Bronchopulmonary sequestration
| Pulmonary sequestration | |
|---|---|
 |
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| Classification and external resources | |
| Specialty | medical genetics |
| ICD-10 | Q33.2 |
| ICD-9-CM | 748.5 |
| DiseasesDB | 32120 |
| eMedicine | ped/2628 radio/585 |
| MeSH | D001998 |
A pulmonary sequestration (bronchopulmonary sequestration or cystic lung lesion), is a medical condition wherein a piece of tissue that ultimately develops into lung tissue is not attached to the pulmonary arterial blood supply, as is the case in normally developing lung. As a result, this sequestered tissue is not connected to the normal bronchial airway architecture, and as a result, fails to function in, and contribute to, respiration of the organism.
This condition is usually diagnosed in children and is generally thought to be congenital in nature. More and more, these lesions are diagnosed in utero by prenatal ultrasound.
Symptoms can vary greatly, but they include a persistent dry cough.
Bronchopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract.
It consists of a nonfunctioning mass of normal lung tissue that lacks normal communication with the tracheobronchial tree, and that receives its arterial blood supply from the systemic circulation.
BPS is estimated to comprise 0.15 to 6.4 percent of all congenital pulmonary malformations, making it an extremely rare disorder.
Sequestrations are classified anatomically.
Intralobar sequestration (ILS) in which the lesion is located within a normal lobe and lacks its own visceral pleura.
Extralobar sequestration (ELS) in which the mass is located outside the normal lung and has its own visceral pleura
The blood supply of 75% of pulmonary sequestrations is derived from the thoracic or abdominal aorta.
The remaining 25% of sequestrations receive their blood flow from the subclavian, intercostal, pulmonary, pericardiophrenic, innominate, internal mammary, celiac, splenic, or renal arteries.
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