A Rare Case of Bilateral Retroperitoneal Ovaries: A Case Report and Review of the Literature
Authors: Gross Sarah , Holliday Nicolette , Holliday Candice 
Published: July 2024

Introduction: Retroperitoneal gynecologic tumors and cysts are rare. They are often asymptomatic or present similarly to their intraperitoneal counterpart with vague abdominal discomfort and distention. Our literature review revealed that many retroperitoneal cysts are found intraoperatively due to their benign predominance consistent with their ultrasound findings. The rarity of retroperitoneal gynecologic tumors and cysts creates uncertainty regarding the best practice management for general gynecologists caught off guard by their location when found intraoperatively. Rarer still are retroperitoneal ovaries. There are few cases of retroperitoneal ovary displacement in the literature, most often associated with female neonatal incarcerated inguinal hernia repair surgery. We found one case report involving an intraoperatively found retroperitoneal ovarian cystadenofibroma in a patient with a hernia repair performed in infancy. To our knowledge, there are no bilateral retroperitoneal ovaries described in the literature.

Objective: To report a rare case of bilateral retroperitoneal ovaries in an 11 year old female.

Methods: Case report.

Results: We present an 11-year-old female with a past medical history of Dandy-Walker malformation and hydrocephalus status post ventriculoperitoneal (VP) shunt with six laparoscopic VP shunt revisions who endorsed hirsutism and irregular, heavy, and painful menstrual periods. Her family history is significant for polycystic ovarian syndrome (PCOS), endometriosis, and Lynch syndrome. Physical exam revealed mild abdominal distention and lower abdominal tenderness. She had an elevated estradiol, luteinizing hormone (LH), and total and free testosterone. Normally, an elevated estradiol causes a negative feedback response that results in a low LH. Due to these abnormal results, a pelvic ultrasound was ordered to rule out a potential estrogen secreting ovarian tumor. Ultrasound revealed a large anechoic structure in the left adnexa not definitively separated from the ovary measuring 13.0 x 7.7 x 10.7 cm. Radiologic differential diagnosis included large simple cyst, hemorrhagic cyst, and serous neoplasm. A referral to an adolescent gynecologist was made where the patient presented five months later with 25-pound weight gain, constipation, and a hard belly. Repeat ultrasound was consistent with a left ovarian simple cyst with an increased size measuring 16.78 x 12.58 x 8.66 cm as well as a new finding consistent with a right ovarian simple cyst measuring 4.96 x 4.48 x 3.61 cm. Due to her history of severe abdominal adhesions secondary to multiple laparoscopic VP shunt revisions, likely endometriosis, and large ovarian cyst, she was referred to a gynecologic oncologist where she was consented for robotic assisted bilateral ovarian cystectomy and lysis of adhesions. In the operating room, extensive pelvic adhesions were lysed to reveal bilateral retroperitoneal ovaries. The right cyst was drained of clear fluid, revealing no internal complexity, and was completely excised. Persistent pelvic adhesions and cyst size prolonged the time required to excise the left ovarian cyst wall. Once open, papillary excrescences were seen inside the cyst raising concern for a borderline ovarian tumor. The decision was made to remove the cyst wall in its entirety regardless of increased risk of ovarian function compromise. Restoration of normal anatomy was not attainable, and her bilateral ovaries remained embedded in the side walls and superior to the iliac vessels. Pathology revealed a benign 8 cm right paratubal cyst and a benign 18 cm left serous cystadenoma with papillary excrescences.

Conclusions: Pelvic anatomy can be greatly distorted secondary to prior abdominal surgeries or certain pathologies including endometriosis and congenital abnormalities. Ultrasound cannot easily discriminate between intraperitoneal and retroperitoneal ovarian cysts but is often the only preoperative imaging obtained prior to cystectomy. Thus, many retroperitoneal cysts are found intraoperatively resulting in diagnostic and therapeutic challenges. Therefore, general gynecologists must be aware of risk factors associated with abnormal pelvic anatomy and prepared to request intraoperative consultation if needed to ensure optimized patient care and safety.