Mani S, Kupferman F, Kumar K, Hazra S, Sokal M, Jean-Baptiste D, Kim R
Division of Neonatology/Nephrology, Department of Pediatrics, Brookdale Hospital Medical Center, New York, New York.
AJP Rep. 2019 Jul;9(3):e209-e212. doi: 10.1055/s-0039-1692419. Epub 2019 Jul 10.
Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male neonates. We report a preterm neonate with PUV who presented as nonimmune fetal hydrops with intestinal obstruction in the antenatal period. The mother of our patient is a 33-year-old woman who started her prenatal care at our hospital at 30 weeks' gestation. Her sonogram done at 32 weeks in our hospital revealed fetal hydrops. It showed polyhydramnios, mild pyelectasis of right kidney, normal left kidney, and fetal ascites. Amniocentesis revealed bile stained amniotic fluid. Ultrasound during the procedure showed dilated fetal bowel loops with increased echoes. Following delivery at 32 weeks postnatal exam showed ascites with absence of skin edema, pleural, or pericardial effusion. The abdominal sonogram showed distended urinary bladder and bilateral hydroureteronephrosis. Bladder catheterization was done which relieved the bladder outlet obstruction. Voiding cystourethrogram was done later which confirmed PUV and bilateral grade 5 vesicoureteral reflux. The formation of urinary ascites in PUV serves as a pop-off mechanism to relieve the intravesical and intrarenal pressure. When this happens by mechanisms other than bladder rupture, it can lead on to transient intestinal obstruction and hepatic synthetic defects.
后尿道瓣膜（PUV）是男性新生儿膀胱流出道梗阻最常见的先天性病因。我们报告一例患有PUV的早产儿，其在产前表现为非免疫性胎儿水肿并伴有肠梗阻。我们患者的母亲是一名33岁女性，在妊娠30周时开始在我院进行产前检查。她在我院32周时的超声检查显示胎儿水肿。表现为羊水过多、右肾轻度肾盂积水、左肾正常以及胎儿腹水。羊膜穿刺术显示羊水被胆汁染色。术中超声显示胎儿肠袢扩张且回声增强。32周分娩后，产后检查显示有腹水，但无皮肤水肿、胸腔或心包积液。腹部超声显示膀胱扩张及双侧输尿管肾盂积水。进行了膀胱插管，解除了膀胱出口梗阻。随后进行了排尿性膀胱尿道造影，证实为PUV及双侧5级膀胱输尿管反流。PUV中尿腹水的形成作为一种安全阀机制来缓解膀胱内和肾内压力。当通过膀胱破裂以外的机制发生这种情况时，可导致短暂性肠梗阻和肝脏合成功能缺陷。
