Thursday, September 27, 2012

Prune belly syndrome



Abdominal distension and prominent wrinkling of the abdominal skin are characteristic of prune belly syndrome. Although the underlying pathophysiology is enigmatic, distension of the abdomen associated with distension of the urinary bladder is present in all cases. (From MacLennan GT, Cheng L: Atlas of Genitourinary Pathology. London, Springer-Verlag, 2011.)



In the classic form of prune belly syndrome, the bladder appears distended, and there is bilateral hydroureteronephrosis. It is unclear whether failure of bladder emptying is a mechanical or a physiologic problem. Mechanical obstructions may include posterior urethral valves, urethral diaphragm, urethral stenosis, atresia or multiple lumina; or the bladder neck may be incompetent, forming a flap-like valve. When seen at autopsy, the bladder is not always massively distended and thin-walled. After decompression, it may be small or normal-sized but markedly thick-walled; in such cases, bilateral upper tract distension and renal abnormalities are readily apparent nonetheless. (From MacLennan GT, Cheng L: Atlas of Genitourinary Pathology. London, Springer-Verlag, 2011.)

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