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Long-Term Management of Problems in Cloacal Exstrophy: A Single-Institution Review
Nora M Haney1, Christian C Morrill1, Ahmad Haffar1
1James Buchanan Brady Urological Institute, Division of Pediatric Urology, Douglas A. Canning MD Exstrophy Database Center, Charlotte Bloomberg Children's Hospital, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Journal of Pediatric Surgery
|October 14, 2023
Summary
Cloacal exstrophy (CE) patients experience significant long-term health issues but can achieve independent lives. Advances in care improve outcomes, with many adults employed and educated despite challenges.
Area of Science:
- Pediatric Surgery
- Urology
- Gastroenterology
- Orthopedics
- Psychology
Background:
- Cloacal exstrophy (CE) is the most severe congenital malformation within the exstrophy-epispadias complex.
- This study investigates the long-term sequelae in a high-volume patient cohort.
Purpose of the Study:
- To analyze genitourinary, gastrointestinal, orthopedic, and psychosocial outcomes in patients with cloacal exstrophy (CE) who are over 10 years of age.
- To evaluate the long-term functional status and quality of life in CE survivors.
Main Methods:
- A prospectively maintained database of 145 cloacal exstrophy (CE) patients (out of 1490 exstrophy epispadias spectrum patients) from 1974 to 2023 was reviewed.
- Analysis focused on CE patients with a minimum follow-up of 10 years, assessing various functional outcomes.
Main Results:
- Of 63 CE patients with ≥10 years follow-up, many had continent catheterizable channels (71.4%) and successful bowel management (58.7% colostomy, 60% continent after PSARP).
- Significant wheelchair use (34.9%) and psychosocial diagnoses (52.4% anxiety/depression, 27.0% chronic pain) were noted, yet 75% were independent in ADLs.
- Adult CE patients demonstrated high rates of college attendance (79.5%) and employment (82.1%).
Conclusions:
- Despite long-term sequelae across multiple organ systems, advancements in care have led to near 100% survival for CE patients.
- Many individuals with cloacal exstrophy lead independent and functional adult lives, highlighting the effectiveness of comprehensive management strategies.
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