Computed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations

Abstract

Purpose: We aimed to identify clinical and computed tomography (CT) features that distinguish among cystic congenital pulmonary airway malformations (CPAMs) and offer new management strategies.

Methods: We retrieved data on 145 children (85 male, 60 female; median age, 14 months) with pathologically confirmed cystic CPAMs from 2008 to 2018 and retrospectively analyzed the clinical and CT characteristics.

Results: Of the 145 patients, 54 had type 1, 72 had type 2, and 19 had type 4 cystic CPAMs. Significantly more male patients had type 4 CPAMs. Type 2 CPAMs had a higher frequency of combined malformations and prenatal diagnosis than types 1 and 4. The median diameter of cystic CPAMs was 2.8 cm; that of type 1, 2, and 4 was 4.6, 1.5, and 8.1 cm, respectively. Regression analysis showed that a cyst of >7.9 cm in diameter was likely to be type 4, that of <2.8 cm was likely to be type 2, and that of 2.8-7.9 cm was likely to be type 1. Smaller cysts were more likely to be type 2 and larger cysts were more likely to be type 4. The incidence of pneumonia was higher in type 2 than in types 4 and 1. The frequency of mediastinal shift and pneumothorax was statistically significant, and both were more common in type 4.

Conclusion: A cyst of >7.9 cm in diameter, mediastinal shift, and pneumothorax were the most important characteristics of type 4 CPAMs. CT features can distinguish type 4 CPAM from other cystic CPAMs.

Keywords: Children; Computed tomography; Congenital cystic adenomatoid malformation; Congenital pulmonary airway malformations; Congenital thoracic malformation; Pleuropulmonary blastoma.