Covered cloacal exstrophy – a poorly recognized condition: Hints for a correct diagnosis

Abstract

IntroductionCovered cloacal exstrophy requires a high index of suspicion for its diagnosis. Low implantation of the umbilical cord, separated pubic bones, and anorectal malformation are the most common signs. MethodsThirty-one patients with this defect were retrospectively analyzed. ResultsBesides the anorectal malformation, the patients had important unique anatomic findings, including a colon shorter than 20cm (17 patients) and absent bladderneck (27 patients). Twenty-four patients underwent a colonic pullthrough; of those, only 5 of them have voluntary bowel movements. Twelve patients underwent a urinary reconstruction. Eleven of them are dry with catheterization, and one leaks in between catheterization. Two patients are urinary continent. ConclusionsCovered exstrophy is a serious condition. Externally, the patients may look like having a rather simple malformation. However, the intra-abdominal findings are similar to those seen in cloacal exstrophy. An early correct diagnosis is important to plan a reconstructive strategy and to adjust the parent’s expectations concerning bowel and urinary function. In addition to the traditional prognostic factors for bowel and urinary control (sacral ratio, tethered cord, and level of the rectum) these patients have other anatomic defects (absent bladderneck and short colon) that negatively affect the functional prognosis.