Portal hypertension - Symptoms

Symptoms and instrumental data in portal hypertension

1. The earliest symptoms of portal hypertension are: flatulence (“wind before rain”), a feeling of fullness in the intestines, nausea, pain throughout the abdomen, and loss of appetite.
2. As a rule, there are “symptoms of poor nutrition” (poorly defined subcutaneous tissue, dry skin, muscle atrophy).
3. Dilation of the veins of the abdominal wall, visible through the skin in the navel area and in the lateral parts of the abdomen. With pronounced blood circulation through the paraumbilical veins or the umbilical vein itself, a venous plexus (the "capita medusa") is formed around the navel.
4. As portal hypertension progresses, ascites develops (it is most typical for intrahepatic portal hypertension), in addition, swelling of the legs, bleeding from varicose veins of the esophagus and stomach, rectum, and nosebleeds are possible.
5. Splenomegaly of varying severity (depending on the nature of the disease that caused the development of portal hypertension). It is often accompanied by the development of hypersplenism (pancytopenia syndrome: anemia, leukopenia, thrombocytopenia).
6. Hepatomegaly of varying severity (depending on the cause that led to the development of portal hypertension). The liver is dense, sometimes painful, its edge is sharp. In liver cirrhosis, it is lumpy, regeneration nodes are palpable (these cases must be differentiated from liver cancer).
7. With long-standing and severe portal hypertension, portal encephalopathy develops, which is manifested by headaches, dizziness, memory loss, sleep disturbances (insomnia at night, drowsiness during the day); with severe encephalopathy, hallucinations, delirium, inappropriate behavior of patients, and neurological signs of damage to the central nervous system appear.

Various forms of portal hypertension (depending on the location of the block) have their own clinical characteristics.

The suprahepatic form of portal hypertension is characterized by:

• early development of ascites that is not amenable to diuretic therapy;
• significant hepatomegaly with a relatively small enlargement of the spleen;
• severe pain in the liver area.

The subhepatic form of portal hypertension has the following features:

• main symptoms are splenomegaly, hypersplenism;
• the liver is usually not enlarged;
• Subhepatic portal hypertension usually develops slowly, and subsequently multiple esophageal-gastric bleeding is observed.

The intrahepatic form of portal hypertension has the following distinctive clinical features:

• early symptoms are persistent dyspeptic syndrome, flatulence, periodic diarrhea, weight loss;
• late symptoms: significant splenomegaly, varicose veins with possible bleeding, ascites, hypersplenism;
• with the predominance of the hepatosplenic type of intrahepatic hypertension, pain is localized in the epigastrium and especially in the left hypochondrium; laparoscopy reveals congestive veins in the greater curvature of the stomach and spleen; with FEGDS, varicose veins are detected even in the high sections of the esophagus;
• with prevalence of intestinal-mesenteric type of intrahepatic portal hypertension, pain is localized around the navel, in the iliac regions or in the liver area; laparoscopy reveals congestive veins mainly in the area of the diaphragm, liver, round ligament, intestines. During esophagoscopy, varicose veins in the esophagus are not clearly expressed.

Clinical features of the main etiological forms of portal hypertension

Increased portal venous blood flow

1. Arteriovenous fistulas.

Arteriovenous fistulas can be congenital or acquired. Congenital fistulas are observed in hereditary hemorrhagic telangiectasia.

Acquired arteriovenous aneurysms are formed as a result of trauma, liver biopsy, rupture of an aneurysm of the hepatic or splenic artery. Sometimes arteriovenous aneurysms accompany hepatocellular carcinoma.

In arteriovenous fistulas, there is a connection between the hepatic artery and the portal vein or the splenic artery and the splenic vein. The presence of fistulas causes increased blood flow to the portal system. Clinically, the patient has symptoms of portal hypertension. 1/3 of patients have abdominal pain. The main method for diagnosing arteriovenous fistulas is angiography.

2. Splenomegaly not associated with liver disease.

In this case, portal hypertension is caused by myeloproliferative diseases, primarily myelofibrosis (subleukemic myelosis).

The main diagnostic criteria for myelofibrosis are:

• pronounced splenomegaly and, less commonly, hepatomegaly;
• leukocytosis with a neutrophilic shift, often with a pronounced rejuvenation of the formula (the appearance of myeloblasts, myelocytes);
• anemia;
• hyperthrombocytosis (platelets are functionally defective);
• three-line metaplasia of hematopoiesis in the spleen and liver (extramedullary hematopoiesis);
• pronounced bone marrow fibrosis in a trephine biopsy from the ilium;
• narrowing of the medullary canal, thickening of the cortical layer on radiographs of the pelvic bones, vertebrae, ribs, and long tubular bones.

3. Cavernomatosis of the portal vein.

The etiology and pathogenesis of this disease are unknown. Many specialists consider it congenital, others - acquired (early thrombosis of the portal vein with its subsequent recanalization). In this disease, the portal vein is a cavernous angioma or a network of numerous small-caliber vessels. The disease manifests itself in childhood with portal hypertension syndrome or portal vein thrombosis, complicated by bleeding from varicose veins of the esophagus and stomach, intestinal infarction, and hepatic coma. The prognosis is unfavorable, life expectancy from the moment of appearance of clinical signs of portal hypertension is 3-9 years. The main method for diagnosing portal vein cavernomatosis is angiography.

4. Thrombosis or occlusion of the portal or splenic veins

Depending on the localization of portal vein thrombosis (pylethrombosis), a distinction is made between radicular thrombosis, in which the splenic vein or (less commonly) other veins flowing into the common trunk are affected; trunk thrombosis - with blockage of the portal vein in the area between the entry of the splenic vein and the portal vein, and terminal thrombosis - with localization of thrombi in the branches of the portal vein inside the liver.

Isolated splenic vein obstruction causes left-sided portal hypertension. It may be caused by any of the factors that cause portal vein obstruction. Of particular importance are pancreatic diseases, such as cancer (18%), pancreatitis (65%), pseudocysts, and pancreatectomy.

If the obstruction develops distal to the place of entry of the left gastric vein, blood through collaterals, bypassing the splenic vein, enters the short gastric veins and then into the fundus of the stomach and the lower part of the esophagus, flowing from there into the left gastric and portal veins. This leads to very significant varicose veins of the fundus of the stomach; the veins of the lower part of the esophagus are slightly dilated.

The main causes of pylethrombosis:

• liver cirrhosis (in which blood flow in the portal vein slows down); liver cirrhosis is found in 25% of patients with pylethrombosis;
• increased blood clotting (polycythemia, myelothrombosis, thrombotic thrombocythemia, post-splenectomy condition, taking oral contraceptives, etc.);
• (pressure of the portal vein from outside (tumors, cysts, lymph nodes);
• phlebosclerosis (as a consequence of inflammation of the vein wall in congenital portal stenosis);
• inflammatory process in the portal vein (pylephlebitis), sometimes due to the transition of inflammation from the intrahepatic bile ducts (cholangitis), pancreas, during sepsis (especially often with umbilical sepsis in children);
• damage to the wall of the portal vein due to trauma (in particular, during operations on abdominal organs);
• primary liver cancer (paraneoplastic process), cancer of the head of the pancreas (compression of the portal vein);
• In 13-61% of all cases of portal vein thrombosis, the cause is unknown (idiopathic portal vein thrombosis).

The main symptoms of acute pylethrombosis:

• more often observed in polycythemia, liver cirrhosis, after splenectomy
• severe abdominal pain;
• bloody vomiting;
• collapse;
• ascites develops rapidly (sometimes hemorrhagic);
• the liver is not enlarged; if acute portal vein thrombosis occurs in a patient with liver cirrhosis, then there is hepatomegaly;
• no jaundice;
• leukocytosis with a neutrophilic shift in the blood;
• when thrombosis of the mesenteric arteries occurs, intestinal infarctions occur with a picture of acute abdomen;
• With thrombosis of the splenic vein, pain appears in the left hypochondrium and an enlarged spleen.

The outcome is most often fatal.

Acute pylethrombosis should be suspected if portal hypertension suddenly appears after abdominal trauma, liver surgery, or portal system surgery.

The prognosis for acute pylethrombosis is poor. Complete occlusion of the portal vein leads to death within a few days from gastrointestinal bleeding, intestinal infarction, and acute hepatocellular failure.

Chronic pylethrombosis lasts for a long time - from several months to several years. The following clinical manifestations are characteristic.

• signs of the underlying disease;
• pain of varying intensity in the right hypochondrium, epigastrium, spleen;
• splenomegaly;
• liver enlargement is not typical, except in cases of pylethrombosis against the background of liver cirrhosis;
• gastrointestinal bleeding (sometimes this is the first symptom of pylethrombosis);
• ascites (in some patients);
• Portal vein thrombosis against the background of liver cirrhosis is characterized by the sudden development of ascites, other signs of portal hypertension and a sharp deterioration in liver function.

The diagnosis of pylethrombosis is confirmed by splenoportography data, less often by ultrasound.

A feature of the diagnosis of idiopathic portal vein thrombosis: laparoscopy reveals an unchanged liver with well-developed collaterals, ascites, and an enlarged spleen.

The prognosis for pylethrombosis is unfavorable. Patients die from gastrointestinal bleeding, intestinal infarctions, and liver and kidney failure.

Acute thrombophlebitis of the portal vein (pylephlebitis) is a purulent inflammatory process in the area of the entire portal vein or its individual sections. As a rule, pylephlebitis is a complication of inflammatory diseases of the abdominal cavity organs (appendicitis, nonspecific ulcerative colitis, penetrating ulcer of the stomach or duodenum, destructive cholecystitis, cholangitis, intestinal tuberculosis, etc.) or the small pelvis (endometritis, etc.).

Main clinical manifestations:

• a sharp deterioration in the patient's condition against the background of the underlying disease;
• fever with severe chills and profuse sweating, body temperature reaches 40°C;
• intense cramping pain in the abdomen, often in the upper right sections;
• as a rule, vomiting and often diarrhea are observed;
• the liver is enlarged and painful;
• 50% of patients have splenomegaly;
• moderate jaundice;
• laboratory data - complete blood count: leukocytosis with a left shift in the white blood cell count; increased ESR; biochemical blood test: hyperbilirubinemia, increased aminotransferase activity, increased levels of gamma globulins, fibrinogen, seromucoid, haptoglobin, sialic acids;
• When cannulating the umbilical vein, pus is found in the portal system.

4. Liver diseases

Diagnosis of liver diseases listed above as causes of portal hypertension is made on the basis of the corresponding symptoms.

• Cirrhosis

All forms of liver cirrhosis lead to portal hypertension; it begins with obstruction of the portal bed. Blood from the portal vein is redistributed into collateral vessels, some of it is directed bypassing hepatocytes and enters directly into small hepatic veins in fibrous septa. These anastomoses between the portal and hepatic veins develop from sinusoids located inside the septa. The hepatic vein inside the fibrous septum shifts further and further outward until a communication with a branch of the portal vein occurs through a sinusoid. Blood supply to the regeneration nodes from the portal vein is disrupted, and blood enters them from the hepatic artery. Larger intervenous anastomoses are also found in the liver with cirrhosis. In this case, about a third of all the blood entering the liver passes through these shunts bypassing the sinusoids, i.e. bypassing the functioning liver tissue.

Part of the portal blood flow is caused by the regenerative nodes compressing the portal vein branches. This should lead to postsinusoidal portal hypertension. However, in cirrhosis, the hepatic vein wedge pressure (sinusoidal) and the pressure in the main trunk of the portal vein are practically the same, and stasis extends to the portal vein branches. The sinusoids apparently provide the main resistance to blood flow. Due to changes in the Disse space caused by its collagenization, the sinusoids narrow; this can be especially pronounced in alcoholic liver disease, in which the blood flow in the sinusoids can also decrease due to swelling of the hepatocytes. Consequently, obstruction develops along the entire length from the portal zones through the sinusoids to the hepatic veins.

The hepatic artery supplies the liver with a small amount of blood under high pressure, and the portal vein with a large amount of blood under low pressure. The pressure in these two systems is equalized in the sinusoids. Normally, the hepatic artery probably plays a minor role in maintaining portal pressure. In cirrhosis, the connection between these vascular systems becomes closer due to arterioportal shunts. Compensatory dilation of the hepatic artery and increased blood flow through it help maintain sinusoid perfusion.

• Other liver diseases that involve the formation of nodes

Portal hypertension can result from various non-cirrhotic diseases that involve the formation of nodules in the liver. They are difficult to diagnose and are usually confused with cirrhosis or "idiopathic" portal hypertension. A "normal" picture on liver biopsy does not exclude this diagnosis.

Nodular regenerative hyperplasia. Monoacinar nodules of cells similar to normal hepatocytes are diffusely determined throughout the liver. The appearance of these nodules is not accompanied by proliferation of connective tissue. The cause of their development is obliteration of small (less than 0.5 mm) branches of the portal vein at the level of the acini. Obliteration leads to atrophy of the affected acini, while neighboring acini, whose blood supply is not impaired, undergo compensatory hyperplasia, causing nodular degeneration of the liver. Portal hypertension is expressed to a significant degree, sometimes hemorrhages in the nodules are observed.

In case of hemorrhage, ultrasound reveals hypo- and isoechoic formations with an anechoic central part. In CT, the tissue density is reduced, and it does not increase with contrast.

Liver biopsy reveals two populations of hepatocytes that differ in size. The biopsy has no diagnostic value.

Most often, nodular regenerative hyperplasia develops in rheumatoid arthritis and Felty's syndrome. In addition, nodules are formed in myeloproliferative syndromes, syndromes of increased blood viscosity and as a reaction to drugs, especially anabolic steroids and cytostatics.

Portacaval shunting for bleeding esophageal varices is usually well tolerated.

Partial nodular transformation is a very rare disease. Nodes are formed in the area of the liver gate. The liver tissue on the periphery has a normal structure or is atrophic. The nodes interfere with normal blood flow in the liver, resulting in portal hypertension. The function of hepatocytes is not impaired. Fibrosis is usually absent. Diagnosis of the disease is difficult, and often only an autopsy can confirm the diagnosis. The cause of the disease is unknown.

5. Action of toxic substances

The toxic substance is captured by endothelial cells, mainly lipocytes (Ito cells) in the Disse space; they have fibrogenic properties and cause obstruction of small branches of the portal vein and the development of intrahepatic portal hypertension.

Portal hypertension is caused by inorganic arsenic preparations used to treat psoriasis.

Liver damage in workers spraying vineyards in Portugal may be due to exposure to copper. The disease may be complicated by the development of angiosarcoma.

When inhaling vapors of polymerized vinyl chloride, sclerosis of the portal venules develops with the development of portal hypertension, as well as angiosarcoma.

Reversible portal hypertension may occur with vitamin A intoxication - vitamin A accumulates in Ito cells. Long-term use of cytostatics, such as methotrexate, 6-mercaptopurine and azathioprine, may lead to presinusoidal fibrosis and portal hypertension.

Idiopathic portal hypertension (non-cirrhotic portal fibrosis) is a disease of unknown etiology, manifested by portal hypertension and splenomegaly without obstruction of the portal veins, changes in the extrahepatic vascular network and severe liver damage.

This syndrome was first described by Banti in 1882. The pathogenesis of portal hypertension is unknown. Splenomegaly in this disease is not primary, as Banti suggested, but is a consequence of portal hypertension. Microthrombi and sclerosis are observed in the intrahepatic portal venules.

Main clinical manifestations and instrumental data:

• splenomegaly;
• ascites;
• gastric bleeding;
• liver function tests are normal or slightly changed, liver failure develops at a late stage;
• liver biopsies reveal periportal fibrosis, possibly with no histological changes (however, portal tracts must be detected);
• absence of signs of portal or splenic thrombosis according to angiography data;
• normal or slightly elevated hepatic venous wedge pressure, high portal vein pressure based on portal vein catheterization or puncture.

Idiopathic portal hypertension can develop in patients with systemic scleroderma, autoimmune hemolytic anemia, Hashimoto's goiter, and chronic nephritis.

The prognosis for idiopathic portal hypertension is considered relatively good, with 50% of patients living 25 years or more from the onset of the disease.

Nodular regenerative hyperplasia of the liver - liver changes (diffuse or focal) of unknown etiology, characterized by the appearance of nodules consisting of proliferating hypertrophied hepatocytes not surrounded by fibrous tissue.

The absence of fibrosis is a characteristic sign of the disease, allowing it to be differentiated from liver cirrhosis.

Main symptoms:

• ascites;
• splenomegaly;
• bleeding from varicose veins of the esophagus and stomach;
• the liver is slightly enlarged, the surface is fine-grained;
• liver function tests show very little change;
• portal pressure is sharply increased;
• portal hypertension has a presinusoidal character; hepatic venous pressure is normal or slightly increased;
• In liver biopsies, proliferation of hepatocytes without the development of fibrous tissue is observed.

The pathogenesis of portal hypertension in this disease is unclear. Probably, there is compression of the portal veins and an increase in splenic blood flow. Nodular hyperplasia of the liver is often observed in rheumatoid arthritis, systemic blood diseases.

Focal nodular hyperplasia is a rare disease of unknown etiology, characterized by the appearance of nodules in the liver parenchyma measuring 2-8 mm, located mainly at the portal of the liver. At the same time, hypoplasia of the main trunk of the portal vein is detected.

The nodules compress normal liver tissue and contribute to the development of presinusoidal portal hypertension. Liver function tests are slightly altered.

Diseases of the hepatic venules and veins, inferior vena cava

Budd-Chiari disease is a primary obliterating endophlebitis of the hepatic veins with thrombosis and subsequent occlusion.

The etiology of the disease is unknown. The role of autoimmune mechanisms is not excluded.

In Budd-Chiari disease, the internal lining of the hepatic veins is overgrown, beginning near their mouths or in the inferior vena cava near the place where the hepatic veins enter; sometimes the process begins in the small intrahepatic branches of the hepatic veins. Acute and chronic forms of the disease are distinguished.

The acute form of Budd-Chiari malformation has the following symptoms:

• suddenly there is intense pain in the epigastrium and right hypochondrium;
• vomiting suddenly appears (often bloody);
• the liver enlarges rapidly;
• ascites develops quickly (within a few days) with a high protein content in the ascitic fluid (up to 40 g/l); often hemorrhagic ascites;
• when the inferior vena cava is involved in the process, swelling of the legs and dilation of the subcutaneous veins in the abdomen and chest are observed;
• high body temperature;
• 1/2 of patients have mild jaundice;
• There is moderate splenomegaly, but it is not always determined due to the presence of ascites.

The patient usually dies in the first days of the disease from acute liver failure.

Thus, the acute form of Budd-Chiari disease can be suspected in the presence of persistent severe abdominal pain and rapid development of portal hypertension, hepatomegaly and liver failure.

The chronic form of Budd-Chiari disease is observed in 80-85% of patients, with incomplete blockage of the hepatic veins.

Symptoms of the disease:

• in the early stages of the process, subfebrile body temperature, transient abdominal pain, and dyspeptic disorders are possible;
• after 2-4 years, a full-blown clinical picture of the disease appears with the following manifestations: hepatomegaly, the liver is dense, painful, and the development of true liver cirrhosis is possible;
• dilated veins on the anterior abdominal wall and chest;
• pronounced ascites;
• bleeding from varicose veins of the esophagus, hemorrhoidal veins;
• increased ESR, leukocytosis, increased serum gamma globulin levels;
• in liver biopsies - pronounced venous congestion (in the absence of heart failure), a picture of liver cirrhosis;
• Reliable diagnostic methods are venohepatography and lower cavography.

The disease ends with severe liver failure. Life expectancy is from 4-6 months to 2 years.

Budd-Chiari syndrome is a secondary disorder of venous blood outflow from the liver in a number of pathological conditions not associated with changes in the liver's own vessels. However, there is currently a tendency to use the term "Budd-Chiari syndrome" to denote a difficult outflow of venous blood from the liver, and regardless of the cause, the blockade should lie on the path from the liver to the right atrium. According to this definition, it is proposed to distinguish 4 types of Budd-Chiari syndrome depending on the location and mechanism of the blockade:

• primary disorders of the hepatic veins;
• compression of the hepatic veins by benign or malignant growths;
• primary pathology of the inferior vena cava;
• Primary disorders of hepatic venules.

The clinical picture of the syndrome and Budd-Chiari disease are similar. The clinical picture should also take into account the symptoms of the underlying disease that caused the Budd-Chiari syndrome.

In recent years, non-invasive (echography, computed tomography, nuclear resonance imaging) research methods have been used instead of invasive (cavography, liver biopsy) to diagnose Budd-Chiari syndrome.

If Budd-Chiari syndrome is suspected, it is recommended to start with an ultrasound of the liver and color Doppler echography. If Doppler echography shows normal liver veins, the diagnosis of Budd-Chiari syndrome is excluded. Using echography, the diagnosis of Budd-Chiari syndrome can be made in 75% of cases.

If the ultrasound is uninformative, one should resort to computed tomography using contrast or magnetic resonance imaging.

If the above non-invasive methods do not allow a diagnosis to be made, cavography, phlebography of the hepatic veins or liver biopsy are used.

Veno-occlusive disease occurs as a result of acute occlusion of small and medium branches of the hepatic veins without damage to larger venous trunks.

The etiology is unknown. In some cases, heliotrope intoxication plays a role (Uzbekistan, Tajikistan, Kazakhstan, Kyrgyzstan, Armenia, Krasnodar Krai, Afghanistan, Iran). Sometimes the cause of the disease may be exposure to ionizing radiation.

Histologically, the following changes are revealed in the liver:

• non-thrombotic obliteration of the smallest branches of the hepatic veins, congestion in the center of the liver lobules, local atrophy and necrosis of hepatocytes;
• In subacute and chronic forms, centrilobular fibrosis develops, followed by liver cirrhosis.

The disease usually develops between the ages of 1 and 6. Acute, subacute and chronic forms are distinguished. The acute form is characterized by:

• sharp pain in the right hypochondrium;
• nausea, vomiting, often bloody;
• ascites (develops 2-4 weeks after the onset of the disease);
• hepatomegaly;
• moderate jaundice;
• splenomegaly;
• significant loss of body weight.

1/3 of patients die from hepatocellular failure, 1/3 develop liver cirrhosis, 1/3 recover within 4-6 weeks.

The subacute form is characterized by:

• hepatomegaly;
• ascites;
• moderate changes in liver function tests;

Later the disease becomes chronic. The chronic form occurs as liver cirrhosis with portal hypertension.

Cruveilhier-Baumgarten disease and syndrome

Cruveilhier-Baumgarten disease and syndrome are rare and may cause portal hypertension.

Cruveilhier-Baumgarten disease is a combination of congenital hypoplasia of the portal vein, liver atrophy and non-closure of the umbilical vein.

The main manifestations of the disease:

• dilated subcutaneous venous collaterals of the abdominal wall ("caput medusae");
• a venous noise above the navel, determined by auscultation, which intensifies if the patient lifts his head from the pillow; the noise can be perceived by palpation and disappears when pressing with the palm above the navel;
• splenomegaly and hypersplenism syndrome (pancytopenia);
• pain in the epigastrium and right hypochondrium;
• gastrointestinal bleeding;
• persistent flatulence;
• ascites;
• high pressure in the portal vein (determined using splenoportometry).

The prognosis is unfavorable. Patients die from gastrointestinal bleeding or liver failure.

Cruveilhier-Baumgarten syndrome is a combination of non-closure of the umbilical vein (recanalization) and portal hypertension of an acquired rather than congenital nature.

The main causes of the syndrome are:

• cirrhosis;
• obliteration or endophlebitis of the hepatic veins.

Cruveilhier-Baumgarten syndrome is more often observed in young women. The clinical manifestations of the syndrome are the same as in Cruveilhier-Baumgarten disease, but unlike the latter, an enlarged liver is observed.

Hepatic portal sclerosis

Hepatoportal sclerosis is characterized by splenomegaly, hypersplenism, and portal hypertension without occlusion of the portal and splenic veins and pathological changes in the liver. There are many unclear pathogenesis of this disease. Other names for it are: non-cirrhotic portal fibrosis, non-cirrhotic portal hypertension, idiopathic portal hypertension. Banti syndrome (a term that has fallen into disuse) also probably belongs to this group of diseases. The disease is based on damage to the intrahepatic branches of the portal vein and endothelial cells of the sinusoids. Increased intrahepatic resistance indicates intrahepatic obstruction of the portal bed. Hepatoportal sclerosis can be caused by infections, intoxications; in many cases, the cause remains unknown. In children, the first manifestation may be intrahepatic thrombosis of small branches of the portal vein.

In Japan, this disease occurs mainly in middle-aged women and is characterized by occlusion of the intrahepatic branches of the portal vein. Its etiology is unknown. A similar disease, called noncirrhotic portal fibrosis, affects young men in India. It is thought to be related to arsenic in drinking water and folk remedies. It is more likely to develop as a result of years of exposure of the liver to recurrent intestinal infections.

Cases of a largely similar disease have been described in the USA and Great Britain.

Liver biopsy reveals sclerosis and sometimes obliteration of the intrahepatic venous bed, but all these changes, especially fibrosis, may be minimal. Autopsy reveals thickening of the walls of large veins near the portal of the liver and narrowing of their lumen. Some changes are secondary, caused by partial thrombosis of small branches of the portal vein with subsequent restoration of blood flow. Perisinusoidal fibrosis is usually present, but it can only be detected by electron microscopy.

Portal venography reveals narrowing of the small branches of the portal vein and a decrease in their number. The peripheral branches have uneven contours and depart at an acute angle. Some large intrahepatic branches may not be filled with contrast medium, while at the same time, very thin vessels are observed to grow around them. Contrast examination of the hepatic veins confirms changes in the vessels; venovenous anastomoses are often detected.

Tropical splenomegaly syndrome

This syndrome develops in individuals living in malaria-endemic regions and is characterized by splenomegaly, lymphocytic infiltration of the sinusoids, hyperplasia of Kupffer cells, increased IgM levels and serum antibody titers to malaria plasmodia. Improvement occurs with prolonged chemotherapy with antimalarial drugs. Portal hypertension is insignificant, and bleeding from varicose veins is rare.

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Objective examination

Liver cirrhosis is the most common cause of portal hypertension. In patients with liver cirrhosis, it is necessary to find out all possible causes, including a history of alcoholism or hepatitis. In the development of extrahepatic portal hypertension, previous (particularly in the neonatal period) inflammatory diseases of the abdominal organs are of particular importance. Blood coagulation disorders and the use of certain drugs, such as sex hormones, predispose to thrombosis of the portal or hepatic vein.

Anamnesis

• The patient has cirrhosis or chronic hepatitis
• Gastrointestinal bleeding: number of episodes, dates, volume of blood loss, clinical manifestations, treatment
• Results of previous endoscopy
• Indications of alcoholism, blood transfusions, viral hepatitis B and C, sepsis (including neonatal sepsis, sepsis due to intra-abdominal pathology or other origins), myeloproliferative diseases, use of oral contraceptives

Survey

• Signs of liver cell failure
• Veins of the abdominal wall:
  • location
  • direction of blood flow
• Splenomegaly
• Liver size and consistency
• Ascites
• Swelling of the shins
• Rectal examination
• Endoscopic examination of the esophagus, stomach and duodenum

Additional research

• Liver biopsy
• Hepatic vein catheterization
• Selective arteriography of abdominal organs
• Ultrasound, computed tomography or magnetic resonance imaging of the liver

Hematemesis is the most common manifestation of portal hypertension. It is necessary to determine the amount and severity of previous bleeding, whether it led to impaired consciousness or coma, and whether a blood transfusion was performed. Melena without hematemesis may be observed with varicose veins. The absence of dyspepsia and pain in the epigastric region, as well as pathology in a previous endoscopic examination, allows us to exclude bleeding from a peptic ulcer.

The stigmas of cirrhosis can be identified - jaundice, spider veins, palmar erythema. It is necessary to pay attention to the presence of anemia, ascites and prodromal symptoms of coma.

Veins of the anterior abdominal wall

In intrahepatic portal hypertension, some blood may flow from the left branch of the portal vein through the periumbilical veins into the inferior vena cava. In extrahepatic portal hypertension, dilated veins may appear on the lateral abdominal wall.

The nature of the distribution and direction of blood flow. The dilated tortuous collateral veins diverging from the umbilicus are called the "head of Medusa". This sign is rare, usually one or two veins are enlarged, most often the epigastric ones. Blood flows away from the umbilicus; with obstruction of the inferior vena cava, blood flows through the collaterals from below upwards, into the system of the superior vena cava. With tense ascites, functional obstruction of the inferior vena cava may develop, which makes it difficult to explain the observed changes.

The veins of the anterior abdominal wall can be visualized by photographing them with infrared light.

Noises

In the area of the xiphoid process or umbilicus, a venous murmur can be heard, sometimes spreading to the precordial area, to the sternum or to the area of the liver. At the site of its greatest expression, vibration can be detected with light pressure. The murmur can increase during systole, during inspiration, in an upright position or in a sitting position. The murmur occurs when blood passes from the left branch of the portal vein through the large umbilical and paraumbilical veins located in the falciform ligament into the veins on the anterior abdominal wall - the superior epigastric vein, internal thoracic vein and into the inferior epigastric vein. Sometimes venous murmur can also be heard over other large venous collaterals, for example, over the inferior mesenteric vein. Systolic arterial murmur usually indicates primary liver cancer or alcoholic hepatitis.

The combination of dilated veins of the anterior abdominal wall, loud venous noise above the navel and normal liver size is called Cruveilhier-Baumgarten syndrome. It may be caused by non-closure of the umbilical vein, but more often it is compensated cirrhosis of the liver.

A murmur extending from the xiphoid process to the umbilicus and the “capita Medusa” indicate obstruction of the portal vein distal to the origin of the umbilical veins from the left branch of the portal vein, i.e. intrahepatic portal hypertension (liver cirrhosis).

Spleen

The spleen is enlarged in all cases, and its dense edge is revealed upon palpation. There is no clear correspondence between the size of the spleen and the pressure in the portal vein. In young patients and in large-nodular cirrhosis, the spleen is enlarged to a greater extent.

If the spleen cannot be palpated or its size is not enlarged upon examination, then the diagnosis of portal hypertension is questionable.

Pancytopenia associated with enlargement of the spleen (secondary "hypersplenism") is detected in the peripheral blood. Pancytopenia is associated with hyperplasia of the reticuloendothelial system rather than with portal hypertension, and does not disappear with the development of portocaval shunts, despite a decrease in portal pressure.

Liver

Both small and enlarged liver sizes are significant, so their percussion determination should be carried out carefully. There is no clear dependence of liver size on portal vein pressure.

It is necessary to pay attention to the consistency of the liver, its soreness and the lumpiness of the surface during palpation. If the liver is soft, one should think about extrahepatic portal vein obstruction. If the consistency is dense, cirrhosis is more likely.

Ascites

Ascites is rarely caused by portal hypertension alone, although a significant increase in portal pressure may be the leading factor in its development. With portal hypertension, filtration pressure in the capillaries increases, causing fluid to leak into the abdominal cavity. In addition, the development of ascites in cirrhosis indicates, in addition to portal hypertension, hepatocellular insufficiency.

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Rectum

Varicose veins of the anorectal area can be detected during rectoscopy; the veins may bleed. They are observed in 44% of cases of liver cirrhosis, and are exacerbated in patients with existing bleeding from varicose veins of the esophagus. They should be distinguished from simple hemorrhoids, which are bulging venous bodies not associated with the portal vein system.