Use of corticosteroid sparing systemic immunosuppression for treatment of corticosteroid dependent optic neuritis not associated with demyelinating disease.

AIM: To describe the authors' experience and that in the published literature regarding the use of corticosteroid sparing systemic immunosuppression for patients with corticosteroid dependent optic neuritis not associated with demyelinating disease. METHODS: The records of 10 patients from the authors' clinical database, and 38 patients from the published literature with corticosteroid dependent optic neuritis, were retrospectively reviewed to determine patient demographics, diagnosis, clinical course, and outcomes. These patients had recrudescence of symptoms, such as decreased vision and pain, with attempted taper of corticosteroid. Many of these patients also suffered side effects from systemic corticosteroid use such as weight gain and uncontrolled hyperglycaemia. Antimetabolites (for example, methotrexate and azathioprine), cyclosporine and/or alkylating agents (for example, cyclophosphamide and chlorambucil) were given to enable taper of corticosteroid while effectively controlling optic neuritis. RESULTS: The study included 43 women and 5 men: 17 patients with systemic lupus erythematosus, 12 patients with sarcoidosis, 3 with other systemic autoimmune diseases, and 16 with no clinically identifiable systemic association. 79% of all patients benefited from the use of systemic immunosuppression in that they had successful corticosteroid taper, control of inflammation, improvement in symptoms, and/or tolerance of adverse effects. Mild toxicity was common and 19% of patients, most often those taking cyclophosphamide, discontinued medication because of adverse effects. 24 of 28 (86%) patients on alkylators benefited clinically, while 20 of 29 (69%) patients on antimetabolites had clinical benefit. CONCLUSION: Systemic immunosuppression may be a safer and more effective treatment alternative to chronic oral corticosteroid use in cases of corticosteroid dependent optic neuritis not associated with demyelinating disease.

Monocular blindness as a complication of trigeminal radiofrequency rhizotomy.

PURPOSE: To raise the awareness of the rare complication of monocular blindness resulting from radiofrequency rhizotomy for trigeminal neuralgia. METHODS: Case series from tertiary referral centers. Patients were referred after complaint of loss of vision. RESULTS: We report three patients who had trigeminal neuralgia and underwent treatment by radiofrequency rhizotomy. Each developed complete loss of vision in one eye immediately after the procedure. One patient died of an unrelated cause, and pathologic analysis revealed changes compatible with acute trauma to the right optic nerve. CONCLUSION: Our third case confirms the hypothesis that traumatic optic neuropathy after trigeminal radiofrequency rhizotomy results from malpositioning of the rhizotomy needle through the inferior orbital fissure into the orbital apex rather than the foramen ovale.

Secondary fractures of Le Fort I osteotomy.

PURPOSE: To report the ophthalmic complications of Le Fort I osteotomy for the correction of dentofacial deformities and to determine the maximal compressive loads applied during pterygomaxillary separation in a cadaver model. METHODS: Two cases of ophthalmic complications arising after Le Fort I osteotomy are reported. Le Fort I osteotomy was performed on five cadavers. The maximal compressive load applied during pterygomaxillary separation was recorded with a 10 kN (3,000 lbf) load cell of a MTS Mini-Bionix servo-hydraulic machine (MTS, Eden Prairie, MN, U.S.A.). A paired t test was used to compare forces applied to the right and left sides. Computed tomography scans of each specimen were obtained after Le Fort I osteotomy to document secondary fractures. The skulls were subsequently stained with 1% fuschin red to highlight secondary fractures. RESULTS: Maximum compressive loads during pterygomaxillary separation ranged from 22 N (5.0 lbf) to 162 N (36.5 lbf), with an average of 106 N (23.8 lbf) (SD 47.6 N [10.7 lbf]). Forces applied on the first operative side were significantly greater than forces applied on the second operative side (p = 0.0034). Secondary fractures were found in three specimens by computed tomography and in two specimens by 1% fuschin red. All secondary fractures occurred on the second operative side. CONCLUSION: Secondary fractures in the Le Fort I osteotomy procedures occurred on the side opposite the greater maximal compressive load and on the second operative side.

Visual field deficits in conventional anterior temporal lobectomy versus amygdalohippocampectomy.

OBJECTIVE: To evaluate and identify the incidence of visual field defects (VFD) after anterior temporal lobectomy (ATL) versus amygdalohippocampectomy (AH). VFD occur frequently after ATL and are usually superotemporal quadrantanopias. Little is known about the incidence of VFD after AH and this surgical method offers the possibility of a seizure-free survival without visual loss. METHODS: Patients with similar characteristics were examined. All patients had intractable seizures and mesial temporal sclerosis, small tumors localized to only the uncus, amygdala, or hippocampus, or no known pathology. Postoperative kinetic field testing using the I4e isopter on a Goldmann perimeter was performed 30 days or more after surgery. RESULTS: Of 29 patients examined, 14 underwent AH and 15 had ATL. Four of 14 AH patients (28%) had a VFD at 10 degrees from center and 11/14 (78%) had VFD at 40 degrees. One of 15 ATL patients (7%) had a VFD at 10 degrees from center and 11/15 (73%) had VFD at 40 degrees. There was no significant difference between surgery types. CONCLUSIONS: AH in this study was associated with a significant number of VFD. No significant difference was found between the frequency of VFD produced from AH and ATL. The mechanism of injury is due to direct trauma to the optic radiations while accessing the mesial temporal structures. Because all patients in the study were asymptomatic for VFD, it remains to be determined whether these VFD are clinically significant.

Features of amiodarone-induced optic neuropathy.

PURPOSE: To report clinical features of amiodarone-induced optic neuropathy and outline the differentiation of amiodarone optic neuropathy from nonarteritic anterior ischemic optic neuropathy. METHOD: We reviewed data from 73 patients reported to have developed an optic neuropathy while taking amiodarone. RESULTS: Amiodarone optic neuropathy is characterized by an insidious onset, slow progression, bilateral visual loss, and protracted disk swelling that tends to stabilize within several months of discontinuing the medication. Nonarteritic ischemic optic neuropathy is characterized by acute, unilateral visual loss that is usually complete at onset, with resolution of disk edema over several weeks. CONCLUSION: Unique clinical features of amiodarone-induced optic neuropathy may help clinicians diagnose and distinguish between amiodarone-induced optic neuropathy and nonarteritic anterior ischemic optic neuropathy.

Severe ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy.

PURPOSE: To report severe ocular and orbital toxicity after administration of intracarotid etoposide phosphate and carboplatin. METHOD: Case report. RESULTS: A 52-year-old man with glioblastoma multiforme underwent left intracarotid administration of eto poside phosphate and carboplatin inferior to the ophthalmic artery. Within 7 hours, a nonpupillary block angle-closure glaucoma developed secondary to uveal effusion in the ipsilateral eye, which was relieved by cycloplegia. Four days later, severe orbital inflammation resulted in a visual acuity of counting fingers, proptosis, optic neuropathy, and total external ophthalmoplegia in the eye. The patient's condition improved after a lateral cantholysis and administration of high-dose intravenous corticosteroids. Two weeks later, an anterior uveitis occurred in the left eye, which responded to topical corticosteroids. During a 2-month period, the patient recovered to a visual acuity of 20/70, near normal motility, and normal intraocular pressure, and the ocular and orbital inflammation resolved. Preexisting ipsilateral chemotherapy-induced maculopathy became more pronounced. CONCLUSION: Ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy is infrequently reported.

Neuro-ophthalmic complications of intracranial catheters.

We report four patients who sustained direct injury to the brain after insertion of intraventricular shunts and pressure monitoring lines, which resulted in permanent neuro-ophthalmic deficits. These included hemianopsia from an optic tract lesion, esotropia and residual bilateral facial paresis from dorsal pontine injury, unilateral blindness from damage to the optic nerve, and dorsal midbrain syndrome from catheter compression in the region of the posterior commissure. Although presumably rare, such injuries should be considered in diagnosing patients with neuro-ophthalmic complaints after insertion of such devices.

Neuro-ophthalmological function of patients with pineal region tumors approached transtentorially in the semisitting position.

To optimize orientation and operative exposure for aggressive resection, the authors approached pineal region tumors transtentorially with the patient in a semisitting position. In the current report, 12 consecutive patients were evaluated to document operative ocular morbidity referable to the brain stem as well as visual deficits secondary to occipital lobe retraction. Before craniotomy, ophthalmological findings related to dorsal midbrain dysfunction were evident in four of the 10 patients who had previously undergone ventricular shunting. The other patients developed a partial or complete Parinaud's syndrome in the early postoperative period and some suffered additional deficits such as cranial nerve palsies. These deficits improved to varying degrees in all patients. Although each had full visual fields preoperatively, an absolute or incomplete left homonymous hemianopsia developed in the immediate postoperative period. Such visual field deficits fully resolved over a variable period of time in 10 of the 12 patients. One patient has a permanent left homonymous hemianopsia, while another has a left homonymous paracentral scotoma. Eight patients were able to return to preoperative pursuits. While ocular abnormalities related to dorsal midbrain dysfunction are most probably independent of operative approach, visual field deficits attributable to occipital lobe retraction were common in patients after a occipital transtentorial approach performed in the semisitting position. Reading difficulties associated with ocular motor dysfunction due to dorsal midbrain involvement represent the principal long-term neuro-ophthalmological complaint of patients who have undergone pineal region surgery.

A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group.

BACKGROUND AND METHODS: The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. RESULTS: Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did not differ from that in the placebo group. In addition, the rate of new episodes of optic neuritis in either eye was higher in the group receiving oral prednisone, but not the group receiving intravenous methylprednisolone, than in the placebo group (relative risk for oral prednisone vs. placebo, 1.79; 95 percent confidence interval, 1.08 to 2.95). CONCLUSIONS: Intravenous methylprednisolone followed by oral prednisone speeds the recovery of visual loss due to optic neuritis and results in slightly better vision at six months. Oral prednisone alone, as prescribed in this study, is an ineffective treatment and increases the risk of new episodes of optic neuritis.

Acute idiopathic blind spot enlargement. A spectrum of disease.

The authors present a report on their experience with 10 patients with acute idiopathic blind spot enlargement. All had enlarged steep-margined blind spots without accompanying changes in optic disc appearance, significantly diminished visual acuity or color vision. Fluorescein angiography was obtained in eight patients. Five patients had retinal appearance that was either normal or could not be attributed to a known disease entity. Three patients had the multiple evanescent white dot syndrome, one had acute macular neuroretinopathy, and at least one had presumed ocular histoplasmosis. It has been proposed that acute idiopathic blind spot enlargement is a subset of multiple evanescent white dot syndrome. The authors' findings suggest that multiple evanescent white dot syndrome and other retinal diseases, some not yet identified, are subsets of acute idiopathic blind spot enlargement.

CT of acquired hyperopia with choroidal folds.

Seven patients with an ophthalmologic diagnosis of acquired hyperopia with choroidal folds were evaluated by high-resolution axial CT of the orbits. Coronal, oblique coronal, and parasagittal reformations were obtained and the thickness of the optic nerve and morphologic appearance of the globes were assessed by measurement and subjective appearance. Flattening of the globe, which caused the globe to assume an ellipsoid shape, was seen in all 11 affected eyes. Mild to moderate optic nerve enlargement was also demonstrated in most patients. In six of 11 affected eyes a visible space was noted between the optic nerve and its sheath, implying expansion of the subarachnoid perineural compartment. These findings were not demonstrated in a control group of five patients scanned in a similar manner. Scans of a phantom revealed no evidence of CT-generated distortion. These findings may help to identify hyperopia with choroidal folds as a benign disease and eventually help to establish its cause.

Visual recovery in combined central retinal artery and central retinal vein occlusion.

We observed two patients who had combined central retinal artery occlusion and central retinal vein occlusion with severely reduced visual acuity and characteristic retinal changes. Over the course of several months, visual acuity and ophthalmoscopic appearance returned to normal. Both patients had a transient visual loss before their occlusive events and a mild nonconcurrent central retinal vein occlusion in their fellow eye. There was no evidence of inflammatory, vascular, or myeloproliferative disease.

Maculopathy associated with combination chemotherapy and osmotic opening of the blood-brain barrier.

Eleven patients with intracranial malignant neoplasms underwent hyperosmotic opening of the blood-brain barrier by rapid mannitol infusion via the internal carotid or vertebral artery. Cyclophosphamide was administered intravenously preceding the mannitol infusion and methotrexate was infused after the mannitol via the same artery. Both the mannitol and methotrexate delivered via the carotid artery have direct access to the ipsilateral choroidal and retinal circulations. In these patients, retinal pigment epithelial changes eventually developed ipsilateral to the carotid infusions. The changes probably represent the toxicity of intraarterial methotrexate possibly potentiated by mannitol-induced opening of the blood-retinal barrier. Functional visual loss was minimal and was not a limiting factor with this therapeutic modality.

The natural history and CT appearance of acquired hyperopia with choroidal folds.

Seven healthy adults with acquired hyperopic change in refractive error had choroidal folds in affected eyes. High resolution computerized tomographic scanning techniques used to obtain coronal, oblique coronal, and parasagittal reformations revealed flattening of the posterior globes and mild to moderate optic nerve enlargement. In 5 of 11 eyes, a space was noted between the optic nerve and its sheath, implying expansion of the sub-arachnoid perineural compartment. The clinical findings and natural history of this syndrome, based on an average follow-up of 4.5 years, are discussed.

Ocular neuromyotonia. A clinical description of six patients.

We report the cases of six patients with ocular neuromyotonia, a disorder believed to result from episodic involuntary discharge of ocular motor nerves producing sustained and inappropriate contraction of their respective ocular muscles. Transient disturbances of ocular motility and diplopia result. Four patients had involvement of ocular muscles supplied by the third cranial nerve: one had presumed involvement of the superior oblique muscle, and one the lateral rectus muscle, suggesting abnormal discharge in the fourth and sixth cranial nerves, respectively. Four of six patients received prior radiation therapy for pituitary tumors, implying a possible pathogenic link. Three patients improved after treatment with membrane-stabilizing medication, suggesting that unstable membranes of injured ocular motor axons may generate spontaneous impulses, which produce involuntary sustained and inappropriate ocular muscle contraction.

Optic nerve head drusen and glaucoma.

The simultaneous occurrence of both optic nerve head drusen and glaucoma may make it difficult to evaluate visual field defects and the appearance of the optic nerve head. We saw five patients with both conditions, of whom four had pigmentary glaucoma or pigmentary dispersion syndrome. Drusen do not preclude the development of cupping, and they make it difficult to interpret its significance. Management of these patients requires serial visual field testing, serial disc photographs, and treatment aimed at keeping the intraocular pressures as low as possible.

Ischemic optic neuropathy. Still the ophthalmologist's dilemma.

Ischemic optic neuropathy, in its arteritic and idiopathic varieties, presents both diagnostic and therapeutic challenges to the clinician, specifically: the role of temporal artery biopsy in management of giant cell arteritis; the preferred treatment regimen for giant cell arteritis, including decisions to begin tapering therapy and stopping therapy; action to take if major steroid complications arise while the disease is still active; distinguishing anterior ischemic optic neuropathy from idiopathic optic neuritis; whether cerebral arteriography is indicated in the assessment of idiopathic ischemic optic neuropathy; and whether steroid therapy is of any value in the treatment of idiopathic ischemic optic neuropathy.

Acquired esotropia. A manifestation of Chiari I malformation.

A patient with acquired esotropia underwent apparently successful strabismus surgery. Subsequent recurrence of esotropia, associated with square-wave jerks and downbeat nystagmus led to further investigation. Although standard CT scan was normal, rescanning after instillation of metrizamide demonstrated a Chiari I malformation. Posterior fossa decompression alleviated the esotropia. Acquired esotropia has not been recognized as a manifestation of Chiari I malformation. Our case illustrates that a high degree of suspicion is required to make the diagnosis of Chiari I malformation. Specialized techniques, such as metrizamide cisternography, or magnetic resonance imaging may be necessary if routine diagnostic measures are unrevealing.

Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma.

The clinical information on 22 patients with orbital optic nerve gliomas and 47 patients with meningiomas was correlated with computed tomographic findings obtained in both axial and coronal studies. Most of the gliomas occurred in children, although 7 patients presented after 20 years of age. Among the patients with meningiomas, the majority were women in early middle age, although two tumors occurred in children less than 20 years of age. Low grades of proptosis (median, 2 mm for both tumors), frequent significant visual field obscurations with eye movements, and opto-ciliary shunt vessels pointed toward the diagnosis of an optic nerve tumor. Patients with gliomas generally manifested massively swollen fusiform optic nerves with clear-cut margins due to circumscription by an intact dura. Kinks and bucklings of the optic nerve as well as infarctive cysts distinguished the glioma CT-scan patterns from the meningiomas. Distinctive axial CT-scan features of the meningiomas not shared by the gliomas were narrowly and diffusely enlarged nerves with polar expansions either at the orbital apex or immediately behind the globe; calcification; irregular excrescent margins signifying extradural invasion into the orbital soft tissues; a negative optic nerve shadow running down the center of the lesion; and bone erosion near the orbital apex. Coronal studies often revealed irregular margins signifying transgression of the dura. A diffusely and narrowly enlarged optic nerve shadow with regular margins (intrasheath lesions) was the one morphologically overlapping pattern displayed by 11 meningiomas and three gliomas. In these cases there tended to be more profound visual loss in the gliomas compared with the meningiomas, as well as the more frequent presence of opto-ciliary vessels in the meningiomas. Arteriography may be helpful in this particular category by demonstrating a tumor blush for the meningiomas, whereas this finding is typically absent with optic nerve gliomas. Meningiomas may be very closely simulated by dural or intraneural inflammations.