[A series of 34 cases of prune belly syndrome in children]

Abstract

The congenital triad of abdominal muscle deficiency, urinary tract anomalies and cryptorchidism is often called prune-belly syndrome because of the wrinkled abdominal skin of these neonates. Other associated anomalies may be noted, such as cardiovascular, gastrointestinal or orthopedic disorders. The incidence is about 1 in 40 000 births. Prune-belly is in fact a spectrum of diseases falling into three main groups with very different prognoses and treatments. In group 1, the severe urinary and pulmonary abnormalities usually lead to stillbirth or death in infancy, and need no surgical management. In group 2, the mean survival rate is high (83%), and surgery may be required in some cases, mainly for urinary diversion. In group 3, the disorders are very mild, and the children are often called "pseudo-prunes". The etiology is unknown, but the syndrome appears to be due to arrested mesenchymal development between the sixth and tenth weeks of gestation. In groups 2 and 3, the main problems are to ensure sterility of the urine by close supervision, and to prevent the severe accidents which may be caused by retrograde radiological exploration. A more conservative approach to the syndrome appears to improve the prognosis, and the spontaneous improvement of big megaureters without infectious complications would seem to favor this line or argument.