Mayer-Rokitansky-Kuster-Hauser syndrome with bilateral inguinal hernia of the adnexa - A rare coexisting anomaly.

Abstract

Abstract: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder characterised by the absence of the uterus and upper vagina. It typically presents in adolescent females with primary amenorrhoea despite normal secondary sexual characteristics and a 46 XX karyotype, with an incidence of 1 in 4500 female births. A 20-year-old married female presented with a chief complaint of bilateral inguinal swelling persisting for the past 5-6 months. She had primary amenorrhoea and was sexually active without dyspareunia or coital difficulties. A general examination was normal. Bilateral reducible inguinal hernias, measuring 4 cm × 5 cm (left) and 2 cm × 2 cm (right), with a positive cough impulse, were noted. Hormonal parameters were normal. Karyotyping showed a normal 46 XX in all cells. Magnetic resonance imaging revealed the absence of the uterus, with both ovaries present in the inguinal canals. Diagnostic laparoscopy confirmed the absence of the uterus, a wide left deep inguinal ring with herniating ovary and Müllerian structures, and an open right deep inguinal ring with a herniating ovary and Müllerian nodule. Hernia reduction followed by pre-peritoneal space dissection was performed. A 10 cm × 15 cm non-absorbable polypropylene mesh (Ethicon) was placed and fixed with tackers. The left round ligament was divided to prevent future recurrence of the hernia. Early diagnosis and management of such cases are essential to prevent complications like ovarian torsion. This case underscores the importance of recognising MRKH syndrome presentations for timely and effective treatment.