Çocukluk Çağında Hemofilik Artropati: Patofizyoloji, Tanı ve Tedavi

Abstract

Hemofili hastalarında yaygın olarak gözlenen artropati, yaşam kalitesini ciddi biçimde bozan bir problemdir. Hemofilik artropatide en sık etkilenen eklemler dizler, ayak bilekleri ve dirseklerdir. Eklem içinde gözlenen tek bir kanama bile sinovyum, kıkırdak ve subkondral kemikler dâhil eklemin tüm bileşenlerinde yıkıcı bir takım etkilere neden olabilir. Kıkırdak hasarı ve artropatinin erken dönem değişikliklerinin incelenmesi için daha çok ultrason ve manyetik rezonans görüntüleme kullanımı önerilmektedir. Profilaksi, hemofilik artropati gelişimini önlemek için standart bir tedavi seçeneği olarak gösterilmiştir. Erken yaşta başlanan primer profilaksi kanamaların önlenmesi açısından çok önemlidir ancak adölesan dönemde başlanan sekonder profilaksinin de tekrarlayan kanamaların önlenmesi ve kıkırdak hasarının gelişiminin geciktirilmesi konularında sağladığı ciddi faydaları bilinmektedir. Eklemin kanamaya maruz kalma süresi arttıkça, kıkırdak matriksinin dejenerasyonu ve buna bağlı kıkırdak kaybı da artacağı için eklem içindeki hematomun aspirasyonu hastalığın ilerlemesinin önlenmesinde önemli rol oynar. Profilaksi ve aspirasyonun tekrarlayan kanamaları engelleyemediği durumlarda ise hastalara sinovektomi uygulanması gerekebilir. Gerek medikal gerekse cerrahi yöntemlerle yapılabilen sinovektominin amacı sorunlu sinovyumu ortadan kaldırarak hemofilik artropatinin ilerlemesini önlemektir. Tıbbi sinovektomi (sinoviyortez) radyosinovektomi ve kimyasal sinovektomi olarak iki farklı başlık altında incelenebilir. Bunlardan radyosinovektominin yaklaşık %85 başarı oranı ile kimyasal sinovektomiye göre daha etkili olduğu bildirilmektedir. Tüm bu önlemlerin ilerleyici kıkırdak hasarını önlemede başarısız olduğu durumlarda, açık veya artroskopik sinovektomi, etkilenen eklemin artrodezi ve hatta artroplasti gibi uygulamalara ihtiyaç duyulabilmektedir. Burada, çocukluk çağında gözlenen hemofilik artropatinin patofizyolojisi, teşhis yöntemleri ve tedavisinin güncel literatür eşliğinde tartışılması amaçlanmıştır.

Keywords

• hemofili
• artropati
• sinovit
• tanısal görüntüleme

Hemophilic Arthropathy in Children: Pathophysiology, Diagnosis and Management

Abstract

Arthropathy is a serious and common problem in patients with hemophilia impairing the patient’s quality of life seriously. The most commonly affected joints in hemophilic arthropathy are knees, ankles and elbows. Even a single bleeding could cause devastating effects to synovium, cartilage and also subchondral bones. Ultrasound and magnetic resonance imaging have been advocated for the studying of cartilage damage. Prophylaxis has been demonstrated as a standard choice of management to prevent hemophilic arthropathy development. Primary prophylaxis starting at early years of age is very important to prevent hemorrhages but secondary prophylaxis in adolescents has also significant success rates. As the duration of exposure to the blood increases in the joint cavity, degeneration of the cartilage matrix and resultant cartilage loss also increase, so the aspiration of the hematoma from joint plays an important role in prevention of the disease progression. Synovectomy may be required in cases where prophylaxis and aspiration does not prevent the recurrent hemorrhages. The purpose of synovectomy either with medical or surgical methods is to remove the problematic synovium to prevent the progression of hemophilic arthropathy. Medical synovectomy (synoviorthesis) has two basic types; radiosynovectomy and chemical synovectomy and the former one is appearing to be more effective with an about 85% success rates. If all of these measures fail to prevent the progressive cartilage damage, open or arthroscopic synovectomy, arthrodesis of the affected joint or even arthroplasty could be necessary. Here we tried to summarize the pathological mechanism, diagnosis and management of hemophilic arthropathy in children.

Keywords

• hemophilia
• arthropathy
• synovitis
• diagnostic imaging

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