Association of outer retinal layer morphology with visual acuity in patients with retinal vein occlusion: SCORE Study Report 13.

PURPOSE: To assess associations between visual acuity (VA) and the status of the photoreceptor inner segment-outer segment (IS-OS) junction in a subset of patients in the Standard Care vs COrticosteroid for REtinal Vein Occlusion (SCORE) Study. METHODOLOGY: High-resolution time domain optical coherence tomography (OCT) scans of study eyes from a single site participating in the SCORE Study were evaluated. Integrity of the IS-OS junction in the central subfield was evaluated using a three-step scale: absent, abnormal or normal. Associations of the IS-OS status with ETDRS VA letter score and center point thickness (CPT) were investigated. RESULTS: Baseline OCTs of 42 eyes were evaluated. The IS-OS junction was absent in 30 (71%) and abnormal in 12 (29%). At month 12, the IS-OS junction was absent in 18 (43%), abnormal in 12 (28%), and normal in 12 (28%) eyes. At baseline, IS-OS status was significantly associated with CPT, but not with VA. At month 12, IS-OS status was significantly associated with CPT and VA, that is, absent or abnormal IS-OS was associated with increased CPT and worse VA. Change in IS-OS status was not associated with change in CPT (P=0.8). Worsening of IS-OS status was associated with loss of VA and improvement in IS-OS status to normal was associated with gain in VA (P=0.03). CONCLUSION: In this data set with long-term follow-up of OCTs as part of the SCORE Study, there is a correlation between change in IS-OS status and VA. This supports further evaluation of outer retinal morphology in larger data sets.

Intraoperative bleeding during vitrectomy for diabetic tractional retinal detachment with versus without preoperative intravitreal bevacizumab (IBeTra study).

AIMS: To compare the amount of intraoperative intraocular bleeding in patients with diabetes with macula-involving tractional retinal detachment (TRD) undergoing pars plana vitrectomy (PPV) with and without preoperative intravitreal bevacizumab (IVB) injection. METHODS: An institutional study was carried out with consecutive patients with diabetic retinopathy and macula-involving TRD of recent (3 months) onset who were randomly assigned to PPV only (PPV group) or PPV combined with one IVB (1.5 mg/0.06 ml) injection 2 weeks prior to surgery (bevacizumab (BEV)/PPV group). All patients underwent 23-gauge PPV 3 weeks after baseline. The main outcome measure was erythrocyte count in the fluid retrieved from the vitrectomy cassette using a Neubauer counting chamber. RESULTS: The study included 20 patients. The mean erythrocyte count was 14,865x10(3) (SD 19,332x10(3); median 4,500x10(3)) cells in the BEV/PPV group, and 176,240x10(3) (SD 108,375x10(3); median 166,600x10(3)) cells in the PPV group. The mean erythrocyte count was significantly lower in the BEV/PPV group than in the PPV group (p<0.0001). No major adverse events were identified. CONCLUSION: Preoperative IVB injection was associated with reduced intraocular bleeding during 23-gauge PPV for diabetic macula-involving TRD. Further studies are needed to confirm our preliminary findings. TRIAL REGISTRATION NUMBER: NCT00690768.

Nonpenetrating deep sclerectomy with reticulated hyaluronic acid implant versus punch trabeculectomy: a prospective clinical trial.

PURPOSE: To compare the intraocular pressure (IOP)-lowering effect and complication rate of nonpenetrating deep sclerectomy (NPDS) with reticulated hyaluronic acid (SK-GEL) scleral implant versus traditional punch trabeculectomy (PT) in the management of primary open angle glaucoma (POAG). METHODS: Prospective, randomized comparative study including 93 patients with uncontrolled POAG. Group 1 (43 eyes) underwent NPDS with SK-GEL scleral implant; Group 2 (50 eyes) underwent PT. Mitomycin C (0.2 mg/mL) was applied intraoperatively in both techniques. Study follow-up evaluations were conducted at 36 and 48 months. Complete success indicated the achievement of the target IOP without antiglaucoma medications, while qualified success indicated the same goal with medications. These categories were assessed at two target IOP levels, <21 mmHg and <18 mmHg. RESULTS: At 36 months for complete and qualified success with a <21 and <18 mmHg target IOP, no significant differences were noted between the two groups. At 48 months postprocedure when a <21 mmHg IOP target was considered, the rate of eyes that achieved complete success was 51.1% in the NPDS group versus 72% in the PT group (p<0.05). As for the <18 mmHg IOP target, the rate of eyes that achieved complete success was 32.5% in the NPDS group versus 44% in the PT group (p<0.05). Complications occurred significantly more frequently after PT than after NPDS. CONCLUSIONS: The IOP-lowering effects of the two procedures were comparable at 36 months. At 48 months PT showed a significantly higher rate of complete success compared with NPDS. Complications were more frequent after PT than after NPDS.

Orbital and ocular manifestations of acute childhood leukemia: clinical and statistical analysis of 180 patients.

PURPOSE: To investigate the association between presence of orbital or ocular lesions and type and stage of leukemia and to investigate whether orbital and ocular lesions are significant in predicting leukemia prognosis. METHODS: The authors evaluated 180 patients with acute childhood leukemia. Lesions associated with leukemia may be classified as specific (due to leukemic infiltration of various ocular tissues), nonspecific (due to one of the secondary complications), or iatrogenic manifestations caused by chemotherapy. Risk-based treatment assignment is based on clinical and laboratory features at diagnosis. Children with presenting white blood cell count below 50,000 mm3 are considered at standard risk for treatment failure, while all others are considered at high risk for treatment failure. RESULTS: Specific lesions were noted in 66% of patients with acute myeloid leukemia (AML) and 11.5% patients with acute lymphocytic leukemia (ALL) (p<0.05), and were more severe in patients with high risk leukemia than in patients with standard risk leukemia. Orbital or ocular lesions were noted more commonly in patients with AML (66.6%) compared to patients with ALL (15.1%). In both the AML and ALL groups, there was a higher frequency of leukemic relapses in the bone marrow and/or central nervous system in patients with specific lesions (63.1%) compared to patients with nonspecific lesions (42%), and in patients without orbital or ocular lesions (29.2%) (p<0.05). CONCLUSIONS: In both the AML and ALL groups, the presence of specific orbital or ocular lesions was associated with a higher frequency of bone marrow relapses and CNS involvement (p<0.05), leading to a lower survival rate.

Intravitreal triamcinolone versus bevacizumab for treatment of refractory diabetic macular oedema (IBEME study).

BACKGROUND/AIMS: The aim of this study was to compare the morphological and visual acuity outcomes associated with a single intravitreal injection of triamcinolone acetonide versus bevacizumab for the treatment of refractory diffuse diabetic macular oedema. METHODS: Twenty-eight patients were randomly assigned to receive a single intravitreal injection of either 4 mg/0.1 ml triamcinolone acetonide or 1.5 mg/0.06 ml bevacizumab. Comprehensive ophthalmic evaluation was performed at baseline and at weeks 1, 4, 8 (+/-1), 12 (+/-2) and 24 (+/-2) after treatment. Main outcome measures included central macular thickness measured with optical coherence tomography (OCT) and best corrected Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity. RESULTS: Twenty-six patients (26 eyes) completed all study visits (two patients missed two consecutive study visits). Central macular thickness was significantly reduced in the intravitreal triamcinolone group compared with the bevacizumab group at weeks 4, 8, 12 and 24 (p<0.05). Logarithm of the minimum angle of resolution (LogMAR) best-corrected visual acuity was significantly higher at weeks 8 (0.69; approximately 20/100(+1)) and 12 (0.74; 20/100(-2)) in the intravitreal triamcinolone group compared with the bevacizumab group (weeks 8 (0.83; approximately 20/125(-1)) and 12 (0.86; 20/160(+2))) (p<0.05). Significant change from baseline in mean intraocular pressure (mmHg) was seen at week 4 (+2.25) only in the intravitreal triamcinolone group (p<0.0001). No patient had observed cataract progression during the study. CONCLUSIONS: One single intravitreal injection of triamcinolone may offer certain advantages over bevacizumab in the short-term management of refractory diabetic macular oedema, specifically with regard to changes in central macular thickness. The actual clinical relevance of our preliminary findings, however, remains to be determined in future larger studies.

Extraocular muscle metastasis 16 years following primary prostate carcinoma.

An 83-year-old man with a history of localized prostate carcinoma treated 16 years prior with transurethral partial prostatectomy and radioactive seed implants presented with a 2-month history of right globe proptosis. Computed tomography demonstrated a right medial rectus mass. Serum prostate specific antigen (PSA) level was normal. Tumor biopsy revealed poorly differentiated carcinoma with diffuse staining of tumor cells with PSA and prostatic acid phosphatase. This case demonstrates that prostate carcinoma may metastasize to an extraocular muscle many years after treatment of the primary tumor and may not be accompanied by elevated serum PSA. Immunohistochemical stains are helpful in establishing the diagnosis.

Endophthalmitis caused by Moraxella species.

PURPOSE: To report the incidence, clinical presentation, antibiotic sensitivities, and treatment outcomes for endophthalmitis caused by Moraxella species. METHODS: Consecutive interventional case series. Medical records were reviewed of all patients treated at Bascom Palmer Eye Institute between 1991 and 2000 for endophthalmitis caused by Moraxella species. RESULTS: Moraxella species were recovered from 9 patients (10 eyes), or 1.3% (10 of 757) of all culture-proven bacterial endophthalmitis cases; Moraxella catarrhalis was recovered from 7 eyes and Moraxella osloensis from 3. Endophthalmitis was delayed-onset (5 months to 10 years postoperatively) and bleb-associated in 9 eyes and trauma-related in 1. All isolates were sensitive to ceftazidime, ciprofloxacin, and the aminoglycosides, and they were resistant to vancomycin; resistance to ampicillin and trimethoprim/sulfa was 11%. Although presenting vision was hand motion or worse in 7 of 10 eyes, all but 3 regained baseline visual acuity (including two eyes in which the post-treatment course was complicated by retinal detachment and one eye with coexistent traumatic injuries). CONCLUSION: Endophthalmitis caused by Moraxella species is usually delayed-onset and bleb-associated. Although patients usually present with a profound decrease in vision, the organisms are sensitive to most antibiotics and, unlike most series of delayed-onset bleb-associated endophthalmitis, visual outcomes are generally good unless coexistent ocular morbidities exist.

Optic neuropathy and chiasmopathy in the diagnosis of systemic lupus erythematosus.

PURPOSE: To report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE). METHODS: Retrospective case series. RESULTS: All patients had a positive antinuclear antibody and elevated anti-double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications. CONCLUSIONS: Visual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.

Corneal graft survival and intraocular pressure control after penetrating keratoplasty and glaucoma drainage device implantation.

OBJECTIVE: To investigate corneal graft survival rates and intraocular pressure (IOP) control in eyes after penetrating keratoplasty (PK) and glaucoma drainage device (GDD) implantation. DESIGN: Retrospective, comparative, consecutive case series. PARTICIPANTS: All patients who underwent PK and GDD implantation at the Bascom Palmer Eye Institute between January 1, 1993 and October 31, 1998. MAIN OUTCOME MEASURES: Graft clarity and IOP control. RESULTS: Of the 72 eyes in 72 patients identified, 47 (65%) underwent combined PK and GDD implantation, and 25 (35%) underwent GDD placement after PK (2-30 months after PK; median, 13 months). The GDD type was Baerveldt 350 mm(2) in 57 eyes, Ahmed in 9, Krupin in 2, and other in 4 eyes. The GDD was placed in the anterior chamber in 54 eyes (75%) and in the vitreous cavity in 18 eyes (25%). Preoperative IOP was 11 to 53 mmHg with or without antiglaucoma medications in 16 eyes (30%) with the GDD implanted in the anterior chamber and in 4 eyes (22%) with the GDD placed in the vitreous cavity (P = 0.76). At 1 year after GDD implantation, the graft was clear in 26 eyes (48%) with the GDD in the anterior chamber compared with 15 eyes (83%) with the GDD in the vitreous cavity (P = 0.013). Forty-eight eyes (89%) with the GDD in the anterior chamber and 18 eyes (100%) with the GDD in the vitreous cavity had IOP between 5 and 21 mmHg with or without antiglaucoma medications (P = 0.33). The mean reduction in IOP, 1 year after surgery, was 12 mmHg among eyes with the GDD in the anterior chamber, compared with 17 mmHg among eyes with the GDD in the vitreous cavity (P = 0.13) CONCLUSIONS: Corneal graft survival at 1 year is significantly higher among eyes with the GDD implanted in the vitreous cavity compared with those in which the GDD is implanted in the anterior chamber. The IOP was significantly lower at 1 year after surgery compared with before surgery in both groups, and there was no significant difference between the groups in IOP control and amount of IOP reduction. There was no significant difference in corneal graft survival or IOP control between eyes with the GDD implanted concurrently with the PK versus after the PK.

Surgical removal of submacular choroidal neovascularization: a clinicopathologic study and factors influencing visual outcomes.

BACKGROUND AND OBJECTIVE: Clinical and histopathologic characteristics of submacular choroidal neovascular membranes (CNV) are investigated and features predictive of postoperative complications or poorer visual outcome following CNV removal are identified. MATERIALS AND METHODS: Records of patients who underwent submacular CNV removal at Bascom Palmer Eye Institute between 1991 and 1998 are reviewed. RESULTS: At 6 months postoperatively, vision was stable or improved in 19/26 (73%) eyes, with 15/26 (58%) having 20/200 to 20/400 vision. Postoperative retinal detachment occurred more commonly in age-related macular degeneration eyes (AMD) (P <0.05), and CNV recurrence occurred most frequently in presumed ocular histoplasmosis (POHS) eyes (P <0.05). The one eye with an extrafoveal CNV in-growth site had the largest improvement in vision postoperatively. Choroid in the specimen was associated with worse postoperative vision (P = 0.05). CONCLUSION: Submacular CNV removal achieves visual stabilization at the 20/200 to 20/400 level in most eyes. Potential risk factors for postoperative complications or poorer visual outcome include the underlying disease process, subfoveal in-growth site of CNV, and presence of choroid in the specimen.

Endogenous Pseudallescheria boydii endophthalmitis in a patient with ring-enhancing brain lesions.

A 46-year-old man, status post liver transplantation and taking immunosuppressive medications, was admitted after suffering a generalized seizure. Magnetic resonance imaging of the brain revealed two ring-enhancing lesions and treatment was begun for presumed toxoplasmic encephalitis. He was already receiving amphotericin B for a skin lesion suspected to be caused by candidiasis. One day after the seizure, he complained of photophobia in the left eye. Intraocular inflammation and a small infiltrate in the macula were seen. Vision deteriorated over the next three days. Vitreous tap and injection of amphotericin B and vancomycin were performed, but the intraocular inflammation continued to increase. On day 15, a vitrectomy was performed. The vitreous specimen ultimately grew Pseudallescheria boydii. The patient died on hospital day 30 from complications of the brain abscesses. Pseudallescheria boydii should be considered in the differential diagnosis of endophthalmitis, especially in patients with immunosuppression, serious medical disease, or ring-enhancing brain lesions.

Late endophthalmitis associated with glaucoma drainage implants.

OBJECTIVE: To report the clinical course of a series of patients who had late endophthalmitis develop after glaucoma drainage implant (GDI) surgery. DESIGN: Noncomparative, interventional, consecutive case series. PARTICIPANTS: Four patients were identified with late endophthalmitis associated with Baerveldt glaucoma implants. METHODS: The medical records of all patients with endophthalmitis associated with a GDI treated at the Bascom Palmer Eye Institute or University of Florida between January 1, 1987 and December 31, 1999 were retrospectively reviewed. Patients with culture-positive endophthalmitis diagnosed more than 1 month after GDI surgery were included in this series. MAIN OUTCOME MEASURES: Visual acuity and intraocular pressure (IOP). RESULTS: Late endophthalmitis associated with Baerveldt glaucoma implants developed 7 weeks to 2 years postoperatively. Exposure of the GDI tube was present in all cases. The implant was removed in three of four patients. Visual acuity worsened from preinfection level in two of four cases. IOP was controlled at last follow-up in all patients, although replacement of the explanted GDI was required in one patient. CONCLUSIONS: Late endophthalmitis may occur after GDI surgery. Exposure of the GDI tube seems to represent a major risk factor for these infections. To prevent this potentially devastating complication, we recommend prophylactic surgical revision with a patch graft in all cases in which there is an exposed GDI tube.

Echography of retinoblastoma: histopathologic correlation and serial evaluation after globe-conserving radiotherapy or chemotherapy.

PURPOSE: To assess the sensitivity of echography in detecting retinoblastoma, compare tumor features observed by echography with histopathology data, and assess the usefulness of echography in serially following retinoblastoma tumors after globe-conserving treatments. METHODS: The medical and echography records of all patients treated for retinoblastoma at the Bascom Palmer Eye Institute between 1991 and 1997 were reviewed. All eyes underwent pretreatment echographic evaluation, and eyes treated with external beam radiotherapy, brachytherapy, or chemotherapy underwent serial follow-up echography. RESULTS: Sixty-nine eyes of 48 patients were identified. Echography demonstrated evidence of retinoblastoma in 69 of 69 (100%) eyes and calcification in 63 (91.3%) eyes. Histopathology was superior to echography in detecting optic nerve invasion, extraocular extension, and presence of calcification. CONCLUSION: Echography is a useful adjunct to indirect ophthalmoscopy in establishing the diagnosis of retinoblastoma. While not as specific as histopathology, echographic evaluation before and after treatment of retinoblastoma permits monitoring of treatment response and may aid in detecting recurrent tumor growth or failure to respond to treatment.

Emotional distress in patients with retinal disease.

PURPOSE: To investigate the prevalence of, and potential risk factors for, emotional distress among patients with retinal disease. DESIGN: Cross-sectional study. METHODS: Cases consisted of 86 consecutive patients at Wilmer Eye Institute Retinal Vascular Center. Fifty-one controls with normal visual acuity and no known ocular disease were frequency-matched to the cases by age, sex, and race. Subjects were interviewed using the Community Disability Scale, a functional status questionnaire, and the General Health Questionnaire, a questionnaire assessing emotional distress. RESULTS: The prevalence of probable (General Health Questionnaire score 4 or greater and less than 10) or definite (General Health Questionnaire score 10 or greater) emotional distress was 59.3% among ophthalmic patients and 2.0% among controls. There were significant relationships between emotional distress, as assessed by General Health Questionnaire score, and degree of 1) visual impairment, as assessed by weighted bilateral average logarithm of minimal angle of resolution (logMAR), and 2) functional impairment, as assessed by Community Disability Scale score (P =.001). Univariate analyses identified significant predictors of emotional distress: shorter duration of ocular disease (P =.019), worse visual acuity (P =.001), increased systemic comorbidities (P =.001), and increased functional impairment (P <.001). Multiple regression analysis demonstrates that worse visual acuity, increased systemic comorbidities, and shorter duration of ocular disease each explain over 10% of the variability in General Health Questionnaire score (r(2) = 0.15, 0.12, and 0.11, respectively). Addition of Community Disability Scale score to the regression model eliminates the significance of visual acuity and systemic comorbidities; Community Disability Scale score explains nearly 30% of the variability in emotional distress, as assessed by General Health Questionnaire score (r(2) = 0.29). CONCLUSIONS: Emotional distress is prevalent among patients with retinal disease; potential risk factors for emotional distress among such patients include shorter duration of ocular disease, worse visual acuity, increased systemic comorbidities, and increased functional impairment.

Visual hallucinations in patients with retinal disease.

PURPOSE: To investigate the prevalence and phenomenologic nature of visual hallucinations among patients with retinal disease and to investigate whether presence of hallucinations is a significant predictor of functional status, quality of life, and/or emotional distress after adjusting for visual acuity. DESIGN: Cross-sectional study. METHODS: Eighty-six consecutive patients at the Wilmer Ophthalmologic Institute Retinal Vascular Center were interviewed using the Sickness Impact Profile, Community Disability Scale, General Health Questionnaire, Visual Phenomena Interview, Eysenck Personality Questionnaire, and Telephone Interview for Cognitive Status. RESULTS: The prevalence of visual hallucinations was 15.1%. Most were formed hallucinations in clear consciousness that lasted for seconds to minutes. The majority of patients had been experiencing visual hallucinations for less than 1 year (61.5%) or for 1 to 2 years (23.1%). Only two of the 13 patients with hallucinations had informed a physician of their hallucinations. Univariate analyses revealed that variables significantly associated with experiencing hallucinations were female sex, worse visual acuity, bilateral visual impairment, emotional distress, decreased functional status, and decreased quality of life. Regression analysis demonstrated that among patients with relatively good vision, those who experienced hallucinations were more emotionally distressed and had a lower quality of life than patients without hallucinations. CONCLUSIONS: Visual hallucinations among patients with retinal disease are common, underdiagnosed, and not associated with cognitive deficits, abnormal personality traits, or a family or personal history of psychiatric morbidity. Among patients with relatively good vision, hallucinations are associated with increased emotional distress and decreased quality of life.

Long-term follow-up of unoperated macular holes.

OBJECTIVE: To investigate the anatomic and visual acuity outcomes among patients with unoperated macular holes and at least 5 years of follow-up. DESIGN: Retrospective, noncomparative case series from an institutional practice setting. PARTICIPANTS: All patients with unoperated full-thickness macular holes evaluated at Bascom Palmer Eye Institute between January 1, 1968 and December 31, 1993 and observed for at least 5 years. METHODS: Demographic and clinical data were abstracted from patients' medical records and ophthalmologic photography records. For patients with bilateral macular holes, only one eye was included. MAIN OUTCOME MEASURES: Visual acuity and clinical features on initial examination, at 5 years, and at final follow-up. RESULTS: The study included 65 eyes of 65 patients with a median age of 65 years (range, 52-85 years) and a median follow-up of 9.3 years (range, 5-29 years). On initial examination at Bascom Palmer Eye Institute, the macular hole was stage 2 in 15 eyes (24%), stage 3 in 23 eyes (37%), and stage 4 in 25 eyes (40%). At final follow-up, the macular hole was stage 3 in 10 eyes (16%) and stage 4 in 53 eyes (84%). Visual acuity was 20/200 or worse in 35 eyes (54%) on initial examination, in 43 eyes (74%) at 5 years, and in 53 eyes (82%) at final follow-up. Poorer visual acuity on initial examination was a significant predictor of poorer final vision (P < 0.01). Other accompanying clinical features such as the presence of operculum, posterior vitreous detachment, and epiretinal membrane were not significantly associated with final vision. Throughout follow-up, there was a redistribution and reduced number of yellow nodular opacities at the level of the retinal pigment epithelium at the base of the macular holes and the development of retinal pigment epithelial atrophy around the macular holes. CONCLUSIONS: Long-term follow-up of unoperated macular holes demonstrates progression in hole size and stage, vision loss which generally stabilizes at the 20/200 to 20/400 level, a redistribution and reduced number of yellow nodular opacities at the level of the retinal pigment epithelium, and the development of retinal pigment epithelial atrophy surrounding the macular hole, resulting in a "bull's-eye" macular appearance.

Echographic evaluation of a patient with diabetes and dense vitreous hemorrhage: an avulsed retinal vessel may mimic a tractional retinal detachment.

PURPOSE: To report that an avulsed retinal vessel may appear as a tractional retinal detachment on echographic evaluation. METHODS: Case report. RESULTS: A 57-year-old diabetic woman presented with a nonclearing vitreous hemorrhage of 2 months duration in the left eye. Echography was consistent with a localized tractional retinal detachment on longitudinal sections; transverse sections demonstrated a pinpoint opacity in the vitreous cavity. Intraoperatively, an avulsed retinal vessel was noted in the area of echographic abnormality. CONCLUSION: An avulsed retinal vessel may mimic tractional retinal detachment on echography. Although trained ophthalmic echographers routinely perform both longitudinal and transverse sections during an echographic evaluation, less skilled observers must be aware of the importance of performing both longitudinal and transverse sections for accurate echographic diagnosis.

Long-term visual outcome in patients with anterior visual pathway gliomas.

OBJECTIVES: To investigate the visual outcomes of patients with gliomas of the anterior visual pathway and the clinical features associated with prognosis. MATERIALS AND METHODS: During retrospective review, demographic and clinical data were abstracted from medical records of patients seen at the Bascom Palmer Eye Institute between January 1, 1970 and December 31, 1998. RESULTS: Of the 42 patients identified, 68% were female, and 55% had neurofibromatosis (NF) type I. There was no substantial difference in presenting signs, symptoms, and visual acuity between the NF(+) and NF(-) groups except for nystagmus, which was more common in the NF(-) group (p = 0.014). Throughout follow-up evaluation, vision in the better eye remained stable in both groups, independent of treatment or NF status. Vision in the worse eye often declined, despite treatment. However, binocular visual status, measured as average weighted logMAR (MAR, minimum angle of resolution) vision, did not change significantly over time, regardless of treatment or NF status. CONCLUSIONS: In the NF(+) and NF(-) groups, vision in the better eye remained stable, regardless of treatment, and vision in the worse eye often declined, despite treatment. Binocular visual acuity (measured as weighted logMAR) did not change significantly over time, regardless of NF status or treatment modality.