Normal Vision despite Narrowing of the Optic Canal in Fibrous Dysplasia

Lee, Janice S.; FitzGibbon, Edmond J.; Butman, John A.; Dufresne, Craig R.; Kushner, Harvey; Wientroub, Shlomo; Robey, Pamela Gehron; Collins, Michael T.

doi:10.1056/nejmoa020742

Cited by 191 publications

(169 citation statements)

References 21 publications

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“…Hypersomatotropinemia in MAS can be associated with significant morbidity due to exacerbation of polyostotic fibrous dysplasia in the presence of elevated GH levels [106,113]. Hypersomatotropinemia has also be implicated in sarcomatous transformation of bone tumors in a MAS patient [114].…”

Section: Molecular Genetics Of Pituitary Tumorsmentioning

confidence: 99%

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Keil

Stratakis

2008

Expert Review of Neurotherapeutics

123

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Pituitary tumors are rare in childhood and adolescence, with a reported prevalence of up to 1 per million children. Only 2 -6% of surgically treated pituitary tumors occur in children. Although pituitary tumors in children are almost never malignant and hormonal secretion is rare, these tumors may result in significant morbidity. Tumors within the pituitary fossa are of two types mainly, craniopharyngiomas and adenomas; craniopharyngiomas cause symptoms by compressing normal pituitary, causing hormonal deficiencies and producing mass effects on surrounding tissues and the brain; adenomas produce a variety of hormonal conditions such as hyperprolactinemia, Cushing disease and acromegaly or gigantism. Little is known about the genetic causes of sporadic lesions, which comprise the majority of pituitary tumors, but in children, more frequently than in adults, pituitary tumors may be a manifestation of genetic conditions such as multiple endocrine neoplasia type 1 (MEN 1), Carney complex, familial isolated pituitary adenoma (FIPA), and McCune-Albright syndrome. The study of pituitary tumorigenesis in the context of these genetic syndromes has advanced our knowledge of the molecular basis of pituitary tumors and may lead to new therapeutic developments.

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Section: Molecular Genetics Of Pituitary Tumorsmentioning

confidence: 99%

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Keil

Stratakis

2008

Expert Review of Neurotherapeutics

123

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“…[36][37][38] In a cross-sectional survey of unselected patients with sphenoid and optic canal involvement, Lee et al 39 found no correlation between visual loss and canal stenosis on quantitative radiography. 39 Typically, the mechanisms for the optic neuropathy in fibrous dysplasia patients include acute compression from a secondary mass lesion such as a bone cyst with or without haemorrhage, sphenoid-ethmoid mucocoele, or sarcomatous degeneration. Chronic optic nerve traction may also play a role in some cases.…”

Section: Fibrous Dysplasia and Other Primary Bone Diseasementioning

confidence: 99%

Optic nerve disorders: role of canal and nerve sheath decompression surgery

Acheson

2004

Eye

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Optic nerve sheath decompression (ONSD) maintains a role in the management of visual loss complicating papilloedema in selected patients primarily with idiopathic intracranial hypertension. The evidence base for this intervention is reviewed and audit data on visual outcomes for patients with acute, chronic, and atrophic forms of papilloedema are contrasted. Optic canal decompression has a role in the management of compressive optic neuropathies complicating mass lesions arising from paranasal sinuses and intracranially and can be achieved by transethmoidal, transbasal, and open craniotomy routes. The evidence base supporting this intervention in traumatic optic neuropathy and in primary bone disease causing canal stenosis (in particular fibrous dysplasia) is reviewed where the indications are more controversial.

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“…precocious puberty and hyperthyroidism) known as the McCune Albright syndrome (MAS) [4,5]. The symptoms associated with FD are well described in the literature, with some series reporting up to one-third of patients having visual complaints, including blurred vision, diplopia, and epiphora [6]. The most devastating consequence of CFD is the loss of vision due to optic nerve compression (ONC).…”

Section: Introductionmentioning

confidence: 99%

“…The most devastating consequence of CFD is the loss of vision due to optic nerve compression (ONC). Although radiological evidence of ONC in CFD is found in 50-90% of the patients [6], the management of these patients is more often based on personal experience and/or expert opinion.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Surgery versus Watchful Waiting in Patients with Craniofacial Fibrous Dysplasia: A Meta-Analysis

Amit¹,

Collins²,

FitzGibbon³

et al. 2012

J Neurol Surg B

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Background: Fibrous dysplasia (FD) is a benign bone tumor which most commonly involves the craniofacial skeleton. The most devastating consequence of craniofacial FD (CFD) is loss of vision due to optic nerve compression (ONC). Radiological evidence of ONC is common, however the management of this condition is not well established. Our objective was to compare the long-term outcome of patients with optic nerve compression (ONC) due to craniofacial fibrous dysplasia (CFD) who either underwent surgery or were managed expectantly.

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Normal Vision despite Narrowing of the Optic Canal in Fibrous Dysplasia

Cited by 191 publications

References 21 publications

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Optic nerve disorders: role of canal and nerve sheath decompression surgery

Surgery versus Watchful Waiting in Patients with Craniofacial Fibrous Dysplasia: A Meta-Analysis

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