[ prune belly syndrome - about 2 cases]

ABSTRACT

The Prune Belly syndrome, or aplasia of the abdominal wall muscles is a rare syndrome. It occurs in 1/40.000 children and almost exclusively in boys. It is characterized by the association of an absence of the abdominal wall musculature, a cryptorchidism and severe anomalies of the urinary tract. The treatment of this syndrome is difficult. We bring back two typical observations. It is about two newborns, the first was admitted at 8 hours of life and the second at 11 days of life for an abdominal distension with a total haematuria without anuria. The clinical examination found a spread and expansive stomach, a crumbled cutaneous coating and two voussures in the flanks, a bilateral cryptorchidism and an overflowing miction. The first patient had a minimal renal insufficiency and a low prothrombine time. In the second patient the biologic balance was normal and the UBCE was negative. The abdominal ultrasonography showed a bilateral hydronephrosis. The IVU showed an important hydronephrosis with the presence in the first case of a bilateral anomaly of the uretero-pelvic junction. The UCG in the second patient revealed a bilateral vesicoureteral reflux. The Prune Belly syndrome was evoked. We decided not to operate the patients and to supervise the renal function and to maintain the urines sterile. The first patient received K1 vitamin. The evolution was marked by the disappearance of the haematuria. The first patient died at 40 days of life, the second didn't return to consultation. The interest of these observations is to recall the clinical, radiological and evolutionary aspects of this syndrome and to raise the importance of the antenatal diagnosis