Durán Peña A, García Sánchez J L, López Martínez H, Puertolas Márquez M A, del Carmen Marten M
Bol Med Hosp Infant Mex. 1980 Mar-Apr;37(2):275-87.
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.
梅干腹综合征是一种非常罕见的病症,其特征为腹肌明显缺失、双侧隐睾以及泌尿系统异常。病因尚不明确;目前最被认可的理论是胚胎学理论,即由于第六至十周发育过程中的干扰所致。组织学上发现肾发育不全,同时输尿管部分或完全缺乏肌纤维,取而代之的是结缔组织,膀胱肥大且神经节细胞正常;前列腺尿道扩张。有三种临床类型:新生儿型、新生儿急症型和晚期发育型。对于这些损伤情况各异的儿童,目前尚未制定出完善的治疗方案。最佳治疗方法是预防,控制感染,避免器械操作和反复进行泌尿外科检查。预后取决于功能性实质的体积及其保存情况。还取决于发育不全的程度、功能不全的程度以及感染引起的继发性损伤。我们报告三例不同病例及其治疗情况;根据其病情演变的特殊特征,我们取得了良好的效果,并预期有良好的预后。