Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature

Abstract

Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with congenital cystic adenomatoid malformation (Stocker Type II and I) with brief review of literature.

Congenital Pulmonary Airway Malformation – Case Presentation

“…In the presented case, the diagnosis of CPAM was supported by the location, size, wall thickness and distribution of cysts, adjacent lung tissue and vasculature aspect and also by associated symptoms. Other entities like congenital diaphragmatic hernia, bronchiectasis, cystic fibrosis, mediastinal masses (teratoma, cystic hygroma, blastoma) and infectious causes, which can sometimes cause problems of differentiation with CPAM, were clinical and imaging excluded (5,11,12).…”

“…Type I is the most common (50-70%), is composed of single or multiple large cysts (> 2 cm), usually affects only one lobe and is not associated with other malformations, with a good prognosis (10,12). Type II (15-30%) has lesions consisting of multiple small cysts (<2 cm), sometimes associated with other anomalies incompatible with life, such as pulmonary hypoplasia, renal agenesis, atresia of the esophagus (4).…”

Retrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years

“…In CPAM, a segment or an entire lobe of the lung is replaced by a non-functional tissue ranging from aplastic to micro or macro cystic pulmonary tissue [2]. It is almost always unilateral and especially Type III CPAM occurs slightly more often in male rather than female patients, [1][2][3].…”

“…Approximately 25-30% of all congenital lung malformations are CPAMs, and 30% of all patients with CPAM are at risk of respiratory failure at birth [2]. The underlying cause for CPAM is traditionally accepted as pulmonary maldevelopment [3].…”

Lung abscess in congenital cystic malformation during early infancy

“…1 If CPAM is suspected as the cause of repeated pulmonary infection limited to single lobe, patient should be treated early with surgical removal of the affected lobe as Type I CPAM the most frequent subtype, is associated with risk of malignant transformation. 2 Lung abscess is a rare condition and much rarer in early infancy.…”