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International Journal of
Case Reports and Images
Contents
Vol. 4, No. 9 (September 2013)
Cover Image
Figure 1: Distal tubule with a large, acellular
hyaline cast that has a lamellated appearance
(H&E stain, x200)
Case Series
Cover Figure:
463 Surgical management of an impacted sharp
metallic foreign body in esophagus
Sharma NK, Yadav VK, Pokharna P, Devgaraha
S, Mathur RM
Case Report
467 Noncompaction cardiomyopathy: A rare
cardiomyopathy
Sachin Kumar Amruthlal Jain, Brijesh Patel,
Timothy R Larsen, Yousif Ismail, Shukri David
471 A young nulliparous woman with right ovarian
serous borderline tumor and left ovarian
micropapillary serous carcinoma
Vinita Jaggi Kumar, Dhruv Jain, Asit Mridha,
Sidharth Nanda, Jyotica Jain, Rajesh Kumar
Grover
477 Sigmoid carcinoma: A rare presentation and
diagnostic challenge
Bassem Amr, Komal Munir, Natasha SantanaVaz, Venkateswarlu Velineni
507Missed retained knife blade injury: A
potentially lethal trap for the unwary
Victor Kong, John Bruce, Leah Naidoo,
George Oosthuizen, Grant Laing, Damian
Clarke
511 Novel surgical extrication of penile
constricting metal ring: A case report
Abimbola Olaniyi Olajide, Amogu Kalu Eziyi,
Folakemi Olajumoke Olajide, Babatunde
Kazeem Beyioku
515 Beta-hemolytic group B Streptococcus
meningitis in a young healthy woman
Li Han, Rohit Gosain, Maria Plataki, Daniel
Horowitz, Daniel Bordea
Case in Images
518 Acardiac twin: Conservative management
Deepa V Kanagal, Mangala Srikantaswamy,
Rashmi M Devaraj, Maitri M Kulkarni,
Mahendra Gangadhariah, Lokeshchandra
C Hanumanalu
481 Neglected case of congenital hypothyroidism in
a 17-year-old female
Mahmoud Medhat Mahmoud
Letter to Editors
485 Fallaciously elevated glucose level by handheld
glucometer in a patient with chronic kidney
disease and hypoglycemic encephalopathy
Praveen Pratap Jadhav, Meera Praveen Jadhav
524 Primary cavernous hemangioma of the
thyroid gland
Devika Gupta, Sunita Kakkar, Pooja
Gupta, Vandana Rana
489 Dual causes of multiple myeloma
Zaw Min, Zipporah Krishnasami, William J
Cook, J Martin Rodriguez
All Articles:
502Recurrent intraosseous ganglia in the
proximal tibia following anterior cruciate
ligament reconstruction using patella
tendon bone autograft with a titanium
interference screw: A case report
Aysha Rajeev, James Henry, Nanjappa
Kashyap
494 Advanced secondary abdominal pregnancy: A
complication of induced abortion
Fateme Lalooha, Khadijeh Elmizadeh, Fateme
Salehi
498 Primary cerebellar B cell lymphoma: A case
report
Anupam Datta, Arunima Gupta, Krishnangshu
Bhanja Choudhury, Aruj Dhyani, Anup
Majumdar
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IJCRI 201 3;4(9):463–466.
Sharma et al.
www.ijcasereportsandimages.com
CASE SERIES
463
OPEN ACCESS
Surgical management of an impacted sharp metallic
foreign body in esophagus
Sharma NK, Yadav VK, Pokharna P, Devgaraha S, Mathur RM
ABSTRACT
Introduction: Ingestion of foreign bodies is
common and the esophagus is the most common
site of impaction in the upper gastrointestinal
tract. Case Series: We report two cases of
impacted metallic foreign bodies in upper
esophagus. The presentating symptoms were
throat pain and dysphagia. Rigid and flexible
esophagoscopy failed to retrieve the foreign
body in both cases hence lateral cervical
esophagostomy was performed for foreign body
removal. Conclusion: Ingestion of foreign
bodies is common. Some metallic foreign bodies
frequently get impacted in the esophagus
because of their large size, rigidity and pointed
edges and are difficult to remove by endoscopy.
Therefore, surgery is required for retrieval of
such impacted, sharp, metallic foreign bodies.
Keywords:
Impactation,
Lateral
esophagostomy, Foreign body
cervical
*********
Sharma NK, Yadav VK, Pokharna P, Devgaraha S,
Mathur RM. Surgical management of an impacted sharp
metallic foreign body in esophagus. International
Journal
of
Case
Reports
and
Images
2013;4(9):463–466.
*********
Sharma NK1 , Yadav VK1 , Pokharna P 1 , Devgaraha S 1 ,
Mathur RM 1
Affiliations: 1 Department of CTVS, S. M. S Medical College,
Jaipur, Rajasthan, India.
Corresponding Author: Dr. Neeraj Kumar Sharma, S. M. S
Medical College Jaipur, Rajasthan, India; Email:
[email protected]
Received: 24 September 201 2
Accepted: 1 6 November 201 2
Published: 01 September 201 3
doi:10.5348/ijcri201309357CS1
INTRODUCTION
Foreign body ingestion is a common problem
encountered in clinical practice. Most of them pass
spontaneously but some are really problematic.
Endoscopic removal of a foreign body is routinely done
but it fails in some cases which require surgical removal
of the foreign body. We present two cases of unusual
foreign body ingestion, one was a sharp metallic folded
tin cover and another was a sharp metallic bottle cap. As
these foreign bodies could not be retrieved
endoscopically, surgical removal of these foreign bodies
was performed successfully. Patients are doing well on
followup after one year.
CASE SERIES
We report two cases of impacted, sharp, metallic
foreign bodies in the upper esophagus. First patient was
a 35yearold male, who was a known case of
schizophrenia. He ingested a sharp metallic folded tin
cover (Figure 1). The patient presented to us with throat
pain and dysphagia. On examination, there was bilateral
air entry, equal on both sides, with no added sound.
Chest Xray revealed foreign body in the cervical
esophagus (Figure 2). The second patient was a 25years
male. He presented to us with a history of accidental
ingestion of sharp metallic bottle cap (Figure 3). At the
time of presentation he had throat pain and dysphagia.
There was no respiratory distress. On examination chest
had bilateral equal air entry without stridor.
In both cases Xray of the cervical spine and chest
(lateral view) were done which reveled foreign body with
sharp edges present in the cervical esophagus (Figure 4).
Flexible and rigid esophagoscopy was done for
therapeutic removal of the foreign body but failed.
Foreign body was impacted in upper esophagus with
surrounding inflammation and edema.
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 9, September 201 3. ISSN – [0976-31 98]
IJCRI 201 3;4(9):463–466.
Emergency surgery was planned. Longitudinal
incision was given on the left side of neck in front of
sternocleidomastoid. Carotid artery and thyroid gland
Sharma et al.
464
were retracted. Esophagus was found to be inflammed
and edematous. Longitudinal incision was given on
esophagus over impacted foreign body and foreign body
was gently disimpacted and removed to prevent further
injury to esophagus. Esophagus was repaired using 3.0
vicryl interrupted sutures in two layers, with Ryle’s tube
left in situ for three weeks. Postoperative period was
Figure 3: Chest Xray showing foreign body in the cervical
esohagus.
Figure 1: Folded metallic tin cover removed from esophagus
(case 1).
Figure 2: Bottle cap removed from esophagus (case 2).
Figure 4: Lateral chest Xray showing foreign body in the
upper esophagus.
IJCRI 201 3;4(9):463–466.
Sharma et al.
uneventful and both the patients are doing well on
followup.
DISCUSSION
It is estimated that up to 90% of all foreign bodies
pass spontaneously from the esophagus. Endoscopic
management is needed in less than 10% cases, whereas
surgery is required for foreign body retrieval or
management of complications in approximately 1%
patients [1–3]. In both our cases, the patients required
lateral cervical esophagostomy, after failed endoscopic
retrieval.
Although most of the foreign bodies in the
gastrointestinal tract are swallowed accidentally, other
conditions such as imprisonment, mental illness,
mental retardation, bulimia, alcohol consumption or
drug abuse may also be involved, particularly in the
Western countries [4]. The majority of foreign body
ingestions occur in the pediatric population with a peak
incidence between six months and six years of age
[2–8]. Edentulous adults are also at greater risk for
foreign body ingestion, including that of dental
prosthesis [8, 9]. One of our cases had an accidental
ingestion of bottle cap while another had a history of
mental illness.
Esophageal objects can cause a foreign body
sensation, drooling, respiratory distress due to tracheal
compression, gagging, dysphonia, vomiting, and
dysphagia, depending on the location and nature of the
foreign body [10]. Although rare, perforating objects are
potentially life threatening because they may lead to the
formation of a fistula between the esophagus and the
innominate artery thus causing catastrophic bleeding
[11, 12]. Young children and those with mental illness
may present with choking, refusal to eat, vomiting,
drooling, wheezing, bloodstained saliva, or respiratory
distress [5, 7, 13, 14]. Our patients had symptoms of
throat pain and dysphagia.
The foreign bodies usually lie close to one of the
three esophageal anatomical constrictions: the
cricopharyngeal ring, the aortic arch narrowing or the
esophagogastric junction [15, 16]. The most frequent
lodgement site of described in literature is the
cricopharyngeus muscle [11, 12].
Plain films (cervical and chest Xrays) are a very
important diagnostic tool, especially in defining the
location of the foreign body [10]. A bariumswallow X
ray study could be useful in cases of nonradioopaque
foreign body, but due to possible barium aspiration and
or irritation of the damaged esophageal mucosa, this
procedure is no longer used [17–19]. Computed
tomography scans can be used to confirm the presence
and location of the foreign body (especially in the cases
of fish bones), and to evaluate any eventual damage to
the neighboring structures [18, 19].
In our cases, it was the clinical history, Xrays and
endoscopy that guided us in diagnosing and defining
the location of the foreign body.
The treatment of choice for esophageal forein bodies
465
depends on various parameters such as patient’s age,
clinical condition; the type, size, shape, site and
number of foreign bodies. [10, 20] Endoscopy is the
preferred method with a reported success rate of 83%
[20]. Today, both rigid or flexible endoscopy, performed
under general anesthesia or conscious sedation,
respectively, are considered to be safe and represent
effective methods in experienced hands. For the
management of sharp and penetrating foreign bodies,
rigid endoscopy is often the treatment of choice. Major
risk during esophagoscopy maneuvers include direct
instrumental wounds and perforations [16]. Surgical
treatment is unavoidable in cases of irretrievable
foreign body or esophageal perforation [10].
In our cases, flexible and rigid endoscopies were
unsuccessful in the removal maneuvers mainly due to
the size and shape of the foreign bodies. Further, the
foreign bodies were impacted in esophagus therefore
surgical treatment was necessary.
CONCLUSION
Ingestion of foreign bodies end their lodgment in the
esophagus are common. Some metallic foreign bodies
get frequently impacted in the esophagus and dose
difficulty in endoscopic retrieval due to their large size,
rigidity and pointed edges. Therefore in such cases
surgery is required for retrieval of the impacted sharp
metallic foreign bodies.
*********
Author Contributions
Sharma NK – Substantial contributions to conception
and design, Analysis and interpretation of data,
Drafting the article, Revising it critically for important
intellectual content, Final approval of the version to be
published
Yadav VK – 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
Pokharna P – Substantial contributions to conception
and design, Acquisition of data, Revising it critically for
important intellectual content, Final approval of the
version to be published
Devgaraha S – Analysis and interpretation of data,
Mathur RM – Analysis and interpretation of data,
Guarantor
The corresponding
submission.
author
is
the
guarantor
Conflict of Interest
Authors declare no conflict of interest.
of
IJCRI 201 3;4(9):463–466.
Sharma et al.
Copyright
© Sharma NK 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
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for more information.)
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IJCRI 2013;4(9):467–470.
Jain et al. CASE REPORT
467
OPEN ACCESS
Noncompaction cardiomyopathy: A rare cardiomyopathy
Sachin Kumar Amruthlal Jain, Brijesh Patel, Timothy R Larsen,
Yousif Ismail, Shukri David
Abstract
Introduction: Noncompaction cardiomyopathy
(NCCM) is a rare form of cardiomyopathy. The
American Heart Association classified it as a
genetic cardiomyopathy. The pathogenesis of this
condition is attributed to the failure of trabecular
myocardium to compact and underdeveloped
microcoronary circulation. Case Report: We
present a case of a 72-year-old African American
female who presented with shortness of
breath and leg swelling and was found to have
severe systolic dysfunction. Stress test showed
ischemia. Left heart catheterization which
included left ventriculogram showed prominent
trabeculations suggestive of noncompaction
cardiomyopathy. Conclusion: This condition is
often associated with other congenital cardiac
defects and arrthymias. When it is present
alone, it is called isolated noncompaction
cardiomyopathy
(INCCM).
The
treatment
protocol for this cardiomyopathy is the same
as other cardiomyopathies, and includes the
management of arrhythmias, heart failure and
potential risk of embolism.
Keywords: Noncompaction cardiomyopathy,
Echocardiography, Heart failure, Congenital
heart disease
Sachin Kumar Amruthlal Jain1, Brijesh Patel1, Timothy R
Larsen1, Yousif Ismail1, Shukri David1
Affiliations: 1MD, Providence Hospital and Medical Center,
Southfield, MI.
Corresponding Author: Sachin Kumar Amruthlal Jain,
MD, Department of Cardiology, Providence Hospital and
Medical Centers, 16001 W. Nine Mile Road, Southfield, MI.
48075; Phone: 248 849 3968; Fax: 248 849 3151; E-mail:
[email protected]
Received: 15 October 2012
Accepted: 23 February 2013
Published: 01 September 2013
*********
Jain SKA, Patel B, Larsen TR, Ismail Y, David S.
Noncompaction cardiomyopathy: A rare cardiomyopathy.
International Journal of Case Reports and Images
2013;4(9):467–470.
*********
doi:10.5348/ijcri-2013-09-358-CR-2
Introduction
Noncompaction cardiomyopathy (NCCM) is a rare
form of cardiomyopathy [1, 2]. The American Heart
Association classifies it as a genetic cardiomyopathy
[1]. The pathogenesis of this condition is attributed
to the failure of trabecular myocardium to compact
and underdeveloped microcoronary circulation. This
condition is often associated with other congenital
cardiac defects and arrthymias. When it is present alone
then it is called isolated noncompaction cardiomyopathy
(INCCM) [1, 2]. Currently, the treatment protocol for this
cardiomyopathy is the same as other cardiomyopathies,
and includes the management of arrhythmias, heart
failure and potential risk of embolism [2].
CASE REPORT
Our patient, was a 72-year-old African-American
female who came to the emergency room (ER)
complaining of lower extremity edema and shortness
of breath for one week. She gave a history of worsening
dyspnea and gradual development of orthopnea. She
denied paroxysmal nocturnal dyspnea, cough, chest pain,
sputum production or palpitation. She was hospitalized
for congestive heart failure and lower extremity edema
in the past. Her past medical history was significant for
severe congestive heart failure, hypertension and chronic
obstructive pulmonary disease. She was a smoker. Her
IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 9, September 2013. ISSN – [0976-3198]
IJCRI 2013;4(9):467–470.
physical examination was significant for shortness of
breath and bilateral lower extremity edema (1+). Her
medication included aspirin, carvedilol, furosemide,
hydralazine, simvastatin and tiotropium. Her electro
cardiogram showed atrial flutter with 2:1 block, bilateral
atrial enlargement, Q-waves in Lead II and III, poor
R-wave progression and prolonged QT interval (499
ms). In the hospital, patient was treated for symptoms
of congestive heart failure, and underwent ablation
procedure for atrial flutter. The patient was in normal
sinus rhythm and with improved symptoms at the time
of discharge.
Image findings and analysis: The patient underwent
echocardiographic study to assess the left ventricular
function and right chamber pressure measurements,
because of her symptoms and the history of severe
congestive heart failure. The study showed enlarged left
ventricle with severe global systolic dysfunction and
depressed ejection fraction of 20%, grade I diastolic
impairment, mildly enlarged left atrium and a trace of
mitral and pulmonary regurgitation. In addition, it showed
trabeculations in the left ventricle (Figure 1). The severe
global systolic dysfunction warranted further evaluation
of her heart. Therefore, she underwent lexiscan cardiolite
nuclear stress test. The stress test showed large partially
reversible inferior and inferolateral defect consistent
with stress induced ischemia and also noted to have
dilated right ventricle. To further evaluate the ischemia,
two days later, she underwent cardiac catheterization/
left ventriculogram that showed ejection fraction of 20%,
severe global impairment in contractility and prominent
left ventricular trabeculation suggesting NCCM (Figure
2), compared to a normal left ventriculogram from another
patient (Figure 3).
Jain et al. 468
DISCUSSION
During the first five to eight weeks of embryonic
life, spongy/trabecular myocardium starts to compact.
These trabecular fibers that are separated by ventricular
recesses link myocardium to the ventricular cavity.
The noncompaction cardiomyopathy ensues when
the myocardium fails to compact fully. The coronary
circulation also develops during this period. When the
fibers fail to compact they often have compromised
coronary microcirculation which makes them more
susceptible to ischemia and hypokinesia [2]. Many
times, cases of noncompaction cardiomyopathy also have
concurrent arrthymias and congenital cardiac defects.
The image studies in our patient did not reveal any
Figure 2: Left ventriculogram demonstrating prominent apical
trabeculae (arrows). (L-left ventricular cavity).
Figure 1: Transthoracic echocardiogram revealing prominent
inferoapical trabeculations (arrow). LV-left ventricle, RV-right
ventricle.
Figure 3: Left ventriculogram from another patient
demonstrating normal left ventricular myocardium for
comparison.
IJCRI 2013;4(9):467–470.
congenital cardiac defect. Therefore, it is called isolated
noncompaction cardiomyopathy.
In 2006, the American Heart Association included
noncompaction
cardiomyopathy
as
a
genetic
cardiomyopathy [1]. However, it may exist in both
sporadic and familial forms. In case of familial form, it
is associated with X-link inheritance linked to G4.5 gene
(named Tafazzin) on Xq28 and a gene located on 11p15 [1].
The actual prevalence of noncompaction cardiomyopathy
is unclear. The increasing number of case reports on this
condition and various imaging options have helped to
approximate the prevalence. Based on echocardiographic
studies, 0.014–0.05% of the general population might be
affected with this condition [1, 2]. The affected patients
may develop systolic or diastolic dysfunction, or both,
at the same time [3]. Echocardiography has remained
as the main modality for diagnosis of this condition
[2]. However, other imaging modalities can offer more
information about the structure and function of the heart.
The differential diagnosis of isolated noncompaction
cardiomyopathy include acquired, ischemic or mixed
cardiomyopathy. Currently, it is managed as other
cardiomyopathies, and includes appropriate treatment
for heart failure, arrthymias and embolism [2]. Growing
literature on noncompaction cardiomyopathy will
enhance the understanding of pathogenesis, management
and prognosis of this condition.
Jain et al. 469
approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Copyright
© Sachin Kumar Amruthlal Jain 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. Espinola-Zavaleta N, Soto ME, Castellanos LM,
Játiva-Chávez S, Keirns C. Non-compacted
cardiomyopathy: Clinical-echocardiographic study.
Cardiovasc Ultrasound 2006;4:35.
2. Weiford BC, Subbarao VD, Mulhern KM.
Noncompaction of the ventricular myocardium.
Circulation 2004;109(24):2965–71.
3. Luong MW, Mettini S, Delgado D. Noncompaction
Cardiomyopathy. UTMJ 2010;87:144–7.
CONCLUSION
Noncompaction cardiomyopathy is a rare congenital
heart condition that presents later in life. Patients with
this condition present with heart failure, arrhythmias
and/or embolism. In conclusion, patients with
noncompaction cardiomyopathy are treated as other
heart failure patients.
*********
Sachin Kumar Amruthal Jain – Conception and design,
Acquisition of data, Analysis and interpretation of data,
Critical revision of the article, Final approval of the
Brijesh Patel – 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
Timothy Larsen – Acquisition of data, Analysis and
interpretations, Critical revision of the article, Final
Yousif Ismail – Acquisition of data, Analysis and interpretations, Critical revision of the article, Final approval
of the version to be published
Shukri David – Acquisition of data, Analysis and
interpretations, Critical revision of the article, Final
IJCRI 2013;4(9):467–470.
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IJCRI 2013;4(9):471–476.
Kumar et al. CASE REPORT
471
OPEN ACCESS
A young nulliparous woman with right ovarian serous
borderline tumor and left ovarian micropapillary serous
carcinoma
Vinita Jaggi Kumar, Dhruv Jain, Asit Mridha, Sidharth Nanda,
Jyotica Jain, Rajesh Kumar Grover
Abstract
Introduction: Serous borderline tumor (SBT) of
low malignant potential (LMP) is heterogeneous
group, neither wholly benign nor frankly
malignant. It usually involves premenopausal age
patients, has good prognosis and may recur even
after 20 years. Decades later, transformation
to low grade serous carcinoma can occur in 7%,
mandating prolonged follow-up. Ten and 20 years
survival in stage I is 95% and 80% respectively.
Micropapillary type and invasive tumor implants
warrant treatment like carcinoma and need
adjuvant chemotherapy. Case Report: We present
the case of a 25-year-old nullipara, married for
four years who had ovarian cystectomy one year
back. She reported to our institute with massive
ascites and failure of anti-tubercular treatment
of three months. Diagnostic tap revealed cells
Vinita Jaggi Kumar1, Dhruv Jain2, Asit Mridha3, Sidharth
Nanda3, Jyotica Jain4, Rajesh Kumar Grover5
Affiliations: 1MBBS, MD, MRCOG, Department of Surgical
oncology (Gynecological Oncology Division, Delhi State
Cancer Institute (Govt. of NCT), Dilshad Garden, Delhi,
India; 2MBBS, MD, Assistant Professor, Department of
Onco Pathology, Delhi State Cancer Institute (Govt. of
NCT), Dilshad Garden, Delhi, India; 3MBBS, MD, Assistant
Professor, Clinical Oncology, Delhi State Cancer Institute
(Govt. of NCT), Dilshad Garden, Delhi, India; 4MBBS, MD,
Associate Professor, Clinical oncology, Delhi State Cancer
Institute (Govt. of NCT), Dilshad Garden, Delhi, India;
5MBBS, MD, PGDHHM, Director, Radiation Oncology, Delhi
State Cancer Institute (Govt. of NCT), Dilshad Garden,
Delhi, India.
Corresponding Author: Dr. Vinita Jaggi Kumar, Delhi State
Cancer Institute (Govt. of NCT Delhi), Dilshad Garden, Delhi,
India; Postal Code-110095; Ph: +91 9560390112; Fax: +9111-22110505; Email: [email protected]
suggestive of metastatic adenocarcinoma.
Bilateral complex adnexal masses with ascites
were seen on computed tomography (CT)
scan. CA125 was 191 U/mL. Thorough staging
laparotomy i.e., inspection and palpation of
abdomen and pelvic organs, ascitic fluid cytology,
bilateral salpingo-oophorectomy, and bilateral
pelvic and aortocaval lymph nodes sampling up
to inferior mesenteric artery level, along with
supracolic and infracolic omentectomy, and
multiple peritoneal biopsies was carried out.
Uterus was preserved. In our patient while right
ovarian tumor was the benign type of SBT, the left
ovarian tumor was the aggressive micropapillary
type of SBT. Conclusion: This case emphasizes
the need of subclassification of serous borderline
tumors showing a broad spectrum of clinical
and biological behavior from benign to low
grade carcinoma. We suggest that in patients
of reproductive age with infertility and adnexal
masses, despite malignant cells in ascites, serous
borderline tumor should be kept as a differential
diagnosis and conservative surgery be offered.
Keywords: Micropapillary serous carcinoma,
Typical SBT, Malignant ascites, Staging laparotomy, Low malignant potential tumors
*********
Kumar VJ, Jain D, Mridha A, Nanda S, Jain J, Grover RK.
A young nulliparous woman with right ovarian serous
borderline tumor and left ovarian micropapillary serous
carcinoma. International Journal of Case Reports and
Images 2013;4(9):471–476.
*********
doi:10.5348/ijcri-2013-09-359-CR-3
Received: 16 November 2012
Accepted: 1 June 2013
IJCRI 2013;4(9):471–476.
Introduction
Serous borderline tumors (SBT) of ovary are an
enigmatic group which are neither wholly benign nor
frankly malignant. They occur mostly in premenopausal
age group, have favorable prognosis, with 10 years
survival rate of nearly 95% for stage 1 tumors. They may
recur even after 20 years, hence they are labeled as low
malignant potential (LMP) tumors. About half of all LMP
tumors are serous tumors [1]. Mean age of borderline
serous tumors is 40 years, which is two decades earlier
than invasive cancer. [2] According to FIGO and WHO,
stromal invasion, defined as destructive infiltrative
growth, is the sole criterion used to distinguish SBT from
invasive serous carcinomas [2].
Furthermore, SBT have been subclassified into
benign and malignant types according to their biologic
behavior. Micropapillary serous carcinoma (MPSC) is a
proliferative, serous, ovarian neoplasm, characterized
by a micropapillary pattern. Though it lacks destructive
infiltrative growth, yet it behaves as a low-grade invasive
carcinoma, hence is more aggressive than a typical
ovarian serous borderline tumor. They are also associated
with extraovarian invasive peritoneal implants and more
frequent recurrences than in typical ovarian serous
borderline tumors. [3] In absence of invasive implants,
MPSC does not imply unfavourable prognosis, and is
much closer in its biologic behaviour to SBT than to serous
carcinomas, hence it is retained in LMP category [4, 5].
Typical SBT as well as those with noninvasive implants
are placed at lower end of proliferative spectrum.
We present the case of a patient who presented with
massive ascites and bilateral ovarian tumors who was
taken up for laparotomy and on surgical staging was
diagnosed as bilateral borderline ovarian tumors in stage
1C with typical non-invasive SBT in right ovary and more
aggressive non-invasive micropapillary type SBT in left
ovary.
CASE REPORT
On 24th June 2010, a nulliparous female, aged 25
years, married for four years, presented with progressive
abdominal distension due to massive ascites (Figure 1).
There was no history of fertility inducing drugs but one
year back, she had undergone ovarian cystectomy in a
private hospital, the details of which were not available.
There was loss of appetite and weight but no history of
hormone intake. She had hypomenorrhea. There were no
signs and symptoms of bowel disturbance or any family
history of cancer. On examination, she was emaciated
with hugely protuberant abdomen due to ascites. There
was mild bilateral pedal edema but no lymphadenopathy,
or hepatosplenomegaly. Vague mass was palpable in
suprapubic area. Perspeculum examination showed,
normal cervix displaced backwards. On bimanual
pelvic examination, uterus was not felt separately but
Kumar et al. 472
incorporated in bilateral adnexal masses, which were felt
through both fornixes. A large tumor about 6x5 cm, hard
to feel was impacted in pouch of douglas. Rectal mucosa
was smooth and mobile. Investigations revealed normal
hepatic, renal and hematological profile. Preoperative
serum Ca125 (marker for ovarian carcinoma) was
191 U/mL, CEA (colon carcinoma), CA19.9 (gallbladder
and pancreatic carcinoma) and CA 72.4 (pancreatic and
gastric carcinoma), b-hCG (germ cell tumor of ovary), AFP
(hepatobiliary malignancy and germ cell tumor of ovary)
were normal. Contrast computed tomography (CT) scan
revealed huge ascites with bilateral, complex, solid cystic
adnexal heterogeneous mass, which measured 8x10 cm
on the right side and 7x8 cm on the left side. Uterus,
liver, gallbladder, spleen, pancreas, both kidneys, bones
and joints were normal. No significant retroperitoneal
lymphadenopathy was seen. Computed tomography scan
was suggestive of bilateral ovarian tumors with ascites.
First diagnostic ascitic fluid tap revealed clear yellow,
serous fluid with atypical cell clusters suspicious of
malignancy, while second tap was suggestive of metastatic
adenocarcinoma (Figure 2). Adenosine deaminase
(ADA) test which is indicative of tubercular etiology was
performed in the ascitic fluid. Its value was in the normal
range, thus ruling out abdominal tuberculosis.
Upper gastrointestinal tract endoscopy revealed grade 1
esophageal varices, at the lower end at 3’ O clock position.
Stomach and duodenum were normal. Colonoscopy
could not be done due to improper bowel preparation.
Endometrial biopsy revealed secretory endometrium with
no evidence of malignancy and PAP smear was normal.
On 25th July 2010, after informed consent the patient
underwent staging exploratory laparotomy. Surgery
included inspection and palpation of abdomen and
pelvic organs, ascitic fluid cytology, bilateral salpingooophorectomy, bilateral pelvic and aortocaval lymph
nodes sampling up to inferior mesenteric artery level,
along with supracolic and infracolic omentectomy, and
multiple peritoneal biopsies. Both the fallopian tubes
were also removed, however, uterus was preserved.
Peroperatively, six liters of clear, straw colored ascitis.
On gross appearance uterus was normal, no normal,
ovary seen. The ovaries were replaced by ovarian tumors.
The ovarian tumors were separate but together they
were impacted in the pelvis. Moreover, the left ovarian
tumor was adherent to the sigmoid mesentery. The
plane of cleavage was maintained. Liver, under surface
of diaphragm, abdominal and pelvic peritoneum, ileal
mesentery, stomach, small and large intestines and
omentum were normal. Left pelvic lymph nodes and
para-aortic lymph nodes were palpable and enlarged
and were thoroughly dissected, while on the right side
they were not enlarged and were palpated to be normal.
Hence, right nodes sided were only sampled.
Histopathology revealed right ovary to be 5.4x5.0
cm, multicystic with solid papillary fronds, without any
breach of capsule (Figure 3). Left ovary was 6.5x5.8 cm
and similar to the right ovary (Figure 3). Right ovarian
IJCRI 2013;4(9):471–476.
tumor did not show any destructive stromal invasion.
Mild nuclear atypia with low mitosis and psammoma
bodies were seen (Figure 4). It was labeled as non-invasive
serous borderline tumor, of typical type following WHO
classification. While no stromal invasion was there in left
ovarian tumor as well, there were foci of fused solid areas
and occasional mitosis, in foci of >5 mm in length (Figure
5). It was labeled as non-invasive SBT, micropapillary type
(micropapillary serous carcinoma). Omentum, appendix,
peritoneal biopsies, pelvic and para-aortic lymph nodes
were devoid of metastasis or implants.
Immediate postoperative recovery was uneventful.
Drain was kept until stitch removal on postoperative day12 as there was substantial amount of fluid from the drain.
Postoperatively her CA 125 was 5.89 U/mL. Subsequent
to discussion with our clinical oncology team, decision
of adjuvant chemotherapy, was taken to give, keeping in
view the biologic behavior of micropapillary type of left
ovarian tumor. Postoperative recovery was uneventful but
after first cycle of chemotherapy she developed parietal
wall abscess. The abscess burst through the surgical
stitch line and drained massive amount of pus which
tested sterile on culture. Tests for tuberculosis performed
on the pus were negative. Wound was debrided and left
to heal by secondary intention. After this initial delay,
she uneventfully completed six cycles of three weekly
chemotherapy with carboplatin and paclitaxel. Currently,
she is disease free for one and half years with latest report
of CA 125 of 4.32 U/mL and normal CT scans of abdomen
and pelvis.
DISCUSSION
Micropapillary serous carcinoma (MPSC) is a
proliferative, serous ovarian neoplasm which is without
destructive infiltrative growth but still behaves as a lowgrade invasive carcinoma and hence considered as an
in situ form of serous carcinoma [6]. In the absence of
invasive implants, MPSC has favorable prognosis with
Figure 1: Massive abdominal distension with ascites.
Kumar et al. 473
Figure 2: Ascitic fluid cytology showing atypical cells suggestive
of metastatic adenocarcinoma. (H&E, x400)
Figure 3: Showing right ovary (5.4x5.0 cm), left ovary
(6.5x5.8 cm). Both were multicystic, with solid papillary fronds
without any breach of capsule.
Figure 4: Photomicrograph showing typical non-invasive
serous borderline tumor in right ovary, depicting thick papillae,
heirarchial branching, no destructive stromal invasion, mild
nuclear atypia, low mitosis and psammoma bodies. (H&E,
x200)
IJCRI 2013;4(9):471–476.
Figure 5: Showing non-invasive SBT, micropapillary
type (micropapillary serous carcinoma) in left ovary with
complex papillae, non-hierarchical branching, foci of fused
solid areas and cribriform pattern, occasional mitosis in area of
>5 mm in length but no destructive stromal invasion.
biologic behavior similar to serous borderline tumors [7,
8].
Twenty-six cases of MPSC were identified in a review
of 400 cases of ovarian SBT. None of the stage 1 patients
had recurrence, but in higher stages 50% died of the
disease. Twenty-four (92%) of MPSC cases were SBT
associated. It was concluded that micropapillary type
of SBT may progress to invasive carcinoma in some
instances [8]. Drescher et al. studied significance of DNA
content and nuclear morphology as prognostic factors to
predict aggressiveness of borderline ovarian tumors and
to guide adjuvant chemotherapy. [9]
Clinical presentation of LMP tumors is with abdominal
distension, pain, and pelvic mass which is same as that
of malignant tumors. Young patients have associated
infertility or pregnancy. History of infertility increases
the risk of LMP tumors with odds ratio (OR) of 1.9, while
use of infertility drugs has OR of 4. [10] In patients with
serous borderline tumors, after ovarian cystectomy,
8–15% patients have recurrence in the ipsilateral or
contra lateral ovarian tissue. Bilaterality or synchronous
and metachronous tumors are common and seen in about
40% patients [10].
In our patient there was bilateral involvement
of ovaries; the tumors in right and left ovary were
separate. While right ovary had benign type of typical
borderline serous tumor, the left ovary had noninvasive
borderline serous tumor of micropapillary type, which
as the literature suggests is a more aggressive subtype.
Preoperatively ascitic fluid cytology did have malignant
cells, however there were no implants detected in the
peritoneal lining.
Kumar et al. 474
About 80% of low malignant potential (LMP) tumors
are diagnosed in stage 1. Some have extraovarian tumor
implants on peritoneal surface of pelvis or abdomen or
both. Though some consider these implants to represent
metastasis, yet others consider them to represent
synchronous extraovarian proliferations arising from
surface coelomic epithelium as a result of multifocal field
change. Spontaneous regression of extraovarian foci has
been reported following resection of ovarian primary
tumor. [11]
These extraovarian tumor implants could be invasive
or non-invasive. Serous borderline tumors with invasive
implants and micropapillary type of ovarian serous
borderline tumors are aggressive subgroups and are
classified as carcinomas. They are associated with poor
prognosis and are treated with adjuvant chemotherapy.
[4, 11]
The LMP or borderline neoplasms have 10 years
survival rate of 95% for stage 1 patients. [2] Overall
survival is 90–95% at 5 years and 80% at 20 years. In
another study, disease-specific survival was >95% for
patients with lowstage (stage I) tumors and approximately
65% for patients with highstage (stage II–IV) tumors.
Thus, although both the typical type of borderline
serous tumors and micropapillary type of serous
borderline tumors appear to be noninvasive histologically,
the former tumor is benign, while the latter behaves
like a low grade carcinoma. Poor prognosis for MPSC
prompts clinicians to use adjunctive chemotherapy [4].
According to FIGO and WHO, stromal invasion defined
as destructive infiltrative growth is the sole criterion used
to distinguish SBT from invasive serous carcinomas. An
aggressive subgroup of proliferative serous lesions with
micropapillary projections are named as micropapillary
serous carcinoma (MPSC). These tumors and SBT with
invasive implants may be associated with malignant
behavior [5].
Serous borderline tumors have a very favorable
prognosis, but complete surgical staging and prolonged
follow-up are advised because pelvic recurrence and
occasionally transformation to invasive carcinoma may
occur, albeit over delayed period of time. Designation
of benign subgroup of SBTs as “atypical proliferative
tumors” is not recommended because it discourages
complete surgical staging and follow-up. Advanced stage
tumors with noninvasive implants are common and
behave in a benign fashion, and can be safely treated
conservatively.
Although histologically ovarian MPSC lack stromal
invasion and therefore are qualified by FIGO and WHO
guidelines for inclusion in borderline category, clinically
they behave more aggressively than typical serous
borderline tumors. Hence, the SBTs associated with
invasive implants and SBT-MPSC could behave as low
grade carcinoma and their management should be seen
in this light.
Various subtypes of serous borderline tumors are
summarized in Table 1. [12]
IJCRI 2013;4(9):471–476.
Kumar et al. 475
Table 1: Classification of serous borderline tumors. [7, 12]
Terms
Typical serous borderline Typical serous borderline Micropapillary serous carcinoma with or
tumor (SBT) without invasive tumor
with
invasive without invasive implants
implants
implants
(Serous tumors of LMP)
Synonym
Atypical proliferative serous
tumor
Clinical behavior
Benign
Histopathology
1. No stromal invasion
2. Heirarchial pattern
branching
Prognosis
Good
Molecular biology
Mutations of kras and braf/
erb2 mutations
Implants
Not seen
Invasive implants present
Recurrences
Not frequently seen
Late recurrences may occur Invasive recurrences are more frequent
Low grade (invasive) or non-invasive
micropapillary serous carcinoma
Low grade malignant
Low grade malignant
Micropapillary pattern with small
of 2. Heirarchial pattern of 2. branching
uniform filiform papillae, with cores
3. Invasive implants
having no stroma
3. At least one confluent area 5 mm in
present
greatest dimension in at least one slide.
4. Extraovarian invasive or non-invasive
implants
Aggressive as compared to May progress to invasive carcinoma
typical ovarian SBT
Mutations of kras and braf and loss of 1p36
and loss of CDKN2A/B
Extraovarian invasive implants are more
frequent
Abbreviation: LMP - Low malignant potential
CONCLUSION
We suggest, that in cases in reproductive age group
with infertility and adnexal masses, despite presence
of malignant cells in ascites we should keep serous
borderline tumors in mind and offer conservative surgery.
Our case suggests that MPSC and typical type of
SBT represent two ends of a broad spectrum of clinical
and biologic behaviour seen in low malignant potential
ovarian tumors. The MPSC are associated and probably
arise from SBT and may account for few cases of SBT
which progress to invasive carcinoma. There is the need
of subclassification of serous borderline tumors.
*********
Vinita Jaggi Kumar – Conception and design, Acquisition
of data, Analysis and interpretation of data, Drafting the
article, Final approval of the version to be published
Dhruv Jain – Conception and design, Analysis and
interpretation of data, Critical revision of the article,
Asit Mridha – Acquisition of data, Drafting the article,
Sidharth Nanada – Conception and design, Drafting the
Jyotica Jain – Analysis and interpretation of data, Critical
revision of the article, Final approval of the version to be
published
R K Grover – Conception and design, Critical revision of
the article, Final approval of the version to be published
Guarantor
Copyright
© Vinita Jaggi Kumar et al. 2013; This article is distributed
under the terms of Creative Commons attribution 3.0
www.ijcasereportsandimages.com/copyright-policy.php
REFERENCES
1.
Russel P, Merkur H. Proliferating ovarian “epithelial”
tumours: a clinico-pathological analysis of 144 cases.
Aus NZ J Obstet Gynecol 1979;19(1):45–1.
IJCRI 2013;4(9):471–476.
2. Epithelial borderline tumors of the ovary. Age
distribution. Annual report gynaecological cancer.
FIGO 1994:22.
3. Piura B, Rabinovich A, Yanai-Inbar I. Micropapillary
serous carcinoma of the ovary: Case report and review
of literature. Eur J Gynaecol Oncol 2000;21(4):374–
6.
4. Seidman JD, Kurman RJ. Subclassification of
serous borderline tumors of the ovary into benign
and malignant types. A clinicopathologic study
of 65 advanced stage cases. Am J Surg Pathol
1996;20(11):1331–45.
5. Prat J, De Nictolis M. Serous borderline tumors
of the ovary: a long-term follow-up study of 137
cases, including 18 with a micropapillary pattern
and 20 withmicroinvasion. Am J Surg Pathol 2002
Sep;26(9):1111–28.
6. Kurman RJ, Trimble CL. The behaviour of serous
tumors of low malignant potential: Are they ever
malignant? Int J Gynecol Pathol 1993;12(2):120–7.
7. Seidman JD, Kurman RJ. Subclassification of
serous borderline tumors of the ovary into benign
and malignant types. A clinicopathologic study
of 65 advanced stage cases. Am J Surg Pathol
1996;20(11):1331–45.
other devices
Kumar et al. 8.
476
Burks RT, Sherman ME, Kurman RJ. Micropapillary
Serous Carcinoma of the Ovary: A Distinctive Lowgrade Carcinoma Related to Serous Borderline
Tumors. Am J Surg Pathol 1996;20(11):1319–30.
9. Drescher CW, Flint A, Hopkins MP, Roberts JA.
Prognostic significance of DNA content and nuclear
morphology in borderline ovarian tumors. Gynecol
Oncol 1993;48(2):242–6.
10. Lim-Tan SK, Cajigas HE, Scully RE. Ovarian
cystectomy for serous borderline tumors: a follow up
study of 35 cases. Obstet Gynacol 1988;72(5):775–81.
11. Hopkins MP, Kumar NB, Morley GW. An assessment
of pathologic features and treatment modalities in
ovarian tumors of low malignant potential. Obstet
Gynecol 1987;70(6):923–9.
12. Jaffery D Seidman, Kathleen R Cho, Brigitte M
Ponnet, Robert J Kurman. Surface epithelial tumors
of the ovary. In: Robert J Kurman, Lora Hedrick
Ellenson, Brigitte M Ronnett editors. Blaustein’s.
Pathology of the Female genital tract. 6th ed. USA,
Springer 2011:679–85.
other devices
IJCRI 2013;4(9):477–480.
Amr et al. CASE REPORT
477
OPEN ACCESS
Sigmoid carcinoma: A rare presentation and
diagnostic challenge
Bassem Amr, Komal Munir, Natasha Santana-Vaz,
Venkateswarlu Velineni
Abstract
Introduction: Adenocarcinoma represents less
than 2% of all bladder carcinomas. Metastatic
adenocarcinoma is the most common form
and usually represents a direct extension
from a primary lesion either in the colon,
prostate or the female genital organs. Other
categories include primary vesical or urachal.
Case Report: We present a case of 79-yearold
female
presented
with
microscopic
heamaturia, frequency, urgency and weight
loss. An adenocarcinoma of the urinary bladder
of intestinal origin was founded. Conclusion:
Primary adenocarcinoma of the urinary bladder
is uncommon neoplasm and the reported
incidence of the primary adenocarcinoma is 0.5–
2%. It represents a diagnostic challenge raising
the inquiry about the site of the lesion. Three
major classes were identified: primary vesical
adenocarcinoma,
urachal
adenocarcinoma
and extravesical adenocarcinoma involving the
bladder. The metastatic adenocarcinoma is the
most common category representing a direct
extension from a nearby organ or metastatic
spread. The most common treatment for
urinary bladder adenocarcinoma is surgery.
Generally speaking, treatment options include:
surgery, radiotherapy, chemotherapy and
immunotherapy. Metastatic adenocarcinoma
Bassem Amr1, Komal Munir1, Natasha Santana-Vaz1,
Venkateswarlu Velineni1
Affiliations: 1Worcestershire Acute Hospitals NHS Trust,
Woodrow Drive, Redditch, B98 7UB.
Corresponding Author: Mr. Bassem Amr, Worcestershire
Acute Hospitals NHS Trust, Woodrow Drive, Redditch, B98
7UB. Ph: +44 1527 50 3030, Mob: +44 78 256 356 24; Email:
[email protected]
Received: 23 July 2012
to the urinary bladder especially of colonic
origin is of particular interest and need to be
ruled out before making a diagnosis of primary
adenocarcinoma of urinary bladder.
Keywords: Bowel
Bladder tumor
cancer,
Adenocarcinoma,
*********
Amr B, Munir K, Santana-Vaz N, Velineni V. Sigmoid
carcinoma: A rare presentation and diagnostic challenge.
2013;4(9):477–480.
*********
doi:10.5348/ijcri-2013-09-360-CR-4
Introduction
Adenocarcinoma represents less than 2% of all
bladder carcinomas [1]. Primary adenocarcinoma is an
uncommon malignant neoplasm and is a source of a
diagnostic challenge. It is less frequently encountered
in areas where bilharziasis is endemic whereas the
squamous cell carcinoma is more prevalent. This
incidence ranges between 5–11.4%. Chronic vesical
irritation and infection are predisposing factors for
metaplastic changes of the urothelium [2]. Based on the
original tumor site, adenocarcinoma of the bladder could
be classified into three categories: primary, urachal and
metastatic. Primary vesical adenocarcinoma needs to
be distinguished from the more common extravesical
metastatic adenocarcinoma (direct spread, lymphatic,
and hematogenous). The principal primary organs to be
considered include prostate, colon, female genital tract,
appendix, stomach and breast. Clinical association with
bladder exstrophy and schistosomiasis has been well
documented. Clinical assessment, imaging, histologic,
and immunohistochemical correlation should be done
IJCRI 2013;4(9):477–480.
while investigating this diagnosis due to histologic and
immunohistochemical overlapping between primary
bladder adenocarcinoma and metastatic adenocarcinoma
[3]. Prognosis and therapeutic options for primary versus
metastatic adenocarcinoma vary widely.
CASE REPORT
A 79-year-old female was presented to her general
practitioner in January 2012 with urinary symptoms in
the form of frequency, urgency and passing dark urine.
She had also noted recent weight loss of 3 kg. Urine
dipstick was positive for leukocytes, protein and blood.
Antibiotic was prescribed by her doctor for urinary
tract infection. The symptoms recurred once the course
of treatment was completed; for which she was given
three separate courses of antibiotics as her mid-stream
urine samples showed E. coli. Her ultrasound scan of the
urinary tract showed a simple cyst on the right kidney
and incidental finding of a bulky uterus; therefore pelvic
scan was undertaken. A retroverted bulky fibroid uterus
measuring 6.5x2.9x4.6 cm was revealed. A heterogenous
myometrium demonstrating echogenic foci throughout
anteriorly was founded and a well-defined echogenic
area within the left adnexa measuring 2.5 cm with small
amount of free fluid was seen on the scan. She was
referred to the hematuria clinic on April 2012, where a
flexible cystoscopy was performed under local anesthesia.
There was an inverted granuloma present at the trigone
and an area of inflammation on the right-hand side of
the posterior wall. Computed tomography (CT) scan of
the abdomen and pelvis showed marked sigmoid colon
thickening with associated diverticulosis suggestive
of complicated sigmoid diverticulitis with a localized
perforation. Also a focal urinary bladder wall thickening
was likely secondary to the colonic abnormality with no
definitive evidence of a colovesical fistula on this scan
(Figure 1). She was admitted to our hospital for cystoscopy
and biopsy. Examination under anesthesia revealed
a 5-cm pelvic mass extending into the left iliac fossa.
Cystoscopy revealed a lesion in the trigone (Figure 2)
which was biopsied. Histopathological findings of the
cystoscopic biopsy revealed moderately differentiated
adenocarcinoma of an intestinal appearance infiltrating
bladder mucosa. Her case was discussed at the urology
and colorectal multidisciplinary team (MDT) meetings. A
staging CT scan showed a sigmoid colon tumor, possibly
involving the roof of the bladder with lymphadenopathy
and no evidence of distant metastatic disease. Her blood
investigations were within normal limits before she was
scheduled for anterior resection, partial cystectomy
and total abdominal hysterectomy. She was admitted
to intensive care unit after a long procedure where
she received noradrenaline support for 48 hours and
transfused two units of blood before discharging her back
to the ward. She made an uneventful recovery before being
discharged with a long-term catheter and an outpatient
Amr et al. 478
appointment for cystogram and chemotherapy. Patient
was followed-up on July 2012 after satisfactory cystogram
and again on October 2012 by consultant surgeon. She
was doing well and managed to maintain her weight. The
final histology confirmed a moderately differentiated
adenocarcinoma of colonic origin (pT4 pN0) invading the
detrusor muscle of the bladder. The myometrium, both
adnexa and cervix were unremarkable.
Figure 1: Thickened wall of sigmoid colon and area of possible
perforation with gas bubbles.
Figure 2: Cystoscopic picture demonstrating a lesion at the
trigone.
IJCRI 2013;4(9):477–480.
Amr et al. DISCUSSION
Adenocarcinoma of the urinary bladder is uncommon,
representing less than 2% of malignant neoplasms at this
site. Adenocarcinoma can arise anywhere in the urinary
bladder. Nevertheless, in most cases they involve the
trigone and posterior bladder wall [1]. About two-thirds
of these tumors present as solitary, discrete lesions,
unlike the ‘usual’ urothelial carcinomas, which tend to be
multifocal [2]. These tumors are more common in men
with a male-female ratio of 3:1. It is usually occurring
in the fifth to seventh decades of life. Direct extension
of adenocarcinoma from adjacent organs such as the
prostate, colon or ovary is more common than primary
adenocarcinoma of the urethra or urinary bladder [3].
Metastatic lesions to urinary bladder often infiltrate the
wall of the bladder rather than ulcerating the mucosa. In
some cases, the bladder is the only genitourinary organ
involved in metastasis, mainly from the breast. Most
of these patients with urinary bladder metastases are
asymptomatic. Symptoms produced by these metastases
occur only when the bladder mucosa is involved [4].
These tumors show varied histological pictures and
degrees of differentiation. Primary adenocarcinoma
of the urinary bladder exhibit several histological
subtypes: glandular not otherwise specified, mucinous
(colloid), colonic (enteric) type, signet ring cell, clear cell
(mesonephric) type and mixed type [5, 6].The majority
of the primary adenocarcinoma is of the enteric type and
it is morphologically indistinguishable from metastatic
adenocarcinoma of colonic origin when evaluated by
the cytological, histopathological, histochemical and
immunological techniques. However certain features,
including the location of the tumor, growth pattern,
and clinical history, have been suggested as useful aides
for differentiating metastatic colonic adenocarcinoma
from primary enteric-type adenocarcinoma of urinary
bladder [7, 8]. It is important to discriminate primary
adenocarcinoma of the urinary tract from metastatic
colonic adenocarcinoma because of their differing
treatment protocols as well as its morphological
similarity. Clinical and radiological correlation and
ultimately histological confirmation are strongly
recommended for confirmation of the diagnosis especially
in cases of unknown primary [9]. Treatment options
include: surgery, radiation therapy, immunotherapy
and chemotherapy. For small bladder tumors, partial/
segmental cystectomy could be performed. However, for
large size tumors radical cystectomy is recommended
[10].
CONCLUSION
The diagnosis of urinary bladder adenocarcinoma
raises the challenging question of whether the lesion is
primary vesical, urachal in origin or metastatic from a
distant or an adjacent organ.
479
*********
Bassem Amr – 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
Komal Munir – Aquisition of data, Revising it critically
for important intellectual content, Final approval of the
Natasha Santana-Vaz – Aquisition of data, Revising
it critically for important intellectual content, Final
Venkateswarlu Velineni – Aquisition of data, Revising
Guarantor
Copyright
© Bassem Amr et al. 2013; This article is distributed
REFERENCES
1.
Gill HS, Dhillon HK, Woodhouse CR. Adenocarcinoma
of the urinary bladder. Br J Urol 1989;64(2):138–42.
2. Thomas DG, Ward AM, Williams JL. A study of 52
cases of adenocarcinoma of the bladder. Br J Urol
1971;43(1):4–15.
3. El-Bolkainy MN, Mokhtar NM, Ghoneim MA, Hussein
MH. The impact of schistomiasis on the pathology of
bladder carcinoma. Cancer 1981;48(12):2643–8.
4. ALLEN TD, HENDERSON BW. Adenocarcinoma of
the bladder. J Urol 1965;93:50–6.
5. Wang HL, Lu DW, Yerian LM, et al.
Immunohistochemical distinction between primary
adenocarcinoma of the bladder and secondary
colorectal adenocarcinoma. Am J Surg Pathol
2001;25(11):1380–7.
6. Torenbeek R, Lagendijk JH, Van Diest PJ, Bril H,
van de Molengraft FJ, Meijer CJ. Value of a panel
of antibodies to identify the primary origin of
adenocarcinomas presenting as bladder carcinoma.
Histopathology 1998;32(1):20–7.
7. Bennett JK, Wheatley JK, Walton KN. 10-year
experience with adenocarcinoma of the bladder. J
Urol 1984;131(2):262–3.
IJCRI 2013;4(9):477–480.
8. Melicow MM. Tumors of the urinary bladder: a
clinicopathological analysis of over 2500 specimens
and biopsies. J Urol 1955;74(4):498–521.
9. Perez-Mesa C, Pickren JW, Woodruff MN, Mohallatee
A. Metastatic carcinoma of the urinary bladder from
primary tumors in the mammary gland of female
patients. Surg Gynecol Obstet 1965;121(4):813–8.
other devices
Amr et al. 480
10. Grignon DJ, Ro JY, Ayala AG, Johnson DE, Ordóñez
NG. Primary adenocarcinoma of the urinary bladder:
a clinicopathologic analysis of 72 cases. Cancer
1991;67(8):2165–72.
other devices
IJCRI 2013;4(9):481–484.
Mahmoud CASE REPORT
481
OPEN ACCESS
Neglected case of congenital hypothyroidism in a 17-yearold female
Mahmoud Medhat Mahmoud
Abstract
*********
Introduction: Congenital hypothyroidism is
one of the most common endocrinal disorders
in newborns. The slow development of obvious
clinical symptoms, coupled with the importance
of early treatment led to the implementation of
widespread newborn screening for this condition.
Case Report: This paper presents a case of
congenital hypothyroidism that was born at
home and was not screened for hypothyroidism.
A 17-year-old female patient complained of short
stature, mental retardation, delayed puberty
and chronic constipation as a result of neglected
hypothyroidism. Conclusion: This case discusses
the importance of neonatal screening programs
for early detection of congenital hypothyroidism
cases that are unfortunately still being neglected
in some areas of developing countries. It
also shows the value of early and adequate
replacement therapy for the detected cases as
this could protect them against irreversible
growth retardation and mental retardation.
This is because any diagnostic delay, inadequate
treatment or even poor compliance to treatment
is always associated with irreversible damage as
those manifested in this case.
Keywords: Hypothyroidism,
Neonatal screening
Short
stature,
Mahmoud Medhat Mahmoud1
Affiliations: 1Alexandria Faculty of Medicine–Fifth Year Medical Student, Alexandria University, Alexandria, EGYPT
Corresponding Author: Mahmoud Medhat Mahmoud, 89
Ebn Monkez St. behind Al Horreya Police Station, Victoria,
Alexandria, EGYPT- 21611; EGY +2 0100-356-7957; Email:
[email protected]
Received: 18 January 2013
Mahmoud MM. Neglected case of congenital
hypothyroidism in a 17-year-old female. International
Journal of Case Reports and Images 2013;4(9):481–484.
*********
doi:10.5348/ijcri-2013-09-361-CR-5
Introduction
Congenital hypothyroidism is defined as thyroid
hormone deficiency present at birth. Problems with
thyroid gland development (dysgenesis) and disorders of
thyroid hormone biosynthesis (dyshormonogenesis) are
the most common causes of congenital hypothyroidism.
Newborn screening for hypothyroidism is not done
in many third world countries. Only an estimated onethird of the worldwide birth population is screened. It
is, therefore, important that clinicians could be able to
recognize and treat the disorder [1]. Also health education
with early signs and symptoms of the condition is very
important especially in areas where neonatal screening
against hypothyroidism is not performed.
This case report describes a neglected case of
hypothyroidism that was left untreated for 17 years.
The reasons behind this negligence are mainly that the
patient was not screened against hypothyroidism at birth.
In addition, her parents were not aware of the signs of the
condition.
CASE REPORT
A 17-year-old patient was brought by her mother to
Alexandria University Hospital, complaining of delayed
puberty, short stature, mental retardation and chronic
constipation. She was born at home to a family of low
socio-economic conditions and was not screened for
hypothyroidism at birth. She reported that the patient
IJCRI 2013;4(9):481–484.
Mahmoud 482
was hypoactive and not interested in the surroundings
since early childhood, but the case was neglected and the
mother never asked for medical advice before.
Upon physical examination the patient was obese.
She had very short stature with a height of 129 cm (< 3rd
percentile). She had large head, short neck, coarse facial
features with myxedematous appearance, macroglossia
and depressed nasal bridge (Figure 1). Her skin was cold
and dry. The patient’s vital signs were normal.
Abdominal examination revealed generalized
abdominal distention (pot belly) with umbilical hernia,
otherwise abdominal ultrasound revealed normal
abdomen. Neurological examination showed generalized
hypotonia and hyporeflexia with positive Woltman
sign (delayed relaxation phase of elicited deep tendon
reflexes). The patient was mentally retarded and had low
IQ level. The computed tomography (CT) scan of brain
was normal. Pulmonary and cardiac examinations were
within normal limits.
Laboratory examination showed high TSH levels.
TSH measured 536 IU/mL, with low levels of free T4 and
T3 (T4 was 32 ng/mL, T3 was 0.6 ng/mL). Accordingly,
diagnosis of primary hypothyroidism was established
and replacement therapy with levothyroxine was started
to normalize the levels of TSH. Chronic constipation was
treated with laxatives.
After two years of treatment, the condition improved
slightly where the patient became more oriented and
interested in the surroundings, constipation was treated
and artificial menstruation was induced at the age of 19
years.
DISCUSSION
The incidence of congenital hypothyroidism based on
neonatal laboratory screening is greater than the incidence
based on clinical signs and symptoms. The incidence of
the condition depending on clinical diagnosis has been
found to vary between 1:5800 and 1:6900, whereas
based on neonatal screening the incidence is between
1:2900 and 1:3600. This discrepancy may be due to the
fact that some children with the disorder go undetected
when general laboratory screening is not utilized. On the
other hand, neonatal laboratory screening will detect
some children with minor disturbance of thyroid function
which are clinically insignificant [2].
Thyroid hormones are important for normal
growth hormone and IGF functions. Hypothyroidism
is associated with a reduction in the pulsatile growth
hormone secretion and in growth hormone response
to stimulatory tests. In hypothyroidism, serum levels
of IGF-I and IGFBP-3 fall dramatically. In a study of 12
patients with hypothyroidism, it was found that long-term
replacement therapy in children with hypothyroidism
is associated with a physiological increase in IGF-1 and
IGFBP-3 [3]. However, studies have also concluded
that catch-up growth in hypothyroidism may be
Figure 1: Short stature (height: 129 cm) and myxedematous
appearance in a 17-year-old female patient with neglected
congenital hypothyroidism.
incomplete if treatment has been started shortly before
or during puberty [4]. So despite treatment, prolonged
hypothyroidism may result in compromised adult height
in some patients. The contributing factors to this height
deficit may include the duration of hypothyroidism, the
height deficit at the time of the diagnosis, etiological
differences and the diminished potential for catch-up
growth in late-diagnosed hypothyroidism [5].
A follow-up study of 30 patients with congenital
hypothyroidism concluded that early detection by
neonatal screening and treatment enables normal
prepubertal and pubertal growth and the achievement
of normal adult height, following normal puberty. Adult
height in congenital hypothyroidism is significantly
correlated with parental height and the mean L-T4 daily
dose administered over the first six months of treatment
[6].
IJCRI 2013;4(9):481–484.
Another complication of neglected or inadequately
treated hypothyroidism is mental retardation. Normal
brain development depends on delivery of adequate
thyroid hormone for the first 2–3 years of life. Low
thyroid levels during this time may result in irreversible
damage, whereas hypothyroidism developed after the age
of 3 years, it leads to reversible effects if it is early and
adequately treated [1].
The New England Congenital Hypothyroidism
Collaborative reported that a subgroup of 18 infants who
had low serum T4 levels (average T4 8.6 µg/dL) and low
l-thyroxine dosing (< 5 µg/kg/day) with a history of poor
compliance in the first three years of life, had a mean IQ
of 87. The larger, adequately treated group, with a serum
T4 in the target range (average T4 11.2 µg/dL), had an IQ
score of 105. This study concluded that T4 values in the
lower part of the normal range are probably incompatible
with maximal intellectual development and every effort
should be exerted to maintain serum T4 levels in the
upper half of the normal range during the first year of life
[7].
It was found that diagnostic delay of hypothyroidism
was associated with a steady decline in mean IQ
(Wechsler intelligence scale) in a retrospective study of
141 hypothyroid children where the mean IQ was 79.5 for
children with congenital hypothyroidism, but was normal
in children diagnosed before the age of 6 weeks [8]. And
many other studies concluded that IQ levels of early
treated congenital hypothyroidism patients detected by
neonatal screening programs can be within normal range
and they can have normal school attainments [9].
CONCLUSION
In conclusion, growth retardation and mental
retardation of children due to hypothyroidism can be
totally prevented by three things. First to follow a strict
neonatal screening program for hypothyroidism that
is unfortunately still being neglected in some areas of
the developing countries. Therefore, health education
to parents in these countries about the main signs and
symptoms of hypothyroidism is very important. Second
is the adequate and very early L-thyroxin replacement
therapy. Third is very careful follow-up of patients and
adjustment of the L-thyroxin dose to maintain serum
T4 levels in the upper half of the normal range during
the first year of life. This is because any diagnostic
delay, inadequate treatment or even poor compliance to
treatment is always associated with irreversible damage
as those manifested in this case.
Mahmoud 483
Drafting the article, Critical revision of the article, Final
Guarantor
Copyright
© Mahmoud Medhat Mahmoud et al. 2013; This article
REFERENCES
1.
Maynika V Rastogi, Stephen H LaFranchi. Congenital
hypothyroidism. Orphanet J Rare Dis 2010;5:17.
2. Alm J, Hagenfeldt L, Larsson A, Lundberg K. Incidence
of congenital hypothyroidism: Retrospective study
of neonatal laboratory screening versus clinical
symptoms as indicators leading to diagnosis. Br Med
J (Clin Res Ed) 1984 Nov 3;289(6453):1171–5.
3. Bona G, Rapa A, Boccardo G, Silvestro L, Chiorboli
E. IGF-1 and IGFBP-3 in congenital and acquired
hypothyroidism
after
long-term
replacement
treatment. Panminerva Med 1998 Jun;40(2):103–6.
4. Boersma B, Otten BJ, Stoelinga GB, Wit JM. Catch-up
growth after prolonged hypothyroidism. Eur J Pediatr
1996 May;155(5):362–7.
5. Kandemir N, Yordam N. Height prognosis in children
with late-diagnosed congenital hypothyroidism. Turk
J Pediatr 2001 Oct-Dec;43(4):303–6.
6. Dickerman Z, De Vries L. Prepubertal and pubertal
growth, timing and duration of puberty and
attained adult height in patients with congenital
hypothyroidism (CH) detected by the neonatal
screening programme for CH--a longitudinal study.
Clin Endocrinol (Oxf) 1997 Dec;47(6):649–54.
7.Characteristics
of
infantile
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discovered on neonatal screening. J Pediatr 1984
Apr;104(4):539–44.
8. Hulse JA. Outcome for congenital hypothyroidism.
Arch Dis Child 1984 Jan;59(1):23–9.
9. Bargagna S, Dinetti D, Pinchera A, et al.
School attainments in children with congenital
hypothyroidism detected by neonatal screening
and treated early in life. Eur J Endocrinol 1999
May;140(5):407–13.
*********
Mahmoud Medhat Mahmoud – Conception and design,
IJCRI 2013;4(9):481–484.
other devices
Mahmoud Access PDF of article on
other devices
484
IJCRI 2013;4(9):485–488.
Jadhav et al. CASE REPORT
485
OPEN ACCESS
Fallaciously elevated glucose level by handheld glucometer
in a patient with chronic kidney disease and hypoglycemic
encephalopathy
Praveen Pratap Jadhav, Meera Praveen Jadhav
Abstract
Introduction: The handheld glucometers are
commonly used for glucose estimation in acutely
ill patients, especially in those with neurological
deficit. However, in a few conditions like chronic
renal failure, it can give fallacious readings. This
has serious implications in management of these
patients, which can lead to misdiagnosis of the
underlying hypoglycemia. Case Report: Here we
present a case of a patient with hypoglycemia
who had a fallacious normal glucose level when
checked by a handheld glucometer (i.e., elevated
on glucometer). The underlying pathology giving
rise to this fallacy was presence of chronic kidney
disease with high uric acid and low hematocrit.
Conclusion: Treating physicians should be aware
of such possibilities and take suitable steps
for appropriate diagnosis of potentially lifethreatening hypoglycemia.
Keywords: Hypoglycemia, Glucometer, Chronic
kidney disease, Uric acid
*********
Praveen Pratap Jadhav1, Meera Praveen Jadhav2
Affiliations: 1MD, Consulting Physician–Omkar Heart Insitute, Nasik, MH, India; 2MS, Chief Medical Officer, Surgeon–
Omkar Heart Institute, Nasik, MH, India.
Corresponding Author: Praveen Pratap Jadhav, Omkar
Heart Institute, Gaikwad Mala, Nasik Road, Nasik, Maharashtra, India - 422101; Ph: 91-9822055612; Fax: 91-2532458585; Email: [email protected]
Accepted: 27 April 2013
Jadhav PP, Jadhav MP. Fallaciously elevated glucose
level by handheld glucometer in a patient with chronic
kidney disease and hypoglycemic encephalopathy.
2013;4(9):485–488.
*********
doi:10.5348/ijcri-2013-09-362-CR-6
Introduction
Precise diagnosis of acute critically ill unconscious
or mentally altered patient is very difficult. Care givers
rely on history, examination, imaging and laboratory
data for exact diagnosis. However, sometimes laboratory
data could be misleading and can lead to hazardous
consequences. One such condition, where laboratory
results can mislead, is the diagnosis of hypoglycemia in
patients with chronic renal failure. Here we present a
case where widely used handheld glucometers provided
a falsely high capillary blood sugar reading causing
diagnostic problems.
CASE REPORT
A 54-year-old lady was presented with a short history,
beginning with altered sensorium and drowsiness and
leading to unconsciousness in one hour. She was a
diabetic with history of chronic kidney disease on regular
treatment. During the initial stages of symptoms, the
relatives tried to feed her with oral glucose powder,
presuming this to be a hypoglycemic attack. However,
the patient was unable to ingest it. The blood sugar level
checked on Accu-Chek glucometer (Roche Diagnostics)
was 187 mg/dL. Since she did not respond to treatment
IJCRI 2013;4(9):485–488.
in a few minutes, she was shifted to the hospital for
further treatment. She was brought to the hospital deeply
unconscious. Her vital parameters were normal, heart
rate 100 per minute, blood pressure 110/70 mmHg and
respiratory rate 18 per minute. She was not responding
to deep pain, had flaccid paralysis of all four limbs, pupils
were constricted but reacting to light, deep tendon reflexes
were depressed and plantar reflexes could not be elicited.
Respiratory, cardiac and alimentary system examinations
were normal. Her capillary sugar checked on a different
Accu-Chek glucometer (Roche Diagnostics) was
167 mg/dL. Differential diagnoses of brain stem
cerebrovascular accident, metabolic encephalopathy
and uremic encephalopathy were considered. Her blood
sample was sent for investigations and her magnetic
resonance imaging (MRI) scan of the brain was done.
The MRI scan revealed bilateral symmetrical hyper
intense lesions in the internal capsule, corona radiata
and centrum semiovale, with reduced apparent diffusion
coefficient. An hour later her blood reports showed
hemoglobin 8.2 g/dL, normal blood counts, hematocrit
20%, creatinine 6 mg/dL, blood urea nitrogen 96 mg/
dL, sodium 132 mEq/L, potassium 5.2 mEq/L, uric
acid 9.2 mg/dL, normal hepatic enzymes and ammonia
levels and plasma glucose 24 mg/dL. She was infused
with 25% dextrose and then with 500 mL of 10% glucose
infusion. She responded dramatically in a few minutes.
She became conscious, started moving all the four
limbs with complete recovery of all neurological deficit.
Temporal event of positive neurological improvement
after intravenous glucose suggested the diagnosis of
hypoglycemic encephalopathy. Patient was treated with
initial intravenous and later oral glucose till plasma
Jadhav et al. 486
levels of sugar were stabilized. She was discharged
asymptomatic within 24 hours.
DISCUSSION
Hypoglycemia is a common emergency situation
in intensive care units. It is life-threatening, yet easily
treatable. The most common way to diagnose this condition
is with handheld glucometers. Some conditions, however,
have the potential to interfere with glucose measurements
by handheld glucometers and provide erroneous readings
of blood glucose. This can lead to misdiagnosis and wrong
treatment in patients with hypoglycemia. Various factors
are attributed to altered glucose measurement on the
strips. Some common reasons are given in Table 1.
Though most of these factors cause a minor variation
in estimated glucose, sometimes, especially with extreme
values of these factors, fallacious readings could be
obtained and these could be hazardous. In this patient,
though it was obvious that patient had hypoglycemia, it
was not detected by glucometer testing.
Glucometers use glucose oxidase (GOD) or glucose
dehydrogenase (GDH) enzymes to detect the presence
of glucose in blood. In these reactions, glucose is
metabolized by GOD or GDH to hydrogen peroxide or
reduced nicotinamide adenine dinucleotide, respectively.
The amount of hydrogen peroxide or rNAD can then
be measured by oxidized dye color change or by
electrochemical reactions to calculate the amount of
glucose present [1]. The glucose oxidase method makes
use of one or both of the following reactions:
Table 1: Sources of glucose monitoring inaccuracy
Factors
Variation
Mechanism
Strip factor
Strip-strip variation
Varying storage conditions. Variation of enzyme quantity in
different strips.
Physical factors
Altitude
Increased sensitivity of GOD biosensor strip to oxygen
concentration.
Temperature
Unpredictable response. Brand specific errors
Meter-strip miscoding
Altered relation between electrical signal produced by strip and
reported blood glucose.
Hematocrit
Glucose in RBCs is in equilibrium with plasma glucose, but at
lower levels. Extreme hematocrit alters the equilibrium.
Triglycerides
Takes up volume, thereby reducing the amount of glucose in
capillary volume.
Uric acid
Uric acid is oxidized by the electrodes in the tip of the strip.
L dopa, ascorbic acid, icodextrin
in dialysate solutions
These drugs interact with the electrodes at the tip of the strip to
give high false values.
Patient factors
Pharmacological factors
GOD – glucose oxidase, RBC – red blood cells
IJCRI 2013;4(9):485–488.
Glucose + O2
Jadhav et al. GOD
gluconic acid + H2O2 (1)
487
published
Guarantor
peroxidase
H2O2 + chromogen
chromophore (color) + H2O (2)
Many conditions can interfere with these chemical
reactions happening at the tip of the glucose measuring
strip. High levels of uric acid, which is a reducing agent,
may result in falsely high glucose readings [1]. Uric acid
lowers the value for glucose as determined by the GODPerid method. Bleaching of the final color is the source
of such interference in this reaction [2]. Reducing agents
other than glucose, such as metabolites of icodextrin,
may also be detected by these methods. This is a chemical
used in the dialysate for patients with end stage renal
failure on long-term peritoneal dialysis, and which is
partly absorbed in the circulation. High or low hematocrit
values can also lead to falsely abnormal glucose readings.
Low hematocrit value has also been shown to increase
glucose estimations on handheld glucometer [3]. Both,
low hematocrit and high uric acid, are common in critically
ill patients as well as in patients with renal failure. Hence,
it can be seen that patients with chronic kidney disease
may have multiple reasons to get falsely high glucose
readings when handheld glucometers are used. This
patient, though never dialyzed, had high uric acid and
low hematocrit values, which could have led to falsely
high glucose levels on the reagent strip. Since uric acid is
a reducing agent and though it is implicated as an agent
to give falsely elevated glucose in handheld glucometer,
we did not come across any reference or case reports
describing the same. The MRI scan of the patient was also
highly suggestive of hypoglycemic encephalopathy [4].
Copyright
© Praveen Pratap Jadhav et al. 2013; This article is
distributed under the terms of Creative Commons
REFERENCES
1.
2.
3.
4.
King DA, Ericson RP, Todd NW. Overestimation by a
Hand-Held Glucometer of Blood Glucose Level due to
Icodextrin. Isr Med Assoc J 2010 May;12(5):314–5.
Chinh NH. Mechanism of interference by uric acid
in the glucose oxidase-peroxidase method for serum
glucose. Clin Chem 1974 Apr;20(4):499–501.
Kang EG, Jeon SJ, Choi SS, Song CJ, Yu IK. Diffusion
MR imaging of hypoglycemic encephalopathy. AJNR
Am J Neuroradiol 2010 Mar;31(3):559–64.
Karon BS, Griesmann L, Scott R, et al. Evaluation of
the impact of hematocrit and other interference on the
accuracy of hospital-based glucose meters. Diabetes
Technol Ther 2008 Apr;10(2):111–20.
CONCLUSION
Hence, we suggest that in patients with chronic kidney
disease, especially due to diabetes, effort should be made
to diagnose and treat hypoglycemia accurately when they
present with altered mental status. This may involve not
over-relying on handheld glucometer, using laboratory
method to estimate blood glucose for confirmation or
empirically treating these patients with intravenous
dextrose without laboratory evidence of hypoglycemia.
*********
Praveen Pratap Jadhav – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
Meera Praveen Jadhav – Substantial contributions to
conception and design, Acquisition of data, Analysis and
interpretation of data, Revising it critically for important
IJCRI 2013;4(9):485–488.
other devices
Jadhav et al. Access PDF of article on
other devices
488
IJCRI 2013;4(9):489–493.
Min et al. CASE REPORT
489
OPEN ACCESS
Dual causes of multiple myeloma
Zaw Min, Zipporah Krishnasami, William J Cook, J Martin Rodriguez
Abstract
Introduction: Patients infected with human
immunodeficiency virus (HIV) infection have
been living longer secondary to dramatic
improvements in their immune status because
of highly active antiretroviral therapy (HAART).
Consequently, there is an increasing incidence
of non-AIDS defining malignancies and chronic
diseases in HIV-infected individuals. The
hepatitis C virus (HCV) co-infection is highly
prevalent in patients with HIV infection. Case
Report: We report a patient with HIV and
HCV co-infection who presented with multiple
myeloma, and explore literature looking for a
plausible causal association between multiple
myeloma, HIV and HCV infections. Conclusion:
Multiple myeloma is not a commonly associated
malignancy with HIV and/or HCV infection
although hyperglobulinemia is often associated.
Clinicians should be aware that multiple myeloma
may occur as a non-AIDS defining cancer in HIVinfected individuals and/or as an extrahepatic
manifestation in HCV-infected patients.
Zaw Min1, Zipporah Krishnasami2, William J Cook3, J Martin
Rodriguez4
Affiliations: 1MD, Infectious Diseases Fellow, Division of
Infectious Diseases, University of Alabama, Birmingham,
Alabama, USA; 2MD, Assistant Professor, Division of Nephrology, University of Alabama, Birmingham, Alabama, USA;
3
MD, PhD, Emeritus Professor, Department of Pathology,
University of Alabama, Birmingham, Alabama, USA; 4MD,
FACP, Associate Professor, Division of Infectious Diseases,
University of Alabama, Birmingham, Alabama, USA.
Corresponding Author: Zaw Min, MD, Infectious Diseases
Fellow, Division of Infectious Diseases, University of Alabama, THT 229, 1530 3rd Avenue South, Birmingham, AL
35294-0006 USA; Ph: 1-205-934-5191; Fax: 1-205-9345155; Email: [email protected]
Keywords:
Multiple
myeloma,
Human
immunodeficiency
virus
(HIV),
Acquired
immune deficiency syndrome (AIDS), Hepatitis
C infection
*********
Min Z, Krishnasami Z, Cook WJ, Rodriguez JM. Dual
causes of multiple myeloma. International Journal of
Case Reports and Images 2013;4(9):489–493.
*********
doi:10.5348/ijcri-2013-09-363-CR-7
Introduction
Human immunodeficiency virus (HIV)-infected
patients have benefited from dramatic improvements in
prognosis and life expectancy because of effective highly
active antiretroviral therapy (HAART). The incidence of
AIDS-defining neoplasms has consequently decreased
[1]. However, non-AIDS defining malignancies and
chronic diseases are increasingly being reported in HIVinfected populations [1–3]. Chronic hepatitis C virus
(HCV) infection is highly prevalent in HIV-infected
patients due to similarities in the epidemiology. Hepatitis
C virus infected patients frequently develop chronic liver
disease, but extrahepatic complications have also been
reported including HCV-associated B cell tumors [4, 5].
We describe a patient with HIV and HCV co-infection who
developed IgG lambda light chain multiple myeloma. We
review literature for a possible causal association between
multiple myeloma, HIV and HCV infection.
CASE REPORT
A 47-year-old African-American female co-infected
with HIV and HCV presented to our HIV clinic for routine
follow-up. She had been diagnosed with HIV and HCV
IJCRI 2013;4(9):489–493.
infections eight years before. She had been treated with
interferon and ribavirin for HCV infection, but she did not
complete therapy due to side effects from interferon. Her
HIV infection was well-controlled with anti-retroviral
therapy (zidovudine, lamivudine and efavirenz) with CD4
count of 1257 cells/µL and HIV-RNA viral load of less
than 20 copies/mL. She complained of fatigue, diffuse
arthralgias and unintentional weight loss (8 kg in two
months). Physical examination was unremarkable. Her
laboratory studies showed normochromic and normocytic
anemia with a hemoglobin of 10 g/dL (normal range
10.3–15.2 g/dL), elevated serum creatinine at 2.5 mg/dL
(normal range 0.4–1.2 mg/dL), and high globulin gap with
a serum total protein of 9.6 g/dL (normal range 6.0–7.9 g/
dL) and a serum albumin of 3.3 g/dL (normal range 3.5–
5.0 g/dL). An extensive work-up to evaluate the etiology
for renal dysfunction was undertaken. A renal sonogram
revealed normal-sized kidneys. A 24-hour urine collection
showed 2.1 g of protein. Anti-nuclear antibodies and
complement levels were normal and HCV-RNA viral load
was 20 million copies/mL with HCV genotype 1a. Serum
cryoglobulin was negative. Serum and urine protein
electrophoreses showed a monoclonal spike (4.26 g/
dL) and immunofixation electrophoreses demonstrated
monoclonal IgG with elevated free lambda light chains.
Serum free monoclonal lambda light chain was elevated
at 3500 mg/L (normal range 5.7 – 26.3 mg/L). Skeletal
survey was negative. The patient underwent a renal
biopsy which revealed light chain casts in the renal
tubules (Figure 1A–B), confirmed as monoclonal lambda
light chain on immunofluorescence (Figure 2). There was
no evidence of HIV-associated nephropathy (HIVAN). A
bone marrow biopsy showed more than 45% of plasma
cell infiltration (Figure 3). After the diagnosis of IgG
lambda light chain multiple myeloma was made, she was
treated with nine cycles of initial induction chemotherapy
(bortezomib and dexamethasone) followed by autologous
hematologic stem cell transplant per the institutional
treatment protocol. Subsequently, her serum creatinine
improved to 1.1 mg/dL. The patient declined anti-HCV
therapy because of her previous intolerance to interferon
treatment.
Min et al. 490
Figure 1: (A) Distal tubule with a large, acellular hyaline cast
that has a lamellated appearance (H&E stain, x200), (B) Dilated
tubule with degenerated epithelium and a layer of dehisced
cells covering the fragmented cast material (Periodic acid-Schiff
hematoxylin stain, x200).
DISCUSSION
The HIV/AIDS cancer match study, one of the largest
epidemiological studies conducted in the United States
(US), linked 15 population-based HIV/AIDS and cancer
registries in the US. The study analyzed 413,080 HIVinfected persons in 34 US states from 1991 through
2005. During that period, an estimated 79,656 cancers
occurred in the AIDS population. It was observed that
the incidence of the AIDS-defining malignancies declined
markedly over that 14-year period, whereas non-AIDSdefining neoplasms became the predominant type of
cancer in HIV-infected persons during the HAART era.
Multiple myeloma is one of those non-AIDS-defining
Figure 2: The casts stained strongly positive for lambda light
chains. There was no staining for kappa light chains. Fracture
lines perpendicular to the long axis of the cast were evident
(immunofluorescence stain, x200).
IJCRI 2013;4(9):489–493.
Figure 3: A bone marrow aspirate smear showed >45% of
plasma cells, evidenced by eccentrically placed nuclei with a
marked prinuclear hof, or cytoplasmic clearing (red arrows).
cancers whose incidence has increased recently in HIVinfected individuals [1].
The HIV-infected patients can present with a
range of plasma cell disorders, from benign polyclonal
hypergammaglobulinemia, or indeterminate monoclonal
gammopathy of unknown significance (MGUS) to
malignant plasma cell dyscrasias [6]. Multiple myeloma is
usually not recognized as a malignancy that is associated
with HIV infection. There have been only about 50
reported cases of HIV-infected patients with multiple
myeloma in literature since the first case was reported in
1983 [7]. Multiple myeloma has also been reported as the
initial clinical manifestation of HIV/AIDS infection [8].
The pathogenesis of multiple myeloma in HIV-infected
patients is multifactorial (Figure 4). There are at least two
major proposed mechanisms:
(i) a monoclonal paraprotein is specifically directed
against the HIV-1 p24 gag antigen [9] and
(ii) continued stimulation by HIV viral antigens
alters T cell regulation of B cells which are transformed
to malignant plasma cells [10].
Another postulated factor is interleukin-6 (IL-6),
secreted from bone marrow stromal cells, which
stimulates the growth of plasma cells as a paracrine
mechanism. Human Herpes Virus-8 (HHV-8) has the
ability to produce viral IL-6 (vIL-6), a human homolog
of growth factor for plasma cells, which may perpetuate
the growth of neoplastic plasma cells in HIV-infected
individuals who are co-infected with HHV-8 [11].
The association of HCV infection with multiple
myeloma is more controversial. Among the
hematological disorders, monoclonal gammopathies
rarely occur in patients with chronic HCV infection. It is
speculated that HCV is lymphotropic and the mechanism
which contributes to the pathogenesis of B cell nonHodgkin lymphomas (NHL) may also play a role in
Min et al. 491
the development of multiple myeloma in HCV-infected
populations (Figure 4) [12]. Thus, it is a relatively
weak association between HCV infection and multiple
myeloma. It is also of interest to note that monoclonal
gammopathy, if present, is more prevalent in patients
with HCV genotypes 2a and 2c [13].
To the best of our knowledge, this is the first reported
case of multiple myeloma in a patient with HIV-HCV coinfection. There are several unique characteristics in our
patient. First of all, she is older than the average age (33
years) of HIV-positive patients with multiple myeloma.
Secondly, the clinical course of multiple myeloma in our
patient was relatively less aggressive than the course
usually noted in HIV-infected patients. Thirdly, her HIV
infection was well-controlled, and there was no parallel
progression of multiple myeloma with HIV infection to
AIDS. Lastly, her HCV genotype was 1a, which is not one
of the most commonly observed genotypes (2a/2c) in
HCV-infected patients with multiple myeloma.
Figure 4: A schematic diagram of proposed major pathogenetic
mechanisms of multiple myeloma in patients with HIV-HCV
co-infection (see text for details). HHV-8 – Human Herpes
Virus-8.
CONCLUSION
In conclusion, chronic infection with human
immunodeficiency virus (HIV) and/or hepatitis C virus
(HCV) appears to increase the risk of developing multiple
myeloma, although their oncogenic role has been less
established. Our case highlights that dual HIV and HCV
infections may possibly have an additive effect that drives
this oncogenic process leading to multiple myeloma.
Further research is warranted to establish a pathogenic
causal relationship. Nonetheless, providers should keep
in mind that multiple myeloma may occur in HIV-monoinfected or HIV-HCV co-infected patients, especially
when serum gammopathy is present.
IJCRI 2013;4(9):489–493.
Min et al. *********
Zaw Min – Conception and design, Acquisition of data,
Analysis and interpretation of data, Drafting the article,
Zipporah Krishnasami – Conception and design,
Drafting the article, Critical revision of the article, Final
William J Cook – Conception and design, Acquisition of
data, Analysis and interpretation of data, Drafting the
Martin Rodriguez – Conception and design, Acquisition
of data, Analysis and interpretation of data, Drafting the
5.
6.
7.
8.
9.
Guarantor
10.
11.
Copyright
© Zaw Min et al. 2013; This article is distributed under
the terms of Creative Commons attribution 3.0 License
which permits unrestricted use, distribution and
12.
13.
492
with hepatitis C virus infection. Haematologica 1998
Oct;83(10):946-8.
Domingo JM, Romero S, Moreno JA, Domingo JA,
Callén L, Gutiérrez M. Hepatitis C virus infection
and mixed cryoglobulinemia in patients with
lymphoproliferative diseases. Haematologica 1999
Jan;84(1):94-6.
Dezube BJ, Aboulafia DM, Pantanowitz L. Plasma
cell disorders in HIV-infected patients: From benign
gammopathy to multiple myeloma. AIDS Read 2004
Jul;14(7):372-4, 377-9.
Israel AM, Koziner B, Straus DJ. Plasmacytoma
and the acquired immunodeficiency syndrome. Ann
Intern Med 1983 Nov;99(5):635-6.
Pouli A, Lemessiou H, Rontogianni D, et al. Multiple
myeloma as the first manifestation of acquired
immunodeficiency syndrome: A case report
and review of the literature. Ann Hematol 2001
Sep;80(9):557-60.
Konrad RJ, Kricka LJ, Goodman DB, Goldman J,
Silberstein LE. Brief report: Myeloma-associated
paraprotein directed against the HIV-1 p24 antigen in
an HIV-1-seropositive patient. N Engl J Med 1993 Jun
24;328(25):1817-9.
Fiorino AS, Atac B. Paraproteinemia, plasmacytoma,
myeloma and HIV infection. Leukemia 1997
Dec;11(12):2150-6.
Rettig MB, Ma HJ, Vescio RA, et al. Kaposi’s sarcomaassociated herpesvirus infection of bone marrow
dendritic cells from multiple myeloma patients.
Science 1997 Jun 20;276(5320):1851-4.
Lakatos PL, Fekete S, Horanyi M, Fischer S, Abonyi
ME. Development of multiple myeloma in a patient
with chronic hepatitis C: A case report and review
of the literature. World J Gastroenterol 2006 Apr
14;12(14):2297-300.
Andreone P, Zignego AL, Cursaro C, et al. Prevalence
of monoclonal gammopathies in patients with
hepatitis C virus infection. Ann Intern Med 1998 Aug
15;129(4):294-8.
ACKNOWLEDGEMENTS
We would like to extend our thanks to Dr Victoria
Johnson and Dr Edward W. Hook, III, for their support
and suggestions on the submitted manuscript.
REFERENCES
1.
Shiels MS, Pfeiffer RM, Gail MH, et al. Cancer burden
in the HIV-infected population in the United States. J
Natl Cancer Inst 2011 May 4;103(9):753-62.
2. Cooley TP. Non-AIDS-defining cancer in HIVinfected people. Hematol Oncol Clin North Am 2003
Jun;17(3):889-9.
3. Lewden C, Salmon D, Morlat P, et al. Causes of
death among human immunodeficiency virus (HIV)infected adults in the era of potent antiretroviral
therapy: Emerging role of hepatitis and cancers,
persistent role of AIDS. Int J Epidemiol 2005
Feb;34(1):121-30.
4. Pérez Sánchez I, Rivera Redondo J, García Monforte
A, et al. B-lymphoproliferative disorders in patients
IJCRI 2013;4(9):489–493.
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IJCRI 2013;4(9):494–497.
Lalooha et al. CASE REPORT
494
OPEN ACCESS
Advanced secondary abdominal pregnancy: A complication
of induced abortion
Fateme Lalooha, Khadijeh Elmizadeh, Fateme Salehi
Abstract
Introduction: Secondary abdominal pregnancies
usually develop as a result of a tubal abortion,
tubal rupture or uterine rupture with intraabdominal implantation. Case Report: We
report a 40-year-old patient gravida IV, para
III suffering from abdominal pain. She was in
the 29th week of gestation according to a 14thweek normal sonogram. She had unsuccessfully
attempted to induce abortion with the assistance
of a home midwife in the 14th week. This caused
severe abdominal pain, but she chose not to be
hospitalized because of fear of prosecution.
Ultrasound demonstrated that the fetus was out
of the uterus and revealed a heterogeneous mass
in the left lower quadrant. Laparotomy revealed
an area of thin fibrotic scar behind the uterus
with normal tubes and ovaries. The fetus in the
amniotic sac was surrounded by intestinal loops.
The placenta, implanted behind the left broad
ligament at the edge of the scar area was removed
with a brisk bleeding, which was controlled. No
postoperative complications were observed.
Conclusion: Presence of little free fluid and a
uterine scar with normal fallopian tubes against
a history of an unsuccessful induced abortion and
severe illness are highly suggestive of a uterine
rupture resulting in secondary implantation on
Fateme Lalooha1, Khadijeh Elmizadeh1, Fateme Salehi2
Affiliations: 1Obstetrician and Gynecologist, Department
of Obstetrics & Gynecology, Qazvin University of Medical
Science, Qazvin, Iran; 2Resident of Obstetrics and
Gynecology, Department of Obstetrics & Gynecology,
Qazvin University of Medical Science, Qazvin, Iran.
Corresponding Author: Khadijeh Elmizadeh, Qazvin, Iran 3415646139; Ph: 00989121813620; Fax: 00982812236378;
Email ID: [email protected]
Received: 04 September 2012
the broad ligament. This is probably the first
case that shows an abdominal pregnancy can
result from unsuccessful induced abortion. In
the countries where abortion is not permitted,
patients with low socioeconomic status are prone
to the dangerous consequences of illegal attempts
to induce abortion. Close observation and special
follow-up care are necessary in such cases.
Keywords:
Induced
Abortion,
Secondary Abdominal Pregnancy
Pregnancy,
*********
Lalooha F, Elmizadeh K, Salehi F. Advanced secondary
abdominal pregnancy: A complication of induced
abortion. International Journal of Case Reports and
Images 2013;4(9):494–497.
*********
doi:10.5348/ijcri-2013-09-364-CR-8
Introduction
Abdominal pregnancy is a rare event and is classified
as either primary or secondary, with the latter being more
common. Secondary abdominal pregnancies usually
develop as a result of a tubal abortion, tubal rupture, or
uterine rupture with intra-abdominal implantation [1].
CASE REPORT
A 40-year-old gravida IV, para III woman was referred
to us in the 29th week of gestation with a complaint of
a two-week-long abdominal pain and weakness. The
accompanying symptoms were vertigo and loss of
appetite. There were signs of anemia. Her vital signs
were stable. She had lost 3 kg in two weeks. Pregnancy
had been confirmed three months earlier through serum
IJCRI 2013;4(9):494–497.
hCG measurement followed by ultrasound examination
showing a normal 14-week pregnancy with antero fundal
placenta. She reported no remarkable medical and
surgical history and claimed that her early pregnancy was
uneventful.
Physical examination upon admission revealed
an abdominal mass rising from the pelvis without
tenderness, rebound, and guarding. Laboratory results
showed hemoglobin level 7.7 g/dL and a hematocrit
value 25.7. Other examinations, including BUN, Cr,
serum electrolytes, coagulation, and liver function tests,
showed were normal. Gestational age was 29 weeks
+4 days according to 14 weeks sonogram. However,
ultrasonographic scan confirmed a breech-lying 26
weeks +2 days normal fetus out of the uterus with
oligohydramnios and a vague heterogeneous 157×79 mm
mass in the left lower quadrant with some free fluid in the
abdominal cavity. The uterus was empty and normally
placed. The MRI scan confirmed the diagnosis (Figure 1).
After the diagnosis was explained to the patient and
her family, they revealed a history of an unsuccessful
attempt to induce abortion with the help of a home
midwife. This attempt caused severe illness, paleness and
crampy abdominal pain, which wore off after two weeks.
Despite severe illness, she had not visited any physician
because of fear of prosecution.
The patient was given four units of packed cell which
increased her hemoglobin to 11.7 g/dL. She underwent
bowel preparation prior to the surgery. Accessibility
of sufficient blood products was confirmed. A team
consisting of two obstetricians/gynecologists, a general
surgeon, a vascular surgeon and an anesthesiologist
prepared for laparotomy under general anesthesia.
The parietal peritoneum was opened through a midline
Figure 1: Magnetic resonance imaging showing uterus, fetus,
and placenta.
Lalooha et al. 495
incision. The amniotic sac was surrounded by intestinal
loops. The baby was delivered. She had no dysmorphic
features and weighed 800 g with an Apgar score of
4–0 at 1–20 minutes. The amniotic membranes were
loosely clinging to the loops of jejunum and ileum and
were easily dissected away. The uterus, fallopian tubes,
and ovaries were normal. The placenta was implanted
behind the uterus, on the left broad ligament and the culde-sac. There was a thin fibrotic scar measuring 3×2 cm
behind the uterus near the edge of the placenta and was
only covered by uterine serosa. Attempting to remove the
placenta resulted in a brisk bleeding, but it was controlled
by packing and suturing. The fallopian tubes were ligated,
and the uterus was repaired. The patient was transfused
with two units of packed cell during operation. No
complications were observed after the surgery. The
patient was discharged after six days in good health.
DISCUSSION
This case revealed the presence of little free fluid and
a uterine scar with normal fallopian tubes. These findings
when considered against backdrop of an unsuccessful
induced abortion and severe illness are highly suggestive
of a uterine rupture resulting in secondary implantation
on the broad ligament.
In a review of available literature, we found only a few
cases of secondary abdominal pregnancy resulting from
uterine rupture. Teng et al. (2007) reported a secondary
abdominal pregnancy following rupture of a uterine
scar after two cesarean sections [2]. They claimed that
their report was the first case of an early scar rupture
resulting in a viable intra-abdominal pregnancy. Amritha
et al. reported a viable secondary abdominal pregnancy
following rupture of a rudimentary horn [3]. We can
assume that our case is the first report of secondary
abdominal pregnancy as a complication of an induced
abortion.
Abdominal pregnancy causes relatively few
symptoms, none of which are individually diagnostic [4].
Once she recovered from the acute symptoms of uterine
damage, our patient was relatively asymptomatic in the
rest of her pregnancy. Thus, a high index of suspicion
is warranted for diagnosing abdominal pregnancy [3].
Ultrasonography is the main method for diagnosis
[5, 6], but it fails to diagnose in half of the cases [4,
5]. The 29th-week sonogram of our patient revealed
an abdominal pregnancy with the following findings:
separation of the uterus from the fetus, extrauterine
placenta, oligohydramnios, abnormal lie of the fetus,
some free peritoneal fluid and poor visualization of the
placenta.
An MRI scan confirmed all the sonographic findings.
Thus, it can be a useful adjunct to the ultrasound and may
help identifying the spread of placental implantation. It
seems wise to administer both tests to obtain maximal
information for surgical planning [3, 4].
IJCRI 2013;4(9):494–497.
We chose to terminate the pregnancy upon diagnosis
because a review of previous case reports showed that
employing expectant management in the hope of fetal
maturity involves a substantial risk of life-threatening
hemorrhage [4, 5].
Fortunately, it was possible to separate the whole
placenta intra-operatively. Placental management is a
highly controversial issue. Retention of the placenta poses
substantial risks. Most authors agree that the placenta
should be removed provided its blood supply is identified
and can be ligated without damaging other organs [3, 5].
Completely removal of the placenta made
postoperative methotrexate administration unnecessary.
Indeed, postoperative use of methotrexate is controversial
in the case of the placenta left in place [4].
Lalooha et al. Conflict of Interest
Copyright
© Fatemeh Lalooha et al. 2013; This article is distributed
REFERENCES
1.
CONCLUSION
Abdominal pregnancy is a rare condition and diagnosis
requires a high index of suspicion. In the countries where
abortion is illegal, patients with a low socioeconomic
status are prone to the dangerous consequences of secret
attempts to induce abortion. So it is imperative that
every primary health care provider closely observe the
patients with unwanted pregnancy and provide special
follow-up care. Prompt treatment upon discovery of the
condition is essential. In managing abdominal pregnancy,
maternal morbidity and mortality can be significantly
reduced through preoperative diagnosis and evaluation,
sufficient blood supply, bowel preparation, availability of
a multidisciplinary surgical team and proper operative
techniques.
ACKNOWLEDGEMENTS
496
Mahomed K. Abdominal Pain. In: James DK, Steer
PJ, Weiner CP, Gonik B, Crowther CA& Robson SC,
editors. High Risk Pregnancy: Management Options.
Philadelphia, Pennsylvania: Saunders 2010:1013–26.
2. Teng HC, Kumar G, Ramli NM. A viable secondary
intra-abdominal
pregnancy
resulting
from
rupture of uterine scar: role of MRI. Br J Radiol
2007;80(955):e134–6.
3. Amritha B, Sumangali T, Priya B, Deepak S, Sharadha
R. A rare case of term viable secondary abdominal
pregnancy following rupture of a rudimentary horn:
a case report. J Med Case Reports 2009;3:38–40.
4.Worley KC, Hnat MD, Cunningham FG.
Advanced extrauterine pregnancy: diagnostic and
therapeutic challenges. Am J Obstet Gynecol 2008
Mar;198(3):297.e1–7.
5. Dahab AA, Aburass R, Shawkat W, Babgi R, Essa
O, Mujallid RH. Full-term extrauterine abdominal
pregnancy: a case report. J Med Case Reports
2011;5:531–4.
6. Yildizhan R, Kolusari A, Adali F, et al. Primary
abdominal ectopic pregnancy: a case report. Cases J
2009;2:8485–9.
Thanks Mr Mahmood Soleimani for his helpful
commends in writing the case report.
*********
Fateme Lalooha – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
Khadijeh Elmizadeh – Acquisition of data, Drafting the
article, Revising it critically for important intellectual
content, Final approval of the version to be published
Fateme Salehi – Acquisition of data, Drafting the article,
Guarantor
IJCRI 2013;4(9):494–497.
other devices
Lalooha et al. Access PDF of article on
other devices
497
IJCRI 2013;4(9):498–501.
Datta et al. CASE REPORT
498
OPEN ACCESS
Primary cerebellar B cell lymphoma: A case report
Anupam Datta, Arunima Gupta, Krishnangshu Bhanja Choudhury,
Aruj Dhyani, Anup Majumdar
Abstract
Introduction: Primary central nervous system
lymphoma (PCNSL) is usually a diffuse large B
cell non-Hodgkin lymphoma that originates in
the brain, spinal cord, leptomeninges or eyes.
Primary central nervous system lymphoma is
a rare malignant tumor comprising less than
3% of all primary brain tumors. The PCNSL in
immunocompetent patients is most commonly
supratentorial, found adjacent to the ventricular
surfaces and in deep white matter and subcortical
structures, such as the basal ganglia, thalamus
and corpus callosum. The lesion is single in
60–70% of patients. Lesions are located in the
hemispheres (38%), thalamus/basal ganglia
(16%), corpus callosum (14%), periventricular
region (12%) and cerebellum (9%). Herein, we
report an extremely rare case of a primary CNS
B cell lymphoma involving cerebellum. Case
Report: A 55-year-old female was presented
with vomiting, ataxia and disorientation. T2Anupam Datta1, Arunima Gupta1, Krishnangshu Bhanja
Choudhury2, Aruj Dhyani1, Anup Majumdar3
Affiliations: 1Post Graduate trainee, Radiotherapy, Junior
Resident, Department of Radiotherapy, Institute of Post
graduate Medical Education and Research, Kolkata-700020,
West Bengal, India; 2MD, Radiotherapy. RMO-cum-Clinical
tutor, Department of Radiotherapy, R.G.Kar Medical College
and Hospital, Kolkata -700004, West Bengal, India; 3MD,
Radiotherapy, Professor and Head, Department of Radiotherapy, Institute of Post graduate Medical Education and
Research, Kolkata-700020, West Bengal, India.
Corresponding Author: Krishnangshu Bhanja Choudhury,
Designation: RMO-cum-Clinical tutor, Department of Radiotherapy, R.G.Kar Medical College and Hospital, Kolkata -700004, West Bengal, India; Address: C-11/4, Green
Tower, Golf Green, Kolkata -700095, West Bengal; Ph: +919830932869; E-mail: [email protected]
Received: 30 July 2012
Accepted: 06 March 2013
weighted axial, T1-weighted and gadoliniumenhanced T1-weighted axial magnetic resonance
imaging scans show a contrast enhancing mass
with peritumoral edema in the right cerebellar
hemisphere. Excision biopsy was suggestive
of non-Hodgkin lymphoma. Diagnosis was
confirmed with immunohistochemistry favoring
non-Hodgkin lymphoma of diffuse large B cell
type. This patient received cytotoxic therapy with
carmustine 200 mg IV 6 weekly 3 cycles followed
by external beam radiation therapy 30 Gy. No
recurrence was noticed in one year follow-up.
Conclusion: Primary B cell lymphoma limited to
the CNS is exceedingly rare in the middle aged
immunocompetent individual. Little is known
regarding etiologic factors, optimal management
and prognosis.
Keywords: Non-Hodgkin lymphoma, Carmustine,
B-cell lymphoma, Cerebellum
*********
Datta A, Gupta A, Choudhury KB, Dhyani A, Majumdar
A. Primary cerebellar B cell lymphoma: A case report.
2013;4(9):498–501.
*********
doi:10.5348/ijcri-2013-09-365-CR-9
Introduction
Primary central nervous system lymphoma (PCNSL)
is usually a diffuse large B cell non-Hodgkin lymphoma
that originates in the brain, spinal cord, leptomeninges or
eyes. The PCNSL is a rare malignant tumor representing
3% of intracranial neoplasms and 4–6% of extra nodal
lymphomas with a yearly incidence of 0.5 case per 100,000
people and with a median survival, if untreated, of 1.5–3.3
IJCRI 2013;4(9):498–501.
months. It is rare in immunocompetent patients. Median
age at diagnosis is 60–65 years. The lesion is single in 60–
70% of patients. Lesions are located in the hemispheres
(38%), thalamus/basal ganglia (16%), corpus callosum
(14%), periventricular region (12%) and cerebellum (9%).
We report unusual primary cerebellar B cell lymphoma in
a middle aged immunocompetent female patient.
Datta et al. 499
showed normal glucose and protein levels, and 2 cells/
mm3, all of which were lymphocytes. She was diagnosed
as primary CNS non-Hodgkin lymphoma and went on to
receive cytotoxic therapy with carmustine 200 mg IV 6
weekly three cycles followed by external beem radiation
therapy (EBRT) 30 Gy. No recurrence was noticed in
one year follow-up. Post treatment CT scan of the brain
revealed no abnormality (Figure 3).
CASE REPORT
A 55-year-old female was admitted in emergency
department with symptoms of ataxia and disorientation
of 1 week duration. She was suffering from headache and
vomiting for last four months. The headache was diffuse,
constant, throbbing and associated with episodic nausea
and vomiting. Concurrently, she had a weight loss of 11 kg,
daily fever and chills over the same duration. Clinical
examination revealed slurred speech, finger to nose
ataxia worse on the right side and truncal ataxia, with
no other significant neurological deficits. A computed
tomography (CT) scan of the brain revealed a solitary,
well circumscribed, cystic, ring enhancing mass in the
right cerebellar hemisphere with surrounding edema. T2weighted axial, T1-weighted and gadoliniumenhanced
T1- weighted axial magnetic resonance imaging (MRI)
scans of brain showed a contrast enhancing mass with
peritumoral edema in the right cerebellar hemisphere
(Figure 1). Complete hemogram, liver function and
renal function tests along with serum electrolytes were
normal. Skiagram of chest and ultrasonography of
abdomen were normal. She was seronegative for HIV 1
and 2, HBsAg and anti-HCV antibodies. Intravenous
dexamethasone was initiated and a subtotal resection
of the cerebellar lesion was performed. Hematoxylin
and eosin preparation revealed a malignant round cell
tumor probably non-Hodgkin lymphoma (Figure 2A–C).
Diagnosis was confirmed with immunohistochemistry
positivity for CD20 and CD10 (dim) and tumor stained
immunonegative for CD3, CD5 and CD23. The Mib-1
labeling index was approximately 60–70%. Therefore,
giving the impression of non-Hodgkin lymphoma (NHL)
of diffuse large B cell type. With this biopsy report further
investigations of CT scan of abdomen and bone marrow
examination were done; all studies were normal. After
three weeks of surgery cerebrospinal fluid (CSF) analysis
Figure 1: Magnetic resonance imaging scan (A) T2-weighted
axial, (B) T1-weighted (C) and Gadolinium-enhanced T1weighted axial. Magnetic resonance imaging scan showing a
contrast enhancing mass with peritumoral edema in the right
cerebellar hemisphere.
Figure 2: (A , B, C) Hematoxylin-eosin stained biopsy sections
showing normal cerebellar tissue and a tumor composed of
sheets of round or oval cells with hyperchromatic nuclei. Mitotic
figures seen, 40x and 100x.
Figure 3: Follow-up magnetic resonance imaging scan after
treatment. (A) T2-weighted axial, (B) T1-weighted axial and (C)
Gadolinium-enhanced T1-weighted axial. There is no evidence
of residual or local recurrence.
DISCUSSION
The PCNSL is defined as lymphoma limited to the
cranial-spinal axis without systemic disease [1]. In the
past, PCNSL was considered a rare disorder, accounting
for 1–2% of all cases of non-Hodgkin lymphoma and fewer
than 5% of all cases of primary intracranial neoplasm [2],
just 13% of these arise in the posterior fossa. The PCNSL
now represents around 3% of intracranial neoplasms and
4–6% of extranodal lymphomas with a yearly incidence
of 0.5 case per 100,000 people.
An increasing incidence of this disease has been
seen among patients with acquired immunodeficiency
syndrome (AIDS) and among other immunocompromised
persons. The incidence of PCNSL in the immunocompetent
population has been reported to have increased more
than 10-fold from 2.5 cases to 30 cases per 10 million
populations [3]. The cause for the increase in incidence
of this disease in the immunocompetent population is
unknown. Two theories have been proposed. Lymphoma
develops within a focus of inflammation in the CNS,
as may occur in other organ systems such as the
gastrointestinal tract. Another possible explanation is
IJCRI 2013;4(9):498–501.
that the cancerous lymphocytes develop elsewhere in the
body, but acquire a receptor on their surface that draws
them to a signal expressed only inside the CNS. The CNS
is an immunologic sanctuary site rendering the neoplastic
lymphocytes which may be systemically eradicated by an
intact immune system, find relative protection within the
central nervous system. In patients with AIDS, infection
with the Epstein–Barr virus, which also causes infectious
mononucleosis, is probably an important trigger for
PCNSL, although the reason for this is not understood.
The natural history of this disorder differs between
patients with AIDS and those without AIDS. The CT scans
may show ring enhancement in 50% of AIDS patients
while patients without AIDS almost always show only
homogeneous enhancement [1]. Both groups do equally
poorly without therapy (1–3 months mean survival), but
the overall survival for treated patients is much better for
patients without AIDS (18.9 months) than for those with
AIDS (2.6 months) [1, 4].
Although more than 95% of patients with PCNSL are
lymphoma of B cell origin, 45 patients with CNS lymphoma
of T cell origin showed no difference in presentation or
outcome in a retrospective series with data collected from
12 cancer centers [5]. Almost all are aggressive neoplasms
of the diffuse large B-cell type. In a retrospective case
series derived from 18 cancer centers in five countries
of 40 patients with low-grade primary CNS lymphoma,
a better long-term outcome was shown (7-year median
survival) than is associated with the usual aggressive CNS
lymphoma [6]. Anecdotal cases of primary CNS Hodgkin
lymphoma have also been reported [7].
The PCNSL in immunocompetent patients is most
commonly found adjacent to the ventricular surfaces and
in deep white matter and subcortical structures, such as
the basal ganglia, thalamus and corpus callosum. Most
of these tumors are supratentorial. In one series, more
than 70% of tumors were in a cerebral hemisphere and
periventricular location (usually involving the corpus
callosum or basal ganglia). Approximately, 50–70%
of patients with this disease presented with solitary
lesions, whereas the remainder had multifocal disease.
These lesions characteristically enhance homogeneously
with the administration of contrast agents [8]. The
incidence of cerebellar involvement in one study in
2005 (16 of 170 lesions) was similar to the findings
of Lanfermann et al. (15 of 174 lesions) [9, 10]. The
exact incidence of cerebellar PCNSL in a middle aged
individuals remains unclear inspite extensive literature
search. The differential diagnosis of PCNSL includes
gliomas, metastatic tumors, demyelinating disorders,
subacute infarcts, and space-occupying lesions due to
an infectious etiology.
The optimal treatment for includes radiation, high dose
methotrexate, or more complicated regimen of de Angelis.
Surgery other biopsy provides no therapeutic benefit
because of the depth and diffuse nature of the tumor and
should be reserved for the rare patient with neurological
deterioration due to brain herniation. Antimetabolites
Datta et al. 500
such as methotrexate (MTX) and cytarabine (ara-C)
remain most common chemotherapeutic agents used in
PCNSL regimens. The MTX doses in excess of 1 gm/m2
result in tumoricidal levels in the brain parenchyma and
doses 3 g/m2 yield tumoricidal levels in the CSF. However,
MTX administration requires intensive monitoring with
serum MTX level measurements. Other drugs used are
carmustine, temozolomide, procarbazine and vincristine.
Whole-brain radiation therapy alone is insufficient for
durable tumor control and is associated with a high-risk of
neurotoxicity in patients over 60 years of age. The initial
response to radiation therapy in immunocompetent
patients is excellent, often resulting in complete
resolution of radiographic abnormalities. Nevertheless,
the duration of response is short and median survival
duration with radiation therapy alone averages only 18
months. Relapse in patients with parenchymal disease is
usually within the brain, though leptomeningeal, vitreous
and, rarely, systemic recurrences are reported.
The prognosis of nonselected patients with PCNSL
is comparable to that of glioblastoma multiforme
patients, with a 5-year survival rate after conventional
chemotherapy and radiotherapy (RT) usually at less than
10% [11]. Unfavorable prognostic factors for survival
are more than 60 years of age, WHO performance
status greater than 2, poor neurologic function, elevated
serum lactate dehydrogenase, extensive tumor spread,
CSF protein level greater than 0.6 g/L, corticosteroid
dependence, and absence of chemotherapy administration
[12–14].
CONCLUSION
Primary B cell lymphoma is limited to the central
nervous system, more so to cerebellum. It is rare in
middle aged, immunocompetent individual. Little is
known regarding etiologic factors, optimal management,
and prognosis.
*********
Anupam Datta –Conception and design, Drafting the
Arunima Gupta – Conception and design, Acquisition of
data, Analysis and interpretation of data, Critical revision
of the article, Final approval of the version to be published
Krishnangshu Bhanja Choudhury – Conception and
design, Acquisition of data, Analysis and interpretation
of data, Drafting the article, Critical revision of the article,
Aruj Dhyani – Conception and design, Drafting the
Anup Majumdar – Conception and design, Drafting the
IJCRI 2013;4(9):498–501.
Datta et al. 6.
Guarantor
Copyright
7.
8.
9.
© Anupam Datta et al. 2013; This article is distributed
REFERENCES
Fine HA, Mayer RJ. Primary central nervous system
lymphoma. Ann Intern Med 1993;119(11):1093–104.
2. Jellinger K, Radaskiewicz TH, Slowik F. Primary
malignant lymphomas of the central nervous system
in man. Acta Neuropathol Suppl 1975;Suppl 6:95–
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3. Corn BW, Marcus SM, Topham A, Hauck W, Curran
WJ Jr. Will primary central nervous system lymphoma
be the most frequent brain tumor diagnosed in the
year 2000? Cancer 1997;79(12):2409–13.
4. Nasir S, DeAngelis LM. Update on the management of
primary CNS lymphoma. Oncology 2000;14(2):228–
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5. Shenkier TN, Blay JY, O’Neill BP, et al. Primary CNS
lymphoma of T-cell origin: a descriptive analysis
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collaborative group. J Clin Oncol 2005;23(10):2233–9.
Jahnke K, Korfel A, O’Neill BP, et al. International
study on low-grade primary central nervous system
lymphoma. Ann Neurol 2006;59(5):755–62.
Gerstner ER, Abrey LE, Schiff D, et al. CNS Hodgkin
lymphoma. Blood 2008;112(5):1658–61.
Moradi A, Tajedini A, Mehrabian A, et al.
Clinicopathogical features of primary central nervous
system lymphoma. Neurosciences 2006;11(4):284–8.
Lanfermann H, Heindel W, Schaper J, et al. CT and
MR imaging in primary cerebral non-Hodgkin’s
lymphoma. Acta Radiol 1997;38(2):259–67.
Küker W, Nägele T, Korfel A, et al. Primary central
nervous system lymphomas (PCNSL): MRI features
at presentation in 100 patients. Journal of NeuroOncology 2005;72(2):169–77.
Blay JY, Lasset C, Carrie C, et al. Multivariate analysis
of prognostic factors with non HIV-related Primary
cerebral lymphoma: A proposal for a prognostic
scoring. Br J Cancer 1993;67(5):1136–41.
Michalski JM, Garcia DM, Kase E, Grigsby PW,
Simpson JR. Primary central nervous system
lymphoma: Analysis of Prognostic variables
and patterns of treatment failure. Radiology
1990;176(3):855–60.
Blay JY, Conroy T, Chevreau C, et al. High-dose
methotrexate for the treatment of primary central
nervous system lymphomas: Analysis of survival and
late neurologic toxicity in a retrospective series. J Clin
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Nelson DF, Martz KL, Bonner H, et al. Non-Hodgkin’s
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other devices
IJCRI 2013;4(9):502–506.
Rajeev et al. CASE REPORT
502
OPEN ACCESS
Recurrent intraosseous ganglia in the proximal tibia
following anterior cruciate ligament reconstruction using
patella tendon bone autograft with a titanium interference
screw: A case report
Aysha Rajeev, James Henry, Nanjappa Kashyap
Abstract
Introduction: Intraosseous ganglia are benign
cystic lesions and often multiloculated lesions
located in the subchondral bone. These lesions
are often asymptomatic but in cases located
close to neurovascular structures or articular
surfaces, they can be symptomatic, causing
pain, neurologic dysfunction or articular
fractures. We report a rare case of recurrent
intraosseous ganglion following an anterior
cruciate ligament (ACL) reconstruction in the
proximal tibia. The ganglion developed after
ten years of ACL reconstruction using patella
tendon bone autograft fixed with a titanium
interference screw. Initially the swelling
mimicked the clinical and radiological features
of a bone tumor. Case Report: A 40-year-old
Caucasian male underwent ACL reconstruction
using patella tendon bone graft and a titanium
interference screw fixation in the tibia. After ten
years of surgery he noticed a lump in the proximal
tibia at the site of tibial screw. The radiological
features were unremarkable. He had excision
of the swelling along with screw removal. The
swelling recurred again within six months. The
lump was excised again and tibial tunnel bone
Aysha Rajeev1, James Henry2, Nanjappa Kashyap3
Affiliations: 1Associate Specialist, Dept. of Orthopaedics
and Trauma, Queen Elizabeth Hospital, Gateshead, uk;
2
Consultant, Dept. of Histo Pathology, Queen Elizabeth
Hospital, Gateshead, uk; 3Consultant, Dept. of Orthopaedics
and Trauma, Queen Elizabeth Hospital, Gateshead,uk.
Corresponding Author: Aysha Rajeev, 25, Fatfield
Park, Washington. Tyne And Wear, UK; Contact No:
00441914190315; Email: [email protected]
Received: 31 October 2012
grafted. After 4 weeks the lump reappeared,
magnetic resonance imaging scan showed a large
cystic lesion in the proximal tibia. After taking
the advice from the oncologist, the lesion was
curetted and bone grafted. The cyst went on to
heal completely in six months time. Conclusion:
The occurrence of intraosseous ganglion with
absorbable interference screw has been reported.
In our case report the intraosseous ganglion
formed after using a titanium interference screw
which has not been reported previously. It is also
unique in the sense that the swelling recurred
thrice and mimicked characteristics of a bone
tumor.
Keywords: Recurrent, Intraosseous, Titanium
interference screw, Mimic bone tumour
*********
Rajeev A, Henry J, Kashyap N. Recurrent intraosseous
ganglia in the proximal tibia following anterior
cruciate ligament reconstruction using patella tendon
bone autograft with a titanium interference screw:
A case report. International Journal of Case Reports and
Images 2013;4(9):502–506.
*********
doi:10.5348/ijcri-2013-09-366-CR-10
Introduction
The occurrence of intraosseous ganglions is not that
common. Ganglions are commonly soft tissue benign
tumors usually arise in close proximity to joints, tendon
or its sheaths [1]. The intraosseous penetration of a
periosteal ganglion-like structures with subsequent
formation of a bone cyst has been described in the past
IJCRI 2013;4(9):502–506.
[2]. Intraosseous ganglia occur subchondrally, with or
without an intact articular cartilage [3]. Sometimes the
lesions occur in the metaphysis with connections to the
joint surface or to the adjacent ligamentous structures [4].
Various theories have been proposed as to the
pathogenesis of these ganglions. These include
degeneration of connective tissue and migration of
synovial fluid from an adjacent joint [1]. The possibility
of acute or chronic trauma as one of the causative factors
in these type of ganglions have also been postulated in
a small percentage of patients [5]. The presence of serosanguinous mucin in the aspirate of these ganglions
support this theory. Trauma sometimes cause to myxoid
or mucinous degeneration which in turn leads to the
pathogenesis of the ganglion [6].
In this case report, the patient developed an intraosseous ganglion almost ten years following an anterior
cruciate ligament (ACL) reconstruction using patella
tendon bone graft. The ganglion was located in the site of
the tibial titanium interference screw. We are reporting
this case as a unique and rare cause of recurrent and
resistant intraosseous ganglion which has never been
cited in English literature.
CASE REPORT
A 40-year-old Caucasian male underwent ACL
reconstruction of the right knee using patella-tendon
bone graft in 1999. He was doing extremely well for 10
years. In 2009 while playing football he had a knock on
the medial aspect of the tibia. He noticed a lump just
below the medial side of the knee which became very
painful. There were no symptoms of instability. He had
pain radiating from the lump on to the front of the upper
leg. He was active and continued with his job.
On examination, there was no deformity of the
knee. There was lump on the medial aspect of the tibial
tuberosity, at the site of the tibial screw insertion for
ACL reconstruction. The swelling was about 4x4 cm,
tender with on redness or signs of inflammation. The
movements were full range and tests for instability were
negative. There was no distal neurovascular deficit.
The radiological examination of the knee showed early
degenerative changes in all three compartments. The
position of the tibial screw was satisfactory with no bony
changes (Figure 1). He was listed for planned procedure
to take the tibial screw out. At the time of surgery the
surgeon noticed a large soft tissue ganglion over the
screw head. This was excised en-bloc and screw removed.
The patient was reviewed back in the clinic two weeks
following the surgery. He had mild ooze from the wound
and swab was send for culture and sensitivity. The patient
was again reviewed back in the clinic after one week
which showed a jelly like material oozing from the wound
but the culture reports were negative. He continued to
receive wound dressings over the next few weeks and the
wound healed satisfactorily, and the lump reappeared.
Rajeev et al. 503
The patient returned six months after the operation
with increased pain and swelling in the medial aspect of
the knee. Physical examination showed a tender lump
about 5x5 cm under the scar tissue. A decision was
taken to excise the lump and bone graft the tibial tunnel.
The scar was opened and the ganglion was excised and
the tibial tunnel was grafted using freeze dried bone.
Postoperatively the patient did well, the wound healed
satisfactorily and the swelling disappeared.
The patient was then re-referred within four weeks by
his general practioner with increasing pain and swelling
at the operative site. On examination, the wound has
healed well and there was only a small tender lump
palpable about 2x2 cm. The X-ray revealed that graft is
incorporating well (Figure 2). In view of the continuing
symptoms he was referred for an MRI scan urgently. The
scan revealed a large cystic lesion in the proximal tibia
(Figure 3). The patient was referred to the regional tumor
unit for expert opinion. The tumor surgeon reported that
the patient had got a recurrent intraosseous ganglion.
A decision was taken to curette and bone graft lesion.
The patient underwent excision of the ganglion and
bone grafting using fresh frozen femoral head and tissue
samples were send for histopathology (Figure 4). The
patient was followed-up second week, sixth week and
sixth month following the operation. The histopathology
showed mature bone with fibro-collagenous tissue
showing myxoid change and chronic inflammation.
The myxoid material extends into bone suggesting an
intraosseous ganglion. The cyst healed (Figure 5) and
patient regained full knee movements. He was discharged
from the outpatient clinic after 1 year.
Figure 1: The radiograph showing tibial screw in-situ with no
bony changes.
IJCRI 2013;4(9):502–506.
Rajeev et al. 504
Figure 4: Histopathology showing bony tissue mixed with
myxoid fibrous tissue consistent with an intraosseous ganglion.
Figure 2: X-ray showing the graph incorporating well after the
first surgery.
Figure 5: Final radiograph showing the cyst well healed after
curettage and bone grafting.
Figure 3: Magnetic resonance imaging scan showing a large
cystic lesion in the proximal tibia.
DISCUSSION
The diagnosis of intraosseous ganglion should be
considered in the absence of previous inflammatory and
degenerative joint lesions. Most of these cases occur
between the ages of 14–73. There are basically two types of
intraosseous ganglia—one is idiopathic and the other one
caused by penetration of an extraosseous ganglion into
the underlying bone [1]. The juxta-articular, intraosseous
ganglion reported in our case belongs to the second type,
in which a soft tissue ganglion developed first and later
penetrated into the underlying proximal tibia.
There are several theories proposed about the
pathogenesis of intraosseous ganglions. The best possible
theory would be a combination of intramedullary
metaplasia followed by fibroblast proliferation. The
fibroblasts secretes both hyaluronic acid and mucin
which tend to accumulate and cause pressure necrosis.
This leads to intramedullary trabecular degeneration and
the formation of intraosseous cyst formation [3].
Most of the intraosseous ganglia lie subchondrally,
with a normal articular cartilage and rarely communicate
with joint or tendon sheath. The intact cyst often appears
relatively smooth, round to oval shaped with an ochreyellow color similar to a soft tissue ganglia, containing a
thick gelatinous material. Microscopically, the walls are
lined by poorly vascularized fibrous tissue and the cavity
with flattened connective tissue cells which sometimes
IJCRI 2013;4(9):502–506.
resemble synovial histiocytes. The secretion is high in
hyaluronic acid and other mucopolysaccharides along
with glucosamine, albumin and globulin.
The radiological features of an intraosseous ganglion
include a scalloped cortical defect with a sclerotic margin.
They appear thick and well defined which indicates a
slow growing lesion [7]. These lesions very rarely shows
calcification [8].
An MRI scan shows features similar to degenerative
cysts with the overlying joint showing a normal
articulation. The intraosseous ganglion may or may not
communicate with the joint. The lesions show a fluid like
appearance with low intensity on T1-weighted images,
moderately low intensity on proton density weighted
images and high homogenous intensity on T2-images.
The differential diagnosis of intraosseous ganglion
is mainly giant cell tumor and chondroblastoma. Giant
cell tumors are locally aggressive composed of giant cells,
connective tissue and stromal cells. They usually occur
between 3rd and 4th decade and predominantly epiphyseal
location after growth plate closure. Chondroblastoma are
commonly seen before skeletal maturity. Radiologically,
they tend to be well defined lucent lesions with a sclerotic
margins with calcification seen in 50% of cases.
The formation of cyst in the tibial tunnel after the use
of an absorbable interference screw have been described
[9]. Majority of the interference screws being used now-adays are made of biodegradable material. The polylactide
material causes hydrolysis and phaocytois [10]. This
causes inflammatory foreign-body reactions responsible
for as osteolytic reaction around the screw. Malhan et al.
postulated that the gradual accumulation of breakdown
products in a closed tibial tunnel ultimately lead to a cyst
formation [11]. In our case report the interference screw
was made of titanium.
The occurrence of pretibial cyst formation in the
proximal tibia after ACL reconstruction has been
reported with patella tendon autograft with the use of
bioabsorbable interference screws [12, 13]. The actual
mechanism by which these cyst forms has not been
established. A mismatch of graft tunnel diameter,
eccentric tibial tunnel, bone necrosis may slow down the
incorporation of the graft into the tibia. This may lead to
a water-channel between the knee joint and the proximal
tibia there by leading to the formation of a cyst [14].
There has been no reports of any cyst formation using a
titanium interference screw.
CONCLUSION
This is a rare case of intraosseous ganglion following
anterior cruciate ligament reconstruction. The intraarticular communication after anterior cruciate ligament
reconstruction of proximal tibia thereby causing the
synovial fluid to flow to the proximal tibia under pressure
may have attributed to the development of intraosseous
ganglion. The clinician should be aware that it can mimic
Rajeev et al. 505
a bone tumor. The pathologic lesion can be successfully
treated with curettage and bone grafting.
*********
Aysha Rajeev – Substantial contributions to conception
and design, Acquisition of data, Analysis and
interpretation of data, Drafting the article, Revising
James Henry – Acquisition of data, Analysis and
Nanjappa Kashyap – Acquisition of data, Analysis and
Guarantor
Copyright
© Aysha Rajeev et al. 2013; This article is distributed
REFERENCES
1.
Schajowicz F, Clavel Sainz M, Slullitel JA. Juxtaarticular bone cysts (intra-osseous ganglia): a
clinicopathological study of eighty-eight cases. J Bone
Joint Surg Br 1979;61(1):107–6.
2. Fisk GR. Bone concavity caused by a ganglion. J Bone
Joint Surg Br 1949 May;31B(2):220.
3. Feldman F, Johnston A. Intraosseous ganglion. Am J
Roentgenol Radium 1973;118(2):328–43.
4. Hicks JD. Synovial cysts in bone. Australian & New
Zealand J Surg 1956;26(2):138–43.
5. DeOrsay RH, Mecray PM, Ferguson LK. Pathology
and treatment of Ganglions. Am J Surg 1937;36:313–
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1928;47:460.
7. Valls R, Melloni P, Darnell A, Munoz J, Canalties J.
Diagnostic imaging of periosteal ganglion. Eur Radiol
1997;7(1):70–2.
8. Resnick D, Niwayama G. Diagnosis of bone and
joint disorders. 2nd ed. Philadelphia: WB Saunders;
1988:1379–82.
IJCRI 2013;4(9):502–506.
9. Thaunat M, Chambat P. Pretibial ganglion-like
cyst formation after anterior cruciate ligament
reconstruction:a consequence of the incomplete bony
integration of the graft? Knee Surg Sports Traumatol
Arthrosc 2007;15(5):522–4.
10. Clatworthy MG, Annear P, Bulow JU, Bartlett RJ.
Tunnel widening in anterior cruciate ligament
reconstruction: a prospective evaluation of hamstring
and patella tendon grafts. Knee Surg Sports Traumatol
Arthrosc 1999;7(3):138–45.
11. Malhan K, Kumar A, Rees D. Tibial cyst formation
after anterior cruciate ligament reconstruction using
a new bioabsorbable screw. Knee 2002;9(1):73–5.
other devices
Rajeev et al. 506
12. Benedetto KP, Fellinger M, Lim TE, Passler JM,
Schoen JL, Willems WJ. A new bioabsorbable
interference screw: Preliminary results of a
prospective, multicentre, randomised clinical trial.
Arthroscopy 2000;16(1):41–8.
13. Feldmann DD, Fanelli GC. Development of a
synovial cyst following anterior cruciate ligament
reconstruction. Arthroscopy 2001;17(2):200–2.
14. Tsuda E, Ishibashi Y, Tazawa K, Sato H, Kusumi T,
Toh S. Pretibial cyst formation after anterior cruciate
ligament reconstruction with a hamstring tendon
autograft. Arthroscopy 2006;22(6):691.e1–6.
other devices
IJCRI 2013;4(9):507–510.
Kong et al. CASE REPORT
507
OPEN ACCESS
Missed retained knife blade injury: A potentially lethal trap
for the unwary
Victor Kong, John Bruce, Leah Naidoo, George Oosthuizen,
Grant Laing, Damian Clarke
Abstract
Introduction: Retained knife blade is an
uncommon injury and they often present in a
spectacular fashion. Concealed retained knife
blade, however, is difficult to diagnose without
a detailed clinical and radiological assessment.
Extraction requires careful planning in a
controlled environment, preferably in the
operating theater. Case Report: We present the
highly unusual case of a 25-year-old male in
whom a retained knife blade following a stab
to the left anterior leg was missed on three
separate visits to a rural hospital. Conclusion:
Whilst diagnosis and management continues to
be challenging, clinicians must always remain
vigilant to the possibility of such injury. When the
presentation is unclear, a high level of suspicion,
Victor Kong1, John Bruce2, Leah Naidoo3, George Oosthuizen4,
Grant Laing2, Damian Clarke4
Affiliations: 1MBChB, Surgical Registrar, Pietermaritzburg
Metropolitan Trauma Service, Department of Surgery,
Edendale Hospital, Pietermaritzburg, KwaZulu Natal, South
Africa; 2FCS(SA), Consultant Trauma Surgeon, Pietermaritzburg Metropolitan Trauma Service, Department of Surgery, Edendale Hospital, Pietermaritzburg, KwaZulu Natal,
South Africa; 3MBChB, Surgical House Officer, Pietermaritzburg Metropolitan Trauma Service, Department of Surgery,
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.
Corresponding Author: Victor Yeewai Kong, Pietermaritzburg Metropolitan Trauma Service, Department of Surgery,
Edendale Hospital, Private Bag X509, Plessislaer, Pietermaritzburg, 3216, KwaZulu Natal, South Africa; Cell No:
+27(0)797411036; Fax: +27(0)333954094; Email: [email protected]
Received: 06 December 2012
Accepted: 22 May 2013
careful clinical assessment and judicious use
of radiography is of paramount importance.
Early referral to a trauma center for definitive
management is crucial.
Keywords: Missed injury, Retained knife blade,
Extraction
*********
Kong V, Bruce J, Naidoo L, Oosthuizen G, Laing G, Clarke
D. Missed retained knife blade injury: A potentially
lethal trap for the unwary. International Journal of Case
Reports and Images 2013;4(9):507–510.
*********
doi:10.5348/ijcri-2013-09-367-CR-11
Introduction
Knife injuries are common and can present with a full
spectrum of pathology, ranging from minor cuts to severe
life-threatening injuries. Retained knife blade injury
logically falls within the scope of this injury spectrum [1].
Delayed presentation due to missed injuries is associated
with significant morbidity [2]. We report a highly unusual
case of a 25-year-old male who presented to our trauma
unit some three weeks after the initial injury, with a
concealed, retained knife blade in the left leg. It emerged
that the actual injury was missed and he had been sent
home by the doctor on multiple occasions.
CASE REPORT
Mr. B was 25-year-old male who presented to a rural
hospital following a single stab injury to the left anterior
leg. He was moderately intoxicated at the time. He was
assessed by a locum doctor at the hospital and was
thought to be an uncomplicated superficial stab wound.
IJCRI 2013;4(9):507–510.
He was discharged home after being given a prescription
for paracetamol. One week after his initial injury, the
patient presented again complaining of increasing pain
from the wound. Apart from some localized tenderness,
there was no discharge or surrounding cellulitis. He was
given a course of oral flucloxacllin, 250 mg, four times
daily for presumable wound sepsis. He represented again
the following week with no improvement and complained
of a sense of fullness and increasing pain in the anterior
leg extending to the calf. It was decided that antibiotics
dose was to be increase and he was again discharged with
a further course of oral flucloxacllin, this time at 500 mg,
four times daily. A week later, he presented for the third
time complaining of worsening of his symptoms, and at
the patient’s insistence, he was referred to our trauma
unit for a second opinion.
On arrival, the patient was in significant discomfort.
His baseline vitals were: heart rate: 90/min, blood
pressure: 135/70 mmHg, temperature 37.5oC. A small
puncture wound (approximately 1.5x1.5 cm) was noted in
the anterior aspect of the left leg (Figures 1 and 2) and
approximately 10 cm inferior to the tibial tuberosity.
The wound edges appear sloughy, with no surrounding
cellulitis. Tenderness was noted that extended from
anterior leg to the calf but no foreign body was palpable.
His pulses in the lower limbs were normal and equal,
with normal Doppler flow signal. Suspicion was aroused
about the retention of a foreign body and an urgent
X-ray was arranged. A large retained knife blade was
seen, with the trajectory in the superior direction, lodged
between the interosseus membrane (Figure 3). He was
commenced on intravenous co-amoxiclav, 1.2 g, and was
taken immediately to the operating theatre for a planned
removal. Intraoperatively, a longitudinal incision was
made extending from the wound edge, and the knife
blade was immediately visible. The knife blade was easily
extracted with minimal hemorrhage (Figure 4). There was
no evidence of osteomyelitis. An on-table angiography
was performed, which showed no vessel injuries. The
wound was thoroughly irrigated and was left to close by
secondary intension. He had an uneventful recovery and
was discharged on fifth day.
Kong et al. 508
Figure 2: Approximate dimension of the wound, note the
sloughy edge.
Figure 3: X-ray depicting the retained knife blade. Note the direction of the blade.
DISCUSSION
Figure 1: Stab wound on the anterior aspect of the left tibia, seen
3 weeks after the initial injury.
Retained knife blade remains a rather uncommon
injury [1]. Most of these injuries usually present in
spectacular fashion and the protruding objects are usually
obviously visible [3, 4]. Heroic attempts at extraction
in the emergency department may result in massive
torrential hemorrhage [5]. Most experience reported in
literatures has been from isolated case reports and several
small case series [1, 6, 7]. Being an uncommon injury,
most centers have limited experience in its management
[1]. Concealed retained knife blade injury is much more
IJCRI 2013;4(9):507–510.
Figure 4: Extracted knife blade. The shaft had clearly broken off
after the initial impact.
unusual. It becomes even more problematic if the initial
retained blade is not recognized. Delayed presentation
is not uncommon and one series reported a delay of up
to eight weeks after the initial injury [7]. Delay can be
associated with significant morbidity relating to sepsis,
even more so with injuries to the extremities.
This highly unusual case highlighted several important
issues worth discussing. Our patient initially presented to
a rural hospital and based on an unclear history related
to intoxication, the injuries was missed. However, with
the subsequent multiple representations, the possibility
of a retained blade injury was never considered, leading
to significant further delay in diagnosis. No radiography
was even taken on the suspected areas as the injury was
never considered, which would have easily revealed the
retained blade. In this case, the old adage of ‘what the
mind does not know, the eyes do not see’ stood the test
of time.
It is equally important to recognize that the appearance
of a stab wound can be deceptive. It is also obvious that
it bears no relationship to the magnitude of the potential
underlying injury. In our case, it was also rather peculiar
having noted that the wound was only approximately
1.5 cm, but the width of the retained blade was over 2 cm.
The most likely explanation of this disparity was probably
related to a degree of wound contraction after a long
delay. However, it is crucial to understand that this type
of presentation could potentially be misleading.
This case, however, is not an isolated incidence
related to suboptimal management for these patients
who present to rural hospitals in South Africa [8]. Rural
hospitals in South Africa and other developing countries,
facilities and resources are often severely limited [8]. This
situation is usually further compounded by a severe and
on-going shortage of medical staff and poorly maintained
infrastructures. The few who provide these essential
health services often lack the training and experience in
managing trauma patients [8]. Furthermore, even in wellstaffed urban trauma centers, diagnosis and management
of these patients is still difficult.
Kong et al. 509
Once the retained knife blade was identified, the issue
with definitive management can be difficult. In most
situations, a spiral computed tomography (CT) scan can
usually help defining the relation between the retained
blade and major significant anatomical structures [5].
This will generally facilitate a far safer extraction plan
[1]. Angiography is generally recommended if initial CT
scan is equivocal, especially if a major vascular injury is
suspected [1]. In our case, we elected to pursue a combined
exploration and extraction in the operating theater due to
the anatomical location of the injury. It was anticipated
that major hemorrhage in the vessels inferior to the
arterial trifurcation of the knee could be safely dealt with
and adequate vascular control achieved. Furthermore,
if there were injuries to one of the small branches from
the trifurcation, it was highly unlikely this would have
significant impact on the lower limb perfusion, and a
simple ligation would have been a safe option [9]. The
subsequent on table angiogram performed proved to be
reassuring. Despite the patient’s protracted delayed to
missed diagnosis on multiple occasions, he fortunately
made a full recovery.
CONCLUSION
Retained knife blade is an uncommon injury.
Concealed, retained knife blade is notoriously to diagnose.
Therefore, a high index of suspicion must be maintained,
coupled with judicious use of imagining, especially if the
history is unclear. Early and carefully planned extraction
in the operating theatre by a suitably trained trauma
specialist remains the cornerstone of good management.
*********
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
John Bruce – Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final
Leah Naidoo – Acquisition of data, Drafting the article,
George Oosthuizen – Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Grant Laing – Acquisition of data, Drafting the article,
Damian Clarke – Acquisition of data, Drafting the article,
IJCRI 2013;4(9):507–510.
Guarantor
2.
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
REFERENCES
1.
Kong et al. Sobnach S, Nicol A, Nathire H, Kahn D, Navsaria P.
Management of the retained knife blade. World J
Surg 2010 Jul;34(7):1648–52.
other devices
510
Madhok BM, Roy DD, Yeluri S. Penetrating arrow
injuries in Western India. Injury 2005;36(9):1045–
50.
3. Kelly IP, Attwood SE, Quilan W, Fox MJ. The
management of impalement injury. Injury
1995;26(3):191–3.
4. Thomson BN, Knight SR. Bilateral thoracoabdominal
impalement: avoiding pitfalls in the management of
impalement injuries. J Trauma 2000;49(6):1135–7.
5. Hanoch J, Feigin E, Pikarsky A, Kugel C, Rivkind A.
Stab wound associated with terrorist attacks in Israel.
JAMA 1996;276(5):388–90.
6. Grobbelaar A, Knottenbelt JD. Retained knife blades
in stab wounds of the face: is simple withdrawal safe?
Injury 1991;22(1):29–31.
7. van Lierop AC, Raynham O, Basson O, Lubbe DE.
Retained knife blades in the ear, nose and throat:
three cases. J Laryngol Otol 2009;123(3):351–5.
8. De Villiers MR, De Villiers PJT. Doctors’ views of
working conditions in rural hospitals in the Western
Cape. SA Fam Pract 2004;46(3):21–6.
9. Ballard JL, Bunt TJ, Malone JM. Management of small
artery vascular trauma. Am J Surg 1992;164(4):316–9.
other devices
IJCRI 2013;4(9):511–514.
Olajide et al. CASE REPORT
511
OPEN ACCESS
Novel surgical extrication of penile constricting metal ring:
A case report
Abimbola Olaniyi Olajide, Amogu Kalu Eziyi,
Folakemi Olajumoke Olajide, Babatunde Kazeem Beyioku
Abstract
Introduction:
Penile
entrapment
with
strangulation is a unique clinical entity which
has remained a challenge because of lack of
standard treatment modality. Treatment of
reported cases has been based on an individual
physicians approach depending upon the
circumstances and facilities available. Some of
these are cumbersome and some involve use of
non-medical facilities. We report a novel simple
surgical approach used in the management of
a case of penile entrapment. Case Report: A
16-year-old male adolescent presented with four
days history of entrapment of his penile shaft
in a hard steel metal ring. There was associated
swelling of penile shaft distal to the ring with
progressive diminution of urine stream. All
initial attempts for removing mettalic ring were
abortive till surgical degloving of penile shaft
was done in the operating room with immediate
reduction of swelling and removal of constricting
ring intact. There was no postoperative
complication and outcome was satisfactory.
Conclusion: Constricting device on a penile shaft
can be removed by a simple surgical technique
Abimbola Olaniyi Olajide1, Amogu Kalu Eziyi1, Folakemi
Olajumoke Olajide2, Babatunde Kazeem Beyioku3
Affiliations: 1Senior Lecturer, Department of Surgery, Ladoke
Akintola University of Technology, Ogbomoso, Oyo State,
Nigeria; 2Lecturer 1, Department of Community Health,
Obafemi Awolowo University, Ile-Ife, Osun State, Nigeria;
3
Resident Doctor, Department of Surgery, Ladoke Akintola
University of Technology Teaching Hospital, Osogbo, Osun
State, Nigeria.
Corresponding Author: Dr. Abimbola O. Olajide, Department
of Surgery, Ladoke Akintola University of Technology
Teaching Hospital, Osogbo, Osun State, Nigeria; Ph:
+2348037251893; E-mail: [email protected]
Received: 26 December 2012
avoiding some of the cumbersome modalities
earlier reported.
Keywords: Entrapment, Strangulation, Penile
*********
Olajide AO, Eziyi AK, Olajide FO, Beyioku BK. Novel
surgical extrication of penile constricting metal ring: A
case report. International Journal of Case Reports and
Images 2013;4(9):511–514.
*********
doi:10.5348/ijcri-2013-09-368-CR-12
Introduction
Penile entrapment with strangulation is a rare clinical
entity requiring urgent decompression [1]. Entrapping
object is usually applied when penis is flaccid or semierect. It becomes impossible to remove when penis
becomes erect leading to swelling of the penis distal to
the object [2]. The management of this condition poses
unique challenges to the treating physician through
variable presentation as well as lack of specific treatment
options. No standard treatment modality is described
in literature; every case needs an individual approach
depending upon the circumstances and facilities available
[3]. Therefore, penile entrapment remains a challenge
occasionally faced by urologists; it is tasking and requires
ingenuity for successful treatment [4].
Methods described in literature include aspiration
of corpora, use of saws, grinders, bolt cutters, and
dental drills. Others include denudation of penile skin
with postoperative skin grafting, amputation or use of
myofascial flap for reconstruction of penis [3].
We report this case to illustrate a surgical technique
used in the management of a penile entrapment with
successful outcome.
IJCRI 2013;4(9):511–514.
CASE REPORT
A 16-year-old boy presented in accident and emergency
department with 4 days history of entrapment of his penile
shaft in a steel ball bearing which he has used for erotic
purpose. The ring had slipped in easily and entrapped
after penile erection with failure of all attempts to remove
the metal ring. He did not seek any help until four days.
Later when he reported to relatives who made several
attempts at remove the metal ring including use of metal
cutting saws with no success. Urine stream has reduced
progressively with increasing straining at micturition.
On examination, stainless steel ring of about 1.5 cm
width impacted at the base of the penile shaft with
marked swelling of the penile shaft distal to the metal
ring and two areas of dark discolorations were noted
on the penile skin (Figure 1). Sensory sensations were
preserved on the penile shaft but arterial pulsation was
not palpable. Initial attempts by lubrication and gentle
traction on ring were unsuccessful following which he
was transferred to the operating room. After failure of
Gigli, orthopedic and metal cutting saws, he was given
general anesthesia through a face mask, a transverse
circumcising incision was made just proximal to the
coronal sulcus, and this was extended vertically on the
midline of the ventral surface of the penis. This incision
was deepened to the sub-dartos plane, skin and dartos
layer were subsequently raised off the Buck’s fascia.
Fluid with blood oozed out of the subcutaneous tissue
with swelling of skin and subcutaneous tissue observed
to reduce immediately. The skin and subcutaneous
tissue then slid easily under the metal ring (Figure 2),
followed by the erectile tissue contained within the
Buck’s fascia (Figure 3). Following removal of the metal
ring, the skin was closed in single layer using nylon 3-0
suture (Figure 4). Size 14 Foley’s catheter was passed
and left in place for 48 hours.
Postoperative period was uneventful and he was
discharged and sutures were removed on 4th and 8th
Figure 1: Preoperative picture showing entrapment and
strangulation of penile shaft in the metal ring.
Olajide et al. 512
postoperative days, respectively. The skin recovered
completely and there was no complaint with urination
and erection thereafter (Figure 5). He attended clinic for
five months and lost to follow-up thereafter.
Figure 2: Reduction of swelling with sliding of skin and
subcutaneous tissue under metal ring after penile degloving.
Figure 3: Metal ring on the erectile tissue after releasing the
skin and subcutaneous tissue from under the metal ring.
IJCRI 2013;4(9):511–514.
Figure 4: Immediate postoperative picture of penile shaft and
the metal ring after extrication.
Olajide et al. 513
Treatment requires urgent removal of the offending
device and decompression of edema to prevent ischemic
injury to the affected tissues. Various methods for removal
of the various constricting devices have been documented
in literature, these often involves consultation of various
services, including emergency services to obtain special
instruments to remove the constricting object [3, 6–9].
Most authors have canvassed for a multi-disciplinary
approach including the assistance of jewelers and
locksmiths and fire department to procure appropriate
tools for removal [1]. A few surgical techniques have also
been described including winding of silk from the glans,
string techniques and aspiration of corpora tissue [5, 10].
None of these methods is considered a standard modality
of treatment; each case needs an individual approach
depending upon the encircling material, assessment of
severity and available facilities and tools [3].
The method presented in this report is applicable and
successful because the swelling in penile entrapment is
due mainly to venous engorgement resulting in swelling
mostly in the skin and the subcutaneous fascia (dartos)
which is loosely attached to the underlying Buck’s fascia.
In addition, thickness of Buck’s fascia and corporeal tissue
resist pressure on the deep vessels, avoiding gangrene of
the erectile bodies [3]. These facts allow for easy elevation
of the penile covering over Buck’s fascia and its content
(degloving). As soon as degloving started, fluid and blood
was observed to egress from the subcutaneous tissues
with immediate reduction of the swelling of the penile
skin and subcutaneous tissue. This gave space for skin
and subcutaneous tissue to slide under the constricting
ring first and the penile shaft thereafter.
This procedure is simple, purely surgical and not
new to most urologists because it is part of some forms
of urethroplasty for hypospadias and urethral stricture.
It obviates need for any special instrument and further
delay in waiting for fire fighters, jewelers or locksmiths.
CONCLUSION
Figure 5: Picture of penile shaft three months after the surgery.
Penile entrapment and strangulation remains a rare
challenge to urologist worldwide with need for emergency
decompression to prevent grave complications. Simple
degloving of the penis reduces the swelling and allows
easy removal of the constricting object.
*********
DISCUSSION
Though rare, penile entrapment can be associated
with serious complications. It causes blockage of the
venous return resulting in swelling of the penile skin
and subcutaneous tissue distal to the object. Delay in
treatment results in penile strangulation with ischemic
necrosis and gangrene of the tissue [5].
Abimbola Olaniyi Olajide – Conception and design,
Acquisition of data, Drafting the article, Final approval of
Amogu Kalu Eziyi – Conception and design, Critical
revision of the article, Final approval of the version to be
published
IJCRI 2013;4(9):511–514.
Folakemi Olajumoke Olajide – Drafting the article,
Babatunde Kazeem Beyioku – Acquisition of data, Final
Guarantor
Copyright
© Abimbola Olaniyi Olajide et al. 2013; This article
ACKNOWLEDGEMENTS
We wish to acknowledge the Ms Funmlayo Atoyebi
and Dr Damola Tanimowo, the perioperative nurse and
anesthesiologist who supported us in the operating room
during the surgery.
other devices
Olajide et al. 514
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1.
Eaton SH, Dickstein RJ, Wiygul JB. Novel use of the
Gigli saw for management of penile entrapment. J Sex
Med 2009;6(2):595–7.
2. Perabo FG, Steiner G, Albers P, Müller SC. reatment
of penile strangulation caused by constricting devices.
Urology 2002;59(1):137.
3. Ivanovski O, Stankov O, Kuzmanoski M, et al. Penile
strangulation: Two case reports and review of the
literature. J Sex Med 2007;4(6):1775–80.
4. Patel C, Kim R, Delterzo M, Wang R. Prolonged penile
strangulation with metal clamps. Asian J Androl
2006;8(1):105–6.
5. Noh J, Kang TW, Heo T, Kwon DD, Park K, Ryu SB.
Penile Strangulation Treated With The Modified
String Method. Urology 2004;64(3):591.
6. Sathesh-Kumar T, Hanna-Jumma S, De’zoysa N,
Saleemi A. Genitalia strangulation-Fireman to the
rescue! Ann R Coll Surg Engl 2009;91(4):W15–6.
7. Silberstein J, Grabowski J, Lakin C, Goldstein I.
Penile constriction devices: Case report, review of the
literature, and recommendations for extrication. J
Sex Med 2008;5(7):1747–57.
8. Kang SP, Chakravarti A, Amer K. Help of the fire
brigade in a case of a strangulated penis. Ann R Coll
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9. Kore RN, Blacklock AR. Ring the fire brigade. Br J
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10. Vahasarja VJ, Hellstrom PA, Serlo W, Kontturi
MJ. Treatment of penile incarceration by the string
method: 2 case reports. J Urol 1993;149(2):372–3.
other devices
IJCRI 2013;4(9):515–517.
Han et al. CASE REPORT
515
OPEN ACCESS
Beta-hemolytic group B Streptococcus meningitis in a
young healthy woman
Li Han, Rohit Gosain, Maria Plataki, Daniel Horowitz, Daniel Bordea
Abstract
Introduction: Group B Streptococcus (GBS)
is the leading cause of bacterial meningitis
and sepsis in neonates but is a rare cause of
meningitis in adults. The GBS infections in adults
include bloodstream infections, pneumonia,
skin and soft-tissue infections, and bone and
joint infections. Case Report: A 23-year-old
woman with a past medical history of stroke
at the age of four and Staphylococcus aureus
toxic shock syndrome at age 10 was presented
to the emergency department with three days of
worsening frontal headache, fatigue and mild
confusion. Patient denied fever, photophobia,
neck stiffness, nausea and vomiting. Brudzinski’s
sign and Kernig’s sign were negative. Laboratory
data showed initially elevated WBC (13.5x103/
µL) and normal metabolic panel. Cerebrospinal
fluid (CSF) analysis was normal with two
white cells, all lymphocytes. After two days of
hospitalization, she was back to her normal state
without any headache or confusion and was
discharged home. One day after discharge, both
urine and CSF culture grew β-hemolytic GBS but
with negative blood cultures. She was readmitted
and placed on ceftriaxone 2g IV twice daily for
14 days. Conclusion: This case illustrates an
unusual presentation of group B Streptococcus
meningitis. Internists should be aware of atypical
group B Streptococcus meningitis and consider
treating patients with empiric antibiotics on
clinical suspicion.
Keywords: Group B Streptococcus, β-hemolytic
*********
Han L, Gosain R, Plataki M, Horowitz D, Bordea D. Betahemolytic group B Streptococcus meningitis in a young
healthy woman. International Journal of Case Reports
and Images 2013;4(9):515–517.
*********
doi:10.5348/ijcri-2013-09-369-CR-13
Introduction
Li Han1, Rohit Gosain2, Maria Plataki3, Daniel Horowitz4,
Daniel Bordea5
Affiliations: 1MD, First Year Resident, Internal Medicine,
Bridgeport Hospital, Bridgeport, Connecticut, USA; 2Dept.
of Internal Medicine, Bridgeport Hospital – Yale University,
Bridgeport, CT; 3MD, Second Year Resident, Internal
Medicine, Bridgeport Hospital, Bridgeport, Connecticut,
USA; 4MD, Third Year Resident, Internal Medicine,
Bridgeport Hospital, Bridgeport, Connecticut, USA;
5MD, Hospitalist, Internal Medicine, Bridgeport Hospital,
Bridgeport, Connecticut, USA.
Corresponding Author: Li Han, MD, First Year Resident,
Internal Medicine, Bridgeport Hospital, Bridgeport,
Connecticut, USA; Email ID: [email protected]
Group B Streptococcus (GBS) is gram-positive
β-hemolytic coccus characterized by the presence of
group B Lancefield antigen and the main virulence factor
is the polysaccharide anti-phagocytic capsule. The GBS
is the leading cause of bacterial meningitis and sepsis
in neonates but it is a very rare cause of meningitis in
adults. The GBS infections in adults include bloodstream
infections, pneumonia, skin and soft-tissue infections,
and bone and joint infections. This organism has been
recognized with increasing frequency as a substantial
cause of morbidity and mortality among non-pregnant
adults.
Received: 19 February 2013
CASE REPORT
A 23-year-old woman with a past medical history
of stroke at the age of four and Staphylococcus aureus
IJCRI 2013;4(9):515–517.
Han et al. 516
toxic shock syndrome at age 10 was presented to the
emergency department with three days of worsening
frontal headache, fatigue and mild confusion. She
complained of continuous throbbing-like headache in the
left frontal lobe, which she rated as 8 on a severity scale
of 10. Patient denied fever, photophobia, neck stiffness,
nausea and vomiting. In the emergency department, she
was found to be confused with behavioral changes such as
trying to scan one of her cards on the Purell hand sanitizer
machine and clicking her car remote control to turn on
the TV. Physical examination was normal except for a
2/6 systolic murmur at the apex with no radiation. No
nuchal rigidity or focal neurological deficit was observed.
Brudzinski’s and Kernig’s signs were negative. Laboratory
examination showed initially elevated WBC 13.5x103/µL
that went down to 9.5x103/µL the next day and normal
metabolic panel. Cerebrospinal fluid (CSF) analysis was
normal with two white cells, both lymphocytes (Table 1).
Computed tomography (CT) scan of the head showed
no acute intracranial abnormality. Magnetic resonance
imaging (MRI) of the brain revealed encephalomalacia
in the left frontal lobe but no acute infarction (Figure 1).
Electroencephalogram (EEG) was slightly abnormal
with occasionally increased amount of intermittent
delta activity, seen mostly in the frontal central areas
bilaterally. This finding could correlate with the patient’s
clinical confusion. Transthoracic echocardiogram
showed mild concentric left ventricular hypertrophy
with ejection fraction 60–65%, trace mitral and tricuspid
regurgitation. After two days of hospitalization without
using any antibiotics, she was back to her normal state
without any headache or confusion and was discharged
home. One day after discharge, both urine and CSF culture
grew β-hemolytic GBS. Blood cultures were negative.
Urinalysis showed specific gravity 1.03, leukocyte esterase
negative, nitrate negative, white blood cell count <5/Hpf,
glucose negative, protein negative, ketones negative.
CSF culture showed 4 colonies of β-hemolytic GBS and
urine culture showed 50,000 organisms/mL. She was
readmitted and placed on ceftriaxone 2 g IV twice daily
for 14 days. Further laboratory work-up included HIV
testing, and immunoglobulin level measurement both
were negative. Computed tomography of the abdomen
and pelvis was performed and abscess was excluded. She
was discharged home to complete 14 days of intravenous
antibiotic treatment.
in pregnant women make them more susceptible to GBS
infections, mainly in the urinary tract where the bacteria
are found in high numbers. However, rarely it may also
affect non-pregnant or immunocompetent adults, such
as those diagnosed with diabetes mellitus, malignancy,
HIV infection, and advanced hepatic renal disease [2].
Incidence of invasive GBS infections has increased by
2–4 folds in the past two decades and approximately twothirds of these cases are observed in adults [3].
Classical cases of bacterial meningitis presents with
diffuse headaches, fever, nuchal rigidity, and change in
mental status [4, 5]. Some patients have temperatures
ranging above 38°C, and some present with hypothermia,
but it is important to note that no one presents with
normal temperature [6]. However, our case was unique
such that the patient denied fever and presented with
headache, confusion and normal CSF analysis.
DISCUSSION
CONCLUSION
The GBS is a gram-positive β-hemolytic streptococci
and bacitracin resistant organism. It is a part of the normal
flora of gastrointestinal tract, upper respiratory tract and
genital tract in both men and 15–45% women [1]. However,
GBS is capable of causing serious infections, primarily in
neonates, pregnant women and immunocompromised
individuals. Neonates acquire GBS infection in utero or
during passage through vagina. Physiological changes
We report a very unique case of group B
Streptococcus meningitis in a 23-year-old non-pregnant
immunocompetent female. The patient did not present
with classical triad of typical bacterial meningitis, rather
with headache, confusion and normal cerebrospinal
fluid analysis. This patient acquired the infection
probably from urinary tract because both the urine and
cerebrospinal fluid cultures are positive for β-hemolytic
Table: Cerebrospinal fluid (CSF) analysis.
Apperance Pressure
(cmH2O)
Clear
12
WBC,
Predom
2,
lymphocytes
Glucose Protein
(mg/
(mg/
dL)
dL)
58
28
Figure 1: Magnetic resonance imaging scan of brain: Small
ovoid focus of signal abnormality in the left frontal lobe, likely
representing encephalomalacia given the clinical history of
stroke as a young child. No evidence of acute infarction or
evidence of acute parenchymal hemorrhage.
IJCRI 2013;4(9):515–517.
group B Streptococcus. Internists should be aware of
atypical group B Streptococcus meningitis and consider
treating patients with empiric antibiotics.
*********
Li Han – Substantial contributions to conception and
design, Analysis and interpretation of data; Drafting the
Rohit Gosain – Substantial contributions to conception
and design, Acquisition of data; Drafting the article,
Maria Plataki – Substantial contributions to conception
and design, Revising it critically for important intellectual
Daniel Horowitz – Substantial contributions to
conception and design, Revising it critically for important
published
Daniel Bordea – Substantial contributions to conception
and design, Revising it critically for important intellectual
Han et al. Copyright
© Li Han et al. 2013; This article is distributed under
the terms of Creative Commons attribution 3.0 License
which permits unrestricted use, distribution and
REFERENCES
1.
2.
3.
4.
5.
Guarantor
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Farley MM. Group B streptococcus disease in
nonpregnant adults. Clin Infect Dis 2001 Aug
15;33(4):556–1.
Jackson LA, Hilsdon R, Farley MM, et al. Risk factors
for group B streptococcal disease in adults. Ann Intern
Med 1995 Sep 15;123(6):415–20.
Skoff TH, Farley MM, Petit S, et al. Increasing
burden of invasive group B streptococcal disease in
nonpregnant adults, 1990-2007. Clin Infect Dis 2009
Jul 1;49(1):85–92.
Domingo P, Mancebo J, Blanch L, Net A, Nolla J.
Fever in adult patients with acute bacterial meningitis.
J Infect Dis 1988 Aug;158(2):496.
Attia J, Hatala R, Cook DJ, Wong JG. The rational
clinical examination. Does this adult patient have
acute meningitis? JAMA 1999 Jul 14;282(2):175–81.
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timing. Ann Intern Med 1998 Dec 1;129(11):862–9.
other devices
IJCRI 2013;4(9):518–523.
Kanagal et al. case in images
518
OPEN ACCESS
Acardiac twin: Conservative management
Deepa V Kanagal, Mangala Srikantaswamy, Rashmi M Devaraj,
Maitri M Kulkarni, Mahendra Gangadhariah,
Lokeshchandra C Hanumanalu
Abstract
Introduction: Acardiac twin is a rare anomaly
occurring in 1 in 35,000 pregnancies. The outcome
is fatal for the acardiac twin with a 50–75%
mortality of the normal pump twin. Management
options include conservative management,
medical therapy, occlusion of connecting
vessels and selective termination of the acardiac
twin. The Management of a twin pregnancy
complicated by an acardiac twin is a challenge
because the continuous growth of the acardiac
fetus is deleterious to the healthy pump twin
leading to cardiac insufficiency, polyhydramnios,
prematurity and even death of the normal twin.
Case Report: We report three cases of acardiac
twinning which presented with different
manifestations, acardius acephalus, acardius
myelocephalus and acardius amorphous. All the
three cases were managed conservatively with
Deepa V Kanagal1, Mangala Srikantaswamy2, Rashmi M
Devaraj3, Maitri M Kulkarni4, Mahendra Gangadhariah4,
Lokeshchandra C Hanumanalu5
Affiliations: 1M.S, DNB, Associate Professor, Department of
Obstetrics and Gynecology, K.S.Hegde Medical Academy,
Mangalore, Karnataka, India; 2M.D, Assistant Professor,
Department of Anatomy, Rajarajeshwari Medical College
Bangalore, Karnataka, India; 3M.S, Assistant Professor, Department of Obstetrics and Gynecology, J.S.S. Medical College, Mysore, Karnataka, India; 4M.S, Assistant Professor,
Department of Obstetrics and Gynecology, Adichunchanagiri Institute of Medical Sciences, Mandya, Karnataka, India;
5
M.D, D.G.O, Professor and Head, Department of Obstetrics
and Gynecology, Mysore Medical College and Research Institute, Mysore, Karnataka, India.
Corresponding Author: Deepa V. Kanagal, Associate Professor, Department of Obstetrics and Gynecology, K.S.Hegde
Medical Academy, Mangalore-575018, Karnataka, India; Ph:
09980164615; E-mail: [email protected]
Received: 19 June 2012
good outcome of the pump twin in all the cases.
We present the cases with review of literature.
Conclusion: Conservative non-intervention may
be appropriate in developing countries with low
resources.
Keywords: Acardiac twins, Twin pregnancy,
Conservative management
*********
Kanagal DV, Srikantaswamy M, Devaraj RM, Kulkarni
MM, Gangadhariah M, Hanumanalu LC. Acardiac twin:
Conservative management. International Journal of Case
Reports and Images 2013;4(9):518–523.
*********
doi:10.5348/ijcri-2013-09-370-CII-14
Introduction
Acardiac twinning or Twin Reversed Arterial
Perfusion (TRAP) is a rare anomaly occurring in
monozygotic multiple pregnancies with an incidence
of 1% and in 1 in 35,000 pregnancies [1]. The mortality
in recipient acardiac twin is 100% whereas the normal
donor twin is also at an increased risk of morbidity and
mortality. This is a report of three cases of acardiac twins
with different presentation. One was acardius acephalus,
another was acardius myelocephalus and the third one
was acardius amorphous. All cases resulted in live birth
of the normal twin.
CASE REPORT
Case 1: A 30-year-old third gravida with previous
two uneventful vaginal deliveries came to us for the
first time at 34 weeks of gestation with twin pregnancy
in active labor. She delivered a normal female baby of
IJCRI 2013;4(9):518–523.
Kanagal et al. 519
1.7 kg followed by a malformed, dead acardiac fetus
of 900 g (Figure 1). The acardiac fetus had a grossly
malformed upper body without upper limbs head and
neck (acardius acephalus) (Figure 2A). The trunk was
partially developed. The lower limbs were developed but
had bilateral congenital Talipes equinovarus deformity
and syndactyly (Figure 2B). External genitalia resembled
female (Figure 2C). There was a single placenta with two
cords , one small and thin belonging to the acardiac fetus.
(Figure 3). Autopsy showed the absence of neural tissue,
(Figure 4) absence of lungs, incomplete diaphragm,
Figure 3: Single placenta with 2 cords (Case 1).
Figure 1: Normal fetus and acardiac (Case 1).
Figure 4: Absent neural tissue (Case 1).
Figure 2: (A) Acardius acephalus (Case 1), (B) Bilateral
Congenital Talipes equinovarus (Case 1), (C) Female Genitalia
(Case 1).
rudimentary cardia, liver tissue, uterus, fallopian tube,
ovary, kidney and ureters. (Figure 5). The normal twin
was fine at the time of discharge three days later.
Case 2: A 26-year-old second gravida with previous
normal delivery came with 24 weeks pregnancy. On
examination, the uterus was of 28 weeks size. Ultrasound
revealed twin pregnancy with one normally formed fetus
of 24 weeks and another fetus with a partially formed head
with hydrocephalus and enlarged lateral ventricle (Figure
6A–B). The spine was partially seen and the lower limbs
IJCRI 2013;4(9):518–523.
Kanagal et al. 520
Figure 5: Thoraco-abdominal autopsy (Case 1).
Figure 7: Abdomen of fetuses, ascites in acardiac twin and
common placenta (Case 2).
Figure 6: (A) Hydrocephalus (Case 2). (B) Enlarged lateral
ventricle (Case 2).
were recognizable. No other anatomical structure was
made out. Gross abdominal edema and ascites was noted
(Figure 7). The placenta was a single large mass. Color
Doppler of umbilical artery of the abnormal fetus showed
reversal of flow thus confirming the diagnosis of acardiac
pregnancy. The parents were counseled regarding the
prognosis of the surviving twin, options of conservative
and invasive management and non availability of invasive
management in our center. After obtaining their consent,
the lady was managed conservatively with regular
follow-up and serial ultrasound examination. At 36th
week of gestation, she developed polyhydramnios and
pre-eclampsia and an emergency cesarean section was
done. A borderline term male fetus of 2 kg weight with
normal morphology was delivered by breech extraction.
The other twin was acardiac weighing 750 grams with
a partially developed head and face. Lower limbs could
be made out. (acardius myelocephalus) (Figure 8A–B).
External genitalia were poorly developed. Autopsy was
not done for the acardiac fetus. The normal twin fared
well and was discharged on seventh postoperative day.
Case 3: A second gravida of 21 years of age with a
previous cesarean section presented at 24th week of
gestation with twin pregnancy. Ultrasonography showed
the first twin to be an acardiac amorphous mass without
any recognizable structures. Only the spine was made
out throughout its length (Figure 9). The second twin
was normally developed. There was a single placenta
(Figure 10). After explaining about the treatment
options, the lady opted for conservative management
in view of the cost and non-availability of facilities for
invasive management at our hospital. She was followed-
Figure 8: (A) Acardius myelocephalus (Case 2). (B) Partially
developed head (Case 2).
Figure 9: Acardius amorphous with spine (Case 3).
up by serial ultrasound examinations. The growth of the
normal fetus was satisfactory without any complications.
The patient had regular antenatal check ups and was
treated for anemia. She went into labor at 36th week of
gestation. An emergency cesarean section was done in
view of previous cesarean section. The first twin was an
amorphous soft, globular mass without any recognizable
structure and was weighing 700 g (acardius amorphous)
(Figure 11). The second twin was a normal female of
weight 2.1 kg. The postoperative period was uneventful.
The mother and the normal twin were discharged on the
eighth postoperative day.
IJCRI 2013;4(9):518–523.
Figure 10: Both fetuses with single placenta (Case 3).
Figure 11: Acardius amorphous specimen (Case 3).
DISCUSSION
Multiple pregnancy accounts for 1.5% of all
pregnancies with a perinatal morbidity and mortality of
10% [2]. Acardiac twinning or TRAP sequence is a rare
congenital anomaly of monozygotic multiple pregnancy
due to abnormal placental anastomosis characterized
by formation of a malformed fetus with an absent or
rudimentary heart (acardius) and other structures. There
Kanagal et al. 521
is usually a normally formed donor twin who may have
features of heart failure. Acardiac fetuses were first
described by Beneditti in 1533 [3]. Acardiac acephalic
variety is the most common type of acardiac fetus.
It has been hypothesized that the TRAP sequence is
caused by a large artery to artery placental shunt often
accompanied by a vein to vein shunt. Within the single
shared placenta, arterial perfusion pressure of the donor
twin exceeds that of the recipient twin who thus receives
reverse blood flow of deoxygenated arterial blood from
its co-twin. This used blood reaches the recipient twin
through its umbilical arteries and preferentially goes to
its iliac vessels. Thus only the lower body is perfused and
disrupted growth and development of the upper body
results [4].
An acardiac twin should be suspected in all
monochorionic, malformed fetuses with cystic hygroma,
generalized edema and an absent cardiac pulsation with a
non-functioning heart. Also, an ultrasonography finding
of twins revealing discordant or grotesque malformation
along with reverse flow in the umbilical artery is usually
diagnostic of an acardiac twin [5]. This can be diagnosed
in first trimester by vaginal scanning and color Doppler
sonography. Serial ultrasonography is indicated to assess
such twin pregnancies.
Failure of the head growth is called acardius acephalus,
a partially developed head with identifiable limbs is
acardius myelocephalus and failure of any recognizable
structure to form is acardius amorphous [6]. Acardiac
twins can also be classified as follows:
Acardius anceps – when head is poorly formed,
Acardius acephalus – if the head is absent
Acardius acormus – when only head is present.
Based on the development of heart, acardiac twins can
be classified as hemiacardius when heart is incompletely
formed and holoacardius if the heart is absent [7]. The
prominent features of the recipient twin are total or
partial absence of cranial vault, holoprosencephaly,
absent facial structures, anophthalmia, microphthalmia,
cleft lip, cleft palate, absent or rudimentary limbs, lungs,
heart, liver and gallbladder, diaphragmatic defects,
esophageal atresia, ventral wall defects, ascites, edema of
skin and single umbilical artery.
Management of twin pregnancy with an acardiac fetus
is a challenge as the continuous growth of the acardiac
fetus is deleterious to the healthy pump twin. It can lead
to cardiac insufficiency, polyhydramnios, prematurity
and even death of the structurally intact twin in up to
50% of cases [8].
Moore et al. reviewed 49 cases of acardiac twins and
reported that perinatal outcome was related to the ratio
of weight of the acardiac twin to the weight of the normal
twin. They stated that when twin weight ratio which is
the acardiac twin weight divided by the normal twin
weight was above 70%, preterm labor, hydramnios and
congestive heart failure in the pump twin were found and
when the weight of the acardiac fetus was less than 25%
compared to the pump twin, the prognosis was better
IJCRI 2013;4(9):518–523.
[8].The weight ratio in this study was derived from post
delivery weights. They also proposed a second order
regression equation to predict weight of the acardiac
twins antenatally: Weight (g) = 1.2 x (longest dimension
(cm)) 2 – 1.7 x longest dimension (cm). Also the use of
abdominal circumference ratios could provide a better
approach to establish the differences in body weights [8].
Brassard et al. reported that low pulsatility indices in
the umbilical artery perfusing the acardiac twin compared
with the pump twin correlated with poor prognosis [9].
Optimal management is controversial. Expectant
management versus prenatal intervention is to be
debated. Many methods of management have been
proposed including termination of pregnancy, serial
ultrasound scans to monitor for signs of decompensation,
medical management of polyhydramnios or by serial
amniocentesis, hysterotomy to remove anomalous twin
and invasive procedures. Goal of prenatal treatment is to
stop blood flow to the acardiac twin without affecting the
pump twin in order to improve its outcome. Platt et al. in
1983 were the first to suggest occlusion of the circulation
to the acardiac twin as the definitive treatment to interrupt
blood supply to it [10]. Minimally, invasive intervention
methods are through cord occlusion techniques or
intrafetal ablation. Cord occlusion has been attempted
by embolization, cord ligation, laser coagulation, bipolar
diathermy and monopolar diathermy while intrafetal
ablation is performed with alcohol, monopolar diathermy,
interstitial laser and radiofrequency [11].
Tan and Sepulveda recommended intrafetal ablation
as the treatment of choice than cord occlusion [11]. They
claimed ultrasound guided intrafetal approach to be
easier, less invasive and with a higher rate of success
than ultrasound and fetoscopy guided cord occlusion
procedures.
Invasive treatment should be restricted to those
pregnancies which would benefit from prenatal
intervention like those where the pump twin is at a
significant risk of prematurity, cardiac insufficiency
or death and should be considered in presence of poor
prognostic factors like polyhydramnios, ultrasound
markers of cardiac insufficiency, large acardiac twin and
rapid growth of or evidence of substantial blood flow
perfusion through the umbilical vessel supplying the
parasitic mass [12].
Sullivan et al. advocated expectant management in all
cases [13]. They reported 90% survival in pump twin in 10
pregnancies with an acardiac twin managed expectantly.
They cautioned against aggressive intervention and
recommended expectant management with close fetal
surveillance. Stamatian et al. gave an opinion that
conservative management is indicated in cases where
the acardiac twin is small and when there are no signs of
cardiovascular impairment in the pump twin [14]. Serial
ultrasound surveillance is important for detecting any
worsening of the condition, which may suggest the need
for interventions to optimize the pump-twin’s chance for
survival [15].
Kanagal et al. 522
CONCLUSION
Expectant management with close antepartum
surveillance deserves consideration in cases of
monozygotic twins with Twin Reversed Arterial Perfusion
sequence. Neonatal mortality of the pump twin diagnosed
antenatally may be considerably less than reported.
The interruption of vascular communication between
the twins is difficult to accomplish. It needs expensive
equipment and trained personnel for the procedures.
Hence invasive treatment should be considered only in
cases with poor prognostic factors. Such pregnancies
should be managed by fetal medicine specialists familiar
with invasive procedures. Intra fetal ablation procedures
are better than cord occlusion techniques. In our
cases, all the three pump twins survived with expectant
management.
ACKNOWLEDGEMENTS
We thank Dr. Praveen Kannan of Kannan Diagnostic
Centre, Karnataka, India for the excellent ultrasound
pictures.
*********
Deepa V Kanagal – 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
Mangala Srikantaswamy – Substantial contributions to
Rashmi M Devaraj – Substantial contributions to
Maitri M Kulkarni – Substantial contributions to
Mahendra Ganagadharaiah – Substantial contributions
to conception and design, acquisition of data, drafting
the article, revising it critically for important intellectual
Lokeshchandra C Hanumanalu –
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
Guarantor
IJCRI 2013;4(9):518–523.
Copyright
© Deepa V Kanagal et al. 2013; This article is distributed
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twinning. Am J Obstet Gynecol 1990;163(3):907–12.
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IJCRI 2013;4(9):524–526.
Gupta et al. 524
letters to editorOPEN ACCESS
Primary cavernous hemangioma of the thyroid gland
Devika Gupta, Sunita Kakkar, Pooja Gupta, Vandana Rana
To the Editor,
Hemangiomas are benign vascular neoplasms that
have a characteristic clinical course marked by early
proliferation and spontaneous involution. They occur in a
number of organs including skin, lips, liver, colon, brain,
etc. [1, 2].
Primary thyroid hemangiomas are extremely
uncommon with only a few cases have been reported [3].
We report a case of cavernous hemangioma presenting as
solitary nodule thyroid.
A 49-year-old male presented with one year history of
slowly enlarging swelling on left side of the neck. There
was no history of any associated pain, voice change or
dyspnea. There was no history of any trauma or previous
fine needle aspiration cytology (FNAC). There was no
family history of thyroid disease.
Local examination revealed an approximately
3x2.5 cm nontender swelling in front and left side of
cricoid cartilage. The swelling was firm to hard with well
defined margins and it moved with deglutition.
Serum thyroid stimulating hormone was 3.8 mIU/L
(normal range 0.4–4.0 mIU/L) and free T4 was 9 pmol/L
Devika Gupta1, Sunita Kakkar2, Pooja Gupta3, Vandana
Rana4
Affiliations: 1MD, Pathology, Assistant Professor, Armed
Forces Medical College, Department of Pathology and
Laboratory Science, Command Hospital, Pune, India;
2
MD, Pathology and OncoPatholgy, Professor, Armed
Forces Medical College, Department of Pathology and
Laboratory Science, Command Hospital, Pune, India;
3
MD, RadioDiagnosis, Department of Radiodiagnosis and
Imaging, Armed Forces Medical College, Pune, India;
4
MD, Pathology, Department of Pathology and Laboratory
Science, Command Hospital, Pune, India.
Corresponding Author: Devika Gupta, MD, Pathology,
Assistant Professor, Armed Forces Medical College,
Department of Pathology and Laboratory Science,
Command Hospital, Pune -411040, India; Ph: 9158984335;
Received: 14 March 2013
(normal range 10.3–24.5 pmol/L). These hormonal levels
were normal and no antithyroid antibodies were detected.
Thyroid ultrasonography revealed a 2.5x2.8 cm
isoechoic lesion in the left lobe of thyroid with a
hypoechoic rim and a prominent vessel having arterial
flow in its superior aspect (Figure 1). Ultrasonography
did not show any areas of calcification or cervical lymph
nodes. Right lobe of thyroid was normal.
The FNAC of the mass was attempted thrice but
was inconclusive as only blood was aspirated. Patient
underwent left hemithyroidectomy. Gross examination
of the cut surface of the specimen revealed a well
circumscribed nodular lesion measuring 2.5x2.8 cm. It
appeared cystic and hemorrhagic in appearance.
Histological examination of the tumor confirmed a
cavernous hemangioma. There were multiple, dilated,
irregular anastomosing vascular channels of varying
sizes. These vascular spaces were lined by bland
endothelial cells. Amidst these were noted an occasional
entrapped thyroid follicle (Figure 2A–B). The patient was
discharged from the hospital after five days with no signs
of complication.
In most cases, a cavernous hemangioma of the thyroid
gland represents a secondary hemangioma occurring
due to previous FNAC. These are formed due to vascular
proliferation in organized hematoma following FNAC
[4, 5]. Organization of the hematoma generally results
in complete resolution, but it can give rise to vascular
and fibroblastic proliferative changes that resemble a
cavernous hemangioma. This is defined as secondary
hemangioma.
Primary hemangioma is a developmental anomaly
resulting from inability of angioblastic mesenchyma to
form canals [6]. Preoperative diagnosis of hemangioma
of the thyroid is difficult since there are no pathognomic
features on FNAC, ultrasonography or computed
tomography scans. The presence of heterogenous signal
intensity and serpentine pattern on magnetic resonance
imaging (MRI) scan is considered highly suggestive of
cavernous hemangioma [7]. In patients with a thyroid
swelling who have a cold nodule on thyroid scan and only
blood is aspirated on repeated FNACs, Technetium-99m
erythrocyte blood pool imaging may be performed to
diagnose hemangioma. Little or no increased activity is
IJCRI 2013;4(9):524–526.
seen soon after injecting the label, and this appearance
of poor perfusion and slow filling of the tumor is
characteristic of cavernous hemangioma. Hemangioma
should be considered in the diagnosis of any pulsatile
mass involving the thyroid gland. Diagnosis before
surgery is difficult and the definite diagnosis relies on
histological findings of surgical specimen.
Gupta et al. 525
Sunita Kakkar – Acquisition of data, Drafting the article,
Pooja Gupta – Acquisition of data, Drafting the article,
Vandana Rana – Acquisition of data, Drafting the article,
Guarantor
Copyright
Figure 1: Ultrasonographic image of the left lobe of thyroid
showing 2.4x2.6 cm well defined isoechoic lesion with
hypoechoic rim and a large prominent vessel within having
arterial flow (marked with arrow).
© Devika Gupta et al. 2013; This article is distributed
REFERENCES
1.
Figure 2: (A) The thyroid nodule shows dilated, congested
varying sized anastomosing vascular channels (H&E stain,
x20), (B) A few scattered colloid filled thyroid follicles amidst
the vascular channels (H&E stain, x40).
*********
Gupta D, Kakkar S, Gupta P, Rana V. Primary cavernous
hemangioma of the thyroid gland. International Journal
of Case Reports and Images 2013;4(9):524–526.
*********
doi:10.5348/ijcri-2013-09-371-LE-15
Kano M, Kameyama K, Hosoda Y, Sugino K, Ito K.
A cavernous haemangioma of the thyroid gland. J
Laryngol Otol 2005 Oct;119(10):828–30.
2. Ríos A, Rodríguez JM, Martínez E, Parrilla P.
Cavernous hemangioma of the thyroid. Thyroid 2001
Mar;11(3):279–80.
3. Kumamoto K, Sugano K, Hoshino M, Utsumi Y,
Suzuki S, Takenoshita S. Cavernous hemangioma of
the thyroid. Thyroid 2005 Oct;15(10):1199–201.
4. Tsang K, Duggan MA. Vascular proliferation of the
thyroid. A complication of fine-needle aspiration.
Arch Pathol Lab Med 1992 Oct;116(10):1040–2.
5. Datta R, Venkatesh MD, Nilakantan A, Joseph B.
Primary cavernous hemangioma of thyroid gland. J
Postgrad Med 2008 Apr-Jun;54(2):147–8.
6. Kumar R, Gupta R, Khullar S, Dasan B, Malhotra A.
Thyroid haemangioma: A case report with a review of
the literature. Clin Nucl Med 2000 Oct;25(10):769–1.
7. Memis A, Arkun R, Ustun EE, Kandiloglu G. Magnetic
resonance imaging of intramuscular haemangiomas
with emphasis on contrast enhancement patterns.
Clin Radiol 1996 Mar;51(3):198–204.
*********
Devika Gupta – Substantial contributions to conception
and design, Acquisition of data, Analysis and
IJCRI 2013;4(9):524–526.
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