Research: Congenital pulmonary airway malformation (CPAM): A case report and review of the literature

Radiol Case Rep

. 2023 Jul 25;18(10):3483-3486.

 doi: 10.1016/j.radcr.2023.07.018. eCollection 2023 Oct.

Congenital pulmonary airway malformation (CPAM): A case report and review of the literature

Krishnaraj Aryal 1Pradeep Raj Regmi 1Gauri Adhikari 2Ujjwal Bhhattarai 3Sagar Prasad Sedhain 1

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Free PMC article


Congenital pulmonary airway malformation (CPAM) is a rare congenital dysplastic malformation characterized by failure of bronchial development and localized glandular overgrowth. Previously known as Congenital Cystic Adenoid Malformation (CCAM), CPAM is classified into 5 types, from type 0 to type IV, depending upon the origin of pulmonary areas of the lung, cyst size, and cyst appearance. CPAM is a rare congenital anomaly typically diagnosed prenatally in ultrasound. However, few cases are diagnosed in childhood and even fewer in adulthood. CPAM can be differentiated from pulmonary sequestration based on the origin of the arterial supply; the former has its arterial supply from the pulmonary artery, whereas pulmonary sequestration has its arterial supply from the systemic circulation. Another differential diagnosis of CPAM includes congenital bronchogenic cyst, congenital lobar emphysema, pleuropulmonary blastoma, congenital cystic bronchiectasis, and congenital diaphragmatic hernia. The most common presentation is recurrent chest infection and chest pain, whereas other presentation includes pneumothorax and hemoptysis. Here, we present a case of a 6-year-old child with recurrent episodes of fever and cough diagnosed as a type II CPAM based on computed tomography findings.

Keywords: Child; Computed tomography; Congenital pulmonary airway malformation.

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