Intestinal lymphangiectasia is an infrequent condition where lymph vessels in the surface of the small intestine are enlarged or plugged. Consequently, fats and proteins which are supposed to be taken to the bloodstream are leaked into the intestinal tract, instead. The result of this is low blood proteins, malabsorption of fats as well as swelling in different parts of body. It may be primary (congenital) or secondary to other diseases and both children and adults have it.
Intestinal lymphangiectasia Symptoms may be mild or severe and may include:
The underlying pathophysiology will be based on whether the lymphangiectasia is primary or secondary:
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To diagnose Intestinal Lymphangiectasia, physicians use blood tests (to investigate protein and lymphocytes levels) and stool tests (to identify fat malabsorption) and endoscopy with biopsy (to study the intestinal lymph vessels directly).
Yes. Although Intestinal Lymphangiectasia is prevalent among children, adults can also get the disease particularly when it comes about as a result of other diseases such as inflammation, infection, heart failure, or tumors.
Flare-ups can be experienced after big fat meals that overwhelm deformed lymph vessels, those related to infections, surgical procedures or fluctuations in drugs that affect lymphatic circulation or the wellness of the intestines.
Cases of Intestinal Lymphangiectasia can be treated with early diagnosis and treatment involving dietary changes, supplements and medical treatment-which is why most individuals with Intestinal Lymphangiectasia lead a normal life with no major influence on life expectancy.
Intestinal Lymphangiectasia (ICD-10), is covered by the code of 90.89 under other malabsorption due to intolerance, not elsewhere classified.
Surgery is rarely required. It might be discussed whether a local lymphatic impairment can be fixed or excised but the majority of patients are successful to diet and medications.
Follow-up consultations with a gastroenterologist should be regularly performed in 3-6 months, or more often, in case of symptom alterations, or in case laboratory test results show the increase of protein loss or malabsorption.
Yes. The cornerstone of the management is a low-fat, high-protein diet enriched with medium-chain triglyceride (MCT) oil. Deficiencies may also be corrected with vitamin supplements (A, D, E, K) and minerals.
Even though some kids improve or get remission due to maturation of their lymphatic system, others are still in need of continued dietary care and medical monitoring till they reach adult age.
