The Journal of Obstetrics and Gynaecology of India
did-you-know
Clinical Pearls of JOGI SERIES OF WEBINARS Click her to view
VOL. 71 NUMBER 2 March-April  2021

Rare Case of Bilateral Ovarian Lymphangioma with Chylous Ascites in Pregnancy with Review of Literature

Rana A. Choudhary1 · Priyanka H. Vora1 · Kedar K. Deodhar2 · Sanket V. Pisat3 · Manjusha K. Ganla3 · Kedar N. Ganla1

Rana A. Choudhary is a Consultant Obstetrician and Gynaecologist, Department of Reproductive Medicine, Ankoor Fertility Clinic, Mumbai, India; Priyanka H. Vora is a Consultant Obstetrician and Gynaecologist, Department of Reproductive Medicine, Ankoor Fertility Clinic, Mumbai, India, Kedar K. Deodhar is a Professor, Department of Pathology, Tata Memorial Hospital, Dr. E. Borges Road, Mumbai, India; Sanket V. Pisat is a Consultant Gynaec Endoscopic (Laparoscopic & Hysteroscopic) Surgeon, Ankoor Fertility Clinic, Mumbai, India; Manjusha K. Ganla is a Consultant Anaesthetist, Ankoor Fertility Clinic, Mumbai, India; Kedar N. Ganla is a Consultant Obstetrician and Gynaecologist, Department of Reproductive Medicine, Ankoor Fertility Clinic, Mumbai, India.

Rana A. Choudhary - dr.ranakhan@gmail.com

  1. Department of Reproductive Medicine, Ankoor Fertility Clinic, Mumbai 400016, India
  2. Department of Pathology, Tata Memorial Hospital, Dr. E. Borges Road, Mumbai, India
  3. Ankoor Fertility Clinic, Mumbai, India
  • Download Article
  • Email Article
  • Print Article
  • Whatsapp Article

About the Author


Dr. Rana A. Choudhary completed her under graduation from Seth G.S. Medical College and K. E. M. Hospital, Parel and later pur-sued  her  post-graduation  in  Obstetrics  and  Gynaecology  from L.T.M.M.C and L.T.M.G. Hospital, Sion Mumbai. She has a keen interest in Reproductive Medicine  and  Infertility  and  went on to do Maters in Repro-ductive Medicine from Homer-ton University, UK and Diploma in Reproductive Medicine from Germany. Due to her interest in the field of research, she worked at the National Institute for research in reproductive health (ICMR) for over 2 years. She has done Diploma in Clinical Research and is actively involved  in  various  research  projects.  She  is  currently  Consultant  Obstetrician, Gynaecologist and Reproductive medicine specialist at Wockhardt. Masina, Jagjivanram Hospital and Ankoor Fertility Clinic, Mumbai.

Lymphangiomas are rare benign tumors caused due to proliferation of the lymphatic vessels. Although they can occur anywhere in the body; head-neck region and axilla are common locations. Only around 20 cases of primary ovarian lymphangiomas have been reported in literature. We report a case of 36 year old female with incidental finding of bilateral ovarian lymphangiomas and chylous ascetic fluid diagnosed at caesarean delivery. As diagnosis was not confirmed, oophorectomy was not done. She came back after a week with breathlessness, abdominal distension (chylous ascetic fluid) and pleural effusion due to the lymphangiomas of the ovary. When reviewed retrospectively, removal of the ovaries during cesarean section should be recommended in such cases.

Case

A 36-year-old primigravida underwent elective caesarean section at 38 weeks of gestation for breech presentation. She had conceived after in vitro fertilization (IVF) done for poor ovarian reserve (Sr. Anti-mullerain hormone <   0.03 and Antral follicular count-3) with donor oocytes. Her antenatal course was uneventful. During her caesarean delivery, upon opening the peritoneum, chylous ascitic fluid was noted in the abdomen. The fluid was collected and sent for routine microscopy, cytology and culture sensitivity testing. Around 400 ml of chylous ascitic fluid was drained. She delivered a healthy baby boy (3 kg) with good APGAR score. The uterus and both fallopian tubes appeared normal. However, bilateral ovaries were bulky (right ovary-8 ×   7  ×   5 cm, left ovary-7 × 6 × 5 cm) with chylous fluid oozing from both the ovaries (Fig. 1). Husband and relatives were explained about the intraoperative findings. After taking their consent, bilateral ovarian tissue biopsies was taken and sent for histo-pathology. Other organs like the stomach, pancreas, jejunum, mesentery, etc. did not show any abnormality or growth. An abdominal drain was kept. The drain had 200 ml of milky serosanguinous fluid on the first post-operative day, which reduced to less than 20 ml on fifth day. The patient was discharged after removal of abdominal drain and advised to follow-up after 7 days. Her investigations showed the fol-lowing results as seen in Table 1.

However, on day 9 of delivery, she came with breath-lessness and severe abdominal distension. Her vitals were stable with oxygen saturation of 96% and reduced air entry on the right lower side of chest with ascites. Her chest x-ray revealed moderate pleural effusion on the right side. Ultra-sonography of the abdomen and pelvis showed moderate ascites with enlarged ovaries suggestive of lymphangiomas. The patient was readmitted. Around 3 l of chylous ascetic fluid was removed and another 500 ml on pleural tapping. These samples were sent for further investigations which confirmed chylous fluid (Table 2). The patient was given intravenous albumin and antibiotics. She was symptomati-cally better and discharged 2 days later.

The histopathology of ovarian biopsies revealed multi-ple dilated channels in ovarian parenchyma with some con-taining eosinophilic fibrinous fluid material (Fig. 2). The channels were lined by uniform flattened endothelial cells and separated by moderate cellular eosinophilic stroma sug-gestive of bilateral ovarian lymphangiomas with differential diagnosis of hemangiomas or adenomatoid tumours (Fig. 2). The immunohistochemistry showed ovarian tissue lining endothelial cells positive for CD31 and focal weak positiv-ity for D2 40, while equivocal for CD34. The cells were cytokeratin and vimantin negative, with no mitotic activ-ity, which was consistent with diagnosis of bilateral ovarian lymphangiomas (Fig. 2).

The patient had recurrence of symptoms after 3 weeks and presented with breathlessness and abdominal disten-sion. On examination, her vitals were stable, with oxygen

saturation of 94%, severe ascites and reduced air entry on the right side of chest. X-ray chest showed right-side moderate pleural effusion, and there was moderate to severe ascites on ultrasonography. In view of recurrence of symptoms, a decision for exploratory laparotomy was taken with the consent of the patient and her husband. Intraoperatively, 3 l of chy-lous ascetic fluid was drained. Both ovaries were enlarged and oozing chylous fluid. Bilateral oophorectomy was done and an abdominal drain kept. The other common sites for lymphangiomas like stomach, jejunum, mesentery etc. were thoroughly checked for any growth or abnormalities. Post-operative course was uneventful and she was discharged on day 5. The patient was asymptomatic on 6-month follow-up.

Discussion

Extravasation of milky chyle rich in triglycerides into peritoneal cavity leads to chylous ascites. True chylous ascites is characterized by high fat (triglyceride >   110 mg/dl). Sec-ondary chylous ascites is seen when existing clear ascitic fluid turn chylous. The literature review shows that chylous ascites during pregnancy is an extremely rare occurrence and could be due to various causes like pancreatitis, inflammation, lymphangioma, etc. (Table 3) [1, 2].

Lymphangiomas are rare benign congenital malformations of lymphatic system, but less than 1% may turn malignant [1]. Its pathogenesis is not very clear and is rarely seen in adults. Lymphangiomas occur due to obstruction of local lymph flow, and in 95% of people, it’s found in the head, neck and axillary region [1, 2]. Only 5% may have involvement of lungs, pleura, pericardium, oesophagus, stomach, jejunum, colon, pancreas, liver, gallbladder, kidney and mesentery [1, 2]. Primary ovarian lymphangioma is extremely rare with only around 20 cases

reported in the literature [1–4]. Three of them had milky ascites with ovarian lymphangioma [1–4]. One woman presented with 33 weeks of gestation with ovarian lymphangioma and torsion [1–4]. Most women were managed with total abdominal hys-terectomy with bilateral salphino-ooprectomy (TAH BSO) [1–4]. There were only 3 case reports (non-pregnant women)

of ovarian lymphangiomas with chylous ascites who under-went unilateral or bilateral oophrectomy [1, 2, 4]. There is only one reported case of pregnant woman with ovarian lymphangi-oma and chylous ascites like in our patient. She presented at 37 weeks of gestation with breathlessness and pre-eclampsia and  underwent  caesarean  delivery  with  unilateral  ovarian  oophrectomy for an enlarged left ovary (16 cm) [3].

Compliance with Ethical Standards

Conflict of interest There is no conflict of interest for the manuscript submitted.
Human and Animal Rights  The manuscript submitted for consideration does not involve any research or experiments on human participants and/or animals.
Informed Consent Informed consent was taken from the patient and her husband before submitting the manuscript for consideration to this journal.

References

  1. Iwasa T. Lymphangioma of the ovary accompanied by chylous ascites. J Obstet Gynaecol Res. 2009;35(4):812–5.
  2. Al-Busafi SA, Ghali P, Deschênes M, et al. Chylous ascites: evalu-ation and management. ISRN Hepatol. 2014;2014:240473. https ://doi.org/10.1155/2014/24047 3.
  3. Daddenavar  A,  Daddenavar  V,  Patil  R,  et  al.  Bilateral  ovarian  cystic lymphangioma with chylous ascitis in pregnancy—a rare case report with review. Int J Reprod Contracept Obstet Gynecol. 2016;5(5):1633–6.
  4. Choudhary  RA,  Vora  PH,  Deodhar  KK,  et  al.  Bilateral  lym-phangioma of ovary—a rare case report. Sch J Med Case Rep. 2016;4(10):723–5.
  • Download Aarticle
  • Email Aarticle
  • Print Article
  • Whatsapp Article