Persistent müllerian duct associated with transverse testicular ectopia

Abstract

A rudimentary uterus and Fallopian tubes, persistent Müllerian duct syndrome (PMDS), associated with transverse testicular ectopia (TTE) was found in a phenotypically normal boy with 46, XY karyotype who presented with bilateral undescended testes. Catheterization of the uterus confirmed its connection to the urethra. Because of the close vicinity of the rudimentary uterus to the vas deferens and bladder neck in PMDS, in spite of infertility in the majority of these patients, hysterectomy should be avoided to prevent iatrogenic damage to the vas and bladder neck. In TTE associated with bilateral and sometimes unilateral maldescent, abdominal exploration may be justified to exclude the possibility of PMDS and to facilitate orchiopexy.