These signs include ascites , pleural effusion , and edema of the limbs and trunk. Weight loss is seen with long-term disease. Rupture of the lymphatics causes protein loss into the intestines. The most common cause of lymphangiectasia was congenital malformation of the lymphatics.

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This article has been cited by other articles in PMC. Abstract Primary intestinal lymphangiectasia PIL is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.

PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present.

In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas confined to the gastrointestinal tract stomach, jejunum, midgut, ileum or with extra-intestinal localizations were reported in PIL patients.

A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids.

Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion s occur.

History and definition In , Waldmann et al. These patients had edema associated with hypoproteinemia, low serum albumin and gammaglobulin levels. The total exchangeable albumin pool, assessed with radio-labeled I-albumin, was low in all patients. Daily fecal excretion of I was twice the highest value obtained in controls. Microscope examination of the small intestine biopsies showed variable degrees of dilation of the lymph vessels in the mucosa and submucosa.

The authors also proposed the term "intestinal lymphangiectasia". Epidemiology The prevalence of clinically overt PIL is unknown.

However, PIL can be asymptomatic; it primarily affects children generally diagnosed before 3 years of age and young adults but may be diagnosed later in adults [ 2 , 3 ]. Clinical description A. Moderate serous effusions pleural effusion, pericarditis, chylous ascites are common, and life-threatening anasarca may occur rarely throughout the course of the disease [ 1 , 5 ].

The edema is pitting because the oncotic pressure is low due to hypoalbuminemia resulting from exsudative enteropathy. PIL may be suspected at birth or during pregnancy based on ultrasonography images, which can detect fetal ascites or lower limb lymphedema [ 6 ]. Childhood particularities In children, PIL is generally diagnosed before 3 years of age [ 5 , 7 , 8 ] and may be complicated by fatigue, abdominal pain, nausea, vomiting and weight loss, inability to gain weight and growth retardation [ 5 ].

Malabsorption may cause fat-soluble vitamin deficiencies and hypocalcemia leading to convulsions [ 5 ]. Other clinical findings - Lymphedema is a rare disorder which is usually not associated with another disease, but it may be associated with intestinal lymphangiectasia.

Lymphedema is less pitting than edema due to hypoproteinemia, and is localized to the lower limbs foot, ankle, calf, rarely thigh and predominantly bilateral. Upper limb with hand and forearm involvement, lymphedema of breast and external genitalia with skin thickening may also be present [ 9 ]. In most patients with lymphedema, edema as a consequence of hypoproprotidemia has also been observed. The two types of edema are not always easily distinguished.


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