Congenital Cystic Lung Diseases

Abstract

Congenital cystic diseases of the lung are a rare but significant cause of morbidity in children and young adults presenting with respiratory distress and repeated chest infections. They consist of cystic adenomatoid malformation, bronchogenic cyst, pulmonary sequestration, and congenital lobar emphysema. Surgical treatment is a safe and an effective method of treatment. Chest X-ray and computed tomography are the key imaging modalities used for diagnosis.

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

“…It has been hypothesized that there are thousands of people living with an undetected CPAM, and they may live a full life without any complication [1,3,4]. Similarly most cases in our series were diagnosed incidentally.…”

“…In addition, CPAMs may have a potential for malignancy, giving further support to those who advocate early resection [18,19]. Some papers have demonstrated lack of consensus among surgeons, even in the same center [4,5,7,10,18]. In this study we documented and classified the children with CPAM and compared the differences with literature findings.…”

“…It was classified into three subtypes in 1977 and expanded into five subtypes with a new acronym as CPAM by Stocker in 2002 [1,4]. All these classifications are based on the similarity in appearance of the lesion with the various areas of the normal tracheobronchial tree [3].…”

A Six-month-old Infant with Respiratory Distress and Cyanosis

“…Aileen Azari-yam (CMC Pathologist): The main pathological differential diagnosis includes cystic adenomatoid malformation type III, which has similar findings on ultrasound. Congenital lobar emphysema is devoid of cystic lesions as compared to cystic adenomatoid malformation (18,19).…”