Anterior Sagittal Transperineal Repair of Posterior Cloacal Malformation: Case Report with Emphasis on Surgical Nuances (A Video)

Abstract

Posterior cloaca is a rare congenital anomaly characterized by a common urogenital channel opening near the anal verge, frequently associated with Müllerian and renal abnormalities. Delayed presentation may result in progressive upper urinary tract deterioration. Surgical reconstruction aims to achieve anatomical separation of the urinary and genital tracts while preserving continence and renal function. A 4-year-old child from a rural setting presented with atypical external genitalia and urinary leakage from a single perineal opening, which was initially suspected to represent a disorder of sexual differentiation. Examination revealed clitoromegaly, reduced anogenital distance, a normally positioned anus, and a single midline common channel opening anterior to it without separately identifiable urethral or vaginal orifices. Investigations confirmed a 46,XX karyotype with renal dysfunction, bilateral hydroureteronephrosis, and bilateral streak ovaries with a hypoplastic uterus. Following urinary diversion with suprapubic catheterization and renal recovery, definitive reconstruction was performed via an anterior sagittal transperineal approach with partial urogenital sinus (UGS) mobilization, feminizing genitoplasty, and protective diverting loop colostomy. Anterior sagittal partial UGS mobilization provides excellent exposure for precise reconstruction in posterior cloaca presenting beyond infancy. Preoperative urinary diversion facilitates renal recovery and optimizes surgical outcomes. Individualized planning is essential in complex cloacal variants with associated Müllerian anomalies.