Congenital chylothorax: Associations and neonatal outcomes
Congenital chylothorax: Associations and neonatal outcomes
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DOI：
10.1111/jpc.12477
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发表时间：
2014-03-01
影响因子：
1.7
通讯作者：
Malhotra, Atul
中科院分区：
文献类型：
--
作者：
Downie, Lilian;Sasi, Arun;Malhotra, Atul
目的先天性乳糜胸是一种罕见但重要的新生儿疾病，发病率和死亡率很高。该研究旨在描述新生儿这种情况的相关关联、治疗和结果。方法这是对过去 15 年来在三级新生儿中心收治的所有先天性乳糜胸病例的回顾性病例系列。结果确定了 10 例先天性乳糜胸病例。八名婴儿在产前得到诊断，三名婴儿进行了产前胸腔引流。大多数婴儿在出生时进行了通气，需要立即进行胸膜穿刺术。产后管理包括引流液体、通气、白蛋白替代、奥曲肽和富含中链甘油三酯的配方奶粉的饮食调整。五名婴儿被发现存在染色体畸变，另外两名婴儿则存在常规基因检测未证实的畸形特征。努南综合症是最常见的潜在遗传诊断。 80% 的队列中存在相关异常和畸形。该系列中有两例死亡，均为患有多种合并症的婴儿。 结论 先天性乳糜胸是一种罕见疾病，根据我们的经验，每年总体患病率不到一例。大多数婴儿患有相关的染色体异常和需要长期重症监护的重大合并症。
AimCongenital chylothorax is a rare but significant neonatal entity with major morbidity and mortality. The study aims to describe the related associations, management and outcomes of this condition in neonates.MethodsThis is a retrospective case series of all cases of congenital chylothorax admitted to a tertiary neonatal centre in the last 15 years.ResultsTen cases of congenital chylothorax were identified. Eight infants were diagnosed antenatally and three infants had antenatal pleural drainage. Most infants were ventilated at birth and required immediate pleurocentesis. Post-natal management included drainage of fluid, ventilation, albumin replacement, octreotide and dietary modification with medium-chain triglyceride-enriched formula. Five infants had chromosomal aberrations identified, while a further two had dysmorphic features not substantiated with routine genetic testing. Noonan's syndrome was the single most common underlying genetic diagnosis. Associated anomalies and malformations were present in 80% of the cohort. There were two deaths in the series, both in infants with multiple co-morbidities.ConclusionsCongenital chylothorax is a rare condition with overall prevalence of less than a case per year in our experience. Majority of infants had associated chromosomal anomalies and significant co-morbidities needing prolonged intensive care.