66 years old male with past medical history of Hypertension, chronic kidney disease, active smoker and alcohol abuse, presented with two episodes of hemoptysis. Patient was admitted initially with working diagnosis of possible hematemesis due to significant bleeding and drop in hemoglobin and started on proton pump inhibitors. Non contrast Computed tomography (CT) of chest was done to rule out any lung masses which suggested mucous or blood in left lung airways. While in hospital, patient started to develop acute hypoxic respiratory failure and was placed on noninvasive ventilation but later intubated. While intubating, blood clots were noted in the airway. Bronchoscopy was done which remained inconclusive. Meanwhile upper gastrointestinal endoscopy and laryngoscopy were done to find the bleeding source but were found to be unremarkable. As the bleeding had stopped spontaneously, patient was extubated and remained stable until next day when he started bleeding again with large clots and fresh blood from his mouth. Patient was electively intubated and bronchoscopy was repeated which showed profuse oozing from left lower bronchus but exact source of bleeding could not be identified. A single lung endotracheal tube was placed for selective ventilation of the right lung. Because of patient’s tenuous state it was felt that interventional radiology which had initially been planned, was not an option and that the only option for this critically ill patient was surgical intervention. Patient was taken to operating room. On thoracotomy it was noted that there was a systemic vessel arising in inferior ligament that appeared to be dividing down in to one of the hiatus of the diaphragm and this was consistent with intralobar sequestration. The vessel was ligated and the left lower lobe was resected. Patient tolerated the procedure well and recovered slowly.
Bronchopulmonary sequestration is a rare congenital anomaly in which a mass of abnormal lung tissue that has no normal connection with the bronchial tree is supplied with the blood from an aberrant artery originating in thoracic aorta. Two forms have been recognized, intralobar and extra lobar sequestration. Intralobar pulmonary sequestration (ILS) in which the lesion is located within a normal lobe and lacks its own visceral pleura while the extra lobar is located outside the lung with its own pleura. ILS has aberrant connections to bronchi, lung parenchyma, or the gastrointestinal tract, and often presents with recurrent infections. The embryologic basis for the development of BPS is unclear. Diagnosis is confirmed by CT scan of lung and magnetic resonance angiography. In symptomatic patients, BPS is treated by surgical excision, which is curative and is associated with minimal morbidity.
Bronchopulmonary sequestration although rare but should be suspected in middle aged and elderly population with hemoptysis.