Fibrous Dysplasia: (Because of 2 Cases)

Abstract

Aim: Fibrous dysplasia is a slow growing skeletal developmental anomaly in which particularly long tubular bones, ribs, skull and facial bones are involved. We aimed to present two cases diagnosed as fibrous dysplasia in our neurosurgey clinic. Case report: The first case was 11 year-old, male and had a craniofacial involvement with a rarely seen humerus involvement polyostotic fibrous dysplasia. The second case was 14 year-old, male and had only craniofacial involvement monostotic fibrous dysplasia. While medical treatment is used to reduce the symptomps of the disease, the surgical treatment is used to correct deformities in fibrous dysplasia. Cranioectomy was carried out for cosmetic purposes in case I, and due to prediagnosis of “osteom” in the Case II. The two cases were diagnosed as fibrous dysplasia as a result of clinical, histopathological and radiological examinations. Follow-ups for both cases were done. Result: Craniofacial involvement with humerus involvement is rarely seen in polyostotic fibrous dysplasia. We evaluated these two cases under the light of literature

Keywords

• Fibrous dysplasia,polyostotic,monostotic,humerus

Fibröz Displazi: (2 Olgu Nedeniyle)

Abstract

Amaç: Fibröz displazi, benign, yavaş ilerleyen, özellikle kraniyofasiyal, uzun kemikler ve kosta tutulumu gösteren iskelet lezyonudur. Kliniğimizde fibröz displazi tanısı koyduğumuz 2 olguyu sunmayı amaçladık. Olgu Sunumu: İlk olgu 11 yaşında kraniyofasiyal tutulumla birlikte humerus tutulumu olan polyostotik fibröz displazi. İkinci olgu 14 yaşında sadece kraniyal tutulumu olan monostotik fibröz displazi. Fibröz displazide medikal tedavi semptomları azaltmak için kullanılır iken cerrahi tedavi deformiteleri düzeltmek amacıyla uygulanır. İlk olguya kozmetik amaçlı kraniektomi, ikinci olguya ise osteom ön tanısıyla kraniektomi yapıldı. Klinik, histopatolojik ve radyolojik inceleme sonucu olgulara fibröz displazi tanısı konuldu. Her iki olguda klinik olarak takibe alındı. Sonuç: Kraniyofasiyal tutulum ile birlikte humerus tutulumu olan polyostotik fibröz displazi oldukça nadir görülmektedir

Keywords

• fibröz displazi,polyostotik,monostotik,humerus

References

1. Ham DW, Lassen LF, Pitman KT: Fibrous dysplasia of the clivus and sphenoid sinus. Military Medicine. 163: 186-189, 1998.
2. Lichtenstein L: Polyostotic fibrous dysplasia. Arch Surg. 36: 874-898, 1938.
3. Lichtenstein L, Jaffe HL: Fibrous dysplasia of bone. A condition affecting one, several or many bones the graver cases of which may present abnormal pigmentation of skin, premature sexual development hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol. 33:777-816, 1942.
4. Lustig LR, Holliday MJ, Mc Carthy EF, Nager GT: Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 127: 1239- 1247, 2001.
5. Dahlin DC, Uni KK: Bone Tumors: General Aspects and Data on 8542 Cases. Springfield,IL: Charles C Thomas, 1986
6. Bruno AM. Fibrous dysplasia. Available from http://www. emedicine. Com / RADIO / topic 284 htm. Cited 30 march 2007.
7. Klempous J, Kuzniar J, Sztuka A, Jaworska M: A case of polyostotic fibrous dysplasia. Wiad Lek. 59(1-2):125-7, 2006.
8. DiCaprio R.M., Enneking F.W: Fibrous Dysplasia. Pathophysiology, Evaluation, and Treatment. J Bone Joint Surg Am. 87:1848-1864, 2005.