Urological outcomes in adult females born with anorectal malformation or Hirschsprung disease
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In brief
In brief
This cross-sectional study examines urological outcomes in 66 adult females born with anorectal malformation or Hirschsprung disease, finding significantly higher rates of incontinence, UTIs, and social impairment in cloaca patients compared to reference populations. Results emphasize the need for lifelong multidisciplinary urologic and nephrologic care.
- Adult females with cloaca have significantly higher rates of urinary incontinence, UTIs, and social impairment versus reference population.
- 26.6% of ARM/HD patients required bladder reconstruction; 18.8% perform catheterization through channel or native urethra.
- Chronic kidney disease or ESRD affects a subset of patients, with three requiring kidney transplantation in this cohort.
- Cloaca subgroup requires lifelong multidisciplinary follow-up including urology and nephrology for urologic sequelae management.
- Self-reported urinary outcomes demonstrate persistent morbidity into adulthood, emphasizing need for transition care protocols.
Written by the GCMD Library team from the article.
Abstract
Introduction
Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population.
Methods
This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher’s exact tests.
Results
Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation.
Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3).
Conclusion
This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca.
Level of Evidence
III.