Ranke A, Schmitt M, Didier F, Droulle P
Service de Chirurgie Pédiatrique B, Hôpital d'Enfants, Vandoeuvre-les-Nancy, France.
Eur J Pediatr Surg. 2001 Aug;11(4):246-54. doi: 10.1055/s-2001-17147.
Multicystic Renal Dysplasia (MRD) was discovered during antenatal ultrasound examination in 138 fetuses between 1980 and 1995. Associated malformations were present in 66 % (42 % urological) and 22 % of the fetuses did not survive the pregnancy or the peri-natal period.Anatomical analysis showed a wider variety of MRD than in classical descriptions. Obstruction of the urinary tract was almost invariable. Like the hypothesis published by Beck in 1971, our view is that, with a very early obstruction of the urinary tract (during the first trimester), there is a dysplastic evolution of renal tissue, while later in pregnancy the same obstruction can induce a hydronephrosis with corticomedullary dysplasia.We advise complete neonatal urological investigation, and surgical removal of multicystic kidneys, to avoid multiple and inadequate evaluations of those children with a single functioning renal unit.
1980年至1995年间，在138例胎儿的产前超声检查中发现了多囊性肾发育不良（MRD）。66%的胎儿存在相关畸形（42%为泌尿系统畸形），22%的胎儿在孕期或围产期未能存活。解剖分析显示，MRD的表现比经典描述更为多样。尿路梗阻几乎是普遍存在的。与贝克1971年发表的假说一样，我们认为，在孕期极早期（孕早期）发生尿路梗阻时，肾组织会发生发育异常演变，而在孕期后期，同样的梗阻可导致伴有皮质髓质发育异常的肾积水。我们建议对新生儿进行全面的泌尿系统检查，并手术切除多囊肾，以避免对仅有一个功能肾单位的儿童进行多次且不充分的评估。
