Prune Belly Syndrome: A Special Case

G Bassir, I Maali*, A Gotni, A Assal, M Bensouda, M Jalal, A Lamrissi and N Samouh

Prune Belly Syndrome (PBS) is an extremely rare anatomo-radiological syndrome that combines aplasia of the muscles of the anterior wall of the abdomen, dilatations of the urinary tract, and testicular malformations, forming the classic triad of the syndrome. However, up to 75% of patients with PBS present with pulmonary, skeletal, cardiac, and gastrointestinal malformations. We report the case of a male neonate born at term to a mother with no particular pathological history, in whom the clinical examination at birth revealed hypoplasia of the muscles of the anterior abdominal wall and congenital dislocation of the hip. Hypoplasia of the abdominal wall muscles was confirmed by abdominal ultrasound and abdominal CT scan, and cardiac malformations were also found on transthoracic ultrasound. The outcome can vary widely, from stillbirth due to major renal and respiratory dysplasia to a virtually normal child. All this explains the great diversity of opinions on the attitude to adopt when faced with this syndrome.

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Published on: Dec 24, 2024 Pages: 44-46

Full Text PDF Full Text HTML DOI: 10.17352/jgro.000130
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