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Portal hypertension: syndrome, signs, treatment

What it is? Portal hypertension is an increase in pressure inside the portal (portal) vein, due to the occurrence of an obstacle to the normal flow of blood through it.

This condition is observed in some diseases of the vessels and internal organs, the outflow from which is included in the portal system. These are unpaired abdominal organs - esophagus, liver, intestines, spleen, etc.

Portal hypertension syndrome is a condition of the body in which a number of specific clinical and morphological manifestations develop in response to the formation of portal hypertension.

For a better understanding of what portal hypertension is, its manifestations and treatment methods, let's consider the development of the disease. For this, it is necessary to plunge a little into the anatomy and the processes occurring in the body ..

Portal hypertension

The liver is an organ with an unusual, double blood loss. Both arterial and venous blood flow in it, which are mixed in the thickness of this organ. Such a system is necessary for the liver to perform its complex and diverse functions.

Venous blood from the intestine, spleen and stomach enters the liver through the portal vein. In the liver, this vein branches into smaller vessels that form a dense network, the so-called sinusoidal capillary network.

Branches of the hepatic artery, which carries arterial blood from the aorta to the liver, flow into it (oxygen and nutrients are supplied to this artery). In sinusoids, arterial and venous blood are mixed, which ensures the active work of liver cells (hepatocytes).

In the future, blood from sinusoids is collected in the hepatic veins, and they, in turn, carry blood to the inferior vena cava. And she already goes to the heart. This closes the circulation with the portal (portal) vein (see photo 2).

The portal vein has a connection with the system of hollow veins not only through the liver, but also in the form of communicating vessels - anastomoses, which play an important clinical significance. Violation of the patency of the portal vein causes blood to look for other outflow pathways. And they are - this is an anastomosis.

Through them, the blood enters the hollow veins on the principle of communicating vessels. When this occurs, specific manifestations that form the picture of this state.

There are 4 types of portal hypertension. It depends on the level of the obstacle:

  1. Pre-hepatic, or pre-hepatic, form. It appears when an obstacle is formed in the vessels before they enter the liver;
  2. Intrahepatic - develops when an obstacle is formed inside the liver. This is the most common option, which has a frequency of 80-90%;
  3. Suprahepatic - in this form, the blockage of blood flow takes place at the level of the vessels that leave the liver, that is, in the hepatic veins;
  4. Mixed form. Represents a combination of intrahepatic variant with suprahepatic or prehepatic forms.

Content

Causes of portal hypertension, the effect of cirrhosis

Causes of portal hypertension

The causes of portal hypertension depend on the variant of this syndrome.

1. A pre-hepatic form may develop as a result of the following causal factors:

  • Congenital disorders of the portal vein structure: cavernous transformation, aplasia, hypoplasia, portal vein atresia.
  • Blood flow closure thrombus - portal vein thrombosis. It is observed in inflammatory diseases such as acute appendicitis with disintegration (destructive), pancreatitis, purulent cholangitis, acute cholecystitis, and also as a manifestation of thromboembolism.
  • Compression of the portal vein by a large tumor or cyst of the pancreas, a parasitic cyst for alveococcosis. Also in these cases, secondary thrombosis of both the portal and splenic veins is possible, which additionally forms portal hypertension.

2. The intrahepatic form is caused by changes in the structure of the liver tissue, due to diseases that disrupt the normal structure of hepatocytes.

Even the early stages are accompanied by portal hypertension in liver cirrhosis. This is the most common cause of the development of an outflow disturbance in the portal system. With this disease, regenerate nodes are formed. They represent connective tissue structures that squeeze the liver cells from the outside.

  • Parasites in the liver - echinococcosis , schistosomiasis;
  • Polycystic, liver tumors, metastasis of tumors;
  • Liver fibrosis;
  • Toxic hepatitis, which occurs when taking a large amount of vitamin A, acute hepatitis;
  • Fatty degeneration of the liver - the accumulation of fat droplets in hepatocytes, leading to cell stretching and compression of their structures;
  • Bone marrow diseases;
  • Sarcoidosis , tuberculosis.

3. The suprahepatic form is the rarest. It occurs during the formation of an obstruction to blood flow in the vessels that leave the liver - the hepatic veins. This form happens under the following conditions:

  • Chiari disease - inflammation of the inner lining of the hepatic veins, which leads to the formation of blood clots, blocking the blood flow;
  • Badda Chiari Syndrome. In this condition, the inferior vena cava overlaps from the inside, due to the formation of connective tissue in the lumen of the vein, or when it is crushed by a tumor, cyst, scars on the outside;
  • Heart diseases: constrictive pericarditis, tricuspid valve insufficiency, right ventricular insufficiency, etc.

4. Mixed form may occur with a combination of diseases. In this form, the worst prognosis, since the possibility of surgical treatment is severely limited. The main causative factors are:

  • The emergence of portal thrombosis of the portal vein in combination with cirrhosis of the liver, which leads to the simultaneous or sequential formation of prehepatic and intrahepatic forms.
  • Secondary cirrhosis and suprahepatic portal hypertension first create increased pressure in the hepatic veins, blood stagnation in the liver, the development of changes in its structure, followed by the formation of intrahepatic portal hypertension.

5. Portal hypertension without obstruction to the bloodstream develops when a fistulous transition is formed between the artery and the vein, through which an increased discharge of blood into the portal vein occurs.

Most often, the fistula is formed between the splenic artery and the splenic vein.

Signs of portal hypertension, photo

Signs of portal hypertension, photo

Photo of characteristic signs "Head of a jellyfish"

Anastomoses between the portal and the hollow veins, through which blood is released when a block occurs in the portal system, are located in 3 zones:

  1. The region of the lower esophagus and the upper part of the stomach;
  2. The lower part of the rectum;
  3. Anterior abdominal wall.

Therefore, portal hypertension has symptoms consisting in changing vessels in these areas:

  • Expansion of the veins of the esophagus;
  • Hemorrhoids - varicose veins of the rectum;
  • "The head of the jellyfish" - the expansion of the veins of the anterior abdominal wall;
  • Enlarged spleen as a result of increased blood filling - splenomegaly, and as a result of the growth of its tissue - hypersplenism. The latter state is accompanied by activation of the splenic functions of destruction of blood cells, so it is often accompanied by anemia and a decrease in the number of peripheral platelets;
  • The accumulation of fluid in the abdominal cavity - ascites.

Also, when the intrahepatic block occurs, the discharge of a part of blood from the portal vein bypassing the liver - into the hepatic veins occurs.

The blood, not getting into the liver, does not undergo purification, all harmful metabolic products enter the brain, and encephalopathy and other manifestations of the intoxication syndrome develop.

Syndrome of portal hypertension will manifest a complex of symptoms. Thus, in general condition, there is weakness, malaise, fatigue.

Dyspeptic complaints appear - nausea, vomiting, discomfort, pain in the stomach and liver. In patients, appetite is sharply reduced, up to and including refusal from food - anorexia. The consequence of this will be a significant weight loss due to the loss of fat and muscle.

Portal Hypertension Syndrome

Portal hypertension syndrome photo

Itching is noted on the skin (due to the large flow of bile acids into the bloodstream, which irritate sensitive nerve endings). The skin becomes jaundiced, there may be sudden bruises, and when the vessels rupture, spontaneous bleeding occurs from the mouth or from the perianal area.

There are swelling in the legs , and with the appearance of ascites, the abdomen increases greatly in volume. The cause of these signs is a decrease in the protein-synthesizing function of the liver. As a result, the fluid easily goes into the interstitial space (oncotic pressure of the blood decreases).

  • Urine is getting dark. This is due to the entry of bilirubin into it, which turns into urobilin.
  • With the development of hepatic encephalopathy, sleep is disturbed, the correct rhythm of sleep and wakefulness is lost, memory and mental activity deteriorates. Behavioral disturbances and personality changes can occur, including suicide attempts.
  • Sexual dysfunction and impotence develop, which is promoted by a violation of the metabolism of sex hormones in the liver. There may be muscle cramps, muscle wasting, the development of Dupuytren's contracture - "twisting" of the fingers.

Manifestations of portal hypertension in children will be minimal and, in general, the course of this syndrome is more favorable.

This is due to the fact that the pressure in the portal vein increases slightly, the blood goes around obstacles along physiological bypass routes (still unclosed in children), there are no marked disturbances in liver function changes, ascites is rare.

However, in the absence of treatment, the disease begins to progress, despite the large compensatory abilities of the child’s body. Requires rapid establishment of the cause and its elimination.

Diagnosis of portal hypertension

The first stage consists of a survey and examination, identifying signs of this syndrome, liver disease and other diseases. Laboratory research is to conduct a general and biochemical analysis of blood and urine. Its results change as follows:

  • Portal hypertension in liver cirrhosis reveals a decrease in the number of platelets, at the late stage - anemia and a decrease in other blood cells.
  • With hypersplenism, a decrease in all cellular elements will be detected - pancytopenia.
  • In hemochromatosis, an increase in hemoglobin in combination with a low color index.
  • With alcoholic cirrhosis, a significant increase in the enzymes AsAT, AlAT, GGTP (gamma-glutamyl transpeptidase) is detected.
  • Primary biliary with cirrhosis is manifested by an increase in bilirubin, a decrease in albumin level, a significant increase in alkaline phosphatase, in the last stage there is a decrease in AST and ALT, which indicates the destruction of hepatocytes.
  • A decrease in the prothrombin index, if the index is below 60%, the prognosis is unfavorable.
  • Blood levels of albumin, creatinine, electrolytes, urea are determined. The results obtained are compared with special scales for assessing the stage of the disease.
  • In urine analysis: red blood cells, white blood cells, protein, uric acid, creatinine. With ascites and edema, it is necessary to determine the amount of daily urine.

The instrumental study consists of an ultrasound scan, which is very informative with this syndrome. It can be used as a screening method. When ultrasound is detected:

  • changes in the volume of blood vessels;
  • presence of bypass vessels;
  • ascites;
  • changes in the size and internal structure of the liver and spleen;
  • determining the presence of blood clots and blood flow velocity.

Can also be carried out: CT scan, MRI, radioisotope scanning.

A method that allows you to see the true picture of blood flow disorders is angiography - an x-ray examination of the vessels with the introduction of a contrast agent.

To inspect the veins of the esophagus spend FGDs. To clarify the causes of portal hypertension, a liver biopsy is performed with subsequent microscopic examination of tissues, as well as laparoscopy.

Treatment of portal hypertension

Treatment of portal hypertension

The complex of therapeutic measures consists in the treatment of the disease that caused the obstruction, as well as in the elimination of increased intraportal pressure. With portal hypertension, the treatment is divided into conservative and surgical.

With the conservative treatment of portal hypertension, drugs are used to reduce the pressure in the portal system:

  1. Vasopressin reduces arterioles, which reduces blood flow to the intestines and reduces pressure in the portal vein. Before its introduction it is necessary to remove the ECG, since it has a narrowing effect on the heart vessels.
  2. Somatostatin - increases the resistance of arteries, affecting smooth muscles, which leads to a decrease in pressure in the portal system. The synthetic analogue of this hormone is octreotide.
  3. Beta-blockers non-selective: propranolol, nadolol, timolol. In 30% of patients are ineffective. In addition, they have a side effect in the form of impotence.
  4. Nitrates: isosorbide-5-mononitrate and others. They are an effective dilator of veins. This group is used in combination with vasopressin.

Surgical treatment of portal hypertension consists in the artificial formation of workarounds to drain blood from the portal vein. With intrahepatic obstruction, surgical treatment is carried out only after the main process subsides in the liver, in the absence of manifestations of liver failure.

The following operations are performed: portosystemic shunting, embolization of the splenic artery, omentinerenopexy (hemming of the greater omentum to the liver and kidney, for the development of new vessels, bypassing the portal vein).

Removal of the spleen reduces the pressure in the portal system, but as an independent operation, it is used only for hypersplenism.

In the treatment of portal hypertension in liver cirrhosis, donor organ transplantation gives a good effect, with the development of irreversible changes in the liver in cirrhosis.

Complications

Complications of portal hypertension are serious enough. They are represented by the following list:

  • Bleeding from the dilated and modified veins of the esophagus. Such bleeding with portal hypertension is the most frequent and dangerous complication, leading to death in 50% of cases;
  • The development of secondary cirrhosis of the liver in subhepatic form of portal hypertension;
  • Hepatic failure - liver failure;
  • Spontaneous bacterial inflammation of the peritoneum;
  • External and internal hernia due to ascites.
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