Fact-sheet: Bronchopulmonary sequestration
Updated on 09/17/2021 at 12:16 PM
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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