Abstract
Prune belly syndrome (PBS) is defined as an association of abdominal wall deficiency, genitourinary anomalies, and, in males, cryptorchism. Although PBS is more common in males, females can also have the condition. In both sexes, expression of the disease is often incomplete and prognosis depends upon the specific abnormalities present. PBS in girls or incomplete expression of PBS is called pseudoprune belly syndrome (PPBS). We recently evaluated two baby girls with PPBS. Both girls had unilateral abdominal wall hypoplasia associated with cardiac, genitourinary, gastrointestinal, pulmonary, and musculoskeletal involvement that ranged from normal to severe. One of the patients also demonstrated congenital cytomegalovirus (CMV) infection and focal jejunal hypoganglionosis. Since CMV has been associated with both aganglionosis and PBS, it is possible that CMV induced both conditions in this patient.
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Donnelly, L.F., Johnson, J.F. Unilateral abdominal wall hypoplasia: Radiographic findings in two infant girls. Pediatr Radiol 25, 278–281 (1995). https://doi.org/10.1007/BF02011100
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DOI: https://doi.org/10.1007/BF02011100