What is plastic bronchitis?

Plastic bronchitis is a lymphatic flow disorder that causes severe respiratory issues. In children with plastic bronchitis, lymph fluid builds in the airways and forms rubbery or caulk-like plugs (known as casts). These casts block the airways, making it difficult to breathe.

Lymphatic flow disorders refer to a group of diseases characterized by abnormal circulation of lymph fluid. Lymph vessels carry lymph fluid to veins, where it returns to the bloodstream, playing a crucial role in immune function and fat and protein transport.

Functioning almost like a sponge, the lymphatic system absorbs excess lymph fluid — watery fluid containing protein, electrolytes, cells and other substances — from the body’s organs and returns it to the blood stream.

Injury to the thoracic duct (the main lymph vessel), congenital abnormalities or excessively high venous pressures can result in lymphatic flow problems and leakage of lymphatic fluid into the chest, abdomen or other body cavities. In plastic bronchitis, the abnormal circulation causes lymph to leak into the airways.

What causes plastic bronchitis?

Plastic bronchitis is most prevalent in patients with certain forms of congenital heart disease who have had the Fontan surgery and those with lymphatic abnormalities. It can also be associated with certain lung diseases, infections, and (very rarely) allergies.

Etiologies of Plastic bronchitis

Primary Plastic bronchitis

Pulmonary lymphangiectasia

• Poor pulmonary efferent lymphatic flow
• Aberrant hepatopulmonary connections 

Lymphatic Channel Disorders (Lymphatic malformations)

• RASopathies
• Central conducting lymphatic anomaly (CCLA)
• Lymphangiomatosis/Generalized lymphatic anomaly (GLA)
• Kaposiform lymphatic anomaly (KLA)
• Gorham-Stout disease (GSD)

Secondary Plastic bronchitis

Mechanical Obstruction

• Elevated central venous pressure (CVP)
• Elevated intrathoracic pressure (ITP)
• Obstructing tumors or masses
• Thoracic duct (TD) occlusion
• Obesity 

Primary Non lymphatic causes

• Eosinophilic 
• Asthma
• Allergies
• Aspergillosis 
• Sickle Cell
• Cystic fibrosis 

What are the signs and symptoms of Plastic bronchitis?

The accumulation of material in the airways leads to airway obstruction and respiratory symptoms such as cough and oxygen deprivation (hypoxia). In many cases, the early symptoms are nonspecific and can resemble asthma.

Patients with plastic bronchitis have difficulty breathing and are prone to uncomfortable coughing fits. During these fits, they may cough up the casts. The casts often return within days, as more lymph fluid leaks into the airways. In severe cases, plastic bronchitis can lead to asphyxia (suffocation).

How is Plastic Bronchitis diagnosed? 

Historically, plastic bronchitis was diagnosed when patients coughed up cast material or cast material was found during bronchoscopy, a procedure used to look inside the airways and lungs. Other tests, such as chest X-ray, can show that the affected lung is not expanding normally, but this is not a diagnostic finding by itself. 

The world-renowned experts in the Jill and Mark Fishman Center for Lymphatic Disorders developed a specialized imaging technique - dynamic contrast magnetic resonance lymphangiography (DCMRL) - that is used to pinpoint the exact source of lymphatic leaks. 

During this procedure, an MRI contrast agent (a safe, injectable dye) is injected directly into the lymphatic system and tracked by MRI. 

There are three points of access through which the contrast agent is injected: 

• Intranodal (inguinal lymph nodes)
• Intrahepatic (liver)
• Intramesenteric (mesentery)

Each point of access for DCMRL provides a different view of the lymphatic system for the most comprehensive lymphatic imaging. Patients with Plastic Bronchitis may receive all three points of access, allowing our physicians to clearly see the anatomy of the lymphatic system, the direction and speed of lymphatic flow and the exact source of any leaks. This is especially important in Plastic Bronchitis diagnosis and treatment to identify the source of leak and to target an intervention.

Pulmonary lymphatic perfusion syndrome  

We have found that pulmonary lymphatic perfusion leading to plastic bronchitis fall into 6 different classifications as depicted below.  (Click images to enlarge).

Type	Image	Causes of PLPS	Thoracic duct outlet	Intervention
Type 1		Retrograde flow in single branch	Patent	Selective lymphatic embolization
Type 2		Retrograde flow in multiple branches	Patent	Selective lymphatic embolization
Type 3		Double thoracic duct with one duct perfusing the lungs	One thoracic duct outlet is patent	Selective lymphatic embolization
Type 4		Retrograde flow in multiple branches	Not patent	Lymphovenous anastomosis or thoracic duct embolization
Type 5		Multiple branches perfusing lungs without central thoracic duct	Not patent	Lymphovenous anastomosis or selective lymphatic embolization
Type 6		Hepatopulmonary perfusion	Patent	Selective lymphatic embolization

In some cases, a special blue dye injected into lymphatic channels can further aid localization of lymphatic leaks. 

How is plastic bronchitis treated?

Plastic bronchitis is treated by a team of experts that specialize in lymphatic imaging and interventions. At The Children's Hospital of Philadelphia, patients have access to a specialized Jill and Mark Fishman Center for Lymphatic Disorders. Treatment depends on the exact cause and the patient’s anatomy.

In all patients, the first step involves careful mapping of the anatomy and flow of the lymphatic system. This is done using a specialized MRI technique called dynamic contrast MR lymphangiography. During this procedure, an MRI contrast agent is injected directly into the lymphatic system.

After the MRI procedure a small catheter is placed into the main lymphatic channel, called the thoracic duct, to further outline the abnormal ducts that are surrounding the airway.

After the abnormal ducts are identified, they are sealed using a procedure called selective lymphatic duct embolization. Your or your child’s doctor will use oil, coils, particles, glue or other bonding agents, inserted through a tiny tube (catheter), to seal the ducts.

With this procedure, they are careful not to block the main thoracic duct if possible, an approach that experts at CHOP believe is important for the success of this procedure. Because lymphangiography allows clinicians to pinpoint the exact spot of the leak, they can target their intervention to the affected area, preserving the thoracic duct.

The treatment of plastic bronchitis in patients with heart disease may also include cardiac interventional procedures such as balloon dilation, stent dilation of a narrow vessel, or embolization of an abnormal blood vessel with coils.

You or your child may also be prescribed medications that can reduce inflammation, lower venous pressures or dissolve the casts.

This video shows selective glue embolization of a lymphatic channel originating from the distal thoracic duct that was causing plastic bronchitis.

What is the long-term outlook for patients with Plastic bronchitis?​

The highly advanced treatment options for Plastic bronchitis are cutting-edge, and there is more to learn about them. The team of experts in the Jill and Mark Fishman Center for Lymphatic Disorders is invested in further research to determine the long-term outlook for plastic bronchitis patients and the role of lymphatic imaging and intervention in the treatment of this condition.​

Follow-up care for plastic bronchitis

Follow-up care will be managed primarily by your or your child’s primary physician in coordination with the physicians from the Jill and Mark Fishman Center for Lymphatic Disorders for specific treatments like medication changes or medication weans. In patients with certain types of congenital heart disease, follow-up care will also be managed in coordination with CHOP’s Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program.

Plastic bronchitis research

Experts are collaborating on research to better understand the lymphatic system, develop new lymphatic interventional procedures, and to improve lymphatic imaging modalities.

Why choose us

The cause of plastic bronchitis was discovered by lymphatic experts here. These experts also developed selective lymphatic embolization and dynamic contrast MR lymphangiography, a minimally invasive procedure used to treat the condition. We are currently the only center in the world to offer this new treatment.

Our publications

Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J: A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. Ann Thorac Surg 98(2): 634-640, Aug 2014.

Dori Y, Keller MS, Rychik J, Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics. 2014 Aug;134(2):e590-5. PMID: 25002668

Itkin MG, McCormack FX, Dori Y.: Diagnosis and treatment of lymphatic plastic bronchitis in adults using advanced lymphatic imaging and percutaneous embolization. Annals of the American Thoracic Society 13(10): 1689-1696, Oct 2016.

Dori Y, Keller MS, Rome JJ, Gillespie MJ, Glatz AC, Dodds K, Goldberg DJ, Goldfarb S, Rychik J, Itkin M. Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease. Circulation. 2016 Mar 22;133(12):1160-70. PMID: 26864093

Geanacopoulos AT, Savla JJ, Pogoriler J, Piccione J, Phinizy P, DeWitt AG, Blinder JJ, Pinto E, Itkin M, Dori Y, Goldfarb SB.: Bronchoscopic and histologic findings during lymphatic intervention for plastic bronchitis. Pediatr Pulmonol 53(11): 1574-1581, Nov 2018.