A complete spectrum of congenital cystic adenomatoid malformation of the lung deceptive clinical presentations and histological surprises; a single institutional study from a tertiary care hospital in North India

Abstract

ABSTRACT Background: Congenital Cystic Adenomatoid Malformations (CCAM) are rare congenital anomalies of the lungs characterised by bronchopulmonary foregut malformations due to a sudden arrest in the development of the bronchial tree in the first trimester of the gestational period. Aim: The present study was aimed to describe the clinical and histopathological profiles of the patients and study patient outcomes after 1 year of surgical resection. Methods: All patients diagnosed with CCAM by histological examination of tissue obtained on surgical resection during the study period were included in the study. Data, such as patient demographics and clinical, radiological and histopathological findings, were recorded, and follow-up information was taken on OPD follow-up till 1 year after surgery regarding respiratory infections, haemoptysis or mortality. Results: Out of 21 patients, 11 were female and included in the study between the ages of 1 month and 32 years, with >50% younger than 2 years. Most patients in the study had recurrent pneumonia, with difficulty in breathing being the second most common presenting complaint. All patients had undergone computed tomography (CT) of the lungs, which was able to diagnose cystic lesions accurately in >80% of cases. Histologically, all cases were classified based on recent Strocker’s classification, and Type 1 was the most commonly observed with 13 cases, followed by Type 2 in five and Type 3 in three patients. There was no evidence of malignant transformation in any of the cases. There was 100% survival at the end of 1 year, with six patients having respiratory infections and none of the patients getting hospitalised over 1 year after surgery. Conclusion: CCAM is a rare congenital anomaly associated with significant morbidity and may present at any age. It can be histologically classified into three subtypes, with Type 1 being the most common. Early surgical management is mandatory to prevent complications such as recurrent infections, respiratory distress, pneumothorax, lung abscess and malignant transformation. All patients included in the study had undergone surgical resection, and there was 100% survival at 1-year follow-up.