Fibrous Dysplasia of the Orbital Region: Current Clinical Perspectives in Ophthalmology and Cranio-Maxillofacial Surgery

Purpose: To describe the multidisciplinary diagnosis and treatment of patients with orbital fibrous dysplasia, a slowly progressive disease that may lead to asymmetry, disfigurement, and functional ocular problems. Methods: Ten patients with orbital fibrous dysplasia underwent bifrontal craniotomy through a coronal flap, with the removal of the supraorbital arch and dysplastic process involving the anterior and middle base of the skull. Four patients underwent superior orbital fissure and optic nerve canal decompression. Reconstruction was performed by using an autologous bone graft for both the adults and children, in whom a rib graft was preferred. The mean follow-up was 53.2 ± 18.3 months (range, 14 to 94 months). The patients’ preoperative status and postoperative status were compared. Results: The immediate and long-term morphologic and aesthetic results were good in all cases. All of the patients complained of some degree of diplopia during the immediate postoperative period, but the problem spontaneously resolved within 1 to 6 months in all but one case. No postoperative reduction in visual function was observed in the patients who underwent optic nerve decompression. The only reported complication was the irregular reabsorption of regrafted dysplastic bone in one patient. Conclusions: A multidisciplinary approach to orbital fibrous dysplasia is fundamental for treatment planning and execution.

[1]  Edmond FitzGibbon,et al.  Normal vision despite narrowing of the optic canal in fibrous dysplasia. , 2002, The New England journal of medicine.

[2]  T. Otsuka,et al.  Optic nerve decompression for orbitofrontal fibrous dysplasia. , 2002, Skull base : official journal of North American Skull Base Society ... [et al.].

[3]  C. Mautalen,et al.  Intravenous pamidronate increases bone mineral density and reduces bone remodeling markers in fibrous dysplasia , 2001 .

[4]  B. Horswell,et al.  Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review. , 2001, Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons.

[5]  F. Biglioli,et al.  Reconstruction of the orbital walls in surgery of the skull base for benign neoplasms. , 2000, International journal of oral and maxillofacial surgery.

[6]  J. Nerad,et al.  Ophthalmic manifestations of fibrous dysplasia: a disease of children and adults. , 1998, Ophthalmology.

[7]  R. Goldberg,et al.  Unilateral blindness after ipsilateral prophylactic transcranial optic canal decompression for fibrous dysplasia. , 1998, American journal of ophthalmology.

[8]  Yu-Ray Chen,et al.  Optic Nerve Decompression in Fibrous Dysplasia: Indications, Efficacy, and Safety , 1997, Plastic and reconstructive surgery.

[9]  徐鎖寬,et al.  [Fibrous dysplasia]. , 1972, Rontgenpraxis; Zeitschrift fur radiologische Technik.

[10]  D. Golding-Wood,et al.  Cranio-facial fibro-osseous dysplasia. A case report and overview of the management of the condition. , 1996, International journal of pediatric otorhinolaryngology.

[11]  C. Sasaki,et al.  Pathology of tumors of the cranial base. , 1995, Clinics in plastic surgery.

[12]  R. Mcfadzean,et al.  Fibrous dysplasia of the orbit. , 1994, The British journal of ophthalmology.

[13]  S. McCarthy,et al.  Monostotic fibrous dysplasia of the orbit: an unusual lacrimal fossa mass. , 1993, The British journal of ophthalmology.

[14]  I. Jackson,et al.  Experience in the use of calvarial bone grafts in orbital reconstruction. , 1992, The British journal of oral & maxillofacial surgery.

[15]  P. Sullivan,et al.  Advances in the management of orbital fractures. , 1992, Clinics in plastic surgery.

[16]  P. Alberius,et al.  Repositioning of craniofacial tumorous bone after autoclaving. , 1991, Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery.

[17]  M. S. Noordhoff,et al.  Treatment of Craniomaxillofacial Fibrous Dysplasia: How Early and How Extensive? , 1990, Plastic and reconstructive surgery.

[18]  R. Ruberg.  Soft Tissue Sarcomas: Histological Diagnosis , 1990 .

[19]  H. Vinters,et al.  Reversible visual loss caused by fibrous dysplasia. , 1990, American journal of ophthalmology.

[20]  Osguthorpe Jd,et al.  Orbital complications of fibrous dysplasia. , 1987 .

[21]  S. Gudeman,et al.  Orbital Complications of Fibrous Dysplasia , 1987, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

[22]  M. Edgerton,et al.  The surgical treatment of fibrous dysplasia. With emphasis on recent contributions from cranio-maxillo-facial surgery. , 1985, Annals of surgery.

[23]  W. Goldhahn,et al.  Fibröse Dysplasie der knöchernen Orbita mit «Neuritis retrobulbaris» , 1985 .

[24]  I. Munro,et al.  Fibrous dysplasia of the orbit in childhood. Clinical features and management. , 1985, Ophthalmology.

[25]  O. Sparrow,et al.  Craniofacial polyostotic fibrous dysplasia. , 1985, Journal of maxillofacial surgery.

[26]  K. Jones,et al.  McCune-Albright syndrome (fibrous dysplasia) associated with an orbital tumor. , 1984, Annals of ophthalmology.

[27]  I. Jackson,et al.  Treatment of cranio‐orbital fibrous dysplasia , 1983, Journal of maxillofacial surgery.

[28]  I. Jackson,et al.  Treatment of cranioorbital fibrous dysplasia , 1982 .

[29]  J. L. Martinez-Lage.  Bony Reconstruction in the Orbital Region , 1981, Annals of plastic surgery.

[30]  Munro Ir,et al.  Radical treatment for fronto-orbital fibrous dysplasia: the chain-link fence. , 1981 .

[31]  I. Munro,et al.  Radical Treatment for Fronto‐orbital Fibrous Dysplasia: The Chain‐Link Fence , 1981, Plastic and Reconstructive Surgery.

[32]  O. Melen,et al.  Fibrous dysplasia of bone with acute visual loss , 1980 .

[33]  G. Liakos,et al.  Ocular complications in craniofacial fibrous dysplasia. , 1979, The British journal of ophthalmology.

[34]  A. Schonder.  Fibrous dysplasia of bone with proptosis. , 1977, American journal of diseases of children.

[35]  E. M. Gamidov.  [Malignant transformation of fibrous dysplasia]. , 1977, Voprosy onkologii.

[36]  R. Retter.  Unilateral proptosis due to monostotic fibrous dysplasia. , 1976, Annals of ophthalmology.

[37]  T. Feintuch.  Chondrosarcoma arising in a cartilaginous area of previously irradiated fibrous dysplasia , 1973, Cancer.

[38]  P. Buxton,et al.  Fibrous dysplasia of the ethmoid presenting with proptosis , 1969, The British journal of surgery.

[39]  J. Sassin,et al.  Neurological complications of fibrous dysplasia of the skull. , 1968, Archives of neurology.

[40]  V. Desai,et al.  Polyostotic Fibrous Dysplasia , 1967, The Journal of Laryngology & Otology.

[41]  Gass Jd.  ORBITAL AND OCULAR INVOLVEMENT IN FIBROUS DYSPLASIA. , 1965 .

[42]  J. Gass.  ORBITAL AND OCULAR INVOLVEMENT IN FIBROUS DYSPLASIA. , 1965, Southern medical journal.

[43]  Irving M. Etkind.  Neoplasms of Bone and Related Conditions: Etiology, Pathogenesis, Diagnosis, and Treatment , 1962 .

[44]  W. Harris,et al.  The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. , 1962, The Journal of bone and joint surgery. American volume.

[45]  A. Tedeschi.  [Surgical treatment of fibrous dysplasia]. , 1958, Minerva ortopedica.

[46]  J. Fries.  The roentgen features of fibrous dysplasia of the skull and facial bones; a critical analysis of thirty-nine pathologically proved cases. , 1957, The American journal of roentgenology, radium therapy, and nuclear medicine.