PART OR NOT TO PART: BRONCHO-PULMONARY SEQUESTRATION, A RARE BUT POTENTIALLY DANGEROUS ANATOMICAL ANOMALY

Abstract

TYPE: Abstract Publication TOPIC: Genetic and Developmental Disorders PURPOSE: Bronchopulmonary sequestration is a rare but potentially dangerous anatomical anomaly. With our case, we hope to increase awareness about this entity. METHODS: 26 year-old female presented with right-sided pleuritic chest pain and associated intermittent dry cough. She denied dyspnea, wheezing, hemoptysis, fever, chills or sick contacts. Medical history was significant for SLE on hydroxychloroquine. Physical exam and initial lab work were unremarkable. CT chest with IV contrast incidentally revealed a focal area of consolidation in the RLL with large feeding vessel arising directly from descending thoracic aorta. No discrete draining veins were identified. She was diagnosed with intralobar RLL bronchopulmonary sequestration. Her chest pain was attributed to serositis and improved with short course of steroids and NSAIDs. But management of her bronchopulmonary sequestration was tricky. RESULTS: It is a rare abnormality of the respiratory tract consisting of nonfunctioning lung tissue lacking communication with tracheobronchial tree and receiving its arterial blood supply from systemic circulation. Usually diagnosed in-utero or in childhood, but can rarely be asymptomatic, eventually leading to major complications like massive hemoptysis, recurrent pneumonias, high-output cardiac failure; occasionally reports of neoplasms arising within the sequestration have been reported. CONCLUSIONS: Management options include observation, surgical resection or coil embolization of feeding artery. We discussed her case in multidisciplinary pulmonary-radiology conference. She did not have high risk features like large lesion ∼20% of hemithorax, bilaterality, multifocal/cystic lesions, but due to risk of infection and hemoptysis, consensus opinion recommended treatment with surgical resection of the RLL sequestration. CLINICAL IMPLICATIONS: Bronchopulmonary sequestration requires careful risk benefit deliberation. DISCLOSURE: No significant relationships. KEYWORD: Bronchopulmonary sequestration