What is Intestinal Lymphangiectasia?

Mary McMahon

Intestinal lymphangiectasia is a rare condition in which the lymph vessels which connect to the small intestine become enlarged, which hinders their ability to transport fluid. This inhibits the body's ability to process fats and absorb protein, and it can lead to serious medical complications. There are a number of causes for intestinal lymphangiectasia to occur, and treatment options usually depend on resolving the cause and adjusting the patient's diet to make it easier for the patient to absorb nutrients.

The lymphatic system, shown in green.
The lymphatic system, shown in green.

In healthy patients, the lymph vessels which connect with the small intestine carry away fats which have been digested by the small intestine, along with proteins. In a patient with intestinal lymphangiectasia, these fluids remain in the intestine instead of being transported in the lymph vessels because the vessels are swollen. The patient experiences diarrhea, and may develop nausea, vomiting, and abdominal pain. Some patients also experience lymphedema.

Intestinal lymphangiectasia disables the small intestine's ability to process fats and absorb protein.
Intestinal lymphangiectasia disables the small intestine's ability to process fats and absorb protein.

This is a malabsorption condition, characterized by difficulties with absorbing necessary dietary nutrients. Intestinal lymphangiectasia is specifically a form of protein-losing enteropathy, meaning that the patient experiences protein loss through the intestines and can develop a condition called hypoproteinemia, characterized by low protein in the blood. Intestinal lymphangiectasia is diagnosed with a patient interview and a biopsy which can be used to identify the enlarged lymph vessels.

In some patients, enlarged lymph vessels are congenital. In others, they may be caused by conditions such as constrictive pericarditis or pancreatitis. In these cases, the patient can be treated, and he or she may recover normal bowel function over time. While the patient is recovering, a specialty diet will be used to keep the patient healthy. This diet is used in the long term for patients with congenital intestinal lymphangiectasia.

The diet for patients with this condition is low in fat and high in protein to facilitate absorption of nutrients. The patient also takes dietary supplements to confirm that she or he gets necessary dietary nutrients, including vitamins and minerals. While it can take time to adjust to the diet, patients with chronic intestinal lymphangiectasia can experience very full lives. When this condition is diagnosed in young children, parents should make sure to educate caregivers about the need for a restrictive diet and the necessity for taking supplements at the right time, to make it clear that the child's diet addresses a health concern and that high-fat treats or snacks should not be provided.

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