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Congenital pulmonary airway malformation

G Khanbabaee, S A Tabatabaei, F Rahimpoor

    Bratislavske Lekarske Listy
    |October 26, 2013
    PubMed

    Insights

    Congenital cystic adenomatoid malformations (CCAMs) are rare lung anomalies. This case highlights a misdiagnosis of hydatid cyst, with surgery revealing CCAM in a pediatric patient.

    Area of Science:

    • Pediatric Pulmonology
    • Thoracic Surgery
    • Developmental Biology

    Background:

    • Congenital cystic adenomatoid malformations (CCAMs) are rare, sporadic lung developmental anomalies.
    • Typically unilateral and single-lobe, CCAMs present as hamartomatous abnormalities with adenomatoid proliferation.

    Observation:

    • A 6-year-old girl presented with a 3-month history of prolonged fever and non-productive cough.
    • Physical examination revealed coarse crackles and decreased breath sounds on the left side.
    • Laboratory tests showed elevated markers for hydatid cyst (IgA and IgM).

    Findings:

    • Initial diagnosis suggested a hydatid cyst based on clinical presentation and serology.
    • Intraoperative findings led to a revised diagnosis of adenomatoid cystic malformation.
    • Surgical resection was performed with a favorable outcome.

    Implications:

    • This case underscores the importance of considering CCAMs in pediatric patients with persistent respiratory symptoms, even with initial atypical presentations.
    • Accurate diagnosis is crucial for appropriate surgical management and patient outcomes.
    • Highlights diagnostic challenges in differentiating CCAMs from other thoracic pathologies.
    Abstract

    Keywords:
    cystic adenomatoid malformation of lung, congenital, pulmonary, pneumonectomy.

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