Фиброзная дисплазия является патологией кости, в рамках которой нормальная костная ткань заменяется фиброзно-костной с возможным наличием трабекул (костных перегородок). По сути, это означает, что мышцы и связки близ очага поражения постепенно превращаются в кости. Процесс способен развиваться локально или затронуть сразу несколько костей. Болезнь относится к разряду опухолевых, но практически никогда не приводит к возникновению злокачественных новообразований.
При проявлении первых симптомов в детстве, заболевание, чаще всего, удается устранить полностью. Если во взрослом возрасте обнаруживается фиброзная дисплазия кости, лечение ее будет более сложным и, в зависимости от стадии, не гарантирует 100%-го результата. Достоверно установить причины развития патологии сегодня все еще не удается, но известно, что в большей мере ей подвержены женщины.
Если у вас или ваших близких обнаружена фиброзная дисплазия, лечение в нашей клинике станет эффективным решением проблемы. Высококлассные специалисты, новейшее оборудование, большой опыт — мы давно занимаемся лечением этой болезни, и поможем вам как можно быстрее вернуться к активной жизни.
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Классификация
Основная классификация патологии проходит из расчета масштабности поражения: монооссальная форма предусматривает наличие очагов на одной кости, полиоссальная — на нескольких, но обычно с одной стороны тела.
В российской медицине используется классификация Зацепина, согласно которой фиброзную дисплазию подразделяют на:
- Внутрикостную дисплазию. Внешнее искривление кости отсутствует, поскольку процесс замещения нормальной ткани на фиброзную происходит только внутри кости
- Тотальную дисплазию. Замещению подвергаются как внутренние, так и внешние ткани кости
- Опухолевую дисплазию. Редкий случай, когда замещение тканей достигает крайне больших размеров
- Синдром Олбрайта. Обычно встречается у детей, характеризуется множественностью очагов, нарушением работы эндокринной системы, заметной деформацией и диспропорцией частей тела
- Фиброзно-хрящевую форму. По сравнению с другими имеет самые высокие риски злокачественных новообразований
- Обызвествляющую дисплазию. Возникает редко, поражает малую и большую берцовую кости
На заметку: какую бы форму не приняла фиброзная дисплазия, лечение должно начинаться с консультации врача-ортопеда.
Причины фиброзной дисплазии
Большая часть специалистов сходится во мнении, что болезнь возникает в результате генетических мутаций и начинает развиваться еще в утробе матери, однако это лишь популярная теория.
Причины фиброзной дисплазии окончательно не установлены и по сей день, но выявлен ряд факторов, при наличии которых болезнь возникает чаще:
- Неоднократные переломы одного и того же участка кости (не слишком распространенный фактор, встречается только у ⅓ обратившихся пациентов)
- Наличие сопутствующих патологий (рудиментарная почка, врожденная атрофия зрительных нервов и пр.)
- Не достаточно быстрая скорость роста костей
- Деформационные процессы кости (вздутие и пр.)
Симптомы заболевания
На ранних стадиях фиброзная дисплазия может никак себя не проявлять и развиваться незаметно. Как правило, в этих случаях ее выявляют случайно в ходе обследования других заболеваний. Явственными симптомами патологии можно считать появление боли (чаще в области бедер), нарушение работы сердца и кровеносной системы, эндокринные нарушения, пигментацию.
Если в ходе обследования обнаружена фиброзная дисплазия кости, лечение назначается индивидуально, в зависимости от степени развития болезни, множественности ее очагов, возраста пациента и других факторов.
Генетический аспект лечения
В 2006 году был открыт ген, из-за мутации которого и возникает фиброзная дисплазия. Сегодня продолжаются работа по поиску возможности блокировки этой мутации, однако все это пока остается на уровне предварительных лабораторных исследований. В настоящее время предотвратить развитие фиброзной дисплазии кости невозможно, а ее лечение, в основном, заключается в хирургическом устранении деформированных тканей и заменой их на трансплантаты.
Фиброзная дисплазия черепа: лечение
Разрастание фиброзной ткани в области черепа всегда приводит к его деформации и асимметрии, но более точные проявления определяются локализацией очага:
- Поражение челюсти характеризуется ее утолщением и визуальным расширением верхней или нижней части лица. Чаще встречается фиброзная дисплазия нижней челюсти. Она развивается близ больших и малых коренных зубов, и выглядит как припухлость щек.
- Патология в области лба или темени представляет особую опасность, поскольку может вызвать смещение костной пластины и давление на мозг
- На фоне эндокринных нарушений болезнь способна спровоцировать разрастание фиброзной ткани у основания черепа и сбой в работе гипофиза. Последствия: диспропорция тела, локальная пигментация, нарушение связи в работе внутренних органов и систем
Если в детском возрасте диагностируется фиброзная дисплазия костей черепа, лечение предусматривает щадящее хирургическое вмешательство. Взрослым будет проведена резекция поврежденного участка кости с последующей трансплантацией. В ходе лечения могут быть показаны препараты, угнетающие развитие очага, снимающие болевой симптом и стимулирующие уплотнение костной ткани.
Фиброзная дисплазия бедренной кости: лечение
Поскольку бедренная кость постоянно подвергается большим физическим нагрузкам, деформационные процессы в ее структуре быстро становятся заметны. Обычно они характеризуются искривлением бедра в наружную сторону и укорачиванием конечности. На первых этапах это ведет к изменению походки, далее — к сильной хромоте. Костные искривления провоцируют развитие остеоартроза, что еще больше отягощает ситуацию.
Так как изменения бедренной кости отражаются на работе всего скелета, особенно важно выявить патологию на начальной стадии. Однако, когда бы не была обнаружена фиброзная дисплазия бедренной кости, лечение обязательно будет предусматривать оперативное вмешательство. Стоит сказать, что в большинстве случаев оно проходит удачно и не влечет осложнений.
Фиброзная дисплазия малой берцовой кости: лечение
Поражение малой берцовой кости, как правило, долго остается незамеченным и не влечет сильной деформации конечности. Даже боли часто возникают не сразу, а лишь через некоторое время. По сравнению с вышеописанным случаем, при диагнозе фиброзная дисплазия малой берцовой кости, лечение проходит и быстрее, и эффективнее, хотя также подразумевает хирургическую операцию с резекцией и трансплантацией.
Чаще всего, воздействие патологии ограничивается локальным участком кости, и не вредит работе внутренних систем организма. Если поражение не обширное и присутствует только на одной кости, прогноз лечения является благоприятным и практически всегда имеет положительный исход.
В случае, если диагностирована деформация костей конечностей или фиброзная дисплазия черепа, лечение следует начинать немедленно, ведь только так можно сохранить максимальное количество здоровой костной ткани. Не теряйте драгоценное время, обратитесь к специалистам нашей клиники за качественной медицинской помощью. Симптоматика проявления всегда разнообразна, а потому где-бы не была обнаружена патология, лечение необходимо подбирать в индивидуальном порядке.
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Диагностика
Диагностика проводится на основании анамнеза и рентгенологического исследования. Проведение последнего обязательно, так как это оптимальный способ изучить состояние скелета и его тканей. Даже если пациент обратится за помощью на первых стадиях болезни, рентген четко покажет очаги деформации. На снимке эти участки будут похожи на матовое стекло и содержать специфические вкрапления. При наличии одного или нескольких факторов, пациент может быть отправлен на денситометрию и КТ кости.
Нужно помнить, что на очень ранних стадиях, при малом количестве симптомов и одном небольшом очаге, даже при рентгенологическом исследовании диагностировать заболевание не всегда просто. Для полноты картины и исключения других возможных причин, пациенту могут быть рекомендованы консультации и обследования у ряда специалистов (кардиолога, онколога и пр.).
Фиброзная дисплазия: противопоказания к операции
Оперативное вмешательство при лечении фиброзной дисплазии имеет свои противопоказания и рекомендовано не всем. Выбирать другие методы воздействия стоит, когда:
- Болезнь только начала развиваться, 1-2 ее очага еще малы и не несут особой угрозы
- Речь идет о ребенке и расположение небольшого очага не подвержено сильной компрессии. В этом случае остается вероятность, что развитие патологии сойдет на нет самостоятельно, однако постоянное медицинское наблюдение все же требуется
- Процесс распространен в больших масштабах и задействовал множество костей. Таким пациентам рекомендованы другие способы лечения, в том числе медикаментозное.
Важно: на какой-бы стадии не была обнаружена фиброзная дисплазия, противопоказания к ее хирургическому лечению — не повод отчаиваться. Сегодня медицина располагает достаточными возможностями, чтобы давать положительные прогнозы в случаях с самыми обширными поражениями.
Профилактика
В качестве профилактических мер может рассматриваться общее оздоровление организма: лечебная гимнастика, укрепление мышечного корсета, массаж, лечебные ванны, профилактика переломов, употребление витаминов и пр. Рекомендуется отказаться от вредных привычек и избегать физических перегрузок.
Риски и осложнения
Фиброзная дисплазия, лечение которой не было проведено вовремя, нередко приводит к инвалидности. Кроме того, последствием ее запущенных форм могут стать: опухолевые процессы (доброкачественные и злокачественные новообразования), развитие сопутствующих заболеваний (нарушение слуха, зрения, работы сердца и пр.), чрезмерная активность гормона роста.
Не рискуйте своим здоровьем, если у вас обнаружена фиброзная дисплазия кости, лечение стоит доверить только высококвалифицированным специалистам, которые занимаются этой патологией уже многие годы. Такую узкопрофильную помощь на должном уровне оказать смогут далеко не везде, тогда как в нашей клинике фиброзная дисплазия является одним из ключевых направлений.
Лечение в нашей клинике:
- Бесплатная консультация врача
- Быстрое устранение болевого синдрома;
- Наша цель: полное восстановление и улучшение нарушенных функций;
- Видимые улучшения после 1-2 сеанса; Безопасные безоперационные методы.
Фиброзная дисплазия – это очень серьезная патология костной ткани с частичным замещением её соединительными волокнами. Естественно, что рубцовая ткань не обладает свойствами кости. Поэтому происходит нарушение целостности, многочисленные переломы в области очагов замещения. При тотальном разрушении кости может формироваться её искривление.
Фиброзная дисплазия костей поражает в основном детей и подростков. Это связано с тем, что в основе патологических изменений лежат внутриутробные нарушения развития. Первичные признаки могут формироваться при наличии сопутствующих факторов риска. Малоподвижный образ жизни, недостаточное развитие мышечной ткани нижних конечностей, дефицит в питании определённых витаминов и минералов – все это является спусковым механизмом для начала развития фиброзной дисплазии костей ног.
Важно понимать, что заболевание хоть и имеет в большинстве случаев врождённую этиологию, легко поддается коррекции и профилактике. Фиброзная дисплазия ноги часто поражает трубчатые длинные кости: бедренную, малую и большую берцовые. При тотальном разрушении может быть выявлена полиоссальная форма. Моноосальная форма – это локализация единичных очагов фиброзного замещения костной ткани в полости одной кости. Редко формируется двухсторонняя трансформация. Обычно страдает только одна конечность.
Если вы заподозрили у себя или своего ребенка наличие подобных изменений, то советуем без промедления обратиться за медицинской помощью. Для этого вы можете записаться на бесплатный прием в нашей клинике мануальной терапии. Опытный врач ортопед проведёт тщательный осмотр. Поставит диагноз и расскажет о том, как можно проводить лечение для остановки патологического процесса и возвращения нормального состояния костной ткани.
Причины фиброзной дисплазии
Знать причины фиброзной дисплазии костей необходимо для того, чтобы иметь возможность их исключать и таким образом проводить эффективную профилактику заболевания.
Начинать следует с факторов воздействия на этапе внутриутробного развития. Высокий риск развития патологии у плода может возникнуть, если женщина во время беременности:
- не соблюдала рекомендации врача;
- не употребляла витаминно-минеральные комплексы;
- использовала по назначения врача гормональные препараты с целью сохранения беременности;
- болела острыми вирусными и бактериальными инфекциями;
- лечилась с помощью антибактериальных и противовирусных средств.
После рождения включается в действие следующая триада негативных факторов:
- отсутствие достаточного ухода за младенцем, включая проводимые массаж, гимнастику, воздушные ванны;
- дефицит витамина D, приводящий к запуску процесса разрушения костной ткани на фоне рахита;
- снижение уровня иммунной защиты при отсутствии длительного периода грудного вскармливания.
Таким образом, можно сделать вывод о том, что в группу риска развития фиброзной дисплазии костей ног входят грудничка из социально неблагополучных семей, недоношенные и находящиеся на искусственном вскармливании. Важна профилактика рахита, особенно в северных регионах, где наблюдается дефицит солнечных дней.
В возрасте старше 3-х лет факторы риска развития дисплазии дополняются дефицитом некоторых элементов в повседневном рационе питания. Известно, что дети в ясельном и дошкольном возрасте отличаются особой привередливостью в выборе продуктов для своего питания. В большинстве своем они отказываются категорически от молока и молочных продуктов. Это ведет к неизбежному дефициту кальция. Начинается тотальное разрушение зубной эмали. Вслед за непроходящим и непобедимым кариесом приходит дисплазия костной ткани. Из неё в большом количестве вымывается кальций. В условиях тотального дефицита этого микроэлемента в составе крови очаги замещаются фиброзными соединительными волокнами.
Таким образом, если у вашего ребенка в возрасте 4 – 7 лет имеется серьёзная проблема с зубками, вы постоянно посещаете стоматолога и все равно это не помогает вылечить кариес, не стоит думать, что малыш просто много кушает сладкого. Вероятнее всего, у него существует дефицит кальция. Нужно срочно сделать биохимический анализ крови с определением этого микроэлемента. Если дефицит действительно присутствует, то важно проводить специфическую профилактику и искать причину патологии.
У взрослого человека фиброзную дисплазию костей ног могут вызывать следующие патологии:
- остеопороз, в том числе спровоцированные гормональными нарушениями и заболеваниями кишечника;
- избыточная масса тела с увеличением физической нагрузки на надкостницу, которая утрачивает способность обеспечивать кровоснабжение кости;
- неправильная постановка стопы;
- малоподвижный образ жизни и преимущественно сидячая работа;
- тотальная дистрофия седалищного нерва на фоне длительно протекающего остеохондроза пояснично-крестцового отдела позвоночника с нарушением иннервации всех тканей нижней конечности с одной или с обеих сторон;
- эндокринные заболевания надпочечников и щитовидной железы;
- деструкция крупных суставов нижней конечности и как следствие ослабление мышечной ткани, обеспечивающей нормальное кровоснабжение.
Фиброзная дисплазия бедренной кости (бедра)
Фиброзная дисплазия бедренной кости может быть врождённой и приобретенной, одно или многоочаговой, кистозной или простой. Форма патологии зависит от возраста пациента. Многоочаговая фиброзно-кистозная фибродисплазия возникает у пожилых пациентов на фоне гормонального остеопороза.
У детей в возрасте до 3-х лет фиброзная дисплазия бедра чаще всего проявляется в виде тотального замещения трабекул костной ткани соединительнотканными рубцами. Быстро наступает искривление конечности с уменьшением её длины. Самостоятельное передвижение невозможно. Фиксируются многочисленные переломы, место которых не срастается типичным образом с помощью формирования костной мозоли.
У детей в возрасте от 3-х до 7-8 лет подобная патология может стать следствием грубого нарушения режима питания и оказываемых на нижние конечности нагрузок. В последнее время большое распространение принимает так называемая гиподинамическая форма дисплазии. Стоит понимать, что для правильного кровоснабжения надкостницы требуется хорошая слаженная работа мышц берда и голени. Хорошо натренированные мышцы стимулируют капиллярное кровоснабжение и ускоряют обменные процессы в костной ткани. Если нет регулярных и полноценных физических нагрузок на нижние конечности, то костная ткань может начать разрушаться. В областях дистрофических изменений происходит рубцевание соединительной тканью.
Фиброзная дисплазия бедра отличается быстрым прогрессирующим течением. Если у ребёнка появились жалобы на боль в области бедренной кости, тазобедренного или коленного сустава, сразу же обращайтесь на прием к опытному ортопеду. Врач назначит лечение, которое остановит течение опасной патологии.
Фиброзная дисплазия коленного сустава
Фиброзная дисплазия коленного сустава – это патология, при которой страдают головки, входящих в сочленение костей (бедренной и берцовой). Остальная трубчатая структура может оставаться без изменений. В области головок формируются многочисленные очаги фиброзного заполнения. Эта патология требует тщательной диагностики с помощью рентгенографических снимков. На них отчетливо видны отграниченные очаги, в полости которых находится не костная ткань, а рубцовая. Она дает эффект затемнения. Требуется дифференциальная диагностика с целью исключения остеосаркомы и других видов злокачественных новообразований.
Патология коленного сустава проявляется следующими симптомами:
- постоянные тупые боли, усиливающиеся после физических нагрузок;
- щелчки и хруст при движении в плоскости данного сочленения костей;
- судороги в области бедра и голени;
- укорочение конечности на 3-5 см;
- хромота и переваливающаяся походка;
- постоянные переломы, вывихи, растяжение связок в области коленного сустава.
При любых неприятных ощущениях следует обращаться за медицинской помощью. Своевременная профилактика и лечение позволяют купировать негативные проявления.
Фиброзная дисплазия большеберцовой и малоберцовой кости
Развивающаяся фиброзная дисплазия берцовой кости на начальных этапах дает неправильную постановку стопы. Ножка в голеностопе подворачивается внутрь или наружу. В зависимости от этого формируется вальгусная или варусная деформация голени. Если поражена только одна кость, то возникает нарушение походки, а в целом возможность самостоятельного передвижения не утрачивается.
Большую опасность в плане будущего прогноза представляет собой фиброзная дисплазия большой берцовой кости, поскольку в этом варианте высока вероятность инвалидности уже на ранней стадии. В большинстве случаев фиброзная дисплазия большеберцовой кости сопровождается сильными болями, отсутствием способности к длительным физическим нагрузкам. быстрая утомляемость мышц голени является первым признаком неблагополучия. Если ребенок жалуется на боль в голени при подъеме по лестнице, то его следует немедленно показать ортопеду.
Фиброзная дисплазия малоберцовой кости редко приводит к стойкой инвалидности. Она разрушается медленнее за счет того, что на неё оказываться меньшая нагрузка. Обычно заболевание выявляется при случайном рентгенографическом снимке по поводу подозрения на перелом малой берцовой кости.
Перед тем, как лечить фиброзную дисплазию
Важно понимать, что подобными симптомами, особенно у детей, может проявляться огромное количество дистрофических, эндокринных и обменных заболеваний. Поэтому, перед тем как лечить фиброзную дисплазии нижних конечностей, нужно провести тщательную дифференциальную диагностику.
Для начала вы можете записаться на прием к опытному ортопеду в нашей клинике мануальной терапии. В ходе первого осмотра опытный доктор сможет поставит правильный предварительный диагноз. Если потребуется, вам будут рекомендованы дополнительные меры обследования. Обычно использования МРТ, при проведении которой диагностика позволяет провести исключение все злокачественных новообразований. Также потребуется сделать биохимический анализ крови для исключения эндокринных и обменных патологий.
Запишитесь на первичную бесплатную консультацию. в нашей клинике. Посетить доктора вы сможете в любое удобное для вас время.
Лечение фиброзной дисплазии кости
Официальная медицина проводит лечение фиброзной дисплазии кости с помощью хирургического вмешательства. В очаги патологически изменённой ткани вводится специальный структурный состав, напоминающий по свойствам костную ткань. Это дает положительный результат только на крайне ограниченный срок. Начинается быстрое разрушение костной ткани, расположенной рядом с заполненными участками за счет еще большего ухудшения процесса кровоснабжения.
Единственное эффективное лечение фиброзной дисплазии заключается в полном устранении причины этой патологии. Мануальная терапия позволяет решить эту задачу кардинальным образом. Остеопатия устраняет нарушения в процессе движения лимфатической жидкости и крови. Массаж ускоряет процессы микроциркуляции. Кинезитерапия и лечебная гимнастика укрепляет мышцы и восстанавливает естественное питание надкостницы.
Рефлексотерапия запускает обменные и восстановительные процессы. В сочетании с рекомендациями диетолога и мануа л ьного терапевта эти методики дают положительный и долгосрочный результат.
Важно начинать лечение на ранних стадиях. Чем раньше вы обратитесь к врачу, тем выше шансы на выздоровление.
Фиброзная дисплазия — опухолеподобное заболевание, при котором происходит локальное или распространенное изменение костных тканей. Соединительные ткани разрастаются, костные — деформируются, нарушается их функционирование, появляются небольшие костные трабекулы. Данная аномалия фиброзных изменений впервые исследована в начале прошлого века медиками Брайцовым и Олбрайт, но детально описана в 40–х гг. 20 века Яффе и Лихтенштейном, поэтому заболевание получило название синдрома Лихтенштейна-Яффе.
Болезнь в основном проявляется в длинных трубчатых костях, но может выражаться патологиями ткани черепа, ребра и бедренной кости. Поражение может охватывать не только один очаг, но и несколько других. Деформацию фиброзного участка сопровождает сильный болевой синдром, изменения характеризуются удлинением или укорочением патологических сегментов, подверженных частым переломам.
Причины патологии
Этиология болезни до конца не выяснена, ее причинами могут быть аномалии внутриутробных генных мутаций. Чаще всего патологии костей наблюдаются у детей дошкольного возраста. Неблагоприятными факторами формирования фиброзных процессов во время протекания беременности являются:
- аномалии развития хрящевых и костных тканей;
- патологии суставов, изменения в мышцах и связках;
- измененный гормональный фон;
- наличие генетической предрасположенности;
- хронические обострения болезни матери;
- влияние тяжелых инфекционных процессов;
- медикаментозные препараты, противопоказанные во время беременности;
- токсические химические вещества;
- алкогольная, никотиновая или наркотическая зависимость беременной;
- гинекологические заболевания, токсикозы;
- нарушенное рациональное питание будущей матери.
Основной группой риска возникновения фиброзов костного скелета являются маленькие дети, но зафиксированы случаи проявления болезни в преклонном возрасте, причем женщины подвергаются костным патологиям во много раз чаще мужчин.
Классификация заболевания
В зависимости от характера поражения различают 3 основные формы фиброзной дисплазии:
- Монооссальная — развивается в 70% случаев, поражает только один очаг и возникает в 20–30-летнем возрасте.
- Мономелическая, отличающаяся патологическим изменением нескольких соседствующих костей конечности, тазовой или плечевой области.
- Полиоссальная — проявляется в 30% случаев, характеризуется фиброзными патологиями нескольких очагов и возникает в дошкольном возрасте.
Патологические фиброзные изменения могут охватывать любые части человеческого скелета. Дисплазия обнаруживается в костных системах:
- черепа, в основном лицевых и челюстных;
- плечевых;
- правой и левой подвздошных костей;
- ребер;
- позвонка;
- тазовых;
- берцовой;
- большеберцовой кости;
- коленного сустава.
Российскими медиками используется клиническая классификация доктора Зацепина, различающая такие формы дисплазии:
- внутрикостные фиброзы, при которых разрастаются патологические ткани монооссального или полиоссального типа, но сохранен корковый слой и отсутствует внешняя деформация;
- тотальные изменения, при которых происходит разрушение как костномозговой части, так и кортикального слоя, образуя деформации и являясь причиной последующих переломов;
- опухолевые фиброзы, отличающиеся большим ростом патологического очага;
- дисплазии синдрома Олбрайта, при которых генерализированное полиоссальное поражение сочетается с эндокринным нарушением, влиянием преждевременного полового созревания девочек на пропорциональное строение тела, очаговыми кожными пигментациями, деформацией скелета;
- фиброзно–хрящевая форма, при которой происходит перерождение хрящевой ткани и появление хондросаркомы;
- обызвествляющие фибромы, поражающие большеберцовые кости.
Черепные дисплазии классифицируются отдельно и имеют собственные формы:
- склеротическую, при которой уплотняется костная ткань и образуются ясно выраженные участки пораженной кости (основание черепа, нос, лоб, верхняя челюсть);
- кистоподобную, поражающую нижнюю челюсть кистообразными образованиями, которые имеют множественный или одиночный характер;
- педжетоподобную, отличающуюся большим возрастанием объемов мозговых полушарий в результате болезни Педжета;
- черубизмы, поражающие верхнюю и нижнюю челюсти.
Характерная симптоматика
Небольшие одиночные дисплазии протекают сначала бессимптомно и выявляются, когда проводится рентген по иным поводам. Постепенно происходит утолщение инфицированного участка, возникает воспалительный процесс, через 2–3 года наблюдаютя болевые симптомы и пигментирование кожных покровов в месте фиброзных изменений.
Первоначальным проявлением фиброзов считается развитие эндокринных расстройств. Небольшие сегменты одиночных монодисплазий выявляются в черепно–лицевых костях, ребрах и костях бедра. Множественная фиброзная форма заболевания способна охватить от двух костей до 80% скелета. Выявляется данная патология в костях тазобедренных, голени, колена. Верхняя часть тела поражается меньше.
Распространены физические уродства в виде асимметрии лица, реберных деформаций и неестественной длины ног. Больше половины больных страдают патологическими переломами и нарушениями сочленения костей.
Клеточно-волокнистая ткань области поражения усеивается кровеносной сосудистой сеткой и разбросанными костными образованиями, имеющими диффузный или очаговый вид. Очаговое поражение трансформирует уплотненную, обособленную от окружающих тканей часть, легко отделяющуюся от кости. Диффузные изменения мешают точному определению размеров поражения из-за пористости и мягкости кости. Происходит истончение коркового слоя, кость превращается в плотную бледно–желтую субстанцию. Могут обнаруживаться кистообразные образования.
Симптоматика различается в соответствии с локализацией заболевания:
- большая берцовая и малоберцовая кости при дисплазии характеризуются укорочением, надломами, хромотой и переломами;
- коленные суставы беспокоят болевыми синдромами (физические нагрузки, перемена погоды), хрустом и увеличением подвижности суставной чашки, изменением формы сустава, ног и походки;
- челюстные фиброзы деформируют лицо, нижняя или верхняя челюсть утолщается;
- патологии черепных костей при дисплазии влияют на нормальное формирование зубов, характеризуются изменениями объема черепной коробки и носовых пазух, деформацией первого позвонка (как следствие — изменение осанки, позвоночника и таза);
- при фиброзах плечевых костей нарушается подвижность рук;
- при синдроме Олбрайта происходят эндокринные расстройства, пропорциональные изменения скелета, пигментации кожи и деформация костей, явления раннего полового развития у девочек.
Диагностика
Установление точного диагноза достигается совместными усилиями врачей различной специализации: хирургов, ортопедов, фтизиатров, эндокринологов, онкологов и травматологов. Кроме того, проводится полное обследование, заключающееся в проведении:
- осмотра больного, во время которого определяется выраженность симптомов методами пальпации и перкуссии;
- сбора анамнеза и поиска этиологических предпосылок болезни;
- рентгенографии и магнитно–резонансной томографии, выявляющих локализации и характеристики патологического изменения, а также помогающих исключить туберкулезные и онкологические заболевания;
- гистологических исследований, позволяющих на микробиологическом уровне изучить коллагеновые волокна и исключить онкологические образования;
- денситометрии и КТ (компьютерной томограммы);
- сцинтиграфического исследования для определения стадии заболевания.
Способы лечения
Избавление от данной патологии предполагает проведение хирургического вмешательства, при котором иссекается очаг фиброза и часть кости трансплантируется искуственным аналогом. При черепных изменениях лицевых костных тканей производят чистку орбитальной области вокруг глазных яблок.
Хирургическими методами фиброзное изменение с успехом лечится в большинстве случаев. При злокачественном осложнении показана ампутация конечности.
Консервативная терапия
Консервативная терапияБессимптомное течение заболевания предполагает клиническое наблюдение характерных волокнистых изменений, для чего каждые полгода проводятся рентгенологические исследования и посещения эндокринолога для лечения системного расстройства. Для купирования болевых ощущений при одиночных формах применяется Памидронат и другие препараты биофосфанатов.
При полиоссальных формах хирургические методы не показаны, проводятся профилактические мероприятия, которые предполагают дополнение лечения:
- курсами лечебного массажа;
- физиотерапевтическими процедурами;
- ЛФК;
- снижением нагрузки на суставы при помощи специальных средств;
- ношением ортопедической обуви.
В случаях патологических переломов и удлинении (либо укорочении) конечностей применяется аппарат Илизарова.
Хирургическое вмешательство
К эффективным хирургическим способам относится метод остеосинтеза, при котором проводится соединение костных обломков и устраняется их подвижность фиксирующими приспособлениями. Используются следующие виды остеосинтеза:
- чрескостный — производится жесткое соединение металлическими спицами или гвоздями обломков кости и их фиксирование специальными дугами;
- монолокальный компрессионный — создается управляемая компрессия (дистракция) аппаратом внешней фиксации;
- билокальный компрессионный дистракционный — дозированно перемещается сформированный фрагмент обломка кости.
Заключение
После хирургической операции или проведения терапевтических методов здоровье пациентов улучшается. Но, в связи с большой вероятностью рецидивов, нужно постоянно наблюдаться у медицинских специалистов. При возникновении боли и деформации в костной системе, трудностях передвижения, рецидивах переломов необходимо своевременно обратиться за медицинской помощью.
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