From Big Medical Encyclopedia

PORTAL HYPERTENSIA [late lat. (vena) of portae portal vein; hypertensia; synonym hypertension portal] — a complex of the changes arising at disturbance of outflow of blood from system of a portal vein. These changes come down to sharp build-up of pressure in system of a portal vein with delay of a blood flow, to developing of a splenomegaly, varicosity of a gullet, stomach and bleeding from them, a phlebectasia of a front abdominal wall, ascites, etc.

The concept «portal hypertensia» is entered into medical practice by fr. clinical physicians by Gilbert (And. N. Gilbert, 1901) and Villaoye (M. of Villaret, 1906), established existence of supertension in a portal vein at ascites.

Depending on localization of the process which caused disturbance portal blood circulation (see), distinguish the suprahepatic, vnutripeche-night, extrahepatic and mixed forms P. of. Differentiation and recognition of separate forms P. of became possible thanks to Russelo's works (L. Rousselot, 1936), Whipple (A. O. of Whipple, 1945) and Bleykmora (A. N. of Blake-more, 1946).

An etiology

such diseases as Kiari's disease (an obliterating endophlebitis or thrombophlebitis of hepatic veins), Badd's syndrome — Kiari are suprahepatic P.'s Reason of (thrombophlebitis of hepatic veins with transition to the lower vena cava, either thrombosis or a stenosis of the lower vena cava at the level of hepatic veins). The most frequent reason of intra hepatic P. of — cirrhosis, is more rare tumoral diseases of a liver. Vnepechen internal P. is caused by inborn pathology of vessels of portal system (an atresia of a portal vein, its cavernous transformation), and also a phlebosclerosis or thrombosis of portal system caused by various inflammatory diseases in an abdominal cavity (pancreatitis, cholecystitis, appendicitis, etc.), a prelum hems and other reasons. The reason of the mixed form P. of — cirrhosis with secondary thrombosis of portal system.

Pathological physiology

Cirrhoses of a liver (see), developing as a result inf. processes or toxic defeats, are characterized by excess proliferation of connective tissue elements on the periphery of hepatic segments or in their center (at centrolobular necroses). Growth of connecting fabric the prelum of portal or hepatic (central) venules, and sometimes is caused by that and others, as leads to essential increase in resistance to a blood-groove in venous vessels of a liver and to difficulty of outflow of blood from a portal vein. Existence of a large number of an arterioportalny anastomosis at various levels of a microcirculator bed through which the arterial blood under big pressure can come to system of a portal vein leads to increase of resistance to a blood-groove on portal vessels owing to emergence of the so-called hydraulic block.

At the extrahepatic (subhepatic) block the obstacle to outflow of blood arises at the level of a trunk portal vein (see), is more rare at the level of a splenic vein.

Build-up of pressure in a portal vein at P. happens considerable — to 600 mm w.g. (it is normal of 50 — 115 mm w.g.).

At cirrhosis only 13 — 15% of blood of portal system reach sinusoid. It limits transport of oxygen on a portal vein that is steadily compensated by activation of an arterial blood-groove. In turn, increase in blood supply of a liver on system of a hepatic artery inevitably deepens the phenomena of the hydraulic block.

Difficulty of outflow of blood from a portal vein on vessels of a liver and P.'s emergence leads to expansion porto-caval anastomosis (see) and activation of collateral blood supply. Especially large amount of blood flows on a porto-caval anastomosis through veins of a cardial part of a stomach, it is less — on veins of a front abdominal wall, a hepatoduodenal sheaf, a rectum, etc. A porto-caval anastomosis usually begins to function with the portal pressure exceeding 300 mm w.g. Stagnation of blood in a portal vein often leads to development of a splenomegaly and a delay of blood in a spleen (see. Hepatolienal syndrome ). At the expressed P., especially if it is a consequence of the intra hepatic block, quite often develops ascites (see). In formation of ascitic liquid the significant role belongs to an excess lymphopoiesis in a liver, to strengthening of an ekstravazation in vessels of its microcirculator bed. The intensification of an ekstravazation leading to formation of ascites is defined not only increase in hydrostatic pressure in sinusoids and venules, but also reduction of oncotic pressure of plasma owing to a hypoproteinemia, and also a delay of sodium and increase in osmotic pressure in tissue of a liver due to increase of molar concentration as a result of the exchange disturbances caused by a hypoxia.

Pathological anatomy

Fig. 1. Macrodrug of a liver: sharply expressed sclerosis of a portal vein with an obliteration of intra hepatic branches and fresh blood clot in a gleam (it is specified by an arrow).

Pathoanatomical changes at an intra hepatic and suprahepatic form P. of are caused by the diseases which caused them (see. Kiari disease , Cirrhoses of a liver ). At extrahepatic P. the liver can be reduced in sizes, without symptoms of cirrhosis, only some fibrosis of portal vessels is noted. The spleen is increased, is frequent with postinfarction hems and massive unions with surrounding fabrics. At gistol, a research of a spleen the fibroadenia of its pulp is defined in a varying degree. Vessels of portal system distalny obstacles are expanded, deformed with unevenly sclerosed walls (fig. 1). Expanded a coronary vein of a stomach (the left gastric vein, T.) and veins of a submucosal layer of a gullet eminate in a gleam, sometimes on nek-rum an extent of their wall are thinned. The main trunks of portal system can be completely obliterated with formation of dense network of the expanded, deformed vessels of a mesentery of a small bowel, area of a spleen and stomach.

The clinical picture

the Clinical picture of a syndrome of P. of is characterized by the following symptoms: a) emergence of collateral circulation — a phlebectasia on a front abdominal wall, a varicosity of a gullet and stomach, hemorrhoidal veins; b) splenomegaly; c) hypersplenism; d) hemorrhagic manifestations (bleeding from veins of a gullet and the cardia of a stomach, a mucous membrane of a nose, gums, uterine bleedings, hemorrhoidal bleedings; e) ascites.

At cirrhosis and Kiari's disease the listed symptoms are shown against the background of liver failure (see).

Fig. 2. The patient with intra hepatic portal hypertensia: expanded veins of a front abdominal wall.
Fig. 3. The patient with suprahepatic portal hypertensia: veins of sidewalls of a stomach are expanded.

Frequent the first symptom revealing a disease (especially at an extrahepatic form P. of), is accidentally found increased spleen or suddenly arisen bleeding from veins of a gullet. Patients complain of a febricula, feeling of weight in left the hypochondrium which sometimes is followed by pains and temperature increase, a meteorism, increase in a stomach. At patients with ascites expanded veins on a front abdominal wall around a navel (the head of a jellyfish) or towards a thorax or suprapubic area (fig. 2) with a nun's murmur, characteristic at auscultation, over them often come to light. A porto-caval anastomosis arises at the vnu-tripechenochny block of portal system as a result of functioning of an umbilical vein and is designated as Kryuvelye's syndrome — Baumgarten (see. Kryuvelye — Baumgarten a syndrome ). At Kiari's disease expanded veins are localized more often on sidewalls of a stomach (fig. 3), on a back and the lower extremities.

The most terrible symptom of P. of is bleeding from veins of a gullet and a stomach (see. Gastrointestinal bleeding ), arising sometimes suddenly against the background of full wellbeing. Bleeding is shown by a plentiful hematemesis (see. Hematemesis ), melena (see). It can recur and quite often comes to an end with the death of the patient. The lethality from bleeding from veins of a gullet remains high, especially at cirrhosis (45 — 60%), patients perish from the acute liver failure developing as a result of blood loss. Patients with extrahepatic P. of have bleeding easier, the lethality at the same time is much lower (5 — 10%). Quite often after massive bleeding patients with cirrhosis for the first time have an ascites, process in a liver is activated, its current worsens. At patients of ascites, as a rule, does not happen to extrahepatic P. At Kiari's disease ascites resistant also prevails over all other symptoms. Long persistent ascites leads to exhaustion, an adynamia, development of liver, renal and cardiopulmonary failure.

At P. the spleen is always increased. The sizes vary it over a wide range. Quite often patients note feeling of weight and pain in left hypochondrium which are caused by extensive unions of a spleen with surrounding fabrics, and also heart attacks spleens (see). At bleeding the spleen is sharply reduced, sometimes ceases to be palpated, and then gradually again increases to the former sizes. Splenomegaly (see) is followed by a hypersplenism, often the quantity of thrombocytes decreases to 80 000 — 30 000, quantity of leukocytes — to 3000 — 1500 in 1 mkl blood. Moderate anemia, after bleeding considerable, difficult giving in to treatment is observed.

The diagnosis

Diagnosis of a syndrome of P. of and its forms is often impossible without special methods of a research, from to-rykh the most informative rentgenoangio-graphical methods are (see. Kavografiya , Mezenterikografiya , Portografiya , Splenoportografiya , Tseliakografiya ), ezofagoskopiya (see), gastroscopy (see), a splenomanometriya (see. Spleen ), etc.

Apply a transdermal sple-nomanometriya to measurement of portal pressure. The technique is carried out by a puncture of a spleen and accession to a needle of the device of Valdman for measurement of venous pressure. At P. pressure in a spleen reflecting pressure in portal system reaches on average 350 — 450 mm w.g. (it is normal of 50 — 115 mm w.g.).

Fig. 4. Splenoportogramma at intra hepatic portal hypertensia: sharply expanded splenoportalny bed with the functioning umbilical vein; 1 — a portal vein, 2 — a splenic vein, 3 — an expanded coronary vein of a stomach, 4 — an umbilical vein.
Fig. 5. Splenoportogramma at extrahepatic portal hypertensia: lack of the main trunk of a splenic vein with a set of again formed vessels (1); retrogradno the filled expanded veins of a stomach and a gullet (2); stenosis of intra hepatic branches of a portal vein and its deformation (3).
Fig. 6. Retrograde mezenterikoporto-gram at impassability of the main vessels of portal system: a conglomerate of expanded vessels in the field of portal fissures, a spleen and a stomach (it is specified by shooters).

About the level of blockade of portal blood circulation and a condition of vessels the splenoportografiya gives the greatest informational content. At intra hepatic P. the splenoportalny bed is passable, expanded with sharp depletion of the vascular drawing of a liver, expanded veins of a stomach and a gullet, an expanded umbilical vein (fig. 4) sometimes come to light. At extrahepatic P. the various picture depending on the level of blockade (fig. 5) is noted, up to full impassability of a splenoportalny bed. In this case apply a returnable mezenterikoportografiya (fig. 6) to establishment of level of blockade. At suprahepatic P. to the greatest information is given by a kavografiya and a puncture biopsy of a liver. Expanded veins of a gullet and a stomach, as a rule, come to light at their X-ray or endoscopic inspection.

Differential diagnosis between various forms P. of and other diseases which are followed by increase in a spleen quite often presents great difficulties. The anamnesis is of great importance. The instruction in the anamnesis on the postponed hepatitis, including virus, hron, shall suggest alcoholism an idea of intra hepatic P. of. The instruction on increase in a spleen since the birth or early children's age, umbilical sepsis, an injury, suppurative processes in an abdominal cavity gives the grounds to suspect, extrahepatic P. The acute beginning with high temperature, pains in right hypochondrium, bystry increase in a liver and emergence of ascites is characteristic of suprahepatic P. (Kiari's disease).

Difficulties are presented by the differential diagnosis of the extrahepatic P. of proceeding without varicosity of a gullet with an initial stage of tumoral diseases of a spleen (a lymphogranulomatosis, a reticulosarcoma, a lymphoma). The diagnosis is established on the basis of more bystry and a severe disease with generalization of process at tumors of a spleen, data of a splenomanometriya, splenoportografiya.


At suprahepatic and intra hepatic P. in the period of activity of inflammatory process in a liver treatment only conservative (see. Kiari disease , Cirrhoses of a liver ). At the arisen bleeding from veins of a gullet treatment can also be conservative, directed to a stop of bleeding and compensation of blood loss in the beginning. Conservative treatment at the same time can be preparation for the emergency operative measure.

Fig. 7. The diagrammatic representation of a stop of venous bleeding from expanded veins of a gullet and a stomach by means of Bleykmor's probe: and — position of the probe in a gullet and a stomach of the patient — Bleykmor's probe; 1 — an esophageal cylinder, 2 — a gastric cylinder.

The most efficient method of conservative treatment is the prelum of veins of a gullet Bleykmor's (fig. 7) probe. The last has two pneumatic cylinders (esophageal and gastric) and three gleams. One gleam is intended for aspiration of gastric contents, and two others — for inflating of gastric and esophageal cylinders. The probe is entered through the nasal course into a stomach and through the tube which is built in in a wall of the probe, in a gastric cylinder force 50 — 70 cm 3 air also tighten the probe to feeling of an emphasis in the field of the cardia of a stomach. Fix the probe to an upper lip an adhesive plaster. Then inflate an esophageal cylinder, entering from 80 to 150 cm into its gleam 3 air. Wash out a stomach. If on the probe fresh blood, then, obviously continues to arrive, a bleeding point are the veins of a stomach which did not undergo a prelum. In this case it is necessary to dismiss cylinders and the probe to remove. At the stopped bleeding the probe is left in a gullet at several o'clock, periodically dismissing cuffs and checking whether bleeding renewed. Bleeding from a gullet and the cardia sometimes manages to be stopped after administration of sclerosing drugs (e.g., Varicocidum) in a gleam varicose of expanded veins through ezofagogastroskop.

Operational treatment of P. of can be made as in the emergency, and planned order. The indication to the emergency operative measure is massive bleeding from varicose expanded veins of a gullet or the cardia, and also a possibility of its recurrence in the next few hours after a stop. Indications to an operative measure in a planned order are existence of a varicosity of a gullet and the cardia, and also a splenomegaly and ascites. At extrahepatic P. treatment generally operational. At the same time indications to it are limited by a recurrence of tromboflebitichesky process in portal system or associated diseases.

Need for conservative treatment arises at posthemorrhagic anemia, a recurrence of a phlebothrombosis of portal system. Hemotransfusion, vitamin therapy, use of anticoagulants, antiinflammatory drugs is effective.

At suprahepatic and intra hepatic P. in many respects are defined of the indication to operation by a condition of the patient and features patol, process in a liver. The most optimum moment for carrying out operation is lack of an active form of hepatitis and compensation of a functional condition of a liver. Operation is contraindicated in a stage of a decompensation, and also in the presence of active hepatitis. From veins of a gullet and the cardia apply to a final stop of bleeding generally the operations directed to the termination of intake of blood in veins of a gullet from portal system. Apply Tanner's operation, an essence the cut consists in full cross crossing of a stomach in a cardial part with its subsequent sewing together. At this operation full dissociation of veins of portal system and an upper vena cava is reached. At its correct performance the direct effect can be reached. M. A. Topchibashev (1950) offered a circular section in cardial department of a stomach without opening of a mucous membrane and underrunning of vessels of a submucosal layer, without getting into a gleam of a stomach.

B. A. Petrov and E. N. Galperin (1963) suggested to cut only a front wall of a stomach, and back to stitch through a mucous membrane. M. D. Patsiora (1959) applied a gastrotomy in a cardial part with underrunning of veins of small curvature of a stomach and a belly part of a gullet through the mucous membrane covering them.

Use of more difficult surgeries at bleeding from veins of a gullet is not justified because of high risk and big percent of a lethality.

Complications in the postoperative period in many respects depend on a reference functional state of a liver and degree of posthemorrhagic anemia. The most frequent complications: liver failure, eventration, ascites, insufficiency of seams of a gullet or stomach.

In the remote period after the operations directed to a stop of bleeding from veins of a gullet a recurrence of bleeding is observed. Therefore the operation executed in emergency situation shall be regarded as the first stage of operational treatment.

The operational treatment of P. of made in a planned order is directed generally to pressure decrease in portal system, improvement of a functional condition of a liver.

The applied methods of operations can conditionally be divided into the following groups: 1) creation of new outflow tracts of blood from portal system (an omentopexy, organopek-this, a porto-caval anastomosis); 2) reduction of inflow of blood to portal system (splenectomy, bandaging of branches of a celiac artery); 3) the termination of communication of veins of a stomach and gullet with veins of portal system (a gastrokavalny anastomosis, a resection of proximal department of a stomach and gullet, Tanner's operation, a gastrotomy with underrunning of veins of a stomach and gullet, thrombosing of veins of a stomach and gullet); 4) the operations directed to strengthening of processes of regeneration of a liver and intra hepatic arterial circulation (a resection of a liver, neurotomy of a hepatic artery).

The most difficult is the choice of a method of operation at cirrhosis and an extrahepatic portal gi-pertekniya. If in the first case the choice of a method of operation limits the broken functional condition of a liver, then in the second — prevalence and extent of damage of veins of portal system.

At suprahepatic P. the operations directed to creation of new outflow tracts of blood from a liver in system of an upper vena cava are preferable (a gepatopnevmopeksiya, a rekanalization of the lower vena cava, a kavopredserdny anastomosis — B. V. Petrovsky).

At intra hepatic P. with the expressed phenomena of a hypersplenism it is shown splenectomy (see) in combination with a resection liver (see) or coagulation of its surface. Existence varicose of expanded veins of a gullet dictates need of use of a vascular porto-caval anastomosis (see), preferably the selection (gastrokavalny, distal splenorenalny) or a splenectomy in combination with a gastrotomy and underrunning of veins of a gullet and the cardia of a stomach.

At extrahepatic P. with existence varicose of expanded veins of a gullet by method of the choice is imposing of a splenorenalny anastomosis in this or that option depending on a condition of vessels of portal system. At impassability of portal system creation of organoanastomoz in combination with bandaging of veins of a gullet and stomach or a splenectomy with bandaging of veins of a gullet and stomach is shown.

The forecast

the Current of a syndrome of P. of is caused by a basic disease. At intra hepatic and suprahepatic forms activation patol, process in a liver increases possibility of bleeding from veins of a gullet, a cut, in turn, promotes progressing of process in a liver, the hepatonephric insufficiency leading to a lethal outcome develops. The disease can be complicated by ascites peritonitis, a hydrothorax, thrombosis of a portal vein, a diabetes mellitus, primary cancer of a liver.

Extrahepatic P.'s current of high-quality, not reducing working capacity if the disease proceeds without complications. With developing of bleedings from veins of a gullet the forecast of a disease sharply worsens. From complications the heart attack of a spleen, mechanical jaundice as a result of a cicatricial stenosis of the extrahepatic bilious courses, stomach ulcer and a duodenum are possible.

Features of portal hypertensia at children

P.'s Syndrome of at children's age differs in a number of features. At children the extrahepatic form P. of prevails. Most often anomalies of development of a portal vein or fibrinferments of vessels of portal system, developing soon after the birth owing to a pylephlebitis are its reason. Pylephlebitis (see) at newborns results from spread of an infection on umbilical and to paraumbilical veins from the inflamed umbilical wound. In rare instances at intestinal diseases migrating of an infection from mezenterialny vessels is possible.

Are the main reason for an intra hepatic form P. of at children cirrhosis and fibrokholangiokis-toz (inborn fibrosis of a liver).

At extrahepatic blockade of a portal blood-groove find considerable changes of a liver in the form of its underdevelopment, diffusion fibrosis and dystrophy in 1/3 patients. Insufficient inflow to a liver of blood from the portal system bearing plastic materials, and reorganization of metabolism of body, necessary for growth, is the cornerstone of these changes. It is expressed in falloff of consumption to cookies of oxygen and dominance of extremely unprofitable anaerobic type of breath in the power relation. At extrahepatic blockade of bleeding from varicose expanded veins arise often. At cirrhosis sub - and the decompensation of portal blood circulation is observed preferential at children of advanced age. Bleedings from varicose expanded veins develop seldom.

At children bleeding from veins of a gullet is observed seldom, it, as a rule, arises from varicose expanded veins of a cardial part of a stomach.

Operational treatment of P. of at children is accompanied by a number of additional difficulties (small diameter of vessels, quite often total absence of the normal anatomic formations of portal system suitable for anastamosing) that considerably limits possibilities of vascular surgery. Therefore in pediatric practice the specific weight of palliative operations is big. According to most of children's surgeons, the hope for functioning of an artificial porto-caval anastomosis is real with a diameter of sewed vessels not less than 7 — 8 mm, i.e. at children of advanced age. At an extrahepatic form P. of and a fibrokholangiokistoza preference is given splenorenalny and-vomezenterialnomu to an anastomosis.

Palliative interventions set as the purpose the prevention of bleedings in the conditions of the remaining P. These objectives can be achieved by means of endovascular an embolus isocies (see. X-ray endovascular surgery ) or an endoscopic sklerozirovaniye varicose expanded veins of a stomach and a gullet, and also direct operative measures on veins of a gastroesophageal zone. The most effective operation is the gastrotomy with underrunning and bandaging of veins of a cardial part of a stomach and a belly part of a gullet from a mucous membrane. Direct interventions on veins provide remissions with duration up to 45 years and keep a possibility of the radical shunting operation in the future.

Bibliography: Akopyan V. G. Surgical hepathology of children's age, M., 1982; Caravans G. G. and Pavlovsky M. P. Cirrhoses of a liver and their surgical treatment, Kiev, 1966; Mansurov of X. X. Portal hypertensia, Dushanbe, 1963; Ying V. V. Steam of and Meerson F. 3. Sketches of clinical physiology of blood circulation, M., 1965; Patsiora M. D. Surgery of portal hypertensia, M., 1974, bibliogr.; Petrovsky B. V. Pi Zarets-k and y V. V. O to differential diagnosis of mitral heart diseases, Vestn, hir., t. 81, No. I; At of l about in F. G. and Koryakin T. O. Surgical treatment of portal hypertensia, L., 1964, bibliogr.; In iirge of To., Wagner K. and. Zimmermann H. - Century of Diagnostik und Therapie des Pfortaderhochdrucks, B., 1975; B u r 1 u i D. The surgery of portal hypertension, Bucure§ti, 1980, bibliogr.; Child C. G. The hepatic circulation and portal hypertension, Philadelphia — L., 1954, bibliogr.; Popper H. u. Schaff-n e r F. Die Leber, Struktur und Funktion, Stuttgart, 1961; Soderlund S. Portal hypertension in children, Nord. Med., v. 64, p. 1068, 1960; Tanner N. C. Gastroduodenal bleeding as a surgical emergency, Proc. roy. Soc. Med., v. 43, p. 147, 1950; Walker R. M. Die portale Hypertension, Stuttgart, 1960.

M. D. Patsiora; S. A. Seleznyov (stalemate. physical.), V. G. Akopyan (it is put. hir.).