Leeners B, Sauer I, Schefels J, Cotarelo C L, Funk A
Department of Obstetrics and Gynaecology, Rhenish-Westphalian Technical University Hospital Aachen, Pauwelsstrasse 30, D-52057 Aachen, Germany.
J Clin Ultrasound. 2000 Nov-Dec;28(9):500-7. doi: 10.1002/1097-0096(200011/12)28:9<500::aid-jcu10>3.0.co;2-8.
The prune-belly syndrome (PBS) consists of abdominal wall distention with deficiency of the abdominal wall musculature, urinary tract abnormalities, and cryptorchidism. The impaired drainage of the bladder leads to oligohydramnios and pulmonary hypoplasia. We present 4 cases of PBS diagnosed by prenatal sonography. In 2 cases, vesicoamniotic shunt therapy was not indicated because of a poor prognosis based on sonographic and laboratory findings; the pregnancies were terminated. In another case, treatment was not performed because of a twin pregnancy, and the neonate with PBS died the day of delivery by cesarean section at 31 weeks' menstrual age. In the other case, vesicoamniotic shunt therapy was successfully performed, and a healthy child was delivered. Several conditions must be met for vesicoamniotic shunt therapy to have a good chance of success: the karyotype must be normal, other malformations must be excluded by careful sonographic examination, and renal function must be normal, as determined by serial analyses of fetal urine. Generally, the shunt should be inserted as early as possible.
梅干腹综合征（PBS）表现为腹壁膨胀伴腹壁肌肉组织缺损、泌尿系统异常和隐睾症。膀胱引流受损导致羊水过少和肺发育不全。我们报告4例经产前超声诊断的PBS病例。其中2例，由于基于超声和实验室检查结果预后不良，未行羊膜腔分流术治疗；终止妊娠。另一例，因双胎妊娠未进行治疗，患有PBS的新生儿在孕31周时剖宫产分娩当天死亡。另一例成功进行了羊膜腔分流术治疗，并分娩出一个健康的婴儿。羊膜腔分流术治疗要获得成功必须满足几个条件：核型必须正常，必须通过仔细的超声检查排除其他畸形，并且根据胎儿尿液的系列分析确定肾功能必须正常。一般来说，分流术应尽早插入。
