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

A 20-year-old female presented with a chief complaint of bilateral reducible inguinal swelling for the past 5–6 months. The patient also reported mild lower abdominal pain that worsened with activities such as coughing and weightlifting. She had primary amenorrhoea but was sexually active without dyspareunia or coital difficulties. On physical examination, the patient’s spine and gait were normal, and secondary sexual characteristics were well developed (Sexual Maturity Rating Stage 5). Local examination revealed bilateral inguinal hernias and a blind-ending vagina, but the cervix was not visualised and no palpable uterus. A per-abdomen examination revealed a cough impulse over the bilateral inguinal region. The hormonal profile showed mildly elevated luteinising hormone at 14.1 mIU/mL (reference range 2–12 mIU/mL) while follicle-stimulating hormone, testosterone and oestradiol were at normal levels. A urine pregnancy test was negative. Karyotyping confirmed a 46 XX genetic makeup. Imaging studies, including ultrasound and magnetic resonance imaging, did not visualise the uterus but showed small ectopic ovaries in the bilateral inguinal regions [ Figure 1 ]. Based on these findings, the patient was diagnosed with MRKH syndrome with bilateral inguinal hernias containing adnexa, and a diagnostic laparoscopy was performed after obtaining informed patient consent. Magnetic resonance imaging images with axial and sagittal T2-weighted images (a and b) showing bilateral ovarian and rudimentary horns (white arrows) at the inguinal canal In this case, diagnostic laparoscopy revealed normal ovaries and fallopian tubes on both sides. The left ovary was densely adhered to the deep inguinal ring, presenting as a solid nodule during clinical examination. Surgical management proceeded with transabdominal pre-peritoneal hernia repair, with the decision to use mesh hernioplasty due to the large size of the deep ring defect. On entering the peritoneal cavity, both rudimentary uterine horns were found entering the inguinal canal. The rudimentary uterus, ovaries and fallopian tubes were reduced into the peritoneal cavity by dividing the round ligament. Both adnexal structures were plicated to prevent ovarian torsion. The procedure concluded with the placement of two meshes in the pre-peritoneal plane, fixed with absorbable tackers and the peritoneal flaps were closed using barbed PDS sutures [ Figure 2 ]. The patient had a normal post-operative recovery and was discharged after 72 h. The patient was also offered and counselled about assisted reproductive techniques, such as surrogacy, oocyte and embryo cryopreservation, but she declined. Diagnostic laparoscopy revealing ovary and rudimentary horns protruding through the right and left inguinal canals (a and b). (c and d) The reduction of the adnexal contents, followed by the placement of prolene mesh on both sides (e and f)

Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare congenital disorder characterised by the agenesis of the Müllerian ducts, resulting in the absence of the uterus and the agenesis of the vagina. It typically presents in adolescent females with primary amenorrhoea despite normal secondary sexual characteristics and a 46 XX karyotype. The incidence of MRKH syndrome is approximately 1 in 4500 female births. This condition can be associated with renal, skeletal, auditory and, in rare cases, ovarian anomalies, but these abnormalities were not present in the present case.[ 1 ] Herniation of the uterus and adnexa has been reported in up to 31% of indirect hernial sacs among female infants. The incidence decreases with age and becomes very rare in adolescent and adult females.[ 1 ] This case presents the rare combination of MRKH syndrome with bilateral adnexal structures in inguinal hernias.

The coexistence of MRKH syndrome and bilateral inguinal hernias containing adnexal structures is extremely uncommon. Early diagnosis and management of such cases are essential to prevent complications like ovarian torsion. This case highlights the importance of recognising MRKH syndrome presentations for timely and effective treatment. The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. There are no conflicts of interest.

Inguinal hernia of the ovary and fallopian tube is commonly seen in infancy and typically presents within the first 2 years of life.[ 2 ] A short inguinal canal, the oblique orientation of the canal in the abdominal wall and the presence of a diverticulum of Nuck are anatomical conditions that can predispose to adnexal entrapment, leading to inguinal hernias involving Müllerian structures. Theories explaining this phenomenon include the non-fusion of the Müllerian ducts, resulting in hypermobility of the ovary or congenitally elongated ovarian ligaments. In addition, a patent processus vaginalis, also known as the canal of Nuck, which typically closes by 1 year of age, may contribute to the development of this condition.[ 3 ] MRKH syndrome exists on a spectrum of varied presentations but can broadly be divided into two types. Type 1, which accounts for 44% of cases, involves an isolated Müllerian abnormality, specifically the congenital absence of the uterus and vagina. Type 2, representing 56% of cases, includes Müllerian duct agenesis along with renal, cardiac, muscular and vertebral defects.[ 4 ] This case represents type 1 MRKH syndrome, involving only a Müllerian duct fusion abnormality.