The impact of the optic neuritis treatment trial on the practices of ophthalmologists and neurologists

OBJECTIVE: To determine whether the Optic Neuritis Treatment Trial (ONTT) results have altered the practice patterns of ophthalmologists and neurologists. DESIGN: Mail survey. PARTICIPANTS: A random sample of 987 ophthalmologists and 900 neurologists practicing in the United States were mailed a questionnaire that inquired into decision-making with regard to management of optic neuritis before and after the publication of the ONTT results. MAIN OUTCOME MEASURES: Responses received from 202 ophthalmologists and 244 neurologists, a response rate of 47%. RESULTS: Following the ONTT reports, nearly all ophthalmologists and neurologists have reduced their use of oral prednisone alone, substituting a regimen that includes intravenous methylprednisolone. A large proportion of practitioners in both specialties mistakenly believe that intravenous methylprednisolone treatment improves final visual outcome. Only 7% of neurologists and 36% of ophthalmologists (P = 0.0001) are adhering to the ONTT suggestion to use magnetic resonance imaging as a basis for initiating treatment. CONCLUSIONS: The ONTT has led to a dramatic reduction in the use of oral prednisone without a preceding course of intravenous methylprednisolone in the treatment of acute optic neuritis. Ophthalmologists and neurologists have changed some of their practices without fully understanding the results of the ONTT.     

Evaluation of the underlying causes of papilledema in children

ObjectiveThe purpose of this retrospective study was to identify the types and relative frequencies of intracranial disorders in pediatric patients who present with papilledema.DesignRetrospective case series.Participants and MethodsThis study was conducted in 2 pediatric ophthalmology clinics, both providing community-based care in a large inner-city urban center in the U.S. Pediatric patients aged between 0 and 16 years diagnosed with papilledema and who had an underlying etiology identified were included in the study. Patient demographic data, ophthalmologic examination findings, and diagnostic work-up results were identified from clinical records.ResultsThe mean age of 38 study patients (19 female, 19 male) was 8.6 ± 4.8 years. Of the 38 patients, 16 (42.1%) had idiopathic intracranial hypertension (IIH) as the underlying cause of the papilledema, 7 (18.4%) had a craniosynostosis disorder, 6 (15.8%) had intracranial tumours, 2 (5.3%) had primary hydrocephalus, and 1 (2.6%) patient each had transverse sinus thrombosis related to sinusitis, hypertensive crisis, subdural hematoma, intracranial abscess, Lyme disease, presumed neurosarcoidosis, and acute disseminated encephalomyelitis. Of the 6 intracranial tumours, 2 (33.3%) presented in the sellar/parasellar region, 2 (33.3%) in the posterior fossa, and 2 (33.3%) were in cortical locations.ConclusionClinicians should have a high index of suspicion for IIH and brain tumours in children presenting with papilledema. Patients with craniosynostosis should have routine eye examinations to monitor for asymptomatic papilledema. Understanding the relative incidence of etiologies for papilledema highlights the urgency of appropriate work-up and the need to consider low-frequency etiologies.     

Subretinal recombinant tissue plasminogen activator injection and pneumatic displacement of thick submacular hemorrhage in Age-Related macular degeneration

OBJECTIVE: To evaluate the anatomic and visual outcomes after a surgical procedure for displacement of thick submacular hemorrhage in patients with age-related macular degeneration. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-nine consecutive eyes of 28 patients with thick submacular hemorrhage secondary to age-related macular degeneration. INTERVENTION: The procedure consisted of a pars plana vitrectomy with subretinal recombinant tissue plasminogen activator injection (125 micrograms/ml) through a translocation microcannula, followed by fluid-air exchange and postoperative prone positioning [correction]. MAIN OUTCOME MEASURES: Intraoperative and postoperative surgical complications, preoperative and postoperative best-corrected visual acuities, postoperative fluorescein angiography results, and additional postoperative treatments. RESULTS: Total subfoveal blood displacement was achieved in 25 eyes, with subtotal displacement in the others. Based on fluorescein angiography results, 8 eyes were eligible for additional postoperative treatments. At 3 months, 17 eyes had gained more than 2 lines of visual acuity, whereas 3 had lost more than 2 lines. The difference between preoperative and 3-month postoperative visual acuity was statistically significant (P = 0.0167). Complications consisted of 2 cases of vitreous hemorrhage that cleared within 4 weeks. CONCLUSIONS: This surgical technique seems useful in displacing thick submacular hemorrhage secondary to age-related macular degeneration, allowing postoperative fluorescein angiography testing and, potentially, subsequent treatments. No significant complication from the procedure was identified. However, further controlled studies will be required to assess its efficacy in the management of this difficult clinical problem.     

Intravitreous tissue plasminogen activator injection and pneumatic displacement in the management of submacular hemorrhage complicating scleral buckling procedures

PURPOSE: To evaluate the efficacy of recombinant tissue plasminogen activator (rTPA) and sulfur hexafluoride (SF6) in displacing submacular hemorrhage in patients who had scleral buckling procedures complicated by the development of submacular hemorrhage. Final visual acuity and complications of the procedures were evaluated. METHODS: Sequential intravitreal injections of 50 microg rTPA in 0.1 mL and 0.4 cc SF6 were performed in eight patients who showed submacular hemorrhage 1 day after a scleral buckling procedure. The fundus was checked daily for 3 days after the injection of TPA and gas, and then was followed every week for 1 month and then every 2 months. Patients were observed for at least 6 months. Visual acuity and the status of complications were evaluated. RESULTS: Submacular hemorrhage was totally or partially displaced extramacularly in all patients on the day after rTPA and SF6 injection. Vitreous hemorrhage was present in all patients. The retina was attached in all patients and no recurrent retinal detachment was noted. Visual acuity was improved at 6 months after treatment in all seven of the patients with macula-off retinal detachments compared to the preoperative visual acuity. The last patient who had a macula-sparing retinal detachment had decreased vision (20/25) at 6 months compared to preoperatively. CONCLUSIONS: Recombinant tissue plasminogen activator and SF6 injection is an easy procedure that is less complicated than and as effective as internal drainage in patients with submacular hemorrhage developing as a complication of scleral buckling procedures.     

Management of submacular hemorrhage with intravitreal injection of tissue plasminogen activator and expansile gas

PURPOSE: To evaluate the clinical outcome of intravitreal tissue plasminogen activator (tPA) and expansile gas injection as a minimally invasive treatment for submacular hemorrhage (SMH). METHODS: This study was a retrospective clinical case series examining 104 eyes that received an intravitreal injection of 30-100 mcg of tPA and expansile gas (SF6 or C3F8) for SMH. The main outcomes evaluated were visual acuities (VA), anatomic displacement of submacular blood, and surgical complications. RESULTS:: A total of 85, 77, and 81 eyes were available at 1 week, 3 months, and 12 months follow up, respectively. Postoperatively, > or = 2 Snellen lines improvement were achieved in 43/85 eyes (51%) at 1 week, 49/77 eyes (63%) at 3 months, and 52/81 eyes (64%) at 12 months. Postoperative VA improvement was significantly associated with preoperative VA, submacular blood displacement, and the underlying cause of SMH. Diagnostic postoperative angiogram and clinical examination were possible at 8.2 +/- 7.4 weeks and 9.5 +/- 7.4 weeks, respectively. The observed complications included breakthrough vitreous hemorrhage in 8 eyes (8%) and retinal detachment in 3 eyes (3%). CONCLUSIONS: In this retrospective series, intravitreal injection of tPA and expansile gas was shown to be a safe and effective technique that can improve VA in most eyes with SMH and assist in the diagnosis of the underlying cause.     

Management of submacular hemorrhage by tissue plasminogen activator and SF6 gas injection

Summary The formation of platelet-fibrin clots causes degeneration of foveolar photoreceptors by subretinal hemorrhage. Patients: Twenty-two patients with submacular hemorrhages were treated by intravitreal injection of 50 μg tpa and 0.5 ml SF₆ gas within 1 h. Biomicroscopy, fundus photography and fluorescein angiography were performed pre- and postoperatively. Further multifocal electroretinograms were recorded. Results: The subfoveolar hemorrhages were dissoluted and displaced out of the macular region. The visual acuity increased in 18 cases and ranged from hand movement and 0.3 to values between 1/10 and 0.5. Conclusions: Toxic and hypoxic damage of foveolar photoreceptors by subretinal hemorrhage can be prevented by early and minimal invasive fibrinolytic therapy. After localization of choroidal neovascularizations, treatment by focal laser coagulation is possible in some cases.      

Associated facial contractions after seventh nerve palsy mimicking jaw-winking

A 51-year-old woman who had recovered from Bell's palsy complained of closure of her right eyelids synchronous with chewing. Examination revealed the closure to be synchronous with contraction of the orbicularis oris muscles, not jaw muscles. Other associated facial movements were detected. Electromyography demonstrated that the decrease in size of the palpebral fissure is from contraction of the orbicularis oculi muscles, not inhibition of the levator muscle, proving that this is the effect of aberrant seventh nerve regeneration. This is one of many mass action effects which can occur with seventh nerve regeneration and should not be termed a syndrome.     

Visual acuity outcomes of patients 50 years of age and older with high myopia and untreated choroidal neovascularization

OBJECTIVE: To evaluate visual outcomes of untreated submacular choroidal neovascularization (CNV) in patients 50 years of age and older with high myopia. DESIGN: Retrospective observational case series. PARTICIPANTS: Twenty-two eyes in 22 patients were studied. All were 50 years of age and older with myopia of 6.0 diopters (D) or greater or an axial length of 25.5 mm or greater. Patients had untreated CNV documented by clinical examination and fluorescein angiography at two medical centers between 1986 and 1997. INTERVENTION: Demographic and clinical data were abstracted from patients' medical records. MAIN OUTCOME MEASURE: Visual acuity at 1 year after CNV diagnosis. RESULTS: The study included 22 eyes of 22 patients (mean age, 63.1 years; mean refraction, -11.0 D). Baseline visual acuity (VA) in the study eye was 20/40 or greater in 2 (9%) patients, 20/50 to 20/150 in 9 (41%) patients, and 20/200 or less in 11 (50%) patients. Drusen were present in seven (32%) eyes, and lacquer cracks were noted in ten (45%) eyes. Mean refractive error was -7.0 D for patients with drusen and -12.5 D for patients without drusen. Choroidal neovascularization was less than 0.25 disc diameters (DD) in 11 (50%) eyes, 0.25 to 0.5 DD in 5 (23%) eyes, and greater than 0.5 DD in 6 (27%) eyes. Visual acuity in the study eye 1 year after CNV diagnosis was 20/40 or greater in 3 (14%) patients, 20/50 to 20/150 in 3 (14%) patients, and 20/200 or less in 16 (73%) eyes. The presence of drusen was significantly associated with older age and a lower degree of myopia but was not associated with size of the CNV or visual acuity outcome. CONCLUSION: When compared to patients younger than 50 years of age with high myopia and CNV reported in previous publications, the patients in the current series generally have poorer visual outcomes.     

Verteporfin photodynamic therapy retreatment of normal retina and choroid in the cynomolgus monkey.

OBJECTIVE: This study evaluated the effect of repeated photodynamic therapy (PDT) applications on normal primate retina and choroid using an intravenous infusion of liposomal benzoporphyrin derivative (verteporfin). DESIGN: This was an experimental study in a primate model. ANIMALS/CONTROLS: Six cynomolgus monkeys were used as experimental subjects and one monkey was used as a control subject. INTERVENTION: Three consecutive PDT treatments at 2-week intervals were applied over the center of the fovea or the optic nerve of each eye. Verteporfin was delivered by intravenous infusion at a dose of 6 mg/m2, 12 mg/m2, or 18 mg/m2. Laser irradiation was then applied using a diode laser (689 nm) with light doses and spot sizes kept constant. MAIN OUTCOME MEASURES: Findings were documented by fundus photography, fluorescein angiography, and light and electron microscopy. RESULTS: A cumulative dose response was seen angiographically and histologically with more severe damage to the retina and choroid noted at higher dye doses. Photodynamic therapy applied to the macula using the 6-mg/m2 verteporfin dose showed recovery of choriocapillaris, with mild retinal pigment epithelium and outer photoreceptor damage at 6 weeks. At this dose, the optic nerve showed few focal sites of axon atrophy and capillary loss. Treatments over the macula using the 12-mg/m2 and 18-mg/m2 doses led to chronic absence of choriocapillaris and photoreceptors at 6 weeks. One of two optic nerves became atrophic after PDT applications using dye doses of 12 mg/m2, and both optic nerves became atrophic in the 18-mg/m2 dye dose group. CONCLUSION: Limited damage to the retina, choroid, and optic nerve was present in primates treated with multiple PDT sessions using 6 mg/m2 verteporfin with light doses and the timing of irradiation kept constant. However, PDT using higher dye doses of 12 mg/m2 and 18 mg/m2 led to significant chronic damage to the normal retina, choroid, and optic nerve.     

Episcleritis in childhood

OBJECTIVE: To describe the characteristics and systemic disease associations of episcleritis in childhood. DESIGN: Retrospective, observational case series. PARTICIPANTS: Twelve children diagnosed with episcleritis between July 1981 and June 1998. METHODS/TESTING: Complete eye and systemic evaluations. MAIN OUTCOME MEASURES: Characteristics of episcleritis and presence and nature of concurrent systemic disease. RESULTS: The 12 children (10 boys and 2 girls) ranged in age from 13 months to 16 years. Five children had bilateral simple episcleritis, one had bilateral nodular episcleritis, and six had unilateral simple episcleritis. The eye examination was otherwise normal and recovery was uneventful in all cases. Six of the nine children older than 5 years of age had one of the following rheumatologic diseases: systemic lupus erythematosus, juvenile rheumatoid arthritis, spondyloarthropathy, inflammatory bowel disease, rheumatic fever, or polyarteritis nodosa. All three children younger than 5 years of age had simple episcleritis, an antecedent viral illness, and presented within 2 months of each other. CONCLUSIONS: Episcleritis is a rare occurrence in childhood, especially in children younger than 5 years of age. In older children, it is frequently associated with rheumatologic disease.     

Management of uveitic glaucoma with Ahmed glaucoma valve implantation

OBJECTIVE: To evaluate the safety and efficacy of Ahmed glaucoma valve implantation for the management of glaucoma associated with chronic uveitis. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nineteen patients (21 eyes) with chronic uveitis underwent Ahmed glaucoma valve implantation for uncontrolled glaucoma between 1995 and 1998. INTERVENTION: All patients had their uveitis controlled before surgery via immunomodulatory therapy. Ahmed glaucoma valve implantation was performed. Immunosuppression was continued in the early postoperative period for strict control of inflammation. MAIN OUTCOME MEASURES: Control of intraocular pressure (IOP). A secondary outcome measure was the number of antiglaucoma medications required to achieve the desired IOP. Visual acuity and complications associated with the surgery were monitored. RESULTS: The postoperative follow-up averaged 24.5 months. At the most recent visit, all 21 eyes had IOPs between 5 and 18 mmHg. The average pressure reduction after Ahmed glaucoma valve implantation was 23.7 mmHg. The average number of antiglaucoma medicines required to achieve the desired IOP was reduced from 3.5 before surgery to 0.6 after surgery. No eye lost even a single line of Snellen acuity at the most recent postoperative visit. Two eyes developed hypotony in the course of follow-up. One resolved without specific intervention, and the other eye required two autologous blood injections and tube ligature to correct the hypotony. One eye underwent Ahmed glaucoma valve replacement for abrupt valve failure. Two eyes underwent penetrating keratoplasty for reasons believed to be unrelated to the glaucoma surgery. Kaplan-Meier life-table analysis showed a cumulative probability of success after Ahmed glaucoma valve implantation of 94% at 1 year. CONCLUSIONS: Ahmed glaucoma valve implantation can be an effective and safe method in the management of uveitic glaucoma. The authors hypothesize that control of the patients' uveitis, through preoperative and long-term postoperative immunomodulatory therapy, may have contributed to the success rate reported herein.