[Importance of prenatally diagnosed portosystemic vascular shunts in clinical outcomes].

Monserrat Valdés Carrillo, Marcela Diaz Caamafto, Pamela Socias Marfán, Pablo Silva Labarca
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引用次数: 0

Abstract

Portosystemic venous shunts (PSVS) are malformations that result from abnormal communications between the portal and hepatic veins or inferior vena cava. Prenatal diagnosis is made by evaluating the fetal venous circulation and it is classified as intrahepatic and extrahepatic, with different evolution and complications.

Objective: To report two cases of prenatal diagnosis of portosystemic vascular shunts and review the importance of this rare pathology in its neonatal and pediatric evolution.

Clinical cases: Case 1: pregnancy with fetal growth restriction, 2nd percentile, polyhydramnios, without fetal malformations and abnormal patterns on fetal Doppler. Abnormal blood flow through the ductus venous and abnormal venous communication in the liver were identified. Normal genetic study. Male newborn (NB) delivered at 36 weeks, because of severe fetal growth restriction, by emergency cesarean section. He evolved asymptomatic, with normal liver function, and did not continue follow-up. Case 2: pregnancy with fetal growth restriction < percentile 1. Agenesis of the ductus venous and abnormal communication between the portal vein and the left suprahepatic vein were identified with no other malformations or signs of heart failure. Severe SGA newborn was delivered by induction of labor at 35 weeks. He evolved asymptomatic. Normal complementary study. A home check-up at 2 months showed persistent vascular anomaly without systemic involvement.

Conclusions: Even though in the cases presented there were no neonatal complications, this kind of malformations require a high index of suspicion in cases with fetal growth restriction, as well as a long-term multidisciplinary follow-up.

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[产前诊断门静脉血管分流对临床结果的重要性]。
门静脉分流(PSVS)是门静脉和肝静脉或下腔静脉之间异常沟通导致的畸形。产前诊断是通过评估胎儿的静脉循环来进行的,分为肝内和肝外两种,其演变和并发症各不相同:目的:报告两例门静脉血管分流的产前诊断病例,并回顾这种罕见病变在新生儿和儿科演变过程中的重要性:病例 1:妊娠合并胎儿生长受限,胎儿百分位数第 2 位,多胎畸形,无胎儿畸形,胎儿多普勒显示异常。发现胎儿静脉导管血流异常,肝脏静脉沟通异常。正常基因研究。男新生儿(NB)在 36 周时因胎儿严重生长受限而紧急剖宫产。新生儿无症状,肝功能正常,未继续随访。病例 2:妊娠期胎儿生长受限<百分位数 1。发现门静脉导管发育不全,门静脉与左肝上静脉之间的沟通异常,但无其他畸形或心力衰竭迹象。严重 SGA 的新生儿在 35 周时通过引产分娩。他无症状。辅助检查正常。2 个月时的家庭检查显示血管畸形持续存在,但未累及全身:尽管上述病例没有出现新生儿并发症,但对于胎儿生长受限的病例,需要高度怀疑此类畸形,并进行多学科的长期随访。
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