Intralobar Pulmonary Sequestration Presenting as Hemothorax Secondary to Spontaneous Pneumothorax: Case Report and Literature Review

Abstract

Introduction: Patients with pulmonary sequestration (PS), a rare congenital lung malformation, are mostly asymptomatic. Recurrent localized infection is a major complication, while sudden hemothorax is extremely rare. We present a case of intralobar PS presenting as hemothorax secondary to spontaneous pneumothorax and comprehensively review the relevant literature.

Case report: A 16-year-old male presented with chest pain after strenuous exercise. Chest X-ray showed a moderate pneumothorax. After admission and conservative treatment, he developed dizziness, amaurosis, and urinary incontinence. Bedside chest X-ray suggested a massive pleural effusion, and hemothorax was further identified via catheter drainage. Contrast-enhanced computed tomography was performed, and no abnormal blood vessels or leakage of contrast agent were observed. As the hemoglobin level continued to drop, exploratory thoracoscopic surgery was performed immediately. The abnormal systemic artery supplying the lung tissue was found to be ruptured; therefore, ligation of the abnormal artery with resection of the diseased lung tissue was performed. Pathological examination revealed non-specific manifestations of PS. He was followed up for 1 year without related complications.

Conclusion: Our case suggests that the abnormal supply vessels of PS are unstable, which may cause sudden hemothorax. Therefore, patients with PS should undergo surgery promptly after diagnosis. In patients with hemothorax, we should consider the diagnosis of PS; however, contrast-enhanced computed tomography or angiography cannot confirm the diagnosis in all cases. Surgical intervention is recommended in emergency settings.

Keywords: hemothorax; intralobar; pneumothorax; pulmonary sequestration; spontaneous.

FIGURE 1

Chest X-ray. (A) Left pneumothorax volume of approximately 30%. (B) Left pneumothorax with massive pleural effusion.

FIGURE 2

Contrast-enhanced chest computed tomography. (A) The lung window shows left hydropneumothorax; dorsal side of left lower lobe shows consolidation, which presents as atelectasis after compression. (B) The mediastinal window shows left hydropneumothorax; consolidation of the dorsal side of the left lower lobe is enhanced, which presents as atelectasis after compression; high-density lesion in the left dorsal pleural cavity has no enhancement, which is a blood clot; no blood vessels with abnormal morphology and density or contrast agent leaking into the thoracic cavity are found.

FIGURE 3

Intraoperative exploration. (A) The abnormal branch of the left subclavian artery is ruptured, with a diameter of 2 mm. The proximal end is spasmodic, and the distal end forms thrombus, which is bleeding slowly. (B) The abnormal lung tissue in the apical segment of the upper lobe of the left lung, measuring 1 × 1 × 1 cm. The ruptured blood vessel supplying the abnormal lung tissue can be seen, with a diameter of 2 mm.

FIGURE 4

Pathological specimen and microscopic findings. (A) The abnormal supply vessel in the upper lobe apical segment of the left lung is ruptured, with a diameter of 2 mm. The surrounding lung tissue erodes and appears red. (B) There are many lymphocytic infiltration in the pulmonary interstitium along with mild hyperplasia of the pleural fibrous tissue. Numerous foam cells, cholesterol crystals, and multinucleated giant cell reactions are seen in the alveoli near the pleura.