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对一名患有梅干腹综合征和严重尿道发育不全的患者进行子宫内干预。

In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia.

作者信息

Perez-Brayfield M R, Gatti J, Berkman S, Eller D, Broecker B, Massad C, Kirsch A, Smith E

机构信息

Department of Urology, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.

出版信息

Urology. 2001 Jun;57(6):1178. doi: 10.1016/s0090-4295(01)00951-7.

Abstract

Prenatal ultrasound scanning of a 20-year-old woman at 17 weeks of gestation revealed findings suggestive of bladder outlet obstruction, including bladder distension, dilated bilateral ureters, urinary ascites, and oligohydramnios. Vesicoamniotic shunts were placed with decompression of the bladder and correction of the amniotic fluid levels. Labor was induced at 36 weeks' gestation. At birth, the infant was noted to have prune-belly syndrome with severe urethral hypoplasia, a variant usually associated with a poor prognosis, necessitating vesicostomy for bladder drainage. We present a case of a patient with prune-belly syndrome and bladder outlet obstruction in whom early intervention resulted in an excellent outcome with preservation of renal and pulmonary function.

摘要

对一名20岁妊娠17周的女性进行产前超声扫描,结果提示膀胱出口梗阻,包括膀胱扩张、双侧输尿管扩张、尿性腹水和羊水过少。放置了羊膜腔分流管以减轻膀胱压力并纠正羊水量。妊娠36周时引产。出生时,婴儿被诊断为梅干腹综合征伴严重尿道发育不全,这是一种通常预后较差的变异型,需要进行膀胱造瘘以引流膀胱。我们报告一例梅干腹综合征合并膀胱出口梗阻的患者,早期干预取得了良好的结果,保留了肾和肺功能。

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