Pulmonary sequestration or bronchopulmonary sequestration, is a rare congenital abnormality of the lung. Lungs are composed of millions of tiny air sacs referred to as alveoli. These alveoli communicate with your airways and allow for the transportation of oxygen into your bloodstream and the removal of carbon dioxide from your bloodstream. Each lung is broken into portions referred to as lobes with the right lung usually consisting of three lobes and the left lung usually having two lobes. Each lobe is further divided into segments. The assignment of areas of the lungs into lobes and segments is based on each area having its own group of alveoli, airway, and arterial and venous blood supply.
In pulmonary sequestration, an abnormal, nonfunctioning mass of lung tissue is present. It lacks normal communication with the airway, referred to as the tracheobronchial tree. Unlike normal lung tissue that receives its arterial blood supply from the right side of the heart, pulmonary sequestration's received their blood supply from blood vessels after they have exited the left side of the heart, referred to as the systemic circulation.
Symptoms:
- Symptoms vary depending on type of sequestration and age of symptom development
- Persistent cough
- Coughing up blood
- Episodes of pneumonia
Types of Pulmonary Sequestrations:
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Intralobar Sequestration
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Extralobar Sequestration
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Within pleura lining of a pulmonary lobe
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Outside of normal pulmonary lobe with its own lining
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Drains into pulmonary veins
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Drains outside of normal lung venous drainage
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Usually cystic, frequently infected
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Isolated from lung, rarely infected
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Most common form accounting for 75% of all sequestrations
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Less common, 25% of all sequestrations
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Presents in adulthood
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Usually presents in infancy
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Male/Female predominance equal
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Male 80%/Female 20%
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2/3 of time seen in lower portion of left chest
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90% seen in left chest
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Rarely associated with other developmental abnormalities
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Commonly associated with congenital abnormalities such as diaphragmatic hernias, congenital heart disease, bowel abnormalities, and spinal abnormalities
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Blood supply may come from abdomen or chest
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Blood supply usually within abdomen
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Diagnostic Tests:
- Freuquently suggested on chest x-ray
- Best seen on CT Scan or MRI
- Evaluation of blood supply important in determining approach
Treatment:
- Surgical removal
- Usually removed through Minimally Invasive Thoroscopic Surgery
- May require pretreatment of abnormal blood supply
- Surgery safe with very low complication rate
Here at White Plains Hospital, the experienced team of thoracic surgeons will assess patients to determine if a minimally invasive thoroscopic procedure can be safely performed. The experience and expertise of the thoracic surgical team with allow customization of the procedure for each individual patient to afford them the best solution to their individual situation.