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Autopsy specimens of common ( n = 3) and covered cloacal exstrophy ( n = 4) with single intraexstrophic and -perineal phallic structures and perineo-exstrophic canals have been analyzed histopathologically. The findings were correlated to normal development to reconstruct the pathogenesis. By identifying a specific cloaca-derived urethra field as distinct from allantois-derived bladder fields, the exstrophic area is found to reflect the original hindgut configuration in embryos of approximately 26\u201329 postovulatory days gestational age (2\u20134 mm). Correlation to normal development suggests malfunctioning of the primitive streak\/caudal eminence as a primary fault that leads to a defective cloacal region in the hindgut disturbing cloacal-intestinal-allantoic dissociation and also causes lengthening of the intestinal region into a blind-ending colon, teratoma-like lesions, and vertebral and muscular anomalies. The current idea that membranes in \u201ccovered cloacal exstrophy\u201d represent persisting cloacal membranes is dismissed by finding an amnion-like structure, which suggests dysfunction of an umbilical ring placode as a simultaneous 2nd fault. This malfunctioning may cause omphalocele by defective demarcation of the umbilical cord and may replace midline stroma of the infraumbilical abdominal wall by extraembryonic tissue that stretches into a weak temporary membrane, may leave a perineo-extrophic canal, and may allow the formation of a single perineal or intraexstrophic phallus. Malfunctioning without replacement may result in a purely epithelial \u201callantoic\u201d membrane, which by disintegrating in combination with the cloacal membrane will expose common cloacal exstrophy.<\/jats:p>","DOI":"10.2350\/07-06-0292.1","type":"journal-article","created":{"date-parts":[[2009,1,13]],"date-time":"2009-01-13T11:25:46Z","timestamp":1231845946000},"page":"430-442","source":"Crossref","is-referenced-by-count":10,"title":["Common (\u201cClassical\u201d) and Covered Cloacal Exstrophy: A Histopathological Study and a Reconstruction of the Pathogenesis"],"prefix":"10.1177","volume":"11","author":[{"given":"S.C.J.","family":"van der Putte","sequence":"first","affiliation":[{"name":"Department of Pathology, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W.G.M.","family":"Spliet","sequence":"additional","affiliation":[{"name":"Department of Pathology, University Medical Centre Utrecht, Utrecht, 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