Síndrome de prune belly. RevisiÓn a propÓsito de un caso

A 26 year-old man had an unusual (one in 28,000 to 50,000) type of the collecting system: the prune belly syndrome; because only 330 cases have been reported in the world literature, we present this interesting study. Of etiopathogenesis still in discussion, this syndrome was first described by Frolich in 1839. The prune belly or Eagle-Barret syndrome is a rare congenital absence of the abdominal musculature associated with severe non obstructive urinary tract dilation and bilateral intra-abdominal testes (cryptorchidism). This disorder, also termed as the abdominal muscle deficiency syndrome, shows a wide spectrum of involvement, ranging from very minimal changes to a full-blown case. The etiology of the prune belly syndrome is thought to be a generalized mesenchymal defect that contributes to the absence of musculature. Can be accompanied by some generalized involvement with a large-capacity bladder, and elongated and dilated ureters. The prognosis is usually unfavorable.