Cloacal Anomalies and Congenital Pouch Colon (CPC) in Girls
摘要
Cloacal anomalies represent the most severe form of anorectal malformation (ARM) in females, constituting around 20% of female ARM. There is a single perineal opening, usually with “small-looking” genitalia. The rectum, vagina and urinary tract open into a “single, common channel” which can be 1–10 cm long. Uterine septation and duplication are common, especially with a long common channel (Fig. 50.2), and the vagina(s) may be distended and full of secretions leading to hydrocolpos. The hydrocolpos can compress the trigone leading to hydroureteronephrosis. “Low” cloacal malformations (<3 cm common channel) usually have a well-developed sacrum, a normal appearing perineum, adequate pelvic nerves and musculature, and a good functional prognosis. Cloacal malformations with a common channel >3 cm have more complex defects. Management involves a diversion colostomy followed by a reconstructive operation at a later stage. A combination of posterior sagittal approach (PSARVUP) along with total urogenital sinus mobilization (TUM) is the operative procedure for reconstruction. CPC in girls pose a significant challenge for ano-rectal and genito-urinary reconstruction.