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December 1980

Prune-Belly Syndrome

Author Affiliations

University of Washington Medical Genetics Seattle, WA 98195

Am J Dis Child. 1980;134(12):1182. doi:10.1001/archpedi.1980.02130240062032

Sir.—The article by Siebert entitled "Small-Intestine Length in Infants and Children" (Journal 1980;134:593-595) reports one instance of an intraabdominal lesion in which the small-intestinal length was greater than 2 SDs from the expected mean length. This was in an infant with the prune-belly syndrome in whom the expected small-intestinal length was 164 ± 54 cm (1 SD) and the observed length was 294 cm, greater than 2.4 SDs from the expected mean. One possible explanation for this observation is that linear growth of the small intestine is, in part, determined by the tension on it and the space available for it. When there is lax or absent abdominal wall musculature, as in the prune-belly syndrome, there will be greater intraabdominal volume and thus additional growth of the small intestine can be accommodated. A similar explanation has been suggested as the basis of linear growth of another tube-like structure, the

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