Vascular bypass surgery in the treatment of extrahepatic portal hypertension in children (review)



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Abstract

In children with extrahepatic portal hypertension (HSV), the most frequent and life-threatening complication is bleeding from varicose veins of the esophagus and stomach. Therefore, the main task of HSV treatment is to prevent bleeding from the veins of the upper gastrointestinal tract. The most effective treatment is portosystem bypass surgery (PSS), the effectiveness of which reaches 94-97%. However, the use of these operations is limited precisely because of a decrease in portal liver perfusion (PPP) and the development of portosystemic encephalopathy (PSE).

The aim is to analyze domestic and foreign publications devoted to the surgical treatment of portal hypertension and the development of portosystemic encephalopathy in the postoperative period.

A systematic search was carried out in the databases PubMed, Web of Science, Scopus, MEDLINE, eLibrary, RSCI, Cyberleninka. 345 references were analyzed, 110 articles were reviewed, 97 publications on surgical treatment of portal hypertension were selected for the review.

The development of PSE in the postoperative period often occurs after the imposition of total PSS. To reduce the risk of developing PSE, selective shunts have been developed, which to some extent preserve the PPP. With distal splenorenal anastomosis (DSRA), the frequency of PE is less than 10-15%. An intermediate position is occupied by a side-to-side splenorenal anastomosis, which has signs of selective surgery.

The results of the analysis of literature sources showed that discussions are still underway regarding the choice of the optimal treatment strategy for patients with HSV, the place and role of endoscopic methods for the prevention of gastrointestinal bleeding, as well as mesoportal shunt (MPSH) in the treatment of patients with extrahepatic PG.

Nevertheless, the majority of world experts consider MPSH to be the most optimal operation for primary and secondary prevention of varicose bleeding and other complications of HSV. And if it is impossible to perform it, the operation of selective bypass surgery of the DSRA can serve as an alternative to performing MPSH.

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THE MAIN TEXT OF THE ARTICLE
Extrahepatic portal hypertension (HSV) is one of the most frequent and dangerous causes of acute bleeding from the upper gastrointestinal tract (gastrointestinal tract). Portal hypertension syndrome (PG) unites a group of diseases characterized by increased pressure in the portal vein system (BB).
The most common form of PG in children is considered extrahepatic, caused by blockage of blood flow along the BB trunk and its branches. According to various sources, the frequency of extrahepatic form among all cases of PG in children ranges from 60-75% [1-3].
The etiology of HSV is diverse. Several factors predisposing to the development of PG have been described [4]. They are divided into three categories: local factors that can cause damage to the portal vein (catheterization of umbilical vessels in the newborn period, infectious processes in the abdominal cavity, iatrogenic injuries), general factors (procoagulant status) and, less often, vascular malformations. The most common cause is catheterization of the umbilical vein in the newborn period. This procedure is performed, according to various authors, in 20-60% of newborns when intensive therapy is necessary [5]. According to DiGiogio and DeAngelis, the lower the frequency of umbilical vein catheterization, the higher the technological level of medical care [2]. Umbilical sepsis, omphalitis, peritonitis, and even osteomyelitis are noted among the infectious processes of the newborn period that can cause BB thrombosis. In recent years, a relatively large number of publications have been devoted to the role of congenital deficiency or qualitative abnormalities of anticoagulant factors (antithrombin III, protein C, protein S and resistance to activated protein C) in the genesis of HSV [6, 7]. For example, in the Grama study, thrombophilia is diagnosed in 35% of cases in children with HSV [6]. For this reason, children with HSV, especially those with combined abnormalities and diseases, should be examined for the presence of hereditary prothrombotic disorders: prothrombin 20210 mutation (PTHR), factor V Leiden (FVL), methylenetetrahydrofolate reductase (MTHFR) gene deficiency or metabolic defects such as hyperhomocysteinemia. Congenital anomalies (BB stenosis, atresia or agenesis) are defined as the cause of HSV in 14% of patients [8-10].
The pathogenesis of HSV is complex and is largely due to systemic reactions of the body. BB occlusion induces a number of pathophysiological processes leading to the emergence of hyperdynamic type of blood circulation and complications of PG. An important sign of HSV is the formation of esophageal varicose veins, through which the collateral outflow of blood from the portal system is carried out, and "portal cavernoma" - a network of hepatopetal collaterals, through which portal blood tends to enter the liver bypassing the impassable section of the BB [11, 12, 13]. There is a triad of classic symptoms of HSV: bleeding from varicose veins of the esophagus and stomach, splenomegaly, ascites. In 50-80% of children, the manifesting symptom is gastrointestinal bleeding from varicose veins of the esophagus and stomach [14, 15]. Splenomegaly and hypersplenism are found with approximately the same frequency [5, 14]. In 70% of children, bleeding from the upper gastrointestinal tract occurs before the age of 10 years [16-18]. However, the cumulative frequency of varicose bleeding continues to increase in adolescence; and as a rule, they are repeated about once a year in children who have not received preventive treatment [19, 20].
Portal hypertension gastropathy is usually present in children with HSV, but, unlike adult patients, it is rarely a source of significant bleeding in children [21]. Other complications/symptoms for primary portal obstruction are quite rare. These include minimal hepatic encephalopathy, portal biliopathy, focal nodular hyperplasia of the liver, ascites, lagging children in physical development [15]. Portal biliopathy is the result of an external compression mechanism of portal cavernoma of extrahepatic and intrahepatic bile ducts, ischemia and prolonged PG with the development of collaterals in the biliary tract. It is more common in adult patients (80-100% of cases) and much less common in children with HSV (4% of cases) [3, 22, 23]. Hepatopulmonary syndrome is a rare but well-known complication of HSV. In the BorkarV study. hepatopulmonary syndrome was detected in 13% (p=0.001) of patients with HSV [24].
On the other hand, in our observations [5] in more than 700 patients with HSV, hepatopulmonary syndrome was found in only 2 children. In the study of K.A. Djuma, it was revealed that in most children with HSV there is tension of the humoral link of immunity (IdM, IdA) and phagocytosis, secondary cellular immunodeficiency with a decrease in the total number of leukocytes, lymphocytes and lymphocyte subpopulations (CD3+, CD4+, CD8+) [25].
Nevertheless, an important difference between HSV and other forms of PG is the preservation of liver functions. Violations of the function of the hepatic parenchyma, characteristic of cirrhosis of the liver, are extremely rare when blood flow is blocked by the BB [5, 16, 17, 26].
The main task of HSV treatment is to prevent bleeding from the veins of the upper gastrointestinal tract. For this purpose, all surgical treatment options are used: endoscopic sclerosing and ligation, devascularization operations, PSS [16, 18, 19, 27]. Also, some authors separately distinguish a group of palliative operations aimed at temporarily stopping acute bleeding from varicose veins of the esophagus and stomach. This group includes gastrotomy with stitching of varicose veins and splenectomy [5].
PSH remained the most effective in the treatment of children with HSV for a long time, since it is aimed directly at eliminating the cause of varicose veins of the esophagus and stomach - high pressure in the BB system [26, 28, 29, 30]. PSS are vascular operations that create blood outflow from the portal system with high pressure to the system of the inferior vena cava with low.
The first side-to-side portocaval anastomosis was performed by Rosenstein in 1912 [31]. Later, other variants of vascular anastomoses for the treatment of PG were proposed. A large number of studies on PSS have clearly shown that these operations relieve the patient from the threat of bleeding in the vast majority of cases. The efficiency of portosystem bypass surgery reaches 94-97% [32-34]. However, the use of these operations is limited precisely because of the decrease in portal pressure and, as a consequence, the IFR. Pavlov first studied this phenomenon in 1893 [5]. He described that total deprivation of the liver of portal perfusion leads to hepatic degeneration and "meat intoxication", i.e. PSE. This negative moment is of the greatest importance for patients with cirrhosis of the liver (CP), with varying degrees of liver failure [35]. It is believed that the type of shunting operation and the diameter of the shunt allow you to adjust the degree of decompression of the portal system, i.e. reduction of portal pressure. Studies of portal hemodynamics in the postoperative period allow us to justify the choice of the optimal treatment method [5, 36]. It was precisely to limit the reduction of the SPP after bypass surgery that research was directed to find new techniques – selective bypass and shunts of limited diameter [28, 29, 36-38]. The selectivity of the shunt is assessed by its effect on the SPP. Total bypass surgery leads to the fact that almost the entire volume of blood from the BB system is discharged through the newly created anastomosis into the systemic bloodstream bypassing the liver. Selective shunts maintain to some extent the blood flow from mesenteric veins to the liver, maintaining the PPP. Total bypass surgery can include portocaval, mesocaval, cavamesenteric anastomoses, proximal (central) splenorenal anastomosis. It should be noted that the end-to-side portocaval anastomosis described by Eck in 1877, which leads to total decompression of the portal system, is not used in the treatment of PG in children. Selective shunts include anastomoses in the splenorenal zone: for example, distal splenorenal shunt [38]. The intermediate position is occupied by splenorenal anastomoses side-to-side and "small diameter" shunts. Operations to create "small diameter" shunts involve the creation of artificial portosystem anastomoses of limited diameter to limit the discharge of blood from the portal system [33, 36, 37, 39].

 

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About the authors

Zorikto Mitupov

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Детская городская клиническая больница им. Н.Ф. Филатова

Author for correspondence.
Email: zmitupov@mail.ru
ORCID iD: 0000-0002-0016-6444
SPIN-code: 5182-1748
Russian Federation, Москва

Aleksander Razumovsky

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Детская городская клиническая больница им. Н.Ф. Филатова

Email: 1595105@mail.ru
ORCID iD: 0000-0003-3511-0456
SPIN-code: 3600-4701
Russian Federation, Москва

Viktor Rachkov

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Клинический госпиталь «Лапино-1»

Email: vrachcov@mail.ru
ORCID iD: 0000-0002-1304-0592
SPIN-code: 9371-5492
Russian Federation, Москва

Nadezhda Kulikova

Детская городская клиническая больница им. Н.Ф. Филатова

Email: dr.kulikovan.v@gmail.com
ORCID iD: 0000-0003-0834-2630
SPIN-code: 4687-1558
Russian Federation, Москва

Sergey Margaryan

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова

Email: sergey.margaryan27@mail.ru
ORCID iD: 0000-0002-9968-2931
Russian Federation, Москва

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