54. Evaluation and Management Considerations of Gynecologic Issues in Adolescents with Anorectal Malformations

Abstract

<h3>Background</h3> Girls born with anorectal malformations (ARMs) and bladder exstrophy have a greater incidence of Mullerian and genital anomalies requiring early gynecologic assessment and frequent surgical intervention. As early childhood surgical and medical management of children with ARMs and bladder exstrophy has improved over recent decades, there remains little guidance or framework for recognizing and managing gynecologic and pubertal concerns in the adolescent and young adult years. <h3>Case</h3> A retrospective review was performed for 10-25-year-old female patients born with ARMs, cloaca, or exstrophy that presented to our multidisciplinary clinic between 2009 and 2019. This resulted in twelve unique patients who presented with gynecologic concerns at an average age of 14.6 years. Nine patients had ARMs (including 2 with cloaca and 4 with cloacal exstrophy). The most common presenting symptom was dysmenorrhea (n=8). Six were identified as having an obstructed Mullerian anomaly and 3 had a gynecologic obstruction due to stenosis from prior surgery. Compared to the younger children with ARMs, this population had concerns about vaginal and introital cosmesis, fertility, gender identity, sexuality, and mental health. An overarching theme was the need for a cohesive multidisciplinary team including the surgical subspecialists, gastroenterologists, and psychologists with a heightened awareness of increasing patient autonomy in the adolescent period. <h3>Comments</h3> Patients with ARMs, cloaca, or exstrophy may re-present during adolescence with a unique set of gynecologic concerns. The adolescent should be actively involved in the decision making, and the physician team should have a heightened awareness of psychological and reproductive issues and considerations of long-term concerns and needs.