INTRODUCTION: Traditionally, the care of children and adults has been arbitrarily separated between pediatric and adult surgeons. Despite progress in pediatric surgical techniques, patients still suffer from significant functional sequelae, which persist into adulthood. In addition, surgeons who care for adults rarely receive training in the care of the functional sequelae of these congenital anomalies. Anorectal malformations (ARM), Hirschsprung disease (HD), and their associated urogenital malformations are not an exception. We aim to describe the needs of adult patients with congenital colorectal malformations.
METHODS: Following IRB approval, a retrospective database review was performed looking for all adult patients who were treated by our group from 1983 until 2017.
RESULTS: We identified 120 cases; 32 were excluded due to lack of congenital disease or incomplete records. Our final analysis included 88 cases. 51 patients suffered from an ARM, 18 cloacas, 9 pre-sacral masses, 3 HD, 2 spina bifida and 5 with other diagnoses (3 vaginal anomalies, 1 cloacal exstrophy, 1 obstructed seminal vesical). The specific problems that affected the patients were: complications from previous operations (41), rectal prolapse (25), fecal incontinence (11), gynecologic concerns (12), urologic concerns (6), and recurrent fistula (3). We performed 83 surgical interventions, including 13 rectal prolapse repair, 13 continent appendicostomies, 44 PSARP or re-do PSARP, 11 resections of pre-sacral masses, 11 vaginoplasties, 2 examinations under anesthesia, and 2 Mitrofanoff procedures. Five patients were treated medically (bowel management program, obstetric, urologic evaluation).
CONCLUSION: There is an increase need to better prepare adult providers to assume the care of patients born with congenital colorectal disease, as they transition to adulthood. A collaboration between specialized pediatric referral centers with adult colorectal surgeons, urologists and gynecologists is a potential pathway for the adequate transition of care.