Rod Foroozan

Orbital apex syndrome.

Purpose of review Visual loss from optic neuropathy and ophthalmoplegia involving multiple cranial nerves are the hallmarks of an orbital apex syndrome. Historically, the terms superior orbital fissure, orbital apex, and cavernous sinus have been used to define the anatomic locations of a disease process. However, the diagnostic evaluation and management is similar for each of these entities. The authors reviewed the literature on the diagnosis and evaluation of disorders involving the orbital apex. Recent findings High-resolution MRI is the preferred modality for evaluating most lesions involving the orbital apex. CT is a useful tool in the setting of trauma, to evaluate bone involvement, or when MRI is contraindicated. Although laboratory studies may be useful adjuncts in the diagnostic evaluation of lesions involving the orbital apex, surgical biopsy is often required for definitive diagnosis. Summary Orbital apex syndromes may result from a variety of inflammatory, infectious, neoplastic, iatrogenic/traumatic, and vascular conditions. A detailed history with review of systems is important in narrowing the differential diagnosis. Management is directed at the underlying cause and may be guided by surgical biopsy. Corticosteroids may be useful if an inflammatory etiology is suspected, but should be used with caution.

Recovery of visual function in patients with biopsy-proven giant cell arteritis ☆

OBJECTIVE To assess the visual function of patients with giant cell arteritis (GCA) who had visual loss from either anterior ischemic optic neuropathy (AION) or central retinal artery occlusion and had a subsequent improvement in visual acuity after treatment with corticosteroids. DESIGN Retrospective, observational case series. PARTICIPANTS Thirty-two consecutive patients with biopsy-proven GCA treated at one institution between January 1992 and December 1997. INTERVENTION Treatment with intravenous methylprednisolone 250 mg every 6 hours for 3 days, followed by oral prednisone 1 mg/kg daily for at least 4 weeks duration. MAIN OUTCOME MEASURES The number of patients with an improvement in visual acuity after treatment with intravenous methylprednisolone; neuro-ophthalmic evaluation, including visual acuity, funduscopy, and visual field examination of these patients. RESULTS Improvement in visual acuity occurred in 5 of 39 eyes (13%) with visual loss from biopsy-proven GCA, and all 5 patients had AION. Despite the improvement of visual acuity in these 5 patients, perimetry revealed marked constriction of the visual field in each affected eye. CONCLUSIONS The prognosis for visual improvement in GCA is poor. Although an improvement in visual acuity occurred in 5 of our patients, marked constriction of the visual field was present in all of them.

Bariatric surgery for the treatment of idiopathic intracranial hypertension

OBJECT The purpose of this study was to review the literature on the effectiveness of bariatric surgery for obese patients with idiopathic intracranial hypertension (IIH) with regard to both symptom resolution and resolution of visual deficits. METHODS The published literature was reviewed using manual and electronic search techniques. Data from each relevant manuscript were gathered, analyzed, and compared. These included demographic data, pre- and postoperative symptoms, pre- and postoperative visual field deficits, bariatric procedure type, absolute weight loss, changes in body mass index, and changes in CSF opening pressure. RESULTS Eleven relevant publications (including 6 individual case reports) were found, reporting on a total of 62 patients. The Roux-en-Y gastric bypass was the most common bariatric procedure performed. Fifty-six (92%) of 61 patients with recorded postoperative clinical history had resolution of their presenting IIH symptoms following bariatric surgery. Thirty-four (97%) of 35 patients who had undergone pre- and postoperative funduscopy were found to have resolution of papilledema postoperatively. Eleven (92%) of 12 patients who had undergone pre- and postoperative formal visual field testing had complete or nearly complete resolution of visual field deficits, and the remaining patient had stabilization of previously progressive vision loss. In 13 patients both pre- and postoperative CSF pressures were recorded, with an average postoperative pressure decrease of 254 mm H(2)O. Changes in weight loss and body mass index varied depending on the reported postoperative follow-up interval. CONCLUSIONS The published Class IV evidence suggests that bariatric surgery may be an effective treatment for IIH in obese patients, both in terms of symptom resolution and visual outcome. Prospective, controlled studies are necessary for better elucidation of its role.

Thrombocytosis in patients with biopsy-proven giant cell arteritis

OBJECTIVE To compare the platelet counts, complete blood counts, and Westergren sedimentation rates (WESR) of patients with a biopsy positive for giant cell arteritis (GCA) with those of patients with negative biopsies. DESIGN Retrospective, case-control series. METHOD The medical records of 91 consecutive patients who underwent temporal artery biopsy for possible GCA, over an 8-year period, were reviewed. MAIN OUTCOME MEASURES Values obtained for the complete blood counts, including platelet counts, WESR, and biopsy results of 91 consecutive patients undergoing temporal artery biopsy were analyzed. RESULTS The mean platelet count of 47 patients with positive temporal artery biopsies (433 x 10(3)/microl) was significantly higher than that of 44 patients with negative temporal artery biopsies (277 x 10(3)/microl), P < 0.0001. There was no statistically significant difference in the mean WESR between the biopsy-positive (82 mm/hour) and biopsy-negative (70 mm/hour) groups, P = 0.12. The sensitivity of an elevated WESR for biopsy-positive patients was greater (79%; 95% confidence interval [CI], 64%-89%) than that of an elevated platelet count (57%; 95% CI, 42%-72%). However, the specificity (91% [95% CI, 78%-97%] versus 27% [95% CI, 15%-43%]), positive predictive value (87% [95% CI, 70%-96%] versus 54% [95% CI, 41%-66%]), and negative predictive value (67% [95% CI, 53%-78%] versus 55% [95% CI, 32%-76%]) favored an elevated platelet count compared with WESR, or to the combination of platelets and WESR, as a better test for diagnosing GCA in the 91 patients studied. The area under the receiver operating characteristic function for platelets (0.72) was greater than that for WESR (0.59) or the combination of platelets and WESR (0.65). CONCLUSIONS In patients suspected of having GCA, an elevated platelet count greater than 400 x 10(3)/microl is a useful marker of a positive temporal artery biopsy.

Ischemic optic neuropathy as the first manifestation of elevated cholesterol levels in young patients.

PURPOSE To investigate the relationship between idiopathic nonarteritic ischemic optic neuropathy (NAION) and serum lipid levels in patients </= 50 years of age. DESIGN Case-control study. PARTICIPANTS Thirty-seven consecutive patients with NAION and 74 age- and gender-matched comparison patients. METHODS AND MAIN OUTCOME MEASURES Serum lipid levels and the presence of several historical diseases and exposures were abstracted from the medical records in cases and controls. RESULTS The mean total cholesterol level was significantly increased in patients with NAION compared with controls (235.4 vs. 204.0 mg/dl, P < 0.001). The odds ratio of having high blood cholesterol (>/= 240 mg/dl) with NAION was 3.3 (95% confidence interval, 1.4-7.8), and the likelihood increased when the comparison was restricted to nondiabetic patients. Diabetes mellitus was more common in cases than controls (P = 0.027), but systemic hypertension was not significantly different (P = 0.63). No patient (0 of 24) had a magnetic resonance imaging study consistent with optic neuritis or central nervous system demyelination. Visual improvement was uncommon. CONCLUSIONS This study demonstrates that hypercholesterolemia is associated with NAION in younger patients. NAION may be the first manifestation of a lipid disorder, a previously unrecognized syndrome. These patients have experienced a focal, microvascular central nervous system ischemic event at a relatively young age. Aggressive treatment of lipid abnormalities in these patients may be warranted.

Acute demyelinating optic neuritis.

Acute demyelinating optic neuritis associated with multiple sclerosis (MS) is the most common cause of inflammation of the optic nerve. The Optic Neuritis Treatment Trial (ONTT) has provided important clinical data on the use of corticosteroids, and demonstrated that patients with characteristic inflammatory lesions within the brain on magnetic resonance imaging had a greater chance of developing clinically definite MS (CDMS). The current approach to patients with optic neuritis has been modified by the results of the Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS). Patients with an initial clinical episode of demyelination (optic neuritis, incomplete transverse myelitis, or brain-stem/cerebellar syndrome) and at least two characteristic demyelinating lesions within the brain were randomized to receive interferon beta-1a or placebo after initial treatment with intravenous corticosteroids. At the 3-year point patients treated with interferon beta-1a showed a 50% less risk of CDMS. The results of this study have set the standard for patients with a first bout of demyelinating optic neuritis.

Outcomes after laser therapy for threshold retinopathy of prematurity

OBJECTIVE To determine the anatomic outcomes of eyes treated with laser photocoagulation for threshold retinopathy of prematurity and to identify potential risk factors for unfavorable outcomes after treatment. DESIGN Retrospective, noncomparative case series. INTERVENTION Photocoagulation of the peripheral avascular retina with an argon or diode laser indirect ophthalmoscope. PARTICIPANTS One hundred twenty eyes of 81 infants with threshold retinopathy of prematurity treated with laser photocoagulation from 1989 through 1997 with at least 12 months of follow-up after treatment. MAIN OUTCOME MEASURES The principal outcome was the presence of an unfavorable anatomic outcome defined as a retinal detachment, macular fold, or retrolental tissue. RESULTS One hundred nine of 120 eyes (91%) had favorable outcomes. Eleven eyes (9%) had retinal detachments, and 1 of the 11 also had retrolental tissue. Zone 1 eyes appeared to be 3.3 times more likely to have an unfavorable outcome compared with zone 2 eyes, but the 95% confidence interval (0.8-14.5) did not support this statistically. Twenty-four of 109 eyes (22%) experienced dragging of the temporal vessels. Those with zone 1 disease were 13.7 times more likely to experience temporal dragging compared with zone 2 eyes (95% confidence interval, 3.3-57.2). CONCLUSIONS After laser photocoagulation for threshold retinopathy of prematurity, 91% of eyes had a favorable anatomic outcome. Compared with zone 2 eyes, zone 1 eyes may be more likely to have temporal dragging of the retinal vessels. Laser therapy is effective in the treatment of threshold retinopathy of prematurity.

Nonarteritic anterior ischemic optic neuropathy.

Nonarteritic anterior ischemic optic neuropathy refers to an idiopathic ischemic process of the anterior portion of the optic nerve. The typical presentation is sudden and painless visual loss with examination features of an optic neuropathy. Among the various associated risk factors are optic disc morphology, advanced age, systemic arterial hypertension, diabetes mellitus, and nocturnal hypotension. Currently, there is no proven effective long-term treatment for this disorder.

Is normal tension glaucoma actually an unrecognized hereditary optic neuropathy? New evidence from genetic analysis.

Normal tension glaucoma and dominant optic atrophy share many overlapping clinical features, and differentiating between these two diseases is often difficult. The gene responsible for dominant optic atrophy is the OPA1 gene located on chromosome 3. This gene encodes for a protein product that is involved in mitochondrial metabolic function. Recent genetic linkage analysis of patients with normal tension glaucoma has shown an association with polymorphisms of the OPA1 gene. This association suggests that normal tension glaucoma may actually be a hereditary optic neuropathy with a pathophysiology based in mitochondrial dysfunction.

Cortical visual impairment in children.

Purpose of review Cortical visual impairment is rapidly becoming a leading cause of visual loss in children in developed countries predominately because of the improved survival rates of premature infants over the past decade. Recent findings Most cases of cortical visual impairment arise from hypoxic ischemic injury to watershed areas of the brain. In the preterm infant the watershed areas are in the subcortex around the ventricles, while in the term infant the watershed areas are between the major arteries with injury to the subcortex and cortex. Therefore, preterm and term injury will manifest different ocular and visual system abnormalities as a result of this damage. Cognitive visual dysfunction, a type of cortical visual impairment, may occur in cases of damage to the peristriate cortex (association areas of the brain). The anterior visual pathways may also be damaged in a retrograde, transsynaptic fashion in cases of cortical visual impairment. Summary Cortical visual impairment is a prevalent cause of visual loss in children. It encompasses a wide range of visual disabilities from no light reception to normal visual acuity with cognitive visual dysfunction.