ABSTRACT
Cloacal exstrophy is the most severe anomaly in the
exstrophyepispadias spectrum. It is a rare and complex malformation that affects between one in 200 000 and one in
400 000 live births, with a male to female ratio of 1:2.1
Despite its complexity, survival is expected, with nearly 100%
of patients now surviving. Management has become focused
on patient outcomes and the achievement of an optimal
quality of life. This has been born out in the last three decades
where the shift for improving the quality of life has included
appropriate gender assignment, urinary and fecal social
continence independent from stoma appliances, improved
physical and social independence, and mobility.25
Cloacal exstrophy, also known as vesico-intestinal fissure,
ileovesical fistula, or extrophia splanchnica, was first described
by Littre in 1709 and again by Meckel in 1812.1 The era of
operative correction began with Rickham’s 1960 report of a
three-stage procedure performed over eight months.6 Although
only 17 of 34 patients survived correction during the years
196876, survival of 13 of 15 patients at a single institution was reported in the early 1980s;1 and since that time, survival has
ranged from 90 to 100% in a variety of reported series.35
Today, survival from cloacal exstrophy is nearly universal,
mortality being a product of associated renal or cardiac
disease and occasionally secondary to short bowel syndrome.
The focus of care has now shifted to urinary, gastrointestinal,
and genital reconstruction, designed to render the patient
appliance-free. Considerable attention is now paid to appro-
priate gender assignment.
