Robotic removal of Müllerian duct remnants in pediatric patients: our experience and a review of the literature

Abstract

Persistent Müllerian duct syndrome is a disorder of sexual development, which features a failure of involution of Müllerian structures. An enlarged prostatic utricle is a kind of Müllerian duct remnant (MDR) with a tubular shaped structure communicating with the prostatic urethra. Treatment is aimed at relieving symptoms when present, preserve fertility and prevent neoplastic degeneration. We describe 3 cases of successful robot assisted-removal of symptomatic MDRs. The first case came to our attention for pseudo-incontinence; the other two for recurrent urinary tract infections. The patients have not presented such symptoms anymore on follow-up. We then reviewed existent literature on authors who have recently investigated the main issues concerning MDRs and have attempted a roboticassisted approach on them. Robot-assisted laparoscopy can be considered a valid, safe and effective minimally-invasive technique for the primary treatment of prostatic utricle.

Cystoscopy-Guided Laparoscopic Excision of Prostatic Utricle: Report of a Case

“…Large and symptomatic PUs should be excised surgicallynot only for relief of the symptoms, but also to obtain evidence of malignancy arising from Müllerian duct remnants. [7][8][9] In addition to the difficulty dissecting PUs deep in the pelvis, preservation of fertility is another challenge in the surgical management of PUs because of their anatomical proximity to the spermatic cords; therefore, several surgical approaches for excision of PUs have been described, 3 although to date, there is no standard approach according PU size.…”

Symptomatic prostatic utricle: various approaches for treatment

“…Symptomatic PU with normal external genitalia is still more challenging to diagnose due to irregularity in the presenting symptoms [9]. The management of such remnants requires a skill for complete excision and preserving fertility [12]. The clinical symptoms of PU includes urinary tract irritative symptoms, post-void dribbling, urethral discharge, stones in the utricle pouch, recurrent UTIs, recurrent epididymo-orchitis, pseudo-incontinence, and rarely outlet obstruction or malignancy in PU [2,9,12].…”

“…The management of such remnants requires a skill for complete excision and preserving fertility [12]. The clinical symptoms of PU includes urinary tract irritative symptoms, post-void dribbling, urethral discharge, stones in the utricle pouch, recurrent UTIs, recurrent epididymo-orchitis, pseudo-incontinence, and rarely outlet obstruction or malignancy in PU [2,9,12]. In our study, 5 cases were with severe hypospadias, chordee, recurrent UTI, and dysuria, 2 cases were with recurrent epididymoorchitis, and 1 case was with antenatally detected abdominal mass with hydronephrosis with postnatal urinary retention are observed.…”

“…Various approaches have been described for surgical intervention in PUs [12]. A flowchart proposing management protocol for symptomatic PU cases is presented in Fig.…”

“…Symptomatic and enlarged PU should be actively treated [9]. The treatment is intended to relieve symptoms, preserve fertility, and prevent neoplastic degeneration [12]. The suitable approach should be based on the age, symptoms, and size and location of the utricle [9].…”