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Surgical treatment of congenital bronchopulmonary disease in children
A J Bogers1, F W Hazebroek, J Molenaar
1Department of Thoracic Surgery, Dijkzigt University Hospital, Rotterdam, The Netherlands.
Insights
Surgery for congenital bronchopulmonary disease in children, including cystic adenomatoid malformation, is safe and effective. Multidisciplinary care leads to acceptable outcomes with low morbidity and mortality.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Congenital bronchopulmonary diseases require surgical intervention in pediatric patients.
- Diverse pathologies include hamartomas, bronchogenic cysts, intrapulmonary sequestration, emphysema, rhabdomyosarcoma, and cystic adenomatoid malformation.
- Some cases present with complex comorbidities like esophageal duplication or require artificial ventilation.
Purpose of the Study:
- To evaluate the outcomes of surgical treatment for congenital bronchopulmonary diseases in children.
- To assess the mortality and morbidity associated with these procedures.
- To determine the effectiveness of multidisciplinary management.
Main Methods:
- Retrospective review of 22 pediatric patients operated for congenital bronchopulmonary disease between 1987 and 1992.
- Surgical procedures included lobectomy, pneumonectomy, and bilobectomy.
- Analysis of in-hospital and late mortality and morbidity, with follow-up ranging from 1 to 26 months.
Main Results:
- Ten patients had cystic adenomatoid malformation, with 6 requiring pre-operative ventilation.
- Three patients with cystic adenomatoid malformation died in-hospital due to bilateral disease or contralateral lung hypoplasia.
- One patient with rhabdomyosarcoma experienced recurrence and was not resectable; late mortality occurred due to this.
- Hospital morbidity included pneumothorax, atelectasis, and prolonged ventilation.
- No late complications were observed during follow-up.
Conclusions:
- Multidisciplinary treatment is crucial for managing congenital bronchopulmonary diseases in children.
- Surgical intervention can achieve acceptable mortality and low morbidity rates.
- Early diagnosis and comprehensive management are key to favorable outcomes.
Abstract:
From 1987 to 1992, 22 children (age 0 days-14 years) were operated for congenital bronchopulmonary disease. One patient had a hamartoma. Four children had a bronchogenic cyst, in 1 patient combined with an esophageal duplication. Intrapulmonary sequestration was diagnosed in 3 children; one of whom had an esophageal duplication as well. Four children had localized emphysema. In 1 patient histology showed rhabdomyosarcoma. A lobectomy was performed following chemotherapy but recurrence was not resectable at a third thoracotomy. Ten patients had cystic adenomatoid malformation, 6 were dependent on artificial ventilation before surgery. Three patients with cystic adenomatoid malformation died in hospital: 2 after pneumonectomy who were shown to have bilateral disease and 1 died after bilobectomy and was shown to have a hypoplastic contralateral lung. Hospital morbidity involved 1 patient with recurrent pneumothorax, 2 with atelectasis of the operated lung and 3 with prolonged artificial ventilation. Late mortality involved 1 patient due to pulmonary rhabdomyosarcoma. After 1-26 months of follow-up there were no late complications. Adequate multidisciplinary treatment allows acceptable mortality and low morbidity in surgery for congenital bronchopulmonary disease in children.