Original ArticleCystic adenomatoid malformation of the lung in adult patients: clinicoradiological features and management☆
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.
Section snippets
Materials and methods
All adult (18 years of age or older) patients diagnosed with CCAM at our institution (Ramón y Cajal University Hospital, a 1000-bed referral hospital in Madrid, Spain) between January 1, 2005, and December 31, 2014, were identified from a combined radiological and surgical data bank. Approval from the institutional review board was not required because the present study was retrospective and observational. There were nine patients that were diagnosed with CCAM, six of which underwent surgical
Results
The demographics and clinical characteristics of nine patients with CCAM are shown in Table 1. The most common form of CCAM in this study was CCAM type I (n= 5). Two asymptomatic patients were found to have other simultaneous developmental anomalies of the lung (one patient had an intralobar sequestration in the same lobe as the CCAM, whereas the other patient had an intrapulmonary bronchogenic cyst in the same lung but in a different lobe).
The most common presenting symptoms were cough (n= 5),
Discussion
CCAM is a rare and complex congenital anomaly of the lower respiratory tract that results from failure of normal bronchoalveolar development with hamartomatous proliferation of terminal respiratory units in a gland-like pattern (adenomatoid) without proper alveolar formation [20]. CCAM is part of the wide spectrum of congenital pulmonary malformations that also includes, among others, bronchopulmonary sequestrations, bronchogenic cysts, congenital lobar emphysema, and bronchial atresia [5], [6]
Conclusion
This study is limited by its retrospective nature and its small sample size. In addition, the fact that six of our patients underwent surgical resection of their malformations whereas 3 patients were conservatively managed introduces a heterogeneity factor that precludes coming to conclusions about the management of adult patients with CCAM. However, gathering a larger number of patients from whom clinical data can be accumulated is quite difficult, since diagnosis of CCAM in adult patients is
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The authors declare no conflicts of interest.