Blane C E, Barr M, DiPietro M A, Sedman A B, Bloom D A
Department of Radiology, University of Michigan Medical Center, Ann Arbor.
Pediatr Radiol. 1991;21(4):274-7. doi: 10.1007/BF02018623.
57 cases of renal obstructive dysplasia (defined as the abnormal development of nephronic and ductal structures due to in utero obstruction of the urinary tract) were evaluated in terms of sonographic findings, renal and other associated anomalies, and current status of the child. More than one-third of the cases had bilateral disease and although not uniformly fatal bilateral involvement was associated with significant morbidity and mortality. In 12 of the 33 cases with unilateral dysplasia there was an association with contralateral renal problems including uretero-pelvic junction obstruction, vesicoureteral reflux and aplasia. Almost one-half of the cases had congenital anomalies, these included VACTERL association, congenital heart disease, cranial abnormalities and gastrointestinal malformations. Fifteen stillborns and 12 of the patients with bilateral involvement and four with unilateral involvement have died. Four patients are on dialysis (two with bilateral involvement and two with unilateral renal obstructive dysplasia). Only one-quarter are otherwise normal. More serious problems are reported in this mixed age population of patients with obstructive renal dysplasia than has been identified in previous studies. Management decisions of the fetus and child must be based on this new age-expanded population.
对57例肾梗阻性发育异常（定义为由于胎儿期尿路梗阻导致肾单位和导管结构的异常发育）进行了超声检查结果、肾脏及其他相关异常以及患儿当前状况的评估。超过三分之一的病例为双侧病变，虽然并非一律致命，但双侧受累与显著的发病率和死亡率相关。在33例单侧发育异常的病例中，有12例与对侧肾脏问题相关，包括输尿管肾盂连接处梗阻、膀胱输尿管反流和发育不全。几乎一半的病例有先天性异常，这些包括VACTERL综合征、先天性心脏病、颅骨异常和胃肠道畸形。15例死产儿以及12例双侧受累患者和4例单侧受累患者已经死亡。4例患者正在接受透析（2例双侧受累，2例单侧肾梗阻性发育异常）。只有四分之一的患者在其他方面正常。与先前研究相比，在这个年龄混合的肾梗阻性发育异常患者群体中报告了更严重的问题。对胎儿和儿童的管理决策必须基于这个新的年龄范围扩大的群体。
