An unusual case of recurrent pneumonia

Background/significance: Bronchopulmonary sequestration (BPS) is a rare disorder of the lower respiratory tract comprising 0.15-6.4 % of all congenital pulmonary malformations. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation. It is usually diagnosed later in childhood or adolescence after presenting with recurrent pneumonia. Results: A 30-year-old male with Hx of recurrent pneumonia presented with fever, chest pain, dyspnea and non-bloody productive cough. On physical examination, he was in moderate respiratory distress. On exam, RR 24/min, PR 116/min and T 38.3°C. Dullness on the right posterior lower lung field with inspiratory crackles was found. The rest of the examination was with unremarkable findings. Diagnostic workup: WBC 12,300 with left shift; rest of hemogram was normal. CT of chest revealed consolidation of right lower lobe with distortion of the structures and multiple air pockets. There was no extension of the tracheobronchial tree into this area. A 3-D CT reconstruction demonstrated an aberrant vessel extending from the upper abdominal aorta in to the intrapulmonary sequestration in the anterior basilar segment of the right lower lobe. There was a cystic mass with abscess cavity and thickened pleura, probably from recurrent infection. The venous drainage was in to the pulmonary vein. The patient was managed with antibiotics and open thoracotomy with right lower lobectomy and abscess drainage. Patient had smooth post-operative course and was discharged improved. No recurrence of pneumonia was reported two years after intervention. Conclusion: This patient presented with an intrapulmonary BPS complicated by recurrent pneumonia. BPS is classified as intrapulmonary or extrapulmonary depending on the visceral pleural investment of the abnormal tissue. Communication with bronchi or lung parenchyma may be present allowing infection to occur. Resolution of infection is usually slow and incomplete due to inadequate bronchial drainage. Surgical resection is the treatment of choice for patients who present with infection or symptoms resulting from compression of normal lung tissue. Intrapulmonary lesions often require lobectomy because the margins of the sequestration may not be clearly defined. In patients who present with recurrent pneumonia at younger age, the possibility of congenital malformations like BPS should be considered and diagnostic workup pursued