Celia S. Chen

A review of central retinal artery occlusion: clinical presentation and management

Central retinal artery occlusion (CRAO) is an ophthalmic emergency and the ocular analogue of cerebral stroke. Best evidence reflects that over three-quarters of patients suffer profound acute visual loss with a visual acuity of 20/400 or worse. This results in a reduced functional capacity and quality of life. There is also an increased risk of subsequent cerebral stroke and ischaemic heart disease. There are no current guideline-endorsed therapies, although the use of tissue plasminogen activator (tPA) has been investigated in two randomized controlled trials. This review will describe the pathophysiology, epidemiology, and clinical features of CRAO, and discuss current and future treatments, including the use of tPA in further clinical trials.

Efficacy of Intravenous Tissue-Type Plasminogen Activator in Central Retinal Artery Occlusion Report From a Randomized, Controlled Trial

Background and Purpose— Central retinal artery occlusion is caused by a platelet-fibrin thrombus or embolic occlusion and is a stroke of the eye. Observational studies suggest that thrombolytics may restore ocular perfusion and visual function. We hypothesized that intravenous tissue-type plasminogen activator (tPA) administered within 24 hours of symptom onset might restore ocular perfusion and visual function. Methods— A placebo-controlled, randomized trial of intravenous tPA versus intravenous saline was performed in patients with clinically defined central retinal artery occlusion within 24 hours of symptom onset. tPA was administered at a total dose of 0.9 mg/kg, with 10% given as a 1-minute bolus and the remainder over 1 hour. An improvement of visual acuity of 3 lines or more was considered significant. Results— Twenty-five percent (2 of 8) of the tPA group experienced the primary outcome at 1 week after tPA versus none of the placebo group. One patient had an intracranial hemorrhage. The visual acuity improvement of these 2 patients was not sustained at 6 months. In both patients, tPA was administered within 6 hours of symptom onset. Conclusions— Although essentially a negative study, it does add to the evidence base of reperfusion in central retinal artery occlusion by showing that the time window for intervention is likely to be <6 hours. Reocclusion is a potential problem and may require adjuvant anticoagulation. Future studies should concentrate on determining the efficacy of thrombolytics in the <6-hour time window. Clinical Trial Registration— URL: http://www.anzctr.org.au. Unique identifier: 83102.

Local Intraarterial Fibrinolysis Administered in Aliquots for the Treatment of Central Retinal Artery Occlusion. The Johns Hopkins Hospital Experience

Background and Purpose— Central retinal artery occlusion results in acute visual loss with poor spontaneous recovery. Current standard therapies do not alter the natural history of disease. Several open-label clinical studies using continuous infusion of thrombolytic agents have suggested that local intraarterial fibrinolysis (LIF) is efficacious in the treatment of central retinal artery occlusion. The aim is to compare the visual outcome in patients with acute central retinal artery occlusion of presumed thromboembolic etiology treated with LIF administered in aliquots with that of patients treated with standard therapy. Methods— We conducted a single-center, nonrandomized interventional study of consecutive patients with acute central retinal artery occlusion from July 1999 to July 2006. Results— Twenty-one patients received LIF and 21 received standard therapy. Seventy-six percent of subjects in the LIF group had a visual acuity improvement of one line or more compared with 33% in the standard therapy group (P=0.012, Fisher exact). Multivariate logistic regression controlling for gender, history of prior stroke/transient ischemic attack, and history of hypercholesterolemia showed that patients who received tissue plasminogen activator were 36 times more likely to have improvement in visual acuity (P=0.0001) after adjusting for these covariates. Post hoc analysis showed that patients who received tissue plasminogen activator were 13 times more likely to have improvement in visual acuity of 3 lines or more (P=0.03) and 4.9 times more likely to have a final visual acuity of 20/200 or better (P=0.04). Two groin hematomas were documented in the LIF group. No ischemic strokes, retinal or intracerebral hemorrhages were documented. Conclusions— LIF administered in aliquots is associated with an improvement in visual acuity compared with standard therapy and has few side effects.

Mapped serial excision for periocular lentigo maligna and lentigo maligna melanoma.

PURPOSE To report the early cure rate for periocular lentigo maligna (LM) and LM melanoma (LMM), using modified Mohs surgery with vertically cut paraffin-embedded sections (mapped serial excision [MSE]). A secondary aim was to identify differences in the clinical features and outcomes between periocular LM and LMM and those found elsewhere on the head and neck. DESIGN Prospective, noncomparative, interventional case series. PARTICIPANTS One hundred thirty-five patients undergoing 141 MSE procedures. METHODS A prospective series of 141 MSE procedures for LM and LMM over a 10-year period (1993-2002) in a single-center Mohs surgical unit. MAIN OUTCOME MEASURES Recurrence, site, size of LM or LMM, invasiveness, prior recurrence, clear margin of excision, size of final defect, and number of levels required for complete excision. RESULTS One hundred forty-one MSE procedures, of which 23% (32/141) were for LMM and 19% (27/141) were for periocular lesions. Location or prior recurrence were not predictive of invasive disease; however, the size distribution of the initial lesion (P = 0.0354) and the final defect after MSE (P = 0.0183) were larger in LMM. Thirty-one percent of LM and 14% of LMM less than 1 mm thick required larger than 5-mm and 1-cm margins, respectively, for complete excision. Mean follow-up of 32 months (range, 1-100 months) revealed 4 recurrences (3%), of which two were periocular (P = 0.188). CONCLUSIONS Our review is the largest prospective series of MSE for LM and LMM and suggests that it is the treatment of choice in these forms of melanoma. Mapped serial excision offers a high early cure rate in conjunction with tissue conservation, which is of particular relevance in the periocular region. There were no significant differences between periocular LM and LMM and those found elsewhere in the head and neck region. It also appears that the current recommendations of 5-mm margins for in situ melanoma (LM) and 1-cm margins for melanoma less than 1 mm thick are insufficient for complete excision of LM or LMM, emphasizing the importance of margin-controlled excision of these lesions.

Craniopharyngioma: a review of long-term visual outcome

Purpose: To assess the clinical presentation and long‐term visual outcome in a series of patients with craniopharyn‐gioma.

Juvenile arthritis-associated uveitis: visual outcomes and prognosis

BACKGROUND The current issues in the management of uveitis associated with juvenile arthritis revolve mainly around the treatment of mild disease and how to treat patients with more severe disease. The aims of this study were to determine the incidence of uveitis in a cohort of patients with juvenile arthritis as well as the nature of treatment and the risk factors for visual loss. METHODS Review of the charts of 71 patients with juvenile arthritis, as defined by the American Academy of Rheumatology, seen between 1992 and 2001 at a combined rheumatology and ophthalmology clinic. Information collected included the patients sex, age at diagnosis of arthritis and uveitis, and date of diagnosis of arthritis and uveitis. The rheumatologic diagnosis, results of serologic testing, and details of systemic and topical treatments were also recorded. RESULTS There were 47 girls and 24 boys ranging in age from 16 months to 13 years. The median age at diagnosis of juvenile arthritis was 4 years and 1 month. Twenty-seven patients (38%) had uveitis. The median age at uveitis onset was 5.9 years, with an average interval of 18 months from the diagnosis of arthritis; 11 patients had uveitis at the time of arthritis diagnosis. There was a positive relation between anti-nuclear antibody positivity and the development of uveitis (p < 0.05). Thirteen (48%) of the 27 patients with uveitis had mild anterior segment inflammation, with fewer than 25 cells in the anterior chamber. This group had spontaneous resolution of uveitis without topical therapy. All the patients without uveitis had a final visual acuity of 6/9 or better. Five of the patients with uveitis had a final visual acuity of 6/36 or worse. Cataract was the most common complication affecting visual outcome. Cataract extraction initially improved the visual acuity, but posterior segment complications and glaucoma compromised the final visual outcome. INTERPRETATION We found an incidence of uveitis of 38% with long-term follow-up of patients with juvenile arthritis; the uveitis was diagnosed an average of 18 months after the arthritis. Almost half of the patients with uveitis had minor anterior segment inflammation. These patients did not receive topical treatment and had good visual outcomes. Patients with uveitis at the time of diagnosis of arthritis tended to have a worse visual prognosis and experienced persistent uveitis despite treatment. In this series, cataract extraction was beneficial in improving visual acuity immediately postoperatively, but posterior segment changes and glaucoma may compromise final visual outcomes.

Management of acute central retinal artery occlusion

Central retinal artery occlusion (CRAO) is considered to be an acute stroke of the eye that results in profound visual loss. Spontaneous recovery rates are poor. Most CRAOs are caused by thromboembolism in the central retinal artery. Current standard therapies for CRAO that aim to restore perfusion to the retina and optic nerve head have not been shown to alter the natural course of the disease. Thrombolytic therapy for acute management of CRAO has shown promise in nonrandomized studies with regard to improving visual outcomes. A randomized controlled trial will be required to confirm the efficacy of thrombolytic therapy before it can be recommended for use in CRAO in daily clinical practice.

Late reactivation of thyroid orbitopathy

Background: Thyroid orbitopathy is characterized by spontaneous remissions and exacerbations. Although data on the natural history are scarce, the active phase is usually self‐limited, encompassing an average period of 18−36 months. Late reactivation, defined as active orbitopathy occurring after more than 5 years of quiescent disease, appears to be an uncommon and poorly documented event. The aim of the present study was to assess the incidence and characteristics of late reactivation of thyroid orbitopathy within a tertiary referral orbital clinic.

Vascular risk factors for central retinal artery occlusion

PurposeTo determine the proportion of patients presenting with thromboembolic central retinal artery occlusion (CRAO) who had undiagnosed vascular risk factors amenable to modification.MethodsA retrospective audit of consecutive patients with non-arteritic/thromboembolic CRAO presenting between 1997 and 2008 in a single tertiary teaching hospital.ResultsThirty-three patients with non-arteritic CRAO were identified. Twenty-one patients (64%) had at least one new vascular risk factor found after the retinal occlusive event, with hyperlipidemia being the most common undiagnosed vascular risk factor at the time of the sentinel CRAO event (36%). Nine patients (27%) had newly diagnosed hypertension or previous diagnosis of hypertension but not optimally controlled. To better control their vascular risk factors 18 patients (54%) were given a new or altered medication. Nine patients had more than 50% of ipsilateral carotid stenosis ; six of these proceeded with carotid endarterectomy or stenting. One patient had significant new echocardiogram finding. Systemic ischaemic event post CRAO occurred in two patients with stroke and acute coronary syndrome.ConclusionsPatients presenting with CRAO often have a previously undiagnosed vascular risk factor that may be amenable to medical or surgical treatment. As this population is at a high risk of secondary ischaemic events, risk factor modification is prudent.

Vision-Related Quality of Life in Patients with Complete Homonymous Hemianopia Post Stroke

Abstract Purpose: The aim is to determine the characteristics of vision-specific quality of life restriction using the National Eye Institute Visual Function Questionnaire (NEI VFQ-25) and Veterans Affairs Low Vision Visual Function Questionnaire (VA LV VFQ-48) in patients with complete homonymous hemianopia (HH) post stroke. Method: Prospective cohort study of patients with complete HH compared to age- and gender-matched subjects with normal visual fields. Results: In the NEI VFQ-25, scores on five subscales were significantly reduced after multiple logistic regression accounting for vision and comorbidities. The five subscales are vision-specific social functioning, vision-specific mental health, vision-specific dependency, driving, and peripheral vision. In the VA LV VFQ-48 postregression analysis, mobility is the only domain that is significantly affected in the people with HH. Conclusion: Identification of the significant areas of visual difficulties and their effects on quality of life is important as it can help better address the patients’ rehabilitation needs. The current study identifies the need for orientation and mobility training as well as independent living rehabilitation in patients with HH to help address the difficulties in their vision-specific quality of life and maximize their residual vision.