Urogenital sinus and anorectal malformation: experience with 22 cases

Urogenital sinus and anorectal malformation is a complex problem. Anatomy must be defined precisely in each to guide surgical reconstruction, details of which can vary greatly. Although primary reconstruction in the newborn has been accomplished, it may be safer in most to start with decompression colostomy and definition of the anatomy. If the vagina is very distended with urine, it should be drained by some means. Separate abdominal perineal pullthrough of the colon without vaginoplasty should never be performed, because it can cause serious obstructive uropathy when the rectal--urogenital sinus fistula is divided. Ureteral reimplantation is often necessary. Many of these children have undergone urinary diversion; most of those are suitable candidates for "undiversion."