Cystic pulmonary malformations: clinical and radiological polymorphism. A report on 30 cases

Objective: This report describes different clinical pictures of cystic pulmonary malformation (CPM) and problems in diagnosis.

Methods: Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in our institution were reviewed.

Results: Thirty-three cases of CPM were diagnosed in 30 children. They consisted of 17 boys and 13 girls ranging from 20 days to 16 years of age at the time of the diagnosis. The CPM included: 17 cases of congenital lobar emphysema (CLE), seven bronchogenic cysts (BC), five cystic adenomatoid malformations (CAM) and four pulmonary sequestrations (PS). Three patients presented two associated lung malformations. The mean ages at the time of diagnosis varied from 2 to 88 months. The symptoms consisted of respiratory distress (n=14, 46.6%); recurrent attacks of respiratory embarrassment (n=6, 20%); pulmonary infection (n=8, 26.6%) associated with haemoptysis in two cases; haemothorax (n=1) and a chance discovery (n=1). Radiological investigations led to the diagnosis in all cases of CLE and CAM although it contributed less to the diagnosis of BC and PS. Twenty-nine patients required chirurgical treatment involving lobectomy (n=22), pneumonectomy (n=2) and cystectomy (n=8). The histopathological examinations confirmed the diagnosis in all cases and rectified the preoperative diagnosis in four cases. Except for one patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia, the postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years). Three patients developed transient and episodic attacks of dyspnoea.

Conclusions: CPM may be responsible for many clinical and radiological pictures that present difficulties in their diagnosis. Polymorphism is related to the type of malformation, its topography and the evolutive complications.