What is chylothorax?
Chylothorax is a rare condition in which lymphatic fluid leaks into the space between the lung and chest wall. When this fluid builds up in the lungs, it can cause a severe cough, chest pain and difficulty breathing.
Chylothorax is a lymphatic flow disorder. This group of diseases is 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.
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.
Causes of chylothorax
Chylothorax has many potential causes, including:
- Injuries to the central lymphatic system due to surgery or trauma (this is also known as traumatic chylothorax)
- Congenital lymphatic malformations such as lymphangiomatosis
- Systemic diseases or infections such as tuberculosis
- Congenital syndromes such as Gorham-Stout, Noonan and Turner syndromes
- Elevated venous pressures due to heart failure, pulmonary hypertension or congenital heart surgery
Symptoms of chylothorax
Initially, chylothorax causes few symptoms. When enough fluid accumulates, affected individuals will have difficulty breathing, a cough and chest discomfort.
Diagnosis of chylothorax
Doctors use a combination of imaging tests to diagnose chylothorax. Tests may include X-ray, CT, ultrasound or MRI. A diagnosis of chylothorax is made when these tests demonstrate the presence of fluid in the pleural spaces.
Your child’s doctor may also test the fluid that has accumulated after it has been drained to see if it originated in the lymphatic system. Additionally, lymphatic imaging modalities, such as intranodal lymphangiography and dynamic contrast magnetic resonance lymphangiography, might be used to determine the exact source of the lymphatic leak.
Treatment for chylothorax
Treatment of chylothorax depends on its cause. At The Children's Hospital of Philadelphia, patients are treated by a team of experts who specialize in lymphatic imaging and interventions through the Lymphatic Imaging and Intervention Program. The team will determine the best treatment approach for each patient’s condition.
When lymphatic leaks are found, they can be sealed using two similar lymphatic interventional procedures:
- Thoracic duct embolization, during which the entire thoracic duct is embolized, or sealed shut
- Selective lymphatic duct embolization, a more targeted procedure in which only certain ducts are embolized, sparing the thoracic duct
Long-term outlook for chylothorax
The long-term outlook for many patients with chylothorax is very good. Nearly all patients with thoracic duct trauma can be cured. Experts at CHOP have also seen encouraging results when treating patients with other types of chylothorax. Treatment often provides a long-term cure.
Follow-up care for chylothorax
Most of the follow-up care will be done by your or your child’s primary physician in coordination with our team for special needs related to the procedure or the lymphatic disorder. This can include recommendations for new medications, medication weans or diet recommendations.
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
Our team has the most experience in the world treating both children and adults with chylothorax. We have developed a variety of innovative imaging and interventional techniques that have advanced the minimally invasive treatment options available to patients.
Savla JJ, Itkin M, Rossano JW, Dori Y. Post-Operative Chylothorax in Patients With Congenital Heart Disease. J Am Coll Cardiol. 2017 May 16;69(19):2410-2422. doi: 10.1016/j.jacc.2017.03.021. PMID: 28494978.
Weissler JM, Cho EH, Koltz PF, Carney MJ, Itkin M, Laje P, Levin LS, Dori Y, Kanchwala SK, Kovach SJ. Lymphovenous Anastomosis for the Treatment of Chylothorax in Infants: A Novel Microsurgical Approach to a Devastating Problem. Plast Reconstr Surg. 2018 Jun;141(6):1502-1507. PMID: 29794709
Vaiyani D., Saravanan M., Dori Y., Pinto E., Gillespie M.J., Rome J.J., Goldberg D.J., Smith C.L., O’Byrne M.L., DeWitt A.G., Ravishankar C. Post-operative Chylothorax in Patients with Repaired Transposition of the Great Arteries. 2021. Pediatric Cardiology. 10.1007/s00246-021-02774-z. PMID: 34841467
Reviewed by Yoav Dori, MD, PhD