There is little guidance for managing pubertally identified Mullerian anomalies in
patients with anorectal malformations (ARMs). We sought to assess these unique issues.
Natal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented
from 2009 to 2019 with a gynecologic concern were included.
Data collection was performed and included the presenting problem, psychological evaluation,
fertility and sexuality concerns, and management strategies for these problems.
Main Outcome Measures
The main outcome was unique needs that had to be addressed in the young adult population
and the type of colorectal and gynecological procedures needed on representation.
Twelve patients were identified; all had gynecologic concerns. Ten had ARMs, including
cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal
revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years
(IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and
providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3).
Goal-directed follow-up is required before surgical management to identify psychological
and reproductive issues in patients with ARMs who have gynecologic concerns. Patient
input and psychologic consultation are helpful for patients requiring staged reconstruction.