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Congenital parenchymatous malformations of the lung
V Evrard1, J Ceulemans, W Coosemans
1Department of Thoracic Surgery, University Hospital Gasthuisberg, Katholieke Universiteit, Herestraat 49, B-3000 Leuven, Belgium.
Insights
Congenital lung malformations require surgical intervention, even when asymptomatic, due to risks of complications. This study reviews 48 pediatric cases, highlighting diagnosis and surgical outcomes for conditions like pulmonary sequestration and bronchogenic cysts.
Area of Science:
- Pediatric Thoracic Surgery
- Congenital Pulmonary Malformations
- Embryology
Background:
- Congenital lung malformations are significant causes of pediatric thoracic surgery.
- These malformations share embryological origins and clinical presentations.
- Early diagnosis and intervention are crucial for managing these conditions.
Purpose of the Study:
- To outline the relationship between embryology and clinical presentation of congenital parenchymatous pulmonary malformations.
- To evaluate the diagnosis and surgical management of these malformations.
- To compare findings with existing literature.
Main Methods:
- Retrospective study of 48 patients operated on between 1979 and 1996.
- Analysis of patient demographics, diagnoses, symptoms, and surgical interventions.
- Review of postoperative outcomes and complications.
Main Results:
- Common malformations included pulmonary sequestration (16), bronchogenic cysts (13), CCAM (14), and CPL (5).
- Mean age at symptom onset was 8.8 years, and at surgery was 9.3 years.
- Lobectomy was performed in 22 cases; lung-preserving surgeries in others. One mortality and 11 complications were reported.
Conclusions:
- Surgical resection is indicated for any thoracic cystic lesion on radiography, even if asymptomatic, due to risks of compression, infection, or malignant transformation.
- Prenatal diagnosis and intrauterine intervention may be considered for fetal intrathoracic masses.
- Effective surgical management of congenital bronchopulmonary malformations is essential in pediatric care.
Abstract:
Congenital lung malformations, primary and secondary, contribute to an important portion of pediatric thoracic surgery. One purpose of this report is to outline the close relation in terms of embryology and clinical presentation of congenital parenchymatous pulmonary malformations. In a retrospective study we also aim to evaluate our experience with the diagnosis and surgical management of congenital parenchymatous bronchopulmonary malformations and to compare our data with the literature. From January 1979 to December 1996 a series of 48 patients, 30 males (62.5%) and 18 females (37.5%), were operated on for congenital bronchopulmonary malformations. Pulmonary sequestration, bronchogenic cysts, congenital lobar emphysema, and congenital cystic adenomatoid malformation were seen in 16, 13, 5, and 14 patients, respectively. The first clinical symptoms occurred at a mean age of 8.8 years (1 day to 62 years), and the mean age at the time of surgical intervention was 9.3 years (1 day to 62 years). The maximum time between first symptoms and surgical treatment was 27 years. A lobectomy was performed in 22 cases; in the other patients more lung-preserving surgery such as enucleation or sequestrectomy was performed. Only one postoperative death occurred following lobectomy for pulmonary sequestration, and it was due to pulmonary hypoplasia and pulmonary hypertension. Eleven other patients presented with postsurgical complications: pneumothorax (n = 5), pleural effusion (n = 3), prolonged air leak (n = 2), portal vein thrombosis (n = 1), and hemorrhage requiring reintervention (n = 1). We conclude that any thoracic cystic lesion expanding on chest radiography should be an indication for surgical resection, even if asymptomatic, because of the risk of pulmonary compression, infection, or malignant degeneration. In the few cases of a fetal intrathoracic mass, prenatal diagnosis and intrauterine intervention may be indicated, and these indications are also discussed.