urmjУральский медицинский журналUral Medical Journal2949-4389Ural State Medical University10.52420/umj.25.1.92DWAOUZurmj-2092Research ArticleЛитературные обзоры | Literature reviewsLiterature reviewsПатогенез, диагностика и лечение малоберцовой гемимелии (литературный обзор)Pathogenesis, Diagnosis and Treatment of Fibular Hemimelia (Literature Review)ИмомовШ. А.ImomovSh. A.

Шохбек Азаматович Имомов — аспирант 

Курган

Shokhbek A. Imomov — Postgraduate Student 

Kurgan 

https://orcid.org/0000-0003-0883-9663ЛеончукС. С.LeonchukS. S.

Сергей Сергеевич Леончук — кандидат медицинских наук, заведующий травматолого-ортопедическим отделением № 6 

Курган

Sergey S. Leonchuk — Candidate of Sciences (Medicine), Head of the Traumatology and Orthopedics Department No. 6 

Kurgan 

leon4yk@mail.ruhttps://orcid.org/0000-0002-7806-7083АрановичА. М.AranovichA. M.

Анна Майоровна Аранович — доктор медицинских наук, профессор, главный специалист травматолого-ортопедического отделения № 17

Курган 

Anna M. Aranovich — Doctor of Sciences (Medicine), Professor, Chief Specialist of the Traumatology and Orthopedics Department No. 17 

Kurgan 

aranovich_anna@mail.ruНациональный медицинский исследовательский центр травматологии и ортопедии имени академика Г. А. ИлизароваРоссияNational Ilizarov Medical Research Center for Traumatology and OrthopedicsRussian Federation20262702202625192–108Copyright © Имомов Ш.А., Леончук С.С., Аранович А.М., 20262026Имомов Ш.А., Леончук С.С., Аранович А.М.Imomov S.A., Leonchuk S.S., Aranovich A.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.umjusmu.ru/jour/article/view/2092Введение

Введение. Малоберцовая гемимелия (МГ) — врожденная аномалия развития (часть или вся малоберцовая кость гипопластична, диспластична или отсутствует), которая сочетается с целым спектром нарушений на больной конечности. МГ представляет собой сложную проблему детской ортопедии.

Цель исследования — на основании анализа научной литературы представить основы патогенеза, возможности диагностики, оперативного и консервативного лечения детей с МГ.

Материалы и методы

Материалы и методы. Проведен поиск публикаций в электронных базах данных PubMed, Google Scholar и eLibrary.ru по следующим ключевым словам: «малоберцовая гемимелия», «эктромелия малоберцовой кости», «продольная эктромелия», fibular hemimelia, congenital absence of the fibula, postaxial anomaly, congenital longitudinal deficiency. Ввиду редкости МГ глубина поиска составила 70 лет.

Результаты и обсуждение

Результаты и обсуждение. В ходе анализа публикаций определено, что в настоящее время нет единого мнения о тактике хирургического ортопедического лечения детей с МГ. Главными вопросами, которые поднимаются хирургами-ортопедами, являются следующие: выполнять раннюю ампутацию или реконструктивные ортопедические вмешательства? когда начинать реконструктивное ортопедическое лечение на больной конечности? в каком возрасте и каком объеме выполнять первое удлинение больной конечности?

Заключение

Заключение. В настоящее время обязательными условиями в успешном лечении больных с МГ являются междисциплинарная курация и возрастная преемственность, которые осуществляют акушер-гинеколог, ортопед, психолог, реабилитолог, эрго- и физиотерапевт, сестринский медицинский персонал и ортезист или протезист. Реконструктивное ортопедическое лечение должно быть индивидуальным, с учетом тяжести аномалии, возраста пациента, мотивации и психологической готовности семьи к лечению. 

Background

Background. Fibular hemimelia (FH) is a congenital anomaly in which part or all of the fibula is hypoplastic, dysplastic, or absent, associated with a wide range of abnormalities of the affected limb. FH is a challenging problem in pediatric orthopedics.

The aim of the study is to present the fundamentals of pathogenesis, diagnostic options, and surgical and conservative treatment approaches for children with FH, based on an analysis of scientific literature.

Materials and methods

Materials and methods. The search was performed in PubMed, Google Scholar and eLibrary.ru databases for publications using the following keywords: fibular hemimelia, fibular ectromelia, longitudinal ectromelia, congenital absence of the fibula, postaxial anomaly, and congenital longitudinal deficiency. Due to the rarity of this disease, the search depth was 70 years.

Results and discussion

Results and discussion. An analysis of the publications revealed that there is currently no consensus on the surgical orthopedic treatment strategy for children with FH. The main questions raised by orthopedic surgeons: to perform early amputation or reconstructive orthopedic interventions? when to begin reconstructive orthopedic treatment on the affected limb? at what age and how much should a limb be lengthened during the first lengthening?

Conclusion

Conclusion. Currently, interdisciplinary care and age-appropriate management involving an obstetrician/gynecologist, orthopedist, psychologist, rehabilitation specialist, occupational and physical therapist, nursing staff, and orthotist/prosthetist are essential for the successful treatment of patients with FH. Reconstructive orthopedic treatment should be individualized, taking into account the severity of the anomaly, the patient’s age, and the family’s motivation and psychological readiness for treatment. 

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The authors declare that there are no conflicts of interest present.