Hirschsprung disease and associated urological morbidities: institutional experience and systemic review.

PURPOSE: Hirschsprung disease (HSCR) may be accompanied by urological comorbidities such as congenital anomalies of the kidney and urinary tract (CAKUT) and lower urinary tract symptoms (LUTS). However, their actual burden remains under-characterized. This study aimed to assess the prevalence and clinical impact of CAKUT and urinary dysfunction in HSCR patients using institutional data and a systematic literature review.

METHODS

We retrospectively analyzed 310 pediatric HSCR patients who underwent definitive surgery between January 2020 and December 2021. All underwent renal ultrasonography; additional imaging (voiding cystourethrogram [VCUG], magnetic resonance imaging [MRI], scintigraphy) was performed as clinically indicated. Postoperative voiding function was evaluated in 160 children ≥ 4 years of age using the Dysfunctional Voiding Scoring System (DVSS). A systematic review was performed per PRISMA guidelines.

RESULTS

Congenital anomalies of the kidney and urinary tract were found in 6.5% (20/310) of patients, predominantly unilateral hydronephrosis. The mean anteroposterior renal pelvic diameter was 13.7 ± 4.8 mm. Surgical intervention was required in 25% of these cases. Only 2 of 160 patients (1.3%) had abnormal DVSS scores. No link was observed between the extent of aganglionosis and urological outcomes. Literature review (24 studies) showed highly variable CAKUT (0–21.8%) and LUTS (0.5–27.9%) prevalence.

CONCLUSION

Congenital anomalies of the kidney and urinary tract and urinary dysfunction are underrecognized comorbidities in HSCR. Routine ultrasonographic screening and selective advanced imaging support early detection. Our low postoperative dysfunction rates suggest favorable outcomes with standardized multidisciplinary care.