Outcomes of urogenital sinus mobilization for congenital adrenal hyperplasia

Abstract

Objective To evaluate the outcomes of urogenital sinus mobilization (UGSM) for congenital adrenal hyperplasia (CAH). Methods From September 2008 to July 2014, 13 cases of CAH underwent cliteroplasty and UGSM in one stage.They were divided into two groups of partial urogenital mobilization (PUM) and total urogenital mobilization (TUM). Their perioperative conditions were assessed.And postoperative postoperative lower urinary tract symptoms (LUTS) were evaluated by DAN-PSS scale and postoperative perineal appearance was graded by improved rating scale. Results The procedures of PUM (n=8) and TUM (n=5) were performed.The median operative age was 3.2 years for PUM versus 5.9 years for TUM, median length of common channel 1.1 cm for PUM versus 3.0 cm for TUM, median operative duration 180 min for PUM versus 200 min for TUM, median follow-up time 42 months for PUM versus 50 months for TUM.In PUM group, 2 cases presented obvious LUTS.One had frequent enuresis and another urinary incontinence.In TUM group, 3 cases presented obvious LUTS, there were moderate incomplete emptying (n=1), enuresis (n=1) and urinary incontinence (n=1). LUTS and perineal appearance had no inter-group difference.All cases had excellent postoperative urinary continence.The cosmetic outcomes were satisfactory (n=4), average (n=6) and unsatisfactory (n=3). Six cases of poor vaginal exposure were recommended for re-operation. Conclusions As an ideal treatment for female CAH, UGSM may be completed in one stage with cliteroplasty.Overall preoperative assessment of urogenital sinus status yields excellent cosmetics and functional outcomes. Key words: Congenital adrenal cortical hyperplasia; Urogenital sinus; Perineum