Department of Pediatrics, Hospital Universitario "12 de Octubre", Madrid, Spain.
Acta Paediatr. 2010 Oct;99(10):1571-7. doi: 10.1111/j.1651-2227.2010.01884.x. Epub 2010 Jun 11.
To analyse the main prenatal and postnatal features of congenital chylothorax (CC), and the outcome including mid-term follow-up.
We searched our databases for CC diagnosed between 1990 and 2006. Data of 29 cases were retrieved and analysed. Follow-up until 3 years of age was available for all patients.
Most patients were diagnosed prenatally (94%) and most cases were complicated by foetal hydrops (66.7%). The overall survival rate at 3 years was 56%. A significantly poorer outcome was observed when foetal hydrops, preterm birth < 34 weeks, large effusions and/or early-onset pneumothorax were present. An important but not significant improvement in the survival rate was observed through the study period; while in 1990-1998, the survival rate was 41.7%, from 1999 to 2006 it was 66.7% (p = 0.19). In the mid-term follow-up, we did not observe any recurrence of CC and most infants remain asymptomatic. However, 27% of survivors were diagnosed as having asthma in early infancy.
CC still carries a significant risk of perinatal mortality. However, continuous advances in foetal and neonatal medicine are improving the prognosis of these patients, and nowadays most of them are likely to survive. Beyond the neonatal period, most survivors have an uneventful outcome.
分析先天性乳糜胸（CC）的主要产前和产后特征，以及包括中期随访在内的结局。
我们检索了 1990 年至 2006 年间诊断为 CC 的数据库。检索到 29 例病例并进行了分析。所有患者均获得了 3 年的随访。
大多数患者在产前被诊断（94%），大多数病例伴有胎儿水肿（66.7%）。3 年的总体生存率为 56%。当存在胎儿水肿、早产<34 周、大量胸腔积液和/或早发性气胸时，预后明显较差。通过研究期间，生存率观察到了显著但无统计学意义的改善；1990-1998 年生存率为 41.7%，1999-2006 年生存率为 66.7%（p=0.19）。在中期随访中，我们没有观察到 CC 的任何复发，大多数婴儿仍然无症状。然而，27%的幸存者在婴儿早期被诊断为哮喘。
CC 仍然存在围产期死亡的高风险。然而，胎儿和新生儿医学的不断进步正在改善这些患者的预后，现在他们中的大多数都有可能存活。在新生儿期之后，大多数幸存者的预后良好。
