Neurological Complications of Fibrous Dysplasia of the Skull

Sassin, Jon F.; Rosenberg, Roger N.

doi:10.1001/archneur.1968.00470340049004

Cited by 81 publications

(46 citation statements)

References 14 publications

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“…Radiological screening must include special views of the optic foramina, and surgical intervention is indicated when compression of the optic nerve becomes imminent. Unilateral involvement is the rule, but cases with bilateral visual impairment are found in the literature (Calderon et al, 1969;Sassis et al, 1968). Finney and Roberts (1976) described a case of fibrous dysplasia of the skull that developed trigeminal neuralgia, and we recently saw a case of 6th nerve palsy that was thought to be due to fibrous dysplasia.…”

Section: Discussionmentioning

confidence: 98%

Ocular complications in craniofacial fibrous dysplasia.

Liakos¹,

Walker²,

Carruth³

1979

British Journal of Ophthalmology

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SUMMARY Three cases of craniofacial fibrous dysplasia are reported, all presenting to the ophthalmologist first. They all had ocular complications due either to direct involvement of the orbital bones or to secondary complications. Sphenoidal mucocele is added to the other complications previously reported, and accordingly a classification of the various ways that fibrous dysplasia can affect the ocular structures is proposed.

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Section: Discussionmentioning

confidence: 98%

Ocular complications in craniofacial fibrous dysplasia.

Liakos¹,

Walker²,

Carruth³

1979

British Journal of Ophthalmology

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“…Visual loss also represents the most common neurological complication of fibrous dysplasia affecting the skull. 44 As such, visual impairment has received considerable attention; much has been published on its causes and putative treatment. Elucidation of underlying causes of visual impairment has important implications for the planning of therapeutic interventions: treatments must be targeted against appropriate pathological substrates and not on coincident and unrelated phenomena.…”

Section: Visual Impairment In Fibrous Dysplasiamentioning

confidence: 99%

“…Advocates of this belief considered that the concomitant observations of fibrous dysplasia involving the optic canal and chronic visual loss proved that the former was the root cause of the latter. 11,17,44 Clearly, optic canal stenosis can be entirely asymptomatic or a coincident occurrence in the face of another cause of visual loss. Moreover, the relationship between the frequency of optic canal stenosis and visual loss has never been proven.…”

Section: Surgical Managementmentioning

confidence: 99%

Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management

Dumont

Boulos²,

Jane³

et al. 2001

FOC

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Fibrous dysplasia is a benign but slowly progressive disorder of bone in which normal cancellous bone is replaced by immature woven bone and fibrous tissue. Significant deformity and both acute and chronic visual impairment can result. A contemporary understanding of fibrous dysplasia, emphasizing the origins of visual impairment, indications for decompressive surgery, and the techniques for correction of the cosmetic deformity are presented. In their experience and review of the literature, the authors found the most frequent clinical presentations to be exophthalmos, displacement of the globe, abnormalities of extraocular motility, cosmetic deformity, and visual impairment. Although traditionally the cause of visual impairment has been ascribed to impingement of the optic canal on the optic nerve, the authors' experience is that the most common cause of visual loss is cystic degeneration of the tumor, particularly with those involving the anterior clinoid process. Exophthalmos and optic canal stenosis are less common causes of visual impairment. Indications for surgical intervention include acute and/or serially radiographically documented and relentless visual impairment and significant cosmetic deformity. Individualized management strategies are also discussed.

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“…Skull involvement occurs in 27% of monostotic patients and in up to 50% of polyostic patients. 16) Clinical findings include asymptomatic enlargement of the involved bone, resulting in facial asymmetry, tilting of the occlusal plane, loss of teeth, and grotesque facial deformity. In some patients, fibrous dysplasia involves most of the craniomaxillofacial bone, causing megacranium and an appearance known as`lion face.'…”

Section: Introductionmentioning

confidence: 99%

Long-Term Outcome of Fibrous Dysplasia: Reconstruction With Dysplastic Bone -Case Report-

Sakamoto

Nakajima

Tamada

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 10-year-old girl presented with facial asymmetry associated with bulging of the right fronto-orbital region with no symptoms. Computed tomography demonstrated enlargement of the right zygomatic, frontal, ethmoid, and sphenoid bones. Abnormal proliferation of the bone had obliterated the sphenoid, right frontal sinus, and right ethmoid sinuses. These radiological findings suggested right optic nerve compression due to fibrous dysplasia. Right optic canal decompression was performed. In preparation for recurrence, the resulting bone defect in the right orbital roof was reconstructed using the outer table of the split lesion bone. The removed frontal bone was divided into intact and lesioned parts, and the intact part was returned. The lesioned part was split and the outer table graft used to reconstruct the frontal region. A temporal musculopericranial flap was used to form a barrier between the opened ethmoid sinus and cranial cavity. A protrusion appeared on the left forehead 10 years later, and was shaved to improve the aesthetic appearance. The patient was followed up for a total of 23 years. The use of dysplastic bone involves the risk of recurrence, but the period of recurrence is delayed and the progression stops after adolescence, so the second operation involved only shaving for aesthetic appearance. This procedure is one of the treatments of choice because of easy reconstruction, easy revision, and good results.

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Neurological Complications of Fibrous Dysplasia of the Skull

Cited by 81 publications

References 14 publications

Ocular complications in craniofacial fibrous dysplasia.

Ocular complications in craniofacial fibrous dysplasia.

Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management

Long-Term Outcome of Fibrous Dysplasia: Reconstruction With Dysplastic Bone -Case Report-

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