Celiac disease
Celiac disease (celiac disease) is caused by the overflow of lymphatic fluid from the intra-abdominal lymphatic system, resulting in a large amount of celiac fluid in the abdominal cavity. This disease is rare and occurs in children, especially in infants under the age of 1 year.
Etiology
The etiology of celiac disease is complex and can be classified as congenital or acquired.
Congenital is due to congenital abnormalities of the abdominal lymphatic vessels, i.e. thoracic duct, mesenteric lymphatic trunk or celiac pond at the developmental insufficiency, defect, stenosis, etc., resulting in increased pressure, dilation and rupture of the intestinal lymphatic vessels, or congenital cleft.
Acquired as trauma damaged the lymphatic vessels leading to the occurrence of celiac disease; infection in the abdominal cavity, especially mesenteric lymph node tuberculosis or tuberculous peritonitis can be secondary to celiac disease; tumor or fiber band compression can block the lymphatic vessels, and the distal lymphatic vessels are stagnant, dilated and ruptured, forming celiac disease.
[Pathology
The fat in the intestinal lumen is digested into fatty acid and mono-triglyceride and then absorbed by the epithelial cells of the small intestinal mucosa to re-synthesize triglyceride, which then enters the lymphatics in the form of celiac particles. Since it is chyme that enters the lymphatic ducts, the lymphatic fluid in the small intestine lymphatic ducts, chyme pools and thoracic ducts is milky, and its physical properties are white milky fluid, odorless, alkaline reaction, specific gravity of 1.010~1.021, and it can be divided into three layers after resting, the upper layer is milky, the middle layer is watery, and the lower layer is white precipitate. The white blood cell count is about 5×109/L, mainly lymphocytes, and no bacterial growth in culture.
When celiac fluid leaked into the peritoneal cavity causing aseptic chemical peritonitis changes, the peritoneum and mesentery were congested, edematous and hypertrophic, the subplasma membrane of the intestinal wall was covered with white fine curved celiac streaks, and the intestinal canal was pale. Histological examination: peritoneal thickening, fibrous tissue hyperplasia, covered with a layer of inflammatory exudate, vasodilatation, congestion, hemorrhage, and scattered chronic inflammatory cell infiltration, granulation tissue formation, showing chronic proliferative peritonitis changes.
Clinical manifestations
This disease can be acute peritonitis type and chronic peritonitis type.
The acute peritonitis type is less common and develops 4-6 hours after a large number of meals, especially fatty meals, as a result of the sudden entry of celiac fluid into the abdominal cavity, leading to acute chemical peritonitis. It is characterized by acute abdominal pain, initially widespread, variable in location, sometimes colic, and gradually increasing. It is accompanied by nausea, vomiting, abdominal distension, total abdominal pressure or limited pressure pain, often with limited pressure pain and muscle tension in the right lower abdomen or left lower abdomen. The bowel sounds are hyperactive in the early stage and diminish in the late stage, which is easily misdiagnosed as acute appendicitis or perforated ulcer disease.
In the chronic peritonitis type, celiac fluid leaks slowly into the peritoneal cavity, which is less irritating to the peritoneum and has a milder inflammatory response, with no obvious signs of peritoneal irritation. It manifests as gradual abdominal distention, weight loss or no weight gain, hypoproteinemia and malnutrition, which may affect respiratory and circulatory functions in severe cases. The abdominal examination shows abdominal distension, angry abdominal wall veins, and mobile turbid sounds on percussion. Some may see scrotal effusion, or scrotal and lower limb edema.
[Diagnosis
Abdominal puncture to extract celiac ascites is the simplest and most reliable diagnostic method. The ascites is milky white, and the sterile classification is dominated by lymphocytes. According to the above characteristics, it can be distinguished from pseudo-cystic ascites and leaky ascites. Ultrasound examination of the abdomen reveals a large amount of ascites. Lymphangiography can not only determine the cause but also the site and extent of lymphatic fluid leakage, but lymphangiography in the lower extremities of breast children is very difficult, and sometimes even if a fistula is found in the abdomen at the time of imaging, it is difficult to clarify the exact location during surgery.
[Treatment].
After diagnosis, treatment should be given as soon as possible, and death is often caused by complications of bacterial infection or hypoproteinemia if treatment is not adequate. Treatment methods include conservative treatment and surgery.
1.Conservative treatment
Including diet therapy or intravenous high nutrition and puncture fluid. Generally, fasting and total intravenous hypernutrition are used first. After fasting, the lymph flow in the abdominal cavity is reduced, which is conducive to the repair and healing of the ruptured lymphatic trunk, and the course of treatment is 2-4 weeks; then special diet therapy is used, with low fat, medium-chain fatty acid, high protein and multivitamin diet, and the intake of long-chain fatty acid is minimized. After absorbed by the small intestine mucosa, medium-chain fatty acids can be transported directly into the portal vein without going through the intestinal lymphatic system.
If abdominal distension affects respiration, at the same time, abdominal puncture and fluid extraction therapy should be performed to relieve respiratory distress, each puncture should try to extract celiac fluid, according to the rapidity of celiac fluid leaking, generally once every 1 to 2 weeks, some cases are cured by the gradual reduction of ascites.
2.Surgical treatment
For acute celiac disease, traumatic celiac disease, those with obvious primary diseases, such as celiac disease caused by tumors, and those who have been ineffective or aggravated by conservative treatment for 4-6 weeks, surgical treatment is feasible, and the purpose of surgery is to relieve the cause of the disease, suture and ligate the leaky hole or perform shunt surgery.
(1) Surgery to remove the cause: celiac disease may be caused by inflammation, tumor or compression of the common lymphatic trunk by the fibrous girdle. Surgery should be performed to remove the tumor and release the compression by loosening the girdle.
(2) Suture ligation of the celiac leak: in some cases, intraoperative laceration is seen near the root of the mesentery of the posterior abdominal wall, and lymphatic fluid is constantly overflowing from the leak, so the laceration should be ligated and drainage placed. In order to find the fissure easily, some people inject Evan blue as a lymphatic duct indicator from the mesenteric root during surgery to help find the lymphatic duct fissure. It has also been suggested that eating a fatty diet and feeding milk containing sultanas 2 to 5 hours before surgery may help to find the lacunae.
(3) Bypass surgery: For those who cannot find the cause and the cleft intraoperatively, bypass surgery is feasible. There are many types of bypass surgery, the most commonly used are.
(1) abdominal saphenous vein shunt. In other words, the femoral triangle is incised, the saphenous vein is freed, its branches are ligated, and the length of freeing is l2-15 cm, then the distal end is cut off, and the proximal end of the saphenous vein is pulled back into the abdominal cavity through the lowest part of the abdominal cavity, and anastomosed with the peritoneum.
The one-way valve keeps the pressure between the vein and the abdominal cavity at 0.294-0.490kPa (3-5cmH20), so that when the abdominal pressure increases, the celiac fluid can flow directly into the vein and a new balance of celiac circulation can be established. (ii) The balance of the celiac circulation is established.
(3) Lymph node venous shunt: Some people have reported that the enlarged lymph nodes in the abdominal cavity were cured by cutting them open transversely or straight, preserving the lymphatic vessels entering the lymph nodes, and then anastomosing the lymph node sections with the inferior vena cava or iliac vein or its branches.
Alternatively, abdominal drainage alone can be performed for those with unidentified etiologies and fissures. After surgery, conservative treatment can be continued and the disease can be cured.
Prognosis]
Most of this disease can be cured by timely and correct treatment, and the recent and long-term results are good, and there are few recurrences.