SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental malformation of the lower respiratory tract. Incidence for this rare condition ranges from 1 per 8,300 to 35,000 live births. CPAM is usually detected in the prenatal and neonatal period. Since 1997, there have only been twelve cases of adult CPAM reported in literature. We here present an unusual case of a 22 year old female with cystic lung disease proven to be CPAM. CASE PRESENTATION: An asymptomatic 22 year old female without any medical history presented for an incidental finding on CT abdomen, which showed lobulated cystic lesions throughout the right lower lobe. A CT chest followed showing a cystic structure in the posterior aspect of the right lower lobe measuring 10 cm. No prior imaging was available. Patient had a healthy childhood. Patient did not report any family history of lung disease. After performing the testing to rule out most common cystic lung diseases in the age group, the plan was to assess the patient with a CT aortogram to assess for pulmonary sequestration. However, the patient’s case was complicated by hospitalization for community acquired pneumonia. At that time, CT chest angiogram was done, which was unable to show any aortic arterial vessel feeding to the cystic lesion ruling out pulmonary sequestration. Also the cystic lesions now had air-fluid levels with dense consolidation. After appropriate antibiotic therapy the patient underwent a right lower lobectomy. Upon pathologic evaluation, pulmonary artery/vein supply and bronchial attachment to the right lower lobe were all normal. Histopathology demonstrated variable-sized cysts, lined by pseudostratified ciliated cells interspersed with mucus cells. No cartilage was seen. The gross histopathologic findings along with the radiographic findings support the diagnosis of Type I CPAM. DISCUSSION: CPAM results from imbalance between cell proliferation and apoptosis during organogenesis. CPAM are hamartomatous lesions with cystic and adenomatous components of tracheobronchial tree. CPAM has been differentiated in 4 types based on the origin and distribution within the lung. With an exception of Type 4 malformations, CPAM has no genetic predisposition. More than two-thirds of the cases are diagnosed during the prenatal and neonatal periods due to the advances in imaging. It is exceedingly rare for cases of CPAM to remain undiagnosed until adulthood. A common presentation is recurrent pneumonia. There are reports that CPAM lesions can disguise as malignant lesions. Specifically, Type I is known to be associated with bronchoalveolar carcinoma. CONCLUSIONS: A conservative approach may be reasonable in a patient who is asymptomatic with CPAM. Our patient’s case was complicated by pneumonia; the patient underwent resection of her right lung, which confirmed Type I CPAM. Reference #1: Barreiro, T. J., Henn, L., Ingnam, S., & Sypert, M. (2016). Congenital pulmonary airway malformation in a 36 year-old female. Respiratory Medicine Case Reports, 17, 34–36. doi: 10.1016/j.rmcr.2015.11.005 Reference #2: Kapralik, J., Wayne, C., Chan, E., & Nasr, A. (2016). Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. Journal of Pediatric Surgery, 51(3), 508–512. doi: 10.1016/j.jpedsurg.2015.11.022 Reference #3: Omar, M. A., Tylski, E., Ghanimeh, M. A., & Gohar, A. (2016). Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease. BMJ Case Reports. doi: 10.1136/bcr-2016-216957 DISCLOSURES: No relevant relationships by Aadil Ahmed, source=Web Response No relevant relationships by daniel kechker, source=Web Response No relevant relationships by ami Vaidya, source=Web Response
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May 22, 2024
Eric J Burks + 2
Open Access