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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care</journal-title><trans-title-group xml:lang="ru"><trans-title>Российский вестник детской хирургии, анестезиологии и реаниматологии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2219-4061</issn><issn publication-format="electronic">2587-6554</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">1295</article-id><article-id pub-id-type="doi">10.17816/psaic1295</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Case reports</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Клинические случаи</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Congenital portosystemic shunts: surgical treatment experience</article-title><trans-title-group xml:lang="ru"><trans-title>Врожденные портосистемные шунты: опыт хирургического лечения</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6181-7036</contrib-id><name-alternatives><name xml:lang="en"><surname>Stepanov</surname><given-names>Alexey E.</given-names></name><name xml:lang="ru"><surname>Степанов</surname><given-names>Алексей Эдуардович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. Sci. (Med.), Doctor of the Department of Reconstructive and reconstructive Surgery of the abdominal organs</p></bio><bio xml:lang="ru"><p>канд. мед. наук, врач отделения реконструктивной и восстановительной хирургии органов брюшной полости</p></bio><email>stepanov_alexey63@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6972-9017</contrib-id><contrib-id contrib-id-type="spin">2363-1150</contrib-id><name-alternatives><name xml:lang="en"><surname>Sukhov</surname><given-names>Maksim N.</given-names></name><name xml:lang="ru"><surname>Сухов</surname><given-names>Максим Николаевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.), Deputy Chief Physician for Surgery</p></bio><bio xml:lang="ru"><p>д-р мед. наук, заместитель главного врача по хирургии</p></bio><email>sukhov79mn@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5106-1215</contrib-id><name-alternatives><name xml:lang="en"><surname>Vasilyev</surname><given-names>Kirill G.</given-names></name><name xml:lang="ru"><surname>Васильев</surname><given-names>Кирилл Германович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Sci. (Med.), Associate Professor of the Department of Pediatric Surgery of the Faculty of Pediatrics</p></bio><bio xml:lang="ru"><p>канд. мед. наук, доцент кафедры детской хирургии педиатрического факультета</p></bio><email>kiravasilyev@yandex.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9554-6414</contrib-id><contrib-id contrib-id-type="spin">7587-9843</contrib-id><name-alternatives><name xml:lang="en"><surname>Polyaev</surname><given-names>Yuri A.</given-names></name><name xml:lang="ru"><surname>Поляев</surname><given-names>Юрий Александрович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor, Head of the Department of Endovascular Surgery</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор, заведующий отделением эндоваскулярной хирургии</p></bio><email>polyaev@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5287-7889</contrib-id><contrib-id contrib-id-type="spin">7590-2400</contrib-id><name-alternatives><name xml:lang="en"><surname>Garbuzov</surname><given-names>Roman V.</given-names></name><name xml:lang="ru"><surname>Гарбузов</surname><given-names>Роман Вячеславович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.), Doctor of the Department of Endovascular Surgery</p></bio><bio xml:lang="ru"><p>д-р мед. наук, врач отделения эндоваскулярной хирургии</p></bio><email>9369025@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0278-8551</contrib-id><name-alternatives><name xml:lang="en"><surname>Golenishchev</surname><given-names>Anton I.</given-names></name><name xml:lang="ru"><surname>Голенищев</surname><given-names>Антон Игоревич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Doctor of the Department of Endovascular surgery</p></bio><bio xml:lang="ru"><p>врач отделения эндоваскулярной хирургии</p></bio><email>a331821@ya.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5106-8852</contrib-id><name-alternatives><name xml:lang="en"><surname>Ashmanov</surname><given-names>Konstantin Yu.</given-names></name><name xml:lang="ru"><surname>Ашманов</surname><given-names>Константин Юрьевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Doctor of the Department of reconstructive Surgery of the abdominal organs</p></bio><bio xml:lang="ru"><p>врач отделения реконструктивной хирургии органов брюшной полости</p></bio><email>ashmanov1964@icloud.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8404-3715</contrib-id><name-alternatives><name xml:lang="en"><surname>Lyvina</surname><given-names>Irma P.</given-names></name><name xml:lang="ru"><surname>Лывина</surname><given-names>Ирма Петровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Doctor of the microvascular surgery Department No. 2</p></bio><bio xml:lang="ru"><p>врач отделения микрососудистой хирургии № 2</p></bio><email>irma_irma@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1502-8121</contrib-id><name-alternatives><name xml:lang="en"><surname>Demushkina</surname><given-names>Alice A.</given-names></name><name xml:lang="ru"><surname>Демушкина</surname><given-names>Алиса Анатольевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. Sci. (Med.), Doctor of the Department of Radiology</p></bio><bio xml:lang="ru"><p>канд. мед. наук, врач отделения лучевой диагностики</p></bio><email>demushkina.alya@bk.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7224-5777</contrib-id><contrib-id contrib-id-type="spin">3502-5812</contrib-id><name-alternatives><name xml:lang="en"><surname>Tereshina</surname><given-names>Anastasia A.</given-names></name><name xml:lang="ru"><surname>Терешина</surname><given-names>Анастасия Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Student of the Pediatric Faculty</p></bio><bio xml:lang="ru"><p>студентка педиатрического факультета</p></bio><email>dr.tereshina@mail.ru</email><xref ref-type="aff" rid="aff2"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University</institution></aff><aff><institution xml:lang="ru">Российская детская клиническая больница Российского национального исследовательского медицинского университета им. Н.И. Пирогова</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Pirogov Russian National Research Medical University</institution></aff><aff><institution xml:lang="ru">Российский национальный исследовательский медицинский университет им. Н.И. Пирогова</institution></aff></aff-alternatives><pub-date date-type="preprint" iso-8601-date="2022-12-23" publication-format="electronic"><day>23</day><month>12</month><year>2022</year></pub-date><pub-date date-type="pub" iso-8601-date="2023-01-19" publication-format="electronic"><day>19</day><month>01</month><year>2023</year></pub-date><volume>12</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>473</fpage><lpage>487</lpage><history><date date-type="received" iso-8601-date="2022-10-06"><day>06</day><month>10</month><year>2022</year></date><date date-type="accepted" iso-8601-date="2022-12-22"><day>22</day><month>12</month><year>2022</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2023, Eco-Vector</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2023, Эко-Вектор</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="en">Eco-Vector</copyright-holder><copyright-holder xml:lang="ru">Эко-Вектор</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by-nc-nd/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://rps-journal.ru/jour/article/view/1295">https://rps-journal.ru/jour/article/view/1295</self-uri><abstract xml:lang="en"><p>Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective.</p> <p>The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed — open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant.</p> <p>Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.</p></abstract><trans-abstract xml:lang="ru"><p>Врожденные портокавальные шунты встречаются редко и могут иметь различную морфологическую структуру (внутри- и внепеченочные шунты, с наличием или отсутствием портального кровотока). Основной метод лечения пациентов с данной патологией — эндоваскулярная окклюзия шунта. Однако в некоторых случаях подобный метод оказывается неэффективным.</p> <p>В статье содержится описание шести клинических примеров хирургического лечения врожденных порто-системных шунтов у детей. В диагностике врожденных порто-системных шунтов ведущая роль принадлежит ультразвуковому исследованию с допплерографией, мультиспиральной компьютерной томографии, ангиографии. Показанием к оперативному лечению стали анатомические особенности шунта, делающие эндоваскулярную окклюзию технически невозможной. В одном наблюдении диагностирован широкий аранциев проток, была выполнена его открытая перевязка. В другом случае воротная вена впадала непосредственно в аневризматическое расширение, выполнена реконструктивно-пластическая операция на сосудах воротной вены. В следующем наблюдении определялся выраженный ретроградный кровоток по расширенной нижней брыжеечной вене, сброс крови через сакральное сплетение во внутреннюю подвздошную вену. Произведено выделение левой внутренней подвздошной вены и ее перевязка, дисплазированная нижняя брыжеечная вена перевязана и частично удалена. У двух пациентов воротная вена впадала непосредственно в нижнюю полую вену в области аневризматического расширения, проведена операция — открытая перевязка шунта. В одном наблюдении диагностирована глубокая гипоплазия внутрипеченочных ветвей воротной вены, в связи с чем восстановление портального кровотока после закрытия шунта невозможно. Ребенок был направлен для решения вопроса о трансплантации печени.</p> <p>Каждый случай врожденных портокавальных шунтов уникален. Хирург определяет тактику непосредственно в ходе операции в зависимости от морфологического строения органов, поскольку предоперационное обследование не всегда дает однозначное представление.</p></trans-abstract><kwd-group xml:lang="en"><kwd>case report</kwd><kwd>congenital portosystemic shunt</kwd><kwd>Abernathy malformation</kwd><kwd>surgical treatment</kwd><kwd>angiography</kwd><kwd>children</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>клинический случай</kwd><kwd>врожденный портосистемный шунт</kwd><kwd>мальформация Абернети</kwd><kwd>оперативное лечение</kwd><kwd>ангиография</kwd><kwd>дети</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">Papamichail M, Pizanias M, Heaton N. Congenital portosystemic venous shunt. European Journal of Pediatrics. 2017;177(3):285–294. 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