Volume 3 Issue 7, July 2016

Parulekar SV

Metaplastic Papillary Tumor Of The Fallopian Tube
Manjrekar V, Parulekar SV, Fernandes G, Shaikh S.

Extensive Parietal Abdominal Wall Endometriosis Post LSCS
George S, Fernandes G, Rojekar A, Gupta AS.

Postpartum Tuberculous Pyoperitoneum
Thakur HS, Madhva Prasad S, Gupta AS.

Rectus Sheath Leiomyoma
Parulekar SV.

Obstetric Care In A Case Of Hemophilia A
Anuranjani L, Shah A, Chauhan AR.

Successful laparoscopic management of a giant paraovarian cyst in an adolescent female
Shah NH, Kale KG, Paranjape SH, Shah VN.

Low Lying Implantation of Ectopic Pregnancy- Is Clubbed Terminology More Apt?
Pai K, Kamath S, Samant PY.

Severe Preeclampsia and Idiopathic Intracranial Hypertension: Possible Autoimmune Connection
Honavar PU,  Samant PY, Bhosle SB.

Paroxysmal Supraventricular Tachycardia In Pregnancy
Panchbudhe S, Satia MN.


Parulekar SV

An abdominal wall mass is usually a general surgical problem. It is not very often that a gynecologist has to address one. However one has to be conversant with the differential diagnosis, so that one does not miss a gynecological cause of such a presentation. Such a lump can be secondary to trauma, infection, neoplasia, or defects in the abdominal wall. Trauma would result in formation of a hematoma. Infections include abscesses – either confined to the abdominal wall or due to communication with an underlying bowel disease adherent to the abdominal wall. A suture granuloma and an injection granuloma are examples of iatrogenic conditions that belong to the infective category. Neoplasms form a large percentage of such masses. Benign neoplasms found in the anterior abdominal wall include a fibroma, lipoma, fibrolipoma, rhabdomyoma, leiomyoma, angioma, neurofibroma, and peripheral nerve tumor. Desmoid tumor is a benign tumor in that it does not metastasize, but it is locally aggressive, and tends to recur after excision. Malignant tumors in the abdominal wall include metastatic disease, lymphoma, and rhabdomyosarcoma. Abdominal wall hernia may occur at different sites. When it becomes irreducible, it may mimic many other conditions producing abdominal wall lumps. Endometriosis is a unique gynecological condition that can be found in the anterior abdominal wall. A hematoma is an acute condition that usually follows trauma. The presence of a coagulopathy predisposes to it. Iatrogenic hematomas follow abdominal surgery, when the hemostasis has not been adequate. It may be found in any layer of the abdominal wall, the subcutaneous tissue and the rectus sheath being the most common ones. It is acute, painful, and associated with features of concealed blood loss. An abscess is another acute painful condition with features of an acute inflammation. Suture granulomas are not very large, usually superficial and easy to diagnose. Injection granulomas are not very common, because the anterior abdominal wall is not a common site for administration of injections. A prior history of an administration of an injection is helpful in making a diagnosis. Benign connective tissue tumors can be found in the anterior abdominal wall as commonly as anywhere else in the body. They have features of such tumors elsewhere. They may be difficult to differentiate from other lesions when they are deep seated. A desmoid tumor tends to arise during a pregnancy or in the scar of a cesarean section. It can occur after trauma or surgery other than a cesarean section too, and with estrogen therapy. It develops from fascia, muscle or aponeuroses. It is locally infiltrative and hence of relative fixity. If the patient has undergone an excision of a tumor at that site and presents with a recurrence, it is more likely to be a desmoid tumor than any other tumor. Breast cancer the most common cancer in a woman that spreads to the abdominal wall, while in a man it is a melanoma. About ten percent of all malignant tumors develop superficial soft tissue metastases. Lymphomas are also known to be found in the anterior abdominal wall. Iatrogenic spread of this type is seen following implantation of malignant cells during abdominopelvic surgery for a malignancy. Anterior abdominal wall hernias can be diagnosed easily when they are reducible – by both expansile impulse on coughing and reducibility. When irreducible, past history of reducibility helps in diagnosis. If not, they mimic benign tumors and need help with imaging techniques for diagnosis. Obstructed and strangulated hernias need urgent surgical care. Clinical history, associate intestinal obstruction, local acute condition and general toxicity, along with imaging clinch the diagnosis. Abdominal wall endometriosis is a condition that can be diagnosed clinical with almost 100% accuracy. It usually follows a cesarean section or a hysterotomy, sometimes any other operation in which the endometrium has been opened. It may be associated with mullerian duct anomalies of obstructive type, associated with intraabdominal and pelvic endometriosis. The lesions may be superficial or deep, usually fixed due to local infiltration and inflammation leading to fibrosis. The lesions are painful with menstruation. They enlarge and become tender during menstruation. A hemogram helps suspect inflammatory conditions. Imaging techniques like ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) are extremely useful in making a diagnosis. In this issue, we have two interesting cases, one of a rectus sheath leiomyoma, and the other of extensive endometriosis. I hope the readers enjoy reading about those.

Metaplastic Papillary Tumor Of The Fallopian Tube

Author Information

Manjrekar V*, Parulekar SV**, Fernandes G***, Shaikh S****.
(* Third Year Resident, *** Professor and Head, Department of Pathology, *** Associate Professor, **** Second Year Resident, Department of Pathology, Seth G S Medical College & KEM Hospital, Mumbai, India.)


Metaplastic papillary tumor of the fallopian tube is an extremely rare condition. It is a benign condition, without any reports of recurrence or malignancy. But its nuclear atypia and papillary architecture suggest a similarity to serous borderline ovarian tumors. There are only eleven cases reported in the world literature. We report the twelfth case.


Metaplastic papillary tumor (MPT) of the fallopian tube is an extremely rare condition. Though its behavior is as of a benign condition in that it does not show recurrence or metastases, it has some features resembling serous borderline ovarian tumors. There are eleven such cases reported so far. We report the twelfth case here, and discuss its etiology and management.

Case Report

A 26 year old primigravida was referred to obstetric emergency room from a private hospital in Mumbai with ultrasonography (USG) suggestive of an unruptured right tubal ectopic gestation. Patient was receiving treatment for primary infertility since 3 months, currently on follicular monitoring and planned relations. She was asymptomatic having no complaints of abdominal pain, bleeding per vaginum and syncope. On examination her vital parameters were in normal limits, systemic examination was unremarkable. The abdomen was soft and nontender. On per speculum examination cervix and vagina were healthy. Vaginal examination revealed a 6 weeks size retroverted uterus with right forniceal tenderness and a 2 cm x 1 cm smooth mass felt through the right fornix with no cervical motion tenderness. An USG done at KEMH showed 1.8 x 2.0 cm hypoechoeic structure suggestive of Right adnexal ectopic gestation with a 3x 2 cm right simple ovarian cyst with no free fluid in the pelvis. Her routine blood investigations and serum biochemistry were within normal range. Serum β-HCG was 5296 mIU/ml and 5319 mIU/ml after 48 h. A plan for conservative line of management was made and Methotrexate 50mg/m2 was given intramuscularly. The patient was monitored and serial abdominal girth measurements were done. Patient complained of acute abdominal pain 6 h post methotrexate administration and USG done at that time revealed moderate hemoperitoneum, suggesting rupture of the ectopic gestation. An emergency exploratory laparotomy was performed under general anesthesia. There was a  5 cm x 4 cm right adnexal mass having a smooth surface. It was made from the right fallopian tube, fimbria and ovary. The uterus was of about 6 weeks' size. Left sided fallopian tube and ovary visualized. Left fallopian tube was short, about 4 cm in length. Left ovary was normal. Moderate hemoperitoneum was noted, the source of bleeding being the posterior surface of the right adnexal mass. The adnexal mass was incised over its superior surface to reveal products of conception, right ovary and right fimbria within it. Right fallopian tube was delineated in the wall of the mass and salpingectomy was performed. The ovarian cyst was enucleated and right ovary was reconstructed. Peritoneal wash was given. Haemostasis was confirmed.Post operatively the patient's condition was stable and course was uneventful. She made an uneventful recovery.
Microscopy showed bits of fallopian tubal structure with blood clot in the lumina. Plenty of chorionic villi were seen embedded in the blood clot. One of the fallopian tubal bits showed an intraluminal polypoidal mass composed of glands and papillae embedded in loose stroma. The glands and papillae were lined by columnar non ciliated cells with eosinophilic cytoplasm and round nuclei with bland nuclear features. Focal pseudostratification of the columnar epithelium was seen. A focus of mucinous metaplasia was also seen. No nuclear atypia, mitotic activity or necrosis was seen. A diagnosis of Papillary Metaplastic Tumor of the fallopian tube was made.

Figure 1. Intraoperative findings: Uterus (white arrow), right ovarian cyst (yellow arrow), right fallopian tube (hollow arrows).

Figure 2. Scanner view of cross section of fallopian tube showing an intraluminal polypoidal mass composed of glands embedded in stroma. Also seen is a focus of mucinous metaplasia. (H&E x 50)

Figure 3. High power view of intraluminal fallopian tubal mass highlighting the glandular architecture. (H&E x 100)

Figure 4. High power view showing glands lined by columnar epithelial lining. No cellular atypia seen. (H&E x 400)

Figure 5. High power magnification of the focus showing mucinous metaplasia. (H&E x 100)

Figure 6. Classic chorionic villi & blood clots also seen in the lumen of the fallopian tube. (H&E x 100)


MPT may be seen as a small lesion in the lumen of the tube postpartum or with a tubal ectopic gestation. Histopathologically it shows papillae lined by stratified epithelium, with abundant, dense, eosinophilic cytoplasm, and nuclei which are bland or with mild atypia. The origin of MPT is debated. In the 1980s, a group led by Saffos et al first described MPT of the fallopian tube incidentally found in four cases of puerperal tubal ligation.[1] They also noticed a close association with pregnancy as well as a significant similarity with serous borderline ovarian tumors. Pang reported such a tumor in a 52-year-old patient, along with bilateral partially calcified chorionic villi [2] This raised the question whether the tumor was newly developed or was present in quiescent for years. Most authors believe it to be a low-grade borderline tumor, but a few believe that it is a reactive condition.[2,3,4,5,6] The immunoprofiles of MPT are positive for cytokeratin and epithelian membrane antigen, and negative for CEA.[5] Salazar et al. also reported positivity for cyclin D1, and hormone receptors.[7] D'Adda et al presented molecular data on MPT showing that it resembles atypical proliferative serous tumors, which are a subtype of borderline ovarian tumors.[8] Some workers have suggested that MPT might represent Arias-Stella reaction.[1,4] They believe it to be due to simultaneous action of estrogen causing proliferative and ciliogenic activity and progesterone causing secretory activity. Changes like variations in proportion and height of cells, stratification, papillary tuft formation, hyperplasia and metaplasia are known to occur in the epithelium of the fallopian tube in conditions like salpingitis, endometrial carcinoma, exposure to endo- or exogenous estrogen, or even without any cause.[1,3,4,5,6,9] Salazar et al. Suggest that since neoplastic condition has not been irrefutably demonstrated in MPT, it should be called fallopian metaplastic papillary polyp instead of MPT.[7]

The differential diagnoses of MPT are papillary tubal hyperplasia, papilloma,  serous borderline tumor of the fallopian tube or spread of such a tumor from an ovarian tumor. A papillary tubal hyperplasia differs from MPT in that it shows papillary tufting and small round clusters of bland epithelium often with psammona bodies.[10] A papilloma differs from MPT in that it maintains normal endosalpingeal cell types. A serous borderline tumor is very rare. It shows complex papillary formation lined by hobnail, ciliated, and mesothelium-like cells in stratification. Mitotic activity is uncommon, but focal nuclear atypia is seen.[6,11,12] In our case, an association with tubal ectopic pregnancy was seen. Curiously, the patient also showed features of old pelvic inflammatory disease, which had resulted in the formation of a tuboovarian mass. The fimbriae had been free, and permitted entry of ovum into the fallopian tube, which resulted in the ectopic pregnancy. It cannot be stated with certainty if the MPT was associated with the tubal ectopic gestation, or was present from the time of the chronic salpingitis.


MPT is a benign condition and its prognosis is good. Hence drastic surgical excisions should not be performed. Since it is a very rare condition, there are not enough cases to study and draw conclusions from. Hence all new cases should be published, so that molecular studies can be performed to determine the pathogenesis of the consition, and long term follow-up can be done to study the behavior of the condition.

  1. Saffos RO, Rhatigan RM, Scully RE. Metaplastic papillary tumor of the Fallopian tube: a distinctive lesion of pregnancy. Am J Clin Pathol. 1980;74:232–6.
  2. Pang LC. Hydrosalpinx due to asymptomatic bilateral tubal pregnancies associated with metaplastic papillary tumor of the Fallopian tube. South Med J. 1999;92:725–7.
  3. Starr AJ, Ruffolo EH, Shenoy BV, Marston BR. Primary carcinoma of the Fallopian tube: a surprise finding in a postpartum tubal ligation. Am J Obstet Gynecol. 1978;132:344–5.
  4. Keeney GL, Thrasher TV. Metaplastic papillary tumor of the Fallopian tube: a case report with ultrastructure. Int J Gynecol Pathol. 1988;7:86–92.
  5. Bartnik J, Powell WS, Moriber-Katz S, Amenta PS. Metaplastic papillary tumor of the Fallopian tube. Case report, immunohistochemical features, and review of the literature. Arch Pathol Lab Med.1989;113:545–7.
  6. Vang R, Wheeler JE. Diseases of the Fallopian tube and paratubal region. In: Kurman RJ, Ellenson LH, Ronnett BM, editors. Blaustein´s pathology of the female genital tract. 6th ed. New York: Springer; 2011. pp. 529–78.
  7. Salazar MF, Moscoso IE, Vázquez LT, García NLL, Abril PAE. Fallopian Metaplastic Papillary Tumour: An Atypical Transdifferentiation of the Tubal Epithelium? Journal ListJ Pathol Transl Medv.2015 Mar 49(2); PMC4367111. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4367111/
  8. D'Adda T, Pizzi S, Bottarelli L, Azzoni C, Manni S, Giordano G. Metaplastic papillary tumor of the salpinx: report of a case using microsatellite analysis. Int J Gynecol Pathol. 2011 Nov;30(6):532-5.
  9. Solomon AC, Chen PJ, LiVolsi VA. Pathologic quiz case: an incidental finding in the Fallopian tube. Fallopian tube, left, tubal ligation: metaplastic papillary tumor of Fallopian tube. Arch Pathol Lab Med. 2003;127:e363–4.
  10.  Feeley L, Quinn CM. Columnar cell lesions of the breast. Histopathology. 2008;52:11–9.
  11. Walker RA, Hanby A, Pinder SE, Thomas J, Ellis IO, National Coordinating Committee for Breast Pathology Research Subgroup Current issues in diagnostic breast pathology. J Clin Pathol. 2012;65:771–85. 
  12. DeLellis RA, Shin SJ, Treaba DO. Immunohistology of endocrine tumors. In: Dabbs DJ, editor. Diagnostic Immunohistochemistry. Theranostic and Genomic Applications. 3rd ed. Philadelphia: Saunders Elsevier; 2010. pp. 291–339.

Manjrekar V, Parulekar SV, Fernandes G, Shaikh S. Metaplastic Papillary Tumor Of The Fallopian Tube. JPGO 2016. Volume 3 Number 6. Available from: http://www.jpgo.org/2016/07/metaplastic-papillary-tumor-of.html

Extensive Parietal Abdominal Wall Endometriosis Post LSCS

Author Information

George S*, Fernandes G**, Rojekar A***, Gupta AS****
(* Fourth Year Resident, **** Professor, Department of Obstetrics and Gynecology; ** Associate Professor, *** Assistant Professor, Department of Pathology, Seth GS Medical College & KEM Hospital, Mumbai, India; )


A 39 year old G1P1L0 patient with previous one cesarean section (LSCS) presented with pain over abdominal scar site during menses and hardness along the scar. A 7x5 cm mass was present in the abdominal parietes. Fine needle aspiration cytology (FNAC) showed endometriosis. Exploration and wide excision of the endometriotic implants along with meshplasty was done. Histopathology report confirmed parietal endometriosis.


Endometriosis is the presence of endometrial glands and stroma outside the uterus, the prevalence of which is 3 to 10 % in reproductive aged women.[1, 2] Pelvis is the most common site but the ectopic endometrial tissue can be seen anywhere in the body. In surgeries, there is a possibility of direct transplantation of the endometrial glands or tissue which serves as an explanation for endometriosis found in abdominal or perineal scars.[1] The association of abdominal wall endometriosis following cesarean sections and hysterectomy was 57 % and 11% respectively.[3] Due to intrinsic abnormalities and defects in cellular immune mechanisms, refluxed and transplanted cells are probably resistant to apoptosis and macrophage mediated immune surveillance and clearance. This results in persistence of ectopic endometrial cells which can exhibit high local hormone production.[1]

Case Report

A 39 year old G1P1L0 patient married since 5 years with previous one LSCS presented with pain over scar site, hardness along the scar site and dysmenorrhea since 1 year. The pain over the scar site was present only during menses. There was no associated fever, vomiting or irregular menstrual cycles. Two years back patient underwent an emergency preterm LSCS in view of bleeding placenta previa. Surgical notes were reviewed and a right lateral tear in the lower segment extending towards the body of uterus was noted. There was no previous history of any other medical, surgical illness or other operative procedures. 
The patient was conscious, oriented and stable. Local examination of the abdomen showed a Pfannenstiel scar.  On palpation a large mass was present in the abdominal parietes about 7 cm in width and 5 cm in height. A 3x3 cm superficial nodule was felt at right angle of the scar. There was no guarding or rigidity present but mild tenderness was present over the scar. Cervix and vagina were healthy on speculum examination and on the uterus was retroverted, normal in size and mobile on bimanual examination. No nodularity or cervical motion tenderness was felt. Serological and biochemical investigations done were all within normal limits.
Multi Detector Computed Tomography (MDCT)  showed two ill defined enhancing soft tissue density lesions in the rectus abdominis muscle in the anterior abdominal wall. The size was approximately 5.0x3.9x2.7 cm on the right side near the right superior iliac spine and 7.0x4.2x2.6 cm in the anterior aspect in the intermuscular plane. It was extending upto the pubic symphysis. It was suggestive of endometriotic deposit/ inflammatory lesions (figure 1). Patient presented to us with these reports and patient was given medical treatment in the form of parenteral Leuprolide ( GnRH antagonist ) 3.75 mg intra muscular for three cycles. Patient was comfortable during these three months period. But patient again started complaining of pain of the same intensity at these same sites after the treatment three cycles.
Therefore along with general surgeons, exploration and excision of the endometriotic implants was performed. On exploration, intraoperative findings showed a localized hard indurated mass of size 3x3 cm at right angle of the scar (figure 2) and one large extensive indurated mass of size 10x4x1 cm was felt extending from umbilicus involving the rectus sheath and rectus muscle up to the pubic symphysis. 
A wide excision of the mass along with free margins was done and the large (approximately 10x5 cm) defect of the rectus sheath was repaired with meshplasty (figure 3).
Around 0.5x1 cm lesion could not be excised since it seemed adherent to the bladder below. Superficial cauterization was done. A polypropylene mesh of 15x15 cm was sutured to the defect. Subcutaneous drains were kept bilaterally and the abdomen was closed. Drain output after the first 24 hours was 200 ml which then gradually decreased and stopped and hence on post operative day 5  the drain was removed. 
On histopathological examination, sections from the subcutaneous tissue as well as from the skeletal muscle showed multiple foci of endometriotic tissue with florid fibrosis and lymphocytic infiltrate surrounding the foci. The endometriotic foci were composed of endometrial glands and stroma. Hemosiderin laden macrophages clusters were also seen. The overall impression was of endometriosis with extensive fibrosis and inflammatory pathology (figures 4 and 5).

Figure 1. CT plate with black arrows pointing at endometriotic implants.

Figure 2. Image showing the excised part of subcutaneous endometriotic implant in right angle of Pfannenstiel scar.

Figure 3. Image showing the partially excised part of the large endometriotic implant.

Figure 4. Microscopic image of rectus sheath showing foci of endometrial gland (black arrow) and stroma (black star) surrounded by fibrosis (white arrow) [H&E x 100]

Figure 5. High Power view of endometrial gland and stroma (white arrow) surrounded by inflammatory cells within the skeletal muscle tissue (black arrow) [H&E x 400]

Patient was reassured postoperatively and decision was taken for three monthly intramuscular  depo provera (depot medroxy progesterone) injections of 50 mg each, which would provide reduction and suppression of the remaining mass and also have contraceptive effect as she did not desire future childbearing.


A treating physician should know that such rare presentations of endometriosis do exist and abdominal wall endometriosis (AWE) should be included in the differential diagnosis.[2] Also the diagnosis can be challenging if a high index of suspicion doesn’t exist. The correct diagnosis can often be missed.[4] Ultrasound, magnetic resonance imaging and CT can be used in the diagnosis.
Nowadays, ultrasound-guided percutaneous cryoablation seems to be promising, but wide surgical excision till date proves to be the mainstay of treatment.[3,4] In surgical treatment for endometriosis, the objectives are to excise and remove all visible disease. To prevent recurrence and proliferation of endometriosis, complete excision of the mass along with a clear margin is very important.[5] In cases where the aponeurosis is involved, we can perform a wide resection of the lesion along with free margins and if the defect is quite large, polypropylene mesh closure is done. The outcome, post surgery is successful.[4]
When the primary objective is relief from pain, post operative medical treatment can be used, particularly in women with residual disease that could not be completely excised as is done in our case as it would have involved excising a part of the urinary bladder.
Our aim is to raise the awareness of extensive endometriosis and the importance of complete excision of the lesion  and adequate closure of the abdominal wall, if needed, with meshplasty.

  1. Fritz MA, Speroff L. Endometriosis. In Clinical Gynecologic Endocrinology and Infertility. 8th ed. Philadelphia,USA: Lippincott Williams and Wilkins; 2011; pp. 1221-48.
  2. Ecker AM, Donnellan NM, Shepherd JP, Lee TTM. Abdominal wall endometriosis: 12 years of experience at a large academic institution. Am J Obstet Gynecol. 2014;211:363 e1-5.
  3. Horton JD, Dezee KJ, Ahnfeldt EP, Wagner M. Abdominal wall endometriosis: a surgeon's perspective and review of 445 cases. Am J Surg. 2008;196(2):207-12.
  4. Vaz-de-Macedo C, Gomes-da-Costa A, Mendes S, Barata S, Alho C, Jorge CC, et al. Abdominal Wall Endometriosis Excision with Mesh Closure - Report of Two Cases. Surg Technol Int. 2016;XXVIII.pii: sti28/718.
  5. Kang J, Baek JH, Lee WS, Cho TH, Lee JN, Lee WK, et al. Clinical manifestations of abdominal wall endometriosis: a single center experience. Arch Gynecol Obstet. 2013;287(2):301-5.

George S, Fernandes G, Rojekar A, Gupta AS. Extensive Parietal Abdominal Wall Endometriosis Post LSCS. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/extensive-parietal-abdominal-wall.html

Postpartum Tuberculous Pyoperitoneum

Author Information

Thakur HS*, Madhva Prasad S*, Gupta AS**
(*Assistant Professor, ** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India)


Tuberculosis peritonitis appearing in postpartum period is a rare entity. A case successfully managed with surgical intervention is presented here. 


WHO statistics, identifies tuberculosis to account for 15-34% of indirect causes of obstetric mortality and this is associated with a six-fold increase in perinatal mortality.[1] While lymphadenitis is the most common extrapulmonary site of tuberculosis, tuberculous peritonitis is a rare entity. A multiparous woman presented in postpartum day 17 with acute abdominal distension. Exploratory laparotomy showed pyoperitoneum with tubo-ovarian abscess.

Case Report

A 32 year old married woman para 4 living 4 presented on 17th day following normal delivery to the emergency department, with complaints of abdominal pain over the past 2 days. She had an indwelling Foley’s catheter on admission. Urosac bag had 200 ml of clear urine. This abdominal pain was generalized, and gradually increasing in severity, not responding to antispasmodic medication given by private practitioner. Patient also complained of gradually progressive breathlessness and giddiness. There were no complaints of vaginal bleeding, vomiting, constipation, urinary complaints or fever. There was no problem in breastfeeding. There was no history of any major medical or surgical illnesses or past history of Kochs’. She had delivered a male child of 2.6 kg uneventfully. She was HIV negative.  
On examination, patient was pale, afebrile, tachypneic with a respiratory rate of 40 cycles per minute ,tachycardia  with pulse of 120 beats/ minute, low volume,  and a  blood pressure 90 /60 mm Hg. Heart sounds were normal and no adventitious sounds were heard on respiratory auscultation.  Abdomen was distended with free fluid, there was generalized tenderness, guarding and mild rigidity. Speculum examination did not show any foul smelling discharge or lochia. Bilateral forniceal fullness was present due to free fluid, and uterine size could not be estimated. Colpopuncture was done which showed greenish, purulent, foul smelling discharge. This was sent for aerobic culture sensitivity and TB PCR.
An ultrasonography scan with the patient showed two mixed echogenic lesions in the right adnexa of 10x9 cm and 4x4 cm size respectively. Right ovary was not seen separately. Sonography was suggestive of complex/hemorrhagic cysts. Uterus was mildly bulky. Moderate ascites with internal echoes was present
Patient was admitted and started on intravenous Piperacillin Tazobactum, Amikacin  Metronidazole,  crystalloids and colloids. Chest x ray was normal. Erect abdominal x-ray showed dilated bowel loops with no obstruction.  Plain CT abdomen and pelvis confirmed presence of hemorrhagic ovarian cyst with feature of rupture. 

Figure 1. CT scan image with arrow pointing towards tubo-ovarian abscess

One unit of blood transfusion was started for hemoglobin 8.6 g/dl. Hypokalemia (serum potassium level of 2.9 mEq/lit) was corrected by parenteral infusion of 20 mEq/litre of potassium chloride. Rest of biochemical parameters were in normal range. Exploratory laparotomy was done along with the surgeon. Upon opening parietal peritoneum approximately 1.5 L of foul smelling pus was drained and sample sent for examination. Uterus was bulky and left sided tube and ovary was normal. Right sided tubo-ovarian mass (T-O mass) around 10x6x5 cm was noted extending to the right pelvic wall, which was discharging pus (figure 2) Thick omental mass was stuck between tubo-ovarian mass and ascending colon. Small bowel loops were matted, (figure 3) stuck but easily separable. Entire length of bowel was traced and no sign of obstruction or injury was seen. The T-O mass was excised by applying clamp between base of the mass and right uterine cornua; base was transfixed with polydiaxanone No.1 suture. Partial omentectomy was done and by blunt and sharp dissection, the omental mass was released from the ascending colon. Specimen was sent for histopathological examination. Thorough intraperitoneal lavage was done with normal saline. Hemostasis was achieved and peritoneal drains were placed – one in Morrison’s pouch and other in pouch of Douglas. In the post operative period, intra-peritoneal drain output gradually reduced from 400 ml on day 1 to 50 ml by day 5; following which it was removed. The drainage was sero-sanguineous in nature.  Serum electrolytes were monitored twice a day and hypokalemia was corrected with slow intravenous infusion of KCl (20 mEq/litre in 500 ml of DNS). 

Figure 2.  Intraoperative image showing pus draining from tubo-ovarian abscess

Figure 3. Intraoperative image showing matted bowel (arrow).

Vaginal swab culture showed no growth. Pus showed gram negative bacilli and gram positive cocci in pairs, but did not grow any micro-organism on aerobic culture. Pus did not show acid fast bacilli on Ziehl-Neelsen staining. TB-PCR did not show evidence of tuberculosis. Final culture also did not show mycobaterial growth. Pus showed dense inflammation composed of predominantly polymorphs, and few lymphocytes with necrosis. It did not show any atypical cells. Adenosine deaminase was 81.8u/l. (cut off value of ADA is 24 u/l)
Gastrointestinal decompression was maintained by indwelling Ryle’s tube for 5 days. It initially drained 320 ml of bilious aspirate in the first postoperative day which gradually reduced to 100ml on day 5; following which it was removed. Patient recovered from paralytic ileus by day 8 and then she was started on oral liquids. Category I anti-tuberculous therapy was initiated in consultation with local RNTCP center. Upon starting oral diet, patient had 8-10 episodes of diarrhoea; all antibiotics were stopped and stool examination was done. It showed no abnormalities. Lactobacillus suspensions were given orally twice a day, and she was kept hydrated with oral rehydration solution. The patient recovered on day 13.

On histopathology ovarian and fallopian tubal structures showed dense inflammatory infiltrates consisting of neutrophils, plasma cells, lymphocytes and macrophages; with caseous necrosis. Histopathology confirmed tuberculous etiology that was acute on chronic presentation.  Patient was advised to continue breastfeeding. No specific anti-tubercular therapy was advised for the neonate. Patient was comfortable; hence discharged on postoperative day 15. Patient followed up on day 24, was compliant to treatment; sutures were removed and wound was healthy.     


As per WHO statistics, approximately 5 lakh women died from TB in 2014. TB accounts for 15-34% of indirect causes of obstetric mortality and is associated with a six-fold increase in perinatal mortality.[1]  While lymphadenitis is the most common extrapulmonary site of tuberculosis, tuberculous peritonitis is a rare entity.[2]  
Our patient probably had latent tuberculosis which had an exacerbation during the postpartum period. Due to increasing levels of progesterone in pregnancy, a Th2-type immune response is favored and the Th1 helper cells are suppressed. This change is reversed in the postpartum period. It is now know that women in early postpartal period are twice at risk to develop tuberculosis than pregnant women.  This is attributed to the “immune reconstitution” occurring in this period, which can result in increased susceptibility to fresh infection and reactivation of tuberculosis.[3,4,5] The common manifestation of tuberculous peritonitis are insidious fever, anorexia, abdominal pain and exudative ascites.[6] However, in our case, the patient presented with abdominal distension and shock. Such an acute and rapid presentation of pelvic tuberculosis with shock in the puerperal period, necessitating operative intervention has been described sporadically.[7]
The peritoneal fluid analysis showed neutrophilic predominance in our patient. Though lymphocytosis is the most common picture, an initial phase of neutrophilia is described as common.[2] Peritoneal fluid ADA levels were high in this patient (81.8u/l). Cut off value of ADA which suggests tuberculous etiology is 24 u/l. Sensitivity, positive predictive value, specificity and accuracy of ADA for diagnosis of tuberculous peritonitis were 87.5%, 95.45%,  83.33% and 86.67% respectively, as reported by Ali et al. However it has a poor negative predictive value of 62.5%.[8,9] A 3 year study was conducted on 300 patients by Rana et al to evaluate the role of various laboratory investigations in the diagnosis of abdominal tuberculosis. It was concluded that though PCR is a highly specific test, but due to high costs, it has limited value in clinical practice.[10] In the same study, FNAC under ultrasonographic guidance resulted in 81% yield and was concluded to be a reliable and cost effective alternative. The gold standard for diagnosis of abdominal tuberculosis is a combined detection on tissue biopsy examination and identification of acid fast bacilli on staining in tissue fluids and tissue cuts. In our patient the acid fast bacilli was not detected in peritoneal fluid, or in the tubo-ovarian abscess. Though tuberculosis is recognized more commonly in the postpartum period, presentation in early pregnancy has also been described.[11] Our patient was started on category one anti-tuberculous therapy in the immediate postoperative period, though patient was breastfeeding. Anti-tuberculous therapy appears to be safe during lactation.[5]  With the introduction of second line therapy and many newer drugs, there has been a plea for increased intake of pregnant and postpartum patients into anti-tubercular drug trials.[12]


Tuberculous peritonitis with shock in a postpartum period is a rare presentation. But high index of suspicion and prompt operative management with early initiation of antituberculous therapy can give good patient outcome.

  1. WHO: Tuberculosis in women. WHO fact sheet. November 2015. Available from:         http://www.who.int/tb/publications/tb_women_factsheet_251013.pdf . 
  2. O’ Brien RJ, Raviglione MC.  Tuberculosis.In: Longo LD, Kasper DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J. eds. Harrison’s Principles of Internal Medicine.New York. 18th edn. McGraw Hill 2014. Pg no 1340-3. 
  3. Mathad JS, Gupta A. Tuberculosis in pregnant and postpartum women: epidemiology, management, and research gaps. Clin Infect Dis. 2012 Dec; 55(11):1532–49.
  4. Zenner D, Kruijshaar ME, Andrews N, Abubakar I. Risk of tuberculosis in pregnancy: a national, primary care-based cohort and self-controlled case series study. Am J Respir Crit Care Med. 2012 April;185(7):779–84.
  5. Ormerod P. Tuberculosis in pregnancy and the puerperium. Thorax. 2001 Jun; 56(6):494–9. 
  6. Dülger AC, Karadaş S, Mete R, Türkdoğan MK, Demirkıran D, Gültepe B. Analysis of cases with tuberculous peritonitis: a single-center experience. Turk J Gastroenterol. 2014 Feb; 25(1):72–8.
  7. Agarwal M, Das A, Singh AS. Pelvic tuberculosis and shock in the puerperium. South Med J. 2011 May; 104(5):358–9.
  8. Mathad JS, Bhosale R, Sangar V, Mave V, Gupte N, Kanade S, et al. Pregnancy differentially impacts performance of latent tuberculosis diagnostics in a high-burden setting. PLoS One [Internet]. 2014 Jan [cited 2016 Feb 9];9(3):e92308.Available from :  http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3962385/ 
  9. Ali N, Nath NC, Parvin R, Rahman A, Bhuiyan TM, Rahman M, et al. Role of ascitic fluid adenosine deaminase (ADA) and serum CA-125 in the diagnosis of tuberculous peritonitis. Bangladesh Med Res Counc Bull. 2014 Dec;40(3):89–91
  10. Rana S, Farooqui MR, Rana S, Anees A, Ahmad Z, Jairajpuri ZS. The role of laboratory investigations in evaluating abdominal tuberculosis. J Family Community Med. Jan 2015;22(3):152–7.
  11. Lahbabi M, Brini J, Massaoudi K. Tuberculous peritonitis in pregnancy: a case report. J Med Case Rep. 2014 Jan;8:3
  12. Gupta A, Mathad JS, Abdel-Rahman SM, Albano JD, Botgros R, Brown V. Toward Earlier Inclusion of Pregnant and Postpartum Women in Tuberculosis Drug Trials: Consensus Statements From an International Expert Panel. Clin Infect Dis. 2016 Mar 15;62(6):761-9. doi: 10.1093/cid/civ991. Epub 2015 Dec 9. Availale from : http://www.ncbi.nlm.nih.gov/pubmed/26658057 

Thakur HS, Madhva Prasad S, Gupta AS. Postpartum Tuberculous Pyoperitoneum. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/postpartum-tuberculous-pyoperitoneum.html

Rectus Sheath Leiomyoma

Author Information

Parulekar SV
(Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)


Anterior abdominal wall is a rare site for the development of a leiomyoma. It may develop there primarily, or may be the result of implantation during myomectomy. A case of rectus sheath leiomyoma which had been wrongly diagnosed as an ovarian tumor is presented.


A leiomyomas is a smooth muscle cell tumor. The uterus is a common site of development of a leiomyoma in a woman.[1] Extrauterine leiomyomas are known to develop in the round ligament, uteroovarian ligament, vagina, and broad ligament. A pedunculated subserosal leiomyoma may get adherent to bowel or omentum, and become a wandering leiomyoma when its pedicle undergoes ischemic necrosis and it derives its blood supply from the structure to which it adherent. Leiomyomas may also develop in the bowel wall. Muscular tumors arising from the abdominal wall tend to be rhabdomyomas, arising from the skeletal muscle. Development of a leiomyoma in the anterior abdominal wall is difficult to explain. A case of rectus sheath leiomyoma which had been wrongly diagnosed as an ovarian tumor is presented.

Case Report

A 28 year old nulliparous woman, married for two years, presented with a complaint of a lump in abdomen for one month. Her menstrual history was normal. There was no history of abdominal pain, pressure effects on bowel, bladder or rectum. Her medical and surgical history was not contributory. Her general and systemic examination revealed no abnormality. Abdominal examination showed a rounded lump measuring 10 cm in diameter on the left side of the midline, below the level of the umbilicus. Its mobility was restricted. A straight leg raising test showed the mass to be intraabdominal. On bimanual pelvic examination there was a 10 cm diameter mass with a smooth surface above and to the left of the uterus. Its lower limit could be reached. Its mobility was restricted. Ultrasonography done prior to presenting to us showed it to be a solid left ovarian tumor. Her CA 125 level was 14 U/mL. Her investigations for fitness for anesthesia showed no abnormality. An exploratory laparotomy was done through an infraumbilical midline vertical incision. It showed a 10 cm diameter mass in the left rectus sheath, attached to the left rectus abdominis muscle and the rectus sheath by loose connective tissue. It was dissected easily and removed completely. Its feeding vessel was on its posterior aspect, branching from the inferior epigastric artery. It was clamped, cut and ligated. The anterior parietal peritoneum was not invaded by the mass. All intraperitoneal structures including the uterus were normal. An extraperitoneal drain was placed in the cavity left behind by the removal of the mass. The abdominal was closed in layers. The patient made an uneventful recovery. Histopathological examination showed it to be a leiomyoma.

Figure 1. Left rectus sheath leiomyoma being dissected out.

Figure 2. Findings after removal of the leiomyoma: cavity left behind after removal of the leiomyoma yellow arrows, edges of posterior rectus sheath and parietal peritoneum (white arrows), peritoneal cavity with normal intraperitoneal structures (pink arrows).


A leiomyomas is a common benign uterine tumor seen in women in the reproductive age group. Extrauterine leiomyomas are uncommon. Most of the extrauterine tumors are located close to the uterus, as in the round ligament, uteoovarian ligament, broad ligament, and vagina.[2,3,4,5,6] A subserous pedunculated leiomyoma may become a wandering leiomyoma when it gets attached to the bowel or omentum and its pedicle undergoes necrosis. Abdominal wall is composed of skeletal muscle, and connective tissue – loose or in aponeurotic form. Muscular tumor of the abdominal wall is expected to be a rhabdomyoma rather than a leiomyoma. Still leiomyomas have been reported in the anterior abdominal wall layers.[7,8,9,10,11,12,13] Though some of them may be due to implantation during open or laparoscopic myomectomies,[4,14.] most of them develop there primarily. These are believed to develop from smooth muscle cells in the walls of blood vessels in the abdominal wall.[15] A leiomyoma incarcerated in an anterior abdominal wall hernia differs from those described before in that it has an abdominal wall defect associated with it and it often has its connection with the uterus.[16] They tend to be asymptomatic, since they cannot cause usual symptoms of a uterine leiomyoma – abnormal bleeding, pain and pressure effects. Usually attention is drawn to them when they become large, or during an examination for some unrelated symptom. Their location in the abdominal wall can be diagnosed clinically by examining the woman in lying down position and then with her raising her extended lower limbs. An abdominal wall tumor becomes more prominent during leg-raising, while an intraabdominal tumor becomes obscured. The diagnosis can be confirmed by imaging techniques, like ultrasonography, and computed tomography. The excision of the mass is usually easy and complete, leaving little residual effect behind, if any. Abdominal wall leiomyoma needs to be differentiated from a fibroma, lipoma, amd a sarcoma. A biopsy may be obtained when a malignancy is suspected.

In the case presented, the straight leg raising test was false negative. Unfortunately the diagnosis was missed on ultrasonography too, and a wrong diagnosis of an ovarian tumor was made. Had a correct diagnosis been preoperatively, a laparotomy could have been avoided, and the tumor could have been excised by opening the abdominal wall only up to the cavity of the left rectus abdominis. 


A leiomyoma, although commonly seen in uterus or gastrointestinal tract, can develop de novo in areas devoid of myomatous tissues or obvious smooth muscle cells. Whether smooth muscle cells in vessels of anterior abdominal wall react to extraneous hormonal stimulation to form a de novo leiomyoma is yet to be established. This condition has to be kept in mind while completing the list of differential diagnosis of anterior abdominal wall tumors.

  1. Kjerulff KH, Langenberg P, Seidman JD, Stolley PD, Guzinsky GM. Uterine leiomyomas. Racial differences in severity, symptoms and age at diagnosis. J Reprod Med. 1996;41(7):483–90.
  2. Stewart EA. Uterine fibroids. Review Lancet. 2001;357(9252):293–8. doi: 10.1016/S0140-6736(00)03622-9. [PubMed] [Cross Ref]
  3. Seims AD, Lube MW. Incarceration of a sessile uterine fibroid in an umbilical hernia during pregnancy. Hernia. [PubMed]
  4. Moon HS, Koo JS, Park SH, Park GS, Choi jg, Kim SG. Parasitic leiomyoma in the abdominal wall after laparoscopic myomectomy. Fertil Steril. 2008;90(4)
  5. Muffly T, Vadlamani I, Weed JC. Massive leiomyma of the broad ligament. Obstet Gynecol. 2007;109(2 pt 2):563–5.
  6. Ostrzenski A. Uterine leiomyoma particle growing in an abdominal wall incision after laparoscopic retrieval. Obstet Gynecol. 1997;89(5 pt 2):853–4.
  7. Igberase GO, Mabiaku OT, Ebeigbe PN, Abedi HO. Solitary anterior abdominal wall leiomyoma in a 31-year-old multipara woman: A case report. Cases J. 2009;2(1):113.
  8. Goyal N, Khurana N. Leiomyoma of rectus sheath: An uncommon entity: Report of two cases. Indian J Pathol Microbiol. 2010;53(3):591–92.
  9. Hamed A. Al-Wadaani. Anterior Abdominal Wall Leiomyoma Arising De Novo in a Perimenopausal Woman. Oman Med J. 2012 Jul; 27(4): 323–325.
  10. Midya M, Dewanda NK. Primary Anterior Abdominal Wall Leiomyoma- A Diagnostic Enigma. J Clin Diagn Res. 2014 Oct; 8(10): NJ01–NJ02.
  11. Narasimha Rao KL, Nagendra Babu SSR, Arjun Prakash J. Sri ram G, Mohan rao N. Solitary Anterior Abdominal Wall Leiomyoma Arising from Tubectomy Scar. Journal of Dental and Medical Sciences 2014;13:04-06.
  12. D’souza C, Bhat S, Purushothaman, Dhanej. De novo growth of leiomyoma from rectus sheath: A rare presentation. Ann Trop Med Public Health 2012;5:390-2
  13. Cho JY, Woo JY, Hong H, Yang l, Lee Y, Hwang J, et al. Anterior Abdominal Wall Leiomyoma Arising De Novo in a Fertile Women: A Case Report. J Korean Soc Radiol 2016;74(1):71-74.
  14. Sreelatha S, Kumar Ashok, Nayak Vedavathy, Punneshetty Sahana, Hanji Nirmala. A rare case of primary parasitic leiomyoma. Int J Reprod Contracept Obstet Gynecol. 2013 Sep;2(3):422–24.
  15. Al-Wadaani Hamed A. Anterior abdominal wall leiomyoma arising de novo in a perimenopausal woman. Oman Med J. 2012;27(4):323–25.
  16. Seims AD, Lube MW. Incarceration of a sessile uterine fibroid in an umbilical hernia during pregnancy. Hernia. 2009 Jun;13(3):309-11.

Parulekar SV. Rectus Sheath Leiomyoma. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/rectus-sheath-leiomyoma.html

Obstetric Care In A Case Of Hemophilia A

Author Information

*Anuranjani L, **Shah A, ***Chauhan AR..
(*Fourth Year Resident, ** Second Year Resident, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)


Hemophilia type A is an inherited bleeding condition which disrupts the hemostasis of pregnancy and has diverse clinical presentation, from hematoma to postpartum hemorrhage. Even though females are carriers of the disease, the severity of complications is inversely related to the level of Factor VIII. We report the management of pregnancy in a known case of hemophilia A carrier who had an uncomplicated pregnancy and vaginal delivery at term utilizing recombinant factor VIII concentrate. The focus of this case report is management of an obstetric case with bleeding disorder.


The hemostatic alterations during antenatal and postnatal phase result in a delicate state of hemostasis which if superimposed with coagulopathies can have cataclysmic outcome. Hemophilia A or classic hemophilia is an X- linked genetic disorder caused by deficiency of clotting Factor VIII (F VIII).[1] Females are typically carriers due to X-linked inheritance and hence female neonates born to them have 50% chance of being carriers of the disease while male neonates have 50% chance of being affected.[1] A 2014 survey by World Federation of Hemophilia states that India has 14,450 cases of Hemophilia A (among population of 1,236,344,631).  
The normal level of F VIII required for hemostasis is > 50 IU/dL. It is estimated that 1 per 1,00,000 women are symptomatic hemophilia A carriers (F VIII < 30 IU/dL) while severe hemophilia A (F VIII < 1 IU/dL) is an extremely rare event.[1] The information regarding the management and outcomes of hemophilia A in pregnancy is limited due to the rarity of the disease.[2] Coagulopathies have an emotional and economic burden, and if inappropriately managed can have disastrous outcomes. We report our experience of a carrier where individualized obstetrical management along with multidisciplinary approach resulted in successful outcome of pregnancy.

Case Report

A 24 year old primigravida, known case of hemophilia A presented at 9 weeks’ gestation to us.. She gave history of development of left labial hematoma due to fall one and half years ago for which she underwent surgical evacuation. Repetitive formation of hematoma at the same site during a span of 26 days was managed by re-evacuation, cauterization and re-suturing along with transfusion of 3 units of blood and 1 of fresh frozen plasma. This prompted further investigation after which she was diagnosed as a carrier of hemophilia A and was given Factor VIII. However no family member could be indexed. She was not on any treatment since then. 
In the present pregnancy, at 9 weeks of gestation, when patient presented to us, she was explained fetal prognosis and advised prenatal detection for hemophilia A but she refused the same and was lost to follow up. She presented for ANC registration at 31 weeks by dates; growth corresponded with her ultrasonography scan. Her physical examination showed no evidence of ecchymosis, petechiae, purpura or vaginal bleeding. Hematology reference was done and no active management was advised. She attended regular ANC clinics and had an uneventful antenatal period. 
She was admitted at 38 weeks of gestation for safe confinement. On admission patient had stable vital parameters and no pallor. On abdominal examination uterus was full term, relaxed, with cephalic presentation and regular fetal heart sounds. Local examination revealed a 3 x 2 cm left labial non-tender scar tissue with no discharge or redness in the region of the previous hematoma. On vaginal examination os was closed, firm, posterior in position and uneffaced.  
All routine investigations were done along with ultrasonography, Doppler and biweekly non- stress test which were within normal limits. Coagulation profile was done where prothrombin time (PT) was 13.4, activated partial thromboplastin time (aPTT) was 30.1, von Willebrand factor was 117 % (normal: 50- 150 %) and factor VIII level was 26% (normal: 50 – 150 % normal pooled plasma) which was below the required values of 50% or 80% for vaginal and cesarean delivery respectively.[3] Screening for Factor VIII inhibitors was negative.
Hematology reference for hemophilia A was done and plan of management charted out for vaginal delivery and cesarean section along with anesthesiologist and neonatologist. Based on prior events, availability of Factor VIII and this being a high risk pregnancy, planned induction of labor was undertaken at 39 weeks of gestation. 1000 IU of Factor VIII was given intravenously just prior to induction and dinoprostone gel was instilled after taking a valid, well informed consent. Due to an unfavorable Bishop’s score dinoprostone gel was re-instilled after 6 hours. Hematologist opinion was re-sought and Factor VIII 1000 IU was repeated during the end of 1st stage of labor. Additional Factor VIII and blood was kept in reserve during the intrapartum and postpartum period anticipating blood loss based on her prior history.
Approximately 14 hours after second instillation of dinoprostone gel, patient delivered a 2.91 kg female child with Apgar of 9/10. Episiotomy was given; 3rd stage was managed actively and there was no postpartum hemorrhage. There was no episiotomy extension or other perineal tears. Following delivery, 500 IU of Factor VIII was given intravenously twice daily for 3 days along with routine antibiotics. Patient complained of moderately increased bleeding on postpartum day 4. There was no evidence of PPH and patient was given 1000 IU of Factor VIII after which no similar complaints were noted. Patient and baby were discharged on post-delivery day 7 with a healthy episiotomy wound. Patient was advised to use LNG IUS at 6 weeks postpartum. The baby was not investigated at birth for Factor VIII levels; however follow up with the pediatrician and hematologist was advised especially as the neonate was a female child.


Hemophilia A disrupts the finely tuned hemostasis of pregnancy and can result in devastating consequences for both mother and neonate. The paucity of cases has made management of a pregnant woman with hemophilia challenging. There is progressive increase in the level of coagulation factors like Factor VIII during pregnancy, but these levels do not increase before second trimester and hence patients are at risk of hemorrhage. Levels of Factor VIII reach a peak during 29 to 35 weeks, normal level being > 50 IU/dL; hence the factor level should be tested at week 28 and again at 34 – 36 weeks.[3] Females are carriers of the disease and can become symptomatic in cases when: (a) female is homozygous hemophilia carrier and male is affected hemophilic (b) hemizygous X chromosome in Turner syndrome or (c) lyonization of Factor VIII alleles.[1]
Complications in pregnancy include first trimester bleeding, spontaneous miscarriage, antepartum hemorrhage, perineal hematomas and both primary and secondary postpartum hemorrhage and disseminated intravascular coagulopathy.[3]  
Successful management of hemophilia in pregnancy requires that the delivery be performed by the least traumatic method. Even though the mode of delivery is debatable, normal vaginal delivery is recommended. There is consensus that vacuum extraction should not be done; use of forceps, scalp electrodes and fetal blood sampling should also be avoided. Cesarean section (CS) is not indicated for mild to moderate cases; however some authors recommend CS in carriers with severe hemophilia with a potentially hemophiliac boy fetus.[3] CS increases the risk of bleeding and need for transfusion and does not eliminate the risk of cranial hemorrhage. Active management of the third stage should be done to prevent postpartum hemorrhage. Mode of delivery should thus be determined on obstetrical grounds.
Among the 41 cases of hemophilia A carriers studied by Chi and Kadir, mean pregnancy and third-trimester FVIII levels in carriers was 46 IU/dL and 121 IU/dL respectively.[4] Studies show that the incidence of primary and secondary PPH is 22% and 11% respectively in hemophilia carriers as compared with 5- 8% and 0.8% respectively in the general population. [2] The risk of PPH increases if FVIII level is < 50 IU/dL and this risk persists up to 3 - 5 weeks postpartum.[2] 
Chances of PPH can be reduced with the use of replacement therapy, altering mode of delivery, minimal trauma to the perineal region and measures to avoid uterine atony. The risk of PPH depends upon factor levels, thus hemostatic replacement is essential. Prophylactic therapy is given to raise FVIII levels to > 50IU/dL for vaginal delivery and ≥ 70 IU/dL for CS.[5] Tranexamic acid and desmopressin can be used in carriers with borderline levels. Low molecular weight heparin is not recommended for thromboprophylaxis.[6] Epidural analgesia can be given if factor levels are > 50 IU/dL.[5] The factor levels should be monitored in the postpartum period for 3 - 4 days after normal delivery and for 7 days following cesarean section. Tranexamic acid, oral contraceptive pills or even Levonorgestrel IUS can be used for late postpartum bleeding.[7]
Neonatal management includes careful observation for signs of bleeding, injection vitamin K and testing of cord blood; however prophylactic factor replacement should not be done due to risk of inhibitor development.[7] Being carriers, female infants appear to be at low risk of bleeding during the neonatal period. Incidence of neonatal cranial hemorrhage is found to be maximum (64%) following vacuum delivery as compared to 3% with vaginal delivery and 15% with CS.[8] 
Another facet in management is prenatal diagnosis of haemophilia. The armamentarium includes techniques like chorionic villus sampling (11-14weeks), ultrasound examination, amniocentesis (> 15 weeks) or by non-invasive technique to detect free fetal DNA (ffDNA) in the maternal circulation (from the 5th week of gestation), or real- time duplex PCR identification of Y-chromosome specific sequences, between 8 – 12 weeks of gestation.[9, 3] Cordocentesis (> 18 weeks) for factor VIII assay is an alternative in India where linkage analysis is not possible or molecular markers for linkage are uninformative or inversion mutation is absent or in sporadic hemophilia families.[10] 
As per the UK HCDO (Hemophilia Center Doctors’ Organization) guidelines, vaccination against Hepatitis B is recommended but should be administered subcutaneously to prevent hematoma formation at the injection site.[11] Studies have shown that desmopressin which increases plasma levels of Factor VIII and vWF, can be given IV, subcutaneously or intranasally even in first and second trimester for treating mild and moderate cases. It has negligible or no oxytoxic effect, does not pass into breast milk in significant amounts, and can be safely used in pregnancy, delivery and postpartum[12].


Women with a family history of a bleeding disorder, menorrhagia, postoperative bleeding, bleeding post dental extraction, PPH or prolonged aPTT should be screened for hemophilia prenatally or antenatally. Prompt pre-conceptional counselling and genetic testing should be advised; there are various centres and federations in India for hemophilia which act as support groups. Most pregnancies in hemophilia A carriers will be uneventful and without bleeding complications, provided there is prudence and preparedness to tackle the risks at various stages of pregnancy. Replacement therapy with Factor VIII remains the mainstay of treatment. Successful management of these cases requires collaboration with hematologist, anaesthesiologist, neonatologist and geneticist. 

  1. Sharma V, Khalid A, Cohen AJ. Management of Pregnancy in a Patient with Severe Hemophilia Type A. Am J Perinatol Rep 2013;3(1):29–32.
  2. Kadir RA, Economides DL, Braithwaite J, Goldman E, Lee CA. The obstetric experience of carriers of hemophilia. Br J Obstet Gynaecol 1997;104(7):803–810.
  3. Kadir RA, Davies J, Winikoff R, Pollard D, Peyvandi F, Garagiola I et al. Pregnancy complications and obstetric care in women with inherited bleeding disorders. Hemophilia 2013;19(4):1–10.
  4. Chi C, Kadir RA. Management of women with inherited bleeding disorders in pregnancy. The Obstetrician and Gynaecologist 2007;9(1):27–33.
  5. Chi C, Lee CA, Shiltagh N, Khan A, Pollard D, Kadir RA. Pregnancy in carriers of hemophilia. Hemophilia 2008;14(1):56–64.
  6. Huq FY, Kadir RA. Management of pregnancy, labour and delivery in women with inherited bleeding disorders. Hemophilia 2011;17(1):20–30.
  7. Dunkley SM, Russell SJ, Rowell JA, Barnes CD, Baker RI, Sarson M et al. A consensus statement on the management of pregnancy and delivery in women who are carriers of or have bleeding disorders. Med J Aust 2009;191(8):460-463. 
  8. Ljung R, Lindgren AC, Petrini P, Tengborn L. Normal vaginal delivery is to be recommended for hemophilia carrier gravidae. Acta Paediatr 1994;83(6):609–611.
  9. Zadeh NM, Namin SA. Evaluation of an improved noninvasive fetal sex determination in hemophilia A patients. J Clin Diagn Res. 2015;9(7): GC01-4.
  10. Panigrahi I, Ahmed RP, Kannan M, Kabra M, Deka D, Saxena R. Cord blood analysis for prenatal diagnosis of thalassemia major and hemophilia A. Indian Pediatr. 2005;42(6): 577-81.
  11. Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat hemophilia and other hereditary bleeding disorders. A United Kingdom Hemophilia Center Doctors’ Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Hemophilia. 2008;14(4):671–84.
  12. Mannucci PM. Use of desmopressin (DDAVP) during early pregnancy in factor VIII-deficient women. Blood 2005;105(8):3382.

Anuranjani L, Shah A, Chauhan AR. Obstetric Care In A Case Of Hemophilia A. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/obstetric-care-in-case-of-hemophilia-a.html

Successful Laparoscopic Management Of a Giant Paraovarian Cyst In An Adolescent Female

Author Information

Shah NH*, Kale KG,** Paranjape SH**, Shah VN***
(* Director, ** Ex-Fellow, ***Anesthetist, Vardann Multispeciality Hospital & 3D laparoscopy center, Mumbai, India.)


Paraovarian cysts constitute 10-33% of all adnexal masses and are usually small and asymptomatic. Rarely, they may attain huge sizes and cause symptoms due to pressure effects. Giant paraovarian cysts have conventionally been managed by laparotomy and pure laparoscopic management is rarely reported in the literature. We present a rare case of a giant paraovarian cyst in a 16 year old girl managed by laparoscopic excision with preservation of adnexa.


Paraovarian cysts are adnexal masses usually derived from the mesothelium lining the peritoneum of broad ligament. They are also thought to be arising from remnants of paramesonephric duct (lined by ciliated columnar cells) or rarely, mesonephric duct (lined by flattened cells).[1, 2] They account for about 10-33% of all adnexal masses and are usually small and asymptomatic.[1, 2] Only occasionally do they become large enough to cause pressure symptoms or undergo torsion or rupture.[1] Although laparoscopy has been used successfully for management of small to moderate sized adnexal cysts, many surgeons still prefer laparotomy for removal of giant paraovarian cysts.[3] Presented herewith is a case of a giant paraovarian cyst in an adolescent female managed laparoscopically.

Case Report

A 16 year old, unmarried girl presented to the outpatient department with complaints of dull aching abdominal pain and distension since last 3-4 months. There was no history of fever, gastrointestinal or urinary complaints, anorexia, loss of weight. Patient had attained menarche at 12 years of age followed by regular menses every 30 days with average amount and duration of blood flow.  Her last menstrual period was 7 days ago. No other significant medical/ surgical history was obtained. On examination, all vital parameters were within normal limits. Abdomen was distended with a huge mass of size corresponding to 34 weeks uterus (Figure 1A), cystic in consistency, restricted mobility. Lower pole of the mass could not be reached suggesting a pelvic origin. Ultrasonography and Computed Tomography revealed a 26x18 cm left ovarian simple cyst, unilocular with smooth surface and absence of any septations or solid areas. Uterus and right ovary were normal. Laboratory investigations including tumor markers (CA-125, β HCG, α fetoprotein & lactate dehydrogenase) were within normal limits. A diagnosis of a benign left ovarian cyst was arrived upon and laparoscopic cyst excision was planned.
A supra-umbilical 10mm primary port was created using direct trocar entry technique. Two 6 mm ports were then placed under vision in left upper and lower quadrants, taking care not to puncture the cyst. Findings at laparoscopy showed large smooth walled paraovarian cyst. The cyst had reached underneath the liver (Figure 1B). Both the ovaries were seen separate from the cyst. A small linear incision was made over the cyst at an area away from the fallopian tube using scissors. The cyst wall was punctured and around 2 liters of clear fluid was drained using a suction cannula, taking care not to cause spillage. Cyst wall was held with a blunt grasper and traction applied upwards to facilitate peeling off from the broad ligament (Figure 1C). Simultaneous coagulation of vessels in the bed of the cyst was performed using bipolar diathermy. Completely excised cyst wall was retrieved through the left lower side port. Thorough peritoneal wash was given with 1 liter of warm normal saline and hemostasis was confirmed (Figure 1D). There were no intra operative complications. Postoperative course was uneventful and patient was discharged on day 3.

Figure 1. (A) Distended abdomen with pelvic mass corresponding to 34 weeks size uterus; (B)Giant paraovarian cyst reaching upto the undersurface of liver; (C) separation of cyst wall from leaves of broad ligament with simultaneous bipolar coagulation of the bed; (D) Final view after complete cyst excision.


Paraovarian cysts are more commonly diagnosed in women in their 3rd – 4th decade of life, possibly because they remain small and asymptomatic for many years before being diagnosed.[1, 2] Occasionally, they may undergo massive enlargement, rupture, hemorrhage or torsion.[2] Reports of giant paraovarian cysts, especially in young females are sparse and there are no strict numerical criteria to define ‘giant/ huge’ paraovarian cysts.[4] We searched the literature on huge paraovarian cysts in young adolescent females using different terms like ‘huge’, ‘giant’, ‘voluminous’ etc. and found eight such reports in females aged between 14- 19 years,[3-10], two case reports in elderly females aged 74  and 40 years,[11, 12] and one report during  pregnancy. [13] Maximum diameter of the paraovarian cysts in these reports ranged from 20 cm to 40 cm.
Paraovarian cysts are often misdiagnosed as ovarian cysts on preoperative imaging using ultrasonography, CT scan and/ or MRI, as with our case.[2] The most probable reason being difficulty in visualizing the ovaries separately from the mass, more so with huge masses.  It is only during surgery that the diagnosis becomes evident.[1, 2] Although these cysts are usually benign, occasional borderline and rarely, malignant tumors have been reported to occur in these cysts.[1] Hence, a complete preoperative workup to rule out malignancy, including serum tumor markers, is mandatory. In the present case, the preoperative workup suggested a benign cyst, hence decision of laparoscopic cystectomy was taken.
Decision of laparotomy vs laparoscopy depends on many factors like size of the cyst, nature of the cyst (benign or malignant), availability of good instruments and expertise for laparoscopic surgery. Most authors opt for standard laparotomy with vertical midline incisions extending above the umbilicus for giant paraovarian cysts.[3, 5-7, 11] Most common reasons of preferring laparotomy over laparoscopy for giant paraovarian cysts is limited space within the abdomen and risk of cyst rupture during laparoscopy.[4] Three case reports advocate minilaparotomy with smaller incisions followed by cyst aspiration to reduce the size of the cyst.[4, 8-9] One author advocated ultrasonography guided cyst aspiration followed by laparoscopic excision of the cyst wall.[12] Only two case reports describe pure laparoscopic excision of giant paraovarian cysts. Rouzi et al  achieved complete cyst excision of a 20 cm paraovarian cyst in a pregnant patient at 20 weeks of gestation.[13] Leanza et al described laparoscopic excision of a huge paraovarian cyst (diameter 30 cm) in a 14 year old obese girl.[10] The surgical techniques and other details of these two cases are given in Table 1. In both cases, there were no reported intra/ post operative complications and ipsilateral ovary and fallopian tube were well preserved, which was true for our patient too.
The two most important aspects of laparoscopic surgery in patients with huge paraovarian cyst are techniques of abdominal entry and tissue retrieval. A supra-umbilical site for primary trocar placement is recommended, as it allows greater degree of freedom for manipulation, gives a better vision and also reduces the chances of injury to the cyst.[14] Entry via Palmer’s point (a point 3 cm below the left subcostal margin in the midclavicular line) or the Lee Huang point (a point over the midline, midway between the xiphoid process and the umbilicus) may also be considered as alternatives to umbilical entry for the same advantages as cited above.[15] The open method (Hasson’s technique), where peritoneum is opened and trocar is placed under vision, is especially useful in patients with suspected intra peritoneal adhesions and may also prevent cyst rupture in cases with huge ovarian/ paraovarian cysts. As far as the use of Veress needle for primary port placement is concerned, the recent Cochrane review states that while the risk of visceral and solid organ injury remains the same, the risk of vascular injuries and failed insertions is definitely decreased with the direct trocar insertion technique as compared to Veress/ trocar insertion.[16] Thus, no one method can be recommended as superior to the other and we believe that a surgeon should choose the method of entry depending on his expertise and the patient characteristics. In the present case, the author utilized a direct supra umbilical trocar entry. For retrieval of the cyst, cyst fluid had to be aspirated to decrease the size followed by complete excision of the cyst wall. The risk of inadvertent spillage of cyst fluid and spread of malignant cells remains a concern; however, with proper patient selection to exclude cases of malignancy, this approach appears to be safe even in huge cysts.[17] Smaller cysts may be completely enucleated, placed in an endobag and then decompressed to avoid spillage of cyst fluid in the peritoneal cavity and also avoid contamination of port site that can occur while retrieving the tissue. However, the endobags have there own disadvantages (they are costly, available in predetermined sizes, difficult to manipulate and may rupture intraoperatively), hence there use should be individualized.[18]   
Laparoscopy has well known advantages over traditional laparotomy like shorter hospital stay, rapid recovery, better cosmesis (desirable especially in adolescent females).[10, 13] Laparoscopy may also allow a better chance to preserve ovary and fallopian tube due to its excellent magnification power.[10] Hence, in our opinion, in cases of giant paraovarian cysts where malignancy is excluded, by detailed preoperative workup and intra operative inspection of cyst, laparoscopy may be safely employed for their excision, provided adequate expertise is available.

Details of the two cases of giant paraovarian cysts managed by pure laparoscopic excision.[10, 13] 

Sr. No.
Case 1 (Leanza et al)[10]
Case 2 (Rouzi et al)[13]
Age of patient
14 yrs, obese
26 years, primigravida, 20 weeks gestation
Size of para-ovarian cyst (Maximum Diameter)
30 cm

20 cm

Nature of cyst (As evident on pre-op  workup)
Abdominal Entry technique
Umbilical trocar insertion by Hasson’s technique
Via Palmer’s point (Veress needle followed by trocar insertion)
Total no. of accessory ports
2 (5mm ports in right & left quadrant)
3 (two 5mm ports in right & left quadrants, one 10mm port in the midline suprapubic region)
Technique of cyst excision
Cyst fluid aspiration, complete separation from leaves of broad ligament and retrieval of cyst wall via umbilical incision
Cyst fluid aspiration, complete separation from leaves of broad ligament and retrieval of cyst wall via suprapubic incision
Preservation of ovary and fallopian tube
Complications (Intra/ post op)
Duration of hospital stay
3 days
< 24 hours


Paraovarian cysts as a differential diagnosis should be kept in mind in women presenting with adnexal masses. A complete preoperative workup is a must to rule out rare possibility of malignancy in these cysts. Laparoscopic excision of these cysts appears to be safe and effective in properly selected cases and in expert hands despite their huge sizes.

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Shah NH, Kale KG, Paranjape SH, Shah VN. Successful laparoscopic management of a giant paraovarian cyst in an adolescent female. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/successful-laparoscopic-management-of.html