Persistent Müllerian Duct Syndrome with Transverse Testicular Ectopia: A Case Report with Literature Review

Abstract

A 39-day-old boy was admitted with a complaint of left inguinal swelling. A left-sided inguinal hernia with bilateral cryptorchidism was found on physical examination. During the exploration of the left inguinal region, pulling of the left gonad in the hernia sac caused protrusion of another gonad (Figure 1). The macroscopic appearance of both gonads was testis associated with fimbria-like structures. Both gonads had vas deferenses and vascular supplies. A right inguinal exploration showed no other gonad. The vas deferenses and vascular supplies of both gonads associated with a uterus-like structure located midline and posterior to the urinary bladder were determined in the abdominal exploration performed through a Pfannenstiel incision. Biopsy specimen were taken from both gonads and the left fimbria-like structure. Frozen section showed them to be testes and tubal fimbriae (Figures 2a, 2b). Histologic appearance of both testes was normal for age. Orchiopexy was performed to the respective sides of both testes. All the other structures associated with the testes were preserved. The chromosome analysis of peripheral leukocytes showed a normal 46,XY karyotype. The postoperative follow-up period was uneventful.