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Purpose: To evaluate the effectiveness of hyperbaric oxygen therapy in reducing optic nerve damage in acute nonarteritic anterior ischemic optic neuropathy., Methods: Twenty-two eyes in 20 patients with acute nonarteritic anterior ischemic optic neuropathy were treated with hyperbaric oxygen (100% oxygen, 2.0 absolute atmospheres of pressure) in two 90-minute inhalation sessions per day for ten days. Changes in mean visual acuity and mean sensitivity loss were compared with those of 27 untreated control patients with acute nonarteritic anterior ischemic optic neuropathy, and proportions of patients with a change in acuity or mean sensitivity loss were compared with controls. Similar analysis was performed on subgroups based on time delay from symptom onset to therapy., Results: Mean values for visual acuity were increased at final examination in both groups; although the increase was greater in controls, the difference was not statistically significant. Mean visual field sensitivity loss was minimally increased; although the increase was smaller in controls, the difference between groups was not significant. There was no significant difference between groups in proportions of patients with change in acuity score or mean sensitivity loss. Stratification by time delay to therapy did not suggest that treatment within nine days produced better visual results than that for either controls or those treated later., Conclusions: Hyperbaric oxygen therapy using 100% oxygen and 2.0 absolute atmospheres of pressure did not produce a significant improvement in visual acuity or visual field for patients with acute nonarteritic anterior ischemic optic neuropathy.

Objective: To determine whether nonischemic optic disc edema is associated with significant delay in fluorescein angiographic optic disc filling., Methods: Fluorescein angiograms from 16 patients with acute papillitis, five with papilledema, and one with optic disc edema from orbital cavernous hemangioma were compared with those of age-matched controls. Early views of the optic disc were evaluated for onset of central retinal artery dye filling and both onset and completion of choroidal and prelaminar optic disc dye filling. Data were compared with our previously published figures for patients with nonarteritic anterior ischemic optic neuropathy (NAION) and a new group of patients aged 46 years and younger with NAION (NAIONy). Subgroup analysis was performed on data from patients with papillitis., Results: Mean onset or completion of filling was not significantly delayed compared with controls for the central retinal artery, choroid, or prelaminar optic disc in patients with nonischemic optic disc edema, including the subgroup of patients with papillitis. In comparison, significant delay had been detected for onset and filling of prelaminar disc in typical NAION; similar significant delay was noted in this study for patients with NAIONy. No patients with nonischemic optic disc edema (including those with papillitis) demonstrated delay of disc filling by at least 5 seconds, while this feature was detected in 76% of patients with typical NAION and 62% of those with NAIONy., Conclusions: Optic disc filling delay is common in typical NAION and NAIONy; it is not a feature of nonischemic optic disc edema. This characteristic may aid in the differentiation of NAION from papillitis.

We wished to determine whether the number of central nervous system (CNS) white matter lesions on magnetic resonance imaging (MRI) is increased in patients with nonarteritic ischemic optic neuropathy (NAION). T2-Weighted axial images of the brain in 13 patients with acute NAION and 16 age-matched controls were used to tabulate the number of subcortical and periventricular white matter lesions. Groups were compared by t test for means, the Wilcoxon-Mann-Whitney rank-sum test, and chi-square test for proportions with at least one lesion. The mean number of CNS white matter ischemic lesions in the NAION group was 4.0 (range 0-20) as compared to 1.4 (range 0-7) in the control group. The difference in these samples suggested a significant increase in NAION (p = 0.069, rank-sum test). The proportions of patients with at least one lesion were not significantly different (53.8% NAION vs. 56.3% controls). The data suggest an increased number of CNS white matter lesions in patients with NAION.

A 71-year-old woman developed chronic progressive visual loss in the right eye and computed tomographic scan showed enlargement of the intraorbital optic nerve consistent with optic nerve sheath meningioma. Over 12 years, the contralateral optic nerve was not clinically affected, and serial neuroradiologic imaging showed no evidence of intracranial tumor extension. Death occurred from metastatic adenocarcinoma of the lung 14 years after initial visual loss. Examination of the postmortem specimen of optic nerve and chiasm revealed extradural extension of meningioma with spread to the region of the optic chiasm and hypothalamus. A large focus of metastatic adenocarcinoma was present within the intraorbital portion of the meningioma. Carcinoma metastatic to intracranial meningioma is rare; to our knowledge, this is the first reported case in an optic nerve sheath meningioma. Neuroimaging may be inadequate to predict the value of tumor excision in preventing intracranial spread of optic nerve sheath meningioma.

Visual acuity and quantitative perimetry (Octopus Program 32) testing was performed in the acute (< 30 days after onset) and convalescent (> 3 months after onset) phases of disease in 27 patients with untreated NAION. Initial group mean acuity and sensitivity loss were compared to final values using the t test. Proportions of patients demonstrating a change of > 2 lines acuity or > 2 dB mean sensitivity were calculated. Patients were classified as either "progressive" (n = 6) or "stable" (n = 21) for purposes of additional subgroup analysis. There was no significant change in group mean visual acuity or field over time. Overall, significant worsening occurred in 11.1% for visual acuity and 22.2% for visual field; improvement occurred in 23.8% for acuity and 24.0% for field. Of 21 "stable" patients, none worsened for acuity and 4.7% showed late worsening of field; 31.3% showed significant improvement for acuity and 31.6% for field. No "progressive" patients demonstrated worsening or improvement after the initial progressive phase.

Fundus fluorescein angiograms from 41 patients with nonarteritic anterior ischemic optic neuropathy of less than three weeks' duration were compared with those from 43 age-matched control subjects. Patients with disease showed statistically significant delay in both the onset and the time to completion of prelaminar optic disk filling. Neither onset nor completion of peripapillary choroidal filling were markedly delayed when compared with control subjects. Frequency of occurrence of delayed filling within peripapillary choroidal watershed zones was not increased in patients with disease. There was no consistent correlation by quadrant between optic disk filling delay, choroidal filling delay, optic disk swelling or hyperfluorescence, and visual field deficit. No trend for change in characteristics was found with increasing time interval from onset of symptoms to performance of angiography. Fluorescein angiography in nonarteritic anterior ischemic optic neuropathy demonstrates delayed optic disk filling without consistent relation to adjacent peripapillary choroidal filling delay or other disease findings.

Purpose: The transition from inpatient to outpatient cataract surgery during the last decade was not accompanied by prospective investigation of its effect on visual outcomes or surgical complications. The authors performed this study to assess the impact of this transition on surgical results., Methods: The authors reviewed 600 extracapsular cataract extractions performed by 4 experienced ophthalmic surgeons during a 36-month period; in 300 cases, patients were hospitalized after surgery (inpatient group), and, in 300 cases, patients were never hospitalized (outpatient group). The same surgical techniques were used in all cases. Visual outcome and rates for operative and postoperative complications were compared., Results: There were no statistically significant differences between the inpatient and outpatient groups for visual acuity. Excluding patients with pre-existing nonlenticular ocular disease, a best-corrected visual acuity of 20/40 or better was achieved in 93.1% of inpatient cases and in 97.2% of outpatient cases 6 months after surgery. Postoperative, clinically apparent cystoid macular edema was more common in the inpatient group (P = 0.03); however, after exclusion of patients with diabetes, hypertension, age younger than 65 years, and eyes with pre-existing nonlenticular disease, there was no statistically significant difference between groups. No significant differences in rates for other operative and postoperative complications were identified, including wound dehiscence, unplanned postoperative filtering blebs, infectious endophthalmitis, retinal detachment, persistent iridocyclitis, glaucoma, and corneal edema., Conclusion: This study does not demonstrate that the transition to outpatient cataract extractions has had an adverse effect on surgical outcomes.

Background: Present trends in medical care suggest that primary care physicians will exert increasing control over patient access to medical specialty consultation and diagnostic testing. Therefore, it is important to determine whether primary care physicians can reliably identify papilledema., Methods: A prospective study involving 429 physicians was undertaken to assess the accuracy of papilledema and pseudopapilledema detection by five groups of physicians, family practice physicians, neurologists, neuro-ophthalmologists, neurosurgeons, and ophthalmologists., Results: Neuro-ophthalmologists and ophthalmologists did better than family physicians, neurologists, and neurosurgeons in identifying both papilledema and pseudopapilledema (P less than .05). Neuro-ophthalmologists more accurately identified pseudopapilledema than all other groups in the study (P less than .05). Family physicians did as well as, or better than, neurologists and neurosurgeons in identifying all classifications of acute and chronic papilledema defined in the study. Family physicians did not perform as well as the other four groups in differentiating pseudopapilledema from papilledema (P less than .05)., Conclusions: Although the sensitivity of detecting papilledema was high (84.5%) for family physicians, the specificity was low (59.3%). Preliminary data indicate that family physicians with prior exposure to clinical ophthalmology in medical school did better than those who had not had training. It is possible that additional exposure to clinical ophthalmology during residency training might yield improved performance.

Spontaneous dural carotid-cavernous fistulas are dural vascular malformations that usually run a benign course. We present a case of a spontaneously occurring dural carotid-cavernous fistula complicated by central retinal vein occlusion and iris neovascularization that led to progressive visual failure.

We describe 3 cases of Chiari type II malformation presenting with bilateral internuclear ophthalmoplegia (INO). Although prominent, the INO was not an isolated sign in any of the patients; superimposed abduction paresis was present in 2, and deficits in smooth pursuit, optokinetic nystagmus, and vestibulo-ocular responses were present in 3. Two had hydrocephalus: 1 was clinically unchanged without therapy after 5 years; the other did not improve with shunting. Findings in our 3 patients, along with the 4 previously reported, confirm that INO is 1 manifestation of widespread brainstem or cerebellar dysfunction. Its origin is probably multifactorial, related to hydrocephalus, vascular compromise, direct neuronal distortion, or congenital neural malformation.

The performance of staff in the ophthalmologist's office will influence the risk that an individual patient may someday file a medical malpractice complaint. Areas of identified risk include improper triage of emergency patients, abandonment, problems in confidentiality, improper maintenance of medical records, and perceived lack of compassion and skill on the part of staff members.

Fibrous dysplasia is a developmental anomaly of bone, often affecting the facial bones. We treated a patient who had fibrous dysplasia involving the right maxillary and sphenoid bones. The patient had a sudden loss of visual acuity to R.E.: counting fingers. Visual fields demonstrated a central scotoma, and retrobulbar neuritis was diagnosed. When vision failed to improve spontaneously, however, radiologic studies were performed. These showed compromise of the optic canal by bony proliferation and an apparent mucocele at the orbital apex. Surgical exploration disclosed fibrous dysplastic bone and a cystic structure overlying the optic nerve. Successful surgical excision of the cyst and debulking of the fibrous dysplasia resulted in visual acuity returning to R.E.: 20/25.

The clinical and histopathologic findings in two adults with craniopharyngioma emphasized the wide age range and diverse initial manifestations of this tumor. A retrospective clinical review of 49 additional patients substantiated these points and disclosed that over 40% were initially examined by ophthalmologists. The initial manifestations fell into four broad categories: (1) visual system abnormalities (77% of the cases), (2) headache (67%), (3) endocrinologic abnormalities (57%), and (4) mental status abnormalities (19%). The frequency of these manifestations varied slightly among different age groups. The radiologic features in the two patients described (a 75-year-old man and a 38-year-old woman) included a distinct appearance on computed tomographic scans. These craniopharyngioma were not calcified and were isodense without contrast but enhanced densely and uniformly with contrast, simulating an aneurysm of the circle of Willis.

Twenty-seven members of a family with dominantly inherited Charcot-Marie-Tooth disease (CMTD) were examined. Fifteen members had CMTD and 13 of these had varying amounts of myotonic pupillary abnormalities similar in some ways to Adie tonic pupil syndrome. Those with graver neurologic disease showed greater pupillary abnormalities. Ten of the 15 patients had pupillary constriction with methacholine chloride (Mecholyl) and some of these had extensive iris atrophy. Several affected patients received symptomatic relief from 0.025% pilocarpine. Seven other patients with CMTD who were not related to our initial family were checked for myotonic pupils; two had findings similar to our initial family. Pupillary abnormalities in certain patients with CMTD appear secondary to a parasympathetic denervation of the iris sphincter and ciliary muscle, as shown by a positive methacholine test, and probably represent part of the autonomic nervous system dysfunction associated with the polyneuropathy in CMTD.

Pupil dilation from commercially available phenylephrine compounds was studied in a group of 11 subjects. Phenylephrine 10% (aq) did not produce significantly more mydriasis than phenylephrine 2.5% (aq) in the general population (P less than 0.05). This suggests that phenylephrine 2.5% can be used instead of phenylephrine 10% for diagnostic dilation.

A case of isolated optic nerve metastasis from breast carcinoma is described. Intraocular tumor was discovered three months following a radical mastectomy and was confined to the optic nerve head and distal optic nerve. Following X-irradiation treatment, central retinal vascular occlusion, rubeosis, and neovascular glaucoma developed during the fifteen months before enucleation. The clinicopathologic features of this case are discussed and the literature reviewed.

Four cases of pneumosinus dilatans of the sphenoid sinus are reported, supplementing the eight cases previously reported in the literature. This rare entity is characterized by expansion of a paranasal sinus that contains only air. In one patient, severe visual loss due to compression of the optic canal by the adjacent enlarged sinus was seen. Galactorrhea occurred in one patient, and three of the four patients reported headaches. There was dehiscence of the sinus roof in two cases, which apparently resulted in a cerebrospinal fluid fistula in one. Previous reports of this entity are reviewed, and the radiographic findings and clinical presentations are discussed. It is proposed that the term "pneumosinus dilatans" be used to describe all dilated, air-filled sinuses with outwardly bulging walls when the primary cause is uncertain.

The Rieger syndrome, characterized by a prominent Schwalbe line, iris strands to the cornea, iris hypoplasia, dental abnormalities, facial malformations, and umbilical defects, is inherited in an autosomal dominant pattern. We studied a boy with the ocular features of the Rieger syndrome, micrognathia, and redundancy of the periumbilical skin. Chromosome analysis revealed an interstitial deletion of the long arm of chromosome 13 involving the distal region of band q14 through band q31. As there was a previous report of the Rieger syndrome in a child with an interstitial deletion of chromosome 13 (q12,q22), we suggest that a gene for this disorder may be located in the segment q14 to q22.

We studied 11 infants and young children fitted with contact lenses for correction of unilateral or bilateral aphakia. The visual-acuity estimates obtained from behavioral cues (alternate-cover test and central-fixation monitoring) were compared with results obtained from pattern-reversal visual-evoked potentials (VEP). We conclude that VEP may be more sensitive than behavioral observation for monitoring the visual progress in certain aphakic children.

The clinicopathologic features of retinal or optic disc astrocytomas are discussed based on eighteen reported cases. In addition, a new case of solitary retinal astrocytoma in a patient without other stigmata of phakomatosis is described. The tumor was prominently vascular and caused total exudative retinal detachment. It doubled in diameter over the seven months prior to enucleation, which was dictated by the occurrence of neovascular glaucoma. The astrocytic nature of the lesion was confirmed by immunohistochemical techniques (glial fibrillary acidic protein stain) and by electron microscopy.

Eyes from 47 autopsy cases of multiple sclerosis were studied pathologically. Lymphocytic or granulomatous retinal periphlebitis was found in four cases (seven eyes); focal lymphocytic or granulomatous retinitis was present in three cases (five eyes). These findings were correlated with optic nerve changes (periphlebitis, neuritis, leptomeningitis, and atrophy), retinal venous sclerosis, uveitis, central nervous system involvement, clinical activity of disease at death, and with ultrastructural and immunopathological retinal findings.

A 39-year-old woman had a large benign intracranial schwannoma of the Gasserian ganglion. Thirty-two years previously, she had an embryonal rhabdomyosarcoma of the orbit. She had been treated by enucleation, local excision, and low-dose supplemental irradiation (1,400 rads). This is the longest survival of which we are aware following any treatment modality in such tumors. No correlation between the two tumors has been established.

A 32-year-old fumigation assistant developed systemic and neuro-ophthalmic manifestations of methyl bromide poisoning, including increased serum bromide level (6.6 mg/100 ml), paresthesias and burning dysesthesia on his hands and feet, and visual impairment. Ocular examination showed mild bilateral decrease in vision, temporal optic nerve head pallor, severely attenuated visual-evoked response amplitudes and normal latencies, a normal electroretinogram, an abnormal electrooculogram, and a severe deuteranomalous (green) defect on Farnsworth-Munsell 100-hue testing. His vision had not improved 12 months after the initial exposure.

Nine patients with maculopathy (macular holes, macular cysts, and lamellar holes) and ten patients with optic neuritis were examined in order to determine changes in the visual evoked potential (VEP) in response to pattern-reversal stimulation. Eyes with lamellar holes had normal P100 latency, but eyes with macular cysts and macular holes had prolonged P100 latency. Eyes with optic neuritis exhibited greater prolongation of the P100 latency than eyes with macular holes. In contrast, eyes with macular holes had a greater reduction in the steady-state VEP amplitude than eyes with optic neuritis. The prolonged latency occurring in maculopathy may be due to a peculiar amplitude summation noted with half-field VEP, rather than to a true conduction delay like that seen in eyes with optic neuritis. The amplitude slope, which is usually positive in normal controls, was negative for 85.7% of eyes with macular holes and 69.2% of eyes with optic neuritis. The negative amplitude slope may represent a subtle defect in retinal ganglion X cells. Eyes with significantly lower values for four or more of the nine central test points on quantitative automated perimetry had negative amplitude slopes and prolonged P100 latency.

Central visual acuity losses were documented in a group of 23 patients with surgically and histologically verified suprasellar meningiomas. The pattern demonstrated was that of acute, gradual or fluctuating loss in one eye, followed by later loss of central acuity in the other eye. Both optic nerves and chiasm were invariably involved either by stretching or compression. Neither preoperative field abnormalities nor central acuity deficits could be correlated with the anatomical location of the tumor, nor could postoperative changes in vision be correlated with tumor size. Lengthy duration of acuity loss and severe visual deficit did not preclude postoperative recovery of vision. Improvement in sight most frequently occurred within the first several weeks after operation, and further return of vision was not noted after 1 year.

Three cases of long-standing intranasal cocaine abuse were associated with orbitopathy or optic neuropathy. All three cases were characterized by chronic sinusitis. Histopathologic examination of involved sinus and orbital tissues revealed chronic and acute nonspecific inflammation, with no evidence of unusual infections or of idiopathic midline destructive disease. In two of the cases, there was radiographic evidence of contiguous orbital inflammation associated with sinusitis. The inflammatory orbital process in these two cases was steroid responsive, but in one case recurrent inflammation occurred in response to steroid tapering. One patient suffered a complete loss of vision in the involved eye due to fulminant orbital inflammation and optic nerve dysfunction.

Two patients (two men, 56 and 59 years old) had sphenoid sinus mucocele originating in the anterior clinoid process. In one case the mucocele initially mimicked diabetic ophthalmoplegia with pupil-sparing palsy of the oculomotor (third) nerve. After resolution of the palsy, severe visual loss developed with minimal recovery of vision after surgery. The second patient had recurrent episodes of retrobulbar optic neuropathy with optic atrophy and decreased vision. Visual loss from sphenoid sinus mucoceles is usually associated with a poor prognosis if surgical treatment is delayed more than seven to ten days.

Patients with contrived histories and/or self-induced physical abnormalities (Munchausen's syndrome) are often successful in deceiving physicians. We recently cared for four patients with ocular Munchausen's syndrome. Self-induced ocular manifestations included voluntary nystagmus, subconjunctival hemorrhages, chronic orbital emphysema requiring exenteration, corneal alkali burns, erosions and ulcerations, and abscesses of the periorbital area. Correct diagnoses of ocular Munchausen's syndrome were made only after extensive medical and surgical investigations. Suggestions for evaluation and treatment will also be discussed.

A young woman developed progressive proptosis of her right eye, accompanied by retinal striae, exposure keratopathy and a severe decrease in visual acuity, reflecting optic neuropathy. An atypical fibrous histiocytoma was found within the orbit. She received orbital exenteration and irradiation. 2 years later she is free of clinical recurrence.

Ipsilateral glaucoma developed in a 51-year-old man with a left-sided temporal lobe arachnoidal cyst. Ultrasonic examination disclosed a cystic orbital lesion adjacent to the optic nerve. Following intracranial decompensation of the arachnoidal cyst, the intraocular pressure dropped markedly. Ultrasonography showed a collapse of the presumed optic nerve sheath cyst. While the exact mechanism producing glaucoma in this patient remains unclear, there was an apparent relationship between the elevated intraocular pressure and the arachnoidal cyst.

To improve understanding and effectiveness of therapy in optic nerve disease, various causes of so-called optic neuritis should be identified when possible. The clinical characteristics of demyelinating optic neuropathy can be contrasted with those of ischemic optic neuropathy, nutritional optic neuropathy, true optic nerve inflammation (e.g., luetic), optic nerve infiltration with tumor, and compression neuropathy caused by adjacent tumor. Radiologic studies and other means of investigating patients with optic neuritis are reviewed. Arguments in favor of, and against, treatment of presumed demyelinating optic neuritis are presented along with representative corticosteroid treatment regimens. The natural tendency toward spontaneous improvement of optic neuritis makes the effect of treatment difficult to assess.

Four patients with clinically localized lesions in the lateral medulla exhibited a tonic bias of their eyes toward the damaged side despite full extracular movements. Each reported that his eyes were being pulled toward the involved side. Although the tonic bias was most prominent when fixation was inhibited, it also occurred with fixation and interfered with saccadic and smooth-pursuit eye movements. Saccades were hypometric when directed against the bias, whereas they were hypermetric when directed toward the side of the lesion. Smooth pursuit toward the intact side was severely impaired, whereas pursuit in the direction of the bias was normal or near normal. Lateropulsion of the eyes seems to be a unique sign of disease in the lateral medullary region of the brainstem.

Craniopharyngiomas are common tumors located in the suprasellar region. Contrast enhancement, cyst formation, and calcification are the three characteristic features of craniopharyngiomas on computed tomographic scan. More than 90% of suprasellar craniopharyngiomas exhibit at least two of these three features, thus providing easy radiologic detection. We treated a 41-year-old man in whom a large suprasellar craniopharyngioma producing severe visual loss was not detected by computed tomography but was easily identified with magnetic resonance imaging. Thus, despite high-resolution computed tomographic scan, large suprasellar craniopharyngiomas can be missed. Magnetic resonance imaging may be superior to computed tomography in detecting these tumors.

Two cases in which medially placed retro-orbital tumors were approached transfrontally have been presented. The computerized tomographic scanner was important in the localization of both lesions. Although of different histologic types, both tumors were friable and vascular. This friability and vascularity would have caused severe difficulties in removal had it not been for the facility with which a cryoprobe froze and grasped tumor tissue. The importance of this instrument, previously unemphasized, has been pointed out.

Quantitative electro-oculographic recording techniques were used to analyze four characteristic eye movement abnormalities in 21 patients with internuclear ophthalmoplegia (INO). The frequency of each of the abnormalities was determined to suggest a pattern that is the most sensitive in detecting the syndrome of INO. Slowing of the adducting saccade was the most frequently found abnormality, being present in all patients. The other characteristic eye movement disorders were found less frequently: dissociated nystagmus at 30 degrees of eccentric gaze, dysmetria of the abducting eye, and limitation of adduction. The most sensitive pattern for detecting an INO seems to be slowing of the adducting saccade combined with either dissociated nystagmus or dysmetria of the abducting eye. Limitation of adduction was seen much less frequently.

The orbital manifestations of Graves' disease frequently constitute the major and distressing portion of the morbidity in this poorly understood process. Patients with optic neuropathy, exposure keratopathy or disfiguring proptosis may be aided considerably by decompression to permit swollen orbital contents to move into the maxillary and ethmoid sinus cavities. Experience with 38 patients treated over a five-year period indicates that antral-ethmoidal decompression is a logical, successful form of therapy and generally free of serious complications. it may provide benefit earlier in the course of Graves' exophthalmopathy than has been accepted in the past.