Long-term urological outcome of patients presenting with persistent cloaca.

Abstract

Persistent cloaca is a complex malformation with variable presentation that remains a difficult reconstructive challenge. Previous reviews have concentrated on surgical technique, and data on long-term outcome are scarce. We evaluate long-term outcome and when possible correlate outcome with anatomy at presentation. The records and radiographs of 64 patients 0.5 to 25 years old with persistent cloaca treated at 1 institution from 1975 to 2000 were retrospectively reviewed to determine the outcome for urinary and fecal continence. Outcome data were available in 61 girls as 3 died. Of these patients 11 were younger than 3 years, making evaluation of continence difficult, and so the remaining 50 patients constitute the study population. Mean patient age at review was 11.3 years (range 4 to 25). Of the 50 patients 10 (20%) are incontinent of urine (4 with urinary diversion) and 40 (80%) are socially continent, including 11 (22%) who void spontaneously, 6 (12%) who perform clean intermittent catheterization alone and 23 (46%) who underwent reconstructive surgery. Fecal continence was achieved in 30 (60%) of patients and 40% are fecal incontinent or have a stoma. The majority of patients born with a cloacal malformation can achieve social fecal and urinary control, although in 46% additional reconstructive surgery was required. The presence of a good bladder neck, short common channel, a normal sacrum and 2 normal kidneys indicates improved prognosis for urinary continence. Achieving normal voiding continence in a child with a cloacal malformation still represents an enormous surgical challenge.