Summary about Disease
Bronchopulmonary sequestration (BPS) is a congenital malformation where a mass of lung tissue does not connect to the normal bronchial airway system and receives its blood supply from the systemic circulation (aorta or its branches) rather than the pulmonary artery. It essentially represents a piece of lung tissue that is separate from the normal lung structure and function. BPS can be intralobar (within the lung) or extralobar (outside the lung).
Symptoms
Symptoms can vary based on the type (intralobar vs. extralobar) and size of the sequestration. Many individuals with BPS are asymptomatic, especially with extralobar sequestration. When symptoms are present, they may include:
Recurrent pneumonia: Most common symptom, particularly in intralobar sequestration.
Chronic cough
Wheezing
Shortness of breath
Chest pain
Hemoptysis (coughing up blood)
Failure to thrive (in infants/children)
Cyanosis (bluish skin due to lack of oxygen) - Less common
Causes
The exact cause of bronchopulmonary sequestration is not fully understood. It is thought to occur during lung development in utero. Theories include:
Accessory lung bud theory: An extra bud develops from the primitive foregut, separating from the normal lung bud.
Traction theory: An anomalous vessel arising from the aorta may exert traction on the developing lung, causing a portion to separate.
Inflammatory theory: Local inflammation during lung development may lead to the sequestration. BPS is considered a congenital anomaly, meaning it's present at birth. It is not typically inherited or related to any specific maternal exposure during pregnancy.
Medicine Used
Medications are typically used to manage symptoms of BPS, particularly infections.
Antibiotics: To treat recurrent pneumonia or other infections. Common antibiotics used might include amoxicillin, azithromycin, or broader-spectrum antibiotics depending on the specific infection.
Bronchodilators: To help open up airways and ease breathing, such as albuterol.
Pain relievers: Over-the-counter or prescription pain medications may be necessary to manage chest pain.
Anti-inflammatory Medications: in rare cases, to manage inflammation within the airways. Important: Medical treatment addresses the complications associated with the sequestration, but it does not treat the sequestration itself. Surgical removal is often necessary for definitive treatment.
Is Communicable
No, bronchopulmonary sequestration is not communicable. It is a congenital malformation, meaning it develops during fetal development and is not caused by an infectious agent. It cannot be spread from person to person.
Precautions
There are no specific precautions to prevent BPS since it is a congenital condition. However, general measures can be taken to manage complications associated with it:
Prompt treatment of respiratory infections: Seek medical attention at the first sign of a respiratory infection (cough, fever, shortness of breath).
Avoid smoking and irritants: Exposure to smoke and other lung irritants can worsen respiratory symptoms.
Maintain good hygiene: Frequent handwashing can help prevent respiratory infections.
How long does an outbreak last?
Since BPS is not an infectious disease, the concept of an "outbreak" is not applicable. Symptoms associated with BPS, such as recurrent pneumonia, may occur periodically, and the duration of each episode depends on the severity of the infection and the effectiveness of treatment. However, the BPS itself is a persistent condition until surgically removed.
How is it diagnosed?
Diagnosis typically involves a combination of imaging and clinical findings:
Chest X-ray: May show an abnormal mass in the lung.
CT scan (Computed Tomography): Provides more detailed images of the lungs and can help visualize the sequestration and its blood supply. Contrast CT is helpful in visualizing the systemic blood supply to the sequestration.
MRI (Magnetic Resonance Imaging): Can also be used to visualize the sequestration, particularly in younger children to avoid radiation exposure.
Angiography (CT angiography or conventional angiography): Visualizes the blood vessels supplying the sequestration (gold standard).
Prenatal ultrasound: In some cases, BPS may be detected before birth.
Bronchoscopy: Used less often, but can help to rule out other causes of recurrent pneumonia or hemoptysis.
Timeline of Symptoms
The presentation of symptoms varies greatly, depending on the type of sequestration.
Prenatal/Infancy (Extralobar): Extralobar sequestration may be diagnosed prenatally through routine ultrasounds. If not found at the prenatal stage, it may be found later in life. Some infants are asymptomatic at birth, while others may develop respiratory distress soon after birth.
Childhood/Adulthood (Intralobar): Intralobar sequestration often presents later in childhood or adulthood with recurrent pneumonia, chronic cough, or other respiratory symptoms. The symptoms may be intermittent, with periods of remission followed by exacerbations. Hemoptysis or other symptoms may develop years after the first onset of pneumonia. There is no set timeline; symptoms can manifest at any point depending on complications and the type of sequestration.
Important Considerations
Differential Diagnosis: It's important to differentiate BPS from other lung conditions like congenital cystic adenomatoid malformation (CCAM), pulmonary infections, and tumors.
Surgical Resection: Surgery (lobectomy or segmentectomy) is often the definitive treatment for BPS, especially when symptomatic or at risk of complications. Minimally invasive techniques (VATS - Video-Assisted Thoracoscopic Surgery) are often preferred.
Management of Complications: Management focuses on preventing and treating complications such as pneumonia, abscesses, and hemoptysis.
Long-Term Follow-Up: After surgical resection, long-term follow-up is important to monitor for any recurrence or complications.
Asymptomatic BPS: Asymptomatic extralobar sequestration may be monitored, but surgical resection is often recommended, especially in children, to prevent future complications.