Pediatric Infectious Diseases: Open Access

Abstract

Disorders of sex development (DSD) is affecting 1 in 10,000 births. Current management is very heterogeneous because of the low volume of patients for persistent Mullerian duct syndrome (PMDS). The persistence syndrome of Mullerian derivatives is a rare form of abnormalities of sex development. The persistence of Mullerian derivatives can be seen as Persistent Mullerian duct syndrome: intraoperative diagnosis in case of cure of a hernia in a boy normally virilized with the discovery of a fallopian duct or a uterus when opening the hernia sac during a laparoscopy, the treatment of a nonpalpable testicle. The treatment is surgical and some author recommends dissection to separate the vas Mullerian duct, orchidopexy and mullerian duct excision. This treatment is done by laparoscopy and is consist of a longitudinal section of the uterus and the cervix to allow lowering each testicle by an internal pathway within the umbilical artery and preserving the testicular vasculature. Four patients admitted in our department of pediatric urology for persistent Mullerian duct syndrome from 2015-2017. After laboratory and radiology work-up, laparoscopy exploration is indicated in all cases. Our option is done by laparoscopy and will consist of a longitudinal section of the uterus, and the cervix to allow lowering each testicle in the corresponding hemi-scrotum by an internal pathway within the umbilical artery and preserving the testicular vasculature and the vas. This is our attitude towards the PMDS syndrome.One case diagnosis of PMDS either inguinal hernia repair, one case either urethrocystoscopy for utero hydronephrosis bilateral, one non palpable testis in two cases.Laparoscopy is best way for PMDS diagnosis. Division of Mullerian duct is a safe way to do orchidopexy without damage of the vas.