Congenital cystic adenomatoid malformation in a toddler: Unusual presentation with pleural effusion

Abstract

Congenital cystic adenomatoid malformation encompasses a series of cystic malformative lesions characterized by aberrant bronchiolar formations of varying size and dispersion. Most cases of this illness are detected in the first few years of life, usually affecting infants. We report a case of CCAM presenting as pleural effusion in a 15-month-old boy who presented with acute respiratory distress. Chest CT revealed a cystic image in the right lower lobe of the lung. The patient had surgical excision, and a pathological examination validated the diagnosis of CCAM type 1 with no malignant material. Following surgery, the patient's general condition improved, and no new respiratory symptoms were observed during an 8-month follow-up period. Increased awareness of this rare condition among pediatricians and radiologists is crucial for facilitating early diagnosis and appropriate treatment.

Keywords: Congenital cystic adenomatoid malformation; Pleural effusion; Respiratory distress; Surgical resection.

Fig. 1

(A) Initial chest X-ray reveals right pleural effusion (arrows). (B) Chest X-ray post chest drainage showing a bulging area (arrows). (C) Chest X-ray after 8 weeks of evolution with aggravation of the bulla image (arrows).

Fig. 2

Axial view of chest CT showing huge bulla originating in the right lower lobe (arrows in [A] and [B]) with compressed lung coupled with emphysema bullae (arrowheads in [A]).

Fig. 3

Histological examination (hematoxylin and eosin (H&E) staining, at 100×) reveals cysts lined by columnar epithelium, with smooth muscle in the cyst walls, with rich capillary vascularization (A). Areas of fibrosis and inflammation were also noted, without any malignancy sign (B).