Overview
Bronchopulmonary sequestration (BPS) is a congenital anomaly characterized by a non-functioning lung tissue mass supplied by a systemic artery, distinct from the normal bronchial circulation. Extralobar BPS involves a separate pleural investment, differing from intralobar BPS which remains within the visceral pleura 3.Diagnosis
Key Diagnostic Criteria: Presence of a peripheral lung mass on imaging, often in the lower lobes 3.
Recommended Tests: Chest X-ray initially, followed by CT or MRI for detailed anatomy and aberrant vascular supply 13.
Aberrant Vessels: Identification of systemic arterial supply to the sequestered tissue is crucial 23.
Radiographic Features: Opacity in lower lung fields suggestive of sequestration in pediatric patients 3.Management
First-Line Treatment: Surgical excision, typically lobectomy or segmentectomy, for definitive treatment 3.
Adjunctive Treatments: Not typically required unless complications like sepsis or aneurysm are present 2.
Surgical Approach: Resection of the sequestered tissue and associated aberrant vessels, especially in cases with vascular anomalies like aneurysms 2.Special Populations
Pediatrics: Extralobar BPS is less likely to present with symptoms but may require surgical intervention to prevent complications like recurrent sepsis 3.
Comorbidities: Cases associated with congenital anomalies (e.g., situs inversus totalis) may require tailored surgical planning 1.Key Recommendations
Suspect BPS in children with unexplained lung opacities, particularly in lower lobes, and confirm with imaging showing systemic arterial supply (Evidence: Moderate 3).
Surgical resection is the standard treatment for both intralobar and extralobar BPS to prevent complications (Evidence: Strong 3).
Consider congenital anomalies in the diagnostic workup, as they may influence surgical approach (Evidence: Expert opinion 1).References
1 Barış MM, Gezer NS, Çelik AO, Kılınç O, Balcı P. A case of concomitant intralobar bronchopulmonary sequestration and situs inversus totalis. The clinical respiratory journal 2017. link
2 Janssen DP, Schilte PP, De Graaff CS, Van Dijk HA. Bronchopulmonary sequestration associated with an aneurysm of the aberrant artery. The Annals of thoracic surgery 1995. link
3 Telander RL, Lennox C, Sieber W. Sequestration of the lung in children. Mayo Clinic proceedings 1976. link