Vaginal Atresia, Left Unicornuate Unicollis Uterus, Left Pelvic Ectopic Single Kidney with Right Inguinal Hernia Containing Ovary in a 14 y Old Female: A Rare MRKHS Variant

Introduction: Vaginal atresia is a component of Mayer–Rokitansky–Küster–Hauser syndrome (MRKHS), which includes variable Mullerian duct abnormalities with normal secondary sexual characteristics. Associated renal anomalies are present in about 30% of cases(1)

Objective: To present a rare variant of MRKHS in a14-y old female with a right ovary in a right inguinal hernia and renal anomalies managed bysigmoid vaginoplasty.

Case Description: A 14-year-old girl presented with primary amenorrhea and cyclic abdominal painone year before, pelviabdominal swelling, right inguinal hernia, and well-developed secondary sexual characteristics with a single introital opening. Investigations revealed a left ectopic pelvic single kidney, hematometra with left hematosalpinx and right-sided inguinal hernia containing the right ovary with a left ovarian cyst. The first procedure included diagnostic laparoscopy and cystoscopy with the combined repair of the right-sided inguinal hernia and laparoscopic drilling of the left ovarian chocolate cyst. The patient was diagnosed with unicornuate unicollis uterus, left hematosalpinx with complete vaginal atresia associated with a left ectopic pelvic single kidney. One month later, sigmoid vaginoplasty was done. At the 3-month follow-up, there was a full-thickness neovaginal prolapse which was managed surgically by resection.

Conclusion: To our knowledge, this is the first description of this variant of MRKH syndrome association, includingvaginal atresia, left unicornuate unicollis uterus with ipsilateral left single pelvic ectopic kidney with right-sided inguinal hernia containing right ovary, in the literature. Renal assessment in cases of vaginal atresia and other mullerian anomalies is mandatory for detecting and managing such anomalies and preserving renal function. Anatomical and functional assessment of the urogenital system is mandatory, and multiple modalities can be integrated to get proper results. Diagnostic laparoscopy and cystoscopy give invaluable information in these cases and can add therapeutic aids.