Portal hypertension: symptoms

Symptoms and instrumental data for portal hypertension

1. The earliest symptoms of portal hypertension are flatulence ("wind before rain"), a feeling of fullness of the intestine, nausea, pain throughout the abdomen, a decrease in appetite.
2. As a rule, there are "symptoms of poor nutrition" (poorly expressed subcutaneous tissue, dry skin, muscular atrophy).
3. Expansion of the veins of the abdominal wall, translucent through the skin in the navel and in the sides of the abdomen. With severe blood circulation along the para-ulbicular veins or the umbilical vein itself around the navel, a venous plexus ("jellyfish head") is formed.
4. With progression of portal hypertension, ascites develops (it is most typical for intrahepatic portal hypertension), in addition, edema on the legs, bleeding from varicose veins of the esophagus and stomach, rectum, nosebleeds are possible .
5. Splenomegaly different degrees of severity (depending on the nature of the disease that caused the development of portal hypertension). It is often accompanied by the development of hypersplenism (a syndrome of pancytopenia: anemia, leukopenia, thrombocytopenia).
6. Hepatomegaly different degrees of severity (depending on the cause that led to the development of portal hypertension). The liver is dense, sometimes painful, its edge sharp. With cirrhosis of the liver it is tuberous, the nodes of regeneration are probed (these cases must be differentiated with liver cancer).
7. With long-existing and severe portal hypertension portal encephalopathy develops , which manifests itself as headaches, dizziness, memory loss, violation of the sleep formula (sleeplessness at night, drowsiness during the day), with severe encephalopathy, hallucinations, delusions, inadequate behavior of patients, neurological signs of central nervous system damage .

Different forms of portal hypertension (depending on the location of the block) have their clinical features.

The superhepatic form of portal hypertension is characterized by:

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

The subheading form of portal hypertension has the following features:

• the main symptoms are splenomegaly, hypersplenism;
• the liver is usually not enlarged;
• Sub-hepatic portal hypertension usually develops slowly, in the future there are multiple esophageal-gastric bleeding.

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

• early symptoms are persistent dyspeptic syndrome, flatulence, periodic diarrhea, weight loss;
• late symptomatology: significant splenomegaly. Varicose veins with possible bleeding, ascites, hypersplenism;
• with predominance of hepatolienne type intrahepatic hypertension, the pain is localized in the epigastrium and especially in the left hypochondrium, laparoscopy reveals stagnant veins in the large curvature of the stomach and spleen; with FEGD, varicose veins are found even in the high esophagus;
• with predominance of intestinal-mesenteric type of intrahepatic portal hypertension, the pain is localized around the navel, in the iliac regions or in the liver region; With laparoscopy, stagnant veins are found mainly in the diaphragm, liver, round ligament, intestine. With 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. Arterio-venous fistulas.

Arterio-venous fistulas are congenital and acquired. Congenital fistulas are observed with hereditary hemorrhagic telangiectasia.

Acquired arterio-venous aneurysms are formed due to trauma, liver biopsy, rupture of an aneurysm of the hepatic or splenic artery. Sometimes arterio-venous aneurysms accompany hepatocellular carcinoma.

In arterio-venous fistulas, there is a combination of the hepatic artery and portal vein or splenic artery and splenic vein. The presence of fistulas causes an increased flow of blood into the portal system. Clinically, the patient has symptoms of portal hypertension. One third of patients have abdominal pain. The main method of diagnosis of arterio-venous 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:

• expressed splenomegaly and less often hepatomegaly;
• leukocytosis with neutrophil shift, often with pronounced rejuvenation of the formula (the appearance of myeloblasts, myelocytes);
• anemia;
• hyperthrombocytosis (platelets are functionally inferior);
• threefold metaplasia of hematopoiesis in the spleen, liver (extramedullary hemopoiesis);
• marked bone marrow fibrosis in trepanobiobtate from the ilium;
• narrowing of the medullary canal, thickening of the cortical layer on the radiographs of pelvic bones, vertebrae, ribs, long tubular bones.

3. Cavernomatosis of the portal vein.

The etiology and pathogenesis of this disease are unknown. Many experts consider it congenital, others - acquired (early-onset thrombosis of the portal vein followed by its 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 as a syndrome of portal hypertension or portal vein thrombosis, complicated by bleeding from varicose veins of the esophagus and stomach, intestinal infarction, hepatic coma. The prognosis is unfavorable, the life expectancy since the appearance of clinical signs of portal hypertension is 3-9 years. The main method for diagnosing cavernomatosis of the portal vein is angiography.

4. Thrombosis or occlusion of the portal or splenic veins

Depending on the localization of portal vein thrombosis (pelletrombosis), radicular (radicular) thrombosis is isolated , in which the splenic vein or (more rarely) other veins that enter the common trunk are affected; trunk - with obstruction of the portal vein in the area between the splenic vein and the portal vein, and terminal - with the localization of thrombi in the branches of the intravenous vein inside the liver.

Isolated obstruction of the spleen vein causes left-sided portal hypertension. Its cause may be any of the factors that cause obstruction of the portal vein. Particularly important are pancreatic diseases, such as cancer (18%), pancreatitis (65%), pseudocysts, and pancreatectomy.

If the obstruction develops distally to the site of the confluence of the left gastric vein, the blood through the collaterals, bypassing the spleen vein, enters the short gastric veins and then into the bottom of the stomach and the lower part of the esophagus, flowing out from there into the left gastric and portal vein. This leads to a very significant varicose veins of the bottom of the stomach; The veins of the lower part of the esophagus widen slightly.

The main causes of pylorbosis:

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

The main symptoms of acute piles:

• more often observed with polycythemia, cirrhosis, after splenectomy
• severe pain in the abdomen;
• bloody vomiting;
• collapse;
• rapidly developing ascites (sometimes hemorrhagic);
• the liver is not enlarged; if acute thrombosis of the portal vein occurs in a patient with cirrhosis of the liver, then there is hepatomegaly;
• jaundice is absent;
• in the blood leukocytosis with neutrophil shift;
• when thrombosis of mesenteric arteries is attached, there are infarctions of the intestine with a picture of the acute abdomen;
• with thrombosis of the splenic vein, there are pains in the left hypochondrium and an increase in the spleen.

The outcome is usually lethal.

Suspicion of acute piles is required if, after abdominal trauma, liver surgery and the portal system, portal hypertension suddenly appears.

The prognosis for acute piles is poor. Complete blockage of the portal vein leads to death within a few days of gastrointestinal bleeding, intestinal infarction, acute hepatic-cell insufficiency.

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

• signs of underlying disease;
• pain of varying intensity in the right upper quadrant, epigastrium, spleen;
• splenomegaly;
• the enlargement of the liver is not characteristic, except for cases of piles with a cirrhosis of the liver;
• gastrointestinal hemorrhage (sometimes this is the first symptom of pilelet disease);
• ascites (in some patients);
• thrombosis of the portal vein on the background of liver cirrhosis is characterized by sudden development of ascites, other signs of portal hypertension and a sharp deterioration in liver function.

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

The peculiarity of diagnosis of idiopathic portal vein thrombosis: with laparoscopy, the unchanged liver is revealed with well-developed collaterals, ascites, enlarged spleen.

The prognosis for piles is unfavorable. Patients die from gastrointestinal bleeding, intestinal infarction, hepatic renal failure.

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

Main clinical manifestations:

• a sharp deterioration in the patient's condition against the background of the underlying disease;
• fever with tremendous chills and puffy sweats, body temperature reaches 40 ° C;
• intense cramping pain in the abdomen, often in the right upper parts;
• as a rule, there is vomiting, often diarrhea;
• the liver is enlarged, painful;
• 50% of patients have splenomegaly;
• mild jaundice;
• laboratory data - general blood test: leukocytosis with a shift of the leukocyte formula to the left; increased ESR; biochemical blood test: hyperbilirubinemia, increased activity of aminotransferases, increased content of y-globulins, fibrinogen, seromucoid, haptoglobin, sialic acids;
• with cannulation of the umbilical vein, pus is found in the portal system.

4. Diseases of the liver

Diagnosis of liver diseases, listed above, as a cause of portal hypertension, is made on the basis of appropriate symptoms.

• Cirrhosis of the liver

All forms of cirrhosis lead to portal hypertension; It begins with the obstruction of the portal channel. The blood from the portal vein is redistributed into the collateral vessels, some of it is directed to bypass the hepatocytes and gets directly into the small liver veins in the fibrosis septa. These anastomoses between the portal and the hepatic vein develop from sinusoids that are inside the septum. The hepatic vein inside the fibrous septum is shifted further outwards until there are messages with the portal vein branch through the sinusoid. The blood supply of the regeneration units from the portal vein is disturbed, the blood in them comes from the hepatic artery. In the liver, cirrhosis also reveals larger intervenous anastomoses. At the same time, about a third of all blood entering the liver passes through these shunts, bypassing the sinusoids, i.e. Bypassing the functioning of the hepatic tissue.

Partially disturbance of the portal blood flow is caused by the regeneration nodes, which compress the branches of the portal vein. This would lead to postsinusoidal portal hypertension. However, with cirrhosis, the pressure of wedging of the hepatic veins (sinusoidal) and pressure in the main trunk of the portal vein are almost the same, and the stasis extends to the branches of the portal vein. Sinusoids, apparently, determine the main resistance to blood flow. Due to changes in the Disse space caused by its collagenization, the sinusoids narrow; especially it can be expressed with alcoholic liver damage, in which the blood flow in the sinusoids can decrease also because of the swelling of the hepatocytes. Consequently, obstruction develops all the way from the portal zones through sinusoids to the hepatic veins.

On the hepatic artery, a small amount of blood is delivered to the liver under high pressure, a large amount and under low pressure enter the portal vein. The pressure in these two systems is equalized in the sinusoids. Normally, the hepatic artery is probably of little importance for maintaining pressure in the portal vein. With cirrhosis, the connection between these vascular systems becomes more crowded due to arterioportal shunts. Compensatory expansion of the hepatic artery and an increase in blood flow along it promote the maintenance of perfusion of sinusoids.

• Other liver diseases that occur with the formation of nodes

To portal hypertension can cause various non-cirrhotic diseases, accompanied by the formation of nodes in the liver. They are difficult to diagnose, and usually they are mixed with cirrhosis or with "idiopathic" portal hypertension. A "normal" picture with puncture liver biopsy does not exclude this diagnosis.

Nodal regenerative hyperplasia. Throughout the liver, monoacinar nodules from cells similar to normal hepatocytes are diffusely determined. The appearance of these nodules is not accompanied by proliferation of connective tissue. The cause of their development is the obliteration of small (less than 0.5 mm) branches of the portal vein at the level of the acini. Obliteration leads to atrophy of affected acini, while adjacent acini, whose blood supply is not disturbed, undergo compensatory hyperplasia, which causes nodular degeneration of the liver. Portal hypertension is expressed to a large extent, sometimes hemorrhages in nodules are noted.

With hemorrhage, ultrasound reveals hypo-and iso-echogenic formations that have an anechoic central part. With CT, tissue density is reduced, and when it is contrasted, it does not increase.

In liver biopsy, two populations of hepatocytes differ in size. The biopsy does not have a diagnostic value.

The most common nodular regenerative hyperplasia develops with rheumatoid arthritis and Felty syndrome. In addition, nodes are formed in myeloproliferative syndromes, syndromes of increased blood viscosity and as a reaction to drugs, especially anabolic steroids and cytostatics.

Portocaval shunting for bleeding varicose veins of the esophagus is usually well tolerated.

Partial nodal transformation is a very rare disease. In the area of the lobes of the liver, nodes are formed. On the periphery, the liver tissue has a normal structure or atrophy. The nodes interfere with normal blood flow in the liver, resulting in the development of portal hypertension. The function of hepatocytes is not disturbed. Fibrosis is usually absent. Diagnosis of the disease is difficult, it is often possible to confirm the diagnosis only with autopsy. 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 development of intrahepatic portal hypertension.

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

The defeat of the liver in workers spraying vineyards in Portugal, can be due to contact with copper. The disease can be complicated by the development of angiosarcoma.

When inhaled vapors of polymerized vinyl chloride develop sclerosis of portal veins with the development of portal hypertension, as well as angiosarcoma.

When vitamin A is intoxicated, reversible portal hypertension may occur-vitamin A accumulates in Ito cells. To presynusoidal fibrosis and portal hypertension may result in long-term use of cytostatics, for example methotrexate, 6-mercaptopurine and azathioprine.

Idiopathic portal hypertension (noncirrhotic portal fibrosis) is a disease of unknown etiology, manifested by portal hypertension and splenomegaly without portal portal obstruction, changes in extrahepatic vasculature and severe liver damage.

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

Main clinical manifestations and instrumental data:

• splenomegaly;
• ascites;
• gastric bleeding;
• liver tests are normal or changed little, liver failure develops in the late stage;
• in liver biopsy specimens periportal fibrosis is revealed, there may be no histological changes (portal tracts must necessarily be found);
• absence of signs of portal or splenic thrombosis according to angiography;
• normal or slightly elevated wedged hepatic venous pressure, high pressure in the portal vein according to the data of catheterization or puncture of the portal vein.

Idiopathic portal hypertension can develop in patients with systemic scleroderma, autoimmune hemolytic anemia, goitre Hashimoto, chronic nephritis.

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

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

Absence of fibrosis is a characteristic sign of the disease, which makes it possible to differentiate it with cirrhosis of the liver.

The main symptoms are:

• ascites;
• splenomegaly;
• bleeding of the esophageal and gastric dilated veins;
• the liver is slightly enlarged, the surface is fine-grained;
• functional tests of the liver have changed very little;
• portal pressure is sharply increased;
• portal hypertension has a presynusoidal character; hepatic venous pressure is normal or slightly elevated;
• in hepatic biopsy notes proliferation of hepatocytes without the development of fibrous tissue.

The pathogenesis of portal hypertension with this disease is not clear. Probably, there is a compression of portal veins and an increase in splenic blood flow. Often, nodular hyperplasia of the liver is observed with rheumatoid arthritis, systemic blood diseases.

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

Nodules squeeze normal liver tissue and promote the development of presynusoidal portal hypertension. Functional tests of the liver have changed little.

Diseases of hepatic venules and veins, inferior vena cava

Badda-Chiari disease is the 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 Badd-Chiari's disease, the growth of the inner lining of the hepatic veins, beginning near their mouths or in the inferior vena cava near the site of the admission of the hepatic veins, is revealed, sometimes the process begins in the small intrahepatic branches of the hepatic veins. Isolate acute and chronic forms of the disease.

The acute form of Badda-Chiari's disease has the following symptoms:

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

The patient usually dies in the first days of illness from acute hepatic insufficiency.

Thus, the acute form of Budd Chiari's disease can be suspected in the presence of constant intense pain in the abdomen and rapid development of portal hypertension, hepatomegaly and liver failure.

Chronic form of Budd-Chiari's disease is observed in 80-85% of patients, with an incomplete occlusion of hepatic veins.

Symptomatology of the disease:

• in the early stages of the process are possible subfebrile body temperature, transient abdominal pain, dyspeptic disorders;
• in 2-4 years there is a developed clinic of the disease with the following manifestations: hepatomegaly, the liver is dense, painful, it is possible to form true cirrhosis of the liver;
• enlarged veins on the anterior abdominal wall and chest;
• marked ascites;
• bleeding from varicose veins of the esophagus, hemorrhoids;
• an increase in ESR, leukocytosis, an increase in the level of y-globulin in the serum;
• in liver biopsies - pronounced venous congestion (in the absence of heart failure), a picture of liver cirrhosis;
• venogepatografiya and lower kavografiya are reliable diagnostic methods.

The disease ends with severe hepatic insufficiency. Life expectancy ranges from 4-6 months to 2 years.

Badda-Chiari syndrome is a secondary impairment of venous blood outflow from the liver in a number of pathological conditions not associated with changes in the own blood vessels of the liver. However, at present a tendency has arisen to use the term "Badd-Chiari syndrome" to refer to a complicated outflow of venous blood from the liver, regardless of the cause of the blockade, which must lie on the way from the liver to the right atrium. According to this definition, it is proposed to distinguish 4 types of Badd-Chiari syndrome depending on the place and mechanism of the blockade:

• primary disturbances of hepatic veins;
• compression of the hepatic veins with benign or malignant growths;
• primary pathology of the inferior vena cava;
• primary disorders of hepaticveoles.

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

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

If suspected of Badda-Chiari syndrome, it is recommended to start with ultrasound of the liver and color Doppler echography. If the data on the normal state of the veins of the liver are obtained with doppler echography, a diagnosis of the Badd-Chiari syndrome is excluded. With the help of ultrasound, the diagnosis of the Budda-Chiari syndrome can be diagnosed in 75% of cases.

In case of non-informative ultrasound, computer tomography using contrast or magnetic resonance imaging should be used.

If these non-invasive methods do not allow diagnosis, use kavografiyu, phlebography of hepatic veins or liver biopsy.

Venous-occlusive disease occurs due to acute blockage of small and medium branches of the hepatic veins without affecting larger venous trunks.

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

Histologically, the following changes are detected in the liver:

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

The disease usually develops between the ages of 1 to 6 years. Isolate acute, subacute and chronic forms. The acute form is characterized by:

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

1/3 of patients die from hepatocellular insufficiency, one-third of patients have cirrhosis, one-third recover after 4-6 weeks.

Subacute form is characterized by:

• hepatomegaly;
• ascites;
• moderate changes in functional liver samples;

Later the disease takes on a chronic course. The chronic form proceeds as cirrhosis of the liver with portal hypertension.

Disease and the Cruevilian-Baumgarten syndrome

Disease and the Cruevelle-Baumgarten syndrome are rare and may be the cause of portal hypertension.

Cruevelle-Baumgarten's disease is a combination of congenital portal vein hypoplasia, liver atrophy, and nebulization of the umbilical vein.

The main manifestations of the disease:

• enlarged subcutaneous venous collaterals of the abdominal wall ("caput medusae");
• defined auscultatory venous noise above the navel, it increases if the patient raises his head from the pillow; noise can be palpable and disappear when pressing the palm above the navel;
• Splenomegaly and hypersplenism syndrome (pancytopenia);
• pain vepigastria and right hypochondrium;
• gastrointestinal bleeding;
• persistent flatulence;
• ascites;
• high pressure in the portal vein (determined by splenometry).

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

The Cruevelle-Baumgarten syndrome is a combination of unpaired umbilical vein (recanalization) and portal hypertension not of an innate but of an acquired character.

The main causes of the syndrome are:

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

The Cruevelle-Baumgarten syndrome is more common in young women. The clinical manifestations of the syndrome are the same as in the case of Cruevelle-Baumgarten disease, but unlike the latter, there is an increase in the liver.

Hepatic-portal sclerosis

For hepatic-portal sclerosis splenomegaly, hypersplenism and portal hypertension without occlusion of portal and splenic veins and pathological changes on the part of the liver are characteristic. In the pathogenesis of this disease, much is unclear. Other names include: noncyrrotic portal fibrosis, noncyrrotic portal hypertension, idiopathic portal hypertension. Banty syndrome (a term that has become obsolete) also probably belongs to this group of diseases. At the heart of the disease is damage to the intrahepatic branches of the portal vein and endothelial cells of sinusoids. Increased intrahepatic resistance indicates intrahepatic obstruction of the portal channel. The cause of hepatic-portal sclerosis can be infection, intoxication; 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. His etiology is unknown. A similar disease, called noncirrhotic portal fibrosis, in India young men are ill. The connection of this disease with the presence of arsenic in drinking water and in alternative medicine is supposed. It is more likely that it develops as a result of long-term effects on the liver of recurrent intestinal infections.

Cases in many ways similar diseases are described in the US and UK.

Liver biopsy reveals sclerosis, and sometimes obliteration of the intrahepatic venous bed, but all these changes, especially fibrosis, can be expressed minimally. In autopsy, a thickening of the walls of large veins near the gates of the liver and a narrowing of their lumen are observed. Some changes are secondary, caused by partial thrombosis of small branches of the portal vein with subsequent restoration of blood flow. Usually there is perisinusoidal fibrosis, but it can be detected only by electron microscopy.

In portal venography, narrowing of the small branches of the portal vein and a decrease in their number are revealed. Peripheral branches have uneven contours and depart at an acute angle. Some large intrahepatic branches may not be filled with a contrasting substance, simultaneously around them there is a proliferation of very thin vessels. Contrastive examination of the hepatic veins confirms vessel changes; often veinovenous anastomoses are found.

Tropical Splenomegaly Syndrome

This syndrome develops in people living in malaria endemic regions, and is manifested by splenomegaly, lymphocytic infiltration of sinusoids, hyperplasia of Kupffer cells, increased levels of IgM, and titer of serum antibodies to malarial plasmodia. With prolonged chemotherapy, antimalarial drugs are improving. Portal hypertension is insignificant, bleeding from varicose-dilated veins develops rarely.

[1], [2], [3], [4], [5], [6], [7]

Objective examination

Cirrhosis is the most common cause of portal hypertension. In patients with cirrhosis of the liver, it is necessary to find out all possible causes of it, including an indication of alcoholism or hepatitis in an anamnesis. In the development of extrahepatic portal hypertension, the inflammatory diseases of the abdominal cavity organs were especially important earlier (in particular, in the neonatal period). Violations of the coagulating system of blood and the intake of certain drugs, such as sex hormones, predispose to thrombosis of the portal or hepatic vein.

Anamnesis

• Presence of a patient with cirrhosis or chronic hepatitis
• Gastrointestinal bleeding: the number of episodes, dates, volume of blood loss, clinical manifestations, treatment
• The results of previous endoscopy
• Indications for alcoholism, blood transfusions, viral hepatitis B and C, sepsis (including sepsis of newborns, sepsis due to intra-abdominal pathology or other origin), myeloproliferative diseases, oral contraceptive use

Examination

• Signs of liver-cell failure
• Veins of the abdominal wall:
  • location
  • direction of blood flow
• Splenomegaly
• The size and consistency of the liver
• Ascites
• Puff edema
• Rectal examination
• Endoscopic examination of the esophagus, stomach and duodenum

Additional research

• Puncture liver biopsy
• Catheterization of the hepatic vein
• Selective arteriography of the abdominal cavity organs
• Ultrasound, computer or magnetic resonance imaging of the liver

Blood vomiting is the most common manifestation of portal hypertension. It is necessary to find out the number and severity of previously transmitted bleeding, whether they led to a breakdown in consciousness or coma and whether blood transfusion was performed. With varicose veins, melena can be observed without bloody vomiting. The absence of dyspepsia and tenderness in the epigastric region, as well as pathology in the previous endoscopic study, makes it possible to exclude bleeding from the peptic ulcer.

You can identify stigmata of cirrhosis - jaundice, vascular sprouts, palmar erythema. It is necessary to pay attention to the presence of anemia, ascites and prodromal coma symptoms.

Veins of anterior abdominal wall

With intrahepatic portal hypertension, a certain amount of blood can flow out of the left branch of the portal vein through the periapical veins into the inferior vena cava. With extrahepatic portal hypertension, enlarged veins can appear on the lateral wall of the abdomen.

The nature of distribution and direction of blood flow. Expanded convoluted collateral veins, diverging from the navel, were called "the head of Medusa." This symptom is rare, usually one or two veins, usually epigastric, are enlarged. Blood flows away from the navel; with obstruction of the inferior vena cava, blood flows through the collaterals from the bottom up, into the system of the superior vena cava. With intense ascites, functional obstruction of the inferior vena cava can develop, which makes it difficult to explain the observed changes.

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

Noises

In the area of the xiphoid process or navel, you can listen to venous noise, sometimes spreading to the precordial region, to the sternum or to the area of the liver. In the place of its greatest expression with a slight pressure can catch the vibration. Noise may increase during systole, with inspiration, in an upright position or in a sitting position. Noise occurs when blood passes from the left branch of the portal vein through the large umbilical and peripump veins located in the crescent ligament, into the veins in the anterior abdominal wall - the upper epigastrium, the inner thoracic vein and the lower epigastric vein. Sometimes venous noise can also be heard over other large venous collaterals, for example, over the inferior mesenteric vein. Systolic arterial noise usually indicates primary liver cancer or alcoholic hepatitis.

The combination of the enlarged veins of the anterior abdominal wall, loud venous noise above the navel and the normal dimensions of the liver is called the Cruevel-Baumgarten syndrome. Its cause may be an unpaired umbilical vein, but more often compensated cirrhosis of the liver.

The noise extending from the xiphoid process to the navel, and the "head of Medusa" indicate obstruction of the portal vein distal to the site of the umbilical veins from the left branch of the portal vein, i.e. On intrahepatic portal hypertension (cirrhosis of the liver).

Spleen

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

If the spleen can not be palpated, or if its dimensions are not enlarged, the diagnosis of portal hypertension is questionable.

In peripheral blood reveal pancytopenia associated with an increase in spleen (secondary "hypersplenism"). Pancytopenia is associated more with hyperplasia of the reticuloendothelial system than with portal hypertension, and does not disappear with the development of portocaval shunts, despite the reduction in portal pressure.

Liver

The importance of both small and large sizes of the liver, so their percussion should be carefully determined. There is no distinct dependence of liver size on pressure in the portal vein.

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

Ascites

Ascites are rarely due to portal hypertension, although a significant increase in portal pressure may be the leading factor in its development. With portal hypertension, the filtration pressure in the capillaries increases, as a result of which the liquid swims into the abdominal cavity. In addition, the development of ascites in cirrhosis indicates, in addition to portal hypertension, and liver-cell failure.

[8], [9], [10], [11], [12]

Rectum

Varicose veins of the anorectal region can be detected during sigmoidoscopy; the veins can bleed. It is observed in 44% of cases of cirrhosis of the liver, and it is strengthened in patients with bleeding from the varicose-dilated esophagus. It should be distinguished from simple hemorrhoids, which is the swelling of venous bodies that are not associated with the portal vein system.