Abstract Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare congenital anomaly characterized by agenesis or hypoplasia of the uterus and upper vagina in phenotypically normal females with a 46, XX karyotype. A 37-year-old female with known MRKH syndrome and prior vaginoplasty presented with an emergency irreducible left inguinal hernia. Preoperative computed tomography suggested herniation of the left fallopian tube and ovary with mesosalpinx. Emergency open surgery revealed a rudimentary uterus with bilateral fallopian tubes and ovaries within the hernia sac. All structures were viable. CT imaging aids emergency decision-making but may underestimate hernia contents in emergency settings. Surgical exploration based on clinical judgment remains essential. MRKH syndrome may rarely present as emergency inguinal hernia. Early surgery and appropriate approach selection ensure safe outcomes.
Vardhan et al. (Thu,) studied this question.