86. Unilateral Ovarian Agenesis: A Systematic Review of the Literature and Report of Two Cases

Abstract

<h3>Background</h3> Ovarian agenesis (OA) is a rare and often incidental finding. Noncompeting embryological, vascular, and mechanical hypotheses comprise possible etiologies, though exact predisposing factors remain unknown. Here, we report two cases of unilateral OA (UOA) and results from the first systematic review. <h3>Case</h3> A 15-year-old girl with history of nephrolithiasis and pelvic pain presented for diagnostic laparoscopy due to suspected endometriosis. Three prior ultrasounds demonstrated normal-appearing ovaries with right corpus luteum cyst. Surgery revealed normal round ligaments, normal postpubertal uterus, right fallopian tube with paratubal cyst and enlarged right ovary. The left ovary, left distal fallopian tube, and left uteroovarian ligament were absent. Multiple peritoneal biopsies were positive for endometriosis. Right paratubal cyst was removed. Postoperative MRI did not identify a heterotopic left ovary. The second patient is a 30-year-old G3P1011 with a history of genital chlamydia, prior laparotomy to retrieve a foreign body, and right tubal ectopic pregnancy treated medically two years prior. She presented with severe abdominal pain and positive pregnancy test. An ultrasound demonstrated normal bilateral ovaries and a cystic right adnexal structure concerning for ectopic pregnancy. Patient elected for unilateral salpingectomy. Operative findings were significant for bleeding gestational sac extruding from the broad ligament. The left ovary and round ligaments were normal, but the right ovary, distal fallopian tube and infundibulopelvic ligament were absent. In both cases a second attending physician was consulted intraoperatively to confirm the absent ovary. <h3>Comments</h3> We conducted a systematic literature review on OA on 6/29/20 using Cochrane Library, Medline, Embase, Google Scholar, Scopus, and Web of Science databases, resulting in 66 relevant articles with 85 cases of OA. Descriptive statistics and univariate analysis were performed in SAS and Excel. The average age of diagnosis was 23.8 years. Ultrasound was the most common imaging technique (49%), and the majority of cases had laparoscopic surgery (65%). Of the cases that mentioned pre-operative imaging results, 13/51 did not mention ovarian anomalies. Abdominal/pelvic pain (27%), and infertility/subfertility (21%) were the most common presenting symptoms. UOA was the most common type of OA (96%) with both sides equally affected. Some ipsilateral fallopian tube absence was present in 81% cases. Approximately 26% of cases had concomitant uterine abnormalities, while 20% had renal abnormalities. These findings are important as they may guide workup and follow-up for females if ovarian agenesis or heterotopic ovarian location is diagnosed.