Kalfa Nicolas, Lopez Christophe, Baud Catherine, Veyrac Corinne, Morin Denis, Averous Michel
Service d'Urologie II, Hôpital Lapeyronie, Montpellier, France.
Prog Urol. 2006 Sep;16(4):481-4.
Congenital giant hydronephrosis due to ureteropelvic junction obstruction is exceptional and treatment often requires nephrectomy of a poorly functioning kidney. However, a more conservative approach by nephroplication is also possible. The objective of this study was to evaluate the long-term functional outcome of these kidneys treated by nephroplication during the neonatal period.
From 1996 to 1998, 5 neonates with congenital giant hydronephrosis were managed in our department (3 antenatal diagnoses). The mean anteroposterior pelvic dilatation was 145 mm (range: 110-180 mm). All infants were treated by pyeloplasty with nephroplication, regardless of the preoperative ultrasound and scintigraphic appearance of the renal parenchyma.
The mean follow-up was 9 years (range: 8-10 years). A marked initial reduction of global kidney volume was constantly observed (more than 50% of the initial volume). The mean relative renal function was 32.4% (range: 10-42%) without any residual obstruction. Renal growth was satisfactory in 4 cases (40 mm at 1 year, 72 mm at 6 years) with an atrophic kidney in 1 case. No child developed hypertension or elevated serum creatinine.
Congenital giant hydronephrosis does not systematically justify primary nephrectomy even in the presence of a healthy contralateral kidney. Despite the pejorative initial ultrasound and scintigraphic appearance, which is always difficult to interpret, early nephroplication improves drainage of pyelocaliceal cavities, decreases the residual dilatation and allows significant nephron sparing in most of these children.
因肾盂输尿管连接处梗阻导致的先天性巨大肾积水较为罕见，治疗通常需要切除功能不佳的肾脏。然而，采用肾成形术的更保守方法也是可行的。本研究的目的是评估新生儿期接受肾成形术治疗的这些肾脏的长期功能结局。
1996年至1998年，我科收治了5例先天性巨大肾积水的新生儿（3例为产前诊断）。肾盂前后径平均扩张为145毫米（范围：110 - 180毫米）。所有婴儿均接受肾盂成形术及肾成形术治疗，无论术前肾脏实质的超声和闪烁显像表现如何。
平均随访9年（范围：8 - 10年）。持续观察到肾脏总体积最初有明显减少（超过初始体积的50%）。平均相对肾功能为32.4%（范围：10% - 42%），且无任何残余梗阻。4例肾脏生长情况良好（1岁时为40毫米，6岁时为72毫米），1例为萎缩肾。无患儿出现高血压或血清肌酐升高。
即使对侧肾脏健康，先天性巨大肾积水也并非必然需要一期肾切除术。尽管最初超声和闪烁显像表现不佳且总是难以解读，但早期肾成形术可改善肾盂肾盏腔的引流，减少残余扩张，并能在大多数此类患儿中显著保留肾单位。
