Galifer R B, Veyrac C, Faurous P
Service de Chirurgie Infantile, CHU St-Charles, Montpellier.
Ann Urol (Paris). 1987;21(4):241-9.
The authors report a survey of the French Group of Visceral Pediatric Surgery, concerning 985 congenital anomalies of the pyeloureteric junction in 883 children. 67.3% of them are boys. This article stresses the recent improvements in prenatal ultrasonography and diagnosis which is now able to individualize a homogeneous group of asymptomatic children ready to be repaired in the early post-natal period. Early reconstruction is associated with the greatest degree of recovery of renal function. In children, the average age is 4 years 9 months at diagnosis. In 43.6% clinical symptoms suggest an infectious problem. Pain is noted in 35.5% and an abdominal mass is discovered in 13.3%. Excretory urography (IVP), ultrasonography and cystography are still the main investigations to be done in all cases. But it is now necessary to use modern isotopic technics such as DMSA and diuretic DTPA to quantify obstruction with accuracy and follow long-term evolution after treatment. Treatment is surgical and conservative in 81.4%. Almost all of the uretero-pyeloplasties are performed according to the dismembered technic usually with temporary nephrostomy drainage. Renal reduction is reserved only for the redundant and decompensated renal pelvis. Morbidity is low (2.5%) and good results can be expected in about 95% of cases.
作者报告了法国小儿内脏外科学组的一项调查，涉及883名儿童的985例肾盂输尿管连接处先天性异常。其中67.3%为男孩。本文强调了产前超声检查和诊断的最新进展，现在能够确定一组无症状儿童，这些儿童可在出生后早期进行修复。早期重建与肾功能最大程度的恢复相关。儿童诊断时的平均年龄为4岁9个月。43.6%的临床症状提示感染问题。35.5%有疼痛，13.3%发现腹部肿块。排泄性尿路造影（IVP）、超声检查和膀胱造影仍是所有病例的主要检查项目。但现在有必要使用现代同位素技术，如二巯基丁二酸（DMSA）和利尿二乙三胺五乙酸（DTPA），以准确量化梗阻并跟踪治疗后的长期演变。81.4%的治疗是手术和保守治疗。几乎所有的肾盂输尿管成形术都是根据离断技术进行的，通常伴有临时肾造瘘引流。肾切除术仅适用于肾盂多余和失代偿的情况。发病率较低（2.5%），约95%的病例可预期有良好效果。
