IPEG 2020 TOP ABSTRACT: Persistent Müllerian Duct Syndrome (PMDS) type 1 in a 46XY Male. MIS Trick to Preserve both Gonads

August 05, 2020
Presenter: Marcela Bailez, MD
Email: mariamarcelabailez4@gmail.com

Authors: Emiro Redondo, MD; Pablo D`Alessandro, MD; Marcela Bailez, MD; Children's Hospital Garrahan

PMDS is a disorder with persistence of Müllerian duct in 46XY. Cryptorchidism is a usual presenting symptom.

Aim: Show a novel MIS technique to preserve both gonads.

A 4.9-year-old boy was referred because of the finding of Müllerian structures during a laparoscopic bilateral 1st stage Fowler Stephens procedure. He had a 46XY cariotype, a low AMH and normal testosterone, LH and FSH A rudimentary uterus with symmetrical fallopian tubes, and testis-appearing gonads without a vas were found.

Trick: As no spermatic pedicle was available and no vas existed we designed the rigth testicle descent using a flap keeping the right tube which was detached from the uterus and the left one using the whole uterus with the left tube and round ligament pedicle. We managed to move them to the scrotum with good vascular supply.

Result: Both testicles present with good size and vascularity in control doppler US.

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