What Is Intestinal Lymphangiectasia?

Intestinal lymphangiectasis is caused by obstruction of intestinal lymphatic reflux and increased pressure in the lymphatic vessels due to primary or secondary causes, causing leakage of lymph fluid from the small intestinal mucosa or lymphatic vessels, causing hypoproteinemia, celiac disease, and chylous The clinical syndrome of ascites and peripheral lymphopenia is a rare gastrointestinal disease, and it is also a representative disease that causes protein-losing bowel disease. According to the cause of the disease, this sign is divided into primary and secondary. Primary small intestinal lymphangiectasia is congenital lymphangiopathy, with an average age of onset of 11 years; secondary small intestinal lymphangiectasis is caused by other diseases, and can occur at any age with an average age of 23 years.

Basic Information

Visiting department: Gastroenterology
Common locations: Small intestine

Common causes: Can be primary or secondary due to tumor, infection, etc.
Common symptoms: Edema, intermittent diarrhea, abdominal pain, bloating, nausea, etc., abdominal or pleural effusion, infection

Causes of Small Intestinal Lymphangiectasia

1. Primary small intestinal lymphangiectasis This symptom is also called Waldman's disease or Milroy's disease. It is caused by a defect in the development of congenital lymphatic vessels, and can be accompanied by a defect in the development of lymphatic vessels in other parts. The patient's lymphatic dilatation and dysfunction caused her lymphatic vessels to not properly receive chylomicrons and lymphatic reflux.
2. Secondary small intestinal lymphangiectasis Secondary small intestinal lymphangiectasis is other diseases that cause small intestinal lymphangiectasis. The following factors are common.
(1) Lymphatic reflux disorders caused by direct involvement of the lymphatic system Autoimmune diseases: systemic lupus erythematosus, dermatomyositis, systemic sclerosis, etc. can cause extensive inflammatory hyperplasia of the mesentery and retroperitoneum, and lymphatic vessels in this area are narrowed or occluded. Causes lymphatic reflux disorder.
Infection: Whipple's disease, filariasis, mesenteric lymph tuberculosis, intestinal tuberculosis, etc. cause inflammation, narrowing or occlusion of lymphatic vessels and surrounding tissues, making small bowel lymphatic reflux disorder.
Tumors: Mesenteric root lymphoma, retroperitoneal fibroma, retroperitoneal mesothelioma, lymph node metastasis, and other tumors infiltrate and compress the intestinal lymphatics, leading to obstruction to reflux.
Abdominal trauma, surgery: Abdominal trauma, surgery, radiation therapy and other damage to lymphatic vessels, resulting in reflux disorders.
Other diseases: chronic pancreatitis, Crohn's disease, Behcet syndrome, etc.
(2) Increased intravascular pressure indirectly affects bowel lymphocytic constrictive pericarditis, congestive heart failure, portal hypertension, Budd-Chiari syndrome, superior and inferior vena cava thrombosis, and postoperative congenital heart disease.
3. The obstruction of other lymphatic reflux leads to an increase in pressure in the intestinal lymphatics, which prevents the absorption of chylomicrons and fat-soluble vitamins, and also prevents small intestinal lymphocytes from entering the peripheral blood circulation for recycling.

Clinical manifestations of intestinal lymphangiectasis

1. Edema Edema is the most prominent clinical manifestation of this disease. Edema is intermittent at first, and then persistent, and may be symmetrical or asymmetric.
2. Digestive system symptoms may include intermittent diarrhea, abdominal pain, bloating, nausea, etc., osmotic or exudative diarrhea, severe cases have fatty diarrhea, and most patients have malabsorption syndrome.
3. Some patients with peritoneal or pleural effusion may have leaky or chylopleural effusion. Due to the involvement of the small intestinal serosa or mesenteric lymphatics, patients may develop chylous ascites.
4. Infections Due to the loss of a large amount of protein, the patient's immunity is low, and opportunistic infections can occur.

Small bowel lymphangiectasis test

1. Physical examination Adolescent patients can suffer from malnutrition and developmental disorders due to a large amount of protein loss. Physical examination shows that the level of growth and development is lower than healthy people of the same age, and edema can be touched.
2. Laboratory examination (1) Blood routine examination: blood cell count decreased, lymphocyte ratio decreased, and erythrocyte sedimentation increased.
(2) Blood biochemical examination: hypoalbuminemia with reduced albumin and globulin.
(3) Electrolyte: Hypocalcemia.
(4) Routine examination of feces: Fecal fat content increases.
(5) Plasma protein detection: transferrin, ceruloplasmin, and fibrinogen decreased slightly.
(6) Immunoglobulin detection: IgG is significantly reduced, IgA and IgM are slightly reduced, and complement is reduced.
3. Imaging examination (1) Barium angiography of the small intestine: thickening of the jejunum ileum mucosa, smooth nodular protrusions or multiple small needle-like filling defects, mild expansion of the jejunum may be accompanied by fluid level, which may indicate the disease .
(2) Capsule endoscopy and enteroscopy: white thick small intestinal villi can be seen, and white nodules can be formed in severe cases. It can also appear as diffusely distributed white spots with characteristic "snowflake-like" changes in the small intestinal mucosa. Endoscopic diagnosis is of high value, and mucosal biopsy can be taken to obtain pathological diagnosis.
(3) Lymphangiography: Mesentery lymph vessels are distorted, narrowed, and varicose. Contrast agent is retained or reversed into the mesenteric lymphatics. If there is a lymphatic intestinal fistula, the contrast agent can leak into the small intestine.
(4) Radionuclide lymph imaging: The small intestine is visualized and radioactive material accumulates in the ileocecal region, which is highly suggestive of the disease; those with chyloascites or pleural effusion may have radioactive concentration in the abdominal or thoracic cavity. Can be used to diagnose lymphedema.
(5) CT examination: diffuse mesenteric edema, thickening, thickening of the intestinal wall of the small intestine, showing a "halo sign", weakening of the intestinal wall. Can be combined with lymphangiography to improve the accuracy of the location and extent of retroperitoneal lymphatic obstruction.
(6) MRI examination: It can be seen that the intestinal wall of the small intestine is thickened, and a large amount of fluid gathers around the mesentery vessels and portal veins. The "halo sign" in CT shows a subserosal or submucosal annular fluid, and the intestinal wall weakens.
4. Pathological examination showed that the lymphatic vessels of the mucosa and submucosa were dilated, the top of the villi was most obvious, and the surrounding inflammation was not obvious. Fat-filled macrophages were seen, and lipofuscin deposition was seen under the serosa.

Small Intestinal Lymphangiectasia

1. Patients may develop gastrointestinal symptoms such as edema, diarrhea, abdominal pain, bloating and nausea, growth retardation caused by nutrient deficiency or lack of fat-soluble vitamins and trace elements.
2. Laboratory tests suggest hypoproteinemia and peripheral blood lymphopenia, which is highly suggestive of the disease. Imaging examination showed that the lymphatic vessels in the mesentery area were twisted and stenoses. Endoscopic biopsy can confirm the diagnosis.

Differential diagnosis of small intestinal lymphangiectasia

1. Malabsorption Malabsorption includes short bowel syndrome, intestinal malabsorption, patients with undigested food in their stools, and blood routinely without lymphocytes are significantly reduced.
2. Chronic wasting diseases of the intestinal tract Chronic wasting diseases of the intestinal tract such as Crohn's disease, intestinal tuberculosis, etc. Patients' stools contain red blood cells and white blood cells, and colonoscopy shows changes in mucosal erosion, granulomas, and inflammatory cell infiltration. No lymphocytes were significantly reduced.
3. Eosinophilic gastroenteritis increased eosinophils in peripheral blood and bone marrow, and infiltration of eosinophils in gastrointestinal mucosa.
4. Patients with allergic purpura are characterized by abdominal pain, bloody stool, rash, and scarlet fever-like bleeding in the intestinal mucosa.
5. Whipple disease Whipple disease is often accompanied by recurrent migratory polyarthritis, systemic lymphadenopathy, bloody stools, anemia, an increase in the number of white blood cells, and an increase in the total number of platelets. PAS (periodate Schiff staining) can be seen on the biopsy tissue. Positive foamy macrophages.

Small Intestinal Lymphangiectasis Treatment

1. Symptomatic treatment of patients with obvious edema can use appropriate diuretics and intravenous infusion of albumin, and those who need surgery and invasive examination can also intravenously inject albumin. Those with gastrointestinal symptoms can be given symptomatic treatment. The patient's immunity is low, but the incidence of opportunistic infections is low, and prophylactic antibiotics are not required.
2. Treatment of basic diseases For patients with secondary small bowel lymphangiectasis, attention should be paid to the treatment of basic diseases. Some of these patients can completely relieve the small bowel lymphangiectasis by treating the primary disease, such as constrictive pericarditis, cavities Venous thrombosis, Budd-Chiari syndrome, etc. Treatment of underlying diseases with permanently obstructed lymphatic vessels does not alleviate the symptoms caused by lymphatic dilatation of the small intestine.
3. Surgical treatment The lymphatic vessel-venous anastomosis shunt can be performed by microsurgical techniques after the location of the obstructed lymphatic vessel itself is determined.
4. Diet therapy should be given to patients with a nutrient-rich, low-fat diet, and medium chain triacylglycerol (MCT) should be used instead of long chain triacylglycerol in the ordinary diet. Medium-chain triacylglycerol is absorbed by the blood and directly enters the portal vein system, which can reduce intestinal lymphatic pressure and reduce lymphatic dilatation. The MCT diet can be used as a basic treatment for this disease, and if it is effective, it can be used for a long time.

Prognosis of small intestinal lymphangiectasia

The disease can progress to lymphoma. Patients with primary intestinal lymphangiectasis have a better prognosis after early diagnosis and early treatment, and have better surgical outcomes. In patients with secondary intestinal lymphangiectasia, if the lymphatic vessels are not permanently occluded, the intestinal dilatation can be completely relieved after the underlying disease is treated.