Management of Ectopic Ovary in the Setting of Mullerian Agenesis [35P

Abstract

INTRODUCTION: Mullerian agenesis can be associated with a complex array of other congenital anomalies, including ectopic ovaries. As a rare finding, clinical recommendations are not clear cut, therefore we present a case report and literature review to develop recommendations for clinical management of an ectopic ovary in the setting of complete Mullerian agenesis. METHODS: An adolescent female with primary amenorrhea underwent complete evaluation at an academic reproductive endocrinology clinic. Clinical management recommendations were developed based on literature review. RESULTS: Physical examination of the 16-year-old amenorrheic female revealed a short blind vagina, fullness of the left inguinal canal, normal sexual hair distribution and breast development. Further evaluation with pelvic MRI demonstrated absence of the uterus, normal right ovary, and ectopic ovarian gonad located in the left inguinal canal. She was additionally found to have absence of the left kidney and a hypertrophic right kidney with a single ureter. We performed extensive literature review to develop clinical recommendation on ectopic ovary in patients with complete Mullerian agenesis, also known as Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. The considerations of gonadal conservation, risk of malignancy, and indications of ovarian transposition were evaluated in preparation of management recommendations. CONCLUSION: Ovarian conservation without ovarian transposition is an optimal clinical management in the case of unilateral ectopic ovary in patients with Mullerian agenesis. Absence of significant increase of malignant transformation should be discussed with these patients. A review of possible oocyte recovery from an ectopic ovary and role of assisted reproductive technology is highly recommended to diminish patient anxiety about future fertility.