Патогенез, диагностика и лечение малоберцовой гемимелии (литературный обзор)

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. 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. 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. 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. 

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