Staged Surgical Management of Intralobar Pulmonary Sequestration with Bilateral Bronchiectasis in an Adolescent: A Rare Case Report

Background: Bronchopulmonary sequestration (BPS) is a rare congenital pulmonary anomaly characterized by nonfunctional lung tissue that lacks communication with the tracheobronchial tree and receives systemic arterial supply. Although BPS is typically localized and unilateral, its association with bilateral bronchiectasis is exceedingly rare. Managing such combined pathology poses significant diagnostic and therapeutic challenges. Case Presentation: We report the case of a 15-year-old Syrian female with a long history of recurrent lower respiratory tract infections and chronic productive cough. Imaging revealed bilateral bronchiectasis and an aberrant artery from the descending thoracic aorta supplying the left lower lobe, consistent with intralobar pulmonary sequestration (ILS). The patient underwent staged open thoracotomies. The first procedure involved right lower lobectomy with partial middle lobectomy and bronchial stump reinforcement using a vascularized muscle and rib flap. The second stage included left lower lobectomy with intraoperative identification and division of the aberrant artery. Histopathological analysis confirmed severe bronchiectasis, abscess formation, hemorrhage, and vascular congestion, with no evidence of malignancy or fibrosis. Discussion & Conclusion: This case highlights an uncommon coexistence of unilateral ILS with bilateral bronchiectasis, a presentation that is rarely documented in the literature. The extent of pulmonary destruction and pleural adhesions necessitated open surgical intervention. Notably, the use of bronchial stump reinforcement underscores the surgical complexity. Early recognition and multidisciplinary management are essential, especially in pediatric patients with persistent respiratory symptoms. This case adds to the limited data on complex BPS presentations and emphasizes the importance of individualized surgical planning.