Major haemoptysis in children with cystic fibrosis: a 20-year retrospective study
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Juerg U Barben1, Michael Ditchfield, John B Carlin
1Department of Respiratory Medicine, Royal Children's Hospital, Parkville, Melbourne, Victoria 3052, Australia.
Insights
Major haemoptysis in children with cystic fibrosis (CF) can occur even without severe lung disease. Bronchial artery embolisation (BAE) is effective but often requires repeat procedures, with survival rates varying by treatment and sex.
Area of Science:
- Pediatric Pulmonology
- Interventional Radiology
- Cystic Fibrosis Research
Background:
- Major haemoptysis affects approximately 1% of pediatric cystic fibrosis (CF) patients.
- This study details the management and outcomes of these children at an Australian tertiary center.
Purpose of the Study:
- To describe the management strategies and long-term follow-up for children experiencing major haemoptysis.
- To evaluate the effectiveness and recurrence rates of bronchial artery embolisation (BAE) versus conservative management.
Main Methods:
- Retrospective review of medical records for 51 children with major haemoptysis between 1980 and 1999.
- Analysis of treatment choices (BAE vs. conservative), success rates, recurrence, and survival data.
Main Results:
- Fifty-one children experienced 102 episodes of major haemoptysis; massive bleeding was not limited to those with FEV1 < 50%.
- Bronchial artery embolisation (BAE) showed a 98% immediate success rate, but 46% required re-embolisation.
- Median survival after first haemoptysis was 70 months, with longer survival for males and those initially treated with BAE.
Conclusions:
- Massive haemoptysis in pediatric CF patients is not always associated with severe lung disease.
- BAE is highly effective for acute episodes, but long-term recurrence risk is similar to conservative management.
- Survival outcomes suggest potential benefits from BAE and highlight the importance of sex in long-term prognosis.
Background:
Major haemoptysis occurs in approximately 1% of children with cystic fibrosis (CF). This report describes management and follow-up of these children at a tertiary centre in Australia.
Methods:
Retrospective review of medical records from 1980-1999.
Results:
Fifty-one children (45% female) had major haemoptysis (102 episodes). Mean age at first episode was 15 years (range 7-19) and mean FEV(1) was 56% predicted (range 14-98). Massive life-threatening haemoptysis was not confined to those with severe lung disease (FEV1 < 50% predicted). Bronchial artery embolisation (BAE) was more likely to be the initial treatment for those with massive haemoptysis and chronic recurrent bleeding tended to be treated conservatively (P = 0.01). Overall, 52 BAE were performed in 28 children with an immediate success rate of 98%; 13 children (46%) had repeated BAE. Four patients died as a direct result of severe haemoptysis. Mean follow-up was 54 months (range 0.5-183). Median survival time (Kaplan-Meier estimate) after first haemoptysis was 70 months, with a significantly longer survival for male patients independent of age (RR 3.8; 95% CI 1.7-8.8; P = 0.001). Median survival time following initial treatment with BAE was longer (103 months) compared to conservative treatment (52 months, P = 0.09).
Conclusions:
Massive haemoptysis was unrelated to the severity of lung disease and was more likely to be treated with embolisation. BAE was highly effective, however, 46% of the children required re-embolisation at some time, which is similar to the recurrence risk for major hemoptysis treated conservatively on longer term follow-up.
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