Although technological advances have contributed much to the management of childhood diseases, they have often resulted in decision quandaries. One such example is that of congenital cystic lung lesions. In their paper, Calvert et al describe the outcome over a period of 10 years of suspected congenital cystic adenomatoid malformations (CCAMs) in 28 children.1
These are often detected on antenatal ultrasound, and parents were previously reassured after a normal chest radiograph in the neonatal period. With the advent of computed tomography (CT) scanning, however, hitherto undetected lesions are now being identified. Calvert et al recommend that all babies with antenatally diagnosed CCAMs have a CT scan, a recommendation with which we agree. However, they also recommend that all asymptomatic lesions should be surgically removed if they have not resolved within the first year. This statement, from surgical authors, is not based on any scientific rationale and may be misleading to parents who have to decide between surgical intervention and conservative management. The authors state that surgery is often considered in asymptomatic patients because of the risk of malignancy, infection, or pneumothorax. However, the evidence to support this comes only from case reports. It is not known whether subjecting patients to a potential lobectomy to remove a small, possibly benign lesion, with an undefined outcome may result in more harm than good.
There is clearly a need for longitudinal studies to assess the outcome of these lesions. To address this, we are currently undertaking a multicentre, long term, follow up surveillance study of prenatally diagnosed cystic lung lesions (the LoTOS, long term outcome study, currently funded by Birth Defects Newlife). In our experience of around 90 cases with a prenatally detected cystic lung lesion, in survivors with radiological findings compatible with cystic adenomatoid malformation, about 58% have had conservative management. Surgical intervention has been carried out in the other 42%, 62% of these because of symptoms and only 38% electively. There is an urgent need to provide an evidence base for the management of Orphan lung diseases, such as CCAM,2 which is currently dependent on physician or surgeon personal bias, as demonstrated by Calvert et al.1
Footnotes
Competing interests: None declared.
References
- 1.Calvert J K, Boyd P A, Chamberlain P C.et al Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991–2001. Arch Dis Child Fetal Neonatal Ed 200691F26–F28. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Jaffé A. Orphan lung diseases in childhood: still unadopted? Thorax 200560892–894. [DOI] [PMC free article] [PubMed] [Google Scholar]