Lymphangiectasia

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Lymphangiectasia is used to describe the dilation of the lymph vessels, especially in the intestine, leading to intestinal lymphangiectasia. It occurs as a result of lymphatic damage due to external causes. The symptoms range from mild to severe and can lead to complications. What is Lymphangiectasia? Lymphangiectasia is used to describe the dilation of the […] Read More

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Lymphangiectasia

Lymphangiectasia is used to describe the dilation of the lymph vessels, especially in the intestine, leading to intestinal lymphangiectasia. It occurs as a result of lymphatic damage due to external causes. The symptoms range from mild to severe and can lead to complications.

What is Lymphangiectasia?

Lymphangiectasia is used to describe the dilation of the lymph vessels, especially in the intestine, leading to intestinal lymphangiectasia.

Lymphangiectasia occurs as a result of lymphatic damage due to external causes, which could result in the blockage of lymphatic drainage, making the lymph rapture and split its content into the colon. It occurs in adults due to late sequels of mastectomy and radiation therapy. 

Intestinal Lymphangiectasia

Intestinal lymphangiectasia is rare conditions in which protein-losing gastroenteropathy involving dilatation of the intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract, resulting in the conditions such as:

  • the hypoproteinemia-low protein contained in the body,
  • edema, 
  • lymphocytopenia-decrease in white blood cell the blood,
  • hypogammaglobinemia; low immunoglobulin G in the blood and
  • immunologic anomalies.

Intestinal lymphangiectasia is generally diagnosed before one is up to 3 years but may be found in older patients. Prevalence is still unknown, but it has its main symptom, predominantly bilateral lower limb edema. 

Types of Intestinal Lymphangiectasia

  • Primary intestinal lymphangiectasia, 

It is also called “Waldmann’s disease”. When the abnormality of the lymph vessels of the intestine occurs in children. It could be congenital as it could result from improperly formed intestinal lymph vessels or blockage of lymph flow from the intestines.

  • Acquire intestinal lymphangiectasia

It is mostly found in adults. One is not born with it but it is could occur due to certain external causes such as amyloidosis.


Complications of Lymphangiectasia

Complications include:

  • Malignant transformation or lymphoma.
  • Multiple cutaneous warts in association with lymphoma.
  • Risk of infection.
  • Gelatinous transformation of the bone marrow.

Diagnosis of Lymphangiectasia

This condition could be diagnosed using:

  • A blood test to ascertain protein level in the blood.
  • Computerize tomography scan.
  • Biopsy from the lining of the small intestine to reveal the rapture and state of the lymphoid vessels.
  • Endoscopic examination.
  • Lymphoscintigraphy can be done.

Treatment

After finding out the main cause of the disorder, treatment can involve:

  • High-protein diet
  • Low fat with medium-chain triglycerides and some other supplements such as antiplasmin, octreotide, etc, are also given to aid manage symptoms.
  • Surgery is recommended in extreme cases.
  • Sometimes albumin infusion is done.
  • Corticosteroid is also administered.

Symptoms

The symptoms of lymphangiectasia include:

In children:

  • Edema moderate or severe.
  • Pericarditis or chylous ascites-stiffening of the sac that envelops the heart.
  • Fatigue.
  • Abdominal pain, with abdominal mass which can be felt at the upper part of the stomach.
  • Weight loss.
  • Inability to gain weight. 
  • Moderate diarrhea fat-soluble vitamin deficiencies due to the inability to absorb nutrients properly.
  • Hypocalcemia-low calcium in the body, leading to convulsions.

In adults, it could also include some symptoms such as:

  • Osteomalacia vitamin D deficiency.
  • Iron deficiency with anemia occurs due to specific multiple ulcers in the small intestine.
  • Malabsorption syndrome can be found.
  • Edema.
  • Inflammation of the pancreas (pancreatitis).

 

Causes

The causes of lymphangiectasia include:

  • Congenital factors
  • Mechanical processes and infiltration such as malignancy and amyloidosis

 

FAQ

  • Can lymphangiectasia be cured?

Lymphangiectasia, unfortunately, has no cure ye but is been managed with dietary restrictions, involving a low-fat diet and supplementation of a specific type of fat that could be more easily absorbed by individuals with this disorder.

  • What is conjunctival lymphangiectasia?

Conjunctival lymphangiectasia can be described as the obstruction of lymphatic channels, with the actual cause often unknown. Here, the conjunctiva is chronically swollen.

  • What is protein-losing enteropathy?

Protein-losing enteropathy (PLE) is said to happen when albumin and other protein-rich substances leak into your intestine. Albumin which contains much protein in the blood serves in transporting hormones and retaining water in the blood.

  • How do you test for protein loss enteropathy?

The test for protein loss enteropathy can be done using Alpha 1 antitrypsin (A1AT), which is an intestinal clearance. It the most common test performed in diagnosing protein-losing enteropathy. It has a high molecular weight, but is minimally degraded in the gut and is excreted intact. Its clearance is calculated after 24 hr stool. 

  • Is Lymphangiectasia contagious?

Lymphangiectasia is not contagious. It is caused by either congenital factors or mechanical processes such as amyloidosis.