Urological and sexual outcome in patients with Hirschsprung disease: A systematic review.

Purpose: There is a paucity of recent evidence regarding long-term urological and sexual outcomes following surgery for Hirschsprung disease (HD). We aimed to undertake a systematic review of all HD literature to define these outcomes.

Materials And Methods: A systematic literature search was conducted on studies from 1966 to 2014. Relevant articles were assessed for urological/sexual operative complications and functional sequelae. Studies were analysed in qualitative (Rangel score) and quantitative syntheses.

Results: Initially 257 reports were assessed, with 24 studies were eligible for inclusion (1972-2014). Mean study quality was 16.5 ± SD 4.8 (range 6-23), indicating overall fair/poor quality. Ten studies (1021 patients) reported operative complications, with ureteric/urethral/vaginal injury occurring in seven (0.7%) patients. In three studies, the primary outcome was urological functional assessment. From 17 studies, 52/2546 patients (2.0%) had reported urinary incontinence. In infants, absent spontaneous erections post-operatively was reported in 3/203 patients (1.5%, 5 studies); of these 3, parents did not note spontaneous erections pre-operatively either. In older patients, erectile dysfunction occurred in 6/498 (1.2%) males. Other sexual outcomes were reported in 10 studies, with 5/10 studies (416 patients) reporting no erectile dysfunction. In the other studies reports ranged from non-specified sexual dysfunction in one study to diverse sexual related problems in nine (7.8%) of their patients in another.

Conclusions: Urological/sexual outcomes are rarely reported after HD surgery (24 studies over 42 years). Study quality is usually poor and a large proportion of the studies are more than 30 years old. In the majority of series it is unclear whether urological and sexual function impairments were not present or if they were not assessed. Prospective reporting of urological/sexual outcome is required, in particular in the era of new surgical techniques/approaches to HD.