Pulmonary Sequestration

Learn about symptoms, causes, management of paediatric pulmonary sequestration, and when to contact a paediatric surgeon in Singapore.

What Is Pulmonary Sequestration?

Pulmonary sequestration is a rare congenital (present from birth) lung condition where a piece of abnormal lung tissue doesn't function like a normal lung. This "sequestered" or separated tissue has its own blood supply coming from unusual blood vessels, and it doesn't connect to the normal airways (bronchi) that bring air in and out of the lungs.
Think of it as a piece of lung tissue that got "left behind" during development. It sits in or near the lungs but doesn't help with breathing. Because this tissue doesn't connect to the airways, it can't participate in normal breathing and may fill with fluid or get infected.
There are two main types of pulmonary sequestration, and understanding which type affects your child is crucial for planning congenital lung sequestration treatment.

Extralobar Sequestration

Less common (about 25% of cases), this anomaly has its own separate covering. The abnormal tissue is completely separate from the normal lung and has its own membrane around it. This type is often found in babies and may be discovered before birth on a prenatal ultrasound or shortly after birth.

Intralobar Sequestration

This is the most common type (about 75% of cases). The abnormal lung tissue is located inside the normal lung, within the same covering (pleura) that wraps around the lung. It's embedded within a lobe of the lung but doesn't function. This type usually shows up later in childhood or even adulthood, and often gets infected repeatedly.
Both types may require bronchopulmonary sequestration surgery to prevent complications and improve lung health.

Common Symptoms of Pulmonary Sequestration

Symptoms vary depending on the type and size of the sequestratio

Extralobar Sequestration (Usually in Babies)

Many babies with this type have no symptoms at all. When found before birth or checked after birth, they may appear completely healthy. Some babies may have:

Trouble breathing if the sequestration is large
Fast breathing
Blue skin colour if oxygen levels are low
Heart problems if the abnormal blood vessels affect heart function

Often, paediatric extralobar sequestration is discovered by chance during tests for other reasons or on prenatal ultrasound.

Intralobar Sequestration (Usually in Older Children)

This type typically causes symptoms because it gets infected repeatedly:

Frequent lung infections (pneumonia) in the same area that keep coming back
Persistent cough that doesn't go away
Coughing up blood in some cases
Chest pain in the affected area
Fever during infection episodes
Shortness of breath
Wheezing or difficulty breathing

An indication for paediatric intralobar sequestration is recurrent pneumonia in the same spot despite antibiotic treatment.

No Symptoms

Some children, especially with small sequestrations, have no symptoms. The condition is found on a chest X-ray or CT scan done for another reason.

When to Seek Care

Contact us if your child has lung infections that keep coming back in the same area, a persistent cough that doesn't improve, coughing up blood, or if sequestration was found on a prenatal ultrasound. We'll evaluate whether bronchopulmonary sequestration surgery is needed for congenital lung sequestration treatment.

What Causes Pulmonary Sequestration in Children?

Pulmonary sequestration develops during pregnancy when the lungs are forming. It's a type of birth defect affecting lung development.
During weeks 4-7 of pregnancy, the lungs start forming and begin to develop their complex structure of airways and blood vessels. Normally, lung tissue develops with proper connections to the windpipe (airways) and gets its blood supply from the pulmonary arteries (the normal lung blood vessels).
In pulmonary sequestration, something goes wrong during this process. A piece of lung tissue develops separately and doesn't connect to the normal airways. It gets blood supply from abnormal arteries, like from the aorta or other body arteries, instead of lung arteries. This creates a piece of "functionless" lung tissue that doesn't participate in breathing.
The exact cause isn't fully understood. It appears to be a random developmental variation that occurs during the complex process of lung formation. Important points for parents:

You didn't cause this during pregnancy
Nothing can prevent it
It's not inherited or genetic in most cases
It happens by chance during development

Consequences of Untreated Pulmonary Sequestration

While some small sequestrations cause no problems, leaving pulmonary sequestration untreated can lead to significant complications.

Repeated Lung Infections

The most common problem, especially with paediatric intralobar sequestration, is recurrent pneumonia. The sequestered tissue doesn't drain properly and can become infected repeatedly. Each infection causes:

Pain, fever, and coughing
Antibiotic treatment that may not fully clear the infection
Missed school and activities
Damage to the surrounding healthy lung tissue over time

Without bronchopulmonary sequestration surgery, these infections keep coming back because the underlying problem - the abnormal tissue - remains.

Bleeding

The abnormal blood vessels supplying pulmonary sequestration can rupture and bleed. This may cause your child to cough up blood or bleed into the chest cavity in severe cases, needing emergency treatment.
Blood vessels in sequestrations are abnormal and fragile, making bleeding more likely than in normal lung tissue.

Abscess Formation

Infected sequestrations can develop abscesses (pockets of pus). This causes severe pain, high fever, and more extensive damage to lung tissue. It may need drainage procedures or more complicated surgery later if not treated promptly.

Heart Strain

The abnormal blood vessels in pulmonary sequestration create extra work for the heart. Large sequestrations with significant abnormal blood flow can cause:

Heart enlargement as it works harder to pump blood
Heart failure in severe, untreated cases, especially in babies
High blood pressure in the lungs (pulmonary hypertension)

This is more common with large paediatric extralobar sequestration found in infants.

Collapsed Lung

Sequestrations can cause air leaks leading to pneumothorax (collapsed lung). This causes sudden chest pain and severe breathing difficulty, requiring immediate medical treatment.

Chronic Breathing Problems

Large sequestrations take up space in the chest and can press on normal lung tissue. Over time, this causes reduced lung capacity, shortness of breath, exercise intolerance, and fatigue.

Rare Cancer Risk

While extremely rare, there have been reported cases of cancer developing in long-standing sequestrations. This small risk is another reason doctors often recommend bronchopulmonary sequestration surgery even for asymptomatic cases.

Impact on Growth and Development

Children with frequent infections and breathing problems may experience poor weight gain and delayed growth. Your child may have frequent absences from school, and cannot participate in sports and activities as much as their peers.
Timely congenital lung sequestration treatment prevents these complications and allows normal childhood development.

How to Treat Pulmonary Sequestration in Children

The definitive treatment for pulmonary sequestration is surgical removal of the abnormal tissue through bronchopulmonary sequestration surgery. At The Little Surgery, we provide comprehensive care starting with a thorough evaluation, expert surgical treatment for both intralobar and extralobar sequestration, and complete follow-up for congenital lung sequestration treatment.

Evaluation and Diagnosis

Dr. Ong will listen to your child's lungs, check for signs of infection, assess breathing effort, and review your child's medical history, including any recurrent infections. Blood tests may also be done to check for infection or assess overall health before surgery. Proper diagnosis requires detailed imaging like X-rays, CT, MRI, and ultrasound scans to see the sequestration and its blood supply.
If your child is diagnosed with an active lung infection, the infection must be treated first with a full course of antibiotics. We typically wait 4-6 weeks after the infection clears before performing bronchopulmonary sequestration surgery. This allows inflammation to settle and makes surgery safer.

Pulmonary Sequestration Surgery

Surgery is the only definitive treatment for pulmonary sequestration. The goal is to remove all the abnormal tissue while preserving as much healthy lung tissue as possible.
For extralobar sequestration found in babies, surgery may be done in infancy (often around 6-12 months of age) if the baby is stable and growing well, or earlier if there are symptoms or complications. Since this type has its own separate covering and is not embedded in a normal lung, it can usually be removed completely without removing normal lung tissue. Some very small, asymptomatic paediatric extralobar sequestrations may be watched carefully with regular check-ups.
For intralobar sequestration, surgery is typically recommended once diagnosed and when the child is otherwise healthy without active infection. Since this type is embedded within normal lung tissue, removing it typically requires removing the section (lobe) of the lung that contains it. This is called a lobectomy. Children's lungs are remarkably adaptable; the remaining healthy lung tissue grows and compensates extremely well. Most children who have a lobectomy for paediatric intralobar sequestration go on to have completely normal lung function.
A critical part of bronchopulmonary sequestration surgery is carefully handling the abnormal blood vessels. These vessels often come directly from the aorta (the body's main artery). They can be large and carry high-pressure blood. This is why congenital lung sequestration treatment should be performed by experienced paediatric thoracic surgeons.
Bronchopulmonary sequestration surgery is performed under general anaesthesia, so your child is asleep and comfortable. The surgery typically takes 2-4 hours, depending on complexity.

Minimally Invasive Surgery

In some cases, thoracoscopic (minimally invasive) techniques can be used with several small incisions, a camera to view inside the chest, and specialised instruments to remove the sequestration. Benefits include smaller scars, less pain, and faster recovery. However, not all sequestrations are suitable for minimally invasive surgery. Dr. Ong will discuss which approach is safest and most effective for your child.

Recovery After Surgery

Most children stay 4-7 days in the hospital after pulmonary sequestration surgery. Care includes:

Pain management to keep your child comfortable
Breathing exercises to help the lungs expand fully
Chest tube monitoring and removal when drainage decreases
Gradual increase in activity
Starting with clear liquids and advancing to a regular diet
At home, recovery continues for several weeks. Your child should avoid strenuous activity and contact sports for 4-6 weeks. Breathing exercises must be done, and prescribed medications taken as instructed. Follow-up appointments will be needed to ensure proper healing and lung expansion.

After successful congenital lung sequestration treatment, most children recover completely. The remaining healthy lung tissue compensates fully, and lung function is typically normal. Children can participate in all regular activities, including sports, with no restrictions.

Why Choose The Little Surgery for Pulmonary Sequestration Treatment

We provide comprehensive congenital lung sequestration treatment, including thorough evaluation with advanced imaging, expert pulmonary sequestration surgery for both paediatric intralobar sequestration and paediatric extralobar sequestration, and complete follow-up care.

Experienced Paediatric Surgeon

Dr. Ong Lin Yin has over 20 years of experience in paediatric surgery, including specialised paediatric thoracic surgery. As the former Head of Paediatric Surgery at KK Women's and Children's Hospital, she has successfully performed numerous pulmonary sequestration surgery procedures with excellent outcomes.

Comprehensive Care

We provide a thorough assessment before recommending surgery, including a detailed physical examination and imaging to assess lung function. Dr. Ong uses precise surgical techniques for congenital lung sequestration treatment to control abnormal blood vessels and preserve maximum healthy lung tissue.
For complex cases, we work with other specialists, like paediatric cardiologists when heart issues are present, or neonatologists for babies with paediatric extralobar sequestration, etc. This approach ensures comprehensive care for pulmonary sequestration.
After pulmonary sequestration surgery, we provide ongoing care, including monitoring of lung healing and re-expansion, follow-up imaging, and management of any concerns so your child can return to all normal activities.

Family Support

We understand that having a child with a lung condition requiring surgery can be stressful. Dr. Ong provides clear explanations of your child’s condition, a comprehensive discussion on surgery, and compassionate support throughout the treatment journey
As a mother herself, she understands family concerns and ensures you feel informed and comfortable.

Contact The Little Surgery Today

If your child has been diagnosed with pulmonary sequestration or any signs of lung problems, we can help.
Contact The Little Surgery today and take the first step toward ensuring your child's health and happiness.