Craniofacial fibrous dysplasia of the fronto-orbital region: A case series and literature review

doi:10.1053/joms.2001.20487

Journal of Oral and Maxillofacial Surgery

Volume 59, Issue 2, February 2001, Pages 157-167

https://doi.org/10.1053/joms.2001.20487 Get rights and content

Abstract

Purpose: Craniofacial fibrous dysplais (CFD), involving the fronto-orbital region often is challenging to treat because of the proximity of neurovascular and ocular structures. This article presents the surgical experience with 6 patients. Patients and Methods: Six patients ranging in age from 7 to 23 years, who had undergone surgery for CFD of the orbital region, were retrospectively reviewed. All patients had progressive complaints of deformity, pain, paresthesia or visual disturbances. Surgery generally involved extensive tumor excision and immediate orbital reconstruction with autogenous bone grafts. Two patients also were reconstructed with resected and treated autogenous bone, which then was immediately reimplanted, using rigid fixation. Three patients also had intracranial microsurgical optic canal decompression. All patients received perioperative corticosteroids. Results: Five patients experienced partial relief of their sensory and visual disturbances. These patients also felt that there had been improvement in facial appearance. One patient with very extensive circumferential optic canal involvement, and who underwent 2-wall decompression, suffered visual loss. Conclusion: Early surgery to address progressive sensory disturbances is recommended so as to avoid the hazards of late-stage decompression. The risks of surgery must be weighed against the benefits of achieving a more functional and aesthetic result. Long-term neuro-ophthalmologic monitoring is essential. © 2001 American Association of Oral and Maxillofacial Surgeons

Section snippets

History

Fibrous dysplasia was first reliably reported 1 century ago by von Recklinghausen,¹ when he described patients with a pathologic condition of the bone characterized by deformity and fibrotic changes that he termed osteitis fibrosa generalisata. In 1938, Lichtenstein and Jaffe² first introduced the term fibrous dysplasia. They also noted that fibrous dysplasia of bone is a condition that can affect single as well as multiple bones. The relationship between patients with these lesions and an

Patients and methods

Six patients with craniofacial fibrous dysplasia involving the orbital region were retrospectively reviewed for clinical presentation, management, and postoperative course. These patients were all treated by the senior author (B.B.H.) through a university-based craniofacial program in a team approach with pediatric neurosurgery and neuro-ophthalmology. All patients had extensive recorded clinical histories and thorough radiologic workups; 4 patients had a tissue diagnosis consistent with

Results

The patients ranged in age from 7 to 23 years, with a mean age of 17 years. There were 3 males and 3 females. All had confirmed CFD involving the orbital region. However, 2 patients, Nos. 1 and 6, had midline involvement at the sella. Five patients had clinical onset of their disease in childhood or early adolescence, but had recently experienced increasing ocular symptoms, primarily diplopia, and blurring of vision. One patient (No. 1) became symptomatic at 21 years of age. However, his

Discussion

The main areas of controversy relative to the management of CFD of the fronto-orbital region relate to the timing of surgery, the role of optic nerve decompression, reconstruction options, and the management of postoperative complications.

Acknowledgements

The authors thank Dr James W. Karesh, Oculoplastic Surgeon and Chief of Ophthalmology, the Sinai Hospital of Baltimore, Baltimore, MD; Dr Remy Blanchaert, Maxillofacial Surgeon, University of Maryland Medical Center, Baltimore, MD; and Dr Arthur DiPatri, Pediatric Neurosurgeon, University of Maryland Medical Center, Baltimore, MD for their continued follow-up and management of these patients.

References (50)

IT Jackson et al.
Treatment of cranio-orbital fibrous dysplasia
J Maxillofac Surg
(1982)
BJ Katz et al.
Ophthalmic manifestations of fibrous dysplasia: A disease of children and adults
Ophthalmology
(1998)
DW Nuss et al.
Craniofacial disassembly in the management of skull-base tumors
Otolaryngol Clin North Am
(1991)
PF Bradley
A 2-stage procedure for reimplantation of autogenous freeze-treated mandibular bone
J Oral Maxillofac Surg
(1982)
PF Bradley
Modern trends in cryosurgery of bone in the maxillo-facial region
Int J Oral Surg
(1978)
YJ Dong et al.
The use of immediate frozen autogenous mandible for benign tumour mandibular reconstruction
Br J Oral Maxillofacial Surg
(1996)
K Saito et al.
Unilateral extradural approach for bilateral optic canal release in a patient with fibrous dysplasia
Surg Neurol
(1990)
IT Jackson et al.
Craniofacial tumors
Clin Plast Surg
(1994)
C Edelstein et al.
Unilateral blindness after ipsilateral prophylactic transcranial optic canal decompression for fibrous dysplasia
Am J Ophthalmol
(1998)
M Perry et al.
The use of computer-generated three-dimensional models in orbital reconstruction
Br J Oral Maxillofac Surg
(1998)

R Brusati et al.

Reconstruction of the orbital walls in surgery of the skull base for benign neoplasms

Int J Oral Maxillofac Surg

(2000)

FD VonRecklinghausen

Die fibrose oder deformierende Ostitis, die Osteomalacie und die osteoplastische Carcinose in ihren gegenseitigen Beziehungen

(1891)

L Lichtenstein et al.

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

(1942)

DJ McCune

Osteitis fibrosa cystica: The case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism

Am J Dis Child

(1936)

F Albright et al.

Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females

N Engl J Med

(1937)

S Hoffman et al.

Fibrous dysplasias: Benign nonodontogenic tumors of the jaws

MT Edgerton et al.

The surgical treatment of fibrous dysplasia with emphasis on recent contributions from cranio-maxillo-facial surgery

Ann Surg

(1985)

IR Munro et al.

Periorbital tumors

IR Munro et al.

Radical treatment for fronto-orbital fibrous dysplasia: The chain-link fence

Plast Reconstr Surg

(1981)

PJ Marie et al.

Increased proliferation of osteoblastic cells expressing the activating Gs a mutation in monostotic and polyostotic fibrous dysplasia

Am J Pathol

(1997)

M Riminucci et al.

Fibrous dysplasia of bone in the McCune-Albright syndrome: Abnormalities in bone formation

Am J Pathol

(1997)

T Yamamoto et al.

Increased IL-6 production by cells isolated from the fibrous bone dysplasia tissues in patients with McCune-Albright syndrome

J Clin Invest

(1996)

RS Weinstein

Long-term aminobiphosphonate treatment of fibrous dysplasia

J Bone Miner Res

(1997)

WH Harris et al.

The natural history of fibrous dysplasia: An orthopedic, pathological and roentgenographic study

J Bone Joint Surg Am

(1962)

ML Davies et al.

Fibrous dysplasia of the skull: Disease activity in relation to age

Br J Radiol

(1991)

Cited by (134)

Craniofacial fibrous dysplasia: Systematic review of facial management
2023, Journal of Stomatology, Oral and Maxillofacial Surgery
Craniofacial fibrous dysplasia (CFD) may be associated with major cosmetic or functional consequences. However, management recommendations for CFD are currently unavailable. Therefore, this systematic literature review aimed to review the existing approaches for CFD management and propose a management algorithm. The focus question was “What are the different options for CFD treatment and their complication rates?” The MEDLINE database was searched, and 33 articles evaluating a total of 1154 patients were reviewed. The bias assessment showed that 20 of the 33 studies had a high or intermediate risk of bias, mainly because of retrospective data collection and small patient numbers. Radical surgery showed a lower recurrence rate than debulking, but its use should be weighed against the morbidity caused by the reconstruction performed in this technique. Orbital decompression using a radical technique or debulking is effective in cases showing exophthalmos or dystopia. Surveillance is a viable option for asymptomatic and/or non-progressive lesions. In cases showing optic nerve compression, prophylactic decompression should be avoided, and decompression should be performed only when patients show diminished visual acuity or visual field defect. Although bisphosphonates have shown efficacy in pain management, their posology requires further discussion. A management algorithm is presented.
Fibrous dysplasia of the zygomatic bone and mandibular coronoid process: A case report
2023, Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
Fibrous dysplasia (FD) is a benign bone disease characterized by the replacement of normal bone with fibro-osseous connective tissue. Although FD may occur in the facial bones, it rarely occurs in both the zygomatic bone and mandible simultaneously. We herein describe a 59-year-old woman who developed FD in the zygomatic bone and mandibular coronoid process. She was referred to our hospital because of the presence of a radiopaque area around the maxillary tuberosity. Computed tomography showed a bell-shaped, radiopaque mass in the right zygomatic bone and upward extension of the right coronoid process. Although we biopsied the lesion of the zygomatic bone, we could not precisely diagnose the lesion. The lesion was excised intraorally under general anesthesia. Histological examination revealed FD in the zygomatic bone and coronoid process. Postoperative computed tomography showed a residual lesion in the zygomatic bone, but there was no enlargement even 1 year postoperatively.
Computer-guided contouring of craniofacial fibrous dysplasia involving fronto-orbital or fronto-cranial region using patient specific surgical depth guide: A prospective case series
2023, Journal of Cranio-Maxillofacial Surgery
The aim of this study was to introduce a new computer guided technique for debulking and contouring the craniofacial fibrous dysplasia involving the fronto-orbital and fronto-cranial regions.
Computer-guided contouring was performed using a modified patient-specific surgical depth guide for six patients with craniofacial fibrous dysplasia involving the fronto-orbital and fronto-cranial regions. Virtual planning was performed to determine the desired amount of bone removal and construct the patient-specific surgical depth guide. Then, the guide was printed using rapid prototyping. In the surgical theatre, the guide was seated in position. Implant drills were inserted through the created depth holes according to the planned fixed depth to create depth holes. Finally, the bone in between the created holes was removed using cutting discs, bone chisels and surgical burs. Satisfaction with facial aesthetics was evaluated by the patients using a Likert scale, and by the surgeons using the Whitaker rating scale. The surgical procedures were uneventful for all the patients. All the patients were satisfied with the post-operative facial esthetics and categorized as category I Whitaker rating scale.
Patient-specific surgical guide technique for recontouring of fronto-orbital and fronto-cranial fibrous dysplasia can be considered an accurate substitution technique that overcomes the drawbacks of the unpredictable conventional one. Further investigations are required.
Benign bony tumors of the paranasal sinuses, orbit, and skull base
2022, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Benign bony tumors of the skull base and paranasal sinuses are uncommon entities, with an overall higher incidence in males. Benign bony tumors may lead to local expansion with resultant mass effect of potentially critical structures. Some benign bony tumors may undergo malignant transformation. This article reviews the presentation and management of benign bone tumors of the skull base and paranasal sinuses with special consideration to involvement of the adjacent orbit, intracranial and critical neurovascular structures. This review covers tumor incidence, location, gross and histologic appearance as well as radiographic findings, treatment, and recurrence rates. Tumors discussed in this article include osteochondromas, osteomas, osteoid osteomas, aneurysmal bone cysts, fibrous dysplasia, giant cell tumors, cemento-ossifying fibroma, ameloblastic fibro-odontoma, ecchordosis physaliphora, chondromyxoid fibroma, primary chronic osteomyelitis, primary chronic osteomyelitis, osteochondromyxoma, and dense bone islands.
Fibrous dysplasia of the clivus – A case study and literature review
2021, Radiology Case Reports
Fibrous dysplasia is a benign, congenital skeletal disorder which leads to the formation of fibro-osseous intramedullary bone lesions. Clival fibrous dysplasia is a rare variant which commonly presents asymptomatically with no findings on examination and is often picked up incidentally on radiological investigation. A 39-year-old female presented with a sudden onset headache of 3 days’ duration alongside diplopia and right lower limb weakness upon examination. Computerized tomography head scan revealed an expansile clivus with a ground-glass appearance, magnetic resonance imaging brain scan revealed a predominantly hypointense signal on T1- and T2-weighted images and subsequent whole-body bone imaging confirmed the diagnosis of monostotic clival fibrous dysplasia. This case highlights the importance of considering monostotic clival fibrous dysplasia as a differential diagnosis in patients presenting with sudden onset symptoms of headache alongside cranial and peripheral nerve involvement, when other more sinister causes have been excluded.
Quantitative evaluation of symmetry after navigation-guided surgical recontouring of zygomatic fibrous dysplasia: a comparative study
2020, International Journal of Oral and Maxillofacial Surgery
Zygomatic fibrous dysplasia is a slowly progressive disorder of bone, which commonly causes facial asymmetry. Precise surgical recontouring can effectively improve facial aesthetics. The aim of this study was to quantitatively evaluate the effectiveness of surgical navigation guidance in correcting zygomatic asymmetry. The study included 26 patients with unilateral zygomatic fibrous dysplasia who underwent bony recontouring. They were divided into two groups according to the use of intraoperative surgical navigation (navigation group and conventional group; n = 13 in each group). Clinical outcomes were evaluated using three-dimensional computed tomography. Six pairs of landmarks were identified, and the coordinates were used to calculate asymmetry indices. The curvature of protruding regions in the surgical area was measured to determine facial skeletal symmetry in three dimensions. The results were compared between the groups. All patients recovered uneventfully and their facial symmetry and aesthetics improved. For three of the six pairs of landmarks, symmetry was better in the navigation group than in the conventional group (all P ≤ 0.02). The postoperative curvature ratios were better (more symmetrical) in the navigation group (P = 0.01). Surgical navigation enhances postoperative facial symmetry. However, the clinical significance of this enhancement when compared to conventional non-navigation surgery needs further investigation.

View all citing articles on Scopus

^*: †Formerly, Assistant Professor, Department of Oral-Maxillofacial Surgery, University of Maryland Medical Systems, Baltimore, MD; Currently, Private Practice, Maplewood, MN.

^**: Address correspondence and reprint request to Dr Horswell: 1560 Beam Ave, Maplewood, MN 55109; e-mail: [email protected]

View full text

Clinical ArticlesCraniofacial fibrous dysplasia of the fronto-orbital region: A case series and literature review*,**

Abstract

Section snippets

History

Patients and methods

Results

Discussion

Acknowledgements

J Maxillofac Surg

Ophthalmology

Otolaryngol Clin North Am

J Oral Maxillofac Surg

Int J Oral Surg

Br J Oral Maxillofacial Surg

Surg Neurol

Clin Plast Surg

Am J Ophthalmol

Br J Oral Maxillofac Surg

Int J Oral Maxillofac Surg

Die fibrose oder deformierende Ostitis, die Osteomalacie und die osteoplastische Carcinose in ihren gegenseitigen Beziehungen

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

Osteitis fibrosa cystica: The case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism

Am J Dis Child

Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females

N Engl J Med

Fibrous dysplasias: Benign nonodontogenic tumors of the jaws

The surgical treatment of fibrous dysplasia with emphasis on recent contributions from cranio-maxillo-facial surgery

Ann Surg

Periorbital tumors

Radical treatment for fronto-orbital fibrous dysplasia: The chain-link fence

Plast Reconstr Surg

Increased proliferation of osteoblastic cells expressing the activating Gs a mutation in monostotic and polyostotic fibrous dysplasia

Am J Pathol

Fibrous dysplasia of bone in the McCune-Albright syndrome: Abnormalities in bone formation

Am J Pathol

Increased IL-6 production by cells isolated from the fibrous bone dysplasia tissues in patients with McCune-Albright syndrome

J Clin Invest

Long-term aminobiphosphonate treatment of fibrous dysplasia

J Bone Miner Res

The natural history of fibrous dysplasia: An orthopedic, pathological and roentgenographic study

J Bone Joint Surg Am

Fibrous dysplasia of the skull: Disease activity in relation to age

Br J Radiol

Clinical Articles
Craniofacial fibrous dysplasia of the fronto-orbital region: A case series and literature review*,**