Clinical presentation and outcome in congenital pulmonary malformation: 25 year retrospective study in Thailand

Congenital pulmonary malformations (CPM) are a group of rare abnormal lung development lesions that can have various presentations. The aim of this study was to define the differences in the clinical presentations of CPM in neonates, infants, and children, and to review the outcomes.

A retrospective study was conducted at a tertiary care hospital in southern Thailand between 1992 and 2016.

Fifty-four patients were diagnosed with CPM, and the median age at onset was 1.7 months (IQR, 0.03-10 months). There were 33 cases (61.1%) of congenital pulmonary airway malformations, two (3.7%) of bronchogenic cyst, eight of (14.8%) congenital lobar emphysema, seven of (13.0%) pulmonary sequestrations, and four of (7.4%) congenital lung cysts. Twenty patients under 1 month old and 16 patients who were 1-12 months old had symptoms of respiratory distress. In contrast, 13 patients >1 year old had symptoms of pulmonary infection. There were significant differences in the numbers of patients who had cyanosis (P = 0.006), cough (P < 0.001), and fever (P < 0.001) between the three age groups. Thirty-eight patients (70%) required surgical treatment involving lobectomy (78.9%). Median follow-up duration was 28.1 months (IQR, 3.7-9.4 months). Nine of 10 patients had abnormal lung function tests, and 80.6% of patients had no subsequent limitations in physical activities.

Respiratory distress is the important clinical feature in neonates and infants, whereas the signs of pulmonary infection usually occur in children >1 year old. Good outcomes usually occur after surgery but need long-term follow up including lung function assessment.