Persistent Mullerian duct syndrome is a disorder of male pseudo-hermaphroditism characterized by persistence of uterus, fallopian tubes and upper two third of vagina in otherwise normally virilized phenotypically and genotypically male (46XY). Aim of study was to create an awareness among the surgeons about the pathophysiological, morphological and clinical presentations of this rare entity along with its management. Between 2009 to 2013 we came across 5 boys with Persistent Mullerian Duct Syndrome (PMDS) with varied clinical spectrum in each, viz hernia, hydrocele, right sided Un-Descended Testis (UDT), left sided UDT and bilateral UDT. All were diagnosed intra-operatively and managed accordingly, 4 by Open Orchidopexy (OP) with 1 requiring midline splitting of the uterus and 1 laparoscopic orchidopexy. Awareness of this rare anomaly associated with inguinal hernia or undescended testis and also diagnostic laparoscopy for impalpable testis helps in appropriate management.
Persistent Mullerian duct syndrome, Hernia uteri inguinale, Undescended testes