Asymptomatic congenital lung malformations

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Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

Section snippets

The arguments

Some authors recommend simple observation of patients with asymptomatic CCAM,41, 42, 43, 44, 45 sequestration46, 47, 48 or prenatally diagnosed “lung masses.”49 However, most authors favor surgical resection, at least for CCAM, intralobar sequestation and bronchogenic cysts.2, 3, 7, 15, 24, 29, 31, 39, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69 Finally, others recommend surgery for “significant” lesions but may observe smaller ones.26, 38 The arguments for

Indications for surgery

Asymptomatic congenital lobar emphysema may resolve and should be observed once underlying lesions are excluded. Bronchogenic cysts require resection. For sequestrations, it is clear that ELS may remain asymptomatic throughout life and may be found because of associated anomalies.3, 97 They may also regress spontaneously. The risk of malignancy appears low despite its frequent association with type 2 CCAM. The risk of infection is small in the absence of cysts or communication with the foregut

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