Volume 3 Issue 2, February 2016

Gupta AS

Successful pregnancy outcome in a case of accessory splenic torsion: A rare clinical entity
Puri J, Thakur H, Gupta AS.

Non Immune Hydrops – An Enigma
Vora P, .Jassawalla MJ, Bhalerao S, Bansal V.

Abdomino-Pelvic Mass In a Teenager - a Diagnostic Dilemma
Anuranjani L, Ostwal P, Chauhan AR.

Lingual Thyroid during Pregnancy Masquerading as Hemangioma of Tongue
Prakash S, Manjrekar V, Samant PY, Parulekar SV.

Is it always an amniotic band?
Prabhu S, Nayak M, Mishra N, Jadhav V.

Pregnancy Outcome In a Case Of Aortoarteritis With Single Kidney
Sinha S, Warke W, Mayadeo NM.

Excision of Cesarean Scar Pregnancy
More VHatkar PShetty A, Gupta AS

Menstrual Red Eye
Niphadkar M, Parulekar SV.

Shah NH, Jethwani L, Paranjpe SH, Shah VN.


Gupta AS

Pregnant women can be affected by acute conditions related to the abdominal viscus. Acute abdomen is an emergency in all patients and a decision regarding surgical management is usually needed. Acute abdomen in pregnancy can be due to conditions specific, associated or incidental to pregnancy. Pregnant women with acute abdomen many times are seen primarily by the obstetrician. The obstetrician should be aware about the incidental causes while evaluating such patients so as not to delay diagnosis and appropriate treatment. A need for multidisciplinary consultation should always be considered and sought. Cause of acute abdomen in pregnancy may be difficult to elicit due to altered anatomy, physiology and altered laboratory parameters. Many of the incidental causes like acute appendicitis, cholecystitis, pancreatitis may mimic the normal symptoms of pregnancy like nausea and vomiting. Leucocytosis that is an important investigation of infectious and inflammatory pathology is also seen in normal pregnancy thus diluting its importance. Abdominal signs of tenderness, guarding and rigidity are also not very prominent as the enlarged gravid uterus has already stretched the peritoneum in advanced pregnancy thus limiting the occurrence of overt peritoneal signs. Examining the woman in the right or the left lateral position and displacing the gravid uterus may help in identifying the organ causing the pain.
A detailed history of onset, duration and progress of the symptoms chiefly pain, period of gestational age, detailed obstetric history, a meticulous general, systemic and local examination with specific attention to the altered anatomy at different gestational ages should be done. This is best illustrated by studying the changing location of the appendix. It is located at McBurney point in the 1st trimester, umblical in the 2nd trimester and right hypochondrium in the 3rd trimester. Acute appendicitis can mimic acute cholecystitis, pancreatitis or even a perforated duodenal ulcer. Precise diagnosis is required especially as some conditions like acute pancreatitis requires conservative management whereas conditions like appendicitis, duodenal ulcer perforation need prompt surgical management. Diagnosis needs to be established by imaging techniques. Ulltrasonography is the main diagnostic technique used in pregnant women. It not only evaluates the extrauterine, the uterine structures but also provides detailed information of the fetus. Risk of exposure to the fetus by ionizing radiation from radiology and CT scan restricts their use. Contrast drugs like gadolinium that cross the placenta also cannot be used. MRI with lower magnetic fields can be used without contrast in the 2nd and the 3rd trimester. Use of MRI in the 1st trimester, even though considered safe is not recommended by the National Radiological Protection Board. Precise diagnosis helps in planning and choosing between conservative and surgical treatment and also the timing of the surgery. In acute conditions when surgery is the treatment of choice and cannot be deferred it should be performed irrespective of the gestational age or else it is best performed in the 2nd trimester when the uterus is quiet and chances of teratogenesis due to the anesthetic agents are absent. Incidence of preterm labor and spontaneous loss is higher in the other two trimesters. Laparoscopy or laparotomy are both safe in expert, trained hands. Even in the 3rd trimester laparoscopy has been performed with good results for both diagnostic and therapeutic indications. Prophylactic, tocolytic administration has no proven benefits though they are still administered. Administration of glucocorticoids for lung maturity can be considered when the risk of preterm labor is significant though the flaring up of infection in the mother can be a real morbid occurrence, and its use should be weighed accordingly. Acute abdomen due to incidental causes is usually not an indication for termination of pregnancy or delivery and many a times it can be dealt with without disturbing the pregnancy. However, if that is not possible then the route of delivery should be decided by obstetric indication only.
We hope our readers will enjoy reading this issue which besides having direct obstetric cases also presents a case of acute abdomen due to splenic torsion.

Successful pregnancy outcome in a case of accessory splenic torsion: A rare clinical entity

Author Information

Puri J*, Thakur H**, Gupta AS***.

(* Third year resident, ** Assistant Professor, *** Professor and Unit Head.
Department of Obstetrics and Gynecology, KEM Hospital, Parel, Mumbai, India.)


A successful outcome of pregnancy with accessory spleen torsion at 14 weeks of gestation that required an exploratory laparotomy in the antenatal period is being presented. Pregnancy was carried till 37 weeks of gestation and an elective LSCS was done as the patient had undergone previous two LSCS.


Accessory spleen is a rare cause of acute abdomen. Accessory spleen was found in 10-15% of autopsy series accidentally.[1] Most of the accessory spleen are asymptomatic, and become problematic only if they get infected, undergo torsion, or necrosis. Most of them present in children. No records were found indicating their presentation and diagnosis in pregnancy on a pubmed, medline and google search. Accessory splenic torsion that occurred at 14 weeks of gestation was treated by exploratory laparotomy and excision of the gangrenous spleen. The pregnancy was continued till term successfully.

Case Report

A 26-year-old G3P2L2, patient with previous two LSCS was posted for elective LSCS and tubectomy at 37 weeks of gestation. Patient delivered a male baby of 2.688 kg, appropriate for gestational age with Apgar score of 9/10.

The patient presented to the emergency room at 14 weeks of gestation with the complaints of acute onset pain in abdomen with pain originating in the left lumbar region and radiating to the back. The pain episode was preceded by one episode of vomiting. The patient had come with an ultrasonography scan (USG) from a private practitioner, suggestive of left psoas abscess. On examination, general and systemic examination was within normal limits. On abdominal examination, uterus of 16 weeks size was palpable; tenderness in left iliac fossa with minimal guarding was elicited. There was no rigidity. Ill-defined fullness was present in the left iliac fossa. On speculum examination, creamy, purulent, non-foul smelling, profuse discharge was seen. Cervix was high up. No fistulous tract or opening was seen in any of the fornices. Sterile swabs were collected and sent for smear and culture. On bimanual pelvic examination, uterus was soft, about 14 weeks in size, deviated to right, os was closed, but cervix was short. No cervical motion tenderness could be elicited. No mass/ cyst was felt in the left fornix. Vagina was not warm. On rectal examination, no mass or collection was felt. Patient underwent a repeat USG scan that was suggestive of a hemorrhagic left ovarian cyst of 9.6 x 6.8 cm, without any vascularity. Due to discrepancy of clinical examination and imaging studies, a magnetic resonance imaging (MRI) for the mass was done. The MRI report was suggestive of a well-defined homogeneous, intraperitoneal mass of size 9.3 x 5.6 x 6.1 cm in the left lumbar region, which was isointense with the spleen. The lesion had a pedicle arising from its lateral aspect just beneath the parietal wall. The pedicle showed fat stranding, up to the splenic vein. Vascular pedicle appeared twisted. MRA (Magnetic Resonance Arteriogram) and MRV (Magnetic Resonance Venogram) revealed no flow in the pedicle, which was suggestive of accessory splenic torsion. Smear did not grow any AFB, bacteria or candida. The patient underwent exploratory laparotomy under general anesthesia. The anesthetist took adequate care regarding adequate oxygenation and vigilant intraoperative monitoring was done. Intra-operatively findings showed an accessory spleen in the left lumbar region with flimsy adhesions to the small bowel loops and the omentum. There was torsion of its pedicle, whose blood supply originated from the splenic artery. There was another native spleen, normal in position and blood supply. Remaining abdominal and pelvic structures were normal. Uterus and adnexae were not handled during the surgery. Patient had an uneventful post-operative stay in the hospital and was discharged on day 5 of the surgery.

Figure 1. MRI showing the torsion of the accessory spleen

Figure 2. Excised gangrenous accessory spleen.

Patient had a regular ANC follow up and care. Progesterone support was given in the form of weekly intramuscular injection of 500 mg of Hydroxyprogesterone, till 37 weeks of gestation. The patient had threatened preterm labor at 33 and 35 weeks of gestation, and she was admitted. Injection Dexamethasone 6mg 12 hourly for 4 doses were given during the first episode. Tocolysis was given with oral Nifedipine 20 mg followed by Nifedipine sustained release 10 mg 12 hourly for 7 days. Urine routine microscopy was within normal limits, urine culture report showed no growth, high cervical swab showed no growth. Patient was conservatively managed and then discharged after 48 hours of observation both the times. Patient underwent an elective LSCS with tubal ligation for previous two LSCS, at 37 completed weeks of gestation. Antibiotics were administered perioperatively as per hospital infection policy. During the LSCS, the scar of the previous LSCS was thinned out, no evidence of dehiscence or rupture was seen. The uterus was a bicornuate uterus. Bilateral adnexa were normal. Bilateral kidneys were palpated. They were normal. The upper abdomen was normal. LSCS surgery was uneventful, and patient tolerated the procedure and anesthesia well. Post-operative stay in the hospital was uneventful. Patient was discharged on Day 5 of surgery. Delayed suture removal was done on day 14. Suture line was healthy.

Figure 3. Bicornuate uterus seen during LSCS

Figure 4. Normal Upper Abdomen during LSCS


Accessory spleen is usually an incidental finding. Accessory spleen develops from dorsal mesogastrium during the embryological development. It is an inert structure, usually does not cause any problems and remains undiagnosed in the lifetime of a person. Accessory spleen was found in 10-30% of autopsies.[1] Torsion of an accessory spleen is a rare clinical event seen in around 0.2-0.3% of splenectomies.[2] Twenty six cases of torsion of accessory spleen have been reported till date.[3] None of these 26 patients was a pregnant woman. The most common locations of accessory spleen (about 22%) are posteromedial to the native spleen.[2] The next most common locations are near the upper pole of the left kidney and around the pancreatic tail.

The most common symptom of torsion of accessory spleen is presentation with acute abdomen, severe abdominal pain originating in the left lumbar region, radiating to the back. The cause of the pain is the necrosis of the splenic tissue. The patient had come with an outside ultrasonography suggestive of left psoas abscess with acute abdomen. A repeat ultrasound was done, which was suggestive of left ovarian cyst, without any vascular supply. Due to unsatisfactory report of ultrasound examination and discrepancy in clinical examination and ultrasound findings, an MRI was done which was suggestive of a 9.3 x 5.6 x 6.1 cm sized accessory spleen with torsion. The patient had undergone an exploratory laparotomy and the devitalized splenic tissue was resected. Preoperative diagnosis of splenic torsion is usually missed and this is detected intraoperatively.[4, 5] However in our patient due to good clinical acumen the ultrasonic diagnosis of psoas abscess and ovarian cyst were disregarded due to mismatch with the abdominal and bimanual pelvic examination and a MRI was obtained which clinched the diagnosis. We feel clinical correlation is essential in such cases and appropriate imaging modalities can reach the final diagnosis preoperatively. Either way in pregnancy the enlarging gravid uterus changes the intra peritoneal dynamics and detection of benign pelvic masses in pregnancy and an acute or sub acute abdomen is always a challenge.

The gold standard for the diagnosis of accessory spleen is MRI. It is better at diagnosing the site of origin, clinical position of accessory splenic tissue, and diagnosing signs of inflammation. CT scan can also be done but it is not advisable in pregnancy. Angiography and nuclear Technetium scans are useful in diagnosing accessory splenic torsion but are useless when there is complete occlusion of arterial supply due to torsion.[4] Angiography is not advised during pregnancy because of fetal irradiation, while Technetium 99m scans can be done when the whole fetal exposure of 0.5 rad is not exceeded.[7]

The differential diagnoses of acute left sided abdominal pain in early pregnancy include genitourinary causes like adnexal torsion; torsion of left ovarian cyst, ureteral calculus. Conditions associated with pregnancy that cause acute abdominal symptoms include torsion of gravid uterus, acute urinary retention due to retroverted uterus, acute red degeneration of a uterine fibroid, and uterine rupture. Vascular causes mimicking acute abdomen include sickle cell crisis, acute splenic artery aneurysm, abdominal trauma, and splenic rupture. Gastrointestinal causes like, acute pancreatitis, peptic ulcer, gastroenteritis, pancreatic pseudocyst, and bowel obstruction. The most common surgical cause of acute abdomen in pregnancy is appendicitis,[7] while the most common gynecologic cause of acute abdomen is ectopic pregnancy.[8]

Exploratory laparotomy or laparoscopy is safe in pregnancy in all trimesters though there is a slight increased risk of preterm labor.[9,10] Patients undergoing exploratory laparotomy for surgical management of acute abdomen was studied in a study conducted by Unal et al. [11] Patients tolerated the procedure well. Our patient probably had threatened labor due to the bicornuate uterus rather than due to the emergency laparotomy that was performed at the start of the 2nd trimester. Prompt diagnosis and management of acute abdomen is the corner stone for optimum maternal and fetal outcome as was seen in our case.


We thank Dr. Gwalani and his team. Department of Surgery, Seth GS Medical College and KEM Hospital for performing the operation.

  1. Corsi A, Summa A, De Filippo M, Borgia D, Zompatori M. Acute abdomen in torsion of accessory spleen. Eur J Rad. 2007; 64:15-17.
  2. Bard V, Goldberg N, Kashtan H. Torsion of a huge accessory spleen in a 20-year-old patient. Int J Sur Case Rep. 2013; 5(2): 67–9.
  3. Ozeki M, Asakuma M, Go N, Ogura T, Inoue Y, Shimizu T, et al. Torsion of an accessory spleen: a rare case preoperatively diagnosed and cured by single-port surgery. Surg Case Rep [Internet]. 2015 Oct 7 [cited 2015 Dec 23];1. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4596154/
  4. Grinbaum R, Zamir O, Fields S, Hiller N. Torsion of an accessory spleen. Abdom Imaging. 2006 Feb; 31(1): 110–2.
  5. Seo T, Ito T, Watanabe Y, Umeda T. Torsion of an accessory spleen presenting as an acute abdomen with an inflammatory mass. US, CT, and MRI findings. Pediatr Radiol. 1994; 24(7): 532–4.
  6. Ohta H, Kohno K, Kojima N, Ihara N, Ishigaki T, Todo G, et al. A case of diaphragm hernia containing accessory spleen and great omentum detected by Tc-99m phytate scintigraphy. Ann of Nuc Med. 1999 Oct;13(5): 347–9.
  7. Soper NJ. SAGES’ guidelines for diagnosis, treatment, and use of laparoscopy for surgical problems during pregnancy. Surg Endosc. 2011 Nov; 25(11): 3477–8.
  8. Kameoka S, Ogawa S. Acute abdomen in pregnancy. JMAJ. 2001; 44(11): 496-500.
  9. Pokharel HP, Dahal P, Rai R, Budhathoki S. Surgical emergencies in obstetrics and gynaecology in a tertiary care hospital. JNMA J Nepal Med Assoc. 2013 Mar; 52(189): 213–6.
  10. Kocael PC, Simsek O, Saribeyoglu K, Pekmezci S, Goksoy E. Laparoscopic surgery in pregnant patients with acute abdomen. Ann Ital Chir. 2015 Apr; 86(2): 137–42.
  11. Unal A, Sayharman SE, Ozel L, Unal E, Aka N, Titiz I, et al. Acute abdomen in pregnancy requiring surgical management: a 20-case series. Eur J Obstet Gynecol Reprod Biol. 2011 Nov; 159(1): 87–90.

Puri J, Thakur H, Gupta AS. Successful pregnancy outcome in a case of accessory splenic torsion: A rare clinical entity. JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/01/successful-pregnancy-outcome-in-case-of.html

Non Immune Hydrops – An Enigma

Author Information

Vora P*, Jassawalla MJ**, Bhalerao S***, Bansal V****.

(* Third year resident, ** Medical Director, *** Honorary, **** Associate Professor,
Department of Obstetrics and Gynecology, Nowrosjee Wadia Maternity Hospital, Mumbai, India.)


A hydropic fetus without maternal Rh isoimmunization is indeed a rare phenomenon. Prognosis of a future pregnancy also poses a challenge to the obstetrician. We present a case of non immune hydrops fetalis (NIHF) in a Rh negative non iso-immunized mother and the challenges faced in reaching the etiology of hydrops.


NIHF is defined as excessive extra vascular collection of fluid in the interstitial compartment secondary to disruption of normal cardiovascular interstitial fluid homeostatic mechanisms.

Case Report

Twenty nine year old primigravida, conceived with assisted reproduction, was referred with an ultrasound finding of hydropic fetus at 29.4 weeks of gestation. Her hemoglobin (Hb) was 9.4 gm% and Hb electrophoresis showed no evidence of hemoglobinopathy. Her blood group (BG) was O negative, husbands BG was O positive, Indirect Coombs test was negative and Rh titres (direct and indirect) were nil. Glucose tolerance test was normal. Her TORCH titres and double stranded DNA were negative. Antinuclear Antibody was weakly positive but hematologist suggested no active management. Color Doppler (CD) at 30 weeks gestation revealed single viable fetus, in breech presentation, with an amniotic fluid index (AFI) of 12 cm, an estimated weight (EBW) of 2.5 kg, a composite gestational age of 33.5 weeks (falsely increased due to the hydrops), with scalp edema, pericardial effusion, cardiomegaly and ascites. Middle cerebral artery – peak systolic velocity(MCA-PSV) was in zone A suggesting fetal anemia (Figure 1 & 2). Fetal 2D echo was done and was normal. Steroids were given for anticipated preterm birth. Under tocolytic and antibiotic cover, cordocentesis was performed and blood was sent for direct Coombs test (DCT), TORCH PCR and Parvovirus B 19 which were negative. BG was O positive and karyotype was normal. However the Hb of the fetus was 7.3 gm% and haematocrit of 22.8. Hence intrauterine transfusion (IUT) was performed and 90 ml of fresh O negative, double packed, CMV negative, irradiated blood was transfused into the umbilical vein at the junction of the umblical cord to the placenta.



Figure 1. USG showing hepatomegaly, spleenomegaly and ascites

Figure 2. USG showing scalp edema

Figure 3 . Pre-transfusion MCA-PSV.

Figure 4. Post transfusion MCA-PSV.

CD two days post procedure showed MCA-PSV reducing and in zone B (37.3 cm/sec) with an impression of resolving hydrops. Hence a repeat CD was planned after a week. However, on the sixth day post procedure, patient complained of abdominal discomfort. On examination mild anemia with a pulse rate of 92/min and blood pressure of 120/80 mm Hg were noted. On per abdomen examination uterus was 32 weeks with breech presentation with fetal heart rate dropping to 60 beats per minute. CD revealed single viable breech with AFI of 21 cm and EBW of 2.7 kg with no retroplacental collection and MCA-PSV in zone A. An emergency lower segment cesarean section was done at 31.5 weeks of gestation. She delivered a male child, 2.6 kg with APGAR score of 5/10, 6/10. The neonate did not cry immediately after birth and was intubated. Heart rate was 180 beats per minute and respiratory rate was 40 beats per minute with shallow breathing and jerky respiration. Neonate was put on ventilator, intravenous fluids and antibiotics. Cord blood showed Hb of 8 gm%, O positive BG and bilirubin of 5mg/dl. An exchange transfusion was performed. Neonatal death occurred within six hours of life due to respiratory distress syndrome. Lactation suppression and injection Anti - D 300 microgram was given to the mother and she had an uneventful recovery.


The relative incidence of hydrops fetalis has changed dramatically in the last 2 decades due to prevention of immune related hydrops fetalis secondary to Rhesus isoimmunization by Rh anti D prophylaxis. With the use of Anti - D prophylaxis immunological causes account for
less than 20% of the cases.[1] NIHF is more common than hydrops fetalis and the incidence of NIHF in Southeast Asia varies from 1 in 500 to 1 in 1500.[1] Maternal causes of NIHF are idiopathic, alpha thalassemia, TORCH infections, thyrotoxicosis, diabetes mellitus, preeclampsia, anemia, hypoprotenemia. Fetal causes are cardiovascular diseases leading to low or high output cardiac failure, chromosomal abnormalities, thoracic masses, intrauterine infections, twin pregnancies, renal malformations, placental abnormalities, metabolic conditions.[1] Lysosomal storage disorders like mucopolysaccharidosis 7, Niemann–Pick disease, galactosialidosis, mucolipidosis and type 2 gaucher disease must be considered when dealing with recurrent hydrops. Mechanism by which these present as NIHF is still unknown.[2,3] Other rare cause of recurrent fatal fetal hydrops is nucleotide substitution in the erythrocyte Beta spectrin gene.[4] Intrauterine investigations done include CD with 2D- ECHO and MCA-PSV. In addition cord blood Hb, DCT, TORCH, parvovirus and karyotype can be offered. In utero treatment includes tertiary care management, intraperitoneal and intrauterine transfusions, fetal thoracocentesis or pericardiocentesis, transplacental drug therapy for fetal dysarrhythmias and treatment of polyhydramnios. The management at birth consists of vigorous and zealous resuscitation, appropriate treatment with fluids, diuresis, dialysis, treatment of cardiac failure, partial or total exchange transfusions. It is also important to screen for metabolic disorders when other common etiologies have been excluded.[1,5] Though rare, obstetricians must keep in mind the possibility of a neonate with NIHF in a Rhesus non immunized mother.

  1. Begum N, Kazal RK, Anwary SA, Nahar KN, Shamsunnahar PA, Akhter N. A neonate with Non –immune Hydrops Fetalis in Rh Non-immunized Mother. Bangladesh J Child Health 2010; 34(2): 70-72.
  2. Van de Kamp JM, Lefeber DJ, Ruijter GJ, Steggerda SJ, den Hollander NS, Willems SM, et al. Congenital disorder of glycosylation type 1a presenting with hydrops fetalis. J Med Genet 2007; 44(2): 277-280.
  3. Cheng Y, Verp MS, Knutel T, Hibbard JU. Mucopolysaccharidosis type 7 as a cause of recurrent non-immune hydrops fetalis. J Perinat Med 2003; 31: 535-537.
  4. Gallagher PG, Weed SA, Tse WT, Benoit L, Morrow JS, Marchesi SL et al. Recurrent fatal hydrops fetalis associated with a nucleotide substitution in erythrocyte beta-spectrin gene. J Clin Invest 1995; 95(3): 1174-1182.
  5. Fatusic Z. Nonimmune Hydrops Fetalis. Donald School Journal of Ultrasound in Obstetrics and Gynecology 2007; 1(1): 105-110.

Vora P, .Jassawalla MJ, Bhalerao S, Bansal V. Non Immune Hydrops – An Enigma. JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/02/non-immune-hydrops-enigma.html

Abdomino-Pelvic Mass In a Teenager - a Diagnostic Dilemma

Author Information

Anuranjani L*, Ostwal P**, Chauhan AR ***.
(* Fourth year resident, ** First year resident, *** Additional Professor and Unit Head.
Department of Obstetrics and Gynecology, KEM Hospital, Mumbai, India.)


Ovarian fibromas are benign ovarian tumorswith incidence of 1-4.7%. Generally found in perimenopausal and menopausal females, they are rarely found in premenarchal teenagers. As they present with nonspecific symptoms they are often misdiagnosed as fibroid or malignant ovarian tumours. Clinical features, ultrasonography in adjunction with tumorsmarkers remain the best preoperative approach to aid diagnosis. Though benign in nature, occasionally raised carcinoma antigen 125 (CA-125) and Meig’s syndrome may be associated and result in poor prognosis. Surgical excision is the treatment of choice and histology remains the gold standard for diagnosis. We report an atypical case of large ovarian fibroma encountered in a premenarchal teenage girl. The case highlights the potential of misdiagnosis due to its presentation, age of occurrence and absence of characteristic symptoms.


Innumerable pelvic masses confront the gynecologist and often lead to diagnostic dilemma. Among these, masses arising from ovaries are not easily identified due to their anatomical location or dynamic nature until they present with symptoms. Amidst the classification of ovarian tumors, ovarian fibromas belong to the group of sex cord stromal tumors; histologically they have stromal origin and are composed of fibroblastic spindle cells which produce collagen. Ovarian fibromas are benign neoplasms of ovary with occurrence of 1 to 4.7%.[1, 2] Though the incidence is low, they are the most common benign solid primary tumors of the ovary.[1] Presenting age is perimenopausal or postmenopausal and rarely below 20 years of age.[1, 2] Even though there is rare documentation of occurrence in a 13-and a 8-year-old patient, till now very few cases have been reported in premenarchal age [1]. Though mostly asymptomatic they may present with nonspecific symptoms like abdominal pain or mass.[1] Generally unilateral and solid in nature, cystic fibromas may also occur.[1, 2] Being solid tumors they are often misdiagnosed as fibroid or as malignant ovarian tumor if ascites and raised CA-125 is found.[3, 4] Due to these reasons, it poses a diagnostic dilemma if encountered in teenage years.

Case Report

A 13 year old unmarried, premenarchal girl presented in our OPD with intermittent pain in abdomen since 20 days. She noticed a lump in the lower abdomen 15 days ago and complained of intermittent fever since 1 month. There was no history of breathlessness, vomiting, edema or any bowel or bladder complaints. No significant medical, surgical or family history was found. On general examination, the patient was afebrile with stable vital signs. Pallor was present while no icterus, cyanosis, lymphadenopathy or edema was found. Both breast and pubic hair were Tanner stage III. Systemic examination did not reveal any abnormality. On abdominal examination, a midline mass around 16 weeks, tender, firm to hard in consistency with restricted mobility was present. No ascites was demonstrated. On local examination hymen was intact. Urine pregnancy test was negative and preliminary diagnosis of fibroid or remote possibility of a hematometra was made.

Trans-abdominal sonography revealed a solid ovarian tumor of 13 x 10 x 9 cm in the right iliac fossa with arterial and venous flow having resistance index (RI) of 0.3. Uterus was normal in size with an ill-defined endometrial echo. The left ovary could not be visualized. A possible diagnosis of malignant ovarian tumor was made. Computed tomography scan reported a smooth surfaced, solid-cystic lesion on left side measuring 6.5 x 9.4 x 11 cm with vascular pedicle arising from left ovarian vessel. Right ovary and uterus were normal. Few sub-centimeter lymph nodes and minimum free fluid in the pelvis was present. A left ovarian neoplasm of malignant nature was suspected. Based on these suspicions, tumor markers were investigated: CA-125, CEA, β-hCG, AFP were found to be within normal limits. Apart from the routine investigations, chest X-ray, ESR and serum testosterone were also within normal limits. LDH was raised (491.6 IU/L).

With a probable diagnosis of left ovarian tumour of malignant origin, patient underwent exploratory laparotomy after a valid, well informed consent from her parents. A smooth, solid mass of 10 x 12 cm arising from the left ovary with dilated vessels was found as seen in Figure 1. No torsion or adhesions were found. The right ovary and both fallopian tubes were normal. Uterus was infantile. Around 10 ml peritoneal fluid was aspirated and sent for cytology. Omental biopsy was done. Undersurface of liver, diaphragm and colon were normal. Para-aortic lymph nodes could not be palpated. Patient underwent left salphingo-opherectomy and the procedure was uneventful. Patient was discharged on post-operative day 7.

Figure 1: Intraoperative findings.

Peritoneal fluid cytology did not reveal any malignant cells. Histopathological report on gross examination showed 10 x 7.5 x 4 cm ovary with smooth surfaced, encapsulated, solid mass with dilated vessels. Cut section showed grey white to yellow areas with few cystic lesions in the ovarian tumor (Figure 2). The initial diagnosis of spindle cell neoplasm suggesting sex cord stromal tumor was given; the final diagnosis was left ovarian fibroma with no atypia or malignancy.

Figure 2: Cut section of ovarian fibroma.


Fibromas are benign stromal tumors composed of spindle, oval or round collagen producing cells arranged in storiform (cartwheel like) pattern. Pathogenesis is obscure but trisomy and tetrasomy 12 have been suggested.[6] These are generally unilateral, solid, mobile tumors of variable size with bilaterality of 0-11.7% [3]. On histopathology, a rare variant called cellular fibroma comprising of absent or minimal nuclear atypia with 1-3 mitoses/10 HPF has been reported. Another variant with incidence < 1% showing moderate nuclear atypia and > 4 mitoses/ 10 HPF having malignant potential is designated as a fibrosarcoma. [2, 3]
Generally diagnosed on ultrasonography as solid masses, fibroma usually appear as homogeneous solid tumors with delayed enhancement on CT. MRI appearance is of a hypo intense mass on T1-weighted images with very low signal intensity on T2-weighted images [1]. The treatment of these tumors is surgical where tumorectomy can be done for young patients or radical treatment for perimenopausal and postmenopausal patients. Even though we performed exploratory laparotomy there are reports of laparoscopic resections with good recovery and shorter hospital stay [3]. A rare but well known syndrome encompassing ascites, pleural effusion and benign solid ovarian tumor known as Meig’s syndrome is found in 1% ovarian tumors and have good prognosis.[2, 5] Ascites and hydrothorax regress after tumour removal.[2] Meig’s syndrome has been commonly found with ovarian fibromas (1-10%).[2, 4] Laparoscopic removal of large ovarian fibroma with torsion and Meig’s syndrome have been reported.[4] There are also documentations of rare associations with Gorlin-Goltz syndrome (multiple basal cell carcinomas, odontogenic keratocysts, calcified falx cerebri, shortened 4th metacarpal), Gardener’s syndrome (multiple adenomatous polyps in colon, sebaceous cysts, osteomas) or Peutz Jeghers syndrome (hereditary intestinal polyposis, melanosis).[1, 2] A relationship between haemolytic anemia and benign ovarian tumors with complete resolution of haemolytic anemia after tumor removal has been reported;[4] however our patient had only mild anemia.


For a solid or cystic adnexal mass where the primary consideration is commonly an epithelial tumor, the possibility of a stromal tumor should also be considered.[1] Due to its rarity and presentation it is often misdiagnosed. Clinical findings, ultrasonography and tumor markers remain the best preoperative approach currently available. Surgical resection is the treatment of choice and histopathology is the gold standard for diagnosis. Prognostic factors have not been fully characterized.

  1. Yen P, Khong K, Lamba R, Corwin MT, Gerscovich EO. Ovarian Fibromas and Fibrothecomas, Sonographic Correlation with Computed Tomography and Magnetic Resonance Imaging: A 5-Year Single-Institution Experience, J Ultrasound Med Jan 2013; vol 32, No.1: 13-18.
  2. Chechia A, Attia L, Temime RB, Makhlouf T, Koubaa A. Incidence, clinical analysis and management of ovarian fibromas and fibrothecomas. Am J Obstet Gynecol. 2008 Nov; 199(5): 473.e1-4.
  3. Son CE, Choi JS, Lee JH, Jeon SW, Hong JH, Bae JW. Laparoscopic surgical management and clinical characteristics of ovarian fibromas. JSLS. 2011;15(1):16–20..
  4. Maccio A, Madeddu C, Kotsonis P, Pietrangeli M, Paoletti AM. Large twisted ovarian fibroma associated with Meig’s syndrome, abdominal pain and severe anaemia treated by laproscopic surgery; BMC Surgery 2014; 14: 38.
  5. Shetty PK, Bafna UD, Balaiah K, Gnana PS. Ovarian Fibroma with Meig’s Syndrome associated with elevated CA-125- A Rare Case. Online J Health Allied Scs. 2010; 9 (2):17.
  6. Roth LM. Recent advances in the pathology and classification of ovarian Sex Cord Stromal Tumors. International Journal of Gynaecological Pathology 2006; 25:199-215.
Anuranjani L, Ostwal P, Chauhan AR. Abdomino-Pelvic Mass In a Teenager - a Diagnostic Dilemma. JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/01/abdomino-pelvic-mass-in-teenager.html