Pulmonary Sequestration: Symptoms, Types, and Treatment

Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026

Quick Answer

Pulmonary sequestration is a rare congenital condition in which a mass of nonfunctional lung tissue is not connected to the normal airways and gets its blood supply from systemic arteries rather than the pulmonary artery. Many people have no symptoms, while others have recurring lung infections or chronic cough. [1] Treatment usually involves surgical removal of the tissue. [1]

What the research says

• In adults, symptoms and infections are common, and tumors turn up in some malformations. The authors of a systematic review of 653 studies covering 980 adults with congenital lung malformations (including intralobar sequestration) note that management of asymptomatic cases remains controversial. In that review, 74.4% of adults came to attention because of symptoms, pneumonia was the most common symptom in congenital pulmonary airway malformation and intralobar sequestration (with over 30% of these patients having recurrent respiratory infections), and 11.7% of the malformations harbored tumors when removed (PubMed: Pederiva et al., Eur Respir Rev, 2025 [2]).

• Removing the tissue in childhood did not harm long-term fitness or quality of life. In a propensity-matched cohort of 194 children with congenital thoracic malformations, including pulmonary sequestration (162 operated on, 32 observed, median follow-up 8.7 years), long-term exercise capacity and quality of life were comparable between the two groups. Thoracoscopic surgery was associated with a shorter postoperative stay, fewer complications, and no deaths (PubMed: Lambrecht et al., Pediatr Surg Int, 2026 [3]).

What Is Pulmonary Sequestration?

Pulmonary sequestration is a developmental anomaly that occurs while a fetus is growing in the womb. [1] It involves the formation of a mass of lung tissue that lacks a connection to the respiratory system, meaning it cannot contribute to the exchange of oxygen and carbon dioxide. A defining characteristic of this condition is its blood supply; the sequestered tissue draws blood from the systemic circulation—specifically the thoracic or abdominal arteries—rather than the pulmonary artery that serves functional lung tissue. [1]

This condition is most frequently identified in the lower left lobe of the lungs. [1] While it is present at birth, many cases are not diagnosed until childhood or even adulthood, often discovered incidentally during imaging for unrelated health concerns. [1] It is distinct from other congenital anomalies, such as congenital pulmonary airway malformation (CPAM), primarily due to its unique systemic blood supply. [1]

Feature	Intralobar Sequestration	Extralobar Sequestration
Location	Inside the normal lung tissue [1]	Separate mass outside the lung [1]
Lining	Shares the lung's pleura	Contained in its own pleura [1]
Prevalence	Most common type [1]	Less common
Discovery	Often in childhood or adulthood	Often diagnosed prenatally

Figure 1: Normal lung anatomy compared with intralobar pulmonary sequestration.

What are the symptoms and complications of pulmonary sequestration?

Many individuals with pulmonary sequestration do not experience noticeable symptoms. [1] When symptoms do occur, they are typically related to respiratory distress or recurrent infections. Children and adults may present with a chronic cough, wheezing, or shortness of breath. [1] One of the most significant indicators is a history of frequent lung infections, such as pneumonia, localized to the same area of the lung.

In severe cases, particularly when a large mass is present during fetal development, complications can arise before birth. These may include pleural effusion (fluid accumulation around the lungs), hydrops fetalis, or heart failure. [1] For adults, the most common issues are hemoptysis (coughing up blood) and persistent infections that do not fully resolve with standard treatments.

How is pulmonary sequestration diagnosed, and how is risk assessed?

Advancements in prenatal care allow many cases of pulmonary sequestration to be identified during routine pregnancy ultrasounds. If a mass is detected prenatally, healthcare providers monitor the fetus closely for signs of fluid buildup or heart strain. In some instances, a shunt may be placed to drain excess fluid before delivery. [1]

For those diagnosed after birth, the primary diagnostic tool is a CT angiogram. [1] This specialized imaging allows doctors to visualize the unique blood supply of the sequestered tissue, which is essential for confirming the diagnosis and planning surgical intervention. Because the condition is not linked to specific genetic changes or family history, it is considered a sporadic developmental event.

Figure 2: From prenatal ultrasound to CT angiography, how pulmonary sequestration is diagnosed.

What are the treatment options for pulmonary sequestration?

The standard treatment for pulmonary sequestration is the surgical removal of the nonfunctional tissue. [1] For children, surgeons typically recommend waiting until the infant is between 6 and 12 months old, provided they are stable and not experiencing severe symptoms. Removing the tissue early helps ensure that the remaining healthy lung tissue has ample space to grow and develop normally.

In adults, the approach may be more conservative. If the sequestration was discovered incidentally and is not causing symptoms or infections, a provider may recommend monitoring rather than immediate surgery. However, if the mass is large or causing complications like coughing up blood, surgical removal remains the preferred path. Following successful surgery, most patients experience an excellent prognosis with no long-term respiratory limitations. [1]

Figure 3: Minimally invasive removal of the sequestration mass, followed by recovery and normal growth.

Conclusion and Next Steps

Pulmonary sequestration is a manageable congenital condition that, in most cases, carries an excellent long-term outlook. Early diagnosis and timely surgical intervention are key to preventing recurrent infections and ensuring healthy lung growth in children. If you or your child has been diagnosed, work closely with a respiratory specialist to determine the best timing for treatment. For those who remain asymptomatic, regular check-ups can help monitor the condition and ensure that any new symptoms are addressed promptly.

Frequently Asked Questions

1. Is pulmonary sequestration hereditary?

No, it is not known to run in families or be caused by specific genetic changes.

2. Can it be diagnosed before birth?

Yes, many cases are found during routine prenatal ultrasounds during pregnancy.

3. Where is sequestered tissue usually found?

Sequestered tissue is most commonly located in the lower left lobe of the lungs.

4. What is the main difference between sequestered and normal lung tissue?

Sequestered tissue is not connected to the airways and receives blood from systemic arteries rather than the pulmonary artery.

5. Do all cases require surgery?

Small, asymptomatic masses located outside the lungs may not require immediate treatment, but surgery is the standard for most cases.

6. What are the common symptoms in children?

Chronic cough, wheezing, and frequent bouts of pneumonia are common signs.

7. Is the surgery dangerous for infants?

While all surgery carries risks, it is typically safe to wait until a child is 6 to 12 months old for the procedure.

8. Can adults be diagnosed for the first time?

Yes, many people are not diagnosed until adulthood, often when they seek care for recurring infections.

9. Does the extra tissue grow over time?

No, the sequestered tissue does not grow larger after birth and may even shrink in some cases.

10. What is the long-term outlook after surgery?

Most children have an excellent prognosis, with their lungs growing normally and no long-term issues.

Related Reading

Bronchogenic Cysts: The Chest Growth You're Born With, Explained covers another lung birth defect found on prenatal or chest imaging.

20-Week Ultrasound (Anatomy Scan): What It Checks, How to Prepare and What Results Mean explains the pregnancy scan where many lung lesions are first seen.

Video-Assisted Thoracic Surgery (VATS): Procedure and Benefits explains the keyhole chest surgery often used to remove sequestrations.

Lobectomy: Lung Surgery, Recovery, Risks and Outlook explains surgery to remove a lung lobe and recovery afterward.

References

1. Cleveland Clinic. Pulmonary Sequestration. Cleveland Clinic. Last updated May 14, 2026. https://my.clevelandclinic.org/health/diseases/pulmonary-sequestration

2. Pederiva F, Dalena P, Pasqua N, et al. Risk of malignant transformation and infections in congenital lung malformations in adults: a systematic review. Eur Respir Rev. 2025;34(176):240254. doi:10.1183/16000617.0254-2024. PMID: 40174959.

3. Lambrecht S, Elrod J, Thater G, et al. Long-term outcomes after operative versus conservative management of congenital thoracic malformations: a propensity-matched cohort study. Pediatr Surg Int. 2026;42(1). doi:10.1007/s00383-026-06444-0. PMID: 42043561.

Medical Disclaimer

This article is for informational purposes only and does not constitute medical advice. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read here.