Lymphatic Disorders

Lymphatic disorders arise when the lymphatic system is disrupted either through congenital malformation, traumatic injury or a change in the lymphatic-systemic circulatory balance.

Overview
Causes
Signs & Symptoms
Diagnosis
Treatment

What are Lymphatic Disorders?

As blood is delivered to the body by the circulatory system, fluid, proteins, immune factors, and cells leak out of blood vessels into body tissues. The lymphatic system is an integrated circulatory system which serves to return this fluid and factors to the circulatory system. It also plays a role in the collection of fats and fat-soluble vitamins from the gastrointestinal (GI) tract. Lymphatic fluid that contains fats is called chyle. Three to five liters of lymph fluid circulate through the human body each day.

Lymphatic disorders arise when this system is disrupted either through congenital malformation, traumatic injury from a medical procedure, or a change in the lymphatic-circulatory balance (e.g. increased central venous pressure (CVP)). Lymphatic disorders may lead to lymphatic leakage resulting in significant losses in critical nutritional, immune, electrolyte and clotting factors. This fluid may also accumulate in parts of the body, such as the chest (chylothorax) or abdomen (chylous ascites). Lymph may also build up in the soft tissues making patients appear puffy (lymphedema). These disorders may pose significant risks to the health of patients.

Who Gets Lymphatic Disorders?

Lymphatic disorders may be seen in several types of patients including those with:

  • Elevated central venous pressure, can be secondary to congenital heart disease or clots in the large veins in the chest
  • Lymphatic outflow obstruction, narrowing or blockage of the largest lymphatic channel in the chest (thoracic duct)
  • Traumatic injury of lymphatic channels following esophageal surgery, lymph node dissection, or other types of surgery
  • Congenital lymphatic disorder, commonly referred to Generalized Lymphatic Anomaly (GLA) or Central Conducting Lymphatic Anomaly (CCLA) 
  • Congenital localized lymphatic malformations, most commonly found in the soft tissues of the head, neck, trunk and extremities

What are the Symptoms of a Lymphatic Disorder?

Symptoms may include:

  • Abnormal accumulation of chyle
    • Chylothorax (chyle within the chest)
    • Chylous ascites (chyle within the abdomen)
    • Chylopericardium (chyle around the heart)
  • Abnormal lymphatic fluid leak or loss
    • Protein-Losing Enteropathy (PLE) (loss through the intestinal tract)
    • Plastic Bronchitis (PB) (leak into the airways)
    • Chylous emesis (loss from the upper GI tract)
    • Chyluria (leak into the urinary tract)
    • Lymphatic leak at surgical sites
    • Lymphedema (leak into the soft tissues of the body wall or extremity)

How are Lymphatic Disorders Diagnosed?

Patients with suspected lymphatic disorders can undergo confirmatory diagnostic testing. Depending on their presenting symptoms, the testing is individualized to the patient. Diagnostic testing may include:

  • Ultrasound evaluation of the blood vessels in the chest 
  • Ultrasound evaluation of the heart (echocardiogram)
  • Ultrasound evaluation of affected soft tissues
  • Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) of affected body parts
  • Cardiac catheterization to identify/treat any cardiac or vascular lesions contributing to lymphatic dysfunction 
  • Direct lymphatic imaging to evaluate functioning of the lymphatic system and to identify abnormal sites of lymphatic leak or accumulation
    • Conventional Intranodal Lymphangiography. Study where contrast dye is directly injected into the lymph nodes and imaged in the lymphatic system by fluoroscopy (X-ray) 
    • Magnetic Resonance Lymphangiography. Study where contrast dye is directly injected into the lymph nodes and imaged in the lymphatic system by MRI
    • Lymphoscintigraphy. Nuclear Medicine study performed by injecting radio-labeled dye into the body and observing its flow through the lymphatic system

How are Lymphatic Disorders Treated?

Lymphatic disorders can be treated in a variety of ways depending on the needs of the individual patient.

Medical therapy

  • Dietary modification 
  • Medication

Minimally-invasive interventional procedures

  • Embolization (occlusion) of leaking lymphatic channels 
  • Opening of blocked/narrowed vessels causing lymphatic leak or abnormal flow
  • Injection of medications to shrink abnormal lymphatic channels (sclerotherapy)

Surgical Treatment

  • Connection of lymphatic channels to veins