Congenital cystic adenomatoid malformation: a rare serial case report

Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung malformation which a part of the lung becomes polycystic. CCAM accounts for 25% of congenital lung malformations and 95% of lung lesions. Case 1 was a 5-month-old female infant who was diagnosed with pneumothorax, with multiple cysts in the right lung, using chest computed tomography (CT). Thoracotomy lung resection was performed. Case 2 was one-day-old newborn infant who had respiratory distress with Downe score 3. Multiple cystic lesions with septations in left lung was observed on chest CT. Lobectomy inferior lobes of left lung was performed. Both patients were diagnosed as CCAM type 1 pathologically. CCAM can be detected in the gestation by ultrasonography or after delivery through the appearance of respiratory distress signs.

Congenital cystic adenomatoid malformation associated with right aortic arch with mirror image branching and absence of left pulmonary artery in an adult

Background Congenital cystic adenomatoid malformation is an uncommon developmental anomaly that is diagnosed prenatally or during early childhood and is rare to present in adulthood. Type 2 congenital cystic adenomatoid malformation is often associated with other anomalies such as congenital cardiac anomalies. We present a case of type 2 congenital cystic adenomatoid malformation in an adult male associated with absence of left pulmonary artery and right aortic arch with mirror image branching. To our knowledge, this is the first case report with the combination of all three anomalies. Case presentation A 33-year-old male presented to the Department of Pulmonary Medicine with high-grade fever, chills and rigor, breathlessness, and productive cough for a duration of one week. He had multiple similar self-limiting episodes in the past which did not require hospitalization. The plain radiograph showed right aortic arch and a small left hilum. CT thorax showed multiple cystic lesions of size 0.5–2.0 cm in the apico-posterior segment of the left upper lobe; anterior, posterior, and lateral segments of the left lower lobe; and the apical segment of the right upper lobe with a maximal cyst wall thickness of 2 mm. Volume of left lung was reduced with trans-thoracic herniation of the medial segment of the right middle lobe; bronchiectatic changes were seen in the apical, anterior, and posterior segments of the right upper lobe; and fibrotic strands were seen in the apical segment of the right lower lobe and the apico-posterior segment of the right upper lobe. Mediastinal window showed right-sided aortic arch with mirror image branching. The pulmonary trunk was seen to continue as the right pulmonary artery with absent left pulmonary artery. Conclusions Though congenital cystic adenomatoid malformation is a disease of infancy and childhood, it should also be considered in adults presenting with recurrent lower respiratory tract infections and/or pneumothorax. Radiologists must be aware of the types of congenital cystic adenomatous malformation (CCAM) and their associations, as type 2 CCAM has a high association with congenital cardiac disease. Proximal interruption of pulmonary artery should be considered if the pulmonary trunk continues as either right or left pulmonary artery.

Surgery in congenital lung malformations: the evolution from thoracotomy to VATS, 10-year experience in a single center

Background Congenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment. We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery. Methods Surgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated. Results A total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy. Conclusions Congenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers.

An optimistic point in COVID-19 pandemic: a case report of large adult congenital cystic adenomatoid malformation

Background Covid-19 pandemic is a major health calamity causing global crisis involving every aspect of the society. CT chest has become an essential diagnostic investigation and as a prognostic tool for assessment for COVID-19 bronchopneumonia. This case report is about an incidental unexpected finding in a young female, who underwent CT chest screening with suspicion of COVID-19 bronchopneumonia. Case presentation A 29-year-old female came with the complaints of sore throat, myalgia, and fever for the past 3 days. She was referred to our department for plain screening CT chest to rule out COVID 19 infection. She was an active sports person since childhood. CT chest revealed a large well-defined bullous cystic lesion of size 16 × 9.5 × 9.5 cm in the left lung lower lobe with partial sparing of its superior, anterior, and posterior basal segments. Imaging diagnosis of large bullous cystic lesion with emphysematous changes was made. No features of COVID 19 bronchopneumonia. Thoracoscopy-guided lobectomy was done, and tissue was sent to histopathological examination. Final diagnosis was large type 1 congenital cystic adenomatoid malformation with mucinous metaplasia. Our case is unique in the sense that large adult CCAM with mucinous metaplasia of the epithelium is a rare presentation. Further it was diagnosed as a part of COVID 19 screening. Conclusion CCAM presentation in adult is rare. Asymptomatic CCAM lesion of this size diagnosed during COVID 19 chest CT screening was rarely described.