Turkiye Klinikleri Journal of Ophthalmology

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Orbital Fibröz Displazi: Olgu Sunumu ve Literatür Araştırması
Orbital Fibrous Dysplasia: Case Report and Review of Literature
Titap YAZICIOĞLUa
aSağlık Bilimleri Üniversitesi Dr. Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi, Göz Hastalıkları Kliniği, İstanbul, TÜRKİYE
Turkiye Klinikleri J Ophthalmol. 2020;29(1):86-91
doi: 10.5336/ophthal.2018-64111
Article Language: TR
Full Text
ÖZET
 Fibröz displazi (FD), normal kemik metabolizmasında, lokal olarak trabeküler kemiğin fibröz doku ile yer değiştirmesi sonucu görülen, etiyolojisi bilinmeyen, benign bir kemik hastalığıdır. Sıklıkla çocukluk çağı ve puberte döneminde büyümekte olan kemikleri etkiler ve yavaş progresyon gösterir. Genellikle asemptomatik olmasına rağmen lokal genişlemelere bağlı olarak kemikte distorsiyon ve zayıflama yaparak patolojik kırık oluşturabilir. Kraniyal sinir paralizilerine, fasiyal asimetri veya proptoz gibi kozmetik problemlere neden olabilir. Hastalığın tanısını koymada bilgisayarlı tomografi önemlidir. Radyolojik bulgular lezyonun matürasyon evresine göre değişkenlik göstermekle birlikte, son safhada görülen buzlu cam opasifikasyonu hastalık için spesifik bir bulgudur. Bu çalışmada, proptozis ve optik disk ödemine neden olan orbital FD tanısı konulan 77 yaşında erkek olgumuz sunulmuştur. Olgumuz FD için nadir görülen bir tutulum bölgesi olan orbitanın etkilenmesi ve erişkin çağda ortaya çıkması nedeni ile sıra dışı bir olgudur.

Anahtar Kelimeler: Fibröz displazi; monostotik; orbita; tomografi
ABSTRACT
 Fibrous dysplasia (FD) is a benign skeletal disorder of unknown etiology, which results from localized change in normal bone metabolism that results in the replacement of all the components of cancellous bone by fibrous tissue. It usually affects growing bones in the childhood period and puberty, and shows slowly progression. Although it is asymptomatic, local expansion of bone causes distorsion and weakness, that results in pathological fractures. It may also cause cosmetic problems such as cranial nerve paralysis, facial asymetry or proptosis. Computed tomography imaging is important in the diagnosis of the disease. Although radiological findings vary depending on maturation stage of the lesion, ground glass opacification is characteristic sign for the end stage of the disease. In this study, we present a 77-year old man who was diagnosed as orbital FD, causing proptosis and optic disc edema. It is an unusual case because of the involvement of the orbit, which is a rare side of FD and occurs in adulthood.

Keywords: Fibrous dysplasia; monostatic; orbit; tomography
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