A previously unreported variant of exstrophy cloaca.
Cloacal exstrophy, a rare and complex congenital anomaly, presents with omphalocele; exstrophied bilateral hemibladders with ureteric or miillerian remnant orifices; central exstrophied ileocecal bowel plate with superior orifice of the terminal ileum, inferiorly, the colon, and centrally, the appendix; bifid rudimentary external genitalia; separated pubic rami; low-set umbilicus; and epispadias in the classic form. A newborn case of cloacal exstrophy presenting without an exstrophied intestine and vesicointestinal fistula is reported. The clinicopathologic features of this previously unreported variant of cloacal exstrophy are discussed with special emphasis on embryologic basis. Exstrophied bowel is the main component of exstrophy cloaca, which makes our case unique with regard to the absence of exstrophied bowel and vesicointestinal fistula. This well-known fact is not applicable to the present case. We think that some other unknown mechanisms must be at work for the development of the cloacal exstrophic anomaly presenting with a shortened intact colon ending with an anteriorly located anus. Normal development of the hindgut primarily depends on the normal formation of the cloacal membrane. The basic morphogenetic processes that consist of cell deposition, fusion, and merging should achieve the precise balance between cell proliferation and apoptotic cell death both in hindgut and cloacal membrane development. Unsatisfactory explanations of many similar malformations are primarily due to the lack of accurate and illustrative findings in different fields of embryology. The present case confirms that further studies are required to clarify the various theories in order to achieve more satisfactory explanations for these types of rare anomalies.
Ciftçi
Arbay O
AO
Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Soyer
Tutku
T
Tanyel
F Cahit
FC
D002988:Cloaca / Q000002:abnormalities* / Q000601:surgery; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D014564:Urogenital Abnormalities / Q000175:diagnosis / Q000601:surgery