Pulmonary sequestration (extralobar)
Extralobar pulmonary sequestration (ELS) is a subtype of pulmonary sequestration, the other type being intralobar pulmonary sequestration (ILS).
Epidemiology
It is usually encountered in infants, most being diagnosed before six months. It is more common in males (M:F 4:1).
Pathology
Extralobar pulmonary sequestration is the less common type of pulmonary sequestration, accounting only for 15-25%.
ELS is covered by its own pleura and this is what differentiates ELS from ILS.
ELS receives vascular supply mainly from the aorta (thoracic or abdominal) or from other arterial vessels (splenic, subclavian, gastric, intercostal or multiple vessels) and venous drainage can be either systemic or pulmonary.
Location
There is strong predilection towards the left lower lobe (65-90%).
Associations
Radiographic features
CT
- well marginated mass
- usually homogeneous, often without internal gas
- may contain cystic areas
- systemic arterial supply
Treatment and prognosis
Surgical excision is the mainstay of treatment.
Differential diagnosis
- intralobar pulmonary sequestration: may not be able to differentiate from ELS on imaging
- CPAM
- neuroblastoma
- bronchogenic cyst
Furthermore, in an infra-diaphragmatic location, consider :
Siehe auch:
- Bronchogene Zyste
- Lungensequester
- Scimitar-Syndrom
- Herzfehler
- arteriovenöse Malformationen der Lunge
- kongenitale pulmonale Atemwegsmalformation (CPAM)
- hybrid lesion: CCAM - pulmonary sequestration
- intralobar pulmonary sequestration
- kongenitale Zwerchfellhernie
- bronchopulmonale Vordarmmalformation