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坎特雷尔五联症:产前诊断、分娩及出生后即刻手术修复

Pentalogy of Cantrell: Prenatal Diagnosis, Delivery, and Immediate Postnatal Surgical Repair.

作者信息

Araujo Júnior Edward, Carrilho Milene Carvalho, Toneto Bruno Rodrigues, Guilhen José Cícero Stocco

机构信息

Department of Obstetrics, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP), São Paulo-SP, Brazil.

Discipline of Cardiovascular Surgery, Department of Surgery, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP), São Paulo-SP, Brazil.

出版信息

J Neonatal Surg. 2017 Apr 15;6(2):32. doi: 10.21699/jns.v6i2.503. eCollection 2017 Apr-Jun.

Abstract

Pentalogy of Cantrell (PC) is a congenital anomaly characterized by a defect in the lower sternum, anterior diaphragm, and anterior abdominal wall; ectopia cordis; and congenital heart disease. It is a very rare congenital anomaly and the prenatal diagnosis is possible in the beginning of second trimester of pregnancy using the conventional ultrasonography. The prognosis is poor with high rates of perinatal mortality. We present a case report of prenatal diagnosis of PC at 22 weeks and 3 days of gestation. We emphasize the prenatal care follow up in a tertiary reference center, the parental counseling, the planning of delivery, and the management of newborn by a multidisciplinary team, including the description of immediate postnatal surgical repair.

摘要

坎特雷尔五联症(PC)是一种先天性异常,其特征为下胸骨、前膈膜和前腹壁缺损;心脏异位;以及先天性心脏病。它是一种非常罕见的先天性异常,在妊娠中期开始时使用传统超声检查可进行产前诊断。预后较差,围产期死亡率很高。我们报告一例妊娠22周零3天时PC产前诊断的病例。我们强调在三级转诊中心进行产前护理随访、对父母的咨询、分娩计划以及由多学科团队对新生儿进行管理,包括对出生后立即进行手术修复的描述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3be7/5538598/070c4ca7093e/JNS-6-32_F1.jpg

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