<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE root>
<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">Perm Medical Journal</journal-id><journal-title-group><journal-title xml:lang="en">Perm Medical Journal</journal-title><trans-title-group xml:lang="ru"><trans-title>Пермский медицинский журнал (сетевое издание "Perm medical journal")</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0136-1449</issn><issn publication-format="electronic">2687-1408</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">514879</article-id><article-id pub-id-type="doi">10.17816/pmj405158-166</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Clinical case</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Случай из практики</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">Creation of elbow joint in children with humeroradial synostosis. A clinical case</article-title><trans-title-group xml:lang="ru"><trans-title>Создание локтевого сустава у детей с плечелучевым синостозом: клинический случай</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8948-9225</contrib-id><contrib-id contrib-id-type="spin">1940-3837</contrib-id><name-alternatives><name xml:lang="en"><surname>Grankin</surname><given-names>D. Yu.</given-names></name><name xml:lang="ru"><surname>Гранкин</surname><given-names>Д. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Researcher, Department of Reconstructive Microsurgery and Hand Surgery</p></bio><bio xml:lang="ru"><p>научный сотрудник отделения реконструктивной микрохирургии и хирургии кисти</p></bio><email>grankin.md@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1319-8979</contrib-id><contrib-id contrib-id-type="spin">8360-8078</contrib-id><name-alternatives><name xml:lang="en"><surname>Golyana</surname><given-names>S. I.</given-names></name><name xml:lang="ru"><surname>Голяна</surname><given-names>С. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Candidate of Medical Sciences, Scientific Supervisor, Department of Reconstructive Microsurgery and Hand Surgery</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук, руководитель отделения реконструктивной микрохирургии и хирургии кисти</p></bio><email>ser.golyana@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7837-4676</contrib-id><contrib-id contrib-id-type="spin">6059-4464</contrib-id><name-alternatives><name xml:lang="en"><surname>Avdeychik</surname><given-names>N. V.</given-names></name><name xml:lang="ru"><surname>Авдейчик</surname><given-names>Н. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Orthopedic Surgeon</p></bio><bio xml:lang="ru"><p>травматолог-ортопед отделения реконструктивной микрохирургии и хирургии кисти</p></bio><email>natali_avdeichik@mail.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1923-7289</contrib-id><name-alternatives><name xml:lang="en"><surname>Safonov</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Сафонов</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Candidate of Medical Sciences, Head of the Department of Reconstructive Microsurgery and Hand Surgery</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук, заведующий отделением реконструктивной микрохирургии и хирургии кисти</p></bio><email>safo125@gmail.com</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3998-4954</contrib-id><contrib-id contrib-id-type="spin">9224-5488</contrib-id><name-alternatives><name xml:lang="en"><surname>Arakelyan</surname><given-names>A. I.</given-names></name><name xml:lang="ru"><surname>Аракелян</surname><given-names>А. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Candidate of Medical Sciences, researcher, Department of Consequences of Injuries and Rheumatoid Arthritis</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук, научный сотрудник отделения последствия травм и ревматоидного артрита</p></bio><email>a_bryanskaya@mail.ru</email><xref ref-type="aff" rid="aff2"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">H. Turner National Medical Research Center for Children’s Trauma Surgery and Orthopedics</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр детской травматологии и ортопедии имени Г.И. Турнера</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр детской травматологии и ортопедии им. Г.И. Турнера</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2023-10-18" publication-format="electronic"><day>18</day><month>10</month><year>2023</year></pub-date><volume>40</volume><issue>5</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>158</fpage><lpage>166</lpage><history><date date-type="received" iso-8601-date="2023-06-27"><day>27</day><month>06</month><year>2023</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/"/></permissions><self-uri xlink:href="https://permmedjournal.ru/PMJ/article/view/514879">https://permmedjournal.ru/PMJ/article/view/514879</self-uri><abstract xml:lang="en"><p>Humeroradial synostosis is a severe rare anomaly of the upper limb, leading to the development of disability. This congenital anomaly may be a separate nosological unit, or a part of a genetic syndrome. Multi-stage surgical treatment is correct for all components of the deformity. The most difficult stage of treatment is the creation of an elbow joint. There are few studies on this problem in the literature. The article presents the results of surgical treatment of two children with humeroradial synostosis, who had the elbow joint created using two techniques. Patient A. underwent microsurgical transplantation of 2–3 metatarsophalangeal joints into the area of the elbow joint that had been absent since birth. Patient B. underwent total arthroplasty of the elbow joint with an individual implant. The unsatisfactory result of the treatment of patient A. is associated with the impossibility of early development of movements in the elbow joint due to the presence of axial pins. The stable position of the endoprosthesis in patient B. made it possible to conduct a course of early development of movements and get a good long-term result. Due to small number of patients, it is impossible to reliably assess the advantage of one of the presented methods. Improvement of the results of treatment in children with humeroradial synostosis requires further research.</p></abstract><trans-abstract xml:lang="ru"><p>Плечелучевой синостоз является тяжелой, редкой аномалией развития всей верхней конечности, приводящей к развитию инвалидности. Данная аномалия развития может быть отдельной нозологической единицей или входить в состав генетического синдрома. Хирургическое лечение – многоэтапное и направлено на коррекцию всех компонентов деформации. Наиболее сложным этапом лечения является создание локтевого сустава. В доступной литературе имеются единичные исследования, посвященные данной проблеме.</p> <p>Представлены результаты хирургического лечения двух детей с плечелучевым синостозом, которым производилось создание локтевого сустава при помощи двух методик. Пациенту А. выполнена микрохирургическая пересадка блока 2–3-го плюснефаланговых суставов стопы в зону отсутствовавшего с рождения локтевого сустава. Пациенту Б. произведено тотальное эндопротезирование локтевого сустава индивидуальным имплантом. Неудовлетворительный результат лечения пациента А. связан с невозможностью ранней разработки движений в локтевом суставе ввиду наличия осевых спиц. Функционально-стабильное положение эндопротеза у пациента Б. позволило провести курс ранней разработки движений и получить хороший отдаленный результат.</p> <p>Ввиду небольшого количества пациентов невозможно достоверно судить о преимуществе одной из представленных методик. Улучшение результатов лечения детей с плечелучевым синостозом требует проведения дальнейших исследований.</p></trans-abstract><kwd-group xml:lang="en"><kwd>congenital anomalies of limb</kwd><kwd>humeroradial synostosis</kwd><kwd>microsurgery</kwd><kwd>elbow joint replacement</kwd><kwd>children</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>врожденные аномалии развития конечностей</kwd><kwd>плечелучевой синостоз</kwd><kwd>микрохирургия</kwd><kwd>эндопротезирование локтевого сустава</kwd><kwd>дети</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Maier W.A. Thalidomide embryopathy and limb defects; experiences in habilitation of children with ectromelias. Arch. Dis. Child. 1965; 40: 154–157.</mixed-citation></ref><ref id="B2"><label>2.</label><citation-alternatives><mixed-citation xml:lang="en">Shvedovchenko I.V., Min'kin A.V. Operativnoe lechenie i protezirovanie detej s vrozhdennym plecheluchevym sinostozom. Vestnik vserossijskoj gil'dii protezistov-ortopedov 2005; 2: 24–33 (in Russian).</mixed-citation><mixed-citation xml:lang="ru">Шведовченко И.В., Минькин А.В. Оперативное лечение и протезирование детей с врожденным плечелучевым синостозом. Вестник всероссийской гильдии протезистов-ортопедов 2005; 2: 24–33.</mixed-citation></citation-alternatives></ref><ref id="B3"><label>3.</label><citation-alternatives><mixed-citation xml:lang="en">McVeigh T.P., Soye J.A., Gordon E., Lynch S.A. Non-syndromic bilateral ulnar aplasia with humeroradial synostosis and oligo-ectrodactyly. American Journal of Medical Genetics Part A 2018; 176 (5): 1180–1183.</mixed-citation><mixed-citation xml:lang="ru">McVeigh T.P., Soye J.A., Gordon E., Lynch S.A. Non-syndromic bilateral ulnar aplasia with humeroradial synostosis and oligo-ectrodactyly. American Journal of Medical Genetics Part A. 2018; 176 (5): 1180–1183.</mixed-citation></citation-alternatives></ref><ref id="B4"><label>4.</label><citation-alternatives><mixed-citation xml:lang="en">Lee Y., Choi J.H., Oh A., Kim G.H., Park S.H., Moon J.E., Ko C.W., Cheon C.K., Yoo H.W. Clinical, endocrinological, and molecular features of four Korean cases of cytochrome P450 oxidoreductase deficiency. Ann Pediatr Endocrinol Metab. 2020 Jun; 25 (2): 97–103. DOI: 10.6065/apem.1938152.076.</mixed-citation><mixed-citation xml:lang="ru">Lee Y., Choi J.H., Oh A., Kim G.H., Park S.H., Moon J.E., Ko C.W., Cheon C.K., Yoo H.W. Clinical, endocrinological, and molecular features of four Korean cases of cytochrome P450 oxidoreductase deficiency. Ann Pediatr Endocrinol Metab. 2020; 25 (2): 97–103. DOI: 10.6065/apem.1938152.076.</mixed-citation></citation-alternatives></ref><ref id="B5"><label>5.</label><citation-alternatives><mixed-citation xml:lang="en">Morton J.E. V. Frentz S., Morgan T., Sutherland-Smith A.J., Robertson S.P. Biallelic mutations in CYP26B1: A differential diagnosis for Pfeiffer and Antley–Bixler syndromes. American Journal of Medical Genetics Part A 2016; 170 (10): 2706–2710.</mixed-citation><mixed-citation xml:lang="ru">Morton J.E. V., Frentz S., Morgan T., Sutherland-Smith A.J., Robertson S.P. Biallelic mutations in CYP26B1: A differential diagnosis for Pfeiffer and Antley–Bixler syndromes. American Journal of Medical Genetics Part A 2016; 170 (10): 2706–2710.</mixed-citation></citation-alternatives></ref><ref id="B6"><label>6.</label><citation-alternatives><mixed-citation xml:lang="en">Gençay I., Vargel I., Büyükkoçak U., Yazc I., Apan A. Anesthetic risks associated with Antley-Bixler syndrome. The Journal of Craniofacial Surgery 2013 Jan; 24 (1): e21-3. DOI: 10.1097/SCS.0b013e318267be0f. PMID: 23348324.</mixed-citation><mixed-citation xml:lang="ru">Gençay I., Vargel I., Büyükkoçak U., Yazc I., Apan A. Anesthetic risks associated with Antley-Bixler syndrome. The Journal of Craniofacial Surgery. 2013; 24 (1): e21-3. DOI: 10.1097/SCS.0b013e318267be0f.</mixed-citation></citation-alternatives></ref><ref id="B7"><label>7.</label><mixed-citation>Oldani E., Garel C., Bucourt M., Carbillon L. Prenatal diagnosis of Antley-Bixler syndrome and POR deficiency. The American Journal of Case Reports 2015; 16: 882.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Morton J.E. V., Frentz S., Morgan T., Sutherland-Smith A.J., Robertson S.P. Biallelic mutations in CYP26B1: A differential diagnosis for Pfeiffer and Antley–Bixler syndromes. American Journal of Medical Genetics Part A 2016; 170 (10): 2706–2710.</mixed-citation></ref><ref id="B9"><label>9.</label><citation-alternatives><mixed-citation xml:lang="en">Zavaruhin V.I. Embriogenez verhnej konechnosti: ot bugorka do slozhnejshego mekhanizma. Voprosy rekonstruktivnoj i plasticheskoj hirurgii 2018; 21 (4): 61–67 (in Russian).</mixed-citation><mixed-citation xml:lang="ru">Заварухин В.И. Эмбриогенез верхней конечности: от бугорка до сложнейшего механизма. Вопросы реконструктивной и пластической хирургии 2018; 21 (4): 61–67.</mixed-citation></citation-alternatives></ref><ref id="B10"><label>10.</label><mixed-citation>Goldfarb C.A., Ezaki M., Wall L.B., Lam W.L., Oberg K.C. The Oberg-Manske-Tonkin (OMT) classification of congenital upper extremities: update for 2020. The Journal of Hand Surgery 2020; 45 (6): 542–547.</mixed-citation></ref><ref id="B11"><label>11.</label><citation-alternatives><mixed-citation xml:lang="en">Zavaruhin V.I. Obshchie principy lecheniya vrozhdennyh anomalij razvitiya verhnej konechnosti. CHast' I. Voprosy rekonstruktivnoj i plasticheskoj hirurgii 2018; 4: 67 (in Russian).</mixed-citation><mixed-citation xml:lang="ru">Заварухин В.И. Общие принципы лечения врожденных аномалий развития верхней конечности. Часть I. Вопросы реконструктивной и пластической хирургии 2018; 4: 67.</mixed-citation></citation-alternatives></ref><ref id="B12"><label>12.</label><mixed-citation>Sénès F.M., Catena N. Correction of forearm deformities in congenital ulnar club hand: one-bone forearm. The Journal of hand surgery 2012; 37 (1): 159–164.</mixed-citation></ref><ref id="B13"><label>13.</label><citation-alternatives><mixed-citation xml:lang="en">Avdejchik N.V., Golyana S.I., Grankin D.YU., Safonov A.V., Tihonenko T.I., Galkina N.S.. Vozmozhnosti primeneniya mikrohirurgicheskoj autotransplantacii kompleksov tkanej u detej. Ortopediya, travmatologiya i vosstanovitel'naya hirurgiya detskogo vozrasta 2020; 8 (4): 437–450 (in Russian).</mixed-citation><mixed-citation xml:lang="ru">Авдейчик Н.В., Голяна С.И., Гранкин Д.Ю., Сафонов А.В., Тихоненко Т.И., Галкина Н.С.. Возможности применения микрохирургической аутотрансплантации комплексов тканей у детей. Ортопедия, травматология и восстановительная хирургия детского возраста 2020; 8 (4): 437–450.</mixed-citation></citation-alternatives></ref><ref id="B14"><label>14.</label><citation-alternatives><mixed-citation xml:lang="en">Rayan G.M., Upton III J., Rayan G.M., Up-ton III J. Elbow Synostosis. Congenital Hand Anomalies and Associated Syndromes 2014; 105–117.</mixed-citation><mixed-citation xml:lang="ru">Rayan G.M., Upton III, J., Rayan, G.M., Upton III, J. Elbow Synostosis. Congenital Hand Anomalies and Associated Syndromes 2014; 105–117.</mixed-citation></citation-alternatives></ref><ref id="B15"><label>15.</label><citation-alternatives><mixed-citation xml:lang="en">Golyana S.I., Avdejchik N.V., Grankin D. YU., Safonov A.V. Blizhajshie rezul'taty endoprotezirovaniya loktevogo sustava u detej s vrozhdennoj loktevoj kosorukost'yu. Sovremennye problemy nauki i obrazovaniya 2021; 4: 76–76 (in Russian).</mixed-citation><mixed-citation xml:lang="ru">Голяна С.И., Авдейчик Н.В., Гранкин Д.Ю., Сафонов А.В. Ближайшие результаты эндопротезирования локтевого сустава у детей с врожденной локтевой косорукостью. Современные проблемы науки и образования 2021; 4: 76–76.</mixed-citation></citation-alternatives></ref></ref-list></back></article>
