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1 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDNeurofibromatosis, type 1, is an autosomal dominant phakomatosis characterized by Lisch nodules of the iris (hamartomas) plexiform neurofibromas, café-au-lait spots on the skin, and axillary freckling. Intracranial tumors such as optic pathway gliomas may occur. Disease/Diagnosis: Neurofibromatosis...
2 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDTuberous sclerosis is an autosomal dominant phakomatosis characterized by astrocytic hamartomas of the retina or optic nerve, adenoma sebaceum of the face, periungual fibromas, and astrocytic hamartomas of the brain, with secondary seizures and mental retardation. Disease/Diagnosis: Tuberous Scleros...
3 Isolated Congenital Optic Disc AnomaliesWilliam Fletcher Hoyt, MDThis patient demonstrates bilateral tilted optic discs. Patients with this congenital optic disc anomaly may be asymptomatic or have bitemporal visual field defects that do not respect the vertical midline. Disease/Diagnosis: Tilted Discs.
4 Isolated Congenital Optic Disc AnomaliesWilliam Fletcher Hoyt, MDThis patient demonstrates bilateral tilted optic discs. Patients with this congenital optic disc anomaly may be asymptomatic or have bitemporal visual field defects that do not respect the vertical midline. Disease/Diagnosis: Tilted Discs.
5 Isolated Congenital Optic Disc AnomaliesWilliam Fletcher Hoyt, MDThis patient demonstrates bilateral tilted optic discs. Patients with this congenital optic disc anomaly may be asymptomatic or have bitemporal visual field defects that do not respect the vertical midline. Disease/Diagnosis: Tilted Discs.
6 Isolated Congenital Optic Disc AnomaliesWilliam Fletcher Hoyt, MDThis patient demonstrates bilateral tilted optic discs. Patients with this congenital optic disc anomaly may be asymptomatic or have bitemporal visual field defects that do not respect the vertical midline. Disease/Diagnosis: Tilted Discs.
7 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDTuberous sclerosis is an autosomal dominant phakomatosis characterized by astrocytic hamartomas of the retina or optic nerve, adenoma sebaceum of the face, periungual fibromas, and astrocytic hamartomas of the brain, with secondary seizures and mental retardation. Disease/Diagnosis: Tuberous Scleros...
8 Traumatic Internal Carotid Artery DissectionSteven Galetta, MDTraumatic dissection of the carotid artery may result in neck pain, an ipsilateral Horner's syndrome (disruption of the pericarotid sympathetic fibers), or ipsilateral arterial occlusions from embolic disease. Pair with images 91_18 and 91_20.
9 Traumatic Internal Carotid Atery DissectionSteven Galetta, MDTraumatic dissection of the carotid artery may result in neck pain, an ipsilateral Horner's syndrome (disruption of the pericarotid sympathetic fibers), or ipsilateral arterial occlusions from embolic disease. Pair with images 91_19 and 91_20.
10 Magnetic Resonance Imaging in Detection of Extracranial Internal Carotid Artery DissectionMarilyn C. Kay, MDThis 28-year-old woman presented with a 4-week history of bilateral visual loss. She had a known history of multiple sclerosis. Her vision was 20/60 OD and 20/40 OS, with an RAPD OS and optic pallor OU. Her fields and MRI are shown. Optic tract lesions usually result in an incongruous homonymous hem...
11 Neuro-Ophthalmic Imaging-Cerebral AngiographyMark J. Kupersmith, MDEhlers-Danlos syndrome is a connective tissue disorder that may affect blood vessels and predispose some affected patients to development of carotid cavernous fistula. Most patients with high-flow direct carotid cavernous sinus fistulas have suffered acute traumatic tears in the internal carotid art...
12 Neuro-Ophthalmic Consequences of TherapyMark J. Kupersmith, MDradiation retinopathy may mimic diabetic or hypertensive optic neuropathy. A history of irradiation to the eye, orbit, or head is mandatory. Radiation retinopathy usually occurs many months after radiation therapy.
13 Neuro-Ophthalmic Consequences of TherapyMark J. Kupersmith, MDradiation retinopathy may mimic diabetic or hypertensive optic neuropathy. A history of irradiation to the eye, orbit, or head is mandatory. Radiation retinopathy usually occurs many months after radiation therapy.
14 Traumatic Internal Carotid Artery DissectionSteven Galetta, MDTraumatic dissection of the carotid artery may result in neck pain, an ipsilateral Horner's syndrome (disruption of the pericarotid sympathetic fibers), or ipsilateral arterial occlusions from embolic disease. Pair with images 91_18 and 91_19.
15 Neuro-Ophthalmic Consequences of TherapyMark J. Kupersmith, MDradiation retinopathy may mimic diabetic or hypertensive optic neuropathy. A history of irradiation to the eye, orbit, or head is mandatory. Radiation retinopathy usually occurs many months after radiation therapy.
16 Neuro-Ophthalmic Consequences of TherapyMark J. Kupersmith, MDRadiation causes a vascular retinopathy that may mimic diabetic or hypertensive retinopathy. It does not develop until many months or several years after radiation therapy to the eye, orbit or head.
17 Neuro-Ophthalmic Vascular DiseaseMark J. Kupersmith, MDAneurysms of the intracranial circulation may act as mass lesions and compress the afferent of efferent visual pathway. Ophthalmic artery aneurysms may compress the optic nerve and result in an optic neuropathy (ie, visual loss, afferent pupillary defect, optic atrophy). Treatment includes endovascu...
18 Neuro-Ophthalmic Vascular DiseaseMark J. Kupersmith, MDAneurysms of the intracranial circulation may act as mass lesions and compress the afferent of efferent visual pathway. Ophthalmic artery aneurysms may compress the optic nerve and result in an optic neuropathy (ie, visual loss, afferent pupillary defect, optic atrophy). Treatment includes endovascu...
19 Optic NeuropathiesMichael Wall, MDOptic disc edema with a macular star figure may occur in infectious diseases (eg, cat-scratch disease, syphilis, tuberculosis, Lyme disease), inflammatory diseases (eg, sarcoid), ischemic diseases (anterior ischemic optic neuropathy), and in papilledema. Infectious causes should be sought in patient...
20 Isolated Optic Neuritis/NeuropathyMark J. Kupersmith, MDPapilledema may produce visual loss due to chronic atrophic papilledema, secondary macular hemorrhage, exudate or edema, secondary ischemic optic neuropathy, or secondary subretinal neovascular membrane formation. Patients with papilledema and visual loss should be suspected of harboring one of thes...
21 Isolated Optic Neuritis/NeuropathyMark J. Kupersmith, MDPapilledema may produce visual loss due to chronic atrophic papilledema, secondary macular hemorrhage, exudate or edema, secondary ischemic optic neuropathy, or secondary subretinal neovascular membrane formation. Patients with papilledema and visual loss should be suspected of harboring one of thes...
22 Systemic Disorders With Optic Nerve and Retinal FindingsMark J. Kupersmith, MDSarcoidosis is an inflammatory granulomatous disease that may result in inflammatory or infiltrative optic neuropathology or retinal vasculitis. Pair with 91_34.
23 Systemic Disorders With Optic Nerve and Retinal FindingsMark J. Kupersmith, MDSarcoidosis is an inflammatory granulomatous disease that may result in inflammatory or infiltrative optic neuropathology or retinal vasculitis. Pair with 91_35.
24 Optic NeuropathiesRalph A. Sawyer, MDOptic disc edema with a macular star figure has been referred to as neuroretinitis.
25 Neuro-Ophthalmic Vascular DiseaseMitchell J. Wolin, MDCarotid cavernous fistulas (CCFs) are connections between the arterial blood flow from the carotid artery system and the cavernous sinus. CCFs may be direct high-flow fistulas or indirect low-flow fistulas. Most direct CCFs are due to trauma. Enlargement of the superior ophthalmic vein may be demons...
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