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Extrapulmonary subpleural pulmonary sequestration

Last edited: 4/15/2026

Overview

Extrapulmonary subpleural pulmonary sequestration is a rare congenital anomaly characterized by nonfunctioning lung tissue supplied by the systemic circulation rather than the pulmonary artery. It often presents as a cystic mass outside the visceral pleura, typically diagnosed incidentally in adults 1.

Diagnosis

  • Key Diagnostic Criteria: Presence of a cystic mass outside the visceral pleura, supplied by a systemic artery.
  • Recommended Tests:
  • - Chest CT scans for initial detection and characterization. - Contrast-enhanced chest MRI to differentiate from other cystic lesions. - CT-guided lung aspiration biopsy (when necessary for tissue diagnosis). - Surgical exploration for definitive diagnosis and management 1.

    Management

  • First-Line Treatment: Surgical resection (lobectomy or wedge resection) for definitive management.
  • Adjunctive Treatments: Not typically required unless complications arise (e.g., infection, hemorrhage).
  • Special Populations

  • Elderly: Diagnosis can occur in elderly patients; surgical intervention remains the mainstay of treatment despite age 1.
  • Key Recommendations

  • Consider pulmonary sequestration in the differential diagnosis of cystic lung masses, especially in adults, during imaging evaluations (Evidence: Moderate 1).
  • Perform contrast-enhanced imaging (MRI or CT) to distinguish pulmonary sequestration from other cystic lung lesions (Evidence: Moderate 1).
  • Surgical resection is recommended for definitive management and to prevent potential complications (Evidence: Expert opinion 1).
  • References

    1 Kim HJ, Shin KE, Park JS, Lee H, Lee JW, Chin S et al.. Intralobar pulmonary sequestration with cystic degeneration mimicking a bronchogenic cyst in an elderly patient: A case report and literature review. Medicine 2020. link

    Original source

    1. [1]

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