Abstract

Extrahepatic portal vein thrombosis is the second most frequent cause of portal hypertension after liver cirrhosis. Risk factors of the thrombosis are divided into systemic: hereditary and acquired thrombophilia (60–70%); and local: abdominal inflammation, trauma, surgery, etc. (20–40%). Acute thrombosis episode is often olygo- or asymptomatic and remains undiagnosed until the development of portal hypertension and its complications: gastroesophageal variceal bleeding, portal biliopathy, etc. Early onset of anticoagulation is recommended and should be prescribed at least for 3 months. In patients with portal cavernoma a long-term (even lifelong) anticoagulation is directed to prevent recurrence and extension of thrombosis, especially in case of thrombophilia. Portocaval shunting is the most radical way to correct portal hypertension and to deliver a patient from variceal bleedings and other complications of the disease. Hepatic encephalopathy is a rare condition and generally subclinical. Endoscopic band ligation and sclerotherapy are effective in acute variceal bleeding, primary and secondary prophylaxis of bleeding and should be supplied by non-selective β-adrenoblockers administration. Patients with extrahepatic portal vein thrombosis are well tolerated to variceal bleeding episodes and have high rates of survival during long-term follow-up due to normal liver function. Mortality in these patients is low (5-year mortality is less than 10%) and generally is associated with comorbidities rather complications of portal hypertension

Highlights

  • Внепеченочный тромбоз воротной вены – вторая по частоте причина портальной гипертензии после цирроза печени

  • Patients with extrahepatic portal vein thrombosis are well tolerated to variceal bleeding episodes and have high rates of survival during long-term follow-up due to normal liver function. Mortality in these patients is low (5-year mortality is less than 10%) and generally is associated with comorbidities rather complications of portal hypertension

  • внепеченочного тромбоза ВВ (ВТВВ) – это обструкция внепеченочной части воротной вены (ВВ), ее долевых ветвей и притоков, характеризуется развитием симптомов острого тромбоза или синдрома портальной гипертензии (ПГ) и кавернозной трансформацией ВВ

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Summary

ВНЕПЕЧЕНОЧНАЯ ПОРТАЛЬНАЯ ГИПЕРТЕНЗИЯ И ТРОМБОЗ ВОРОТНОЙ ВЕНЫ

Внепеченочный тромбоз воротной вены – вторая по частоте причина портальной гипертензии после цирроза печени. Проявления острого тромбоза, как правило, малосимптомны, и он остается недиагностированным до развития осложнений портальной гипертензии: варикозных кровотечений, портальной билиопатии и пр. Ключевые слова: тромбоз воротной вены, портальная кавернома, внепеченочная портальная гипертензия, тромбофилия, миелопролиферативные заболевания, варикозное расширение вен, антикоагулянтная терапия, портокавальное шунтирование, эндоскопическое лигирование, склеротерапия, портальная билиопатия. Patients with extrahepatic portal vein thrombosis are well tolerated to variceal bleeding episodes and have high rates of survival during long-term follow-up due to normal liver function. Mortality in these patients is low (5-year mortality is less than 10%) and generally is associated with comorbidities rather complications of portal hypertension. ВТВВ выступает причиной до 30% кровотечений из варикозных вен пищевода и желудка [1, 6,7,8, 12,13,14], а также является главной причиной ПГ и портальных кровотечений у детей (68–84%) [18,19,20,21,22]

Этиология и патогенез
Фактор риска
Патологическая анатомия и гемодинамика
Клиническая картина и классификация
Findings
Инструментальная и лабораторная диагностика
Full Text
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