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Completely covered cloacal exstrophy: recognition of a new clinical sub-entity
Authors:Makoto Komura  Yoshiaki Tsuchida  Toshiro Honna  Yoshiyuki Kamii  Shu-ichiro Kitahara  Shin-pachi Ishizone
Affiliation:(1) Department of Surgery, National Children's Hospital, 3-35-31 Taishido, Setagaya-ku, 154 Tokyo, Japan;(2) First Department of Surgery, Shi-shu University, 3-1-1 Asahi, 390 Matsumoto, Japan;(3) Department of Pediatric Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, 113 Tokyo, Japan
Abstract:
A case showing many of the typical visceral features of cloacal exstrophy is reported. The patient had fn imperforate anus, a cecal-cloacal fistula, dehiscence of the pubiic symphysis, and lumbosacral spina bifida with synsingomyelia, but the lower abdominal wall was intact without any visceral extroversion. The pertinent literature was reviewed, and it was found that this case corresponded to t typical case of ldquocompletely covered cloacal exstrophy.rdquo Only six cases, including the present one, have so far been reported in the literature. From a clinical viewpoint, it apparently occupies an intermediate position in the wide spectrum of cloacal anomalies between ldquoclassical cloacal exstrophyrdquo and ldquoimperforate anus with recto-cloacal fistula,rdquo but anatomatically and embryologically it is definitely a variant of cloacal exstrophy. In other words, it looks like an imperforate anus with recto-cloacal exstrophy, but should be treated as a variant of loacal exstrophy. The anatomy, classification, embryology, diagnosis, and management of this peculiar surgical condition are discussed, and recognition of this entity is urged.
Keywords:Cloacal exstrophy  Imperforate anus  Recto-cloacal fistula  Short colon  Spina bifida
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