Volume 2 Issue 2, February 2015

Gupta AS

Stage III Endometriosis In Term Pregnancy
Puri J, Desale S, Gupta AS, Valvi D.

Skeletal Dysplasia: A prenatal Diagnostic Challenge.
Niphadkar M, Parulekar SV.

Portal Venous Thrombosis And Related Complications In Pregnancy: Management.
Sarkar P, Mayadeo NM, Mali K, Mirchandani A.

Rupture Of Uterus Remote From Term
Pranali Ahale P, Chauhan AR, Khadkikar R.

Torsion Of A Large Pedunculated Subserosal Leiomyoma
Amit P, Gupta AS.

Morbidly Adherent Placenta
More V, Dalvi P, Panchbuddhe S, Parulekar SV.

Vulval Lymphangiectasia In Pregnancy
Mhaske N, Fonseca MN, Kharat D, Prasad M.

Sclerosing Stromal Tumor Of The Ovary: A Rare Entity
Kakade AS, Kulkarni YS, Kumar P.

Diagnostic dilemma in a rare case of primary ovarian ectopic pregnancy
Chawla LJ, Gokhale A.

Focal Morbid Adherent Placenta on LSCS Scar
Kulkarni A, Qureshi S, Gupta AS.

Quiz February 2015


Gupta AS

Tertiary referral medical college centers like ours are seeing an increased number of cases with morbidly adherent placenta. With the rising incidence of cesarean births the incidence of placenta previa and placenta accreta has increased. Any cause that prevents a good decidual reaction like previous cesarean, hysterotomy, myomectomy scars, Asherman syndrome, multiparity can result in a morbidly adherent placenta.  The decidua basalis and the Nitabuch's layer interface between the invading trophoblast and the myometrium is deficient due to the scar tissue. This allows the invading trophoblast to invade the myometrium or the uterine serosa to varying depths. The degree of invasion determines the 3 categories of the adherent placenta, accreta, increta or percreta. The placenta may be totally adherent or only part of the placenta may be focally or partially adherent. Antenatally about 50% cases are diagnosed. Antenatal detection rates can be improved with increased awareness and meticulous effort by the obstetrician, sonologist especially in cases of previous hysterotomies with an anteriorly implanted placenta. USG, Doppler flowmetry and MRI have allowed almost 100% detection rates.
The clinician can encounter either a totally or a focally adherent placenta. Antenatal diagnosis allows the clinician to organize a proper management strategy and team. Proper counseling and consent of the patient, availability of adequate cross matched blood, blood products, team of anesthetists, interventional radiologists, urologists, or surgeons can be arranged prior to delivery. Both the varieties pose management challenges especially in a woman desiring future child bearing. In a totally adherent placenta accreta, increta or percreta a classical cesarean section with ligation of the cord at its placental root, followed by post operative chemotherapy with methotrexate and antibiotics with close monitoring is the mainstay of treatment in a woman desiring future pregnancies. Treatment in cases with involvement of adjacent viscus like the bladder in placenta percreta is very tricky. Any attempts to separate the placenta can lead to torrential life threatening bleeding. Inflation of balloons placed preoperatively in the anterior division of the internal iliac arteries after delivery of the child, intra operative blood replacements, postoperative oxytocics and methotrexate with intensive monitoring may be the best management protocol for patients with placenta percreta. Hysterectomy after the cesarean birth is probably preferred with placenta accreta and increta where the patient does not desire future child birth.  
Focally adherent placenta separates out partially compelling the obstetrician to remove the adherent area of the placenta piecemeal at the time of delivery or proceed with a hysterectomy to control the profuse bleeding from the separated placental bed. In patients desiring future childbearing an attempt to preserve the uterus can be tried by underrunning the placental bed with delayed absorbable sutures. Affronti's, Cho and B Lynch sutures may be used to control bleeding from the placental bed. Balloon tamponade, selective devascularization of the uterine vasculature have been attempted.
In this issue of JPGO we bring two cases of focally adhered placenta with varied presentations which were managed successfully. We hope the readers gainfully benefit from the various interesting cases present in this issue.  

Stage III Endometriosis In Term Pregnancy

Author Information

Puri J*, Desale S**, Gupta AS***, Valvi D****.
(* Second Year Resident, ** Fourth Year Resident, *** Professor, Assistant Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)


Endometriosis is defined as presence of endometrium including the glands and the stroma at locations other than the uterine cavity. It causes inflammation, scarring, fibrosis, and adhesions resulting in distortion of pelvic anatomy, causing infertility. Our patient had conceived spontaneously and was found incidentally to have widespread endometriosis during cesarean section.


Patients with endometriosis usually present with symptoms of chronic pelvic pain, dyspareunia, infertility, dysmenorrhea, inguinal pain, and or pain during exercise. About 20-25% of women have asymptomatic endometriosis.[1] Thirty to fifty percent of patients with endometriosis are infertile.[1]

Case Report

A 22-year-old primigravida, married for one and a half year, conceived spontaneously without any history of infertility was registered with us for antenatal care. Labor was induced at 40 weeks and 4 days of gestation. A trans cervical Foley’s catheterization was done for cervical ripening as her Bishop’s score was less than 4. She was taken up for lower segment cesarean section (LSCS) in view of failure of induction. After the delivery of fetus, the right ovary was found to have an endometriotic cyst measuring 4x4 cm. It got ruptured and chocolate like material flowed out of the cyst. Cauterization of the base of the cyst and powder burn marks was done. Two pearly white cysts of about 3x3 and 3x3 cm were also seen in the right ovary. Right ovary was stuck to the posterior surface of right side of uterus. Powder burns were seen on the right side, on the posterior surface of uterus. Left fallopian tube and ovary were normal but stuck deep in the pouch of Douglas. Our patient had Stage III (moderate) endometriosis as seen in Figures 1, 2, and 3. Her course in the ward was uneventful. Patient was discharged on Day 5 of LSCS.

Figure1. Endometriotic Chocolate cyst in Right Ovary.

Figure 2. Powder burn marks of active endometriotic lesions.

Figure 3. Scarring of Endometriosis)


The prevalence of endometriosis is 6-10% in women of reproductive age group.[2] Nearly 20-25% of patients are asymptomatic.[1]  Short duration of menstrual cycle and longer duration of menstrual flow are associated high risk for endometriosis as retrograde menstruation is more commonly associated with such menstrual flow patterns. Pregnancy has a protective effect as it decreases the menstruation. Two pearly white ovarian cysts, seen intra-operatively were unlikely to be polycystic ovaries, as polycystic ovaries are more common bilaterally. Moreover, patients with polycystic ovaries usually do not ovulate spontaneously. The cysts could be probably due to adhesions on the surface of the ovary due to endometriosis. Endometriosis most frequently starts in the most dependent areas of the pelvis like, ovaries (most common site)[3] posterior pouch of Douglas, uterosacral ligaments, posterior uterus and posterior broad ligaments. Women with endometriosis are more likely to be delivered by caesarean section.[4] The endometriotic lesions seen were active lesions, powder burn marks, and chocolate cyst with its lining. Scarring was seen on the posterior surface of the uterus, which was due to healing effects probably due to pregnancy.  Decidualization of the endometrial tissue was not seen. Histopathology of the tissue was not sent because of obvious findings of endometriosis. One treatment modality for endometriosis is pseudo-pregnancy with combined oral contraceptive pills for nine months. Interestingly, this patient had active lesions even after nine months of amenorrhea. Rate of conception after surgical laparoscopic treatment by removing the endometriotic implants seen during laparoscopy and adhesiolysis as per different stages of the disease is as follows, Stage I-35.7%, Stage II-44.4% and for Stage III-53.3%. Rate of conception for Stage IV is 20%. However, the rates of conception are not dependent on severity of endometriosis.[5] The patient has been explained about the endometriotic lesions. The patient is further expected to be in amenorrhea during her lactational period. The treatment of endometriosis by cauterizing the powder burns and the endometriotic cyst wall and the protective effect from pregnancy and lactation increases the chances of patient being free from the disease and a good obstetric career. The further plan of management for the patient is to follow up with serial ultrasonography scans, if she becomes symptomatic when she starts to menstruate and check if ovarian endometriosis recurs.


  1. Bulletti C, Elisabetta M, Battistoni CS, Borini A. Endometriosis and Infertility, Journal of Assisted Reproduction and Genetics. 2010;27(8):441-447.
  2. Raffi F, Amer S, “Endometriosis” Obstetrics, Gynaecology and Reproductive Medicine. 2011; 21(4):112-117.
  3. Gylfason JT, Kristjansson KA, Sverrisdottir G, Jonsdottir K, Rafnsson V, Gerisson RT. Pelvic endometriosis diagnosed in an entire nation over 20 years. Am J Epidemiol 2010;172 (3):237–243.
  4. Stephansson O, Kieler H, Granath F, Falconer H. Endometriosis, assisted reproduction technology and risk of adverse pregnancy outcome. Human Reproduction: Oxford Journals. 2009;24(9):2341-2347.
  5. Lee HJ, Lee JE, Ku SY, Kim SH, Kim JG, Moon SY, et al. Natural conception rates following laparoscopic surgery in infertile women with endometriosis. Clinical and Experimental Reproductive Medicine. 2013;40(1):29-32.

Puri J, Desale S, Gupta AS, Valvi D. Stage III Endometriosis In Term Pregnancy. JPGO 2015. Volume 2 No. 2. Available from: http://www.jpgo.org/2015/02/stage-iii-endometriosis-in-term.html

Skeletal Dysplasia: A prenatal Diagnostic Challenge

Author information

Niphadkar M*, Parulekar SV**
(* Second year resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G.S medical college and KEM hospital, Mumbai, India)


Skeletal dysplasias are a very wide and diverse group of conditions related to abnormal bone growth and development. The prenatal diagnosis by ultrasound is  challenging due to wide variety of clinical manifestations and changes occurring due to advancing gestational age. Most of these babies are born at  or near term. Here we report an image of a second trimester fetal demise with the abortus having  abnormally short limbs.


The most common type of lethal skeletal dysplasia occurring in the neonatal period is thanatophoric dyplasia. 1 These conditions pose a challenge for antenatal sonography diagnosis because of large number of dysplasias, variability in time at which the features appear, phenotypic variability with overlapping features and lack of molecular diagnosis.

Case Report

A 24 year old woman, married for 9 years (non-consanguineous marriage) , gravida 5, para 3, living 2, abortion 1, neonatal death 1 presented with 5 months of amenorrhea. She was referred from a private hospital in view of ultrasonography suggestive of intrauterine fetal demise at 14.5 weeks with malformed fetal limbs. The patient had no complaints of pain in abdomen, bleeding per vaginum, any bowel or bladder disturbances. She had no history of any significant medical or surgical illness. Obstetric history was significant, as follows:
G1:  female of 6 years, full term normal delivery (FTND)
G2:  female who died on day 1 of FTND. Patient gave history that the baby had absent proximal limb bones.
G3:  male of 1.5 years, FTND.
G4:  spontaneous abortion at 2 month amenorrhea, check curettage was done
G5:   present pregnancy 

The pregnancy was terminated by induction with tablet Misoprostol 400 μg per vaginally 4 hourly. The patient aborted with three doses. The abortus was found to have abnormally short limbs (figure 1).

Figure 1. Abortus with hupoplastic upper limbs.

It showed short, stunted limbs, apparently missing proximal limb bones, without polydactyly or syndactyly. It was somewhat macerated and its sex could not be determined. Its long bones could not be assessed due to very less gestational age of the abortus. Fetal karyotyping and fetal biopsy could not be done due to maceration of tissues. After considering the history and examination of the abortus, the most probable diagnosis of thanotophoric dysplasia, though osteogenesis imperfecta type 2, achondroplasia and other less common dysplasias could not be ruled out with certainty. The patient was explained regarding the autosomal dominant inheritance of the condition resulting in 2 of her pregnancies getting affected and was advised to avoid consanguineous marriages of her children to avoid the future generations being affected by the condition.


Skeletal dysplasias are a wide heterogeneous group of conditions caused by disturbances of bone growth, beginning in the early stages of fetal development and evolving throughout life due to active gene involvement.[1] The  osteochondrodysplasias  are  disorders  of  development  and/or  growth  of  cartilage and/or  bone, causing affection of long  bones  in  a  generalized and symmetric  manner  and dwarfism. Various causes include thanatophoric dwarfism, achondrogenesis, amelia, osteopetrosis, cleidocranial dysplasia, Ellis-van Creveld syndrome, short rib polydactyly syndrome, Jarcho Levin syndrome, femoral hypoplasia – unusual face syndrome, Ol type 1-3, spondyloepiphyseal dysplasia congenital, SADDAN, fibrochondrogenesis etc.[1,2] Prenatal diagnosis may be possible with ultrasonography (long bone measurements at or less than the 5th centile or >3 SD below the mean.[3,4] Perinatal lethality can be assessed by ultrasonographic chest circumference to abdominal circumference ratio (<0.6) and/or femur length to abdominal circumference measurement ratio (0.16). [3] Molecular testing helps identify homozygosity or compound heterozygosity. Potdelivery/postmortem diagnosis can be done with radiography, autopsy, histopathology and Immunohistochemistry. [3,4] However if a patient aborts, the clinicians need to have knowledge of the appearance of such fetuses so that the abortus can be studied further. The published literature does not have many images of such abortuses. Hence this image is presented.

  1. Dighe M, Fligner C, Cheng E, Warren B, Dubinsky T. Fetal Skeletal Dysplasia: An Approach to Diagnosis with Illustrative Cases. RSNA RadioGraphics 2008;28(4). DOI: http://dx.doi.org/10.1148/rg.284075122
  2. Krakow D, Rimoin DL. The skeletal dysplasias. Genetics in Medicine 2010;12:327–341.
  3. Krakow D, Lachman RS, Rimoin DL. Guidelines for the prenatal diagnosis of fetal skeletal dysplasias. Genet Med. 2009;11(2): 127–133. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2832320/
  4. Gnoli M, Pedrini E, Mordenti M, Tremosini M, Sangiorgi L. Skeletal dysplasias: approach to the clinical diagnosis and implication of appropriate diagnosis for knowledge and research studies in these rare diseases. Hereditary multiple Osteochondromas as example/paradigm. Italian Journal of Pediatrics 2014;40(Suppl 1):A8  doi:10.1186/1824-7288-40-S1-A8 Available from: http://www.ijponline.net/content/40/S1/A8

Niphadkar M, Parulekar SV. Skeletal Dysplasia: A prenatal Diagnostic Challenge. JPGO 2015. Volume 2 No. 2. Available from:  http://www.jpgo.org/2015/02/skeletal-dysplasia-prenatal-diagnostic.html

Portal Venous Thrombosis And Related Complications In Pregnancy: Management

Author Information

Sarkar P*, Mayadeo NM**, Mali K***, Mirchandani A***.
(* Fourth Year Resident, ** Professor, *** Assistant Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)


Portal pressure physiologically increases in pregnancy, leading to exacerbation of preexisting portal hypertension, thereby causing esophageal varices and variceal hemorrhage. Portal vein thrombosis is an uncommon cause of portal hypertension complicating pregnancy. The coexistence of the manifestations of portal hypertension as well as hypersplenism and esophageal varices together with Factor V deficiency during pregnancy is a rare presentation and poses a real challenge for diagnosis and management. We report the clinical follow-up of a 25-year-old pregnant woman who is a known case of Factor V Leiden deficiency and portal vein thrombosis with cavernoma formation, for which she underwent splenectomy and lieno-renal shunt placement 5 years before conception. Antenatal period was uncomplicated and both maternal and fetal outcome was good.


Portal venous cavernoma secondary to portal venous thrombosis is a rare entity and presents with various complications to both mother and fetus. Literature regarding its management protocol presents with various conflicts. Hence we present this unique case and its antenatal management.

Case Report

A 25 year old primigravida who was a known case of extrahepatic portal venous obstruction was registered at our antenatal clinic from first trimester. From the age of 5, she was diagnosed with portal venous obstruction. At the time of diagnosis a splenoportogram showed portal hypertension with portal vein thrombosis with left gastric and short gastric varices with portal cavernomas. She had an uneventful course till the age of 20 years when she presented with acute abdomen, hematemesis, epistaxis, hematochezia and hematuria. CT angiography showed extrahepatic portal venous obstruction with multiple porta-systemic collaterals and splenomegaly with thrombosis of the main portal vein. On examination she had pallor and massive splenomegaly. Hemogram showed pancytopenia, following which bone marrow examination was done which revealed features of hypersplenism with dimorphic anemia and thrombocytopenia. Subsequently, splenectomy was done with proximal lieno-renal shunt placement. Blood counts improved in the post operative period. One year later, hepatoportal system Doppler showed cavernoma formation with patent lieno-renal shunt. Thrombophilia work up revealed Factor V Leiden deficieny, following which she received oral warfarin for six months. After 5 years, she conceived spontaneously and her antenatal period was uneventful and she was started on oral aspirin prophylactically from second trimester which was continued till 36 weeks. No prophylactic anticoagulant was given. Her hemogram, liver function tests (LFT) and coagulogram were monitored in each trimester and found to be normal. She was normotensive throughout pregnancy and had no evidence of gastrointestinal bleed. Anti-phospholipid antibody (APLA) and rest of thrombophila work up was normal. Regular sonography showed adequate growth of the baby. She was initially planned  for trial of vaginal birth, but at 37 weeks she presented in labor and emergency cesarean section was done in view of oblique lie. She delivered a baby girl of 2.5 kg with APGAR scores of 8, 9 and there was no intra-operative complication. Post operative period was uneventful.


Extra hepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in the developing countries and second to cirrhosis in developed countries.[1] Common presentations of this condition are variceal bleed, recurrent thrombosis and hypersplenism.[2] EHPVO can either present as acute (recent) or chronic. Recent EHPVO usually presents with abdominal pain, ascites, fever, and jaundice  whereas chronic EHPVO presents with repeated, well tolerated bleeding from esophageal varices. The outcome of chronic portal venous thrombosis (PVT) has improved over the past years.[2]
Because of intensified prophylaxis for bleeding related to portal hypertension and control of underlying prothrombotic conditions survival among women with PVT has increased remarkably. As a result, desire for pregnancy has become a major issue for young women with well-controlled chronic PVT.[3] Thrombosis is the leading cause of maternal morbidity during pregnancy in a case of portal hypertension as in most of the cases there is underlying thrombogenic condition.[4,5]
The mode of delivery did not appear to influence the risk of bleeding. As a cesarean section is associated with a significantly increased risk of thromboembolic complications, which may be risky in patients with portal hypertension, it seems safe to recommend vaginal delivery whenever possible, restricting cesarean section to obstetrical indications. In PVT patients with prothrombotic conditions, or with thrombosis involving the mesenteric veins, who are at greater risk of intestinal ischemia, anticoagulation therapy can be considered during pregnancy, as recommended  elsewhere for patients with other forms of thrombosis.[7] Management strategy is based on a systematic portal hypertension screening before pregnancy in known cases of chronic EHPVO. Prophylaxis of portal hypertension bleeding/ thrombosis and interruption of anticoagulation for a short duration at delivery are required. In acute cases management can be surgical or medical. Surgical treatment includes shunts and band ligation of esophageal varices. Medical treatment includes use of β-blockers to decrease the flow in portal circulation. Index patient was a chronic case with pre-pregnancy USG Doppler showing normal flow and patent shunt.  Hence we did not advice prophylactic anticoagulation in this case. Feto-maternal outcome was favorable and post operative course was uneventful.

  1. Aggarwal N, Chopra S, Raveendran A, et al: Extra hepatic portal vein obstruction and pregnancy outcome: largest reported experience. J Obstet Gynaecol Res 2011;37:575-80.
  2. Hoekstra J, Seijo S, Rautou PE, et al: Pregnancy in women with portal vein thrombosis: results of a multicentric European study on maternal and fetal management and outcome. J Hepatol 2012;57:1214-9.
  3. Cheng YS: Pregnancy in liver cirrhosis and/or portal hypertension. Am J Obstet Gynecol 1977;128:812-22.
  4. Griesshammer M, Grunewald M, Michiels JJ: Acquired thrombophilia in pregnancy: essential thrombocythemia. Semin Thromb Hemost 2003;29:205-12.
  5. James AH, Jamison MG, Brancazio LR, et al: Venous thromboembolism during pregnancy and the postpartum period: incidence, risk factors, and mortality. Am J Obstet Gynecol 2006;194:1311-5.
  6. Kochhar R, Kumar S, Goel RC, et al: Pregnancy and its outcome in patients with noncirrhotic portal hypertension. Dig Dis Sci 1999;44:1356-61.
  7. Tsochatzis EA, Senzolo M, Germani G, et al: Systematic review: portal vein thrombosis in cirrhosis. Aliment Pharmacol Ther 2010;31:366-74.

Sarkar P, Mayadeo NM, Mali K, Mirchandani A. Portal Venous Thrombosis And Related Complications In Pregnancy: Management. JPGO 2015. Volume 2 No. 2. Available from:  http://www.jpgo.org/2015/02/portal-venous-thrombosis-and-related.html

Rupture Of Uterus Remote From Term

Author Information

Ahale P*, Chauhan AR**, Khadkikar R***.
(* Fourth Year Resident, ** Additional Professor, *** Assistant Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)


Uterine rupture is classified as either complete when all layers of the uterine wall are separated, or incomplete when the uterine muscle is separated but the visceral peritoneum is intact (uterine dehiscence). Morbidity and mortality rates are high when rupture is complete. The greatest risk factor for either form of rupture is prior cesarean delivery. We report a case of a primigravida with 24 weeks’ pregnancy with rupture of the uterine fundus, with past history of hysteroscopic injury, most probably uterine perforation, which was repaired in order to conserve the uterus.


Rupture of uterus during labor is a common event. Overall incidence of uterine rupture is 1 in 1146 pregnancies (0.07%)  and  mostly they occur following previous cesarean section involving  the lower segment. Spontaneous uterine rupture in the second trimester is very rare. Previous reported cases show that second trimester uterine rupture is associated with invasive choriocarcinoma, placenta increta or percreta,[1] pregnancy in a rudimentary horn of a malformed uterus,[2] previous myomectomy,  previous cesarean section scars, previous hysterolaparoscopy injury[3,4] and previous dilatation and curettage.

Case Report

A 34 year old primigravida, married for 8 years, was referred with 24 weeks of pregnancy and uterine rupture . The patient was a known case of primary infertility who had undergone diagnostic hysteroscopy and laparoscopy one year ago in a private hospital; she had been verbally told that there was a uterine perforation during hysteroscopy; however, no documentation of the same was available. She conceived within five months of the procedure and had an uneventful first trimester.
She was admitted in the same private hospital for the last 3 days in view of threatened preterm labor, for which she was given isoxuprine intravenous infusion for one day and corticosteroids for lung maturity. However, her pain intensified over 2 to 3 days and she developed gradual abdominal distension. She was referred to our tertiary care center with ultrasound showing a 2 cm rent in the uterine fundus and a large fluid collection with internal echoes superior to the rent, outside the uterine cavity with moderate hemoperitoneum. The fetus was seen in cephalic presentation with severe bradycardia. The amniotic fluid index was zero.

Figure 1. Ultrasound showing hemoperitoneum.

On examination, the patient was in hypovolemic shock; her general condition was poor, with a low volume pulse of 110/min and blood pressure of 80/60 mm Hg. Abdominal examination revealed tense abdominal distension up to 36 weeks’ uterine size. Uterine contour was absent. There was no vaginal bleeding and the cervix was closed. Emergency exploratory laparotomy was performed. Approximately 1000 ml of blood with clots was removed. Uterine rupture at the fundus measuring approximately 7 x 7 cm, extending from 2 cm above right cornua, to approximately 1 cm above left cornua was seen, with part of amniotic sac and placenta expelled out through the ruptured site. A 690 g stillborn female fetus with placenta and membranes was removed from uterus through the ruptured site. Repair of ruptured uterus was done in 2 layers with No. 1 polyglactin. The tubes and ovaries were normal. She was transfused with two units of whole blood. Post operative course was uneventful and she was discharged after thorough counseling about avoidance of conception and possibility of pregnancy through surrogacy in the future.

Figure 2. Repaired fundal rupture.


Rupture uterus is not uncommon following previous cesarean section. Most of these occur during labor. Surgeries in which uterine scarring occurs on upper segment like hysterotomy, upper segment cesarean section, myomectomy, previously repaired uterine rupture, surgeries done for mullerian anomalies like metroplasty, and LSCS with upward, J shaped  or inverted T-shaped extension are more prone to uterine rupture  even before labor starts, unlike the lower segment scars which are likely to rupture only during labor.
The initial signs and symptoms of uterine rupture are nonspecific making the diagnosis difficult; this can cause delay in definitive therapy.  Fetal morbidity and mortality increase due to fetal anoxia occurring as a result of catastrophic hemorrhage and uterine spasm. The premonitory signs and symptoms of uterine rupture are inconsistent, and the short time for instituting action makes uterine rupture a much feared event for medical practitioners. The signs and symptoms largely depend on the time of onset, site, and severity of the uterine rupture. Rupture at the site of a previous uterine scar is less dramatic because of relatively reduced vascularity.
In our case, there was a rupture involving fundal region at 24 weeks’ of pregnancy with a history of diagnostic hysteroscopy and laparoscopy done 1 year back for primary infertility.
There might have been injury to fundal region during hysteroscopy which might have weakened the myometrium and resulted in rupture in this pregnancy. The decision for repair rather than hysterectomy was taken to maintain the menstrual function.
In contrast to many reports in which rupture of uterus during second trimester was diagnosed in previously scarred uterus, our patient had no history of previous cesarean section. However, advanced maternal age and history of hysteroscopic injury may have been the cause in this patient.

  1. De Roux SJ, Prendergast NC, Adsay NV. Spontaneous uterine rupture with fatal hemoperitonium due to placenta accrete percreta: a case report and review of the literature. Int J Gynecol Pathol. 1999;18(1):82-6.
  2. Ayoubi JM, Fanchin R, Lesourd F, et al. Rupture of a uterine horn after laparoscopic salpingectomy. A case report. J Reprod Med 2003;48(4):290-2.
  3. Dubuisson JB, Fauconnier A, Deffarges JV, et al. Pregnancy outcome and deliveries following laparoscopic myomectomy. Hum Reprod. 2000;15(4):869-73.
  4. Matsue K, Shimoya K, Shinakai T, et al. Uterine rupture of caesarean scar related to spontaneous abortion in the first trimester. J Obstet Gynecol Res 2004;30(1):34-6.

Pranali Ahale P, Chauhan AR, Khadkikar R. Rupture Of Uterus Remote From Term. JPGO 2015. Volume 2 No. 2. Available from: http://www.jpgo.org/2015/02/rupture-of-uterus-remote-from-term.html

Torsion Of A Large Pedunculated Subserosal Leiomyoma

Author Information

Patel A*, Gupta AS**.
(* Second Year Resident, ** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)


We report a case of torsion of a large pedunculated fundal uterine leiomyoma. A 45 year old nulligravida presented to us with abdominal pain. With preoperative diagnosis of leiomyoma of the uterus patient was operated for total abdominal hysterectomy, but intra operatively a large fundal subserosal peduculated leiomyoma with one and half turn twist in its thick pedicle was seen. Mostly torsion of pedunculated leiomyoma presents with acute abdomen and needs emergency intervention but in our case the torsion was not suspected clinically. It was a chronic condition.


Uterine leiomyomas or leiomyomas are smooth muscle tumors with benign course. They are the most common gynecological tumors. In 25% women in the reproductive age group leiomyomas were diagnosed clinically and in almost 80% patients they were detected in surgically excised uteri. [1] Leiomyomas can be sub mucosal, intramural, and sub serosal.[2] Sometimes subserosal myomas can become pedunculated and rarely can they undergo torsion to give acute symptoms. A torsion may cause ischemia and rapid clinical deterioration. [3] There are very few cases reported till now. We report a case of pedunculated sub serosal uterine myoma that underwent torsion.

Case Report

A 45 year old nulligravida, a treated case of hyperthyroidism presented to us with complain of mild continuous abdominal discomfort. The patient did not have any acute symptoms, menstrual complaints or any difficulty in defecation or micturition. The pain was chronic dull aching in nature. On abdominal examination there was a 24 weeks size, non tender, hypogastric mass with side to side mobility. The mass was more towards right of the midline. On vaginal examination the uterus was not felt separately from the mass. Clinically a large single fundal leiomyoma was diagnosed. All hematological, biochemical and serological investigations were performed. All were within normal limits except that the peripheral blood smear showed target cells and occasional sickle cells.  A hemoglobin electrophoresis was done which was negative for any hemoglobinopathies. Fragility test for sickling was negative. The patient was posted for elective total abdominal hysterectomy. Surgery was done under combined spinal and epidural anesthesia. The abdomen was opened by a midline infra-umbilical vertical incision which had to be extended 2 cm above the umbilicus. On entering the abdomen there was a large subserosal leiomyoma around 12x15 cm in size with a thick pedicle. The pedicle of the leiomyoma had twisted and the leiomyoma was lying behind the uterus. On exteriorizing the leiomyoma the thick pedicle showed a one and a half circle turn. The myoma was very vascular, congested with large dilated veins on its surface. The uterus, ovaries and fallopian tubes were normal. The leiomyoma weighed 1.2 kg. Hysterectomy was done. Histopathology was suggestive of a benign leiomyoma. The peripheral blood smear was repeated twice within the next 15 post operative days, but no abnormal cells were seen.

Figure 1. Large fibroid with a thick pedicle.

Figure 2. Posterior surface of the uterus.

Figure 3. Anterior surface of the fibroid.


Torsion of a subserosal uterine leiomyoma is very rare. Torsion is mostly seen in ovarian tumors. Torsion first occludes venous and then arterial supply and causes gangrene. The ischemia due to arterial occlusion leads to acute abdominal pain. Small subserous leiomyomas with thin pedicles may undergo torsion.[4] In our case the pedicle was thick about 5 cm in width and hence the 1 and a 1/2 turn of the pedicle probably could out occlude the arterial supply. This obstructed the venous drainage only leading to congestion and a dull aching discomfort. The torsion was probably triggered by the large weight of the leiomyoma and also by the fact that the leiomyoma was broader than its height looking like a mushroom. The turning movements of the patient in recumbent position could have triggered the rotation of the pedicle. In leiomyomas with thin pedicles the torsion may be severe enough to obstruct arterial blood supply leading to ischemic necrosis and a surgical emergency. Hematological changes like anemia, polycythemia, thrombosis, thrombocytosis, and coagulopathies have been reported. In our case the the finding of target cells and few sickle cells were reported on peripheral smear, the significance of which remained undetermined. After the surgery the peripheral smear was again studied twice 15 days apart by the same laboratory senior scientific officer but no trace of those target cells or sickle cells could be found. We postulate that due to torsion and venous congestion the red blood corpuscles were distorted in the twisted pedicle which were then released in the circulation. No report regarding such cells could be found on Medscape, Pubmed, or internet search. In majority of the reported cases the patients presented mostly with acute pain and tenderness over abdomen needing urgent surgical intervention.  On redirect questioning our patient gave history of intermittent episodes of moderate intensity pain which could be due to sub-acute episodes of torsion. Torsion was not detected on ultrasound Doppler signals as its blood supply was intact.


This case is interesting as a large leiomyoma underwent torsion. Thick pedicle rather than a thin pedicle was twisted and abnormal red blood cells were found preoperatively in the peripheral smear which disappeared after the twisted leiomyoma was removed.


1.        Cramer SF, Patel A. The frequency of uterine leiomyomas. Am J Clin Pathol.1990;94(4):435-8.
2.        Berek JS. Chapter 13: Benign Disease of the Female Reproductive Tract. In: Novak’s Gynecology, 13th edition. Philadelphia: Lippincott Williams & Wilkins, 2002:380-7.
3.        Grover S, Sharma Y, Mittal S. Uterine torsion: a missed diagnosis in young girls? J Pediatr Adolesc Gynecol. 2009;22(1):e5-8.
4.        Shrestha E, Ngangbam HS, Yang Y, Li X. Torsion of Pedunculated Subserous Myoma. Journal of Medical Cases. 2011;2(2), 62-3.


Patel A, Gupta AS. Torsion Of A Large Pedunculated Subserosal Leiomyoma. JPGO 2015. Volume 2 No. 2. Available from: http://www.jpgo.org/2015/02/torsion-of-large-pedunculated.html

Morbidly Adherent Placenta

Author Information

More V*, Dalvi P**, Panchbuddhe S*, Parulekar SV***.
(*Assistant Professor, ** First Year Resident, *** Professor and Head of Department., Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)


Adherent placenta is a not uncommon indication for emergency obstetric hysterectomy. We report a case of partially adherent placenta diagnosed intraoperatively during an emergency hysterotomy, which was performed for antenatal hemorrhage due to separation of a placenta implanted in the other segment. In view of intractable post-partum hemorrhage emergency obstetric hysterectomy was performed.


Placenta accreta is a rare and life threatening condition with high maternal mortality and morbidity. Placenta normally is confined to the decidual lining of the uterus, but it may sometime invade the myometrium due to defect in decidua basalis. This condition is known as placenta accreta. It is a not uncommon indication for emergency obstetric hysterectomy. The incidence of placenta accreta is on rise due to increase in rate of cesarean section.

Case Report

A 26 year old women, married for 7 months, second gravida with 19 weeks of pregnancy presented to the emergency department with blood stained vaginal discharge for 10 hours. She had no other complaints. She had a previous one spontaneous abortion at two months of gestation for which dilatation and evacuation was done. She had no significant medical or surgical illness. On examination her vital parameters were stable. Abdominal examination revealed relaxed uterus of 18 weeks with positive external ballottement and regular fetal heart sounds. On speculum examination leak was seen. On vaginal examination cervical os was 2 3 cm dilated, 40-50% effaced with absent membranes and fetal lower limbs palpable. Ultrasonography was done which revealed intrauterine gestation of 18.3 weeks with severe oligohyadraminous (amniotic fluid index - 1). In view of premature prolonged rupture of menbrane with inevitable abortion, a decision for termination of pregnancy with oxytocin augmentation was taken.  Injectable antibiotics were started. Oxytocin infusion was augmented as per uterine activity. She was reassessed after 24 hours. Clinical findings were similar to previous findings, hence decision for administration of misoprostol 400 microgram vaginally was taken. After second dose of misoprostol 400 micrograms per vaginum after four hours, she complained of vaginal bleeding. On vaginal examination findings were again similar to the previous findings with fresh bleeding of around 300 ml. Uterine rupture was suspected and a decision for exploratory laprotomy with hysterotomy was taken. Intraoperatively the uterus was of 18 weeks’ size, with ballooned out lower segment. There was no rupture. Transverse incision was made on the lower uterine segment and the fetus was delivered. The placenta was posterior reaching lower segment. It was partially separated in  the upper portion and was densely adherent on the lower segment due to which it had to be removed piecemeal. Bleeding continued to occur from the lower segment. Multiple hemostatic stitches were taken on the lower segment and bilateral uterine artery ligation was done. Hemostasis could not be achieved. Total obstetric hysterectomy was performed in view of intractable hemorrhage. Intraoperatively three units of whole blood were transfused.  Histopathological examination of the specimen showed invasion of the lower uterine segment endometrium and myometrium by chorionic villi. Post operative course was uneventful.

Figure 1. Figure1: Black arrow upper portion of the uterus and green arrow distended lower segment of the uterus. A small subserous leiomyoma is also seen.


The incidence of placenta accreta was approximately 1 in 2500 in the 1980s, 1 in 535 in 2002 and 1 in 210 in 2006.[1] Placenta accreta is described as an implantation in which there is an abnormally firm adherence of the placenta to the uterine wall. Placenta accreta occurs due to defects in the decidua basalis causing the placenta to adhere to or invade the myometrium. It is further classified: accreta, in which placenta is adherent to the myometrium; increta, in which placenta invades the myometrium; and percreta, in which placenta penetrates the myometrium and serosa and it may be attached to the adjacent structure. Total placenta accreta/increta/percreta is one in which all the lobules of the placenta are abnormally adherent, while partial type involves only a few to several lobule - as was seen in our case in which only lower half was morbidly adherent. Defective decidual formation is commonly in the lower uterine segment over the previous cesarean section scar or after uterine curettage. In our case, the patient had an abortion in the past, for which curettage was performed. The incidence of adherent placenta is increasing because of increasing rate of cesarean section.[2] Other risk factors for placenta accreta  include advanced maternal age, multiparity, previous myomectomy, thermal ablation of the endometrium and uterine artery embolization. Partially separated adherent placenta causes intractable postpartum hemorrhage requiring emergency obstetric hysterectomy.[3] In our case also emergency obstetrics hysterectomy was performed for intractable postpartum hemorrhage. Excessive bleeding in placenta accreta is either due to the involved lobule being pulled off the myometrium, or the lobule getting torn from the placenta. In most of the cases, placenta accreta is not identified till third stage of labor. Ultrasonography is only 33% sensitive in detecting placenta accreta.[4] Magnetic resonance imaging is more accurate in identifying placenta accreta.

  1. Stafford I, Belfort MA. Placenta accreta, increta, and percreta: A team-based approach starts with prevention. Contemp Ob/Gyn April: 2008; 53(4), 76-8.
  2. Silver RM, Landon MB, Rouse DJ, Leveno KJ, Spong CY, Thom EA, et al. Maternal morbidity associated with multiple repeat cesarean deliveries. Obstet Gynecol 2006; 107(6):1226–1232. 
  3. Zelop CM, Harlow BL, Frigoletto FD Jr, Safon LE, Saltzman DH: Emergency peripartum hysterectomy. Am J Obstet Gynecol May 1993;168(5):1443-1448.
  4. Lam H, Pun TC, Lam PW: Successful conservative management of placenta previa accreta during cesarean section. Int J Obstet Gynecol 2004;86(1):31-32.

More V, Dalvi P, Panchbuddhe S, Parulekar SV. Morbidly Adherent Placenta. JPGO 2015. Volume 2 No. 2. Available from: http://www.jpgo.org/2015/02/morbidly-adherent-placenta.html

Vulval Lymphangiectasia In Pregnancy

Author Information

Mhaske N*, Fonseca MN**, Kharat D***, Prasad M****.
(* Second Year Resident, ** Additional Professor, *** Assistant Professor, **** Senior Resident. Department of Obstetrics/Gynecology, LTMMC and LTMGH, Mumbi, India)


 Lymphangiectasias are acquired lymphatic disorders. Vulval lymphangiectasias can mimic other disorders. Vulval lymphangiectasias occurring with pregnancy is a rarity. A case of post-tuberculous vulval lymphangiectasia accompanying pregnancy is described here.


Common vulval disorders in pregnancy include viral warts and herpetic lesions. Rare lesions such as lymphangiectasia can also exist, especially in the setting of risk factors like previous tuberculosis or malignancy and radiotherapy.

Case Report

A 24 year old primigravida presented in outpatient department in early second trimester with vulval vesicular lesions. Patient had tuberculous lymphadenitis of neck three years prior, with no involvement of other systems, for which treatment was completed. Lesions in the vulval region, initially noticed few months prior to pregnancy, were gradually progressive. There were no complaints of pain, bleeding, discharge or pruritus.
Local genital examination revealed multiple erythematous grouped vesicles over the vulval and pubic region, resembling the classically described “frogspawn” appearance.   There was no regional lymphadenopathy or lower limb edema.  Specialist dermatological opinion was taken and clinical diagnosis of lymphangiectasia was reached. Watchful expectancy was maintained. Investigations including urinalysis, HIV, HBsAg, HCV,VDRL were all negative. Antenatal visits were regular, uneventful.

Figure 1. Multiple papulovesicular lesions over the vulva.

Onset of labor was spontaneous at 38 weeks of gestation. Abdominal examination revealed a full term uterus with a live fetus in cephalic presentation and per vaginal examination revealed adequate pelvis. As shown in figure 1, vulval examination revealed multiple vesicular lesions. 
In the second stage of labor, with descent of the presenting part, the labia splayed out and the vesicular lesions got displaced laterally. Thus there was adequate room for a mediolateral episiotomy, delivering a healthy female child of 2570 g. Episiotomy was sutured without disturbing the lesions. Oral antibiotics were given as per usual hospital protocol. Post-delivery period was uneventful.
Post-delivery biopsy (figure 2) showed hyperkeratotic atrophic epidermis, multiple dilated lymphatic channels, chronic inflammatory cells and dilated capillaries, suggestive of lymphangiectasis secondary to chronic infective condition. Post-delivery follow up showed a mild regression in the size of the lesions, and the patient continues to be asymptomatic.

Figure 2. Histopathological appearance of the lesion.


Lymphangiectasia is a disorder of superficial lymphatics which results from an obstruction of previously normal deep lymphatics.[1] It has been long recognized that vulval skin is susceptible to lymphangiectasia, the symptoms of which include vesicular lesions, pain, bleeding and itching.[2]
Few cases of vulval lymphangiectasia have been reported[3] and this entity has been described as a diagnostic challenge.[4] While many causes exist, the associations include malignancy, radiotherapy, tuberculosis and prior pelvic surgical procedures.[5,,6,7,8] Female genital mutilation has also been described as a cause.[9]
Lymphangiectasias associated with pregnancy and spontaneous regression postpartum have been described as a rarity that needs to be reported.[10] Management options include watchful observation, carbon dioxide laser, immunotherapy and surgery.[11]
In pregnancy, a confident diagnosis is needed, because this entity needs to be distinguished from conditions like viral warts and herpes, [3,12]  which have different labor management,  including avoidance of vaginal delivery. It is hoped that this report will add to existing knowledge about this entity during pregnancy.

  1. Huilgol SC, Neill S, Barlow RJ. CO2 laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surg 2002;28(7):575–7.
  2. Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med 1989;65(5):335–7.
  3. Haneef NS, Ramachandra S, Metta AK, Haritha K. Lymphangiectasias of vulva. Indian Dermatol Online J. 2011; 2(1): 40–42. Available from: http://www.idoj.in/text.asp?2011/2/1/40/79854
  4. Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol  2003;101(5 Pt 1):946–54.
  5. Ghaemmaghami F, Karimi Zarchi M, Mousavi A. Major labiaectomy as surgical management of vulvar lymphangioma circumscriptum: three cases and a review of the literature. Arch Gynecol Obstet. 2008;278(1):57–60
  6. Spratt EAG, Batra P, Fischer MK, Pomeranz MK. Vesicular lesions on the vulva. Arch Dermatol. 2012;148(6):755–60.
  7. Stewart CJR, Chan T, Platten M. Acquired lymphangiectasia (’lymphangioma circumscriptum') of the vulva: a report of eight cases. Pathology 2009;41(5):448–53.
  8. Riyaz N, Nair VL. Cutaneous lymphangiectasia secondary to lymph node tuberculosis. Indian J Dermatol Venereol Leprol; 2000;66(6):314–5.
  9. Franco G, Toma L, Nosotti L, Muscardin LM, Morrone A. Vulvar lymphangiectases mimicking genital warts in female genital mutilation. Eur J Dermatol 2006;16(5):587–8.
  10. Verma S. Pregnancy-induced lymphangiectasias of the vulva. Int J STD AIDS. 2008; 19(3):211-2. doi:10.1258/ijsa.2007.007239
  11. Loche F, Schwarze HP, Bazex J. Treatment of acquired cutaneous lymphangiectasis of the thigh and vulva with a carbon dioxide laser. Acta Derm Venereol. 1999;79(4):335.
  12. Sharma R, Tomar S, Chandra M. Acquired vulval lymphangiectases mimicking genital warts. Indian J Dermatol Venereol Leprol; 2002;68(3):166–7.

Mhaske N, Fonseca MN, Kharat D, Prasad M. Vulval Lymphangiectasia In Pregnancy. JPGO 2015. Volume 2 No. 2. Available from: http://www.jpgo.org/2015/02/vulval-lymphangiectasia-in-pregnancy.html

Sclerosing Stromal Tumor Of The Ovary: A Rare Entity

Author Information

Kakade AS*, Kulkarni YS*, Kumar P**.
(* Associate Professor, **Second Year Resident. Department of Obstetrics & Gynecology, Bharati Vidyapeeth Deemed University Medical College, Bharati Hospital & Research Center, Pune, India.)


Sclerosing stromal tumors of ovary are rare benign tumors, which have distinctive clinical and pathologic features. They are characterized by the pseudolobular pattern of the cellular and hypocellular areas with marked vascularity and heterogeneity of the cellular area. They tend to occur more commonly during second to third decade of life and more than 80% of these tumors occur below the age group of 30 years. We present such rare case of sclerosing stromal tumor of the ovary in a 38 years old patient. The ultrasound and gross findings were suggestive of a malignant neoplasm which turned out to be sclerosing stromal tumor with a very favorable outcome.


Ovarian sex cord stromal tumors represent approximately 8% of primary ovarian neoplasms.[1] In 1973, Chalvardjain and Scully first described sclerosing stromal tumors (SST) as a distinct disease entity in ovarian sex cord stromal tumors. SST is a benign ovarian stromal tumor, which has distinctive clinical and histological characteristics. It is prevalent in young age groups and has heterogeneous pattern, which distinguishes it from the fibromas, thecomas and other types of ovarian stromal tumors. [2] SST is a benign neoplasm and is distinguished from other ovarian stromal by the production of collagen and a pseudolobular pattern with cellular areas separated by edematous and collagenous areas. [3]

Case Report

A 38 years old woman came to the out-patient department with complaints of pain in lower abdomen for 10-12 days. The pain was cramping in nature, continuous, non-radiating and the intensity of pain reduced in lying down and left lateral position. There were no associated bowel or bladder complaints. She did not have any menstrual complaints. Her last menstrual period was one month ago. Her previous cycles were normal. She had three full term vaginal deliveries and underwent a puerperal tubal ligation 14 years ago. She did not have any significant past medical, surgical or personal history. She was given two doses of intravenous iron sucrose by a general practitioner two days back for mild anemia (hemoglobin 8.7 g/dL).
She had a pulse rate of 90 beats per minutes, blood pressure of 120/70 mm Hg. She had mild pallor. Lymph nodes were not palpable. Her cardiovascular and respiratory system did not reveal any abnormality. Her breast and thyroid were normal. The abdomen was soft on palpation and there was a mildly tender suprapubic mass of 9x5 cm, which was firm and with reduced mobility. There was no clinical evidence of free fluid in the abdomen. Per Speculum examination revealed cervical erosion and visual inspection with acetic acid was negative and Pap smear was taken. Per vaginal examination revealed a 9x5x5 cm cystic and solid mass with lobular surface in the right and anterior fornix. It was tender and was felt separately from the uterus. Mobility was reduced and left fornix appeared free.
She was admitted with a suspicion of tubo-ovarian mass undergoing torsion. Her repeat hemoglobin was 8.6 g/dL and CA-125 value was 53.7 U/ml. Her ultrasound revealed a heterogeneous predominantly hypoechoic lobulated mass measuring 10.1x10.0x 6.7 cm which is pushing the uterus caudally. It showed increased perfusion with resistance index of 0.34 (tumoral vascularity). Few areas of necrosis were noted within it. Left ovary was not seen separately from the mass. The right ovary was normal. There was minimal free fluid in the pelvis and Morrison’s pouch. There was no other abnormality in the abdomen. In the settings of raised CA 125 a suspicion of malignancy was kept in mind.
She received one unit of packed cell volume and underwent a staging laparotomy with total abdominal hysterectomy with bilateral salpingo-oophorectomy.
Intraoperative a large lobulated mass of 10 x 10 x 8 cm arising from the left ovary with single twist torsion of the pedicle was seen.

Figure 1. Large lobulated mass arising from the left ovary.

There were cystic and solid areas with edematous ovarian tissue. There were no para-aortic lymph node enlargement and her liver and other organs did not reveal any palpable tumor. The peritoneal fluid was negative for malignant cells.
The cut section of the tumor was yellowish, homogenous, and lobulated and showed a glistening, translucent surface. Histopathological examination showed a benign stromal tumor composed of many lobules of polygonal to spindle cells with uniform round to oval nuclei and eosinophilic cytoplasm (figure 2).

Figure 2. Histopathological appearance of the tumor.

The islands of tumor cells were scattered in an edematous ovarian stroma with many dilated lymphatic and vascular channels within it. The superficial ovarian cortex was relatively spared. The findings were suggestive of sclerosing stromal tumor of ovary with massive edema due to torsion.
She received one unit of packed cells post operatively and had an uneventful recovery. She was discharged after suture removal on day 8 of surgery. She did not have any symptoms on follow up after one month of the surgery.


SST is a benign subtype of ovarian stromal tumor, described as a distinct entity in 1973 by Chalvardjian and Scully. Sex cord stromal tumors have an approximate occurrence in 8% of ovarian neoplasms and SST compromises less than 5% of these.[1] It differs from fibromas, thecomas, and lipid cell tumors in clinical presentation and histopathological features. SST usually occurs in the second and third decades of life, younger than the mean age of patients with other types of stromal cell tumors. The symptoms associated with these tumors are altered menstrual patterns and lower abdominal pain. They may have some functional activity and very rarely may have associated ascites.[2, 4, 5]
Histopatholgical examination of these tumors reveals a pseudolobular pattern in the cellular and hypo cellular areas. There is a prominent vasculature with hemangiopericytomatous pattern. There are areas of cellular heterogeneity in the luteinized theca like cells and there are many spindle shaped, fibroblast like cells seen.[6, 7]
The term ‘sclerosing stromal tumor’ was attributed because the cellular areas of this tumor revealed collagenous sclerosis. Immunohistochemistry plays an important role in distinguishing these tumors from massive ovarian edema and Krukenberg’s tumor. [3]
The luteinized theca like cells may secrete vascular permeability factor and vascular endothelial growth factor causing vasodilatation leading to hypervascularity, hyperpermeability and edema.[7] This zonal edema is a distinctive feature of SST. The edematous stroma of these tumors may show some vacuolated and signet ring cells which can be confused with signets cells of Krukenberg tumor of ovary.[8] Blood markers like calcitonin, inhibin, CD34, and α –glutathione S-transferase positivity have been found to be useful to differentiate SST from thecomas, fibromas and other sex cord stromal tumors.[9]
The etiology of SST remains largely unknown. By virtue of their structural morphology they are proposed to arise from the immature pluripotent stromal cells of the ovary.[10] An alternative origin from the muscle specific actin positive elements of the theca cells called as perifollicular myoid stromal cells has also been reported.[7]
The reported case was a rare occurrence of SST in fourth decade with symptoms and signs suggestive of torsion, and a malignant neoplasm which turned out to be benign on histopathology. The massive edema seen in the ovary of this patient can be because of the torsion and the vascular hyperpermeability induced by VPF & VEGF.
In conclusion SST should be considered in women when clinical findings suggest a solid and cystic adnexal mass with multiple lobulations, with an ultrasound showing low PI and RI values and no ascites. The ultrasound features of SST may mimic those of malignant ovarian tumors, and hence color Doppler ultrasound is advisable to reveal the prominent peripheral vascularity. [11]


1.      Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex cord-stromal and sertoli cell tumors of the ovary. Radiographics 1998;18:1523-1546.
2.      Chalvardjian A, Scully RE. Sclerosing stromal tumors of the ovary. Cancer 1973;31: 664-670.
3.      Peng H, Chang T, Hsueh S. Sclerosing stromal tumor of the ovary. Chang Gung Med J 2003;26:444-447.
4.      Matsubayashi R, Matsuo Y, Doi J, Kudo S, Matsuguchi K, Sugimori H. Sclerosing stromal tumor of the ovary: radiologic findings. Eur Radiol 1999;9:1335-1338.
5.      Damjanov I, Drobnjak P, Grizelj V, Longhino N. Sclerosing stromal tumor of the ovary: a hormonal and ultrastructural analysis. Obstet Gynecol 1975;45:675-679.
6.      Kumar R, Mukherjee G, Umadevi K et al. Sclerosing stromal tumor of the ovary. Indian J Cancer 1994;31:92-95.
7.      Kawauchi S, Tsuji T, Kaku T, Kamura T, Nakano H, Tsuneyoshi M. Sclerosing stromal tumor of the ovary: a clinicopathologic, immunohistochemical, ultrastructural, and cytogenetic analysis with special reference to its vasculature. Am J Surg Pathol 1998;22: 83-92.
8.      Tiltman AJ, Haffajee Z. Sclerosing stromal tumors, thecomas, and fibromas of the ovary: an immunohistochemical profile. Int J Gynecol Pathol 1999;18: 254-258.
9.      Akbulut M, Colakoglu N, Soysal ME, Duzcan SE. Sclerosing stromal tumor of the ovary: report of a case and review of literature. Agean Pathology Journal 2004;1:84-89.
10.  Lam RM, Geittmann P. Sclerosing stromal tumor of the ovary. A light, electron microscope and enzyme histochemical study. Int J Gynecol Pathol 1988;7:280-290.
11.  Lee MS, Cho HC, Lee YH, Hong SR. Ovarian sclerosing stromal tumors. J Ultrasound Med 2001;20:413-417.


Kakade AS, Kulkarni YS, Kumar P. Sclerosing Stromal Tumor Of The Ovary: A Rare Entity. JPGO 2015. Volume 2 No. 2. Available from:  http://www.jpgo.org/2015/02/sclerosing-stromal-tumor-of-ovary-rare.html