Mayer-Rokitansky-Küster-Hauser syndrome with inguinal hernia, left renal fusion, and malrotation: a rare case

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital abnormality of sexual development characterized by agenesis of the uterus and vagina. It may be confined to the genital tract (MRKH type I) or be accompanied by abnormalities of the urinary or skeletal systems (MRKH type II). We report a case of an 8-year-old girl who was admitted to the hospital because of a "reducible mass in the left groin." Upon examination, a mass of about 3 × 2 × 2 cm³ in size was found in the left inguinal region on increasing abdominal pressure, and a left inguinal oblique hernia was diagnosed. The vulva developed normally, and the vaginal vestibule had only a urethral opening but no vaginal opening; abdominal ultrasound revealed that the right kidney was absent, and the left kidney seemed to be fused by two renal cavities; ultrasound of the uterine appendages showed no uterus, and the left and right ovaries were normal; thus, the patient was diagnosed with MRKH type II syndrome. Laparoscopic examination verified the above findings, and it was also found that no obvious fallopian tubes were found around both ovaries. High ligation of the left inguinal hernia was performed to treat the inguinal oblique hernia. When a patient with MRKH syndrome presents with an unexplained inguinal mass, an ovarian or uterine inguinal hernia should be suspected. Early detection of this anomaly will aid in reproductive outcome management and appropriate surgical management.