[Prognosis of prune belly syndrome].
The Prune Belly syndrome (PBS) is unfrequent. Fourteen cases have been followed in our unit during the last 20 years. Four infants (29%) died during the first months of life, because of neonatal sepsis (2 cases) or end-stage renal failure (2 cases). Among the other 10 cases, 6 (43%) had normal glomerular filtration rate at a mean age of 10 years 6 months (6 months to 15 years), 4 had chronic renal failure, including 3 cases who developed end-stage renal failure at 8, 8 years 8 months and 17 years respectively. Resection of an urethral obstruction was performed in 2 cases. This surgical indication remains widely accepted, while the current tendency is to limit ureteral surgery in PBS. Orchidopexy was performed in 4 children, 3 of them less than 6 years 6 months of age. Fertility of these early operated children remains to be established, as all adults reported in the literature remain sterile when orchidopexy was not performed or was performed after age 6.
Service de Néphrologie, Hôpital Robert-Debré, Paris.
G
G
Pillion
M A
MA
Macher
Y
Y
Aigrain
C
C
Loirat
D000015:Abnormalities, Multiple; D002648:Child; D002675:Child, Preschool; D003456:Cryptorchidism / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities*; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D011379:Prognosis; D011535:Prune Belly Syndrome / Q000150:complications / Q000175:diagnosis*
Pronostic du syndrome d'aplasie des muscles de la paroi abdominale.