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International Journal of
Case Reports and Images
Contents
Vol. 4, No. 12 (December 2013)
Cover Image
Figure 1: (B) Grade 3 clubbing of fingers, and
(C) Bilateral pretibial nodular hyperpigmented,
asymmetrical and non-pitting lesions non-pitting
with plaques between and over the nodules.
Review Article
Cover Figure:
666Effect of Crocus sativus and its active
compounds for the treatment of several
diseases: A review
Rafie Hamidpour, Soheila Hamidpour, Mohsen
Hamidpour, Mina Shahlari
671 Sage: The functional novel natural medicine for
preventing and curing chronic illnesses
Rafie Hamidpour, Soheila Hamidpour, Mohsen
Hamidpour, Mina Shahlari
Case Report
678 Collagenous gastritis: Gastroesophageal reflux
disease in an adult patient without collagenous
colitis
Yousef Usta, Denise Millstine, Fredrick Kenny
682Prostatic ductal adenocarcinoma presenting
with hemospermia
Tafadzwa P Makarawo, Jeffrey L O’Connor
686 Chest X-Ray appearance of total opacification
of the hemithorax following central venous line
insertion: A cautionary tale
Victor Kong, Leah Naidoo, Damon Jeetoo,
George Oosthuizen, Grant Laing, Damian
Clarke
691 Idiopathic double telescoping intussusception
in an adult
Manash Ranjan Sahoo, Manoj Srinivas Gowda,
Raghavendra Mohan Kaladagi
695 Pretibial myxedema mimicking elephantiasis
Pinaki Dutta, Viral N Shah
698 Amphetamine abuse and acute thrombosis of
left circumflex coronary artery
Eyad Khattab, Asaad Shujaa
All Articles:
702 Pseudotumor cerebri caused by uncontrolled
Graves’ disease in an adolescent
Ashutosh Kumar, Sasikumar Kilaikode, Paul
Saenger
705Gastric malignancy presenting as adrenal
insufficiency: A case report
Harsh Vardhan Tevethia, Baskaran S, Tony
Mathew Kadavanu, Riyaz M Panchbhaya,
Sunny DAN, Siva PK
SUBMISSION INSTRUCTIONS
709Talon cusp in primary dentition: A case
report
Ravi Prakash Sasankoti Mohan, Sankalp
Verma, Udita Singh, Neha Agarwal, Soumi
Ghanta, Kuber Tyagi
714
Unique case of dual gynecological
malignancies co-existing with an uncommon
thyroid lymphoma
Ravi Prakash Sasankoti Mohan, Sankalp
Verma, Udita Singh, Neha Agarwal, Soumi
Ghanta, Kuber Tyagi
719 Unexpected
brain
atrophy
following
administration of intratumoral interferon
alpha-2b for cystic craniopharyngioma: A
case report
Khadiga Elfadil Ahmed Mohammed, Kellie
R Alleyne Mike, Jeannette Parkes
723 Cholesterol granuloma of the breast
suspected as breast carcinoma
Takaaki Fujii, Reina Yajima, Hiroki Morita,
Satoru Yamaguchi, Soichi Tsutsumi,
Takayuki Asao, Hiroyuki Kuwano
727 Severely ectatic left circumflex coronary
artery with fistula to coronary sinus
presenting with acute coronary syndrome
Ahmed Sayed
731 Bronchogenic cyst: A case report
Aniruddha Sarkar, Narayan Pandit
735 Reversible
cerebral
vasoconstriction
syndrome in HELLP syndrome
Shinji Katsuragi, Masato Osaki, Rieko
Suzuki, Tomoaki Ikeda, Kazunori Toyoda,
Jun Yoshimatsu
739 Air bubble in the heart: An unrecognized
complication of IV contrast injector
Carlos
Gonzalez,
Dioben
Aquino,
Balavenkatesh Kanna
Case in Images
742 Unexpected Crohn’s Disease after a Free
Gastrointestinal Perforation
René Rodríguez-Gutiérrez, Mario RodarteShade, José Gerardo González-González
745 Hemophilic Arthropathy
Babajide O Olubaniyi, Olubukola Ajala,
David C Howlett
Letter to Editors
748 Plasma cell leukemia of IgG secreting
type: A rare case
Parth Anil Desai, Sagar Dholariya, Sanjeev
Gupta, Tejinder Singh
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IJCRI 2013;4(12):666–670.
www.ijcasereportsandimages.com
Hamidpour et al. review article
666
OPEN ACCESS
Effect of Crocus sativus and its active compounds for the
treatment of several diseases: A review
Rafie Hamidpour, Soheila Hamidpour, Mohsen Hamidpour, Mina Shahlari
Abstract
The purpose of this article is to use literature
search engines such as PubMed and SciFinder
to locate scholarly journal articles pertaining
saffron, its preparation, analysis, and its use
in cancer treatments. Saffron comes from the
dried red stigmas of the Crocus sativus L. flower.
Along with its use in cooking and in traditional
medicine, it has numerous applications as an
antitoxic, anti-oxidant, and anti-cancer agent,
due to its secondary metabolites and their
derivatives are safranal, crocins, crocetin and
dymethyl-crocetin. This study demonstrate
that Crocus sativus extract (CSE) and its major
constituents, crocin, and crocetin, significantly
inhibited the growth of certain cancer cells while
not effecting normal cells. Crocus sativus L.
extract should be investigated further as a viable
agent in the treatment of prostate, pancreatic or
other types of cancer.
Keywords: Crocus sativus L., Tumor inhibitor,
Saffron components, Nutritional supplements
*********
Rafie Hamidpour1, Soheila Hamidpour2, Mohsen Hamidpour3,
Mina Shahlari4
Affiliations: 1PhD, President, Pars Bioscience, Leawood,
Kansas, United States; 2MD, Pars Bioscience, Leawood,
Kansas, United States; 3PhD, Pars Bioscience, Leawood,
Kansas, United States; 4BA, Pars Bioscience, Leawood,
Kansas, United States.
Corresponding Author: Rafie Hamidpour, Pars Bioscience
LLC, 14109 Cambridge Lane, Leawood, Kansas, United
States 66224; Ph: (913) 432-0107; Fax: (913) 432-5708;
Email: rafi@parsbioscience.com
Received: 16 November 2012
Accepted: 12 January 2013
Published: 01 December 2013
Hamidpour R, Hamidpour S, Hamidpour M, Shahlari
M. Effect of Crocus sativus and its active compounds for
the treatment of several diseases: A review. International
Journal of Case Reports and Images 2013;4(12):666–670.
*********
doi:10.5348/ijcri-2013-12-407-RA-1
Introduction
Saffron is one of the most expensive spices in the
world, derived from the dry stigmata of Crocus sativus L.
—a member of the Iridaceae (Iris) family. Saffron is handharvested during the flowering season. This process is
very time consuming which involves picking the stigmata
by hand and then carefully drying the stigmata to produce
a quality product. One stigma of saffron weighs about
2 mg and each flower has three stigmata. In order to
obtain 1 kg of spice 150,000 flowers must be picked [1].
Saffron (Crocus sativus L.) is mostly cultivated
in Spain, Iran, India, Greece, China and some other
European and Asian countries. The quality and chemical
composition of saffron are affected by the region in which
saffron is grown, the drying process, the conditions of
packaging, storage of saffron and the analytical extraction
methods which have been used [1, 2].
The nutritional supplement value of saffron (Crocus
sativus L.) which was provided by pars bioscience LLC
in powder form, to Covance, Madison. WI laboratory
was analyzed and shown to include the following
contents: NL-Proximate (moisture, ash, protein, fat, total
carbohydrates, calories, and calories from fat), results of
these analyzes are given in Table 1. Vitamins (vitamin A,
vitamin C, and folic acid), and minerals (calcium, copper,
iron, magnesium, manganese, phosphorus, potassium,
sodium, and zinc) are detailed in Table 2. The results of
analysis of saffron fatty acid profile are given in Table 3.
The analysis of the saffron was conducted by grinding
and extracting saffron, and analyzing the extract using
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):666–670.
www.ijcasereportsandimages.com
HPLC/UV-MS analysis to identify the major components
of saffron (Figure 1).
To date, the following components have been
identified in saffron: safranal which is the principal
substance responsible for the aroma of saffron, dimethylcrocetin, crocetin esters (cis-crocetin and trans-crocetin),
picrocrocin is the substance responsible for bitter taste
of saffron [1], crocin which are the major components
responsible for the color of saffron, trans-crocin-2, transcrocin-2’, trans-crocin-3, trans crocin-4, cis-crocin-1,
cis-crocin-2, cis-crocin-3, cis-crocin-4, cis-crocin-5,
anthocyanin, carotene, and lycopene [1–10].
The main uses of saffron are in cooking, food coloring,
in perfume and cosmetics [1, 11]. Saffron has also
traditionally been regarded as a highly valued medicinal
plant to treat wide variety of ailments such as depression,
respiratory problems, colds, asthma and heart diseases.
[12]. More recently, as the current culture has been
changing, more researches have been done analyzing the
effects of traditional herbs and spices as treatment for the
severe diseases [11].
Several studies have been performed on the use of
saffron or its constituents in the treatment of a variety
of cancers including colorectal cancer cells (HCT-116,
SW-480, and HT-29) [4], breast cancer cells (MCF7 and MDA-MB-231) [7], non-small cell lung cancer
(NSCLC) cells [4], lung adenocarcinoma cells (A549),
lung fibroblasts cells (WI-38), VA-13 cells (WI-38 cells
transformed in vitro by SV40 tumor virus) [12–14], lung
cancer-bearing mice [15], skin carcinogenesis in mice[16,
17], leukemia cells (HL-60) [4,9], osteosarcoma [4,12],
fibrosarcoma [4, 12], ovarian carcinoma [4, 12], and
cervical epithelioid carcinoma cells (HeLa) [12, 14, 18].
Saffron significantly inhibited the growth of colorectal
cancer cells while not affecting normal cells [4]. Saffron
showed a dose-dependent inhibitory response on breast
cancer cells [7]. Crocetin inhibited the three malignant
human cell lines (HeLa, A549, and VA13) [14]. Overall,
saffron inhibits tumor growth in vivo and in vitro and
could be used for the treatment of cancer, either alone or
in combination with other treatments [10, 19].
Table 1: Nutritional Supplement (NL-Proximate) Analysis of
Saffron.
Analysis
Results
(per 100 g serving size)
Moisture
7.7 g
Ash
4.6 g
Protein
15.6 g
Fat
5.5 g
Total Carbohydrates
69.6 g
Calories
363 cal
Calories from Fat
22.1 cal
Hamidpour et al. 667
Table 2: Nutritional Vitamins and minerals of Saffron
Vitamins
Vitamin A
< 100 IU
Vitamin C
< 1.0 mg
Folic Acid
800 mg
Minerals
-
Calcium
124 mg
Copper
0.908 mg
Iron
23.7 mg
Magnesium
154 mg
Manganese
2.44 mg
Phosphorus
404 mg
Potassium
1750 mg
Sodium
39.0 mg
Zinc
4.15 mg
Table 3: Nutritional Fatty acids of Saffron
Analysis
8:0 Caprylic
10:0 Capric
12:0 Lauric
14:0 Myristic
14:1 Myristoleic
15:0 Pentadecanoic
15:1 Pentadecenoic
16:0 Palmitic
16:1 Palmitoleic
17:0 Heptadecanoic
17:1 Heptadecenoic
18:0 Stearic
18:1 Oleic
18:2 Linoleic
18:3 Gamma Linolenic
18:3 Linolenic
20:0 Arachidic
20:1 Eicosenoic
20:2 Eicosadienoic
20:3 Eicosatrienoic
20:4 Arachidonic
22:0 Behenic
Saturated Fat
Monounsaturated Fat
Polyunsaturated Fat
Sum of Fatty Acids
Results (per 100 g serving
size)
< 0.003 g
< 0.003 g
0.011 g
0.012 g
< 0.003 g
0.003 g
< 0.003 g
0.425 g
0.008 g
0.006 g
< 0.003 g
0.030 g
0.314 g
1.20 g
< 0.003 g
0.394 g
< 0.003 g
0.012 g
0.036 g
< 0.003 g
< 0.003 g
0.008 g
0.471 g
0.321 g
1.56 g
2.46 g
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Figure 1: Chromatogram of the Crocus sativus L. sample
analyzed by ultraviolet at 250 nm.
DISCUSSION
Saffron is a very valuable spice with many traditional
medicinal usages. The high amount of carotenoids in
saffron including crocin, crocetin and dimethyl-crocetin
are responsible for some biological functions of saffron.
Most of the studies on the effect of saffron, indicates
the significant inhibitory effects of the Crocus sativus
components on the synthesis of nucleic acids in different
human cancer cell lines [24].
As the studies have shown, diets rich in antioxidants
will lower the risk of several chronic diseases and protect
the body against the development and growth of tumor
cells. Therefore, saffron and its constituents with their
antioxidant properties can act as a protecting agent for
the prevention of some serious diseases like cancer [25].
Crocus sativus L. extract used in several studies were
prepared from stigmas of Crocus sativus. It contains
several pharmacologically active constituents. Saffron
has antioxidant properties; these have been showed in
humans, where saffron (50 mg, twice a day) decreases
the lipoprotein oxidation susceptibility [20]. Also crude
methanol extract of saffron and its compound crocin
have been exhibited high antioxidant and scavenging
properties [21].
The oral administration of the saffron ethanolic
extract (200 mg/kg body wt) increased the life span of
Swiss albino mice intraperitoneally transplanted with
sarcoma-180 (S-180) cells, Ehrlich ascites carcinoma
(EAC) or Dalton’s lymphoma ascites (DLA) tumors [22],
and it has an inhibitory effect on chemical carcinogenesis
in mice using two stage assay system [16].
Crocetin protects body against free radicals and the
studies have shown its role as an antitumor agent [15]
The effect of crocetin on two different types of animal
tumors, skin papillomas and Rous sarcoma have been
studied and shown that crocetin decreased the number
of tumor cells and delayed the onset of the tumors as
well [23]. A recent study showed that crocetin (20
mg/ kg) reverted the level of lipid peroxidation induced
by Benzo (a) pyrene(B(a)P), also increased the activities
Hamidpour et al. 668
of the enzymic antioxidants and glutathione metabolizing
enzymes. Showing that crocetin is a scavenger of free
radicals and a potent antitumor agent [15].
Crocin inhibits the growth of HeLa cells and
suggested apoptosis induction and showed important
inhibitory effects on skin-tumor initiation and promotion
induced by 7, 12-dimethylbenz[a]anthracene (DMBA)
and
12-0-tetradecanoylphorbol-13-acetate
(TPA),
respectively [18].
Many studies during the last decade, demonstrated the
inhibitory effect of saffron and its components in vitro,
on several cancer types such as carcinoma, leukemia,
prostate, pancreatic, and several other tumor cells [26].
SAFETY: The toxicity of saffron has been studied by
many researchers and the levels of toxicity found to be
very low. The studies showed that the concentration of
0 to 5 g/kg was non-toxic to mice [7].
Also hematological and biochemical studies on the
toxicity of saffron extract indicates that there are no
severe toxicological sign found in kidney, liver or bladder
within the normal range of use [22].
CONCLUSION
In summary, our study suggests that crocin and
crocetin found in Crocus sativus L. may be efficacious
in treating several or certain type of cancer. Considering
the popularity of locally used chemical drugs in cancer
patients, Crocus sativus L. should be investigated further
as a natural viable option in the treatment of prostate,
pancreatic, or other types of cancer.
*********
Author Contributions
Rafie Hamidpour – Conception and design, Analysis
and interpretation of data, Critical revision of the article,
Final approval of the version to be published
Soheila Hamidpour – Analysis and interpretation of
data, Critical revision of the article, Final approval of the
version to be published
Mohsen Hamidpour – Analysis and interpretation of
data, Critical revision of the article, Final approval of the
version to be published
Mina Shahlari – Conception and design, Critical revision
of the article, Final approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Rafie Hamidpour et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
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reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
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Hamidpour et al. review article
671
OPEN ACCESS
Sage: The functional novel natural medicine for preventing
and curing chronic illnesses
Rafie Hamidpour, Soheila Hamidpour, Mohsen Hamidpour,
Mina Shahlari
Abstract
Sage (Salvia) species have been used for centuries,
worldwide as culinary herbs as well as traditional
remedies for the treatment of many common
health complications and their symptoms.
In recent decades, more studies have been
performed on the effect of sage in preventing,
controlling, or curing various diseases naturally
and more safely. A number of sage family members
especially Salvia officinalis (common sage) and
their components have shown to be beneficial in
protecting the body against oxidative stress, free
radical damages, angiogenesis, inflammation,
bacterial and virus infection, etc. Several studies
suggest that sage might potentially provide novel
natural treatments for the relief or cure of many
serious and life-threatening diseases in addition
to treating minor common illnesses such as
depression, dementia, obesity, diabetes, lupus,
autism, heart disease and cancer.
Keywords: Sage (Salvia species),
officinalis, Traditional remedies
Salvia
*********
Rafie Hamidpour1, Soheila Hamidpour2, Mohsen Hamidpour3,
Mina Shahlari4
Affiliations: 1PhD, President, Pars Bioscience, Leawood,
Kansas, United States; 2MD, Pars Bioscience, Leawood,
Kansas, United States; 3PhD, Pars Bioscience, Leawood,
Kansas, United States; 4BA in Biology, Pars Bioscience,
Leawood, Kansas, United States.
Corresponding Author: Rafie Hamidpour, Pars Bioscience
LLC, 14109 Cambridge Lane, Leawood, Kansas, UNITED
STATES. 66224; Ph: (913) 432-0107; Email: rafi@
parsbioscience.com; Fax: (913) 432-5708
Received: 16 November 2013
Accepted: 11 January 2013
Published: 01 December 2013
Hamidpour R, Hamidpour S, Hamidpour M, Shahlari
M. Sage: The functional novel natural medicine for
preventing and curing chronic illnesses. International
Journal of Case Reports and Images 2013;4(12):271–277.
*********
doi:10.5348/ijcri-2013-12-408-RA-2
Introduction
The genus Salvia, commonly called sage, is the largest
member of Lamiaceae or mint family containing over 900
species throughout the world [1, 2]. The plants are mostly
aromatic, perennial, with flowers in different colors
[3]. Many species of Salvia, including Salvia officinalis
(common sage) are native to Mediterranean region and
some of the Salvia species have been used worldwide as
flavoring spices as well as traditional herbal medicine [3, 4].
Sage tea has been traditionally used for the treatment
of digestive and circulation disturbances, bronchitis,
cough, asthma, angina, mouth and throat inflammations,
depression, excessive sweating, skin, and many other
diseases [5–7]. Essential oils of Salvia have been used
in the treatment of large range of diseases such as,
nervous system, heart and blood circulation, respiratory,
digestive, metabolic, and endocrine diseases. In addition,
sage essential oil has shown to have carminative,
antispasmodic, antiseptic and astringent properties [8, 9].
The essential oil of Salvia species can have various
compositions depending on the genetic, climates, season,
and environmental factors [10] (Table 1). There are some
chemical compounds such as flavonoids, terpenoids and
essential oils present in different species of Salvia [3].
Essential oils are very important sources for the screening
of anticancer, antimicrobial, antioxidant, and free radical
scavenging agents [11]. Salvia officinalis is considered to
have the highest amount of essential oil compared to the
other species of Salvia [5, 12].
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IJCRI 2013;4(12):671–677.
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Hamidpour et al. In all analyzed samples of S. officinalis, the major
components, although in different extent are: 1,8-cineole,
camphor, borneol, bornyl acetate, camphene, α-and
β-thujone, linalool, α-and β-caryophyllene, α-humulene,
α- and β-pinene, viridiflorol, pimaradiene, salvianolic
acid, rosmarinic acid, carnosolic acid, ursolic acid,
etc. [7, 12]. Studies have shown that some biological
properties of the essential oil of salvia, depend on
camphor, 1,8-cineole, α-thujone and β-thujone [9].
The essential oil of sage contains about 20% camphor,
and as the leaves expand, the camphor contents also
Table 1: Essential oil composition (% of major components) of
sage Salvia officinalis collected as a sample [5].
Compound1
S. Officinalis2
S. Officinalis3
(1R)- (+)-a-Pinene
3.70
4.50
(-)- Camphene
2.60
5.00
B- Pinene
6.00
5.20
Sabinene
-
0.30
B- Myrcene
3.00
3.50
a-Terpinene
-
0.40
(R)-(+) Limonene
-
-
1, 8 – Cineole
62.0
55.0
Y- Terpinene
0.30
0.50
P-Cymene
0.60
0.60
Terpinolene
-
0.20
(-)-a-Thujone
1.38
1.80
B-Thujone
0.72
1.50
Camphor
8.0
10.0
(-)-Linalool
0.80
0.80
Linalyl acetate
0.60
0.30
(-)-TransCaryophyllene
2.00
1.00
Monoterpene
1.26
1.10
(+)-Menthol
-
-
Borneol
5.00
4.50
a- Terpineol
0.20
-
Geranyl acetate
0.30
-
Geraniol
0.10
0.25
Phytol
0.18
-
Thymol
0.80
0.70
Carvacrol
0.20
0.40
Farnesol
0.20
-
T r a n s - t r a n s - 0.06
Farnesol
0.15
Total Components
30
45
Compounds of essential oil extracted from fresh green leaves
and flowering top.
2
Collected at 100 m above the sea level.
3
Collected at 500 m above the sea level.
1
672
increases [13]. The better radical scavenging activities of
S. officinalis essential oil might be because of the high
content of 1,8-cineole [11].
Sage is also a natural source of flavonoids and
polyphenolic compounds (e.g., carnosic acid, rosmarinic
acid and caffeic acid) possessing strong antioxidant,
radical-scavenging and antibacterial activities [14].
The majority of the phenolic acids in salvia species are
derivatives of caffeic acid which is the building block of
a variety of plant metabolites. Caffeic acid plays a central
role in the biochemistry of the Lamiaceae and occurs
mainly in dimmer form as rosmarinic acid (Figure 1) [15,
16]. Carnosic acid and rosmarinic acid which are present
at high concentration in the extract of sage plants, have
shown strong antioxidant properties [17]. Ursolic acid,
a component of sage, has strong anti-inflammatory
properties and in sage preparations, is considered as a
quality control measurement for the anti-inflammatory
effects of different solutions [18].
Antioxidant: Antioxidants play a very important
role to protect the body against the oxidative stress and
free radical damages which are the cause of various
ailments such as diabetes, heart diseases, cancer, brain
dysfunction, weakened immune system and many more
[19, 20].
In a study done on the antioxidant activity of many plant
extracts, like sage (Salvia officinalis), it was found that the
phenolic and flavonoid compounds are mainly responsible
for the antioxidant and free radical scavenging effect of
these plants [20, 21]. Phenolic compounds such as carnosol,
carnosic and rosmarinic acids, rosmadial, rosmanol,
epirosmanol, methyl carnosate and luteolin-7-O-betaglucopyranoside have a high antioxidative activity and are
usually extracted from sage with ethanol [22]. The phenolic
compounds can either stimulate endogenous antioxidant
defense systems or scavenge reactive species [23].
The antioxidant properties of sage have been studied
intensively, and are found to be related to the presence of
rosmarinic acid and carnosic acid [24, 25]. In addition,
salvianolic acid which is a rosmarinic acid dimmer,
isolated from the sage extract showed a great antioxidant
activity and is a very significant scavenger of free radicals
[25] (Figure 2). The aqueous extract of S. officinalis has
shown to have antioxidant and antiviral effect and in a
study, after drinking of sage tea for two weeks the liver
antioxidant status improved as a result [26].
Memory: Amongst many herbal extracts, Salvia
species are known for the beneficial effects on memory
disorders, depression, and cerebral ischemia [27].
Salvia officinalis, Salvia lavandulaefolia (Spanish
sage), and Salvia miltiorrhiza (Chinese sage) have been
used for centuries as restoratives of lost or declining
mental functions such as Alzheimer’s disease [19, 27].
In Alzheimer’s disease, the enzyme acetyl cholinesterase
(AChE) is responsible for degrading and inactivating
acetylcholine, which is a neurotransmitter substance
involved in the signal transferring between the synapses.
Acetyl cholinesterase inhibitor drugs act by counteracting
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IJCRI 2013;4(12):671–677.
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Figure 1: Danshensu and monoterpenoids from S. miltiorrhiza.
All of these compounds contain catechol functionalities [16].
Figure 2: Salvianolic acids from S. miltiorrhiza. All of these
compounds contain catechol moieties [16].
the acetylcholine deficit and enhancing the acetylcholine
in the brain. Essential oil of Salvia officinalis have
shown to inhibit 46% of acetylcholinesterase activity at a
concentration of 0.5 mg mL-1 [28].
A study shows that Salvia officinalis improves the
memory and cognition, and with the increase of the
dosage, the mood elevation increases as well as alertness,
calmness, and contentedness [29]. A randomized, double
blind clinical study has shown that an ethanolic extract
from S. officinalis and S. lavandulaefolia is effective
in the management of mild to moderate Alzheimer’s
disease, and this study did not show any adverse effects
on patients while taking sage [30, 31]. Administration of
Salvia lavandulaefolia has been reported to be effective
on the improving the speed of memory and mood. Salvia
essential oil also has been reported to improve immediate
word recall [19].
A number of studies have investigated the effects
of the aromas of plant essential oils on cognition and
mood. Salvia officinalis aroma produced a significant
enhancement effect for the quality of memory factor
derived from cognitive drug research system. The findings
suggest that the aromas of essential oils of Salvia species
have some but not all of the effects found following the
oral consumption of the herb [32]. The antioxidant and
anti-inflammatory properties of the S. officinalis or S.
Hamidpour et al. 673
lavandulaefolia may offer a long-term protection in the
pathogenesis of the dementia. Also the mood enhancing
properties of the herb may have applications in the
treatment of advanced dementia, in which disturbed
mood and agitation feature as a major problem. There
is no report of negative side effects associated with
S. officinalis or S. lavandulaefolia despite of many years
of usage span [29].
The cytoprotective effect of sage against Aβ (amyloid
beta plaques) toxicity in neuronal cells also has been
proven by the data presented in a study which provides
the pharmacological basis for the traditional use of sage
in the treatment of Alzheimer’s disease. Rosmarinic
acid as a component of sage has shown neuroprotective,
antioxidative, and anti-apoptotic effects against Aβ
toxicity and this could contribute, at least in part, to the
neuroprotective effect of sage. Therefore, it is possible that
rosmarinic acid, this very low toxic natural compound
could be used as a therapeutic agent in the treatment of
Alzheimer’s disease [31].
Diabetes: Salvia officinalis has been used as a
traditional remedy against diabetes in many countries
and its glucose-lowering affects have been demonstrated
in animal studies. In a study, it was found that methanolic
extracts of S. officinalis significantly decreased
serum glucose in type I diabetic rats without affecting
pancreatic insulin production. An aqueous extract of
Salvia officinalis have been found to exhibit insulin-like
activities [33].
In a study, drinking of sage tea, (300 mL, twice
a day) showed the increase in antioxidant defenses
and improved the lipid profile, without causing any
hepatotoxicity or inducing any adverse affects, such as
changes in blood pressure, heart rate and body weight
which these may indirectly improve the diabetic condition
[23]. Tea-infusions of Salvia officinalis have shown to be
as effective as metformin, which is an oral anti-diabetic
drug used for the treatment of type II diabetes and act by
reducing liver glucose production as well as increases the
action of insulin [33].
Cancer: Cancer is characterized by abnormal growth
of cells which tend to proliferate in an uncontrolled way
and in some cases spread to other parts of the body. The
important factor in proliferating and spreading of cancer
cells is the ability of tumors to produce large number of
new blood vessels, known as angiogenesis. Most primary
solid tumors are dependent on angiogenesis for survival,
growth, invasion and metastasis. In a study, it was
found that Salvia officinalis extract at pharmacological
concentrations inhibits angiogenesis in vivo which could
be a novel starting point for the development of a new
anti-angiogenic drug [34]. Ursolic acid found in sage
effectively inhibits angiogenesis, invasion of tumor cells
and metastasis and suppresses the lung colonization of
B16 melanoma cells in vivo [35].
Colorectal cancer is a common type of cancer and
significant cause of mortality in Western societies. It
develops by genetic and epigenetic alterations which
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IJCRI 2013;4(12):671–677.
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transfer normal colon cells to proliferating cells. The
study has shown that dietary compounds can change the
epigenetic status. Many food plants are rich in bioactive
compounds and have shown to posses anticancer
properties. The effects of sage (Salvia officinalis) herbal
tea drinking were studied on the prevention of colon
cancer in rats. It was found that Salvia officinalis water
extract significantly decreased the oxidative H2O2induced DNA damage in vitro [36].
Some diterpenoids isolated from the roots of S.
officinalis have shown to have cytotoxic and DNAdamaging activity in human colon carcinoma Caco-2 cells
and human hepatoma HepG2 cells in vitro conditions
[10]. The sesquiterpene fraction of S. officinalis with the
presence of α-humulene, demonstrate a strong cytotoxic
activity in human prostate carcinoma LNCaP cells [10].
Also trans-caryophyllene which is main component of
sesquiterpene fraction in Salvia officinalis shows high
cytotoxic activity against the melanotic melanoma and
renal adenocarcinoma cells [8]. Presence of α-humulene
as a component of S. officinalis demonstrated a strong
cytotoxic activity on the human prostate carcinoma
LNCaP cells [8].
Salvia libanotica (Lebanese sage) is one of the
largely used sage species in traditional medicine which
have been used for many years to cure diseases such as
abdominal pains, headaches, indigestions, and heart
disorders [2]. The oil extract of this species was shown
to possess strong antimicrobial and antitumor effects
[2]. The components of Lebanese sage essential oil
was identified by gas chromatography and three of the
components which contains on average, 9.1% Camphor
(Ca), 1.3% α-Terpineol (Te) and 1.1% Linalyl acetate (Ly)
were found to be responsible for the oil antibacterial,
antifungal, anti-inflammatory and antitumor effects [2].
In a study Ly, Te, and Ca synergistically induced cell
cycle arrest and apoptosis resulting in the inhibition of
the growth of human colon cancer cell lines, HCT-116
(P53+/+ and P53-/-) without any effect on the growth of
normal human intestinal cell lines [2].
Cholesterol: The metabolite profile of Salvia
miltiorrhiza is similar to common sage and recently, it
was shown that an extract of Salvia miltiorrhiza was able
to lower plasma cholesterol, LDL, and triglycerides as
well as increase HDL levels in lipidemic rats [33].
The extract of Salvia officinalis is found to activate
peroxisome proliferator-activated receptor gamma
(PPARγ) which is a regulator of genes involved in energy
spending as well as lipid and glucose metabolism and
its activation improves the HDL/LDL ratio and lowers
triglycerides in serum, reduces insulin resistance and
reduces size of adipose (fat) tissue [33].
Extracts from some sage species have been shown to
be effective in the prevention of cardiovascular disease
due to, at least in part, prevention of LDL-C oxidation
[23].
Obesity: Overweight and obesity are recognized to be
important risk factors for type II diabetes, dyslipidemia,
Hamidpour et al. 674
hypertension and many other diseases. To regulate fat
absorption, the effective way is, to reduce body weight
and obesity. Pancreatic lipase is well known to play an
important role in lipid digestion [37]. In several studies
on anti-obese components from natural medicine, the
effect of Salvia officinalis and its active components
in pancreatic lipase activity and lipid digestion were
investigated. The methanolic (MeOH) extract from the
leaves of Salvia officinalis L. significantly inhibited
the pancreatic lipase activity, and suppressed serum
triglyceride (TG) elevation in olive oil-loaded mice.
Carnosic acid and carnosol are two of the diterpenes
isolated from the methanolic extract of the Salvia
officinalis with inhibiting activity on pancreatic lipase.
Carnosic acid also significantly inhibited triglyceride
elevation in olive oil-loaded mice and reduced the gain
of body weight and the accumulation of epididymal fat
weight in high fat diet-fed mice after 14 days. In the course
of several studies on anti-obese components from natural
medicine, the extract of Salvia officinalis leaves showed
inhibitory effect against the pancreatic lipase activity
and eventually was effective to reduce body weight and
obesity [38].
Hot flashes: Menopause is considered as
physiological adjustment process to an altered hormonal
balance [39]. Menopausal symptoms include hot flashes,
insomnia, night-time sweating, dizziness, headaches,
and palpitations. These symptoms reflect adaptation of
the body to estrogen deprivation which affects various
central neurotransmitters.
Sage (Salvia officinalis) has been traditionally used
to treat sweating and menopausal hot flashes as well as
to alleviate associated menopausal symptoms [39]. The
efficacy of sage for the treatment of hot flashes during
menopause was proven by multi center open clinical
trial [6]. A fresh sage preparation demonstrated clinical
value in the treatment of hot flashes and associated
menopausal symptoms. Once-daily application of the
fresh sage extract, demonstrated good clinical value in
terms of safety, efficacy, and tolerability in the treatment
of menopausal hot flashes and climacteric symptoms,
validated by statistical analysis and the clinically relevant
verdict of patients and physicians. The study findings
provide a scientific rationale for sage’s use in folk
medicine, offering a valuable option for patients and
healthcare providers, seeking alternative approaches for
the treatment of menopausal hot flashes and climacteric
complaints [39].
Anti-bacteria: In a study done on the antibacterial
effect of sage against selected food spoiling bacteria in
vitro, indicates that the sage aqueous extract exerted
significant antibacterial activity and it was most effective
against Bacillus mycoids, Bacillus subtilis, Enterobacter
cloacae, and Proteus sp. The work showed that sage
along with different plant extracts was comparable to
synthetic preservatives and the result confirmed that
the aqueous extract of Salvia officinalis can be used in
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IJCRI 2013;4(12):671–677.
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biotechnological field as a natural preservative ingredient
in food industry [26].
The study of antibacterial activities of the essential
oil of Salvia officinalis proved that sage essential oil in
higher concentration exhibited a better efficiency than
antibiotics against bacteria, especially against resistant
bacteria to the antibiotics [5]. This has made sage essential
oil a good alternative to the traditional antibiotics as well
as food preservatives [5].
The finding of a study support the view that the
hydroalcoholic extracts of Salvia officinalis has growth
inhibitory effect on some dental caries causing bacteria,
such as Streptococcus mutans, Lactobacillus rhamnosus
and Actinomyces viscosus. Based on this study, and
increasing interest on using traditional treatments
instead of chemical solutions, Salvia officinalis with
the bactericidal effect could be a natural remedy for the
treatment of mouth and teeth diseases [40].
Anti-diarrhea: Based on the medicinal use of sage
in diarrhea and abdominal spasm, the crude extract of
sage was tested for its anti-diarrheal and antispasmodic
activities using the in-vitro and in-vivo assays. A
study demonstrated that the crude extract provides
protection against diarrhea through inhibitory effect
on gut motility by the presence of some gut relaxant
components [7]. The data in a study suggests that the
crude extract of S. officinalis possess anti-diarrheal and
antispasmodic activities, mediated possibly through
activation of voltage sensitive K+ channels together with
weak Ca++ antagonist effect [7]. Therefore, this study
provides pharmacological basis for the medicinal use
of S. officinalis in hyperactive gut disorders such as
abdominal colic and diarrhea [7].
Saftey: There are no reports of negative side effects
as far we know associated with Salvia lavandulaefolia
(or S. officinalis) despite of their usages for many
centuries [29]. The normal usage of sage is very safe.
However, there might be adverse effects for somebody
using Salvia officinalis in excessive amount, which can
be caused by the high content of thujone [6]. The studies
have shown that Salvia lavandulaefolia compare to
Salvia officinalis has similar compositions without the
thujone content, which makes it more suitable to use for
somebody concerned about the excessive usage of sage
as a treatment [37].
CONCLUSION
Salvia species may represent natural, safe, and
effective treatments for many diseases and their
symptoms. In recent decades, with the increase of
pharmacological knowledge about the beneficial effects of
sage especially Salvia officinalis, these herbal medicines
with antibacterial, antioxidant, anti-inflammatory, free
radical scavenging and antitumor activities, have found
to be very effective in the development of novel natural
drugs to prevent, control and treat many minor health
Hamidpour et al. 675
problems as well as more serious and complicated
diseases such as diabetes, Alzheimer’s and cancer.
*********
Author Contributions
Rafie Hamidpour – Substantial contributions to
conception and design, Analysis and interpretation of
data, Revising it critically for important intellectual
content, Final approval of the version to be published
Soheila Hamidpour – Analysis and interpretation of data,
Revising it critically for important intellectual content,
Final approval of the version to be published
Mohsen Hamidpour – Analysis and interpretation of
data, Revising it critically for important intellectual
content, Final approval of the version to be published
Mina Shahlari – Acquisition of data, Drafting the article,
Final approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Rafie Hamidpour et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
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IJCRI 2013;4(12):678–681.
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Usta et al. CASE REPORT
678
OPEN ACCESS
Collagenous gastritis: Gastroesophageal reflux disease in
an adult patient without collagenous colitis
Yousef Usta, Denise Millstine, Fredrick Kenny
Abstract
Introduction:
Collagenous
gastritis,
first
described in 1989, is a very rare disease with only
about 50 cases ever reported to date. This is a
histologic diagnosis characterized by a thickened
subepithelial collagen bands in the gastric
mucosa. It is associated with an inflammatory
infiltrate with mixed chronic inflammatory
infiltrate of the lamina propria and surface
epithelial damage. We present an adult case
with symptoms of reflux that does not fit the
recent literature that has combined all adult
collagenous gastritis cases with collagenous
colitis and chronic watery diarrhea. Case Report:
A 55-year-old female presented to us with
chronic heart burn. She was clinically diagnosed
with gastroesophageal reflux disease. Upper
endoscopy was done and she was found to have
gastric and gastroesophageal junction collagen
deposits on biopsy. She did not have collagenous
colitis on colonoscopy biopsies or any other
systemic disease and she denied any lower
gastrointestinal symptoms including diarrhea.
All the blood tests performed were normal.
Conclusion: To the best of our knowledge, this
is the first case of collagenous involvement of
the esophageal gastric junction in addition to
Yousef Usta , Denise Millstine , Fredrick Kenny
Affiliations: 1MD, Internal Medicine, Mayo Clinic Scottsdale,
13400 E. Shea Blvd. Scottsdale, AZ 85259; 2DO, Internal
Medicine, St Joseph Hospital and Medical Center, 350 West
Thomas Rd Phoenix, Arizona, 85013; 3MD, Gastroenterology,
St Joseph Hospital and Medical Center, 350 West Thomas
Rd Phoenix, Arizona, 85013.
Corresponding Author: Yousef Usta, 3302 N 7th St Apt 248.
Phoenix Arizona, 85014 USA; Ph: 6023089575; Email:
yosusta@hotmail.com
1
2
Received: 21 March 2013
Accepted: 05 August 2013
Published: 01 December 2013
3
the stomach. It is the first case of collagenous
gastritis in an adult patient presenting with upper
gastrointestinal symptoms without collagenous
colitis, which is usually only seen in pediatric type
disease. Considering the rarity of the disease,
we feel that is still too premature to categorize
collagenous gastritis into two distinct adult type
and pediatric type disease.
Keywords: Collagenous gastritis, Collagenous
colitis,
Gastroesophageal
reflux
disease,
Malabsorption, Diarrhea
*********
Usta Y, Millstine D, Kenny F. Collagenous gastritis:
Gastroesophageal reflux disease in an adult patient
without collagenous colitis. International Journal of Case
Reports and Images 2013;4(12):678–681.
*********
doi:10.5348/ijcri-2013-12-409-CR-3
Introduction
Collagenous gastritis, first described in 1989 [1], is a
very rare disease with only about 50 cases reported to
date [2]. This is a histologic diagnosis characterized by
thickened subepithelial collagen bands in the gastric
mucosa associated with an inflammatory infiltrates [3].
Recent case reports have started grouping cases into two
major groups with distinct clinical presentations seen
in children and adults, and adult type disease [2, 4–6].
The two groups are described as children or young adults
presenting with isolated collagenous gastric disease with
anemia and gastric nodularity on endoscopy and the other
distinct group includes older adults with both collagenous
gastritis (CG) and collagenous colitis (CC) associated
with chronic diarrhea and erythema on colonoscopy [6].
The incidence of CC has increased significantly over time
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):678–681.
www.ijcasereportsandimages.com
and is much more commonly seen than CG. Patients with
CC tend to present with lower gastrointestinal symptoms
including pain, bloating, diarrhea, and malabsorptive
symptoms [7]. Since most adults present with both CC
and CG, there is likely a common factor that causes both
disease processes. We present an adult case that does
not meet the adult type categorization of CG. Our case
is a 55-year-old female who presented with symptoms
of gastroesophageal reflux disease (GERD) and no lower
gastrointestinal symptoms. Our case did not fit the recent
literature that has categorized all adult CG cases with CC,
chronic watery diarrhea, and other systemic diseases.
Considering the rarity of the disease, we feel that it is
still too premature to categorize the patients into these
two distinct groups based on age or upper and lower
gastrointestinal symptoms.
Usta et al. 679
staining. There was fibrosis in the muscularis mucosa
underlying the area of amplified mucosa, and there
was no sign of autoimmune gastritis secondary to the
presence of intact portions of oxyntic mucosa. A section
of the gastroesophageal junction showed similar changes
seen in the stomach. There was an area of fibrosis under
the luminal surface consistent with collagen deposits,
which may be consistent with collagenous esophagitis
CE (Figure 1). She had two colonoscopies performed with
random biopsies to rule out accompanying collagenous
colitis, and all the biopsies showed normal mucosa. There
was no evidence of CC and the colon appeared grossly
normal.
CASE REPORT
Herein, we present a 55-year-old female with a 10year history of reflux and heartburn. Her past medical
history includes iron deficiency anemia that responded
to iron supplements, osteoporosis, and a resected
acoustic neuroma 20 years before. Family history
was negative for gastrointestinal diseases. She denied
odynophagia, nausea, vomiting, or anorexia.
She
denied fever, chills, diarrhea, weight loss, hematochezia,
or melena. She denied aspirin, nonsteroidal antiinflammatory medications, alcohol abuse, or any toxic
chemical ingestion. All laboratory examinations were
normal including antithyroid antibody, antiparietal
cell antibodies, anti tissue transglutaminase, thyroid
stimulating hormone, erythrocyte sedimentation rate,
complete metabolic panel, and complete blood count.
An esophagoduodenoscopy (EGD) was performed
which showed a grossly normal esophagus and duodenum.
Grossly, the stomach had abnormal vascularity and
atrophy of the mucosa with no bleeding. Biopsies
performed on the duodenum showed no evidence of celiac
disease. Histology of the antrum of the stomach showed
evidence of chemical gastropathy. There was villiform
transformation and muscular stranding along mucosal
congestion with no acute or chronic inflammation.
Biopsy results were negative for Helicobacter Pylori, and
there was no evidence of intestinal metaplasia, abnormal
infiltrate, or neoplasia. Biopsies of the body and fundus
of the stomach showed evidence of chronic atrophic
gastritis, patchy collagenous deposits, focal lymphocytic
gastritis, severe mucosal atrophy and fibrosis, and focal
intestinal metaplasia. Some of the tissue showed an
intense lymphoplasmacytic infiltrate in the lamina propria
which in some places completely replaces the glandular
mucosa. There were a very large number of plasma cells
and beta cells seen but the majority of the cells were T
cells. There was marked deposition of collagen beneath
the surface in many of the areas particularly where there
was significant epithelial destruction seen with trichrome
Figure 1: A photomicrograph of a biopsy from gastric
mucosa showing sub-epithelial collagen accumulation
(arrows), lymphocytic inflammation of the epithelium, and
inflammation in the lamina propria (Magnification x100).
DISCUSSION
Our case does not fit the recent literature that
has categorized all adult CG cases with CC including
symptoms of chronic watery diarrhea, and other systemic
diseases such as lymphocytic gastritis, celiac disease,
lymphocytic colitis, and autoimmune disorders [5]. To
date, there is no evidence of an association of CG with
a pan systemic collagenous disease. When comparing
to other described biopsy results of CG, our pathology
was very similar. As seen in our case, the collagenous
deposits showed surface epithelial injury and entrapped
inflammatory cells, and the distribution in the antrum
and body was variably patchy and diffuse [5]. She was
started on omeprazole 40 mg by mouth every twelve
hours for treatment of her GERD symptoms. Although
there seems to be no known or proven therapy for CG,
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):678–681.
www.ijcasereportsandimages.com
our patient’s symptoms were markedly improved with
omeprazole and a low fat and high fiber diet. Repeat
upper endoscopy one year later with surveillance
biopsies showed stable and unchanged CG despite
improvement of her symptoms.
Long-term maintenance therapy with oral
budesonide was found to be efficacious and well
tolerated for preventing relapse in patients with
collagenous colitis [7], but there have also been many
cases documenting spontaneous resolution of CC in
literature [8]. Histological endpoints are difficult to
evaluate because the sub-epithelial collagen deposits
tend to be patchy, so treatment has most often focused
on symptom resolution using anti-inflammatory
medications [8]. Since our patient’s symptoms resolved
on high dose omeprazole, we felt that treating with
steroids would likely be more harmful than beneficial
at this stage in her disease. There is a possibility that
like CC, her CG will spontaneously resolve on its own.
There is no documentation of malignancy associated
with chronic CC and CG to date.
CONCLUSION
To the best of our knowledge, this was the first case
of collagenous involvement of the esophageal gastric
junction in collagenous gastritis. She is the first adult
patient who presented with collagenous gastritis with
upper gastrointestinal symptoms with no manifestations
of collagenous colitis, and no lower gastrointestinal
symptoms including diarrhea which is consistent with
pediatric type categorization of the disease. Considering
the rarity of the disease, we feel that is still too premature
to categorize collagenous gastritis into two distinct adult
type and pediatric type disease. Our patient’s symptoms
resolved with a proton pump inhibitor, but histologically,
our patient showed no improvement on one year
esophagoduodenoscopy biopsy surveillance.
*********
Usta et al. 680
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Yousef Usta et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
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5. Leung ST, Chandan VS, Murray JA, Wu TT. Collagenous
gastritis: Histopathologic features and association with
other gastrointestinal diseases. Am J Surg Pathol 2009
May;33(5):788–98.
6. Lagorce-Pages C, Fabiani B, Bouvier R, Scoazec JY,
Durand L, Flejou JF. Collagenous gastritis: A report of
six cases. Am J Surg Pathol 2001 Sep;25(9):1174–9.
7. Bonderup OK, Hansen JB, Teglbjaerg PS, Christensen
LA, Fallingborg JF. Long-term budesonide treatment
of collagenous colitis: A randomised, double-blind,
placebo-controlled trial. Gut 2009 Jan;58(1):68–72.
8. Freeman HJ. Complications of collagenous colitis.
World J Gastroenterol 2008 Mar 21;14(11):1643–5.
Author Contributions
Yousef Usta – Substantial contributions to conception
and design, Acquisition of data, Analysis and
interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Denise Millstine – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Fredrick Kenny – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):678–681.
www.ijcasereportsandimages.com
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681
IJCRI 2013;4(12):682–685.
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Makarawo et al. CASE REPORT
682
OPEN ACCESS
Prostatic ductal adenocarcinoma presenting with
hemospermia
Tafadzwa P Makarawo, Jeffrey L O’Connor
Abstract
Introduction:
Ductal
(endometrioid)
adenocarcinoma is a rare form of prostate
cancer that most often presents with local
obstructive or irritative symptoms such as acute
urinary obstruction and hematuria, often in the
presence of a normal prostate specific antigen
(PSA) value. Hemospermia is a symptom rarely
associated with prostate malignancy. Therefore,
this was not investigated extensively in the
past, requiring only patient reassurance and
empiric antibiotic treatment. Case Report: A
56-year-old male presented with complaints of
hemospermia. His physical examination was
unremarkable and serum PSA was within normal
limits. He was, therefore, reassured and given a
course of antibiotics. However, due to persistent
hemospermia symptoms, he underwent a
flexible cystoscopy which identified a polypoid
lesion within the verumontanum of the prostatic
urethra, which upon resection was found to be
prostatic ductal adenocarcinoma. Following
an initial period of surveillance, the patient
underwent a radical prostatectomy from which
he made an uneventful recovery and remained
disease-free up to the last follow-up six months
before. Conclusion: Hemospermia is a symptom
largely considered to be benign but in some
Tafadzwa P Makarawo1, Jeffrey L O’Connor1
Affiliations: 1Providence Hospital and Medical Centers,
16001 West Nine Mile, Rd, Southfield, MI.
Corresponding Author: Dr. Tafadzwa P. Makarawo, MD,
Department of Surgery, Providence Hospital and Medical
Centres, 16001 W Nine Mile Road, Southfield, Michigan,
United States, Tel: +1-248-849-3000, Fax: +1-248-8495324; Email: taffyowm@yahoo.com
Received: 05 June 2012
Accepted: 20 August 2012
Published: 01 December 2013
patients it should motivate thorough clinical
evaluation for ductal prostatic adenocarcinoma.
Keywords:
Ductal
adenocarcinoma,
Endometrioid, Hemospermia, Prostate
*********
Makarawo TP, O’Connor JL. Prostatic ductal
adenocarcinoma presenting with hemospermia.
International Journal of Case Reports and Images
2013;4(11):682–685.
*********
doi:10.5348/ijcri-2013-12-410-CR-4
Introduction
Prostatic ductal (endometrioid) adenocarcinoma is
a rare variant of prostate adenocarcinoma presenting
in 0.13–6% of all prostate cancers [1]. As with acinar
prostate adenocarcinoma, ductal adenocarcinoma
affects elderly men over the age of sixty-five. However,
in ductal adenocarcinoma, there exist some important
differences in its presentation. Whereas acinar prostate
adenocarcinoma cases are most often diagnosed through
screening by asymptomatic increases in prostate specific
antigen (PSA), ductal adenocarcinoma is known to
present with local obstructive or irritative symptoms such
as acute urinary obstruction and hematuria, often in the
presence of a normal PSA value [1].
Hemospermia is an uncommonly reported symptom
that historically did not warrant further investigation
at presentation [2]. In recent years, there has been a
growing body of evidence in support of its association
with urological malignancy [3]. We add to this body of
evidence a case of prostatic ductal adenocarcinoma
presenting with hemospermia, with only one other case
describing this association in literature [4].
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):682–685.
www.ijcasereportsandimages.com
Makarawo et al. 683
CASE REPORT
A 56-year-old male was presented with complaints
of hemospermia. His PSA was below 0.1, urinalysis was
negative, and digital rectal examination was normal. He
was, therefore, reassured and given a course of antibiotics.
One month later, his symptoms had not resolved
despite a course of antibiotics. Flexible cystoscopy was,
therefore, performed and revealed no evidence of bladder
malignancy but noted an inflammatory-looking polyp
extending off the right portion of the verumontanum.
A transurethral resection of the abnormality
revealed histological features consistent with ductal
adenocarcinoma (Figures 1 and 2). Specifically, tall,
pseudostratified, columnar cells with abundant cytoplasm
arranged in a predominantly papillary pattern were seen
on H&E stain (Figure 1), with PSA immunohistochemistry
staining verifying the prostatic origin of the malignancy
(Figure 2) and distinguishing it from colorectal cancer
invading the prostate.
Following an initial period of active surveillance
elected by the patient, he eventually underwent a radical
prostatectomy. Final pathological evaluation of the
prostate specimen indicated a focus of Gleason score 7
(4+3) ductal adenocarcinoma with two foci of acinar
adenocarcinoma of Gleason score 8 (4+4). Surgical
margins were negative.
The patient had an uneventful postoperative recovery
and remained disease-free on follow-up visits.
Figure 1: Resected prostatic urethral polyp showing tall,
pseudostratified, columnar cells with abundant cytoplasm
arranged in a predominantly papillary pattern, features
consistent with ductal adenocarcinoma (H&E stain, x400).
DISCUSSION
Ductal adenocarcinoma has unique morphological
and clinical features. Histologically, it is characterized
by the presence of tall, pseudostratified, columnar cells
with abundant cytoplasm arranged in a papillary pattern
[1]. Typically, these lesions grow as exophytic lesions into
the urethra, most commonly around the verumontanum
[1]. The exophytic growth into the urethra accounts for
the clinical presentation of obstruction and hematuria.
Mimickers of ductal adenocarcinoma include prostatic
urethral polyps, hyperplastic benign prostate glands,
High-grade prostatic intraepithelial neoplasia (PIN),
colorectal adenocarcinoma, and papillary urothelial
carcinoma. The diagnosis of ductal adenocarcinomas
is made more challenging by the fact that they may not
always present with an elevated PSA and transrectal
ultrasound biopsy can miss these largely central tumors,
diagnosis often being made incidentally following radical
prostatectomy [1, 5].
Hemospermia is defined as the presence of fresh or
altered blood in the ejaculate [2]. The exact incidence of
hemospermia remains unknown as most ejaculates go
unnoticed during intercourse. The etiology of hemospermia
has been described as largely idiopathic, reported in as
many as 70% of all presenting cases [2], with a variety of
other less common causes described previously (Table 1).
Figure 2: Immunohistochemical stain showing PSA expression,
typical for ductal adenocarcinoma of the prostate (PSA, x400).
Table 1: Etiology of Hemospermia
CongenitalSeminal Vesical/ejaculatory duct cysts
InflammatoryUrethritis, prostatitis, epididymitis,
Cytomegalovirus (CMV), HIV
ObstructionProstatic and Seminal Vesical and ejaculatory
duct calculi, benign
TumorsProstate, Bladder, Seminal vesical, Urethra,
Testis, Epididymis
VascularProstatic varices, prostatic telangiectasia,
hemangioma
TraumaPerineum, testicle, Self-instrumentation,
Transrectal ultrasound biopsy
SystemicHypertension, hemophilia, Purpura, Scurvy,
Bleeding diatheses
Idiopathic
Prostate acinar adenocarcinoma has a documented
association with hemospermia. Recent evidence provided
by our colleagues found that of the screened prostate
cancer patients that presented with hemospermia (0.5%),
13.7% of this cohort of patients was diagnosed with
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):682–685.
www.ijcasereportsandimages.com
prostate cancer [3]. Due to the largely benign nature of
most causes of hemospermia (Table 1) with the majority
resolving spontaneously, it was previously advocated that
hemospermia required no further investigation, requiring
patient reassurance and empiric antibiotic treatment
[6]. However, as this case demonstrates, hemospermia
identified in the elderly population should always raise
the suspicion for malignancy and as such reassurance
should only be provided once extensive evaluation has
excluded such etiologies. Transrectal ultrasound biopsy
followed by magnetic resonance imaging (MRI) or
computed tomography (CT) to further assist in visualizing
the anatomy of pelvic organs are essential components in
the evaluation of these patients [7]. Flexible cystoscopy
has been suggested to be the final step in the evaluation
of hemospermia, being useful in direct visualization of
urethritis, urethral polyps or calculi, vascular anomalies,
foreign bodies and bladder neck varicosities.
We accept that acinar prostate adenocarcinoma has
a reported, though rare, association with hemospermia,
but to our knowledge, this is only the second reported
case in English literature, specifically, describing this
symptom in association with the ductal variant of
prostatic adenocarcinoma.
Our case also reiterates some of the difficulties
associated with diagnosing ductal adenocarcinoma of the
prostate. Physical examination and serum PSA levels were
both non-specific in identifying the cause of the patient’s
symptoms and in the end, the presence of persistent
obstructive urinary symptoms and hemospermia
influenced the decision to perform flexible cystoscopy
earlier in the evaluation, a step that eventually yielded the
diagnostic result.
CONCLUSION
Makarawo et al. 684
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Tafadzwa Makarawo et al. 2013; This article is
distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1.
Epstein JI. Prostatic Ductal Adenocarcinoma: A mini
review. Medical Principles and Practice 2010;19:82–
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2. Szlauer R, Jungwirth A. Hematospermia: Diagnosis
and Treatment. Andrologia 2008;40:120–4.
3. Han M, Brannigan RE, Antenor JA, Roehl KA,
Catalona WJ. Association of hemospermia with
prostate cancer. Journal of Urology 2004;172:2189–
2.
4. Rubinowicz DM, Soloway MS, Lief M, Civantos F.
Hemospermia and expressed tumor in the urethra:
An unusual presentation of ductal carcinoma of the
prostate. Journal of Urology 2000;163:915.
5. Tu SM, Lopez A, Leibovici D, et al. Ductal
Adenocarcinoma of the Prostate. Clinical Features
and Implications After Local Therapy. Cancer
2009;115:2872–80.
6. Kumar P, Kapoor S, Nargund V. Hematospermia- a
systematic review. Annals of the Royal College of
Surgeons of England 2006;88:339–42.
7. Fuse H, Nishio R, Murakami, Okumura A.
Transurethral incision for hematospermia caused by
ejaculatory duct obstruction. Archives of Andrology
2003;49:433–8.
Hemospermia is a symptom largely considered to be
benign but in elderly patients with co-existing urological
symptoms, the increased odds of prostate cancer
detection should motivate thorough clinical evaluation
for ductal prostatic adenocarcinoma.
*********
Author Contributions
Tafadzwa Makarawo – Substantial contributions to
conception and design, Acquisition of data Drafting the
article, Revising it critically for important intellectual
content, Final approval of the version to be published
Jeffrey O’Connor – Substantial contributions to
conception and design, Drafting the article, Final approval
of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):682–685.
www.ijcasereportsandimages.com
Access full text article on
other devices
Makarawo et al. Access PDF of article on
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IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
685
IJCRI 2013;4(12):686–690.
www.ijcasereportsandimages.com
Kong et al. CASE REPORT
686
OPEN ACCESS
Chest X-Ray appearance of total opacification of the
hemithorax following central venous line insertion:
A cautionary tale
Victor Kong, Leah Naidoo, Damon Jeetoo, George Oosthuizen,
Grant Laing, Damian Clarke
Abstract
Introduction: Central venous line insertion
is associated with significant complications.
Procedural complications relating to lung injury
is relatively common, and these may require tube
thoracostomy for management. Although a postprocedure chest X-ray is routinely undertaken in
many centers, erroneous interpretation can lead
to potentially incorrect and unnecessary further
intervention. Case Report: We report a case of
a 25-year-old male who had a central venous
line inserted, with the Chest X-ray appearance
of a massive opacification of the hemithorax
misdiagnosed as a hemothorax and planned tube
thoracostomy. It was subsequently confirmed
as a complete pulmonary collapse caused by a
large mucus plug with obstruction of the main
Victor Kong1, Leah Naidoo2, Damon Jeetoo3, George
Oosthuizen4, Grant Laing5, Damian Clarke5
Affiliations: 1MBChB, Surgical Registrar, Pietermaritzburg
Metropolitan Trauma Service, Department of Surgery,
Edendale Hospital, Pietermaritzburg, KwaZulu Natal, South
Africa; 2MBChB, Surgical House Officer, Pietermaritzburg
Metropolitan Trauma Service, Department of Surgery,
Edendale Hospital, Pietermaritzburg, KwaZulu Natal,
South Africa; 3MBChB, Radiology Registrar, Department of
Radiology, Edendale Hospital, Pietermaritzburg, KwaZulu
Natal, South Africa; 4FCS(SA), Senior Consultant Trauma
Surgeon, Pietermaritzburg Metropolitan Trauma Service,
Department of Surgery, Edendale Hospital, Pietermaritzburg,
KwaZulu Natal, South Africa; 5FCS(SA), Consultant Trauma
Surgeon, Pietermaritzburg Metropolitan Trauma Service,
Department of Surgery, Edendale Hospital, Pietermaritzburg,
KwaZulu Natal, South Africa.
Corresponding Author: Dr Victor Kong, Department of
General Surgery, Edendale Hospital, Edendale Main Road,
Pietermaritzburg, 3200. Email: victorywkong@yahoo.com
Received: 29 November 2012
Accepted: 04 June 2013
Published: 01 December 2013
bronchus. This completely resolved after an
awake flexible bronchoscopic clearance, without
the need for tube thoracostomy. Conclusion:
Whilst Chest X-ray allows identification of most
mechanical complications of central venous line
insertion, accurate interpretation and correct
clinical correlation are absolutely critical in
order to avoid unnecessary interventions.
Keywords: Central venous line, Hemothorax
*********
Kong V, Naidoo L, Jeetoo D, Oosthuizen G, Laing G,
Clarke D. Chest X-Ray appearance of total opacification
of the hemithorax following central venous line insertion:
A cautionary tale. International Journal of Case Reports
and Images 2013;4(12):686–690.
*********
doi:10.5348/ijcri-2013-12-411-CR-5
Introduction
Central venous line insertion is associated
with significant complications which are often
underappreciated [1]. Mechanical complications,
especially pneumothorax, are common, and injuries
to major structures (including the major vessels)
are reported to be as high as 20% [2]. A chest X-ray
post-insertion of a central venous line is commonly
performed [3]. Technical complications need to be
addressed in a timely fashion, but requires correct
interpretation of radiological findings. We report a
case of a 25-year-old male who had misinterpreted post
insertion chest radiography and a tube thoracostomy
was subsequently planned inappropriately by the
junior surgical resident.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):686–690.
www.ijcasereportsandimages.com
Kong et al. 687
CASE REPORT
A 25-year-old male was presented who had a
laparotomy for a gastric perforation following a stab
injury. Due to poor peripheral venous access, a right
subclavian central venous line was inserted by the
anaesthetic resident intra-operatively. However, several
attempts were undertaken due to difficult positioning of
the patient. Ultrasound guidance was not utilised because
the machine was not available due to maintenance. The
central line remained patent and functional, and the
patient remained well throughout the operation. The
surgery was uneventful and he was discharged to the
general ward.
A routine post procedural chest X-ray (Figure 1)
was done immediately, but this was not reviewed by
the surgical resident until 12 hours later. The chest
X-ray appeared to show that the central venous line
entered the right subclavian vein, and then traversed
the brachiocephalic vein before re-entering the left
subclavian vein. There was no pneumothorax or
hemothorax noted. The patient had no complains and
was not in distress. Therefore, it was decided that the
central line should be removed as soon as the patient
could tolerate orally, likely to be the following day.
However, he began complaining of abdominal pain
and increasing dull pain of the right chest throughout
the night. He had poor respiratory effort, refused chest
physiotherapy and was unable to mobilize out of the bed
due to ongoing pain.
On the following morning, he was noted to be in mild
respiratory distress, with a respiratory rate of 24/min,
shallow pattern, SpO2: 90% (FiO2: 100% via a Hudson
mask with reservoir bag) with marked dullness and
reduce air entry on the right chest. His heart rate was
90/min and blood pressure 105/70 mmHg. Laboratory
results were: hemoglobin 11.5 g/dL, white blood cell
15x103/µL. A repeat chest X-ray (Figure 2) showed a
complete opacification of the right hemithorax.
The junior resident who reviewed the chest X-ray
felt it was due to a delayed presentation of iatrogenic
hemothorax related to the insertion of the central
line, and a tube thoracostomy was planned. However,
prior to tube thoracostomy, the patient was eventually
reviewed by the duty attending trauma surgeon who
advised on an emergency computed tomography (CT)
scan of the thorax prior to proceeding. The CT scan
(Figure 3) revealed a complete collapse of the right
lung, with a large mucus plug obstructing the right main
bronchus. An emergency awake flexible bronchoscopy
was performed (under conscious sedation), and a large
amount of mucus was removed using suction. Clinical
condition of the patinent improved immediately. A
subsequent chest X-ray, six hours after the procedure,
showed a complete expansion of the right lung. Following
this, his analgesia regime was further escalated, and he
was treated with aggressive chest physiotherapy, which
was well tolerated. He had an uneventful recovery,
Figure 1: Malpositioned right subclavian line.
Figure 2: Total opacification of the right hemithorax
misdiagnosed as hemothorax or pneumothorax. Note the
tracheal deviation towards the side of opacity, which was
suggestive of volume lost of the right hemithorax and thus
suspicious of atelectasis.
Figure 3: (A, B) Computed tomoraphy scan of thorax depicting
a large mucus plug obstruction of the right main bronchus, with
complete collapse of the right lung. Arrow depicting the location
of the mucus plug.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):686–690.
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did not eventually require a tube thoracostomy, and
subsequently discharged on the sixth day.
DISCUSSION
Central venous line insertion is a common procedure
which is frequently performed by hospital doctors, but
is associated with significant complications. Overall
complication rate varies, but it has been reported to be
as high as 20% [1]. Complications are generally divided
into mechanical, thromboembolic and infectious. Of the
mechanical complications, vascular and lung injuries
(hemothorax, pneumothorax) are the most common [4].
The choice of insertion site, however, is often dictated
by the operator, usually dependent on the individual level
of skills and experience [4]. Malpositioning has been
reported in up to 15%, even when inserted by experienced
clinicians [3]. Current evidence appears to suggest that
the subclavian route is more likely to be associated with
malposition in comparison with the internal jugular route
[1]. However, there is no evidence to suggest any difference
in the incidence of hemothorax, pneumothorax and vessel
occlusion between the two different routes [1]. It is also
important to stress that although free venous back flow
must be present after insertion, it does not absolutely
exclude malpositioning or even subsequent migration
[5]. Whilst insertion under ultrasound guidance has
become increasingly common, the traditional anatomical
landmark technique continues to be used frequently [6],
especially in developing countries where the resources
and facilities may be limited.
Death following significant subclavian artery injury
has been reported [7]. The artery is particularly vulnerable
due to its close proximity to the subclavian vein.
Furthermore, this complication appears to be directly
proportional to the number of attempts undertaken. More
than three attempts are associated with a complication
rate of approximately 50% [8].
Several important issues are highlighted in this unusual
case. Firstly, the initial placement position was clearly
incorrect, but this was only recognised 12 hours after
the insertion. Also, because the line appeared functional,
this initially gave a false sense of security. Although some
literatures suggest that chest radiography may not be
absolutely necessary in all cases, especially when inserted
by experienced operators, most clinicians continue to do
so, especially when difficulties are encountered or when
multiple attempts have been made [3]. In the context of
central line insertion, the appearance of pneumothorax
or hemothorax on the chest X-ray is usually obvious,
but erroneous interpretation (as in this case) almost
leads to unnecessary further procedures (such as tube
thoracostomy) being carried out. Whilst the appearance
of total opacification of the hemithorax could be caused
by hemothorax (secondary to vascular injury), another
important diagnosis to consider is complete pulmonary
Kong et al. 688
collapse, which has a number of causes. In this case, the
patient remained hemodynamically stable for more than
24 hours after the original procedure (with hemoglobin
of 11.5 g/dL), with predominantly respiratory symptoms,
makes the diagnosis unlikely. The importance of accurate
interpretation of radiograph and the integration of these
findings within the clinical context cannot be overemphasized. Erroneous interpretation and failure to
recognize alternative diagnoses could have potentially led
to a disastrous situation. It is important to re-emphasize
that one has to remain opened to alternative diagnoses
and constantly question oneself if the findings do not add
up to the clinical picture.
Secondly, tube thoracostomy is usually reserved for
the treatment of significant symptomatic hemothorax
and pneumothorax. Given the significance of the patient’s
symptomatology, it was probably reasonable for the junior
resident to consider this as an option. However, tube
thoracostomy itself is invasive which is also associated
with appreciable morbidity, and even mortality [9].
Therefore, it should only be performed if absolutely
necessary. In our case, even if tube thoracostomy had
been performed based on the initial suspicion of a
hemothorax, it would have been highly unlikely to have
resolved the situation appropriately.
Finally, postoperative pulmonary complications are
common and may occur in up to 20% of all emergency
surgical patients [10]. Pulmonary collapse (atelectasis)
is one of the most common complications following
major abdominal surgery [10]. While numerous factors
contribute the development of pulmonary collapse, it is
generally agreed that the major precipitating factors are
limited chest movement and bronchial obstruction caused
by a thick mucus plug [11]. Suboptimal analgesia, poor
pulmonary toileting, and immobility were the most likely
contributory factors in this case. These were not recognised
early and contributed to the progressive deterioration
in this patient. This was subsequently recognized prior
to embarking on tube thoracostomy an appropriate
definitive intervention was performed, albeit somewhat
delayed. Earlier recognition and preventative measures
would probably have enabled the avoidance of a potentially
dangerous situation.
CONCLUSION
Central venous line insertion is associated with
significant morbidity, and is often under-appreciated.
Although vascular and lung injuries are relatively
common, chest radiographic findings may be easily
misinterpreted. Whilst clinicians must always remain
vigilant of potential complications, it is equally
important to remain open to alternative diagnoses,
especially if the overall clinical picture does not seem
to fit together.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):686–690.
www.ijcasereportsandimages.com
*********
Author Contributions
Victor Kong – Substantial contributions to conception and
design, Acquisition of data, Analysis and interpretation
of data, Drafting the article, Revising it critically for
important intellectual content, Final approval of the
version to be published
Leah Naidoo – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Damon Jeetoo – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
George Oosthuizen – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Grant Laing – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Damian Clarke – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Kong et al. vein: Is postprocedural chest radiography always
necessary? Crit Care Med 1999 Sep;27(9):1819–23.
4. McGee DC, Gould MK. Preventing complications of
central venous catheterization. N Engl J Med 2003
Mar 20;348(12):1123–3.
5. Tocino IM, Watanabe A. Impending catheter
perforation of superior vena cava: Radiographic
recognition. AJR Am J Roentgenol 1986
Mar;146(3):487–90.
6. Mehta N, Valesky WW, Guy A, Sinert R. Systematic
review: Is real-time ultrasonic-guided central line
placement by ED physicians more successful than the
traditional landmark approach? Emerg Med J 2013
May;30(5):355–9.
7. Deogaonkar K, Shokrollahi K, Dickson WA.
Haemothorax: A potentially fatal complication of
subclavian cannulation--A case report. Resuscitation
2007 Jan;72(1):161–3.
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Coussaye JE, Eledjam JJ. When subclavian vein
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pulmonary complications following abdominal
surgery. Chest 1997 Mar;111(3):564–71.
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Victor Kong et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
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2002 Feb;30(2):454–60.
Mansfield PF, Hohn DC, Fornage BD, Gregurich MA,
Ota DM. Complications and failures of subclavianvein catheterization. N Engl J Med 1994 Dec
29;331(26):1735–8.
Gladwin MT, Slonim A, Landucci DL, Gutierrez DC,
Cunnion RE. Cannulation of the internal jugular
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Sahoo et al. CASE REPORT
691
OPEN ACCESS
Idiopathic double telescoping intussusception in an adult
Manash Ranjan Sahoo, Manoj Srinivas Gowda,
Raghavendra Mohan Kaladagi
Abstract
Introduction: Intussusception is uncommon in
adults compared with the pediatric population.
Unlike its pediatric counterpart, intussusception
in adults is associated with obvious pathology.
Small bowel intussusceptions are much less
common than the ileocolic type, with jejunoileal
intussusceptions being amongst the rarest.
There are no reports of double intussusception
in literature. We report a first case of double
telescoping intussusception in an adult where the
proximal ileum (intussusceptum) is telescoping
into distal ileum (intussuscipiens) and this as a
whole is again telescoping into terminal ileum.
Case Report: A 46-year-old woman came to the
emergency ward with a three-day history of pain
abdomen, distension and multiple episodes of
bilious vomiting. The abdomen was distended
and there was rebound tenderness on palpation.
There was a palpable mass just below the
umbilicus. Plain X-ray abdomen showed multiple
air fluid levels. Ultrasonography of abdomen
showed dilated bowel loops, with target sign
giving an impression of ileoileal intussusception.
Intraoperatively double telescoping ileoileal
intussusception was present one foot proximal to
Sahoo Manash Ranjan1, Gowda Manoj Srinivas2, Kaladagi
Raghavendra Mohan2
Affiliations: 1MBBS, MS (General Surgery), Associate
Professor, Department of General Surgery, SCB Medical
college and Hospital, Cuttack, Odisha, India; 2MBBS, MS
(General Surgery), Post Graduate, Department of General
Surgery, SCB Medical college and Hospital, Cuttack,
Odisha, India.
Corresponding Author: Manash Ranjan Sahoo, Associate
Professor Surgery, SCB Medical College and Hospital,
Cuttack, Odisha, India - 753007; Ph: +919937025779;
Email: manash67@gmail.com
Received: 20 February 2013
Accepted: 25 April 2013
Published: 01 December 2013
ileocecal junction. Resection of the ileal segment
bearing the intussusception with end ileostomy
was done. Conclusion: Intussusception in adults
is very rare and has an identifiable cause in 80%
of cases. Double telescoping intussusception
is the condition where there will be a proximal
segment intussuscepting in to the distal segment
and this whole thing will again intussuscept into
the still farther segment of intestine.
Keywords:
Intussusception,
Intussusceptum, Intussuscipiens
Laparotomy,
*********
Sahoo MR, Gowda MS, Kaladagi RM. Idiopathic double
telescoping intussusception in an adult. International
Journal of Case Reports and Images 2013;4(12):691–694.
*********
doi:10.5348/ijcri-2013-12-412-CR-6
Introduction
Intussusception was first reported in 1674 by Barbette
from Amsterdam and further presented in a detailed
report in 1789 by John Hunter as ‘introssusception’.
Intussusception is defined as the telescoping of a
segment of the gastrointestinal tract (intussusceptum)
into an adjacent one (intussuscipiens). This may lead
to intestinal obstruction and loss of blood supply to
the area. More rarely, the intussusceptum may become
strangulated, necrotic, and gangrenous and lead to
sepsis or death. Intussusception is uncommon in adults
compared with the pediatric population. It is estimated
that only 5% of all intussusceptions occur in adults and
approximately 5% of bowel obstructions in adults are the
result of intussusception [1]. Clinically, intussusception
in adults often presents with nonspecific symptoms such
as abdominal pain, nausea, diarrhea, and rectal bleeding.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
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Sahoo et al. 692
The classical triad of symptoms seen in children of
sausage-shaped palpable mass, red currant jelly stools,
and acute abdominal pain is less often seen in adults
[2, 3]. In contrast to childhood intussusception, which
is idiopathic in 90% of cases, adult intussusception has
a demonstrable lead point, which is a well-definable
pathological abnormality in 70–90% of cases [2–5]. Since
90% of pediatric cases are due to unidentifiable cause, only
pneumatic or hydrostatic reduction of intussusception
is sufficient. In contrast, adult intussusception needs
surgical intervention due to associated pathologic lead
point.
There is no report of double telescoping
intussusception in literature. We report the first case of
double telescoping intussusception in an adult without
any obvious pathology.
CASE REPORT
A 46-year-old woman came to the emergency ward
with a three-day history of abdomen pain, distension
and multiple episodes of bilious vomiting. She also
had complaint of constipation for last two days. There
was no significant past medical history. On physical
examination, she had tachycardia and hypotension;
temperature was normal. The abdomen was distended
and there was rebound tenderness on palpation. There
was a palpable mass just below the umbilicus. Laboratory
investigations showed leukocytosis. Liver function tests
were normal. Plain X-ray abdomen showed multiple air
fluid levels. Ultrasonography of abdomen showed dilated
bowel loops, with target sign giving an impression of ileoileal intussusception. With all these findings suggestive
of acute intestinal obstruction, patient was planned for
urgent exploratory laparotomy. Intraoperatively double
telescoping ileoileal intussusception was present one
foot proximal to ileocecal junction (Figure 1). The bowel
proximal to this area was dilated and the intussusceptum
was gangrenous. There was no intramural mass or any
other type of lead point of intussusceptions (Figure 2).
There was no free fluid and inter bowel adhesions. The
resection of the ileal segment bearing the intussusception
with end ileostomy was done. Even though primary
anastomosis is the standard after resection in this case,
end ileostomy was done due to poor general condition
of the patient and gangrenous bowel segment. The
histopathological study of the specimen did not reveal
any pathology. Patient recovered well in postoperative
period and was discharged on 10th postoperative day.
Patient was doing well during follow-up, and after three
months ileo-ascending anastomosis was done.
DISCUSSION
Adult intussusception is an uncommon clinical
entity encountered by surgeons. Common sites of
Figure 1: Resected specimen showing double telescoping
intussusception.
Figure 2: Cut section of the resected specimen.
intussusception are the junctions of mobile and immobile
bowel segments, i.e., ileocolic, rectosigmoid junction [6].
Intussusception in adults affects mainly the small
intestine (52–55%), while the colon is affected in 38–45%
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):691–694.
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of cases [7]. Various methods have been applied in the
classification of intussusception. Based on location, it has
been classified into four categories:
(i) entero-enteric, confined to the small bowel,
(ii) colocolic, involving the large bowel only,
(iii) ileocolic, defined as the prolapse of the terminal
ileum within the ascending colon and
(iv) ileocecal, where the ileocecal valve is the leading
point of the intussusception [7, 8].
In addition adult intussusception has been classified
based on etiology as idiopathic, benign and malignant. In
the small intestine, an intussusception can be secondary
either to the presence of intra- or extra-luminal lesions
[9]. Only 30% of cases of small bowel intussusceptions
are due to malignancy, where as 66% of large bowel
intussusceptions have malignant etiology [4]. Although
our patient presented with an ileoileal intussusception,
no extra- or intra-luminal lesion was seen. This atypical
presentation is seen in 8–20% of cases [7]. The exact
mechanism of the development of intussusception is not
well defined in these cases.
On the other hand, in secondary intussusception
the lead point will alter normal peristalsis and initiate
an invagination of one segment of bowel in to other [4,
10]. Usually, proximal segment telescopes into distal
segment to cause intussusception. There are no reports
of double telescoping in literature where the proximal
segment intussuscept into the distal segment and this
whole thing will again intussuscept into the still farther
segment of intestine. Intussusception leads to bowel
obstruction and inflammatory changes ranging from
thickening to ischemia of the bowel wall. This thickening
or ischemia of the intussusceptum might have acted as
the lead point for the proximal segment to intussuscept
again in to the distal segment causing double telescoping
intussusception. This is a case of an idiopathic type of
ileoileal double telescoping intussusception which as per
our knowledge has not been reported earlier.
CONCLUSION
Intussusception in adults is very rare and has an
identifiable cause in 80% of cases. We report an unusual
cause of small bowel obstruction in an adult patient,
secondary to double telescoping intussusception with
no lead point. Double telescoping intussusception is
the condition where there will be a proximal segment
intussuscepting into the distal segment and this whole
thing will again intussuscept into the still farther segment
of intestine. Since most cases are secondary to lead point
surgery remains the mainstay of management.
*********
Author Contributions
Sahoo et al. 693
Drafting the article, Critical revision of the article, Final
approval of the version to be published
Manoj Srinivas Gowda – Conception and design,
Acquisition of data, Analysis and interpretation of data,
Drafting the article, Critical revision of the article, Final
approval of the version to be published
Raghavendra Mohan Kaladagi – Acquisition of data,
Drafting the article, Critical revision of the article, Final
approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Manash Ranjan Sahoo et al. 2013; This article is
distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1. Zubaidi A, Al-Saif F, Silverman R. Adult
intussusception: A retrospective review. Dis Colon
Rectum 2006 Oct;49(10):1546–1.
2. Azar T, Berger DL. Adult intussusception. Ann Surg
1997 Aug;226(2):134–8.
3. Eisen LK, Cunningham JD, Aufses AH Jr.
Intussusception in adults: Institutional review. J Am
Coll Surg 1999 Apr;188(4):390–5.
4. Begos DG, Sandor A, Modlin IM. The diagnosis and
management of adult intussusception. Am J Surg
1997 Feb;173(2):88–94.
5. Weilbaecher D, Bolin JA, Hearn D, Ogden W 2nd.
Intussusception in adults. Review of 160 cases. Am J
Surg 1971 May;121(5):531–5.
6. Sachs M, Encke A. Entero-enteral invagination of the
small intestine in adults. A rare cause of “uncertain
abdomen”. Langenbecks Arch Chir 1993;378(5):288–
91.
7. Schiud F, van Gansbeke D, Ansey J. Intussusception
in adults--report of 3 cases. Acta Chir Belg 1985 JanFeb;85(1):55–60.
8. Nagorney DM, Sarr MG, McIlrath DC. Surgical
management of intussusception in the adult. Ann
Surg 1981 Feb;193(2):230–6.
9. Ishii M, Teramoto S, Yakabe M, et al. Small intestinal
intussusceptions caused by percutaneous endoscopic
jejunostomy tube placement. J Am Geriatr Soc 2007
Dec;55(12):2093–4.
10. Takeuchi K, Tsuzuki Y, Ando T, et al. The diagnosis
and treatment of adult intussusception. J Clin
Gastroenterol 2003 Jan;36(1):18–21.
Manash Ranjan Sahoo – Conception and design,
Acquisition of data, Analysis and interpretation of data,
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694
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Dutta et al. 695
CASE reportOPEN ACCESS
Pretibial myxedema mimicking elephantiasis
Pinaki Dutta, Viral N Shah
Abstract
Introduction: Elephantiasic pretibial myxedema
is a rare manifestation of autoimmune
thyroid disease. Herein, we report a case of
elephantiasic pretibial myxedema. Case Report:
A 45-year-old male presented with bilateral firm
confluent pretibial polypoid hyperpigmented
nodular and plaque lesions. He had a history of
thyroidectomy for toxic goitre in the past and
was not on any thyroxine replacement therapy.
On examination, he had dry skin, myxedematous
appearance and grade 3 clubbing of fingers.
Investigations
revealed
elevated
thyroid
stimulating hormone and thyroid stimulating
hormone receptor stimulating antibody levels.
Histopathology of the skin lesion was suggestive
of pretibial myxedema.
The patient was
started with thyroxine replacement and steroid
occlusive dressing over lesions. His condition
was improved remarkably with treatment.
Conclusion: Elephantiasic pretibial myxedema
is rare. This condition should be recognized
promptly and should be treated with steroid,
Pinaki Dutta1, Viral N Shah2
Affiliations: 1DM, Assistant Professor, Department of
Endocrinology, 4th Floor, F Block, Nehru Hospital, Post
Graduate Institute of Medical Education and Research,
Sector 12, Chandigarh, India; 2DM, Department of
Endocrinology, Post Graduate Institute of Medical Education
and Research, Sector-12, Chandigarh, India.
Corresponding Author: Dr. Pinaki Dutta, DM, Assistant
Professor, Department of Endocrinology, 4th Floor, F
Block, Nehru Hospital, Post Graduate Institute of Medical
Education and Research, Sector 12, Chandigarh, India 160012; Ph: +91-172-2756584; +91-935711477; Fax: +910172-2747944; Email: pinaki_dutta@hotmail.com
Received: 08 February 2013
Accepted: 27 April 2013
Published: 01 December 2013
occlusive dressing and treatment of underlying
thyroid disease.
Keywords: Pretibial myxedema, Graves’ disease,
Thyroid stimulating hormone receptor antibody,
Hypothyroidism
*********
Dutta P, Shah VN. Pretibial myxedema mimicking
elephantiasis. International Journal of Case Reports
and Images 2013;4(12):695–697.
*********
doi:10.5348/ijcri-2013-12-413-CR-7
Introduction
Pretibial myxedema (PTM) is an immune mediated
localized form of thyroid associated dermopathy often
found in patients with autoimmune thyroid disease,
particularly Graves’ diseases [1]. Rarely, it is also
seen in patients with hypothyroidism [2]. The skin
lesions are diffuse, non-pitting in majority. However,
plaques and nodular forms have also been describe in
literature. Elephantiasis form of (PTM) which is known
as elephantiasis nostra verrucosa (ENV) is extreme rare
form which is found late in the course of Graves’ disease
[3]. We describe a case who remained hypothyroid for a
long period of time, eventually developed elephantiasic
PTM.
CASE REPORT
A 45-year-old male presented with large irregular
nodular swelling over anterior aspects of tibia bilaterally.
These swellings were gradually increasing in size for past
two years. He had a history of thyroidectomy for large
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):695–697.
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toxic goitre seven years back in an outside hospital. He
was not on thyroxin replacement therapy and was never
on follow-up. He neither lived nor had any visit to filarial
endemic area. On examination, he had dry skin, sallow
complexion and periorbital puffiness (Figure 1A). He also
had grade 3 clubbing of fingers (Figure 1B). The bilateral
firm confluent pretibial polypoid nodular lesions were
hyperpigmented, firm, asymmetric and non-pitting
and there were plaques between and over the nodules
(Figure 1C). His pulse was 64 per minute and regular
rhythm and blood pressure was 130/84 mmHg. The
thyroid function test revealed severe hypothyroidism,
his TSH level was more than 100 mIU/mL (normal
limit 0.4–4.4 mIU/mL). Blood examination did not
reveal the presence of filarial parasite and furthermore,
filarial antigen and antibody tests were negative. Thyroid
stimulating receptor antibody titer was 40IU/L (normal
limits <1.5 IU/L). Biopsy of one of the nodular skin
lesions revealed separation of collagen fibers, extensive
deposition and stellate fibroblasts consistent with the
findings of pretibial myxedema (Figure 2). He was started
with 125 µg/day of thyroxine and steroid ointment with
tight occlusive bandage for pretibial swelling. After three
Figure 1: (A) Showing dry skin, sallow complexion and
periorbital puffiness, (B) Grade 3 clubbing of fingers, and (C)
Bilateral pretibial nodular hyperpigmented, asymmetrical and
non-pitting lesions non-pitting with plaques between and over
the nodules.
Dutta et al. 696
months of therapy, there was regression of symptoms of
hypothyroidism and modest decrease in nodular pretibial
myxedema. Currently, he is planned for intralesional
glucocorticoids.
DISCUSSION
Pretibial myxedema is an infrequent manifestation
of autoimmune thyroid disease as a result of immune
mediated phenomenon [1]. About 0.5–4.3% of patients
with a history of thyrotoxicosis have thyroid dermopathy,
and 15% of patients with severe Graves’ ophthalmopathy
have this cutaneous manifestation [4]. According to
one study, PTM was also found with frequency of 17.1%
in Hashimoto’s thyroiditis, and 6.5% in idiopathic
hypothyroidism [5]. The elephantiasic pretibial
myxedema constitutes less than 1% of PTM. Our patient
had toxic nodular goitre and underwent thyroidectomy,
subsequent hypothyroidism and eventually developed
PTM.
Classically, histopathology of these lesions shows
increase in levels of glycosaminoglycan (GAG) in the
reticular dermis with excess increase in hyaluronic acid
concentration [6]. The proposed mechanism for this is
thought to be due to fibroblast stimulation by thyroidstimulating hormone receptor (TSH-R) antibodies [6,
7]. Histopathological findings in our case is same as
describe in literature and the level of TSH-R stimulating
antibodies was also elevated suggesting the role of TSH-R
stimulating antibodies in fibroblast proliferation and
synthesis of GAG.
The most commonly describe therapy for PTM is
tight occlusive steroid dressing [6] which has also been
given in our case. The response rate varies with topical
steroid occlusive dressing is 26-50%. Our case responded
dramatically with local steroid as well as thyroxine
replacement.
CONCLUSION
Elephantiasic pretibial myxedema is a rare disease.
This condition should be recognized promptly and should
be treated with steroid occlusive dressing and treatment
of underlying thyroid disease.
ACKNOWLEDGEMENTS
We are grateful to Department of Photography
(Mr. Brij Lal and Mr. Abijeet) for the taking patient
photographs. We also thank Mr. Prakamya Gupta for
manuscript editing.
Figure 2: Biopsy of one of the nodular skin lesions showing
separation of collagen fibers, extensive deposition and stellate
fibroblasts (H&E stain, x400).
*********
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):695–697.
www.ijcasereportsandimages.com
Author Contributions
Pinaki Dutta – Substantial contributions to conception
and design, Acquisition of data, Analysis and
interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Viral N Shah – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Pinaki Dutta et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
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Dutta et al. 697
REFERENCES
1. Beierwaltes WH. Clinical correlation of pretibial
myxedema with malignant exophthalmos. Ann Intern
Med 1954 May;40(5):968–84.
2. Reed Larsen, P, Davies TF, Hay IA. The thyroid gland
in Williams Textbook of Endocrinology 9th Edition
WB Saunders 1998:390–498.
3. Humbert P, Dupond JL, Carbillet JP. Pretibial
myxedema: An overlapping clinical manifestation of
autoimmune thyroid disease. The American journal
of medicine 1987 Dec;83(6):1170–1.
4. Kriss JP. Pathogenesis and treatment of pretibial
myxedema. Endocrinol Metab Clin North Am 1987
Jun;16(2):409–15.
5. Salvi M, De Chiara F, Gardini E, et al.
Echocardiographic diagnosis of pretibial myxedema
in patients with autoimmune thyroid disease. Eur J
Endocrinol 1994 Aug;131(2):113–9.
6. Schwartz KM, Fatourechi V, Ahmed DD, Pond GR.
Dermopathy of Graves’ disease (pretibial myxedema):
Long-term outcome. J Clin Endocrinol Metab 2002
Feb;87(2):438–6.
7. Fatourechi V, Ahmed DD, Schwartz KM. Thyroid
acropachy: Report of 40 patients treated at a single
institution in a 26-year period. J Clin Endocrinol
Metab 2002 Dec;87(12):5435–41.
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IJCRI 2013;4(12):698–701.
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Khattab et al. CASE REPORT
698
OPEN ACCESS
Amphetamine abuse and acute thrombosis of left
circumflex coronary artery
Eyad Khattab, Asaad Shujaa
Abstract
Introduction:
Currently,
amphetamine
abuse is a widespread problem. While the
use of recreational drugs is more common in
young populations, drug abuse within elderly
populations is increasing, though largely
unrecognized. The exact nature and dose of
recreational drugs are difficult to identify when
a patient presents requiring urgent care, notably
amphetamine-induced acute coronary syndrome
(ACS). Case Report: A 54-year-old male smoker
presented with classical anginal chest pain after
using amphetamines. Electrocardiography (ECG)
showed T-wave inversion in leads I, aVL, and
V4-V6. Laboratory data revealed total creatine
kinase (CK) level 1759 IU/L, CK-MB 87 IU/L,
and a troponin-I of 13.38 ng/mL. A diagnosis
of non-ST elevation myocardial infarction
(NSTEMI) was made. The emergency room
physician commenced the NSTEMI protocol,
but the patient experienced worsening chest
pain unresponsive to nitrates or morphine, with
no change in the ECG pattern. The cardiologist
decided to proceed with percutaneous coronary
intervention. Coronary angiography revealed
100% stenosis in the proximal portion of the left
circumflex artery, with a large clot burden. The
artery was stented with excellent results. Later
testing showed a positive urine toxicology screen
Eyad Khattab , Asaad Shujaa
Affiliations: 1MD, MPH, Demonstrator, Department of
Emergency Medicine, King Khalid University Hospital, King
Saud University, Riyadh, Saudi Arabia. Resident, Department
of Internal Medicine, University of Toledo Medical Center,
Toledo, Ohio, United States of America.
Corresponding Author: Eyad Khattab, P.O. Box 7805,
Riyadh, SAUDI ARABIA 11472; Ph:+966-503327350; Fax:
+966-14672439; Email: eyad.khattab@yahoo.com
1
2
Received: 12 April 2013
Accepted: 04 May 2013
Published: 01 December 2013
for amphetamines. Conclusion: Regardless of
the age or gender of the patient, asking questions
about stimulant drugs use is crucial with anginal
chest pain presentation. Drug abuse is not only for
young males but older males and females are also
susceptible, and can have unique responses to
amphetamine abuse. In this case, amphetamine
abuse caused myocardial ischemia due to blood
clot formation.
Keywords: Amphetamine, Left Circumflex
Coronary Artery, Non-ST elevation myocardial
infraction (NSTEMI)
*********
Khattab E, Shujaa A. Amphetamine abuse and
acute thrombosis of left circumflex coronary artery.
International Journal of Case Reports and Images
2013;4(12):698–701.
*********
doi:10.5348/ijcri-2013-12-414-CR-8
Introduction
Currently, amphetamine abuse is a universal problem.
Drug abuse is more common in young population.
However, the use of recreational drugs by elderly
populations is largely unrecognized and ignored, but
increasing [1]. The prevalence of illicit drug use in general
populations is difficult to identify due to the covert nature
of the activity, especially in one of the most conservative
societies, Saudi Arabia, which is tightly surrounded by
tradition and religious conservatism. Furthermore, the
exact nature and dose of recreational drugs are difficult to
establish when a patient presents need urgent care.
As the name suggests, sympathomimetic drugs such
as amphetamine work by activating the sympathetic
nervous system. As a result, heart rate increases and the
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):698–701.
www.ijcasereportsandimages.com
oxygen supply decreases, causing the oxygen demand of
the heart to increase. Serious cardiovascular problems are
more common among male users than female, although
amphetamine-induced acute coronary syndrome (ACS)
is an infrequent occurrence. Acute occlusion of the left
circumflex artery (LCX) is complicated to diagnose.
Patients having this condition rarely present with
STEMI and are not likely to have percutaneous coronary
intervention (PCI) [2]. A review of 220 articles reported
the activation of the sympathetic nervous system because
of amphetamine use can cause myocardial infarction
(MI) secondary to coronary artery spasm, and concluded
the induction of coronary vasospasm was the likely
underlying mechanism of amphetamine-related acute
MI [3]. The blood supply to the heart occurs during the
diastolic phase of the cardiac cycle unlike other body
organs that get their blood supply during the systolic
phase of the heart cycle [4]. Moreover, amphetamine is
known to increase blood pressure, platelet aggregation,
and trigger atherosclerotic plaque rupture [5].
Khattab et al. 699
creatine kinase (CK) level 1759 IU/L, CK-MB level 87U/L,
and troponin-I level 13.38 ng/mL, a urine toxic screening
test was positive for amphetamine.
Based on the history, physical examinations and
investigations, the diagnosis of non-ST elevation
myocardial infarction (NSTEMI) was made. The patient
was given aspirin 324 mg chew, clopidogrel 300 mg per
oral, morphine sulfate 2 mg IV every 10 minutes as needed,
subcutaneous low molecular weight heparin 60 mg (1 mg/
kg) every 12 hours, and nitroglycerin infusion (10 to 20
µg/min). The patient began to experience a worsening
angina pain and was not responding to treatment.
However, there were no ECG changes from previous tests.
The cardiologist decided to do percutaneous coronary
intervention (PCI) with approximately 11 hours spanning
from the presentation to emergency room visit. Coronary
arteriography revealed a 100% stenosis in the proximal
portion with large clot burden at LCX coronary artery.
Finally, stent inflation was used with excellent results.
He was discharged from the hospital after one week
with orders to take baby aspirin per oral once a day and
CASE REPORT
A 54-year-old male, known to have bronchial asthma
(mild) well controlled with albuterol inhaler, was brought
by his family to our emergency medicine department three
hours after the onset of pressure like chest pain, which was
central and radiated to the left arm. The patient reported
taking an oral dose of amphetamine about seven hours
before the presentation. The patient told that the chest
pain was neither decreased nor increased by anything.
The pain was associated with shortness of breath, nausea
and vomiting. The patient had no history of palpitation,
cough, wheezing, abdominal pain or back pain. He had no
history of similar symptoms before, such as ischemic heart
disease, diabetes mellitus, hypertension, hyperlipidemia,
or atrial fibrillation. He had no family history of similar
symptoms or coronary artery disease. He was not on
any medication. He was not allergic to anything. He
smoked one pack of cigarettes per day over the last 10
years. He used amphetamine tablets occasionally and
had no history of drinking alcohol. At the time of arrival
to the hospital, his blood pressure was 137/94 mmHg,
heart rate was 77 beat per minute, respiratory rate was
17 breaths per minute, oral temperature was 37.2°C and
oxygen saturation was 99% on room air. Generally, he
appeared in severe respiratory distress, but alert and
oriented. Cardiovascular examination revealed first and
second heart sounds were within normal limits, point
of maximum impulse was not displaced, and no rub,
murmur or gallop sounds were exhibited. His lungs were
clear to auscultation bilaterally.
A 12-lead electrocardiogram showed a regular sinus
rhythm (79/min) and T-wave inverted in lead I, aVL, (V4V6). The patient had no previous ECG, echo, stress, or
lipid profile reports for comparison Because of his lack
of prior related symptoms. Laboratory data showed total
Figure 1: ECG showing T-wave inverted in lead I, aVL, (V4-V6).
Figure 2: Left coronary angiograph (A, B) Showing full occlusion
of left circumflex coronary artery at proximal portion, (B, C)
Showing left circumflex coronary artery during stenting, (E, F)
Showing patent left circumflex coronary artery after successful
stenting.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):698–701.
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clopidogrel 75 mg per oral once a day for six months.
Additionally, his medications for bronchial asthma were
maintained and he was given an appointment for the
cardiac clinic after one week. At follow-up, his blood
pressure, lipid profile, and fasting blood sugar were
within the normal range.
DISCUSSION
Cardiovascular problems related to amphetamine use
are common. However, most of the cases and population
studies in literature concerning drug-induced acute
myocardial infarction (AMI) have identified young
male patients presenting with elevated ST levels and
vasospasms in the left anterior descending artery and/or
right coronary artery. What is unique in the present case
is the age of the patient, the apparent contributing factors
of the AMI, and the location. To our knowledge, there are
no publications of amphetamine-induced NSTEMI with
selective thrombosis of the LCX and very little focus on
oral amphetamine abuse in older adults. According to
a population-based observational study, amphetamine
abuse contributes to AMI among persons aged 18-44
years, but does not address the incidence among those
older than 44 years [5]. Another study of the frequency
of acute coronary symptoms due to amphetamine use
among patients aged between 32-48 years [6]. Thus, the
age of our 54-year-old patient is unique among literature
regarding coronary symptoms due to amphetamine use.
Moreover, the lack of vasospasms and the presence
of thrombosis in our case are unusual in literature. The
mechanism of drug-induced MI is usually associated with
coronary spasms [3]. One exception in literature is the
case of a young male patient who presented with acute
MI and tested positive for amphetamine. An arteriogram
showed acute thrombosis, but early PCI did not help and
the patient died later [3]. Our patient was fortunate to
have survived. Additionally, many cases of drug-induced
AMI show ST-segment elevation, for instance a young
male who presented to the emergency room with chest
pain after taking Captagon and showed ST elevation due
to a narrowing in the proximal portion of the left anterior
descending (LAD) artery [7]. However, LCX occlusions, as
in our case, do not usually result in elevated ST segments
[8].
While LCX occlusions are usually NSTEMI, it is rare
for the cardiovascular problems among amphetamine
users to be located in the LCX artery in the first place.
In contrast, cases of narrowing in the LAD artery [7] and
the right coronary artery due to amphetamine use are not
unusual [9]. In a rare case, screening of the LCX artery
showed thrombosis, but it was secondary to thrombosis
of the larger LAD artery [10]. The underlying reason for
the rarity of amphetamine-induced LCX thrombosis is
speculative, but perhaps it is related to age and pre-existing
cardiovascular issues. Exposure to amphetamine may
trigger AMI thrombotic occlusion at the site of multiple
Khattab et al. 700
‘vulnerable’ plaques, while most amphetamine abusers
are young men who are less likely to have vulnerabilities
in the LCX artery. Due to the confluence of unique
factors regarding advanced age, the MI mechanism,
and the location of the problem, we find this case of
amphetamine-induced AMI to be of particular interest
for understanding the range of possible cardiovascular
issues we may encounter in our emergency room.
CONCLUSION
This case demonstrates why asking questions about
stimulant drugs use is crucial with anginal chest pain
presentation, even for an older patient. Drug abuse
is not only for young adults and men but middle aged,
older persons and women are also susceptible, and can
present unique amphetamine-induced acute coronary
syndrome etiology. In some cases, as in this present case,
sympathomimetic drugs may cause myocardial ischemia
due to blood clot formation.
*********
Acknowledgements
Acknowledgment is owed to Dr. Sabry Elmalah for his
help diagnosing and treating the patient and to Clayton
Chiarelott for proofreading and editing the article.
Author Contributions
Eyad Khattab – Conception and design, Acquisition of
data, Analysis and interpretation of data, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
Asaad Shujaa – Conception and design, Acquisition of
data, Drafting the article, Final approval of the version to
be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Eyad Khattab et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
REFERENCES
1.
Beynon CM, McVeigh J, Roe BA. Problematic drug
use, aging and older people: Trends in the age of
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):698–701.
www.ijcasereportsandimages.com
drug users in northwest England. Ageing Society
2007;27:799–810.
2. From AM, Best PJ, Lennon RJ, Rihal CS, Prasad A.
Acute myocardial infarction due to left circumflex
artery occlusion and significance of ST-segment
elevation. Am J Cardiol 2010 Oct 15;106(8):1081-5.
3. El Menyar AA. Drug-induced myocardial infarction
secondary to coronary artery spasm in teenagers and
young adults. Journal of Postgraduate Medicine 2006
Jan-Mar;52(1):51–6.
4. Heusch G. Heart rate in the pathophysiology of
coronary blood flow and myocardial ischaemia:
Benefit from selective bradycardic agents. British
Journal of Pharmacology 2008 Apr;153(8):1589-601.
5. Westover AN, Nakonezny PA, Haley RW. Acute
myocardial infarction in young adults who abuse
amphetamines. Drug Alcohol Depend 2008 Jul
1;96(1-2):49–56.
6. Turnipseed SD, Richards JR, Kirk JD, Diercks
DB, Amsterdam EA. Frequency of acute coronary
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Khattab et al. 701
syndrome in patients presenting to the emergency
department with chest pain after methamphetamine
use. Journal of Emergency Medicine 2003
May;24(4):369–73.
7. Uluçay A, Arpacik Kargi C, Aksoy MF. Acute
myocardial infarction associated with Captagon use.
Anadolu Kardiyol Derg 2012 Mar;12(2):182–5.
8. Stribling WK, Kontos MC, Abbate A, et al. Left
circumflex occlusion in acute myocardial infarction
(from the National Cardiovascular Data Registry).
The American journal of cardiology 2011 Oct
1;108(7):959–63.
9. Furst SR, Fallon SP, Reznik GN, Shah PK. Myocardial
infarction after inhalation of methamphetamine.
The New England Journal of Medicine 1990 Oct
18;323(16):1147–8.
10. Lan WR, Yeh HI, Hou CJY, Chou YS. Acute Thrombosis
of Double Major Coronary Arteries Associated with
Amphetamine Abuse. Acta Cardiologica Sinica
2007;23(4):268–72.
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other devices
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):702–704.
www.ijcasereportsandimages.com
Kumar et al. 702
CASE reportOPEN ACCESS
Pseudotumor cerebri caused by uncontrolled Graves’
disease in an adolescent
Ashutosh Kumar, Sasikumar Kilaikode, Paul Saenger
Abstract
doi:10.5348/ijcri-2013-12-415-CR-9
Introduction:
Hyperthyroidism
causing
pseudotumor cerebri is very rare in children. Case
Report: We are reporting a case of a 17-year-old
girl with uncontrolled Graves’ disease presenting
with pseudotumor cerebri (PTC). Patient initially
did not respond to common management of
PTC with therapeutic lumbar puncture and
acetazolamide, but improved after treating
hyperthyroidism by radioablation therapy.
Conclusion: We emphasize that uncontrolled
hyperthyroidism should be suspected as a
secondary cause of pseudotumor cerebri in
children and treated aggressively to avoid its
dreaded complications.
Keywords: Pseudotumor cerebri (PTC), Benign
intracranial
hypertension
(BIH),
Graves’
disease, Papilledema, Radioablation therapy,
Hyperthyroidism
*********
Kumar A, Kilaikode S, Saenger P. Pseudotumor cerebri
caused by uncontrolled Graves’ disease in an adolescent.
International Journal of Case Reports and Images
2013;4(12):702–704.
*********
Ashutosh Kumar , Sasikumar Kilaikode1, Paul Saenger2
Affiliations: 1MD, Resident, Pediatrics, The Brooklyn Hospital
Center, Brooklyn, New York, USA; 2MD, Attending, Pediatric
Endocrinology, The Brooklyn Hospital Center, Brooklyn,
New York, USA.
Corresponding Author: Sasikumar Kilaikode, 539 85th Street
Apt 1C, Brooklyn, New York, USA. 11209; Ph: 6466249458;
Email: sak9072@nyp.org
1
Received: 24 April 2013
Accepted: 20 June 2013
Published: 01 October 2013
Introduction
Pseudotumor cerebri (PTC), also known as benign
intracranial hypertension (BIH), is a syndrome of
increased intracranial pressure in the absence of
change in ventricular size, with normal cerebrospinal
fluid (CSF) analysis and normal neuroimaging [1].
Presenting symptoms are similar to those associated with
hydrocephalus and typically include headache, vomiting,
and changes in vision. Neurological examination is
typically normal except papilledema which is almost
always noted in adults, but may be inconsistent in
children and adolescents.
Pseudotumor cerebri can be idiopathic or secondary
to numerous underlying causes, including endocrine,
rheumatologic, immunologic conditions and medications
[1]. Both hypothyroidism and hyperthyroidism have been
reported to cause PTC. Hyperthyroidism causing PTC is
rare. There are only a few case reports of PTC caused by
hyperthyroidism in adults and two case reports in the
pediatric population [2, 3].
We are reporting a case of a 17-year-old girl with
uncontrolled Graves’ disease presenting with PTC.
CASE REPORT
A 17-year-old African-American female with
congenital HIV controlled on anti-retroviral therapy
(Complera, Gilead Sciences, Inc. Foster City, CA, USA),
mild intermittent asthma and Graves’ disease diagnosed
five years ago, managed by methimazole with poor
compliance, presented with one day history of severe
pounding bi-frontal headache unrelieved by analgesics.
There was no history of vomiting, fever, visual problems,
weakness of extremities, seizures, neck stiffness, or
trauma. On examination, patient had tachycardia and
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):702–704.
www.ijcasereportsandimages.com
Kumar et al. other vital signs were normal. Her weight was 48 kg
and body mass index was 19.3 kg/m2. Her physical
examination showed diffuse enlargement of the thyroid
gland and exophthalmos. On neurological examination,
there was bilateral papilledema without any focal
neurological deficit or altered sensorium. Computed
tomography (CT) scan of head was normal. On lumbar
puncture, her CSF opening pressure was raised (430 mm
of H2O), no cells, with normal glucose and protein
levels. The CSF was removed therapeutically which did
not relieve her headache significantly. The CSF studies
were negative for any bacteria, fungi, or viruses. Repeat
magnetic resonance imaging (MRI) scan of brain with
and without contrast was normal except for the finding
of stable cerebellar tonsillar ectopia which was consistent
with the previous MRI. Cerebral vascular thrombosis
was ruled out by magnetic resonance venography (MRV)
and magnetic resonance angiography (MRA) studies.
Blood analysis showed thyroid stimulating hormone
(TSH) level <0.1 µIU/mL (normal limits 0.35–4.94 µIU/
mL), FT4 2.5 ng/dL (normal limits 0.9–1.5 ng/dL), total
T4 17.7 µg/dL (normal limits 4.8–1.7 µg/dL) and total
T3 310 ng/dL (normal limits 60–160 ng/dL). Thyroid
uptake study was consistent with diffuse toxic goiter with
increased uptake 74.3% compared to the previous study
61%.
Patient was initially treated with atenolol,
acetazolamide and other supportive measures, which
only partially relieved her symptoms. Patient received
radioablation therapy with 15 mCi of I-131 and reported to
feel better. She was followed up in pediatric endocrinology
clinic one month after radioablation therapy and found
to be asymptomatic. Her thyroid functions were slowly
recovering (Table 1).
Table 1: Changes in thyroid function test with treatment
Thyroid
Function Tests
Prior to
Radioablation
therapy
4 weeks
after
therapy
6 weeks
after
therapy
TSH (µIU/mL)
<0.1
<0.1
<0.1
Total T4(µg/dL)
17.7
12
10.9
Free T4 ( ng/dL)
2.5
1.7
1.4
Total T3 (ng/dL)
310
230
180
703
PTC. Although acetazolamide can cause symptomatic
relief of PTC, our patient mostly improved after
radioablation therapy of thyroid. Therefore, we concluded
that PTC was associated with Graves’ disease in this case.
This was also supported by improvement in her thyroid
hormone levels along with symptomatic relief.
The pathophysiologic basis of PTC is still not clearly
understood, but a relationship has been established
with elevated intracranial venous pressure [4, 5]. The
increase in resistance of CSF absorption is thought to be
caused by an insufficiently high driving pressure gradient
from the subarachnoid space to the venous system.
Thyroxine, being a major regulator of sodium transport,
can contribute to altered CSF dynamics [6]. The effect of
thyroid hormone raising venous pressure may justify the
association between those two entities [6].
CONCLUSION
We would like to emphasize that uncontrolled Graves’
disease should be considered as a rare secondary cause
of pseudotumor cerebri in children. Hyperthyroidism
should be treated aggressively to reduce the complications
associated with pseudotumor cerebri, most notably
permanent visual impairment.
*********
Author Contributions
Ashutosh Kumar – Conception and design, Acquisition
of data, Analysis and interpretation of data, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
Sasikumar Kilaikode – Conception and design,
Acquisition of data, Analysis and interpretation of data,
Drafting the article, Critical revision of the article, Final
approval of the version to be published
Paul Saenger – Conception and design, Acquisition of
data, Analysis and interpretation of data, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
DISCUSSION
Hyperthyroidism causing PTC is rare in children
with only 2 reported cases [2, 3]. In one report,
hyperthyroidism was associated with hypovitaminosis A.
We excluded other causes of raised intracranial
pressure in our patient and confirmed the diagnosis of
© Ashutosh Kumar et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):702–704.
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REFERENCES
1. Rudolph CD, Rudolph AM, Lister GE, First LR,
Gershon AA. Rudolph’s Pediatrics, 22nd Ed. New
York: McGraw-Hill Medical; 2011. P.2176.
2. Merkenschlager A, Ehrt O, Müller-Felber W, Schmidt
H, Bernhard MK. Reversible benign intracranial
hypertension in a child with hyperthyroidism. J
Pediatr Endocrinol Metab 2008 Nov;21(11):1099–1.
3. Roos RA, Van der Blij JF. Pseudotumor
cerebri associated with hypovitaminosi A and
hyperthyroidism. Dev Med Child Neurol 1985
Apr;27(2):246–8.
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Kumar et al. 4.
704
Skau M, Brennum J, Gjerris F, Jensen R. What is
new about idiopathic intracranial hypertension?
An updated review of mechanism and treatment.
Cephalalgia 2006 Apr;26(4):384–99.
5. Bateman GA. Arterial inflow and venous outflow
in idiopathic intracranial hypertension associated
with venous outflow stenoses. J Clin Neurosci 2008
Apr;15(4):402–8.
6. Coutinho E, Silva AM, Freitas C, Santos E. Graves’
disease presenting as pseudotumor cerebri: A case
report. Journal of Medical Case Reports 2011;5:68.
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IJCRI 2013;4(12):705–708.
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Tevethia et al. CASE REPORT
705
OPEN ACCESS
Gastric malignancy presenting as adrenal insufficiency:
A case report
Harsh Vardhan Tevethia, Baskaran S, Tony Mathew Kadavanu, Riyaz M
Panchbhaya, Sunny DAN, Siva PK
Abstract
Introduction: Carcinomas in the lung, breast,
and lymphomas are known to metastasize to
the adrenal gland. However, these metastases
initially do not present with signs and symptoms
of adrenal insufficiency leading to a decreased
chance of adrenal insufficiency being diagnosed.
We report a case of adrenal insufficiency due to
bilateral adrenal metastases as the presenting
manifestation of gastric carcinoma. Case Report:
A 72-year-old male was admitted to our hospital
due to altered mental status, weakness, anorexia,
hematemesis and urinary incontinence for three
days. Hyponatremia, hyperkalemia, anemia,
and mild metabolic acidosis were detected.
A diagnosis of gastric carcinoma along with
bilateral metastases of the adrenal glands was
made after biopsy computed tomography and
positron emission tomography scans. Initiation
of mineralocorticoid replacement therapy,
improved the condition of patient. Conclusion:
There are only a few reports of adrenal
insufficiency or true Addisonian crisis being the
Harsh Vardhan Tevethia , Baskaran S , Tony Mathew
Kadavanu1, Riyaz M Panchbhaya3, Sunny DAN1, Siva PK3
Affiliations: 1MBBS, Resident, Department of Internal
Medicine, Mahatma Gandhi Medical College and Research
Institute, Puducherry, Puducherry, India; 2MB, DNB,
Consultant physician, Department of Internal Medicine,
Mahatma Gandhi Medical College and Research Institute,
Puducherry, Puducherry, India; 3MB, MD, Assistant Professor,
Department of Internal Medicine, Mahatma Gandhi Medical
College and Research Institute, Puducherry, Puducherry,
India.
Corresponding Author: Harsh Vardhan Tevethia, MBBS,
Resident, Department of Internal Medicine, Mahatma
Gandhi Medical College and Research Institute,
Puducherry, Puducherry, India; Ph: +91-915902634; E-mail:
hershey4686@gmail.com
1
Received: 03 June 2013
Accepted: 09 July 2013
Published: 01 December 2013
2
presenting manifestation of underlying malignant
tumors of the lung, colon, or lymphomas. One
should also consider the possibility of malignancy
as a differential in such cases.
Keywords:
Adrenal
insufficiency,
Gastric
malignancy, Hyponatremia, Addison’s disease
*********
Tevethia HV, Baskaran S, Kadavanu TM, Panchbhaya
RM, Dan S, Siva PK. IGastric malignancy presenting as
adrenal insufficiency: A case report. International Journal
of Case Reports and Images 2013;4(12):705–708.
*********
doi:10.5348/ijcri-2013-12-416-CR-10
Introduction
Carcinomas in the lung, breast, and lymphomas are
known to metastasize to the adrenal gland [1]. However,
in most cases they do not attain clinical significance
since these are diagnosed at autopsy. We report a case of
adrenal insufficiency due to bilateral adrenal metastases
as the presenting manifestation of gastric carcinoma.
CASE REPORT
A 72-year old male, without any known comorbidities,
was admitted to our hospital due to altered mental
status, weakness, anorexia, hematemesis and urinary
incontinence for three days. He complained of abdominal
distension and dyspepsia for the last two years. There was
a loss of appetite and weight of about 10 kg. At the time
of admission to hospital, physical examination revealed
blood pressure 110/80 mmHg, tachycardia 120 bpm
with no other specific systemic finding. The patient was
severely dehydrated, presented with decreased reflexes
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):705–708.
www.ijcasereportsandimages.com
and his skin showed hyperpigmentation (Figure 1).
Laboratory tests demonstrated hyponatremia (serum
sodium 125 mEq/L), hyperkalemia (serum potassium
5.6 mEq/L), anemia (Hb 7.3 g/dL) and mild metabolic
acidosis (pH 7.25). The electrocardiogram showed
normal QRS complexes. There was a mild rise in serum
urea 47 mg/dL and normal serum creatinine levels 0.8 mg/
dL. After ruling out tuberculosis and drug induced
adrenalitis, computed tomography (CT) scan of chest and
abdominal was done. Gastric carcinoma with perigastric,
periceliac and para aortic lymphadenopathy along with
bilateral metastases of the adrenal glands was found
(Figure 2). We performed a whole body positron emission
tomography (PET) scan which further validated the CT
findings (Figure 3). The biopsy showed a moderate to
poorly differentiating adenocarcinoma in the gastric
antrum. Based on the patient’s clinical status and the
laboratory test results, in addition to the magnitude of
the adrenal masses, we decided to perform a high dose
cosyntropin stimulation test. The serum cortisol level
was 420 nmol/L before the intramuscular administration
of cosyntropin (250 µg), and 61,438 nmol/L 60 minutes
after the administration. These results combined with the
elevated levels of adrenocorticotropic hormones (ACTH)
165.7 pg/mL before the administration, and the diagnosis
of adrenal insufficiency had been established.
After fluid substitution, administration of glucose/
insulin, calcium gluconate, sodium bicarbonate,
Tevethia et al. 706
Figure 2: Computed tomography scan of abdomen.
Figure 3: Whole body positron emission tomography scan.
salbutamol, furosemide, cation exchange resin, and
initiation of replacement therapy with glucocorticoids
and fludrocortisone, the patient improved within a
few days. Sodium and pH levels normalized after two
days. The patient returned to normal life activity under
methylprednisolone administration and was able to
undergo chemotherapy for his primary disease.
DISCUSSION
Figure 1: Skin hyperpigmentation of the patient.
Addison’s disease refers to partial or complete adrenal
insufficiency. According to recent series, its prevalence
is estimated at 93 to 117 per million [2–4]. When this
‘disease of the suprarenal capsules’ was originally
described by Thomas Addison, tuberculosis was the
main cause of adrenal insufficiency. Today, autoimmune
adrenalitis is responsible for Addison’s disease in 69–93%
of the cases, while the rest are caused by tuberculosis,
drugs, infections, adrenal hemorrhage, infarction, or
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):705–708.
www.ijcasereportsandimages.com
thrombosis [5–8]. Metastatic infiltration of the adrenal
glands is a common finding in malignant tumors,
especially in adenocarcinomas. The most common
primary tumors sites are breast, lung, esophagus, kidney,
colon, rectum, liver and bile ducts. They are also observed
in lymphomas and melanomas [9, 10]. In this patient, the
primary lesion was in the gastric antrum. Unfortunately,
adrenal insufficiency is characterized by nonspecific
clinical features. Usually, patients complain of vague
constitutional symptoms such as generalized weakness,
fatigue, malaise, and weight loss. Many patients may
present with hypotension, dehydration, postural
hypotension and in case of adrenal crisis, syncope,
or shock. Hyperpigmentation is evident in nearly all
patients, especially over the extensor surfaces of the body
and the mucosa.
Laboratory tests often reveal hyponatremia,
hyperkalemia, mild metabolic acidosis, hypoglycemia,
anemia, and lymphocytosis. In most of these cases, the
metastases are unilateral without clinical significance. In
this patient, bilateral adrenal metastases were present
at the time of the initial diagnosis, being responsible for
clinical evident adrenal insufficiency. There are only a
few reports of adrenal insufficiency or true Addisonian
crisis being the presenting manifestation of underlying
malignant tumors of the lung, colon, or lymphomas
[11]. One also needs to be aware of the possibility of
malignancy as a differential in cases presenting as adrenal
insufficiency.
Tevethia et al. Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Harsh Vardhan Tevethia et al. 2013; This article
is distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1.
2.
3.
4.
5.
CONCLUSION
6.
There are only few reports of adrenal insufficiency or
true Addisonian crisis being the presenting manifestation
of underlying malignant tumors of the lung, colon, or
lymphomas. One should also consider the possibility of
malignancy as a differential in such cases.
*********
7.
8.
Author Contributions
Harsh Vardhan Tevethia – Acquisition of data, Critical
revision of the article, Final approval of the version to be
published
Bhaskaran S – Conception and design, Critical revision of
the article, Final approval of the version to be published
Kadavanu Tony Mathew – Conception and design,
Acquisition of data, Drafting the article, Critical revision
of the article, Final approval of the version to be published
Panchbhaya Riyaz – Analysis and interpretation of data,
Drafting the article, Final approval of the version to be
published
Sunny DAN – Conception and design, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
SIva P K – Conception and design, Critical revision of the
article, Final approval of the version to be published
707
9.
10.
11.
Bullock WK, Hirst AE Jr. Metastatic carcinoma of the
adrenal. Am J Med Sci 1953 Nov;226(5):521–4.
Willis AC, Vince FP. The prevalence of Addison’s
disease in Coventry, UK. Postgrad Med J 1997
May;73(859):286–8.
Laureti S, Vecchi L, Santeusanio F, Falorni A. Is the
prevalence of Addison’s disease underestimated? J
Clin Endocrinol Metab 1999 May;84(5):1762.
Erichsen MM, Løvås K, Skinningsrud B, et al. Clinical,
immunological, and genetic features of autoimmune
primary adrenal insufficiency: Observations from a
Norwegian registry. J Clin Endocrinol Metab 2009
Dec;94(12):4882–90.
Irvine WJ, Barnes EW. Adrenocortical insufficiency.
Clin Endocrinol Metab 1972;1:549.
Zelissen PM, Bast EJ, Croughs RJ. Associated
autoimmunity in Addison’s disease. J Autoimmun
1995 Feb;8(1):121–30.
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Alguacil G. Addisonian crisis as the presenting feature
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Mohan et al. CASE REPORT
709
OPEN ACCESS
Talon cusp in primary dentition: A case report
Ravi Prakash Sasankoti Mohan, Sankalp Verma, Udita Singh,
Neha Agarwal, Soumi Ghanta, Kuber Tyagi
Abstract
Introduction: Talon cusp is a well delineated
accessory cusp projecting from the cingulum
area of an anterior tooth. It is named so due to
its resemblance with eagle’s talon, which is the
shape of eagle’s claw when hooked on to its prey.
Various alterations in shape in talon’s cusp may
be present like it may be either sharp or spiked,
teat like or may have rounded and smooth tips.
Only two cases have been reported in Indian
population till date. Case Report: Hereby, we
report a unique case of talon’s cusp in a fouryear-old boy. Conclusion: The occurrence of
talon’s cusp in mandibular primary dentition is
extremely rare.
Keywords: Cingulum, Accessory, Talon cusp
*********
Ravi Prakash Sasankoti Mohan1, Sankalp Verma2, Udita
Singh3, Neha Agarwal3, Soumi Ghanta3, Kuber Tyagi4
Affiliations: 1MDS, Professor & Head of the Department,
Oral Medicine and Radiology, Kothiwal Dental College and
Research Centre, Moradabad, Uttar Pradesh, India; 2MDS,
Assistant Professor, Oral medicine and Radiology, Kothiwal
Dental College and Research Centre, Moradabad, Uttar
Pradesh, India; 3BDS, Post Graduate Student, Oral Medicine
and Radiology, Kothiwal Dental College and Research
Centre, Moradabad, Uttar Pradesh, India; 4MDS, Assistant
Professor, Oral Medicine and Radiology, Teerthanker Dental
College and Research Centre, Moradabad, Uttar Pradesh,
India.
Corresponding Author: Dr. Ravi Prakash Sasankoti Mohan,
C/o Dr. R. P. Singh (MS), Dhanwantri Nursing Home,
Sarai Khalsa, Behind Head Post Office, Moradabad, Uttar
Pradesh, INDIA. Pin-244001; Mob: 09997119919; Email:
sasan_ravi@rediffmail.com
Received: 29 May 2013
Accepted: 11 July 2013
Published: 01 December 2013
Mohan RPS, Verma S, Singh U, Agarwal N, Ghanta S,
Tyagi K. Talon cusp in primary dentition: A case report.
International Journal of Case Reports and Images
2013;4(12):709–713.
*********
doi:10.5348/ijcri-2013-12-417-CR-11
Introduction
Talon cusp is a rare dental anomaly, which originates
during the morphodifferentiation stage of tooth
development. It is defined as, “an additional cusp that
predominantly projects from the lingual surface of
primary or permanent anterior teeth, is morphologically
well delineated and extends at least half the distance from
cementoenamel junction to incisal edge” [1]. Although
it affects both the primary and permanent dentition
but permanent dentition is found to be involved thrice
more commonly than the primary dentition. Several
studies have reported a male and female ratio to be 2:1
[2, 3]. Talon cusp is not entirely innocuous and may
pose difficulties like predisposition to caries, occlusal
interferences and unpleasant esthetics [4]. This article
focusses on an extremely rare case presenting with talon
cusp in mandibular primary dentition.
CASE REPORT
A four-year-old boy was reported to the outpatient
department with a chief complaint of decaying of right
lower back tooth region since 1–2 months. There was no
remarkable medical or dental history. He was apparently
healthy child born to non-consanguineous parents at full
term. Nothing significant findings were elicited in general
physical examination. Intraoral examination revealed
mixed dentition with good oral hygiene. Dental caries was
present in right lower back teeth (in relation to 73, 74).
Apart from this, an ‘extra cusp’ was seen projecting from
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IJCRI 2013;4(12):709–713.
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the lingual surface in relation to mandibular primary
lateral incisor (72) as shown in Figure 1A. On close
examination of the involved tooth (72), an accessory cusp
was present, projecting from cingulum and involving
more than half of the lingual surface (Figure 1B). Intraoral
periapical radiograph confirmed the presence of talon
cusp in relation to 72 (Figure 2). As talon cusp caused
no difficulties to the patient, topical fluoride sealant was
applied and patient was kept on follow-up at six monthly
interval.
Figure 1: A 4-year-old boy presenting with talon’s cusp in
primary mandibular left lateral incisor: (A) Clinical photograph
of intraoral view showing talon’s cusp in relation to 72. (B)
Clinical photograph of intraoral view clearing showing talon
cusp projecting from cingulum of 72 and extending to almost
half the lingual surface.
Figure 2: Intraoral periapical radiograph of mandibular anterior
region confirming the presence of talon cusp in relation to 72.
Note that no involvement of permanent tooth buds was seen.
Mohan et al. 710
DISCUSSION
More than two centuries ago, William Mitchel
described accessory cusp on the lingual surface of
permanent upper central incisor of a woman and described
it as, “process of horn like shape curving from the base
downwards to the cutting edge”. In 1972, Mellor and Ripa
named it talon cusp owing to its close resemblance with
an eagle’s talon [3]. Since then , many names have been
used for this dysmorphic disturbance of teeth, namely,
dens evaginatus, supernumerary cusp, horn, hyperplastic
cingulum, evaginated odontome, cusped cingulum,
accessory cusp and supernumerary lingual tubercle [5].
The usage of multiple terms added up to confusion which
was cleared when Mader suggested that the term talon
cusp should be reserved only for those anomalous cusp
that prominently projected from the lingual surface of
a succedaneous tooth, that are morphologically well
delineated and extend at least half the distance from
cementoenamel junction to the incisal edge. Smaller cusp
like projections in the cingulum should be referred to as
enlarged or prominent cingulum [6].
The prevalence of talon cusp in primary dentition is
extremely low as concluded from the review of literature
from 1977 to 2013 (Table 1). Till date 34 cases of talon
cusp in primary dentition have been reported from all
over the world. Out of these 34 cases, only two are in
the mandibular primary dentition and to the best of
our knowledge, ours is the third reported case of this
uncommon anomaly.
The etiology still remains unclear. Various hypotheses
regarding its etiology have been put forward. Some of
these are; it is supposed to be a consequence of outward
folding of inner enamel epithelial cells or may be due to
hyperactivity of dental lamina [2]. Another hypothesis
suggests genetics to be a causative factor of talon cusp based
on its occurrence in a family. Trauma and other localized
forces on tooth germ have also been held responsible
for talon cusp [2, 4, 10]. Lee proposed that hyperactivity
of cells of tooth germ may lead to development of talon
cusp, which is genetically determined but the degree is
influenced by environmental factors [16].
Clinically, it presents as an accessory cusp that
projects from the cingulum area of anterior teeth and
involves more than the half of the lingual or palatal
surface. Hattab et al. classified talon’s cusp based on the
degree of formation and extension into three categories.
These are [15]:
•
Type 1 (True talon): A well-delineated additional
cusp that predominantly projects from the
palatal or lingual surface of an anterior teeth and
extends half way from cementoenamel junction
to the incisal edge (as seen in our patient).
•
Type 2 (Semi talon): An additional cusp of a
millimetre or more but extending less than half
the distance from cementoenamel junction to
incisal edge. It may blend with palatal surface or
strand away from the crown
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IJCRI 2013;4(12):709–713.
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Mohan et al. 711
Table 1: The prevalence of talon cusp in primary dentition from 1977 to 2013.Till date 34 cases of talon’s cusp in primary dentition
have been reported from all over the world. Out of these 34 cases, only two are in the mandibular primary dentition and to the best of
our knowledge, ours is the third reported case of this uncommon anomaly
S. No
Name of Author
Year
Maxillary
Dentition
Mandibualr
Dentition
Age of Patient
1
S.K. Mallineni et al.[7]
2013
51
-
4 year+ 2 months
Female
Chinese
2
S.K.Mallineni et al.[7]
2013
51
-
2 year+9 months
Male
,,
3
,,
,,
61
-
2 year +9 months
Male
,,
4
Prabhu et al.[8]
2012
62
-
4 years Male
South Indian
5
Swaminathan Kavitha 2012
et al.[9]
-
82
5 years Male
,,
6
S.Hedge et al.[10]
-
72
Not available
,,
7
Praveen P et al.
2011
51
-
8 years Male
,,
8
A.Kapur et al.[12]
2011
51
-
4 years male
,,
9
Chun Kei Lee et al.[13]
2008
52
-
Chinese
10
,,
,,
52
-
,,
11
,,
,,
52
-
12
Yoon RK & Chussid S[14] 2006
51
-
14 months Male
New York
13
Tsaii AL & Chang P[14]
2003
51
-
13 months Female
Chinese
14
Gungor H et al.
2000
51
-
14 months Male
,,
15
Hattab FN & Yasin 1996
OM[14]
51
-
17 months Male
,,
16
Seadano et al.[15]
1989
Prevalence of talon cusp in primary dentition= 0.06%
Mexican
17
Chawla et al.[15]
1983
Prevalence of talon cusp in primary dentition=7.7%
North Indian
18
Liu JF &Chen JR
1955
51
-
12 months
Female
Chinese
19
,,
,,
51
-
,,
Chinese
20
,,
,,
51
-
3 years+6 months
Female
Chinese
21
,,
,,
51
-
,,
Chinese
22
Meon R et al.[14]
1990
51
-
4 years Male
,,
23
Morin CK et al.
1987
51
-
12 months Male
,,
24
Chen RJ & Chen HS
1986
51
-
10 months Male
,,
25
,,
,,
51
-
6.5 years
,,
26
,,
,,
51
-
9 months Male
,,
27
,,
Not
available
51
-
13 months Male
,,
28
,,
,,
51
-
4 years Male
,,
29
,,
,,
51
-
6 years Male
,,
30
Davis PJ
AH[1,14]
51
-
5 years Male
-
31
,,
,,
51
-
5 years Male
-
32
Natkin E
1983
51
-
5 years Male
-
33
Mass et al.
1978
51
-
1 year Male
-
34
Henderston HZ et al.[14] 1977
51
-
4 years Female
-
2011
[11]
[14]
[14]
[14]
[14]
[14]
[14]
&Brooke 1986
,,
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•
ype 3 (Trace Talon): Enlarged cingulum and
T
may present as conical bifid or tubercle shaped.
Histologically, it may or may not contain pulpal tissue.
Radiographically, it appears to be superimposed over the
tooth on which it develops [4]. Talon cusp may be seen
in association with Mohr syndrome, Rubinstein–Taybi
syndrome, Sturge–Weber syndrome, Ellis–van Creveld
syndrome [7].
The clinical problems associated with talon cusp
are predisposition to caries due to the presence of deep
grooves and resultant stagnation of food debris, periapical
lesions, occlusal interference, and irritation to tongue
during mastication or speech. But our patient presented
with no difficulties.
Treatment is required only for symptomatic cases.
In case of deep developmental grooves, these should be
thoroughly cleaned to get rid of debris and sealed using
fissure sealant. In case of carious grooves, restoration of
the involved tooth becomes mandatory. If due to deep
carious lesion or occlusal interferences, a portion of talon
cusp has to be removed then grinding should be done
gradually on consecutive visits at six weeks intervals so as
to ensure deposition of reparative dentin. Pulp therapy is
done in cases of pulpal involvement [7, 8, 11].
CONCLUSION
In this article, we have reviewed literature regarding
talon cusp in primary dentition and have added
significantly to the present knowledge by documenting a
rare case report.
*********
Author Contributions
Ravi Prakash Sasankoti Mohan – Substantial
contributions to conception and design, Acquisition of
data, Analysis and interpretation of data, Drafting the
article, Revising it critically for important intellectual
content, Final approval of the version to be published
Sankalp Verma, Udita Singh – Substantial contributions
to conception and design, Acquisition of data, Analysis
and interpretation of data, Revising it critically for
important intellectual content, Final approval of the
version to be published
Neha Agarwal – Substantial contributions to conception
and design, Acquisition of data, Analysis and
interpretation of data, Revising it critically for important
intellectual content, Final approval of the version to be
published
Soumi Ghanta – Substantial contributions to
conception and design, Acquisition of data, Analysis and
interpretation of data, Revising it critically for important
intellectual content, Final approval of the version to be
published
Kuber Tyagi – Substantial contributions to conception and
design, Acquisition of data, Analysis and interpretation
Mohan et al. 712
of data, Revising it critically for important intellectual
content, Final approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Ravi Prakash Sasankoti Mohan et al. 2013; This article
is distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1. Davis PJ, Brook AH. The presentation of talon
cusp: Diagnosis, clinical features, association and
possible aetiology. British Dental Journal 1986 Feb
8;160(3):84–8.
2. Shafer WG, Hine MK, LevyBM. A textbook of oral
pathology. 4th ed. Philadelphia: WB Saunders
1983:40–1.
3. Mellor JK, Ripa LW. Talon cusp: A clinically
significant anomaly. Oral Surg Oral Med Oral Pathol
1970 Feb;29(2):225–8.
4. Goaz PW, White SC. Oral Radiology. Missouri: CV.
Mosby Company 1987;2:447.
5. Gardener DG, Girgs SS. Talon cusps: A dental anomaly
in the Rubinstein-Taybi syndrome. Oral Surg Oral
Med Oral Pathol 1979 Jun;47(6):519–21.
6. Marder CL. Talon Cusp. J Am Dent Assoc 1981
Aug;103(2):244–6.
7. Mallineni SK, Manan NM, Lee CK, King NM. Talon
cusp affecting primary dentition in two siblings: A
case report. Rom J Morphol Embryol 2013;54(1):211–
3.
8. Rachana V Prabhu, Prasanna Kumar Rao, Veena
KM, Prathima Shetty, Laxmikanth Chatra, Prashanth Shenai. Prevalence of Talon cusp in Indian
population. J Clin Exp Dent2012;4(1):e23–7.
9.Swaminathan Kavitha, Haridoss Selvakumar,
Rajendran Barathan. Mandibular talon cusp in
primary lateral incisor: A rare case report. Case Rep
Dent 2012;2012:670745.
10. Hedge S, Ashok Kumar BR. Mandibular talon cusp:
Report of 2 cases. Int Journal of Paediatric dentistry
2004;9(4):303.
11. Praveen P, Anantharaj A, Karthik Venkataraghavan,
Prathibha Rani, Jaya AR. Talon Cusp In A Primary
Tooth. Journal of dental science and research
2011;2(1):35–40.
12. Kapur A, Goyal A, Bhatia S. Talon cusp in a primary
incisor: A rare entity. Journal of Indian society of
Pedodontics and Preventive dentistry 2011 JulSep;29(3):248–50.
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13. Chun-Kei Lee, Nigel M King, Edward CM L, Shiuyin Cho. Talon cusp in primary dentition-literature
review and report of 3 cases. Journal of clinical
pediatric dentistry 2008;30(4):153–6.
14. Yoon RK, Chussid S. Dental management of a talon
cusp on a primary incisor. Pediatric dentistry 2007
Jan-Feb;29(1):51–5.
15. Hattab FN, Yassin OM, Al-Nimri KS. Talon cusp in
permanent dentition associated with other dental
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anomalies: Review of literature and report of 7 cases.
ASDC J Dent Child 1996 Sep-Oct;63(5):368–76.
16. Lee CK, King NM, Lo EC, Cho SY. Talon cusp in the
primary dentition: Literature review and report of
three rare cases. J Clin Pediatr Dent 2006;30(4):299–
305.
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IJCRI 2013;4(12):714–718.
www.ijcasereportsandimages.com
Alleyne-Mike et al. CASE REPORT
714
OPEN ACCESS
Unique case of dual gynecological malignancies co-existing
with an uncommon thyroid lymphoma
Kellie Alleyne-Mike, Khadiga Elfadil Ahmed Mohammed,
Adriaan Leon van Wijk, Francois Cornelius Jacobus Botha
Abstract
Introduction: Synchronous primaries are
uncommon in medical literature but pose many
diagnostic and therapeutic challenges which
require a multi-disciplinary approach. Case
Report: A 64-year-old female was presented
with a vulva lesion and bilateral inguinal
lymphadenopathy. She was also noted to have a
palpable abdominal growth and an enlarged neck
mass. Blood evaluation yielded elevated CA-125
and thyroid stimulating hormone levels. All other
tests were unremarkable. Computed tomography
of the neck showed a large thyroid mass with
bilateral lobe involvement and abdominal
scanning showed a large cystic lesion causing
right hydronephrosis and hydroureter. She
was diagnosed with a primary vulva squamous
carcinoma in a background of lichen sclerosus
which was managed surgically. The patient also
underwent laparotomy and was found to have a
borderline ovarian malignancy. A neck biopsy
yielded a thyroid lymphoma which was treated
with chemotherapy. Prophylactic radiation to
the groin was performed after multi-disciplinary
consult. There was an excellent clinical response
Kellie R Alleyne-Mike1, Khadiga EA Mohamed1, Leon van
Wijk2, Francois CJ Botha3
Affiliations: 1Senior Resident, Radiation Oncology
Department, Groote Schuur Hospital, Cape Town, South
Africa; 2Consultant, Radiation Oncology Department, Groote
Schuur Hospital, Cape Town, South Africa; 3Resident,
Division of Pathology,Groote Schuur Hospital, Cape Town,
South Africa.
Corresponding Author: Kellie Rozelle Alleyne-Mike, LE 33,
Groote Schuur Hospital, Anzio Road, Observatory 7925,
Cape Town, South Africa Ph: 27-21-791926866 or 868-7580192; Fax Number: 27-21-4485707; Email: kmike.tt@gmail.
com
Received: 07 June 2013
Accepted: 11 July 2013
Published: 01 December 2013
to the treatment. Conclusion: This is the first
report of concurrent vulva and ovarian pathology
associated with a hematologic malignancy.
Specific immunologic and genetic abnormalities
have not yet been identified in literature which
may link these supposedly random occurrences.
However, the immune dysregulation associated
with lichen sclerosus and its role in the
pathogenesis of vulva squamous carcinoma is
of interest in the background of a co-existing
hematological malignancy, and an inherent
compromised immune response. In addition,
ongoing genetic research may yet elucidate
a common link between these synchronous
primaries.
Keywords: Synchronous primary malignancies,
Thyroid lymphoma, Vulva cancer, Ovarian cancer
*********
Alleyne-Mike KR, Mohamed KEA, van Wijk L, Botha
FCJ. Unique case of dual gynecological malignancies
co-existing with an uncommon thyroid lymphoma.
International Journal of Case Reports and Images
2013;4(12):714–718.
*********
doi:10.5348/ijcri-2013-12-418-CR-12
Introduction
Synchronous primaries often pose unique management
challenges. A careful analysis, discussion of patient and
disease-related factors are required to guide appropriate
management. It has been suggested that the upper female
reproductive tract is an extension of the Mullerian system
and thus carcinogenic exposure to this unit has the potential
to produce multicentric lesions [1]. However, the lower
female genital tract does not originate from the Mullerian
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system and the presence of synchronous primaries in both
the upper and lower genital tract, as is presented in this
case, cannot be explained by common embryonic origin.
Further, a dual gynecological malignancy synchronously
coexisting with a hematological malignancy is an unusual
occurrence and will be discussed in view of the therapeutic
challenges presented.
CASE REPORT
A 64-year-old female, with a medical history of
hypertension, ischemic heart disease, and previous total
abdominal hysterectomy for a benign indication, presented
to the gynecology department with a six month history of
a pruritic sore to the right labium minorus. Clinically, the
lesion was 3 cm in diameter and extended partially across
the midline with involvement of the clitoris. Two clinically
palpable, 1 cm ipsilateral mobile inguinal lymph nodes
were noted. On further clinical examination, an abdominal
mass was palpated arising from the pelvis and extending
upwards to above the umbilicus. Additionally, she had
experienced progressive dysphagia for the previous three
months. An enlarged, smooth thyroid gland with bilateral
lobe involvement was present.
Investigations showed an elevated CA-125 558
kU/L and TSH 14 mIU/L. Full blood count, lactate
dehydrogenase (LDH), renal and liver function tests
were normal. HIV and syphilis serology were negative.
Computed tomography (CT) scan of neck showed a
massively enlarged thyroid gland with a maximum
diameter of 9.4 cm (right lobe) and 9.1 cm (left lobe)
(Figure 1). There was associated esophageal compression
and the mass displaced the airway anteriorly without
compromise. The CT scan of chest was negative for
malignancy and CT scan of abdomen showed an
enlarged liver and spleen as well as a central pelvic lesion
extending into the abdomen (Figure 2A) causing right
hydronephrosis and hydroureter (Figure 2B).
Biopsy of the vulva lesion was reported as a nonkeratinizing squamous cell carcinoma (Figure 3A) with
associated lichen sclerosus, while that of the thyroid
gland was consistent with a diffuse large B cell lymphoma
(DLBCL) (Figure 3B–D). Repeated cytological aspiration
(biopsies) of the inguinal nodes failed to confirm
malignancy. Further staging investigations included a
bone marrow biopsy which was clear.
The patient was discussed in a multi-disciplinary
clinic with input from clinical oncology, gynecology,
hematology, radiology, pathology and anesthesiology.
After extensive discussion, it was agreed to forgo
surgical management of the groin due to concerns over
potential morbidity which would delay commencement of
chemotherapy. The patient underwent laparotomy during
which a cystic left ovarian tumor was found. There was
no ascites, peritoneal or omental nodules seen during the
abdominal exploration. Peritoneal cytology was obtained
in a standard manner, followed by bilateral salpingo-
Alleyne-Mike et al. 715
oophorectomy and an infracolic omentectomy. Surgical
management of the vulva primary was done under the
same anesthesia and consisted of a radical wide local
excision. The postoperative course was unremarkable.
The histology of the vulvar lesion was of a moderately
differentiated squamous cell carcinoma which measured
23 mm at its widest diameter. The depth of invasion was
6.2 mm. The closest resection margin was 6.5 mm. The
stage was at least FIGO IB, though incomplete because
of the unknown nodal status. The ovarian histology was
of borderline serous cystadenocarcinoma, FIGO stage IA
(Figure 3E–F).
Following surgery, the CA-125 level normalized.
It was decided to start chemotherapy for the DLBCL
concurrently with prophylactic radiation to the groin
bilaterally because inguinal node dissection was not
performed. Treatment was planned using 12 MeV
to deliver 45 Gy in 25 fractions (maximum depth of
penetration 90% at 3 cm after measurement of lymph
node depth was undertaken using CT imaging). The
systemic therapy for the thyroid lymphoma, staged as
IE, was with CHOP (cyclophosphamide, vincristine,
prednisone and adriamycin) for a total of six cycles. Prior
to chemotherapy, the patient’s left ventricular ejection
fraction (LVEF) was estimated with an equilibrium
radionuclide angiocardiography (ERNA) to be 84%. A
follow-up ERNA did not indicate any LVEF compromise.
Figure 1: Coronal computed tomography of the chest and neck
prior to chemotherapy showing massively enlarged thyroid with
airway displacement. The right thyroid lobe measures 94x52
mm and the left measures 91x42 mm.
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Figure 2: Axial computed tomography of the abdomen prior to
surgery (A) showing a large well homogenated central pelvic
lesion measuring 150x190x224 mm extending up into the
abdomen, (B) with right hydronephrosis and hydroureter due
to mass effect by the large abdominal mass.
Figure 3: (A) Invasive moderately differentiated keratinizing
squamous cell carcinoma with adjacent mild lichen sclerosis
demonstrated by hyalinization of the papillary dermis and
underlying chronic inflammation (H&E stain, x400), (B)
Core biopsy of the thyroid gland (H&E stain, x100), (C) With
a diffuse infiltrate of large atypical B-lymphocytes highlighted
by CD-20 (H&E stain, x200), (D) Scattered residual thyroid
follicles (H&E stain, x200), (E) Borderline serous tumor of
the ovary with large papillary excrescences (H&E stain, x100),
and (F) Showing complex hierarchal branching and epithelial
stratification (H&E stain, x200).
DISCUSSION
In gynecologic oncology, the most frequently
documented synchronous lesions are endometrial and
ovarian malignancies [2]. Synchronous vulva and ovarian
primaries are rare. The DLBCL is an aggressive malignancy
Alleyne-Mike et al. 716
which requires urgent chemotherapy. However, this
patient’s other lesions required surgical management
and commencement of chemotherapy for the lymphoma
would mean an extensive delay of the surgery. Thus,
prompt surgical intervention was undertaken as the first
course of action.
It was agreed to forgo surgical management of
the groin as this has been shown to be associated with
significant morbidity. In an article by Gaarenstroom et al.
[3], almost 70% of the patients exhibited complications
from the procedure, which is also the experience at the
authors’ institution. These problems are mostly wound
breakdown and infection (among others). This risk of
delayed wound healing would have impacted the urgent
commencement of chemotherapy for DLBCL.
The main indication for adjuvant radiotherapy (RT)
to the vulva at our institution is the resection margin
status— positive or <8 mm. In this patient, all margins
were >6.5 mm (Lymphovascular invasion was not present
but the depth of invasion was 6.5 mm.) The closest
excision margin of 6.5 mm was accepted as a reasonable
compromise and adjuvant RT of the tumor bed was not
performed.
Furthermore, RT to the vulva is often associated with
moist desquamation which, coupled with impending
cytotoxic chemotherapy for her primary lymphoma, would
increase her risk of local infection, and cause unnecessary
delays in treatment. Re-excision of the primary site
would also have further delayed commencement of
chemotherapy.
Concern over surgical complications associated with
management of the groin prompted the consideration of
prophylactic RT to the groin. A Cochrane analysis update
by van der Velden suggested that although primary groin
irradiation is inferior to primary groin surgery in terms of
survival, the morbidity associated with primary surgery
was significant [4]. Inadequate RT technique may have
been the cause of worse outcome in the RT group. Petereit
et al. argued that there was no difference in a three-year
cause specific survival between primary RT and primary
surgery to the groin but documented greater toxicity in
the surgery group including infections in 44% and wound
separation in 68% of patients [5]. An interesting study by
Kalidas et al. measured the lymph node depths of patients
with lower gynecological malignancies and found wide
variations based on body habitus [6]. Of the 84 deep
inguinal nodes measured, all were located at a depth of
>3 cm. The study suggested that no substitute can be
made for measurement of inguinal lymph nodes and that
radiation treatment plans in previous studies were often
suboptimal as generalizations were made concerning
lymph node depth, which cannot be applicable to all
patients.
Older patients should be initiated with the same
dose intensity as young patients where possible with
subsequent adjustments made depending on tolerance.
Our patient, aged 64 years, had a good performance status
and controlled co-morbidities, which prompted the use
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):714–718.
www.ijcasereportsandimages.com
of CHOP at typical doses. At our institution rituximab is
restricted to young, fit patients with early stage disease
due to resource limitations, thus this was not a part of the
regimen employed in our patient. She received six cycles
of CHOP with one delay at cycle four due to neutropenia
which did not require hospital admission. The patient is
currently disease free from last eight months.
A specific mechanism has not been identified to link
these individual entities. However, immunological and
genetic abnormalities may be potential contributors to
the pathogenesis of such synchronous neoplasms. Hu et
al. commented that a compromised immune surveillance
was inherent in DLBCL and that this could predispose
such individuals to dual neoplasms [7]. Interestingly, the
pathogenesis of squamous cell carcinoma from lichen
sclerosus has been linked to immunologic dysregulation
[8]. In addition, genetic factors such as RASSF2A, MGMT,
TSP-1, p 53 and microsatellite instability have also
been identified and may play a vital role in this process
[9]. With regards to ovarian pathologies, syndromes
genetically associated with BRCA1, BRCA2 or DNA repair
genes such as hMSH2 and hMLH1 have been identified
[10]. However, whether any parallels can be drawn to this
situation is a subject for further research.
CONCLUSION
The findings outlined in this case are of unknown
significance due to the rarity of the occurrence. This is
the first documented presentation of such a case and thus
may propel interest in further research into the genetics
of multiple synchronous malignancies. The accumulation
of case reports may ultimately be of benefit for the
systematic analysis of multiple neoplasms as the reasons
for their occurrence are likely multi-factorial.
*********
Author Contributions
Kellie Alleyne-Mike – Conception and design, Acquisition
of data, Analysis and interpretation of data, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
Khadiga Elfadil Ahmed Mohammed – Analysis and
interpretation of data, Critical revision of the article,
Final approval of the version to be published
Adriaan Leon van Wijk – Analysis and interpretation of
data, Critical revision of the article, Final approval of the
version to be published
Francois Cornelius Jacobus Botha – Acquisition of data,
Critical revision of the article, Final approval of the
version to be published.
Alleyne-Mike et al. 717
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Kellie Alleyne-Mike et al. 2013; This article is
distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1. Eisner RF, Nieberg RK, Berek JS. Synchronous
primary neoplasms of the female reproductive tract.
Gynecol Oncol 1989 Jun;33(3):335-9.
2. Zaino R, Whitney C, Brady MF, DeGeest K, Burger RA,
Buller RE. Simultaneously detected endometrial and
ovarian carcinomas--a prospective clinicopathologic
study of 74 cases: a gynecologic oncology group study.
Gynecol Oncol 2001 Nov;83(2):355-62.
3. Gaarenstroom KN, Kenter GG, Trimbos JB, et al.
Postoperative complications after vulvectomy and
inguinofemoral lymphadenectomy using separate
groin incisions. Int J Gynecol Cancer 2003 JulAug;13(4):522-7.
4. van der Velden J, Fons G, Lawrie TA. Primary groin
irradiation versus primary groin surgery for early
vulvar cancer. Cochrane Database Syst Rev 2011 May
11;(5):CD002224.
5. Petereit DG, Mehta MP, Buchler DA, Kinsella TJ.
Inguinofemoral radiation of N0, N1 vulvar cancer may
be equivalent to lymphadenectomy if proper radiation
technique is used. Int J Radiat Oncol Biol Phys 1993
Nov 15;27(4):963-7.
6. Kalidas H. Influence of inguinal node anatomy
on radiation therapy techniques. Med Dosim
1995;20(4):295-300.
7. Hu XR, Hu YX, Fu HR, et al. Diffuse large B-cell
lymphoma with concurrent gastric adenocarcinoma:
case report and literature review. J Int Med Res
2011;39(5):2051-8.
8. Regauer S. Immune dysregulation in lichen sclerosus.
Eur J Cell Biol 2005 Mar;84(2-3):273-7.
9. Gutierrez-Pascual M, Vicente-Martin FJ, LopezEstebaranz JL. Lichen sclerosus and squamous cell
carcinoma. Actas Dermosifiliogr 2012 Jan;103(1):218.
10. Russo A, Calò V, Bruno L, Rizzo S, Bazan V, Di Fede
G. Hereditary ovarian cancer. Crit Rev Oncol Hematol
2009 Jan;69(1):28-44.
Guarantor
The corresponding author is the guarantor of submission.
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IJCRI 2013;4(12):714–718.
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Mohammed et al. CASE REPORT
719
OPEN ACCESS
Unexpected brain atrophy following administration
of intratumoral interferon alpha-2b for cystic
craniopharyngioma: A case report
Khadiga Elfadil Ahmed Mohammed, Kellie R Alleyne Mike, Jeannette Parkes
Abstract
Introduction: The use of intracystic treatments
is relatively new in the treatment of
craniopharyngioma and has the potential for
decreased morbidity and long-term control in
patients with recurrent cysts after radiotherapy
as well as delaying radiotherapy treatment in
young children. We describe a case in which the
therapeutic use of interferon alpha-2b (IFNa-2b)
was associated with an adverse effect that has not
yet been documented in the medical literature.
Case Report: An eight-year-old boy with a history
of a craniopharyngioma was presented with
radiological evidence of recurrence. He was
first diagnosed at the age of eight months and
had undergone repeated subtotal resections.
At the age of three, he also received adjuvant
radiotherapy after one of these resections. On
this presentation of recurrence, a decision was
made to offer him intracystic treatment with
IFNa-2b. Although the typical pre-treatment
leakage test was positive, the planned treatment
decision was maintained following extensive
multi-disciplinary consult with both local and
international teams who advised that any
Khadiga Elfadil Ahmed Mohammed1, Kellie R Alleyne Mike1,
Jeannette Parkes2
Affiliations: 1Senior Resident (FC RadOnc SA), Department
of Radiation Oncology in Groote Schuur Hospital, Cape
Town, Western Cape, South Africa.; 2Head of Radiation
Oncology Unit, Consultant Oncologist, Department of
Radiation Oncology in Groote Schuur Hospital, Cape Town,
Western Cape, South Africa.
Corresponding Author: Dr. Khadiga Elfadil Ahmed
Mohammed, Contact address: South Africa, Western
Cape, Cape Town, LE32 Clinic, Groote Schuur Hospital,
Observatory, 7925; Tel: *27-21-404-4270; Fax: *27-21-4485707; Email: khadijaelfadil22@hotmail.com
Received: 13 June 2013
Accepted: 24 July 2013
Published: 01 December 2013
extravasation of IFNa-2b was likely innocuous.
During the course of the treatment the patient
demonstrated severe lethargy. Two weeks after
completing the full course he presented with
urinary incontinence, confusion and blood
investigations confirmed hypernatremia and
hyperthyroidism. Radiologic imaging showed
a stable cystic mass, worsening hydrocephalus
and extensive brain atrophy when compared
with pre-treatment imaging. Conclusion: The
potential for brain atrophy with intracystic IFNa2b has not been documented previously. While
we have found no evidence to support a causal
association between IFNa-2b administration
and neurotoxicity, the implications of such
toxicity warrant further investigation and careful
observation for similar events in the future.
Keywords: Craniopharyngioma, Intratumoral
interferon alpha, Brain atrophy, Neurotoxicity
*********
Mohammed KEA, Mike KRA, Parkes J. Unexpected
brain atrophy following administration of intratumoral
interferon alpha-2b for cystic craniopharyngioma: A case
report. International Journal of Case Reports and Images
2013;4(12):719–722.
*********
doi:10.5348/ijcri-2013-12-419-CR-13
Introduction
Craniopharyngiomas (CPs) are of pituitary embryonic
origin and are the third most common intracranial
tumor of childhood; most of these tumors are cystic in
nature. They are histologically benign, but can behave
aggressively due to their location. Surgery has been
the mainstay of treatment, but complete resection is
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):719–722.
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sometimes impossible without undue damage to vital
structures. Radiotherapy also has a role in unresectable
and partially resected disease as well as recurrent disease.
However, devastating side effects have been reported
following both surgical resection and radiotheraphy [1,
2].
The long-term morbidity of patients with CPs is
substantial and mainly involves endocrine, visual,
hypothalamic, neurobehavioral and cognitive sequelae.
This compromises normal psychosocial integration
and adversely affects quality of life. The disease course
is characterized by multiple recurrences prompting
repeated interventions, and management decisions can
become increasingly challenging as the adverse effects of
earlier treatments accumulate.
Mohammed et al. 720
The first cycle was given intratumorally at a dose
of 3,000,000 international units. This dose was then
administered three times a week for a total of four weeks.
During these treatment sessions, the patient exhibited
severe lethargy. Two weeks after completing the full
planned course he presented with confusion, urinary
incontinence and was found to have hypernatremia and
hyperthyroidism. Magnetic resonance imaging (MRI) of
the brain, at that point revealed significant brain atrophy,
striking volume loss with no significant change in the
tumor size and an increase in hydrocephalus (Figure
3A–B).
CASE REPORT
An eight-year-old boy was referred by an
ophthalmologist to the Pediatric Oncology Center
(Cape Town) in 2004. He presented with features of
hypopituitarism and bilateral optic atrophy. His imaging
studies showed a ring enhancing lesion with solid and
cystic components arising from the suprasellar area and
compressing the third ventricle (V3) with associated
hydrocephalus.
A subtotal resection and left subdural shunt was
performed at that time, and a histological diagnosis of CP
was made hormonal replacement was initiated.
A year later following his first recurrence the patient
was treated with repeat debulking resection and at this
time an Ommaya reservoir was placed. Further subtotal
resection to the growing lesion, followed by adjuvant
radiotherapy (54 Gy) was done the following year after he
had turned three years old.
At this point, multi-modality treatment allowed for
disease stability for another three years, when evidence
of disease progression was again noted. This prompted
surgical re-intervention and a debulking procedure was
again performed.
In June 2011, at the age of eight, radiological evidence
of tumor recurrence was again noted (Figure 1A–B)
and after multi-disciplinary discussion, treatment with
intracystic IFNa-2b was proposed as an alternative method
of gaining local control and thereby controlling disease
progression. A pre-treatment leakage test was done via
the Ommaya reservoir and this showed extensive leakage
around the catheter into the right ventricle (Figure 2).
There was thus concern over the potential for leakage
during IFNa-2b treatment and the clinical implications
of this were researched. The treating multi-disciplinary
team enlisted further advice from international colleagues
regarding the safety of IFNa-2b administration in such
a situation. Following this advice and the information
obtained through documented research, a decision was
made to proceed with the therapy as intra-ventricular
spillage of IFNa was considered to be innocuous.
Figure 1: (A, B) Showing the latest recurrence with a well
defined, complex suprasellar mass with both cystic and solid
components. Associated mass effect is noted in the ipsilateral
ventricle with midline shift.
Figure 2: Computed tomography scan of the brain showed
leakage of the contrast inside the ventricle during the leakage
test.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):719–722.
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Figure 3: (A, B) Showing post treatment generalized brain
parenchyma atrophy and residual tumor with associated
hydrocephalus.
The patient was admitted and his condition stabilized
following appropriate management. His hormonal
replacement medications were adjusted and a shunt was
inserted to drain the re-accumulating hydrocephalus.
He was seen again in the combined Pediatric NeuroOncology clinic a month later, and was clinically stable.
It was thus decided to forgo further IFNa-2b therapy due
to concern about further complications. He will thus be
followed at regular intervals for symptom control and
monitoring of his endocrine functions.
DISCUSSION
Craniopharyngioma is considered to be a benign
chronic condition that is subject to increasing symptoms
and deterioration as was observed in the current case
scenario. The intractable tumor growth and treatment
side effects lead to substantial long-term morbidity
and impaired quality of life for these patients. IFNa has
emerged as quite a promising therapy in the long-term
control of the disease. It appears to be far less neurotoxic
than the previously used intratumoral bleomycin, which
has been fatal in some cases, and its administration
requires less technical expertise than is required when
using beta emitting radionuclides such as yttrium [3].
IFNa is the 1st cytokine produced by recombinant
DNA for treatment of infection and malignant diseases.
It has an important role in the induction of apoptosis; cell
regulation and anti-proliferative effects [1, 3].
The first reports in the early 2000 by Jakacki et al.
looked at the safety and efficacy of systemic IFNa in
the treatment of CP. Tumors with a cardinal cystic
component appear to be more likely to respond to IFNa
than solid tumors. However, they reported significant
side effects, which led to the reduction or interruption of
the treatment [1].
Chamberlain et al. used IFNa as an intrathecal drug
for neoplastic meningitis, and they reported both safety
and feasibility of its administration into the circulating
cerebrospinal fluid system [4]. The side effects were
minimal and did not impede its continuation as in the
Mohammed et al. 721
case with systemic treatments.
Based on these reports, a Brazilian group treated nine
patients with intracystic IFNa-2b, empirically; 3,000,000
units of IFNa were injected intratumorally, three times a
week for four weeks [5]. The results were impressive, with
all the patients experiencing some degree of response and
reduction of the tumor volume at the end of the treatment
[5].
More recently the same Pediatric Neurosurgery
Department, documented findings of a multicentric
prospective study in which they used intracystic IFNa in 60
patients. The same doses were employed and the presence
of leakage was not considered a contraindication for the
treatment as there was no concern over neurotoxicity. In
this study 78.3% of the patients obtained tumor control
but 31.3% exhibited new endocrine dysfunction [6]. Of
note is that 30% experienced side effects (headache,
palpebral edema, fever, chronic fatigue syndrome and
arthritis), though this did not prevent the continuation
of the treatment.
Another retrospective study was undertaken to further
assess the effectiveness of intratumoral IFNa in cystic CP
[7]. Nineteen patients were reviewed, and in this study
there was also variable degree of tumor reduction with
treatment being well tolerated and no fatalities associated
with its administration.
Side effects commonly attributed to IFNa
administration are transient and include chemical
arachnoiditis, chronic fatigue syndrome, fever, loss of
weight, loss of appetite and behavioral changes. However,
no major neurotoxicity has even been identified [2, 6].
Most centers will perform a leakage/permeability
study prior to the start of intracystic treatment. Leakage
can develop from increased permeability of the cyst
during treatment or as a result of tumor shrinkage and
subsequent catheter exposure. Hence, even in cases
where the leakage test is initially negative, leakage of
an intracystic substance remains a potential risk as
the treatment progresses. Therefore, a non-neurotoxic
intracystic medication such as IFNa presents a safer
treatment option than bleomycin or radioisotopes and
can achieve impressive results, as has been shown in
several other patients treated in a similar manner at the
same site [2, 6].
The leakage test was positive in our patient and he was
treated with IFNa. Unfortunately, he developed cerebral
atrophy following this intracystic treatment which has
not been identified in any of the available literature to
date.
Treatment guidelines on the management of CPs are
still evolving due to the rarity of the disease and further
investigation is still needed. Due to the high incidence
of recurrence and the potential for significant treatment
related morbidity, much still needs to be done by way of
identifying innovative drugs that can change the course
of this disease.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):719–722.
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CONCLUSION
This is the first documented case of a possible IFNa
induced brain atrophy. Although a causal association
between IFNa administration and neurotoxicity has not
been elucidated, the gravity of the adverse effect warrants
further investigation of the mechanism of occurrence and
extreme vigilance for similar events in the future.
*********
Author Contributions
Khadiga Elfadil Ahmed Mohammed – Conception and
design, Acquisition of data, Analysis and interpretation
of data, Drafting the article, Critical revision of the article,
Final approval of the version to be published
Kellie R Alleyne Mike – Acquisition of data, Drafting the
article, Critical revision of the article, Final approval of
the version to be published
Jeannette Parkes – Analysis and interpretation of data,
Critical revision of the article, Final approval of the
version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Khadiga Elfadil Ahmed Mohammed et al. 2013; This
article is distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
Access full text article on
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Mohammed et al. 722
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1.
Jakacki RI, Cohen BH, Jamison C, et al. Phase II
evaluation of interferon-alpha-2a for progressive or
recurrent craniopharyngiomas. J Neurosurg 2000
Feb;92(2):255–60.
2. Bartels U, Laperriere N, Bouffet E, Drake J. Intracystic
therapies for cystic craniopharyngioma in childhood.
Front Endocrinol (Lausanne) 2012 Mar 27;3:39.
3. Steinbok P, Hukin J. Intracystic treatments for
craniopharyngioma.
Neurosurg
Focus
2010
Apr;28(4):E13.
4. Chamberlain MC. A phase II trial of intra-cerebrospinal
fluid alpha interferon in the treatment of neoplastic
meningitis. Cancer 2002 May 15;94(10):2675–80.
5. Cavalheiro S, Dastoli PA, Silva NS, Toledo S, Lederman
H, da Silva MC. Use of interferon alpha in Intratumoral
chemotherapy for cystic craniopharyngioma. Childs
Nerv Syst 2005 Aug;21(8-9):719–24.
6. Cavalheiro S, Di Rocco C, Valenzuela S, et al.
Craniopharyngiomas: Intratumoral chemotherapy
with interferon-alpha: A multicenter preliminary
study with 60 cases. Neurosurg Focus 2010
Apr;28(4):E12.
7. Dastoli PA, Nicácio JM, Silva NS, et al. Cystic
craniopharyngioma: Intratumoral chemotherapy
with alpha interferon. Arq Neuropsiquiatr 2011
Feb;69(1):50–5.
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IJCRI 2013;4(12):723–726.
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Fujii et al. 723
CASE reportOPEN ACCESS
Cholesterol granuloma of the breast suspected as breast
carcinoma
Takaaki Fujii, Reina Yajima, Hiroki Morita, Satoru Yamaguchi, Soichi
Tsutsumi, Takayuki Asao, Hiroyuki Kuwano
Abstract
Introduction: Cholesterol granuloma of the
breast is a rare benign condition which is
difficult to differentiate from breast cancer. Case
Report: We herein report the case of a 56-yearold Japanese asymptomatic female who, on a
computed tomography examination for sigmoid
colon cancer, was found to have a palpable
nodule in the upper external quadrant of the left
breast. Breast carcinoma could not be ruled out
on the basis of clinical and radiological findings,
but excisional biopsy revealed cholesterol
granuloma. A histological examination implied
that this case of cholesterol granuloma might
have been related with mammary duct ectasia.
Conclusion: Although cholesterol granuloma is
a rare disorder in the breast, this case indicated
that a better awareness of this benign breast
disease is important for avoiding misdiagnosis
and unnecessary surgery.
Keywords: Cholesterol
carcinoma, Ductal ectasia
granuloma,
Breast
Fujii T, Yajima R, Morita H, Yamaguchi S, Tsutsumi S,
Asao T, Kuwano H. Cholesterol granuloma of the breast
suspected as breast carcinoma. International Journal of
Case Reports and Images 2013;4(12):723–726.
*********
doi:10.5348/ijcri-2013-12-420-CR-14
Introduction
Cholesterol granuloma consists of fibrous granulation
tissue containing cholesterol crystals with foreign body
giant cells. Cholesterol granuloma occurs commonly in
the mastoid region and in various organs such as the
peritoneum, kidney, parotid gland, thyroglossal duct,
lymph node, liver, and spleen. However, cholesterol
granuloma of the breast is a very rare tumor-like lesion
and has been rarely reported. We herein report a rare
case of cholesterol granuloma of the breast. Cholesterol
granuloma should be considered in the differential
diagnosis of breast lesion suspected to be malignant [1–6].
*********
Takaaki Fujii1, Reina Yajima1, Hiroki Morita1, Satoru
Yamaguchi1, Soichi Tsutsumi1, Takayuki Asao1, Hiroyuki
Kuwano1
Affiliations: 1Department of General Surgical Science,
Graduate School of Medicine, Gunma University, Gunma,
Japan.
Corresponding Author: Takaaki Fujii, MD, PhD, Department
of General Surgical Science, Graduate School of Medicine,
Gunma University, 3-39-22 Showa-machi, Maebashi,
Gunma 371-8511, JAPAN; Ph: +81-027-220-8224; Fax:
+81-027-220-8230; Email: ftakaaki@med.gunma-u.ac.jp
Received: 20 June 2013
Accepted: 27 July 2013
Published: 01 December 2013
CASE REPORT
A 56-year-old Japanese asymptomatic female was
admitted to our hospital with a diagnosis of sigmoid colon
cancer. Computed tomography (CT) scan of her chest
and abdomen was done as a preoperative examination of
her colon cancer, and showed a nodule in the left breast
(Figure 1). The patient had no family history of breast
cancer, no history of mammary trauma or biopsy and no
history of lipid metabolism abnormality.
On admission, physical examination revealed a
2.0x2.0 cm palpable nodule which was elastically firm
and mobile in the upper external quadrant of the left
breast with no associated skin findings. There was no
axillary or supraclavicular lymphadenopathy, and no
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):723–726.
www.ijcasereportsandimages.com
abnormal nipple discharge. Mammography showed a
2.0 cm in diameter high-density shadow lesion, which
corresponded to the palpable mass, with partly ill-defined
margins and without specula or microcalcification
(Figure 2). An ultrasonography of the left breast revealed
a hypoechoic and relatively irregularly shaped mass with
an ill-defined margin (Figure 3). A core needle biopsy of
the lesion showed fibrosis and a small granulomatous
lesion infiltrated by lymphocytes and foamy histiocytes,
and there was no evidence of malignancy. From the
physical examination, CT scan, ultrasonographic findings,
a diagnosis of breast cancer could not be ruled out, and an
excisional biopsy was also done when the sigmoidectomy
was performed.
The surgical specimen measured 2.4 cm in diameter
and was a cystic and firm mass. The histological
evaluation revealed a cystic mass surrounded by fibrous
granulation tissue in which needle-like crystals were
deposited and accompanied by giant cells (Figure
4). These findings were compatible with cholesterol
granuloma. Our follow-up, the patient was remained
uneventful since 60 months.
Fujii et al. 724
Figure 1: Computed tomography scan of chest showing a nodule
in the left breast.
DISCUSSION
We herein report a rare case of cholesterol granuloma
of the breast. Cholesterol granuloma of the breast
is composed of fibrous granulation tissue in which
cholesterol crystals accompanied by giant cells are
deposited. The pathogenesis of cholesterol granuloma is
not fully understood. Physical and infectious factors have
been considered and some reports postulate that they are
caused by a reactive response to an unknown stimulus. In
the breast, cholesterol granuloma is thought to represent
a rare manifestation of duct ectasia. Lipid-rich material
is normally found in ectatic ducts. It is postulated that
leakage of cholesterol crystals from the ectatic ducts
causes a foreign body giant cell reaction which results
in granuloma formation. Mammary duct ectasia is a
disorder of middle-aged women. Mammary duct ectasia
is a frequent disorder of the breast that has a wide variety
of clinical manifestations, and this disorder sometimes
involves an extensive disruption of the dilated duct
and is occasionally noticed as a palpable mass. In rare
instances the mass, microscopically, contains cholesterol
crystals. In the pathologic specimen in this case, a
cystic lesion surrounded by fibrous granulation tissue,
chronic inflammation, cholesterol crystals, and scattered
multinucleated giant cells was observed. The lesion was
considered to be cholesterol granuloma associated with
duct ectasia [1–6].
The main concern with breast cholesterol granuloma is
performing differential diagnosis. Clinically, it resembles
breast cancer. The clinical and radiological features of
cholesterol granuloma mimic those of a carcinoma of
the breast. Thus, it is difficult to distinguish cholesterol
granuloma from carcinoma of the breast based on the
Figure 2: Mammography showing a 2.0 cm in diameter highdensity shadow lesion, which corresponded to the palpable
mass, with partly ill-defined margins and without specula or
microcalcification.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):723–726.
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Fujii et al. 725
Author Contributions
Figure 3: An ultrasonography of the left breast revealed a
hypoechoic and relatively irregularly shaped mass with an illdefined margin.
Takaaki Fujii – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Reina Yajima – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Hiroki Morita – Substantial contributions to conception
and design, Drafting the article, Final approval of the
version to be published
Satoru Yamaguchi – Substantial contributions to
conception and design, Drafting the article, Final approval
of the version to be published
Soichi Tsutsumi – Substantial contributions to conception
and design, Drafting the article, Final approval of the
version to be published
Takayuki Asao – Substantial contributions to conception
and design, Drafting the article, Final approval of the
version to be published
Hiroyuki Kuwano – Substantial contributions to
conception and design, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Guarantor
Figure 4: The histological evaluation revealed a cystic mass
surrounded by fibrous granulation tissue in which (A) needlelike crystals were deposited, (B) accompanied by giant cells
(H&E stain, 200x).
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
imaging results. The diagnosis of cholesterol granuloma
is histologic. It is not difficult to distinguish between
cholesterol granuloma and breast cancer histologically,
and core needle biopsy is thought to be useful for
differential diagnosis. However, it should be noted that
breast cholesterol granuloma could be accompanied by
cancer. Thus, cases in which malignancy cannot be ruled
out clinically, a complete removal of the tumor helps in
making a definitive diagnosis [1–6].
© Takaaki Fujii et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
REFERENCES
1.
CONCLUSION
We here reported a rare case of breast cholesterol
granuloma mimicking breast cancer. Reports on breast
granuloma are rare. However, duct ectasia itself,
which is related with cholesterol granuloma, is a very
frequent disorder. To make an accurate diagnosis of this
rare disorder and rule out the possibility of cancer, a
histological examination is required.
*********
Reynolds HE, Cramer HM. Cholesterol granuloma
of the breast: a mimic of carcinoma. Radiology
1994;191(1):249–50.
2. Osada T, Kitayama J, Nagawa H. Cholesterol
granuloma of the breast mimicking carcinoma: report
of a case. Surg Today 2002;32(11):981–4.
3. Garofalo S, Casolino C, Accurso A, Falleti J. Choesterol
granuloma of the breast with unusual ossification
features (osseous metaplasia). Pathol Res Pract
2008;204(5):353–6.
4. Smith GL, Hicks P, Wijesinghe DP, Holme TC.
Cholesterol granuloma of the breast presenting as an
intracystic papilloma. Br J Radiol 1997;70(839):1178–
9.
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IJCRI 2013;4(12):723–726.
www.ijcasereportsandimages.com
5.
Ishizaki M, Ohsumi S, Takashima S, Mandai K. Two
cases of cholesterol granuloma of the breast. Breast
Cancer 2001;8(2):158–61.
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Fujii et al. 6.
726
Furuhira C, Ohshima A, Shimada K, et al. A case of
breast cholesterol granuloma accompanied by cancer.
Breast Cancer 2004;11(2):210–3.
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IJCRI 2013;4(12):727–730.
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Sayed et al. CASE REPORT
727
OPEN ACCESS
Severely ectatic left circumflex coronary artery with
fistula to coronary sinus presenting with acute coronary
syndrome
Ahmed Sayed
Abstract
Introduction: Coronary artery fistulas are
uncommon anomalies. They occur in 0.1–0.8%
of patients undergoing coronary arteriography.
The circumflex coronary arteriovenous fistulae
(CAF) associated with aneurysmal dilatation
and drainage into coronary sinus (CS) are even
less common. We present a case of a 56-yearold male with an acute coronary syndrome who
was found to have a tortuous and aneurysmal
circumflex coronary artery terminating into the
CS. Case report: A previously healthy 56-yearold male developed acute retrosternal chest pain
while skiing. Physical examination revealed a
grade II/VI continuous murmur at the left lower
sternal border, but was otherwise unremarkable.
Laboratory investigations revealed an elevated
troponin and ST depression in the inferior
leads on his electrocardiography. Coronary
angiography showed a diffusely aneurysmal
circumflex artery with a fistula to the CS. The
patient underwent successful surgical ligation
of the fistula. Conclusion: Our patient presented
with an acute coronary syndrome caused by
an uncommon form of coronary artery fistula.
Conventional coronary angiography failed to
fully define the anatomy of the fistula (origin,
pathway, and outflow). Multidetector computed
tomography was complementary to demonstrate
the complex anatomy of the fistula. The fistula
was surgically ligated and he remains well.
Ahmed Sayed
Affiliations: MD, Department of Cardiology, University of
Calgary, Calgary, Canada.
Corresponding Author: Ahmed Sayed, MD, Department of
Cardiology, University of Calgary, Calgary, Canada; Email:
sayed326@yahoo.com
Received: 05 July 2013
Accepted: 05 August 2013
Published: 01 December 2013
Keywords: Coronary artery fistula, Coronary
artery angiogram, Acute coronary syndrome
*********
Sayed A. Severely ectatic left circumflex coronary artery
with fistula to coronary sinus presenting with acute
coronary syndrome. International Journal of Case
Reports and Images 2013;4(12):727–730.
*********
doi:10.5348/ijcri-2013-12-421-CR-15
Introduction
An aneurysmal left circumflex coronary artery (LCX)
with associated fistulous connection to the coronary
sinus (CS) is an extremely uncommon coronary artery
anomaly. A few patients have been reported in literature.
The majority of these cases were asymptomatic and
presented late in life. Accurate diagnosis of these fistulas
often requires multiple imaging modalities to delineate
the entire pathway of the fistula. Multiple treatment
options are available including surgery, trans catheter
closure, and conservative management.
CASE REPORT
A 56-year-old male, who was on a skiing trip, was
presented with sudden onset of retrosternal chest
pressure with shortness of breath and diaphoresis.
There was no family history of congenital heart disease
or other inherited abnormalities. Physical Examination
revealed normal vital signs and a grade II continuous
murmur loudest at the left sternal border in the fourth
intercostal space. The electrocardiography showed
inferior ST segment depression and his high sensitivity
troponin-T was 417 ng/L. A posteroanterior and
lateral X-ray of chest showed only mild cardiomegaly.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
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Sayed et al. 728
Transthoracic echocardiography (TTE) showed a dilated
structure in the atrioventricular groove and there was
mild hypokinesis of the basal inferior and inferolateral
walls of the left ventricle. The right atrium was mildly
enlarged. Coronary angiography revealed a tortuous
and ectatic left circumflex artery with a large arteriovenous fistula draining into the CS without evidence
of atherosclerothic narrowing of the coronary arteries
(Figure 1). The computed tomography (CT) angiography
confirmed the fistula between circumflex artery and the
CS (Figure 2A, B). The patient was initially treated as an
acute coronary syndrome with ASA, Plavix, enoxaparin in
addition to an ACEi and a statin. Following diagnosis of
the congenital coronary fistula, he underwent successful
surgical ligation. He was subsequently discharged home
on ASA, a statin and an ACEi.
DISCUSSION
Coronary arteriovenous fistulas (CAFs) are considered
a major coronary anomaly [1], with an incidence of 0.1%
to 0.87% [2, 3]. The majority of cases originate from the
right coronary and left anterior descending coronary
arteries. Coronary artery fistulas originating from the
circumflex coronary artery account for only 18% of all
coronary arterial (venous) fistulas [3]. More than 90% of
the fistulas drain into the right side of the heart with the
most common sites of drainage being the right ventricle,
right atrium, and pulmonary artery [4, 5]. Rarely, they
drain into the CS in 7% and the superior vena cava in
1% of cases [4]. A fistula originating from the circumflex
artery that drains into the coronary sinus is rare. In one
literature review a total of 14 cases of LCX to CS fistula
were reported [6]. Most of these cases were found
incidentally in male and most common presentation
was either dysnpnea on exertion or chest pain, rarely
can remain asymptomatic. Two cases involved inferior
and inferolateral myocardial infarction related to the
CAF with patent epicardial coronary arteries [6]. The
dilation of the coronary artery may relate to prolonged
hyperdynamic circulation from the fistula.
Our patient presented in his late fifties. He was
healthy with no limitation in physical activity. His initial
presentation was one of an acute coronary syndrome
which was confirmed by his electrocardiography and
troponin abnormalities. His symptoms were likely
secondary to significant shunt-induced ischemia triggered
by increased physical activity during skiing. The diagnosis
of the fistula in our patient was established by invasive
coronary angiography and CT coronary angiography.
The management of coronary arteriovenous fistulas
remains controversial. There are significant differences
in the clinical presentation, size and anatomical
connection of coronary artery fistulas, which has led to
a lack of consistent guidelines for treatment [7]. In the
most recent American College of Cardiology/American
Heart Association Guidelines for the Management of
Figure 1: Coronary angiogram showing: dilated LCX artery
and the fistula to the CS. RA (right atrium).
Figure 2: (A, B) Three-dimensional computed tomography
image showing the ectatic LCX, fistula to the CS and the blood
blush to the RA. LM (left main artery).
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):727–730.
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Sayed et al. Adults with Congenital Heart Disease [8], percutaneous
or surgical closure is a Class I recommendation for
large fistulae regardless of symptoms and for smallto moderate-size fistulae with evidence of myocardial
ischemia,
arrhythmia,
ventricular
dysfunction,
ventricular enlargement or endarteritis. The optimal
treatment modality for a patient depends on their age,
the morphology and size of the fistula and the presence
or absence of associated heart disease. Our patient was
a candidate for surgical intervention due to the presence
of symptoms of ischemic heart disease because he had a
large, tortuous aneurysm. As in the previously reported
cases, the fistula in our case was successfully ligated at
the origin and at the site of termination into the CS.
CONCLUSION
Coronary artery fistulas are uncommon and can have
different presentations ranging from asymptomatic to
acute coronary syndromes. The management of coronary
artery fistulae depends on the severity and the clinical
presentation of these congenital anomalies.
*********
Author Contributions
Ahmed Sayed – Substantial contributions to conception
and design, Acquisition of data, Analysis and
interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Guarantor
The corresponding
submission.
author
is
the
guarantor
of
Conflict of Interest
Authors declare no conflict of interest.
729
REFERENCES
1.
Ogden JA. Congenital anomalies of the coronary
arteries. Am J Cardiol 1970 Apr;25(4):474–9.
2. Angelini P, Villason S, Chan AV Jr, et al. Normal and
anomalous coronary arteries in humans. In Angelini
P (ed): Coronary Artery Anomalies: A Comprehensive
Approach. Lippincott Williams & Wilkins, Baltimore,
1999, pp. 27–79.
3. Fernandes ED, Kadivar H, Hallman GL, Reul GJ,
Ott DA, Cooley DA. Congenital malformations of the
coronary arteries: The texas heart institute experience.
Ann Thorac Surg 1992 Oct;54(4):732–40.
4. Dodge-Khatami A, Mavroudis C, Backer CL.
Congenital heart surgery nomenclature and database
project: Anomalies of the coronary arteries. Ann
Thorac Surg 2000 Apr;69(4 Suppl):S270–97.
5. Darwazah AK, Hussein IH, Hawari MH. Congenital
circumflex coronary arteriovenous fistula with
aneurysmal termination in the pulmonary artery. Tex
Heart Inst J 2005;32(1):56–9.
6. Said SA, van der Sluis A, Koster K, Sie H, Shahin GM.
Congenital circumflex artery-coronary sinus fistula
in an adult female associated with severe mitral
regurgitation and myelodysplasy--case report and
review of the literature. Congenit Heart Dis 2010
Nov-Dec;5(6):599–606.
7. Angelini P. Coronary fistulae. Which ones deserve
treatment, and what kind of treatment do they need?
Tex Heart Inst J 2007;34(2):202–3.
8. Warnes CA, Williams RG, Bashore TM, et al. ACC/
AHA 2008 guidelines for the management of adults
with congenital heart disease: a report of the American
College of Cardiology/American Heart Association
Task Force on Practice Guidelines (Writing
Committee to Develop Guidelines on the Management
of Adults With Congenital Heart Disease). Developed
in Collaboration With the American Society
of Echocardiography, Heart Rhythm Society,
International Society for Adult Congenital Heart
Disease, Society for Cardiovascular Angiography and
Interventions, and Society of Thoracic Surgeons. J
Am Coll Cardiol 2008 Dec 2;52(23):e143–263.
Copyright
© Ahmed Sayed 2013; This article is distributed under
the terms of Creative Commons attribution 3.0 License
which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
ACKNOWLEDGEMENTS
I would like to thank Dr. Wardal for the time and
effort that he spent in the critical revision of this article.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):727–730.
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730
IJCRI 2013;4(12):731–734.
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Sarkar et al. CASE REPORT
731
OPEN ACCESS
Bronchogenic cyst: A case report
Aniruddha Sarkar, Narayan Pandit
Abstract
Introduction
Introduction:
Bronchogenic
cyst
is
an
incidentally detected mediastinal mass which is
usually asymptomatic in most of the cases. The
bronchogenic cyst appears to be a well-defined,
nonenhancing cystic mass with peripheral
calcification and presence of milk of calcium in
some cases. It may be said congenital if it detected
in very early age group. Case Report: A case of
bronchogenic cyst was incidentally detected by
computed tomography scan in a 35-year-old
male presenting with the heaviness in the chest.
Conclusion: The case is reported here to give an
emphasis on its formation and mode of treatment
accordingly due to its so hazardous position in
the mediastinum.
Bronchogenic cysts result from the abnormal or late
budding of the ventral lung bud or the tracheobronchial
tree during the process of the development. Most of the
bronchial branches are formed within the 15th week of
development in fetal life, but they continue to divide
and completed in eighth year. Bronchogenic cysts can be
detected in fetus or in stillbirths and it is well recognized
in babies or infants. But mediastinal type bronchogenic
cysts could not be clinically detected usually until adult
life [1]. Bronchogenic cysts accounts for 10–15% of all
primary mediastinal masses, and can be classified as
either intrapulmonary or mediastinal. Overall, 72% of
bronchogenic cysts produce some symptoms, but 90%
of mediastinal type bronchogenic cysts are reported to
be asymptomatic. Mediastinal type bronchogenic cysts
are classified into five types: paratracheal, carinal, hilar,
paraoesophageal, or miscellaneous [2]. The paratracheal
or carinal types can produce symptoms such as dyspnea or
chest pain, due to compression of the trachea or bronchi. A
giant carinal type of mediastinal type bronchogenic cysts
can compress the left atrium of heart due to its proximity
to the heart. The cyst is lined by the ciliated, secratory
respiratory epithelium with cartilage, smooth muscle,
fibrous tissue and mucous glands. The bronchogenic
cyst may be filled with fluid or air or both according to
the communication with the tracheobronchial tree [3].
The cysts are usually asymptomatic or it may produce
pressure symptoms to the surrounding structures. The
patient might complain of heaviness in chest especially
on exertion and the electrocardiogram can reveal left
atrial overload [1]. The echocardiography and computed
tomographic (CT) scan can find out the exact cause of the
atrial overload. The most common radiological position is
in the subcarinal region but it can affect any compartment
of the mediastinum or even within the lung. On CT, the
cysts are well-defined, rounded, nonenhancing masses.
The CT appearance of the fluid can vary from water density
to higher density according to secretion type. Some
bronchogenic cysts may have flecks of calcium within the
fluid, the so-called milk of calcium. Magnetic resonance
imaging (MRI) appearance may vary according to the
Keywords: Bronchogenic cyst, Mediastinal mass
*********
Sarkar A, Pandit N. Bronchogenic cyst: A case report.
International Journal of Case Reports and Images
2013;4(12):731–734.
*********
doi:10.5348/ijcri-2013-12-422-CR-16
Aniruddha Sarkar1, Narayan Pandit2
Affiliations: 1MD, Associate Professor, Department of
Anatomy, Midnapore Medical College, Midnapore,
West Bengal, India; 2Associate Professor, Department
of Radiodiagnosis, North Bengal Medical College,
Sushrutanagar, Siliguri, West Bengal, India.
Corresponding Author: Dr. Aniruddha Sarkar, 48, Sreenagar
Main Road, Kolkata-700094, West Bengal, India; Mob: (+91)
9474474468; Email: drani77@gmail.com
Received: 12 July 2013
Accepted: 03 August 2013
Published: 01 December 2013
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):731–734.
www.ijcasereportsandimages.com
Sarkar et al. 732
nature of the fluid of the cyst, low (grey to black) or high
(white to grey) signal intensity in T1-wieghted imaging,
and bright (white) signal intensity in T2-weighted
imaging. In T1-weighted images, fat, proteinaceous and
hemorrhagic fluids appear in white; and water appears in
low signal intensity. In T2-weighted images water, with
or without proteins, appears in high signal intensity [4].
CASE REPORT
A 35-year-old male patient was presented with sense
of heaviness in chest and shortness of breath even at
rest for past two years. He gave history of intermittent
low grade fever, cough, right sided pain chest since his
adolescence and was frequently treated with antibiotics.
Chest X-ray showed a right sided mediastinal mass with
ipsilateral peripheral pleural calcification in the mid/
lower zone. Electrogram and echocardiography were
within normal limits. The CT scan of thorax showed a
right sided posterior mediastinal, unilocular, cystic mass
lesion having diameter of 10.9x8 cm with a homogeneous,
increased fluid attenuation value of +15 HU (Figure 1). On
contrast study the cyst was thin-walled, nonenhancing
except a small part of its right edge suggesting focal
thickening of inflamed mediastinal pleura or compressed
part of lung adjacent to the cyst (Figure 2). A fluid was
found at its dependent part, both in supine and in prone
position due to presence of a small amount of higher
density fluid (Figure 1, Figure 2 and Figure 3). A focus
of calcification was found in a part its periphery (Figure 3).
Absence of air within the cyst proved that the cyst was
noncommunicating. Right intermediate bronchus was
partially compressed and slightly displaced by the mass
(Figure 4). Associated ipsilateral periopheral pleural
calcification was present. The CT guided aspiration
revealed its content to be whitish, opalescent fluid with
high protein, epithelial cells, amorphous calcium. Culture
report was negative. Fibreoptic bronchoscopy revealed
mucosal thickening and mild narrowing of lumen of right
intermediate bronchus due to extrinsic compression.
Bronchoscopic biopsy specimen revealed inflammatory
changes and no malignant cell was detected.
Figure 1: Axial computed tomography scan of thorax at the level
of aortic root; mediastinal window, supine position.
Figure 2: Axial computed tomography scan of thorax at the level
of ventricles; mediastinal window, prone position.
DISCUSSION
Bronchogenic cysts are the cystic malformations which
are having the respiratory epithelial lining. As it is a space
occupying lesion in the mediastinum, so the appearance
of the symptoms of patients having the bronchogenic
cysts depend on the position and most importantly the
size of the tumor. As they are enlarge, they may produce
symptoms by compression of the surrounding structures.
A few of the cysts might become infected and can rupture
into the nearest bronchus causing mucopurulent sputum,
hemoptysis and fever. Bronchogenic cysts are usually
asymptomatic in early lives. Bronchogenic cysts can be
suspected in early childhood having noisy breathing,
cough, dysphagia, anorexia and a prompt CT scan can
conclude the diagnosis. When the diagnosis is made with
the certainty of the bronchogenic cysts, it will become very
important whether the cysts should be operated. Khalil et
al. suggested surgical excision should be preferred because
of its future complications but according to Mawatari et
al. the treatment should depend on the size and location
of the cyst and adherence with the surrounding structures
[2, 5]. The small cyst, carinal in location and free from
the surrounding structures could be operated as soon
as it is diagnosed before creating complications. But the
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):731–734.
www.ijcasereportsandimages.com
Sarkar et al. 733
CONCLUSION
The bronchogenic cysts are rare mediastinal mass
which are very much important in treatment point
of view when it is diagnosed. There are a few cases of
bronchogenic cysts have been reported till now. The case
is presented herein with a hope that it will open new way
of better treatment of such developmental mass having a
knot of complications which is tightening with the delay
of its removal.
*********
Author Contributions
Figure 3: Computed tomography scan guided aspiration of
bronchogenic cyst; needle in situ, mediastinal window, prone
position.
Aniruddha Sarkar – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Narayan Pandit – Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Aniruddha Sarkar et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
REFERENCES
Figure 4: Axial computed tomography scan of thorax at the
level of carina; mediastinal window, prone position.
operation sometimes become complicated as there may
adhesions with surrounding structures. So, the complete
separation of the cyst from the surrounding structures
becomes impossible. The remnant of the cyst can either
flare up or can create problem later. So, conservative
treatment should be preferred and excision of the cyst
will be chosen when it is creating complications such
as fistula formation, bronchial ulceration, bleeding or
infection.
1.
Schmidt FE, Drapanas T. Congenital cystic lesions
of the bronchi and lungs. Ann Thorac Surg 1972
Dec;14(6):650-7.
2. Mawatari T, Itoh T, Hachiro Y, et al. Large bronchial
cyst causing compression of the left atrium. Ann
Thorac Cardiovasc Surg 2003 Aug;9(4):261-3.
3. Harvey S. Mediastinum. In: Computed Body
Tomography with MRI correlations (Joseph KT., Lee
MD., et al, eds). Lippincott Williams and Wilkins,
Philadelphia 1998 Vol. 1, 271-72.
4. Tarver RD, Conces DJ. Mediastinal disease. In:
Computed Tomography and Magnetic Resonance
Imaging of the whole body (Hagga R.,ed). Mosby,
London 1994:756-7.
5. Khalil A, Carette MF, Milleron B, Grivaux M, Bigot
JM. Bronchogenic cyst presenting as mediastinal
mass with pleural effusion. Eur Respir J 1995
Dec;8(12):2185-7.
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Katsuragi et al. CASE REPORT
735
OPEN ACCESS
Reversible cerebral vasoconstriction syndrome in HELLP
syndrome
Shinji Katsuragi, Masato Osaki, Rieko Suzuki, Tomoaki Ikeda,
Kazunori Toyoda, Jun Yoshimatsu
Abstract
Introduction:
Reversible
cerebral
vasoconstriction syndrome (RCVS) is characterized
by severe and hyperacute headaches with or
without additional focal neurological deficits,
and evidence of vasoconstriction of cerebral
arteries which resolves spontaneously within
1–3 months. However, the precise mechanism of
RCVS is not fully understood. Case Report: We
report a 33-year-old postpartum woman with
RCVS with HELLP syndrome and eclampsia.
Magnetic resonance angiography (MRA) of
head revealed transient segmental stenosis and
dilatation. Especially, most part of the middle
cerebral artery (MCA) was irregularly thinner
at the acute phase than have ever previously
reported. Transcranial color flow imaging
(TCCFI) showed the highest peak systolic velocity
of middle cerebral artery that has ever reported
in RCVS cases (330 cm/s). Histologically, the
large areas that showed cytotoxic edema on
magnetic resonance imaging at acute phase,
which indicated possible infarction at the chronic
Shinji Katsuragi1, Masato Osaki2, Rieko Suzuki3, Tomoaki
Ikeda4, Kazunori Toyoda5, Jun Yoshimatsu6
1
Affiliations:
MD,
Chirman,
Obstetric
Cardiology,
Sakakibara Heart Institute, Fuchu, Tokyo, Japan; 2MD,
Resident, Cerebrovascular Medicine, National Cerebral
and Cardiovascular Center, Suita, Osaka, Japan; 3MD,
Staff, Cerebrovascular Medicine, National Cerebral and
Cardiovascular Center, Suita, Osaka, Japan; 4MD, Professor,
Obstetrics and Gynecology, Mie University, Tsu, Mie, Japan;
5
MD, Chirman Cerebrovascular Medicine, National Cereral
and Cardiovascular Center, Suita, Osaka, Japan; 6MD,
Chairman, Perinatology and Gynecology, National Cerebral
and Cardiovascular Center, Suita, Osaka, Japan.
Corresponding Author: Shinji Katsuragi, MD, 3-16-1 AsahiCho, Fuchu, Tokyo, JAPAN. 183-0003; Email: skatsura12@
yahoo.co.jp; Tel: +42-314-3111; Fax: +42-314-3150
Received: 22 July 2013
Accepted: 27 August 2013
Published: 01 December 2013
stage, have eventually become normal in chronic
stage. Conclusion: This RCVS case occurred to
a postpartum woman in HELLP syndrome after
eclampsia. The peak velocity of MCA measured
by TCCFI was the highest so far ever reported in
RCVS cases, and vasoconstriction on MRA was
severe. However, the severe vasoconstriction did
not last long enough to cause infarction in brain
tissue in this case. TCCFI also shows the steep
decline of the peak velocity of MCA after acute
phase and through this case we reconfirm the
usefulness of TCCFI as this bed-side test reflects
quite well the changes of vasoconstriction in
severe RCVS case.
Keywords:
RCVS
(Reversible
Cerebral
Vasoconstriction Syndrome), Pregnancy, TCCFI
(Transcranial color flow imaging), peak systolic
velocity
*********
Katsuragi S, Osaki M, Suzuki R, Ikeda T, Toyoda K,
Yoshimatsu J. Reversible cerebral vasoconstriction
syndrome in HELLP syndrome. International Journal of
Case Reports and Images 2013;4(12):735–738.
*********
doi:10.5348/ijcri-2013-12-423-CR-17
Introduction
Reversible cerebral vasoconstriction syndrome
(RCVS) is characterized by severe headaches, often
thunderclap headaches, with or without focal deficits
and seizures, and a multifocal constriction of cerebral
arteries, which resolve spontaneously within three
months. This syndrome has a female preponderance
and a mean age of onset of approximately 42 years [1,
2]. The pathophysiology of RCVS is not fully understood.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):735–738.
www.ijcasereportsandimages.com
Katsuragi et al. 736
Here, we report a case of RCVS in a patient with HELLP
syndrome who developed an eclamptic attack and had
caudate hemorrhage. The time-course of reversible
vasoconstriction was documented by magnetic resonance
angiography (MRA), and transcranial color flow imaging
(TCCFI).
CASE REPORT
A 33-year-old primipara women was referred to
our hospital because of severe headache on third day
postpartum following vaginal delivery at 37th week.
Her delivery course at a previous hospital was eventful
because she developed an eclamptic attack just before
delivery and was diagnosed with HELLP syndrome based
on findings of hemolysis, elevation of liver enzymes
(AST/ALT: 1455/663 U/L), and a low platelet count
(40×103/μL). Her blood pressure on tonic-clonic seizure
was 192/108 mmHg. On admission to our hospital,
the patient was alert and conscious, but had severe
headache over the temples with a positive Kernig’s sign.
Computed tomography of head showed hemorrhage in
the left caudate nucleus and magnetic resonance imaging
(MRI) of the corpus callosum on both sides showed fluid
attenuated inversion recovery (FLAIR) and diffusionweighted image (DWI) hyperintensity, and apparent
diffusion coefficient (ADC) map hypointensity. The
anterior cerebral arteries (ACA) and middle cerebral
arteries (MCA) were barely detectable by brain MRA
(Figure 1A). Therapy for brain edema and continuous
intravenous infusion of nicardipine were performed as
conservative management.
On hospital day 2, TCCFI indicated MCA peak
systolic velocities (PSV) of 330 (right side) and 266 (left
side) cm/s (Figure 2). On hospital day 3, nicardipine
was discontinued and replaced by oral administration
of a calcium blocking agent, which maintained blood
pressure at approximately 130/80 mmHg. Headache
was also relieved from hospital day 7. TCCFI on hospital
day 13 showed that the PSV of both MCAs had improved
to approximately 150 cm/s. MRA on hospital day 14
and at 43rd day postpartum revealed progressive
improvement of the lesions in the ACAs and MCAs
(Figure 1B–C). On hospital day 17, cerebral angiography
showed improvement of segmental changes of the ACA
and a larger diameter of the MCA compared to that in
previous tests (Figure 3). MRI scan of head at 43rd day
postpartum showed decreased caudate hemorrhage and
disappearance of the lesions in the corpus callosum.
DISCUSSION
The pathophysiology of eclampsia is thought to involve
vasoconstriction [3]. The patient in this case developed
juvenile cerebral hemorrhage during puerperium, and
the observation of multiple reversible stenoses and
Figure 1: Magnetic resonance angiography in hospital (A) Day 1:
Horizontal portions of the bilateral middle cerebral artery were
severely restricted (arrow) and poorly visualized, and several
segmental stenoses were present in the anterior cerebral artery
(arrowhead). (B) Day 14, and (C) at 43rd day after delivery show
horizontal portions of both middle cerebral arteries (arrow)
were much better visualized, and no segmental stenosis was
observed in the anterior cerebral artery (arrowhead).
Figure 2: Middle cerebral artery peak systolic velocities (MCAPSV) measured by transcranial color flow imaging. Both right
and left MCA-PSVs were severely increased on hospital days
2-3, but had gradually decreased to an almost normal range
by hospital day 13. The gray area indicates the normal velocity
range (100-130 cm/s).
Figure 3: Cerebral angiography (A) On hospital day 3, and (B)
On hospital day 17. (A): Segmental stenosis and dilatation at
the origin of the A2 segment of the anterior cerebral arteries
(arrow head). The middle cerebral arteries was constricted, but
neither focal stenosis nor dilatation was observed (arrow). (B)
Segmental changes of anterior cerebral arteries were improved
and the diameter of the middle cerebral arteries was larger than
that in the earlier test.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):735–738.
www.ijcasereportsandimages.com
the absence of findings suggesting vasculitis indicated
the presence of reversible cerebral vasoconstriction
syndrome (RCVS). Calabrese et al. suggested that the
important factors for diagnosis of RCVS are as follows [1]:
(i) angiography, CTA, or MRA shows the presence of
multifocal, segmental cerebral vasoconstriction
(ii) the absence of evidence of subarachnoid
hemorrhage from a ruptured aneurysm
(iii) near normal results in examination of
cerebrospinal fluid
(iv) the presence of severe acute headache
(v) reversibility of cerebral vasoconstriction findings
(normalization within 12 weeks).
Reversible cytotoxic edema and cerebral hemorrhage
were observed in our patient. In MRI, the corpus
callosum showed FLAIR and DWI, and hypointensity
on an ADC map in DWI, which indicated development
of cytotoxic edema that was probably due to RCVS, since
segmental stenosis and dilatation were found in the ACA,
which supplies the corpus callosum. Of further interest,
the lesions in the corpus callosum eventually almost
disappeared, although they were relatively widely spread,
which indicated possible infarction at the chronic stage.
This unusual phenomenon may be explained as follows.
A sudden increase of blood pressure in eclamptic seizure
during delivery may have increased the cerebral blood
flow volume [3] and then administration of ergometrine
and packed red blood cell transfusion triggered
vasoconstriction [1, 2]. Under these circumstances, blood
perfusion to the ACA and MCA areas may have suddenly
increased and then decreased. This vasoconstriction
occurred suddenly and intensively enough to cause
cytotoxic edema, but did not persist for long enough to
result in irreversible tissue damage.
The measurement of MCA blood flow velocity in
patients with RCVS can be achieved with TCCFI, which
is a combination of transcranial color Doppler imaging
and transcranial Doppler sonography [5, 6]. Recent
developments in diagnostic equipment have led to
significant upgrades of TCCFI, which is now widely used
in clinical settings [7–9]. The mean blood flow velocity of
the right and left MCAs in our patient were quite rapid,
at 275 and 249 cm/s, respectively, and much higher than
that found in a prospective study in 32 RCVS patients
in Taiwan [10], in which no subjects had a blood flow
velocity >200 cm/s. This is well supported by the severely
constricted MCA on MRA at admission. Improvement
of the MCA–PSV was coincident with the improvement
of blood flow in the MCA observed by angiography and
MRA. This indicates that TCCFI can be used to monitor
the therapeutic effect of drugs used for treatment of
RCVS.
This case shows that the cerebral circulation
changes dramatically after an eclamptic seizure attack.
It is of particular interest in this case that cytotoxic
edema disappeared with time. Posterior reversible
encephalopathy syndrome (PRES) is another possible
cause of eclampsia [3] that is mainly confined to the
Katsuragi et al. 737
occipital lobe, but does not, necessarily, involve a
severe headache such as that in RCVS. Based on our
observations in this case, we hypothesize that certain
regions of cytotoxic edema related to eclampsia may
also be reversible encephalopathy, similarly to PRES,
even though the etiology of PRES is vasogenic edema
and completely differs from RCVS. This hypothesis
requires testing by physical examinations, blood pressure
measurements, and longitudinal investigations using
MRI, MRA, angiography and TCCFI in future cases. The
results of these tests will provide further important clues
to understanding of the mechanism of eclampsia.
CONCLUSION
The peak velocity of middle cerebral artery by
transcranial color flow imaging was the highest in
reversible cerebral vasoconstriction syndrome cases
that have ever reported, and large areas of cytotoxic
edema disappeared in the chronic stage. These facts
and magnetic resonance angiography/angiography
reflects the vasoconstriction was most severe but rapidly
normalized which was clearly shown by transcranial color
flow imaging. These results further support the idea that
transcranial color flow imaging demonstrates the rapid
change of vasoconstriction even in the most acute type of
reversible cerebral vasoconstriction syndrome.
*********
Author Contributions
Shinji Katsuragi – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Masato Osaki – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Rieko Suzuki – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Tomoaki Ikeda – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Kazunori Toyoda – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Jun Yoshimatsu – Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):735–738.
www.ijcasereportsandimages.com
Guarantor
Katsuragi et al. 4.
The corresponding author is the guarantor of submission.
Conflict of Interest
5.
Authors declare no conflict of interest.
Copyright
© Shinji Katsuragi et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
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Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB.
Narrative review: Reversible cerebral vasoconstriction
syndromes. Ann Intern Med 2007 Jan 2;146(1):34–4.
Ducros A, Fiedler U, Porcher R, Boukobza M, Stapf
C, Bousser MG. Hemorrhagic manifestations of
reversible cerebral vasoconstriction syndrome:
Frequency, features, and risk factors. Stroke 2010
Nov;41(11):2505–11.
Gilbert JS, Ryan MJ, LaMarca BB, Sedeek M, Murphy
SR, Granger JP. Pathophysiology of hypertension3
during preeclampsia: linking placental ischemia with
endothelial dysfunction. Am J Physiol Heart Circ
Physiol 2008 Feb;294(2):541–50.
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6.
7.
8.
9.
10.
738
Hinchey J, Chaves C, Appignani B, et al. A reversible
posterior leukoencephalopathy syndrome. N Engl J
Med 1996 Feb 22;334(8):494–500.
Martin PJ, Evans DH, Naylor AR. Transcranial colorcoded sonography of the basal cerebral circulation.
Reference data from 115 volunteers. Stroke 1994
Feb;25(2):390–6.
Krejza J, Mariak Z, Walecki J, Szydlik P, Lewko
J, Ustymowics A. Transcranial color Doppler
sonography of basal arteries in 182 healthy subjects:
age and sex variability and normal reference values
for blood flow parameters. AJR Am J Roentgenol
1999 Jan;172(1):213–8.
Baumgartner RW, Mattle HP, Schroth G. Assessment
of >/=50% and <50% intracranial stenoses by
transcranial color-coded duplex sonography. Stroke
1999 Jan;30(1):87–92.
Kimura K, Yasaka M, Wada K, Minematsu K,
Yamaguchi T, Otsubo R. Diagnosis of middle cerebral
artery stenosis by transcranial color-coded real-time
sonography. AJNR Am J Neuroradiol 1998 NovDec;19(10):1893–6.
Ogata T, Kimura K, Nakajima M, Naritomi H,
Minematsu K. Diagnosis of middle cerebral artery
occlusive lesions with contrast-enhanced transcranial
color-coded real-time sonography in acute stroke.
Neuroradiology 2005 Apr;47(4):256–2.
Chen SP, Fuh JL, Chang FC, Lirng JF, Shia BC, Wang
SJ. Transcranial color Doppler study for reversible
cerebral vasoconstriction syndromes. Ann Neurol
2008 Jun;63(6):751–7.
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IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):739–741.
www.ijcasereportsandimages.com
Gonzalez et al. CASE REPORT
739
OPEN ACCESS
Air bubble in the heart: An unrecognized complication of
IV contrast injector
Carlos Gonzalez, Dioben Aquino, Balavenkatesh Kanna
Abstract
Introduction:
Venous air embolism is a
predominantly iatrogenic complication that
occurs when atmospheric gas is introduced
into the systemic venous system. The incidence
of venous air embolism associated with the
injection of contrast for computed tomography
scan reportedly is 11–23%. Yet, most patients are
asymptomatic, it can be fatal if the amount of
air is enough to cause hemodynamic instability.
Case report: We report a case of a 20-year-old
female patient who presented with air bubbles
in the right ventricle after an IV contrast
computed tomography of the abdomen. Venous
air embolism is a potential complication after an
IV contrast injection. Conclusion: We conclude
that is imperative that clinicians pay special
attention to the prevention, association, and
early recognition of venous air embolism when
performing imagines studies with IV contrast.
Keywords: Venous air embolism, Intravenous
contrast injector
Carlos Gonzalez1, Dioben Aquino2, Balavenkatesh Kanna3
Affiliations: 1MD: PGY-2 Internal Medicine, Lincoln Medical
and Mental Health Center of Weill, NY; 2MD: PGY-3 Internal
Medicine, Lincoln Medical and Mental Health Center of Weill,
NY; 3MD, MPH: Attending physician, associated program
director, Department of Internal Medicine, Lincoln Medical
and Mental Health Center of Weill, NY.
Corresponding Author: Carlos Gonzalez, MD, Department
of Internal Medicine, Lincoln Medical and Mental Health
Center, 234 E. 149th St #8-32, Bronx, NY 10451; Tel:
7185794739-9172665705; Fax: 7185794836; E-mail:
carlosgonzalez0521@yahoo.com,
carlos.gonzalez.
lengua@nychhc.org
*********
Gonzalez C, Aquino D, Kanna B. Air bubble in the heart:
An unrecognized complication of IV contrast injector.
International Journal of Case Reports and Images
2013;4(12):739–741.
*********
doi:10.5348/ijcri-2013-12-424-CR-18
Introduction
Venous air embolism (VAE) is predominantly an
iatrogenic complication that occurs when atmospheric gas
is introduced into the systemic venous system [1]. Venous
air embolism has been described as a complication of
neurosurgical procedures performed in a seated position,
central venous catheterization, penetrating and blunt
chest trauma, high-pressure mechanical ventilation,
thoracentesis, hemodialysis, and several other invasive
procedures [1]. However, since the advent of relatively
newer radiological technologies with power intravenous
(IV) contrast injectors, it has become an important
contributor to the incidence of VAE [1–3]. The incidence
of venous air embolism associated with the injection of
contrast for computed tomography (CT) scan reportedly
is 11–23% [1]. Venous air embolism associated with IV
contrast injection is now widely recognized as a cause
of VAE, yet a few cases have been reported in literature.
Clinicians should be aware of the association between
VAE and radiologic exams with IV contrast. We report a
case of a 20-year-old female patient presented with air
bubbles in the right ventricle after an IV contrast CT scan
of the abdomen.
CASE REPORT
Received: 18 October 2012
Accepted: 17 November 2012
Published: 01 December 2013
A previously healthy 20-year-old female patient
presented to the emergency department with an
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):739–741.
www.ijcasereportsandimages.com
accidental stab wound in the left buttock. She denied any
other complaint at the time of the initial assessment in the
emergency department and only mild bleeding associated
with the wound was noted. She was hemodynamically
stable, with normal vital signs and physical examination
was only remarkable for 3 cm wound in the left gluteal
area that compromised skin, fascia, and muscle with no
active bleeding. The wound was successfully closed with
five sutures by the emergency medicine team without any
complication.
While the patient was in the emergency department,
she started to complaint of abdominal pain and the
patient was sent for an abdominal and pelvis CT scan
with contrast. Computed tomography scan showed no
intra-abdominal or pelvic injury, but it was positive for
air bubbles in the right ventricle without obstruction of
right ventricle outflow tract (Figure 1A–B). Based on the
CT scan, a diagnosis of right ventricular air embolism as
a complication of IV contrast injection was made. The
patient did not report any symptoms at that time. She was
immediately placed in supine Trendelenburg position,
and 100% oxygen was given. The patient was admitted to
telemetry unit for close observation. The patient remained
asymptomatic, telemetry monitoring was significant only
for asymptomatic sinus bradycardia, and a new CT scan
showed resolution of the air embolism (Figure 2). The
Figure 1: (A, B) Right ventricular air embolism in a 20-yearold female patient: Contrast computed tomography scan of the
abdomen, upper sections showing multiple air bubbles in the
right ventricle without obstruction of right ventricle outflow
tract (arrow).
Gonzalez et al. 740
patient was discharged home without further complication
the next day.
DISCUSSION
Power contrast injectors have the ability to deliver
400 mL of air in 5 seconds if inappropriately timed
[4]. Although the volume of air required to cause death
is believed to be approximately 200 to 300 mL [2,
5, 6], the amount required to induce hemodynamic
instability is unclear. Most cases of VAE associated with
contrast injection are clinical asymptomatic and often
go undiagnosed [7]. Yet, when the amount of air is large
enough to cause symptoms these may include, but are
not limited to: cardiopulmonary and central nervous
symptoms such as cardiac arrhythmias, chest pain,
electrocardiography changes, dyspnea, altered mental
status, focal deficit, coma, and death; if it is not detected
and treated in time [8]. Other factors associated with the
severity of VAE include the rate of infusion, position of
the patient and the patient baseline health [2, 3, 8].
When acute right ventricular failure secondary to
venous air embolism occurs, it is due to an increase in
the pressure of the pulmonary circulation as a result
of mechanical blood flow obstruction by an air bubble.
Once the right ventricle fails, there may be a secondary
decrease of the venous return to the left ventricle, leading
to decreased cardiac output and systemic cardiovascular
collapse [1].
The management of VAE consists of stopping and
preventing any ongoing air entry, hemodynamic support
with fluid, pressors or inotropes if needed, and increasing
the patient’s oxygenation. To achieve this, the patient
should be started on 100% oxygen to decrease air bubble
size and placed in a Trendelenburg position that displaces
the air bubbles in the right ventricle to a more buoyant
position [1, 3, 4, 8]. Direct right heart air aspiration and
hyperbaric oxygen also has been described with a good
success rate and should be used when available and
indicated [8–12].
CONCLUSION
Figure 2: Right ventricular air embolism in a 20-year-old female
patient: Non-contrast computed tomography scan of the chest,
done one day after the first CT scan, showing no air in the right
ventricle.
Even though most cases of venous air embolism are
asymptomatic, mortality rate in symptomatic cases can
vary from 30% in cases related to catheter placement
up to 80% in trauma patients. Unfortunately, there is
not enough data about mortality related to IV contrast
injections in literature but the high mortality rates
illustrated above gives us an idea of how dangerous
this silent but potentially life threatening entity can be.
Therefore, it is imperative that clinicians pay special
attention to the prevention and early recognition of
venous air embolism when performing procedures that
pose a risk for this condition.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):739–741.
www.ijcasereportsandimages.com
*********
Author Contributions
Carlos Gonzalez – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Dioben Aquino – Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Balavenkatesh Kanna – Acquisition of data, Drafting the
article, Revising it critically for important intellectual
content, Final approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© Carlos Gonzalez et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
REFERENCES
1.
Muth CM, Shank ES. Gas embolism. N Engl J
Med 2000 Feb 17;342(7):476–82.
2. Woodring JH, Fried AM. Nonfatal venous
air embolism after contrast-enhanced CT.
Radiology 1988;167(2):405–7.
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Gonzalez et al. 741
3. Groell R, Schaffler GJ, Rienmueller R, Kern R.
Vascular air embolism: Location, frequency, and
cause on electron-beam CT studies of the chest.
Radiology 1997;202(2):459–62.
4. Ball CG, Rajani RR, Feliciano DV, Nicholas
JM. Venous air emboli and computed axial
tomography power contrast injectors. American
Journal of Surgery 2009;197(6):e57–8.
5.Yeakel AE. Lethal air embolism from
plastic
blood-storage
container.
JAMA
1968;204(3):267–9.
6. Toung TJ, Rossberg, MI, Hutchins GM.
Volume of air in a lethal venous air embolism.
Anesthesiology 2001;94(2):360–1.
7. Pham KL, Cohen AJ. Iatrogenic venous air
embolism during contrast enhanced computed
tomography: a report of two cases. Emergency
Radiology 2003;10(3):147–51.
8. Mirski MA, Lele AV, Fitzsimmons L, Toung
TJ. Diagnosis and Treatment of Vascular Air
Embolism. Anesthesiology 2007;106(1):164–77.
9. Blanc P, Boussuges A, Henriette K, Sainty JM,
Deleflie M. Iatrogenic cerebral air embolism:
Importance of an early hyperbaric oxygenation.
Intensive Care Med 2002;28(5):559–63.
10. Bedford RF, Marshall WK, Butler A, Welsh JE.
Cardiac catheters for diagnosis and treatment
of venous air embolism: a prospective study in
man. J Neurosurg 1981;55(4):610–4.
11. Sviri S, Woods WP, van Heerden PV. Air
embolism--a case series and review. Crit Care
Resusc 2004 Dec;6(4):271–6.
12. Ho AM, Ling E. Systemic air embolism after lung
trauma. Anesthesiology 1999 Feb;90(2):564–
75.
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IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):742–744.
www.ijcasereportsandimages.com
Clinical image
Rodríguez-Gutiérrez et al. 742
OPEN ACCESS
Unexpected Crohn’s Disease after a Free Gastrointestinal
Perforation
René Rodríguez-Gutiérrez, Mario Rodarte-Shade,
José Gerardo González-González
CASE REPORT
A 46-year-old male with history of chronic intermittent
diarrhea and chronic lower-back pain was presented to
our clinic with sudden diffuse abdominal pain irradiated
to both shoulders. He had been on nonsteroidal antiinflammatory drugs (NSAIDs) and prednisone (5–20
mg POq24h) for three years with partial back pain relief.
Physical examination revealed diffuse tenderness and rigid
abdomen with no bowel sounds. X-ray of chest showed a
pneumoperitoneum (Figure 1). A diagnosis of a possible
peptic ulcer perforation was made. He was immediately
managed with intravenous volume, nasogastric tube,
intravenous proton-pump inhibitor (PPI), broadspectrum antibiotics, and urgent laparotomy. During
surgery a 3–4 mm perforation 50 cm before the ileocecal
valve was found along with multiple focal mucosal
ulcerations (Figure 2). Partial bowel resection with an
ileostomy was made. Erythrocyte sedimentation rate
and C-reactive protein were elevated and a macrocytic
anemia with low levels of serum B12 was documented.
The ileum biopsy revealed focal ulcerations across the
ileum wall with acute/chronic inflammation and no sign
Figure 1: Chest X-ray showing air under both diaphragms
confirming the presence of pneumoperitoneum.
René Rodríguez-Gutiérrez1, Mario Rodarte-Shade2, José
Gerardo González-González3
Affiliations: 1MD, Chief Resident, Department of Internal
Medicine, University Hospital “Dr. José E. González”
Universidad Autonoma de Nuevo León, Monterrey, MÉXICO;
2
MD, Chief Resident, Surgery Department, Hospital San
José, Instituto Teconológico y de Estudios Superiores de
Monterrey, Monterrey, MÉXICO; 3MD, Ph.D, Vice Dean of
Research and Professor of Internal Medicine, University
Hospital “Dr. José E. González” Universidad Autonoma de
Nuevo León, Monterrey, MÉXICO.
Corresponding Author: René Rodríguez-Gutiérrez, MD,
Department of Internal Medicine, Dr. José E. González”
University Hospital Universidad Autonoma de Nuevo León,
Monterrey, MÉXICO; Ph: +521 811 474 9146; Fax: 5281
8348 3066; Email: renerodriguez@investigacion-meduanl.
com
Received: 14 August 2012
Accepted: 27 July 2013
Published: 01 December 2013
Figure 2: Ileum perforation 50 cm before the ileocecal valve.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):742–744.
www.ijcasereportsandimages.com
of malignancy. Ziehl-Neelsen and culture for tuberculosis
were negative. Colonoscopy revealed focal ulcerations
and pseudopolyps adjacent to areas of normal appearing
mucosa with the characteristic cobblestone appearance
and the same pathologic findings of the previous ileum
biopsy. The compatible clinical history along with the
laboratory, endoscopic and pathology findings made
the final diagnosis of Crohn’s disease. Treatment with
azathioprine and mesalamine was initiated. The patient
had an uneventful recovery and at a six-month followup he was asymptomatic. An unexpected diagnosis of
Crohn’s disease was made, probably partially masked over
the last three years by the chronic glucocorticoid therapy
used for the chronic lower-back pain that was finally
attributed to a spinal disc hernia. Human Leukocyte
Antigen (HLA)-B27 was negative and there was no sign
of spondylitis or sacroiliitis.
Rodríguez-Gutiérrez et al. 743
requires prompt diagnosis and urgent management
with intravenous fluids, broad-spectrum antibiotics, and
immediate exploratory laparotomy. Good prognosis is
inversely proportional to the time taken for an appropriate
treatment.
*********
Rodríguez-Gutiérrez R, Rodarte-Shade M, GonzálezGonzález JG. Unexpected Crohn’s Disease after a Free
Gastrointestinal Perforation. International Journal of
Case Reports and Images 2013;4(12):742–744.
*********
doi:10.5348/ijcri-2013-12-425-CL-19
*********
DISCUSSION
Crohn’s disease is a chronic illness of uncertain
etiology that is characterized by a transmural
inflammation of the entire gastrointestinal tract. Crohn’s
complications are fistulae, localized peritonitis, abscesses,
bowel obstructions, gastrointestinal perforation, and
hemorrhage. Of them free gastrointestinal perforation,
which is a life-threatening complication, is the most rare
and described only in 1–2% of these patients. It is more
common as the initial manifestation of the illness, but
has also been described during the course of the disease
[1]. Most of the patients present instead with an intraabdominal abscesses or fistulae. The most common
site of free perforation is the ileum or jejunum and
chronic steroid use has been proposed as risk factor for
perforation. Pneumoperitoneum, as found in this case,
is an uncommon feature of small intestine perforation
[2]. Diagnosis is made by the characteristic clinical
presentation of acute abdominal pain along with acute
systemic inflammatory response syndrome or sepsis and
the characteristic findings on the chest and abdominal
X-rays or computed tomography. Immediate management
to achieve hemodynamic stability with high-volume
intravenous fluids and sometimes catecholamines along
with broad-spectrum antibiotics is essential. Nevertheless,
urgent surgical management with exploratory laparotomy
is the cornerstone of the treatment. Prognosis compared
to ulcerative colitis perforation is formidable with a
mortality rate of less than 5% if managed promptly [3].
In this case, the steroids that could have treated and
“masked” the illness for a long time could have also been
the triggering cause of the gastrointestinal perforation.
CONCLUSION
Free gastrointestinal perforation in Crohn’s disease
is an unusual but life-threatening complication that
Author Contributions
René Rodriguez-Gutierrez – Served as the principal
investigator and made substantial contributions in the
conception, design and acquisition of the data, Drafting
and revising it critically for important intellectual content,
Approved the final version to be published
Mario Rodarte-Shade – Made substantial contributions
in the conception, design and acquisition of the data,
Drafting and revising it critically for important intellectual
content, Approved the final version to be published
José Gerardo González-González – Made substantial
contributions in the conception, design and acquisition of
the data, Drafting and revising it critically for important
intellectual content, Approved the final version to be
published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© René Rodriguez-Gutierrez et al. 2013; This article
is distributed under the terms of Creative Commons
attribution 3.0 License which permits unrestricted use,
distribution and reproduction in any means provided
the original authors and original publisher are properly
credited. (Please see www.ijcasereportsandimages.com/
copyright-policy.php for more information.)
REFERENCES
1.
Freeman HJ. Spontaneous free perforation of the
small intestine in Crohn’s disease. Can J Gastroenterol
2002 Jan;16(1):23–7.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):742–744.
www.ijcasereportsandimages.com
Rodríguez-Gutiérrez et al. 2. Werbin N, Haddad R, Greenberg R, Karin E, Skornick
Y. Free perforation in Crohn’s disease. Isr Med Assoc
J 2003 Mar;5(3):175–7.
3. Greenstein AJ, Sachar DB, Mann D, Lachman
P, Heimann T, Aufses AH Jr. Spontaneous free
perforation and perforated abscess in 30 patients
with Crohn’s disease. Ann Surg 1987 Jan;205(1):72-6.
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744
IJCRI 2013;4(12):745–747.
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Olubaniyi et al. clinical image
745
OPEN ACCESS
Hemophilic Arthropathy
Babajide O Olubaniyi, Olubukola Ajala, David C Howlett
case report
A 34-year-old male with hemophilia A presented to
the emergency department with pain and swelling in his
left knee following a minor fall. He was known to suffer
from advanced arthropathy and was awaiting a total knee
replacement. On clinical examination, the left knee was
swollen and tender with a reduced range of movement.
Plain radiograph (antero-posterior and lateral views)
performed (Figures 1 and 2) excluded a fracture or
dislocation but demonstrated classic appearances
of hemophilic arthropathy with severe secondary
degenerative changes. He was discharged with adequate
simple analgesia and outpatient follow-up appointments
with his hematologist and physiotherapist.
DISCUSSION
Hemophilia is an inherited, X-linked recessive
disorder caused by deficiency of plasma clotting factors
VIII, IX or XI. It manifests in males while females
are asymptomatic carriers. Hemophilic arthropathy
Babajide O Olubaniyi1, Olubukola Ajala2, David C Howlett3
Affiliations: 1FRCR, Department of Radiology, Eastbourne
District General Hospital, King’s Drive, Eastbourne, East
Sussex, BN21 2UD, United Kingdom; 2MRCP, Department
of Diabetes and Endocrinology, Western Sussex NHS Trust,
Worthing Hospital, Lyndhurst road, BN11 2DH, United
Kingdom, 3FRCR, Department of Radiology, Eastbourne
District General Hospital, King’s Drive, Eastbourne, East
Sussex, BN21 2UD, United Kingdom.
Corresponding Author: Dr. Babajide O Olubaniyi, Department
of Radiology, Eastbourne District General Hospital, King’s
Drive, Eastbourne, East Sussex, BN21 2UD, United
Kingdom; Tel: +441323417400, Extension 4673; Email: jide.
olubaniyi@nhs.net
Received: 03 June 2013
Accepted: 30 July 2013
Published: 01 December 2013
Figure 1: Anteroposterior radiograph of the knee showing
widened intercondylar notch, bulbous femoral condyles,
flattened condylar surfaces, periarticular osteoporosis, marked
loss of articular cartilage, sclerosis, and osseous proliferation.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):745–747.
www.ijcasereportsandimages.com
Olubaniyi et al. 746
rest. Patients who suffer from repeated hemarthrosis
despite optimal conservative management may benefit
from synovectomy. Patients with end stage disease may
require surgical arthrodesis or total joint replacement to
control pain and restore some function as planned for our
patient.
CONCLUSION
Hemophilia can cause progressive joint destruction
and loss of function. Early prophylactic treatment in
children with severe hemophilia can prevent or delay
joint destruction.
*********
Olubaniyi BO, Ajala O, Howlett DC. Hemophilic
Arthropathy. International Journal of Case Reports and
Images 2013;4(12):745–747.
*********
doi:10.5348/ijcri-2013-12-426-CL-20
Figure 2: Lateral radiograph of the knee showing squared
inferior margin of the patella and severe degenerative changes
in the patellofemoral and tibiofemoral compartments.
results from repeated episodes of hemarthrosis, blood
within the joint damages the articular cartilage directly
[1]. Large joints (knee, ankle, elbow, and shoulder) are
usually affected. The classic appearance of hemophilic
arthropathy in the knee is shown in Figures 1 and 2, i.e.,
widened intercondylar notch, bulbous femoral condyles,
flattened condylar surfaces and squared inferior
margin of the patella [2]. Due to repeated episodes of
hemarthrosis, our patient also had severe secondary
osteoarthritic changes with near-total joint space loss.
Hemophilic arthropathy most often begins in the first
or second decade of life and bleeding episodes tend to
recur in the same joint with gradual progression to joint
destruction. Recurrent hemarthrosis inevitably leads to
synovial hyperplasia, subchondral resorption, erosions,
articular cartilage destruction, joint space narrowing,
and osseous proliferation. Periarticular osteoporosis and
epiphyseal overgrowth occurs due to associated joint
hyperemia. Clinical presentation varies according to
disease severity with pain and joint swelling during acute
episodes of hemarthrosis and progressive loss of joint
function in longstanding cases. Prophylactic replacement
of deficient clotting factors in the first or second decade
of life may prevent or delay joint destruction in children
with severe hemophilia [3]. Patients who present with
an acute bleeding episode require adequate pain relief,
prompt administration of deficient clotting factor and
*********
Author Contributions
Babajide O Olubaniyi – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Olubukola Ajala – Acquisition of data, Drafting the
article, Revising it critically for important intellectual
content, Final approval of the version to be published
David C Howlett - Substantial contributions to conception
and design, Acquisition of data, Revising it critically for
important intellectual content, Final approval of the
version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© BO Olubaniyi et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):745–747.
www.ijcasereportsandimages.com
REFERENCES
1.
Jansen NW, Roosendaal G, Bijlsma JW, DeGroot
J, Lafeber FB. Exposure of human cartilage tissue
to low concentrations of blood for a short period of
time leads to prolonged cartilage damage: An in vitro
study. Arthritis Rheum 2007 Jan;56(1):199–207.
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Olubaniyi et al. 2.
3.
747
Llauger J, Palmer J, Rosón N, Bagué S, Camins A,
Cremades R. Nonseptic monoarthritis: Imaging
features with clinical and histopathologic correlation.
Radiographics 2000 Oct;20 Spec No:S263–78.
Manco-Johnson MJ, Abshire TC, Shapiro AD, et al.
Prophylaxis versus episodic treatment to prevent
joint disease in boys with severe haemophilia. N Engl
J Med 2007 Aug 9;357(6):535–44.
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IJCRI 2013;4(12):748–749.
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Desai et al. 748
Letter to EditorOPEN ACCESS
Plasma cell leukemia of IgG secreting type: A rare case
Parth Anil Desai, Sagar Dholariya, Sanjeev Gupta, Tejinder Singh
To the Editor,
Plasma cell leukemia is very rare comprising 2%
plasma cell dyscrasias [1]. We report a case of a 49-yearold male with complaints of chest pain, breathlessness,
and acute attacks of gastroenteritis. On examination,
there was marked pallor and hepatosplenomegaly.
X-ray and computed tomography (CT) scan revealed
multiple paratracheal lymph node enlargements.
However, there was no diffuse osteopenia or multiple
osteolytic lesions. Laboratory analysis revealed high
serum calcium 11.5 mg/dL, low hemoglobin 5 g/dL,
normal WBC count, thrombocytopenia 22000/mm3
and elevated erythrocyte sedimentation rate (ESR).
Peripheral smear showed many atypical cells (41%) with
blastoid morphology, giving a blood picture of acute
leukemia. However, on closer inspection many of them
appeared plasmacytoid. There was marked rouleaux
formation of red cells in the background. Bone marrow
aspirate and biopsy also revealed near total replacement
by such atypical cells with plasmacytoid morphology.
Serum electrophoresis revealed a prominent M spike
and immunofixation revealed IgG heavy chain and kappa
light chain immunostaining ruling out the possibility
of Waldenström’s macroglobulinemia. Plasma cell
Parth Desai1, Sagar Dholariya2, Sanjeev Gupta3, Tejinder
Singh4
Affiliations: 1MBBS, Resident Department of Pathology,
MAMC, Maulana Azad Medical College, New Delhi; 2MBBS,
Resident Department of Biochemistry, MAMC, Maulana
Azad Medical College, New Delhi; 3DM Hematopathology,
Professor AIIMS, Maulana Azad Medical College, New
Delhi; 4MD Pathology, Director Professor, MAMC, Maulana
Azad Medical College, New Delhi.
Corresponding Author: Parth Anil Desai, MBBS, Resident,
Department of Pathology, MAMC, Maulana Azad Medical
College, New Delhi; Email: parth1410@gmail.com
Received: 07 May 2013
Accepted: 27 June 2013
Published: 01 December 2013
Figure 1: (A) Peripheral Smear (Giemsa): Blastoid cells with
plasmacytoid cells and background rouleaux formation,
(B) Bone marrow aspirate smear: Plasmacytoid cells with
prominent nucleoli, (C) Bone marrow biopsy: Replacement by
atypical plasmacytoid cells. Kappa positive and lambda (inset)
negative immunostaining, (D) Serum electrophoresis (left)
showing M spike and immunofixation (right) showing G heavy
chain and kappa light chain.
leukemias are predominantly IgD and IgE secreting
type [2–4]. This case is rare in that it is IgG secreting
type. Plasma cell leukemias are very aggressive and with
higher incidence of organomegaly, lymphadenopathy,
thrombocytopenia, hypercalcemia, renal impairment and
surprisingly lesser occurrence of lytic bony lesions as in
our case [1, 2, 4].
*********
Desai PA, Dholariya S, Gupta S, Singh T. Plasma cell
leukemia of IgG secreting type: A rare case. International
Journal of Case Reports and Images 2013;4(12):748–
749.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 12, December 2013. ISSN – [0976-3198]
IJCRI 2013;4(12):748–749.
www.ijcasereportsandimages.com
*********
doi:10.5348/ijcri-2013-12-427-LE-21
*********
Author Contributions
Parth Anil Desai – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Sagar Dholariya – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Sanjeev Gupta – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published
Tejinder Singh – Substantial contributions to conception
and design, Acquisition of data, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the version to be published.
Desai et al. Copyright
© Parth Anil Desai et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
License which permits unrestricted use, distribution and
reproduction in any means provided the original authors
and original publisher are properly credited. (Please see
www.ijcasereportsandimages.com/copyright-policy.php
for more information.)
REFERENCES
1.
García-Sanz R, Orfão A, González M, et al. Primary
plasma cell leukaemia: Clinical, immunophenotypic,
DNA ploidy, and cytogenetic characteristics. Blood
1999 Feb 1;93(3):1032–7.
2. Kyle RA, Maldonado JE, Bayrd ED. Plasma cell
leukaemia. Report on 17 cases. Arch Intern Med 1974
May;133(5):813–8.
3. Pruzanski W, Platts ME, Ogryzlo MA. Leukemic form
of immunocytic dyscrasia (plasma cell leukaemia). A
study of ten cases and a review of the literature. Am J
Med 1969 Jul;47(1):60–74.
4. Dimopoulos MA, Palumbo A, Delasalle KB, Alexanian
R. Primary plasma cell leukaemia. Br J Haematol
1994 Dec;88(4):754–9.
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
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