ABSTRACT

Congenital pulmonary airway malformation, formerly called cystic adenomatoid malformation is an alteration in the development of airway terminals, lack of alveolar development and inadequate organization of lung mesenchyme. It is an hamartromal injurie that may even compress and affect the development of neighboring organs. It is rare, with an incidence of 1:25,000 to 35,000 and corresponds to 25% of pulmonary congenital malformations. The underlying cause is unknown. The anomaly is mainly attributed to a maturation defect, specifically the arrest in pseudoglandular phase (weeks 7-17 of gestation) generating a compensatory hyperplasia with replacement of lung tissue by cysts of varying size and distribution. It can be diagnosed in utero by ultrasound. Clinical manifestations range from severe respiratory distress with neonatal death until asymptomatic lesions. The surgical resection by thoracotomy or thoracoscopy are the elective treatment. Stocker classification describes 5 types. The Congenital pulmonary airway malformation type II is rare and represents only 10-15% of cases, so a clinical case report and a literature review is presented.

KEYWORDS

Congenital cystic adenomatoid malformation, congenital cystic lung malformation, lung cyst, surgery, thoracotomy.

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