[Prune bell syndrome].
The prune belly syndrome is a very rare entity, characterized by a marked deficiency of the abdominal muscles, bilateral cryptorchidism and urinary tract abnormalities. The etiology is still unknown; the best accepted theory is the embryologic, due to disturbance in development between the sixth to tenth week. Histologically renal dysplasia is found together with partial or total absence of muscle fibers in the ureter, instead of which there is connective tissue, hypertrophy of the bladder with normal ganglionar cells; the prostatic urethra is dilated. There are three clinical types: neonatal, neonatal urgency and late development. There is not as yet a good management program set out for these children in whom the damage is varied. The best treatment is prophylaxis, control of infection avoiding instrumentation and repeated urological examinations. The prognosis depends on the volumen of functional parenchima and its preservation. Depends also on the degree of dysplasia and of insufficiency and secondary damage due to infection. We are reporting three differents cases and their management; we have obtained good results and we expect a good prognosis according to the special characteristics of their evolution.
D000009:Abdominal Muscles / Q000002:abnormalities* / Q000000981:diagnostic imaging; D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013737:Testis / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014564:Urogenital Abnormalities; D014567:Urography
Síndrome de abdomen en ciruela pasa.