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Know How Intestinal Lymphangiectasia is Diagnosed and Treated in Children

Children Paediatrics

Gastrointestinal Sciences

23 December, 2022

A child aged 1 year 8 months presented with extreme generalized edema & increased frequency of stools of 7 months duration. The edema was insidious and gradually progressive, involving the left side more than the right side.

On examination he had a pitting type of anasarca except for the left upper limb, systemic examination showed ascites. Lab evaluation showed gross hypoalbuminemia. Gastroduodenoscopy revealed multiple whitish raised spots in the duodenum-which were suggestive of lymphangiectasia. Histopathological evaluation showed dilated lymphatics, containing eosinophilic proteinaceous substance (lymph), diagnostic of intestinal lymphangiectasia.

The child was advised a low-fat diet and substitution long chain fatty acids with medium-chain fatty acids. Generalized edema decreased. On follow-up, the child has shown symptomatic improvement. Consultation with our best gastroenterologist in India has always been very effective for children's health.

What is Intestinal Lymphangiectasia?

Intestinal lymphangiectasia (idiopathic hypoproteinemia) is a rare disorder in which the lymph vessels supplying the lining of the small intestine become obstructed. This disorder is the result of improperly formed lymph vessels. Diarrhoea is the main symptom. The diagnosis is based on the results of a biopsy.

Once the specific cause of the disorder is treated, following a low-fat, high-protein diet and taking supplements can help manage symptoms.

The lymph vessels from the digestive tract carry digested fats that were absorbed by the small intestine. Sometimes, these lymph vessels are improperly formed at birth, causing blockage of the flow of the lymph fluid. Less commonly, these lymph vessels may become blocked later in life as a result of such conditions as inflammation of the pancreas (pancreatitis) or stiffening of the sac that envelops the heart (constrictive pericarditis). In both cases, pressure builds up in the abnormal lymph vessels. The increased pressure causes lymph fluid to leak back into the intestine, preventing fat and proteins from being absorbed into the bloodstream.

How is Intestinal Lymphangiectasia Diagnosed?

A person with intestinal lymphangiectasia has swelling of one or both legs and diarrhoea. Few of the symptoms like Vomiting, Abdominal pain, Nausea, and Fatty stools might develop. Children have delayed growth. Doctors will use an endoscope (a flexible viewing tube with a light source attached and a camera through which a small clipper can be inserted) to make the diagnosis and remove tissue (biopsy) from the small intestine. A microscope is used to remove the tissue. Depending on the situation, a gastro expert will inject a dye that can be seen through x-rays going into lymph vessels in the foot. The dye travels to the abdomen and chest and can show abnormal intestinal lymph vessels.

Complications are diagnosed with the use of blood tests Patients might have low levels of protein, cholesterol, and WBCs in the blood. The low protein levels lead to tissue swelling. To know more about the diagnosis, visit our gastroenterology hospital in India.

How is Intestinal Lymphangiectasia Treated?

When intestinal lymphangiectasia is caused by a specific condition, the underlying condition is treated. Symptoms can be helped by:

  • Eating a high-protein and low-fat diet.

  • Taking dietary supplements rich in calcium, vitamins, etc.

  • Certain Triglycerides (medium-chain triglycerides) that are absorbed directly into the blood.

Department of Gastrointestinal Sciences

Manipal Hospitals Global