Cystic adenomatoid malformation of the lung in adult patients: clinicoradiological features and management

Abstract

Purpose

Congenital cystic adenomatoid malformation (CCAM) of the lung in adults is very rare. We aimed to evaluate the clinicoradiological features of adult patients with CCAM.

Methods

Adult patients diagnosed with CCAM were evaluated for clinicoradiological presentation and management.

Methods

Nine patients were diagnosed with CCAM (four were incidentally diagnosed on chest imaging). Most cases appeared on computed tomography as thin-walled multiseptated cystic lesions. Two patients had another concurrent pulmonary developmental anomaly. Five patients were surgically treated and three conservatively.

Conclusions

CCAMs might be incidentally diagnosed on routine chest imaging in adults and can be safely treated with minimally invasive techniques.

Introduction

Congenital cystic adenomatoid malformation (CCAM) is a rare and complex developmental anomaly of the lower respiratory tract that is usually diagnosed either prenatally or in neonates and infants [1], [2], [3]. Also known as congenital pulmonary airway malformation, CCAM is considered part of the wide spectrum of bronchopulmonary foregut malformations that also includes, among others, bronchopulmonary sequestrations, bronchogenic cysts, congenital lobar emphysema, and bronchial atresia [4], [5], [6], [7]. Its presentation or detection in adulthood is very rare, with fewer than 60 reported cases in the literature [8], [9], [10], [11], [12]. Adult patients with CCAM usually present with recurrent lower respiratory infections, nonspecific thoracic symptoms (cough, dyspnea), and hemoptysis [13], [14], [15]. However, adult patients with CCAM may be asymptomatic [16]. In fact, with the increasing use of thoracic computed tomography (CT), it is more likely that developmental pulmonary anomalies might be incidentally detected on routine chest radiographs or on CTs performed for other reasons. Encountering a developmental lung anomaly in an adult can be a challenge, since the congenital abnormality may be radiologically mistaken for something more sinister in this age group than in the pediatric population [17].

Although there are a few published reports of results of surgical management of congenital lung malformations in pediatric patients, there are very few data regarding the evolution, natural history, or optimal management of CCAMs discovered in adulthood, with only a few case series on the surgical management of this entity [9], [10], [11], [18], [19]. Based on the few reports of CCAMs in adults that have been published and on the fact that these lesions may be incidentally (and increasingly) detected in asymptomatic patients, we believe that the management of CCAMs in adulthood deserves a different approach than that of the pediatric population. The purpose of this work was, therefore, to analyze our experience with adult patients diagnosed with CCAM regarding the clinical and radiological presentation, as well as the management (including surgical treatment) of this group of patients.