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Congenital chylothorax: from foetal life to adolescence
S Caserío1, C Gallego, P Martin
1Department of Pediatrics, Hospital Universitario "12 de Octubre", Madrid, Spain. caseriocs@hotmail.com
Insights
Congenital chylothorax (CC) often presents prenatally and impacts survival, particularly with complications like fetal hydrops. However, advances in neonatal care are improving outcomes for affected infants.
Area of Science:
- Perinatal Medicine
- Neonatology
- Pediatric Surgery
Background:
- Congenital chylothorax (CC) is a rare condition characterized by lymphatic fluid accumulation in the pleural space.
- Prenatal diagnosis and understanding associated complications are crucial for management.
- The prognosis of CC has historically been guarded, necessitating improved therapeutic strategies.
Purpose of the Study:
- To analyze the key prenatal and postnatal features of congenital chylothorax (CC).
- To evaluate the outcomes and mid-term follow-up of infants diagnosed with CC.
- To identify factors influencing survival rates in CC patients.
Main Methods:
- Retrospective analysis of 29 CC cases diagnosed between 1990 and 2006.
- Inclusion of prenatal and postnatal data, including complications and interventions.
- Follow-up data available up to 3 years of age for all patients.
Main Results:
- Most CC cases (94%) were diagnosed prenatally, with 66.7% complicated by fetal hydrops.
- Overall 3-year survival rate was 56%, significantly lower with fetal hydrops, preterm birth (<34 weeks), large effusions, or early pneumothorax.
- While not statistically significant, survival improved from 41.7% (1990-1998) to 66.7% (1999-2006).
- Mid-term follow-up showed no CC recurrence, but 27% of survivors developed asthma.
Conclusions:
- Congenital chylothorax (CC) continues to pose a significant risk of perinatal mortality.
- Advances in fetal and neonatal medicine are improving CC patient prognosis, with most now expected to survive.
- Survivors generally experience an uneventful outcome post-neonatal period, though early childhood asthma is a noted concern.
Aim:
To analyse the main prenatal and postnatal features of congenital chylothorax (CC), and the outcome including mid-term follow-up.
Methods:
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.
Results:
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.
Conclusion:
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.
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