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Living with Congenital pulmonary sequestration

A rare lung condition present from birth in which a piece of lung tissue does not work and has its own odd blood supply.

Understanding Congenital pulmonary sequestration

Congenital pulmonary sequestration is a rare condition present from birth in which non-working lung tissue is separate from the rest of the lung.

The separate lung tissue gets blood from an unusual source, often an artery from the main body circulation. It is called intralobular or extralobular depending on where it sits. It may cause a long-lasting or repeated cough, trouble breathing, or lung infection. It is noticed in newborns.

Signs & symptoms

GARD doesn't list a full symptom list for this condition. Its summary mentions these:

  • Long-lasting or repeated cough
  • Trouble breathing (respiratory distress)
  • Lung infection

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and lung and breathing specialists.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

  4. Connect with expert centers

    Fetal Health Foundation may keep lists of experts for this condition.

Care & treatment

Care team
Genetic specialists (geneticists); Lung and breathing specialists (pulmonologists)
Expert care
Expert lists: Fetal Health Foundation
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Fetal Health Foundation
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Is the sequestration inside or outside the normal lung (intralobular or extralobular)?
Does it need treatment, or can it be watched?
Which specialists should follow my child?

Quick facts

Symptoms usually startNewborn

GARD says changes in genes cause this condition. It doesn't name a gene.

GeneticRespiratory

Also known as: bronchopulmonary sequestration; congenital bronchopulmonary sequestration; congenital sequestration of lung; pulmonary sequestration; sequestered lobe (pulmonary sequestration)

Patient organizations

Source: NIH GARD · Congenital pulmonary sequestrationGARD ID 4593 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Congenital pulmonary sequestration.

Explore the Congenital pulmonary sequestration blog

Explore research for Congenital pulmonary sequestration

Explore actively recruiting Congenital pulmonary sequestration studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Congenital pulmonary sequestration with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • Eligibility is confirmed by the study team

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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

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