Totally extraperitoneal repair of inguinal hernia containing Mullerian remnants in a patient with Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

BACKGROUND: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by aplasia of the uterus and upper part of the vagina in females with normal secondary sex characteristics. Inguinal hernia containing ovaries, fallopian tubes and uterus occurs rarely in adult females and its association with MRKH syndrome is even more rare and probably unreported.

CASE PRESENTATION: A 43-year-old female who had already undergone a vaginoplasty 20 years ago presented to our clinic with left groin swelling and pain for 3 weeks. Physical examinations revealed a 3-cm palpable mass in the left inguinal region that was difficult to reduce into the abdominal cavity. The diagnosis of MRKH syndrome was considered along with inguinal hernia according to the intraoperative findings within the hernia sac. Although most of the MRKH patients with inguinal hernia underwent open surgery, totally extraperitoneal repair (TEP) operation and mesh placement was performed on this patient and the resected specimen proved to be rudimentary uterus and left oviduct. The patient was discharged 2 days after surgery and free of any complications at 6 months of follow-up.

CONCLUSION: MRKH syndrome may remain undiagnosed until incidentally discovered during surgery for unrelated conditions. TEP approach offers excellent visualization, avoids intraperitoneal adhesions, safe resection of Mullerian remnants and preserves peritoneal integrity for future fertility options.