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

• bronchogenic cysts 

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 :

• suprarenal neuroblastoma
• teratoma
• foregut duplication cyst
• mesoblastic nephroma
• adrenal hemorrhage

Siehe auch:

• Bronchogene Zyste
• Lungensequester
• Scimitar-Syndrom
• Herzfehler
• arteriovenöse Malformationen der Lunge
• hybrid lesion: CCAM - pulmonary sequestration
• intralobar pulmonary sequestration
• kongenitale pulmonale Atemwegsmalformation (CPAM)
• kongenitale Zwerchfellhernie
• bronchopulmonale Vordarmmalformation

und weiter:

• Zwerchfelldefekte