Overview
Anterior sinusoidal portal hypertension refers to obstruction of the anterior part of the sinusoids in the liver due to various reasons, which leads to obstruction of blood flow in the portal vein and stagnation of blood, and causes elevation of portal vein pressure. For example, in schistosomal cirrhosis, the deposition of schistosome eggs in the portal vein branches and the confluence area causes luminal obstruction and granulomatous hyperplasia, leading to pre-sinusoidal obstruction and resulting in elevated portal vein pressure.
Etiology
Common etiologies are early schistosomiasis, congenital liver fibrosis, idiopathic portal hypertension, early primary biliary cirrhosis, cholangitis, hepatomegaly, arsenic poisoning, azathioprine hepatotoxicity, myelofibrosis (early stage), tuberculosis, and myeloproliferative disorders.
Symptoms
1. Splenomegaly, hypersplenism
Patients have different degrees of splenomegaly, and the spleen may be temporarily reduced when hemorrhage occurs. Accompanied by hypersplenism, the reduction of platelet and white blood cell counts is the most obvious.
2. Upper gastrointestinal bleeding
When the esophagogastric fundic varices rupture and bleed, the patient will vomit a lot of blood, black stools, etc. Due to portal hypertension, impaired coagulation mechanism and thrombocytopenia, it is often difficult to stop the bleeding on its own.
3. Ascites
Patients often have different degrees of ascites, manifested by abdominal distension, decreased urine volume, dark yellow urine color, etc.
4. Signs of liver disease
Patients often have the appearance of liver disease, with yellow skin and sclera, liver palms and spider nevus. Superficial varicose veins can be seen on the abdominal wall, and those with large amount of ascites look like frog’s abdomen; enlarged liver and spleen can be touched; gynecomastia can be seen in patients with chronic disease.
Examination
1. Blood test
The white blood cell and platelet counts are obviously reduced, and there may be anemia, most of which is orthocytosis and a few of which are normocytosis and hypocytosis.
2. Liver function tests
Liver function indexes are mostly normal or only mildly abnormal.
3. X-ray
X-ray barium meal imaging is the first choice of clinical X-ray. It is the first choice of X-ray examination method, which is convenient, safe and non-invasive. It can show that the esophageal mucosa below the arch of the aorta is worm-like or bead-like filling defects.
4.CT
CT scan is very important for the diagnosis of intrahepatic and extrahepatic portal hypertension, which can not only clearly show the shape of the liver and its contour changes, but also show the changes of the parenchyma and intrahepatic blood vessels, and accurately determine the volume of the liver.
5.MRI
MRI can clearly show the opening of portal vein and its branches, and the detection rate of portal-branch circulation is high compared with that of arterial-portal venography. MRI can clearly show the thrombus of portal vein and its branches and the spongy deformation of portal vein, which is important for the diagnosis of extrahepatic portal hypertension. The imaging parameters are multiple and can be arbitrarily imaged to reflect the collateral circulation more accurately.MRI angiography (MRA), can understand the changes of intra- and extra-hepatic portal vein. However, it is expensive and not suitable for screening.
6. Peritoneal puncture
Peritoneal puncture is used to extract ascites, and routine, biochemical and culture tests are performed on the ascites.
7. Endoscopy
To show esophagogastric fundus varices.
8. Pressure measurement
Portal vein manometry; esophageal varices manometry (EVP).
9. Liver tissue biopsy
Liver tissue changes are still the “gold standard” for the diagnosis of cirrhosis, and every patient with cirrhosis should obtain biopsy specimens through fine needle aspiration or laparoscopic biopsy, dissection, or transvenous biopsy as far as possible for histologic diagnosis.
Diagnosis
The diagnosis of portal hypertension is generally not difficult and can be made based on the three main features of splenomegaly, hypersplenism, lower esophageal varices or upper gastrointestinal bleeding and ascites. In addition to confirming the diagnosis of portal hypertension, it should also determine whether it is intrahepatic or extrahepatic; what is the cause of obstruction. Auxiliary tests: routine blood tests, gastroscopy or barium meal, color Doppler ultrasound, portal venography and liver puncture biopsy. Sometimes it is also necessary to differentiate from other diseases with similar signs and symptoms.
Treatment
1 General treatment and dietary treatment
When the condition of patients with portal hypertension is stabilized and there are no obvious other complications, comprehensive treatment focusing on the cause or related factors can be adopted.
(1) Rest: those with mild condition can participate in general work appropriately, but should reduce the labor time and intensity, pay attention to the combination of work and rest, in order not to feel fatigue to the extent. Those who are more seriously ill or have a recent history of complications such as gastrointestinal hemorrhage should stop working to ensure sufficient bed rest and sleep time to prevent fatigue.
(2) Diet: It is advisable to give sufficient calories and foods rich in various vitamins, which should contain various inorganic salts and trace elements in addition to high sugar, high protein (those with hepatic encephalopathy should limit protein intake), appropriate fat and vitamins. Hard and rough food should be avoided to cause mechanical damage to the esophagogastric mucosa, try to control spicy and stimulating food, and strictly prohibit alcohol consumption.
(3) Etiologic treatment: treatment for the cause of cirrhosis is the basis for reducing portal hypertension, and the cause should be actively eliminated. For example, hepatitis cirrhosis viral replicators are given interferon, immune ribonucleic acid, thymosin and other treatments; alcoholic cirrhosis patients should be forbidden to drink; schistosomiasis cirrhosis can be given praziquantel, nitrothiocyanamide.
(4) Hepatoprotective, enzyme-lowering and anti-yellowing treatments: Vipramine, Hepatol, biphenyl dibenzoate, silymarin tablets, glycyrrhizin, potassium magnesium menthylate, arbutinic deoxycholic acid, sodium protoporphyrin, inosine, coenzyme A, hepatocyte growth-promoting hormone, bitter yellow, rhubarb, and other agents can also be used for treating the disease.
(5) anti-hepatic fibrosis treatment: prevention and treatment of liver fibrosis research in recent years, although there is greater progress, but the clinical efficacy is not ideal. Interferon, prostaglandin, polyunsaturated lecithin, colchicine, penicillamine, monoamine oxidase inhibitors can be given to inhibit the synthesis of collagen fibers, which has a certain effect. Chinese medicinal preparations such as danshen, compound turtle shell tablets, cordyceps mycelium, hanfengji methylin, etc. can also be given.
(6) Treatment of ascites: the appearance of ascites is a manifestation of liver function damage to a certain extent, the worse the liver function, the more difficult to eliminate ascites. It should be treated by controlling sodium intake and promoting sodium discharge. The main methods to promote the discharge of water and sodium are: giving diuretics, catheterization, peritoneal puncture and drainage or ascites autotransfusion treatment. There is a decrease in plasma serum protein, serum protein and plasma can be imported. If there is secondary abdominal infection, broad-spectrum antibiotic treatment can be given. If the ascites is cancerous, according to the nature of the cancer abdominal cavity puncture chemotherapy.
2. Emergency hemostatic treatment for acute hemorrhage
In case of rupture and bleeding of esophagogastric fundus varices, vasopressin and triple-lumen double-bladder tube can be used to stop bleeding.
3. Endoscopic treatment
With the wide development of gastroscopy, especially the deepening of the clinical application of emergency endoscopy, not only the diagnosis of esophagogastric fundal varices caused by portal hypertension and emergency treatment of variceal rupture and bleeding have achieved remarkable efficacy, but also due to the continuous development of endoscopic treatment technology, it can be effective in preventing bleeding. Commonly used methods include sclerotherapy, ligation therapy, tissue adhesive embolization therapy and metal clip hemostasis therapy.
4. Interventional therapy
One is to embolize part of the splenic artery through interventional methods to reduce the splenic vein blood flow, lower the portal vein pressure and control the symptoms of hypersplenism to a certain extent. Secondly, transjugular intrahepatic intraportal vena cava stent shunt (transjugular route intrahepatic stent portosystemic shunt, TIPS) is a new interventional radiology therapy for the treatment of portal hypertension and upper gastrointestinal bleeding. It utilizes the principle of surgical shunt, through the use of a series of interventional instruments, to establish an artificial shunt channel between the hepatic vein and portal vein in the liver parenchyma, thus reducing the portal pressure, reducing or eliminating the symptoms of esophageal varices rupture bleeding and ascites due to portal hypertension.
5.Surgery
The most commonly used and classic surgical methods for treating portal hypertension are shunt surgery and interruption of flow. According to the timing of surgery, it can be categorized into preventive surgery for those who have no history of gastrointestinal bleeding, emergency surgery in case of hemorrhage, and elective surgery to prevent rebleeding after bleeding stops. Emergency surgery is required when acute upper gastrointestinal hemorrhage fails to be treated with non-surgical conservative treatment. Because persistent bleeding can lead to severe liver dysfunction, coupled with surgical trauma, the mortality rate of Child?C?grade patients undergoing emergency conventional surgery is as high as 40% to 70%, and the best option is to perform emergency liver transplantation; if there is no condition for liver transplantation, emergency shunt surgery is preferred because shunt surgery will further damage liver function and the surgical mortality rate is even higher, so it should be performed with caution.