Comprehensive classification of cloacal malformation provides a useful guide for individualized targeted...

Review the usefulness of a comprehensive classification system to guide the type of uro-genital reconstruction needed in cloacal malformation.

A retrospective analysis of cloacal malformation operated from 1991 to 2021 was done, corelating the cloacal anatomy to the uro-genital reconstruction needed. A comprehensive classification system for cloacal was developed. This classification takes into account all components of cloaca, presence of bladder neck and proximal urethra; rectal and vaginal entry into the cloaca and vaginal duplication with or without significant hydrocolpos. This severity classification system provided guidance to the type of urogenital reconstruction needed. The cloacal reconstruction done focused on opening the common channel and leaving the anterior vaginal wall, urethra and bladder neck complex in-situ attempting to avoid damage to bladder and genital function. A novel technique was then used to reconstruct the posterior vaginal wall in continuity with the posterior vestibule of the introitus. This pedicle graft from the distal rectum could also be used for labial minora reconstruction in the more severe types of cloaca. The adequacy of the vaginal and introital reconstructions were assessed at the time of closure of the colostomy which occurred between 3 to 6 months after the cloacal reconstruction.

A total of 59 patients were reviewed in this study. 4 patients with type 1 cloaca needed only a skin cutback of the perineum or a limited introitoplasty. All 12 girls with type 2 cloaca had a distal rectal stump rotation flap augmentation of the posterior vestibule. 39 girls with type 3 and 4 cloaca needed an abdominal component to mobilize the high rectum, The distal part of pulled through rectum could be used for augmentation of the posterior vaginal wall in continuity with the introitus; and inner surface of labia minora. In 5 patients the distal rectal segment became avascular, so the distal rectal segment used as a full thickness free graft. At the time of colostomy closure, 2 out of the 59 patients were noted to have introital stenosis. 1 out of the 5 patients with a free graft developed introital stenosis as compared to only 1 in those who 34 who had a vascularised pedicle graft. All 6 of the Type 3b patients had initial vaginostomy to drain the perinatal hydrocolpos prior to their cloacal reconstruction. The exact cloacal type was unclear in 4 patients who were referred at a later age for uro-genital reconstruction after they already had their anorectal reconstruction.

This comprehensive classification of cloacal malformation provided a clear guide to the type of reconstruction needed each patient depending on the severity of her cloaca. Urogenital reconstruction in severe cloacal could be successfully done using a minimally disruptive novel technique which did not disrupt the anterior vaginal wall-urethra -bladder neck complex.