Transverse Testicular Ectopia and Persistent Mullerian Duct Syndrome: Video-assisted Treatment

Introduction

Persistent Mullerian duct syndrome (PMDS) is a rare autosomal recessive disorder in which, due to a deficiency of the Mullerian inhibiting substance (MIS), the complete development of the Mullerian duct structures occurs in the presence of a male phenotype and a 46 XY karyotype. In these patients, a uterus, tubes and vagina are often present which open into a normal male prostatic urethra. These female structures are usually found during surgical correction of an inguinal hernia or during a laparoscopic exploration for non palpable testis. Transverse testicular ectopia (TTE) is frequently present.

Here we present the case of a 6-month-old boy with this rare association and its treatment by video-assisted excision of the Mullerian remnant, video-assisted orchidopexy and laparoscopic closure of the inguinal defects.

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