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Am J Dis Child. 1965;109(6):571-572. doi:10.1001/archpedi.1965.02090020573013.
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Denouement and Discussion 


Major Manifestations  An enlarged flaccid abdomen with complete or partial absence of the abdominal musculature. The abdomen bulges out into the flanks. The skin is wrinkled and redundant. Abdominal organs are easily palpable, and peristalsis and intestinal loops may be seen. Major abnormalities of the genitourinary or gastrointestinal tracts are frequently present and may cause the death of the infant.

Genetics  Does not appear to be genetically determined.

Sexual Incidence  Appears almost exclusively in the male.

Physical Findings  The abdomen is enlarged and flaccid, and the flanks are bulging. The condition may at first glance be confused with congenital ascites. The skin of the abdomen is overabundant and wrinkled. The transverse, rectus, and oblique muscles are usually affected. The abdominal organs are easily palpable, and the intestinal loops and peristalsis can be seen. The enlarged kidneys (hydronephrotic or polycystic), ureters,


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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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