Fact-sheet: Bronchopulmonary sequestration
Updated on 09/17/2021 at 12:16 PM
View all RADEOS cases associated with this fact-sheet
Definition
Pulmonary territory separated from its normal vascular +/- bronchial connections:
- intrapulmonary: intralobar sequestration (ILS)
- extrapulmonary: extralobar sequestration (ELS): intrathoracic or
extrathoracic
No functional vascularization: arterial supply to the sequestration replaced by an abnormal systemic artery
Male predominance
Age at diagnosis < 25–30 years
Pryce classification
Clinical features
Intralobar sequestration:
- Recurrent bronchopulmonary suppuration
- Chronic bronchorrhea
- Hemoptysis
- Pleural effusion
- Heart failure (associated cardiopathy)
- Clinical examination: very rarely cyanosis, digital clubbing,
thoracic asymmetry
Extralobar sequestration:
- incidental finding, asymptomatic, adult
- hemothorax, adult, massive
- in utero, ultrasound or MRI
- pediatric: respiratory distress in the newborn
Laboratory findings
Abnormal development of an accessory pulmonary diverticulum from the primitive foregut
Possible genetic origin: increased expression of
- Homeobox gene: required for normal branching of the
bronchial tree
- MUC1 growth factor
Several forms: associated bronchopulmonary and vascular malformations
- orthotopic (normal position): - Halasz syndrome
- ILS
- heterotopic (abnormal position): - mediastinal cyst
- ELS
Radiography
Polymorphic imaging features:
- single or multiple cystic
- air-fluid levels
- infiltrative
- pseudotumoral
- Unilateral +++ lower lobe ++ left ++, posterobasal
Rare features:
- tubular image of the artery
- atelectasis-like appearance
- pleural-like appearance
CT
CT angiography:
- visualizes the anomalous artery
- assesses the parenchyma
- visualizes the draining vein (ILS/ELS)
- imaging of the chest and upper abdomen
- injection with aortic phase timing
Parenchyma: findings
- single or multiple
- cystic or with air-fluid levels
- pseudotumoral
- alveolar filling: superinfection, hemorrhage
- peripherally: ground-glass opacity, air-trapping zone
- variable appearance over time depending on superinfections and
bronchial-cystic fistulization
Pryce classification:
- type 1: pulmonary artery absent, normal bronchi, abnormal
systemic artery supplies the ventilated territory
- type 2: pulmonary artery absent, bronchus partially absent,
systemic artery supplies both territories (ventilated and non-ventilated)
- type 3: pulmonary artery absent, bronchus absent, abnormal systemic
artery supplies the non-ventilated territory
Vascular intervention
ILS: - Surgery: Lobectomy +++ / Segmentectomy
- Endovascular: vaso-occlusion of the aberrant artery
- if hemoptysis or hemothorax
- pure arterial sequestration Pryce type I
Differential diagnosis
- Pleuropericardial or bronchogenic cyst
- Cystic adenomatoid malformation
- Bronchocele / bronchial atresia
- Arteriovenous fistula
- Bronchial adenoma
- Bronchogenic carcinoma
