Overview
Intralobar congenital pulmonary sequestration (also called intralobar pulmonary sequestration or intralobar sequestration) is a rare birth defect of the lungs. In this condition, a mass of abnormal lung tissue forms within the normal lung but does not connect to the airways like healthy lung tissue does. This means the abnormal tissue cannot function in breathing. Instead, it receives its blood supply from an abnormal artery, usually coming directly from the aorta (the body's main artery), rather than from the normal pulmonary arteries. Because this tissue is trapped inside the normal lung lining (the pleura), it is called 'intralobar,' distinguishing it from the 'extralobar' type, which sits outside the lung's covering. Many people with intralobar sequestration may not have symptoms early in life, but the abnormal tissue is prone to repeated infections. Common symptoms include recurrent pneumonia, chronic cough, and sometimes coughing up blood. Most cases are found in the lower lobes of the lung, especially on the left side. Diagnosis is often made after someone has repeated lung infections that keep coming back in the same area. The main treatment is surgical removal of the affected portion of the lung, which is usually curative. In most cases, surgery leads to excellent outcomes and prevents further infections. Some newer approaches include blocking the abnormal blood vessel using minimally invasive techniques (embolization), though surgery remains the standard of care. Early diagnosis and treatment help prevent complications such as chronic lung damage from repeated infections.
Also known as:
Key symptoms:
Recurrent pneumonia in the same area of the lungChronic coughCoughing up blood (hemoptysis)Chest painShortness of breathFever with lung infectionsWheezingFatigue from repeated infectionsAbnormal breath sounds on one side of the chestLung abscess formationChronic sputum production
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Intralobar congenital pulmonary sequestration.
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Specialists
View all specialists →No specialists are currently listed for Intralobar congenital pulmonary sequestration.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Intralobar congenital pulmonary sequestration.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Where exactly is the sequestration located in my (or my child's) lung, and how large is it?,What type of surgery do you recommend, and can it be done with minimally invasive techniques?,When is the best time to have surgery — should we wait or act now?,What are the risks of surgery compared to the risks of leaving it untreated?,How many surgeries like this have you performed, and what outcomes do you typically see?,Will my (or my child's) lung function be normal after surgery?,Are there any long-term follow-up appointments or imaging studies needed after treatment?
Common questions about Intralobar congenital pulmonary sequestration
What is Intralobar congenital pulmonary sequestration?
Intralobar congenital pulmonary sequestration (also called intralobar pulmonary sequestration or intralobar sequestration) is a rare birth defect of the lungs. In this condition, a mass of abnormal lung tissue forms within the normal lung but does not connect to the airways like healthy lung tissue does. This means the abnormal tissue cannot function in breathing. Instead, it receives its blood supply from an abnormal artery, usually coming directly from the aorta (the body's main artery), rather than from the normal pulmonary arteries. Because this tissue is trapped inside the normal lung lin
How is Intralobar congenital pulmonary sequestration inherited?
Intralobar congenital pulmonary sequestration follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.