Fibrous dysplasia of the skull: neurological manifestations

Freitas, Paulo Eduardo; Ribeiro, Telmo Ramos; Coutinho, Mario Ferreira; Anicet, Adão

doi:10.1590/S0004-282X1989000200010

Abstracts

The neurological manifestations of eight patients with osseous fibrous dysplasia of skull were retrospectively studied. In this series, the frontal bone was involved in 75% of the patients, the sphenoid or temporal bones in 37.5%, and the parietal in 25%. It was observed that cranial deformities occurred in all but one case, headache was present in 87.5% of the patients, visual disturbances in 50%, proptosis in 25%, and auditive symptoms in 12.5%. Six out of the eight patients were operated on: three of the four who presented progressive visual loss had postoperative improvement. In their follow up, none presented, until now, evidence of recurrence or worsening of symptoms, confirming the benign nature of fibrous dysplasia.

Foram estudados, retrospectivamente, oito pacientes com displasia fibrosa de ossos do crânio, analisando-se suas manifestações neurológicas. Na presente série, o osso frontal estava comprometido em 75% dos pacientes, o esfenóide ou o temporal em 37,5% e o parietal em 25%. Observou-se que, além das deformidades ósseas (presentes em todos os casos com exceção de um), 87,5% dos pacientes queixavam-se de cefaléia, 50% apresentavam diminuição da acuidade visual, 25% tinham proptose e em 12,5% predominavam sintomas auditivos. Dos seis pacientes submetidos a tratamento neurocirúrgico, quatro apresentavam alterações oftalmológicas progressivas, havendo melhora acentuada pós-operatória em três deles. Todos os casos foram seguidos ambulatorialmente, sem que em qualquer deles fosse observada a recidiva ou piora dos sintomas, confirmando a natureza benigna da displasia fibrosa.

Displasia fibrosa do crânio: manifestações neurológicas

Fibrous dysplasia of the skull: neurological manifestations

Paulo Eduardo Freitas^I; Telmo Ramos Ribeiro^II; Mario Ferreira Coutinho^III; Adão Anicet^IV

^IServiço de Neurologia e Neurocirurgia Prof. Mario Coutinho, Porto Alegre: Neurocirurgião do Serviço

^IIServiço de Neurologia e Neurocirurgia Prof. Mario Coutinho, Porto Alegre: Neurocirurgião

^IIIServiço de Neurologia e Neurocirurgia Prof. Mario Coutinho, Porto Alegre: Chefe do Serviço

^IVServiço de Neurologia e Neurocirurgia Prof. Mario Coutinho, Porto Alegre: Neurorradiologista do Serviço

RESUMO

Foram estudados, retrospectivamente, oito pacientes com displasia fibrosa de ossos do crânio, analisando-se suas manifestações neurológicas. Na presente série, o osso frontal estava comprometido em 75% dos pacientes, o esfenóide ou o temporal em 37,5% e o parietal em 25%. Observou-se que, além das deformidades ósseas (presentes em todos os casos com exceção de um), 87,5% dos pacientes queixavam-se de cefaléia, 50% apresentavam diminuição da acuidade visual, 25% tinham proptose e em 12,5% predominavam sintomas auditivos. Dos seis pacientes submetidos a tratamento neurocirúrgico, quatro apresentavam alterações oftalmológicas progressivas, havendo melhora acentuada pós-operatória em três deles. Todos os casos foram seguidos ambulatorialmente, sem que em qualquer deles fosse observada a recidiva ou piora dos sintomas, confirmando a natureza benigna da displasia fibrosa.

SUMMARY

The neurological manifestations of eight patients with osseous fibrous dysplasia of skull were retrospectively studied. In this series, the frontal bone was involved in 75% of the patients, the sphenoid or temporal bones in 37.5%, and the parietal in 25%. It was observed that cranial deformities occurred in all but one case, headache was present in 87.5% of the patients, visual disturbances in 50%, proptosis in 25%, and auditive symptoms in 12.5%. Six out of the eight patients were operated on: three of the four who presented progressive visual loss had postoperative improvement. In their follow up, none presented, until now, evidence of recurrence or worsening of symptoms, confirming the benign nature of fibrous dysplasia.

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1. Albright F, Buttler AM, Hampton AO, Smith P - Syndrome characterized by Osteitis Fibrosa Disseminata, areas of pigmentation and endocrine dysfunctions with precocious puberty in females: report of five cases. N Engl J Med 216: 727, 1937.
2. Barrionuevo CE, Marscallo FA, Coelho A, Cruz GA, Mocellin M, Patrocinio JA - Fibrous dysplasia and the temporal bone. Arch Otolaryngol 106: 298, 1980.
3. Belaval GS, Schneider RW - Fibrous dysplasia of bone. Clev Clin Quart 21: 158, 1953.
4. Calderon M, Brady HR - Fibrous dysplasia of bone, with bilateral optic foramina involvement. Am J Ophtalmol 68: 513, 1969.
5. Cohen A, Rosenwasser H - Fibrous dysplasia of the temporal bone. Arch Otolaryngol 89: 31, 1969.
6. Daffner RH, Kirks DR, Gehweilor JA Jr. Heaston DK - Computed tomography of fibrous dysplasia. AJR. 139: 943, 1982.
7. Donosso LA, Magargal LE, Eiferman RA - Fibrous dysplasia of the orbit with optic nerve decompression. Ann Ophtalmol 14: 80, 1982.
8. Feiring W, Feiring EH, Davidoff LM - Fibrous dysplasia of the skull. J Neurosurg 8: 377, 1951.
9. Fernandez E, Colavita N, Moschini M, Faleni A - «Fibrous dysplasia>> of the skull with complete unilateral cranial nerve involvement. J Neurosurg 5 2: 404, 1980.
10. Finney HL, Roberts TS - Fibrous dysplasia of the skull with progressive cranial nerve involvement. Surg Neurol 6: 341, 1976.
11. Fries JH - The roentgen features of fibrous dysplasia of the skull and facial bones: a critical analysis of thirty-nine pathologically proved cases. Am J Roentgenol 77: 71, 1957.
12. Graf CJ, Perret GE - Spontaneous recurrent hemorrhage as an unusual complication tf fibrous dysplasia of the skull. J Neurosurg 52: 570, 1980.
13. Harris WH, Dudley AR, Barry RJ - The natural history of fibrous dysplasia: an orthopaedic, pathological, and roentgenographlc study. J Bone Joint Surg 44: 207, 1962.
14. Harrison DFM - Osseous and fibro-osseous conditions affecting the cranio tacial bones. Ann Otol Rhinol Laryngol 9 3:199, 1984.
15. Ishiguro S, Ikeda M, Shimizu H, Hayashi M - Arteriovenous fistula as an unusual complication of polyostotic fibrous dysplasia of the skull. Surg Neurol 24: 681, 1985.
16. Ito H, Waga S, Sakakura M - Fibrous dysplasia of the skull with increased vascu.ar.ty in the angiogram. Surg Neurol 23:408, 1985.
17. Jackson IT, Armstrong HH, Gomuwka PK, Laws ER Jr, Langford K - Treatment of cranio-orbital fibrous dysplasia. J Max-Fac Surg 10: 138, 1982.
18. Kinnman JEG, Hong CE, Lee EB, Shin HS - .fibrous dysplasia of the face and skull. Pract Oto-Rhinolaryng 31: 11, 1969.
19. Lambert PR, Brackmann DE - Fibrous dysplasia of the temporal bone: the use of computerized tomography. Otolaryngol Head Neck Surg 92: 461, 1984.
20. Leeds N, Seaman WMB - Fibrous dysplasia of the skull and its differential diagnosis: a clinical and roentgenographic study of 46 cases. Radiology 78: 570, 1962.
21. Lichtenstein L - Polyostotic fibrous dysplasia. Arch Surg 36: 874, 1938.
22. Lichtenstein L, Jaffe HL - Fibrous dyplasia of bone. Arch Pathol 33: 777, 1942.
23. Lin JP, Goodkin R, Chase NE, Kricheff II - The angiographic featured or fibrous dysplasia of the skull. Radiology 92: 1275, 1969.
24. Mendelsohn DB, Hertzanu Y, Cohen M, Lello G - Computed tomography of craniofacial fibrous dysplasia. J Comput Assist Tomogr 8: 1062, 1984.
25. Montoya G, Evarts CM, Dohn DF - Polyostotic fibrous dysplasia and spinal cord compression: case report. J Neurosurg 29:102, 1968.
26. Nager GT, Holliday MJ - Fibrous dysplasia of the temporal bone: update w.th case reports. Ann Otol Rhinol Laryngol 93: 630, 1984.
27. Nishioka K, Masuda Y, Inokuchi I, Iyoda K, Tanaka T - A case of monostotic fibrous dysplasia of the temporal bone associated with epileptic seizure. Acta Med Okayama 36: 453, 1982.
28. Ramsey HE, Strong EW, Frazell EL - Fibrous dysplasia of the craniofacial bones. Am J Surg 116: 542, 1968.
29. Sassin JF, Rosenberg RN - Neurological complications of fibrous dysplasia of the skull. Arch Neurol 18: 363, 1968.
30. Schlumberger HG - Fibrous dysplasia of single bones (monostotic fibrous dysplasia). Milit Surgeon 99: 504, 1946.
31. Schwartz DT, Alpert M - The malignant transformation of fibrous dysplasia. Am J Med Sci 247: 1, 1964.
32. Sharp M - Monostotic fibrous dysplasia of the temporal bone. J Laryngol Otol 84: 697, 1970.
33. Taborelli A, Ballarini U, Monolo L, Dorizzi A - Cystic fibrous dysplasia of the skull presenting as an extradural mass: case report. J Neurosurg Sci 27: 253, 1983.
34. Zanini SA, Psillakis JM, Migowski W Jr, Tershakowec M - Craniofacial fibrous dysplasia. Ann Plast Surg 14: 378, 1985.

Publication Dates

Publication in this collection
06 June 2011
Date of issue
June 1989

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Albright F, Buttler AM, Hampton AO, Smith P - Syndrome characterized by Osteitis Fibrosa Disseminata, areas of pigmentation and endocrine dysfunctions with precocious puberty in females: report of five cases. N Engl J Med 216: 727, 1937.

[2] 2. Barrionuevo CE, Marscallo FA, Coelho A, Cruz GA, Mocellin M, Patrocinio JA - Fibrous dysplasia and the temporal bone. Arch Otolaryngol 106: 298, 1980.

[3] 3. Belaval GS, Schneider RW - Fibrous dysplasia of bone. Clev Clin Quart 21: 158, 1953.

[4] 4. Calderon M, Brady HR - Fibrous dysplasia of bone, with bilateral optic foramina involvement. Am J Ophtalmol 68: 513, 1969.

[5] 5. Cohen A, Rosenwasser H - Fibrous dysplasia of the temporal bone. Arch Otolaryngol 89: 31, 1969.

[6] 6. Daffner RH, Kirks DR, Gehweilor JA Jr. Heaston DK - Computed tomography of fibrous dysplasia. AJR. 139: 943, 1982.

[7] 7. Donosso LA, Magargal LE, Eiferman RA - Fibrous dysplasia of the orbit with optic nerve decompression. Ann Ophtalmol 14: 80, 1982.

[8] 8. Feiring W, Feiring EH, Davidoff LM - Fibrous dysplasia of the skull. J Neurosurg 8: 377, 1951.

[9] 9. Fernandez E, Colavita N, Moschini M, Faleni A - «Fibrous dysplasia>> of the skull with complete unilateral cranial nerve involvement. J Neurosurg 5 2: 404, 1980.

[10] 10. Finney HL, Roberts TS - Fibrous dysplasia of the skull with progressive cranial nerve involvement. Surg Neurol 6: 341, 1976.

[11] 11. Fries JH - The roentgen features of fibrous dysplasia of the skull and facial bones: a critical analysis of thirty-nine pathologically proved cases. Am J Roentgenol 77: 71, 1957.

[12] 12. Graf CJ, Perret GE - Spontaneous recurrent hemorrhage as an unusual complication tf fibrous dysplasia of the skull. J Neurosurg 52: 570, 1980.

[13] 13. Harris WH, Dudley AR, Barry RJ - The natural history of fibrous dysplasia: an orthopaedic, pathological, and roentgenographlc study. J Bone Joint Surg 44: 207, 1962.

[14] 14. Harrison DFM - Osseous and fibro-osseous conditions affecting the cranio tacial bones. Ann Otol Rhinol Laryngol 9 3:199, 1984.

[15] 15. Ishiguro S, Ikeda M, Shimizu H, Hayashi M - Arteriovenous fistula as an unusual complication of polyostotic fibrous dysplasia of the skull. Surg Neurol 24: 681, 1985.

[16] 16. Ito H, Waga S, Sakakura M - Fibrous dysplasia of the skull with increased vascu.ar.ty in the angiogram. Surg Neurol 23:408, 1985.

[17] 17. Jackson IT, Armstrong HH, Gomuwka PK, Laws ER Jr, Langford K - Treatment of cranio-orbital fibrous dysplasia. J Max-Fac Surg 10: 138, 1982.

[18] 18. Kinnman JEG, Hong CE, Lee EB, Shin HS - .fibrous dysplasia of the face and skull. Pract Oto-Rhinolaryng 31: 11, 1969.

[19] 19. Lambert PR, Brackmann DE - Fibrous dysplasia of the temporal bone: the use of computerized tomography. Otolaryngol Head Neck Surg 92: 461, 1984.

[20] 20. Leeds N, Seaman WMB - Fibrous dysplasia of the skull and its differential diagnosis: a clinical and roentgenographic study of 46 cases. Radiology 78: 570, 1962.

[21] 21. Lichtenstein L - Polyostotic fibrous dysplasia. Arch Surg 36: 874, 1938.

[22] 22. Lichtenstein L, Jaffe HL - Fibrous dyplasia of bone. Arch Pathol 33: 777, 1942.

[23] 23. Lin JP, Goodkin R, Chase NE, Kricheff II - The angiographic featured or fibrous dysplasia of the skull. Radiology 92: 1275, 1969.

[24] 24. Mendelsohn DB, Hertzanu Y, Cohen M, Lello G - Computed tomography of craniofacial fibrous dysplasia. J Comput Assist Tomogr 8: 1062, 1984.

[25] 25. Montoya G, Evarts CM, Dohn DF - Polyostotic fibrous dysplasia and spinal cord compression: case report. J Neurosurg 29:102, 1968.

[26] 26. Nager GT, Holliday MJ - Fibrous dysplasia of the temporal bone: update w.th case reports. Ann Otol Rhinol Laryngol 93: 630, 1984.

[27] 27. Nishioka K, Masuda Y, Inokuchi I, Iyoda K, Tanaka T - A case of monostotic fibrous dysplasia of the temporal bone associated with epileptic seizure. Acta Med Okayama 36: 453, 1982.

[28] 28. Ramsey HE, Strong EW, Frazell EL - Fibrous dysplasia of the craniofacial bones. Am J Surg 116: 542, 1968.

[29] 29. Sassin JF, Rosenberg RN - Neurological complications of fibrous dysplasia of the skull. Arch Neurol 18: 363, 1968.

[30] 30. Schlumberger HG - Fibrous dysplasia of single bones (monostotic fibrous dysplasia). Milit Surgeon 99: 504, 1946.

[31] 31. Schwartz DT, Alpert M - The malignant transformation of fibrous dysplasia. Am J Med Sci 247: 1, 1964.

[32] 32. Sharp M - Monostotic fibrous dysplasia of the temporal bone. J Laryngol Otol 84: 697, 1970.

[33] 33. Taborelli A, Ballarini U, Monolo L, Dorizzi A - Cystic fibrous dysplasia of the skull presenting as an extradural mass: case report. J Neurosurg Sci 27: 253, 1983.

[34] 34. Zanini SA, Psillakis JM, Migowski W Jr, Tershakowec M - Craniofacial fibrous dysplasia. Ann Plast Surg 14: 378, 1985.