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后尿道瓣膜的胎儿手术:出生后的长期结局

Fetal surgery for posterior urethral valves: long-term postnatal outcomes.

作者信息

Holmes N, Harrison M R, Baskin L S

机构信息

Department of Urology and Pediatrics, University of California, San Francisco, California, USA.

出版信息

Pediatrics. 2001 Jul;108(1):E7. doi: 10.1542/peds.108.1.e7.

Abstract

OBJECTIVE

Fetal intervention for obstructive uropathy was first performed at the University of California, San Francisco in 1981. Indications for treatment were bilateral hydronephrosis with oligohydramnios. Preintervention criteria included fetal urinary electrolytes with beta-microglobulin levels, karyotyping, and detailed sonography specifically looking for renal cortical cysts. We reviewed the outcomes of children who underwent fetal intervention with specific long-term follow-up in patients who were found postnatally to have posterior urethral valves.

METHODS

A retrospective review of the University of California, San Francisco fetal surgery database was performed for patients with a prenatal diagnosis of obstructive uropathy. Medical records from 1981 to 1999 were reviewed. Long-term follow-up was documented if the cause of the urinary tract obstruction was posterior urethral valves. We collected data points, focusing on time and type of intervention, fetal urinary electrolytes, appearance of fetal kidneys, present renal function, length of follow-up, and present status of the urinary tract.

RESULTS

Forty patients were evaluated for fetal intervention; 36 fetuses underwent surgery during this time period. Postnatal confirmation of posterior urethral valves was demonstrated in 14 patients. All patients had favorable fetal urinary electrolytes. Mean gestational age at intervention was 22.5 weeks. The procedures performed included creation of cutaneous ureterostomies in 1, fetal bladder marsupialization in 2, in utero ablation of valves in 2, and placement of vesicoamniotic catheter in 9. Six deaths occurred before term delivery with premature labor and the newborns succumbing to respiratory failure. One pregnancy was terminated electively because of shunt failure and declining appearance of fetal lungs and kidney. The remaining 8 living patients had a mean follow-up of 11.6 years. Chronic renal disease with abnormal serum creatinine was present in 5 patients. Two patients have undergone renal transplantation, and 1 is awaiting organ donation. Five of the 8 living patients have had urinary diversion with vesicostomy, cutaneous ureterostomy, or augmentation cystoplasty with later reconstruction.

CONCLUSIONS

Fetal intervention for posterior urethral valves carries a considerable risk to the fetus with fetal mortality rate of 43%. The long-term outcomes indicate that intervention may not change the prognosis of renal function or be a predictor for possible urinary diversion. Despite all of these patients' having favorable urinary electrolytes, this did not seem to have any implication postnatally. When counseling families about fetal intervention, efforts should be focused on that intervention may assist in delivering the fetus to term and that the sequelae of posterior urethral valves may not be preventable. Fetal surgery for obstructive uropathy should be performed only for the carefully selected patient who has severe oligohydramnios and "normal"-appearing kidneys.

摘要

目的

1981年加利福尼亚大学旧金山分校首次对梗阻性尿路病进行胎儿干预。治疗指征为双侧肾积水伴羊水过少。干预前标准包括胎儿尿电解质及β-微球蛋白水平、染色体核型分析,以及专门检查肾皮质囊肿的详细超声检查。我们回顾了接受胎儿干预的儿童的结局,并对出生后被发现患有后尿道瓣膜的患者进行了特定的长期随访。

方法

对加利福尼亚大学旧金山分校胎儿手术数据库进行回顾性研究,纳入产前诊断为梗阻性尿路病的患者。回顾了1981年至1999年的病历。如果尿路梗阻的原因是后尿道瓣膜,则记录长期随访情况。我们收集了数据点,重点关注干预时间和类型、胎儿尿电解质、胎儿肾脏外观、目前的肾功能、随访时间以及尿路目前状况。

结果

40例患者接受了胎儿干预评估;在此期间36例胎儿接受了手术。14例患者出生后确诊为后尿道瓣膜。所有患者胎儿尿电解质均正常。干预时的平均孕周为22.5周。实施的手术包括1例行皮肤输尿管造口术、2例行胎儿膀胱造袋术、2例行宫内瓣膜消融术、9例行膀胱羊膜腔置管术。6例患者在足月分娩前死亡,因早产和新生儿死于呼吸衰竭。1例妊娠因分流失败以及胎儿肺和肾外观恶化而选择性终止。其余8例存活患者的平均随访时间为11.6年。5例患者存在血清肌酐异常的慢性肾病。2例患者接受了肾移植,1例正在等待器官捐赠。8例存活患者中有5例进行了尿流改道,包括膀胱造口术、皮肤输尿管造口术或扩大膀胱成形术并随后进行重建。

结论

后尿道瓣膜的胎儿干预对胎儿有相当大的风险,胎儿死亡率为43%。长期结局表明,干预可能不会改变肾功能的预后,也不是可能进行尿流改道的预测指标。尽管所有这些患者的尿电解质均正常,但这在出生后似乎并无任何意义。在为家庭提供关于胎儿干预的咨询时,应重点说明干预可能有助于胎儿足月分娩,而后尿道瓣膜的后遗症可能无法预防。梗阻性尿路病的胎儿手术仅应针对精心挑选的、有严重羊水过少且肾脏外观“正常”的患者进行。

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