Portal vein cavernous transformation

　　Portal vein cavernous transformation can be divided into primary and secondary according to the etiology. Among children, the causes of onset are:

　　1. Congenital malformation of the portal vein, where there is abnormal hyperplasia of the venous plexus between the umbilical mesentery and the hepatic vein after the occlusion of the venous duct, to replace the occluded portal vein.

　　2. Portal vein cavernous transformation itself is a type of vascular tumor of the portal vein.

　　3. The outcome of portal vein thrombosis, neonatal sepsis, umbilical infection, and abdominal infection, as well as inflammatory lesions involving the portal vein system, ultimately lead to portal vein occlusion and the formation of collateral veins around the portal vein.

　　Adult portal vein cavernous transformation is mostly secondary, characterized by the obstruction of portal vein blood flow, blood stasis, or increased blood flow, and increased pressure due to the original normal portal vein system lumen structure being damaged by portal vein inflammation, periportal fibrous tissue inflammation, thrombosis, coagulation disorders (polycythemia), tumor invasion, pancreatitis, etc. To alleviate the pressure, collateral circulation is established around the portal vein. The portal vein becomes widened and presents with a solid change, with small tortuous vessels seen around the portal vein.

　　It is for portal hypertension and secondary esophageal and gastric fundus varices rupture and (or) accompanied by portal hypertension-related gastric disease. Occasionally, the cavernous transformation collateral vessels can compress the common bile duct, causing obstructive jaundice. The amount of bleeding is large, and more than 80% of children have the first esophageal and gastric fundus varices rupture and bleeding between the ages of 1 to 6. In addition, there are splenomegaly, splenic hyperfunction, jaundice (secondary to bleeding, infection, traffic), venous compression of the common bile duct, ischemic liver cell injury, etc.

　　When there is no portal hypertension, patients with primary CTPV (portal vein cavernous transformation) may have no discomfort, while patients with secondary CTPV mainly show the manifestations of the primary disease. After the formation of portal hypertension, the main manifestations are portal hypertension and secondary esophageal and gastric fundus varices rupture and (or) accompanied by portal hypertension-related gastric disease. Patients may repeatedly vomit blood and black stools, accompanied by mild to moderate splenomegaly, splenic hyperfunction. Therefore, for such patients, the liver function is good, so it is rare to have ascites, jaundice, and hepatic encephalopathy. Occasionally, the cavernous transformation collateral vessels can compress the common bile duct, causing obstructive jaundice.

　　Adults should have a normal sleep time of 8 hours, which should start around 11 pm. From 1 to 3 am in the morning, it is the time to enter deep sleep, which is the best time to nourish the liver blood. Conversely, the blood will not be nourished enough. Therefore, we appeal to everyone to try not to stay up late. If it is inevitable to become a member of the late-night group, one should take in more adequate nutrition to protect oneself and minimize the harm to the body caused by staying up late. Vitamin A, an essential element for protecting the liver, can prevent liver cancer. The liver is the 'warehouse' of vitamin storage in the human body. When the liver is damaged, the ability of the 'warehouse' to store vitamins also decreases. Studies have shown that vitamin A can protect the liver, prevent and inhibit the proliferation of cancer cells in the liver, and can restore the function of normal tissues.

　　The examination items and content of portal vein cavernous transformation (CTPV) are divided into the following 3 points:
　　1. Abdominal ultrasound: The normal portal vein structure disappears, replaced by irregularly curved vascular shadows, or honeycomb-like, with blood flow visible inside, and the direction of blood flow is irregular; the vascular wall thickens and the echo is enhanced, and thrombi can be seen inside the blood vessels. Ueno classified CTPV into 3 types based on the color Doppler imaging: Type I shows unclear normal portal vein structure, only displaying a honeycomb-like structure in the portal vein area, and primary CTPV belongs to this type; Type II shows that the main portal vein can be displayed, but it is filled with emboli inside, and collateral veins are visible around it; Type III shows that there is a mass echo near the portal vein, and the portal vein is compressed to form collateral veins, and Types II and III are manifestations of secondary CTPV.
　　2. Abdominal CT: The blood flow direction is irregular, and thrombi can be seen in the blood vessels.
　　(1) Disordered structure in the portal vein course area, the normal portal venous system structure disappears, and a soft tissue network-like structure similar to a mass is visible along the portal vein course, which is unclear between each other. After enhancement scanning, the portal vein is obviously strengthened and intertwined into a network, sinusoid-like or tubular soft tissue structures, and there are fine linear density increased shadows extending towards the portal vein around the hilum of the liver.
　　(2) Abnormal liver parenchymal perfusion, in the arterial phase, contrast medium accumulates around the liver parenchyma, forming a high-density band shadow, and sometimes it can also be seen that the adjacent artery is expanded, while in the portal vein phase, the whole liver shows a uniform isodensity shadow.
　　(3) In patients with portal hypertension, collateral circulation vessels that are tortuous and expanded in the shape of crawling can be seen in the coronary vein, umbilical vein, retroperitoneal cavity, hepato-gastro-duodenal ligament, and fundus-esophageal junction area, and in severe cases, they are tortuous into a mass-like shape. Enhancement scanning shows obvious enhancement in the portal vein phase.
　　3. Digital subtraction angiography (DSA): It is mainly manifested as unclear display of normal portal vein structure in the portal vein course area, normal portal vein is replaced by a sponge-like vascular expansion that is out of proportion and tortuous, showing a parallel venous network with tortuous expansion, splenic vein expansion, coronary vein and esophageal vein tortuous expansion.

　　It is advisable to eat celery, chrysanthemum, leek, winter melon, black plum, persimmon cake, sesame, lotus seeds, and sea cucumber. The patient's diet should be light and easy to digest, eat more vegetables and fruits, rationally match the diet, and pay attention to adequate nutrition. In addition, patients should also pay attention to avoiding spicy, greasy, and cold foods.

　　The treatment of portal cavernoma mainly aims at portal hypertension, and secondary esophageal and gastric fundus varices rupture bleeding and portal hypertension-induced gastric disease. Surgical treatment is the main method, and drug therapy only plays an auxiliary role.
　　One, drug therapy
　　Application of drugs to reduce portal vein pressure, so that the resistance of the portal venous system and its collateral circulation is reduced, visceral vessels are contracted, the blood flow and pressure of the portal vein and its collaterals are reduced, so that the blood flow at the bleeding site is reduced, achieving hemostasis, with a hemostasis rate of about 60%. Commonly used drugs include posterior pituitary extract, 0.4µ. g/min continuous intravenous infusion. 14-mer somatostatin, the initial dose is 250µ. g intravenous bolus injection, followed by 250µ. g/h continuous intravenous infusion. 8-mer peptide analog (octreotide), the initial dose is 100µ. g intravenous bolus injection, followed by 250µ. g/h continuous intravenous infusion.
　　Two, interventional radiological therapy
　　Selective abdominal arteriography, after determining the bleeding site and cause, percutaneous catheter drug infusion or embolization therapy can effectively control bleeding.
　　Three, endoscopic treatment
　　Endoscopic injection of sclerosing agents or ligation of esophageal varices, and if necessary, injection of tissue adhesive embolization into the fundus varices to achieve hemostasis. This method may cause esophageal perforation, stricture, and occasionally thrombosis of other veins (such as splenic vein, superior mesenteric vein, etc.).
　　4. Surgical Treatment
　　Patients with good liver function and splenic hyperfunction are suitable for surgical treatment.
　　1. Shunt Operation; Including mesenteric superior vein-inferior vena cava shunt operation, splenic vein-left renal vein shunt operation, and distal splenic renal vein shunt operation. Although shunt operation can reduce portal vein pressure and control gastrointestinal bleeding, excessive shunting of portal vein blood flow not only causes a decrease in inflow to the liver but also may cause the occurrence of hepatic encephalopathy.
　　2. Shunt Operation: Including mesenteric superior vein-inferior vena cava shunt operation, splenic vein-left renal vein shunt operation, and distal splenic renal vein shunt operation. Although shunt operation can reduce portal vein pressure and control gastrointestinal bleeding, excessive shunting of portal vein blood flow not only causes a decrease in inflow to the liver but also may cause the occurrence of hepatic encephalopathy.
　　3. Shunt and Shunt: Currently, most of the combined shunt and shunt operations are used. By relieving splenic hyperfunction and reducing portal vein pressure, the purpose of acute hemostasis and prevention of recurrent hemorrhage in the long term is achieved.
　　4. Splenectomy: For splenic enlargement and hyperfunction.
　　5. Others: Such as balloon occlusion shunt operation in the portal vein to control acute hemorrhage, intraoperative placement of an abdominal catheter in the splenic vein, and postoperative placement of a balloon expansion catheter through the splenic vein catheter under radiological intervention to expand the main portal vein, thus relieving portal vein obstruction and effectively reducing portal vein hypertension.
　　6. Combined Treatment: Clinical findings show that each technique has its drawbacks. The rebleeding rate of simple splenectomy can reach as high as 90%, and it can cause life-threatening post-splenectomy sepsis, which should be avoided as much as possible. Splenectomy combined with shunt operation will further increase portal vein pressure. Although it can stop bleeding immediately in acute hemorrhage, it is inevitable that new collateral circulation will be established over time, making rebleeding difficult to avoid. There are reports that portosystemic shunt operation combined with portocaval shunt operation is the best choice for the treatment of this disease. In particular, splenectomy + gastroesophageal variceal ligation + esophagogastric fundus resection and anastomosis (Phemister surgery) may achieve a good long-term hemostatic effect.