Bahram Rahmani

The Cause-specific Prevalence of Visual Impairment in an Urban Population: The Baltimore Eye Survey

BACKGROUND Whereas population-based data on the causes of bilateral blindness have been reported, little information is available on the distribution of causes of central vision loss less severe than the criteria used to define legal blindness. This visual impairment is responsible for a high proportion of eye care service use and results in important reductions in functional status. METHODS Data from the Baltimore Eye Survey were used to estimate the cause-specific prevalence of visual impairment (best-corrected visual acuity worse than 20/40 but better than 20/200) among black and white residents of east Baltimore who were 40 years of age or older. Eligible subjects underwent a screening examination at a neighborhood location and, for those whose best-corrected visual acuity was less than 20/30, a definitive ophthalmologic examination at the Wilmer Eye Institute. RESULTS The prevalence of visual impairment was 2.7% in whites and 3.3% in blacks; the age-adjusted relative prevalence (B/W) was 1.75 (P = 0.01). The leading causes of visual impaired eyes were cataract (35.8%), age-related macular degeneration (14.2%), diabetic retinopathy (6.6%), glaucoma (4.7%), and other retinal disorders (7.3%). Cataract, diabetic retinopathy, and glaucoma were more common as a cause of visual impairment among blacks, whereas macular degeneration was more frequent among whites. More than 50% of all subjects had the potential for improvement in vision with appropriate surgical intervention. CONCLUSION Visual impairment is a prevalent condition among inner city adults 40 years of age or older. The distribution of causes suggests that improvements in the visual health of the population could be achieved with more effective delivery of efficacious ophthalmologic care.

Racial differences in the prevalence of age-related macular degeneration: The Baltimore Eye Survey

OBJECTIVE To determine the prevalence of age-related macular degeneration (AMD) and signs of age-related maculopathy in a population-based sample of blacks and whites 40 years of age or older from East Baltimore. DESIGN Cross-sectional population-based study. PARTICIPANTS A total of 5308 black and white subjects received a screening eye examination that included fundus photography. MAIN OUTCOME MEASURES Stereoscopic color fundus photographs were graded for the presence and severity of drusen, pigmentary abnormalities, geographic atrophy, and choroidal neovascularization in the macula. RESULTS Drusen > or = 64 microm were identified in about 20% of individuals in both groups, but large drusen (>125 microm) were more common among older whites (15% for whites versus 9% for blacks over 70). Pigmentary abnormalities were also more common among older whites (7.9% for whites versus 0.4% for blacks over 70). Age-related macular degeneration was more prevalent among whites than blacks. The prevalence of AMD was 2.1 % among whites over 70 years of age. No cases of AMD were detected among 243 black subjects in this age group. Logistic regression adjusting for age, sex and smoking (current, former, or never) detected an odds ratio of 4.4 (95% confidence interval: 1.5-12.4) for whites with AMD compared with blacks. CONCLUSION Although drusen are common in both blacks and whites over the age of 40, more severe forms of age-related maculopathy and late AMD are more prevalent in older whites.

An analysis of risk for secondary hemorrhage in traumatic hyphema

OBJECTIVE Factors such as size of hyphema, intraocular pressure, initial visual acuity, and use of steroids or antifibrinolytic drugs may be associated with the likelihood of rebleeding in traumatic hyphema. The association of the visual outcome with secondary hemorrhage has been questioned. DESIGN Randomized, placebo-controlled, clinical trial. PARTICIPANTS Two hundred and thirty-eight patients who had hyphema develop after blunt trauma. INTERVENTION Eighty patients received oral tranexamic acid, 80 patients received placebo, and 78 patients received oral prednisolone. MAIN OUTCOME MEASURES Secondary hemorrhage and vision at the time of discharge from the hospital were measured. RESULTS Rebleeding occurred in 43 (18%) of the patients and was prevented significantly by oral tranexamic acid compared with the placebo (odds ratios [OR] = 0.39; 95% confidence interval [CI], 0.17, 0.89). Occurrence of secondary hemorrhage had weak associations with initial high intraocular pressure (OR = 2.7; 95% CI, 0.99, 7.3) and initial visual acuity of 6/60 or less (OR = 1.8; 95% CI, 0.9, 3.7). Secondary hemorrhage had no statistical association with age, gender, oral prednisolone, size of hyphema, and retinal damage. Visual acuity of 6/60 or less at the time of discharge was significantly associated with rebleeding (OR = 10.5; 95% CI, 3.7, 29.2), initial visual acuity of 6/60 or less (OR = 9.9; 95% CI, 2.8, 38.0), retinal damage (OR = 14.6; 95% CI, 3.8, 55.8), and male gender (OR = 6.5; 95% CI, 1.4, 31.9). Final visual acuity had no significant statistical association with age, use of oral prednisolone or tranexamic acid, and size of hyphema. CONCLUSIONS High intraocular pressure and low vision at the time of first examination may be associated with increased chance of rebleeding. Retinal damage, secondary hemorrhage, male gender, and initial poor vision are associated with a worse visual outcome in patients with traumatic hyphema.

Comparison of tranexamic acid and prednisolone in the treatment of traumatic hyphema : A randomized clinical trial

OBJECTIVE Oral antifibrinolytics, oral steroids, and no oral treatment are the preferred medical treatments for traumatic hyphema. Antifibrinolytics and steroids have decreased the chance of rebleeding in some studies but failed to alter the clinical course in others. Rate of secondary hemorrhage seems variable among different geographic and ethnic groups of patients. Comparison of the treatments in each population is necessary to document the most effective method of preventing recurrent hemorrhage. DESIGN Randomized, placebo-controlled, clinical trial. PARTICIPANTS Two hundred thirty-eight patients in whom hyphema developed after a blunt trauma entered the study. INTERVENTION Eighty patients received 75 mg/kg per day oral tranexamic acid (TA) divided into 3 doses, 80 patients received a placebo with the same number of tablets and frequency as those of the TA group, and 78 patients received 0.75 mg/kg per day oral prednisolone divided into 2 doses. MAIN OUTCOME MEASURE Secondary hemorrhage during the hospital course was measured. RESULTS Secondary hemorrhage occurred in 8 patients (10%) of the TA group, 14 patients (18%) of the prednisolone group, and 21 patients (26%) of the placebo group. The difference between the incidence of rebleeding between TA and placebo groups was statistically significant (P = 0.008). Patients receiving a placebo had a greater chance of secondary bleeding than did patients receiving TA (odds ratios = 3.2; 95% confidence interval = 1.3, 7.5). The incidences of rebleeding were not significantly different in placebo versus prednisolone groups (P = 0.21) and TA versus prednisolone groups (P = 0.15). CONCLUSION In a population with a high rate of secondary bleeding, TA is more effective than oral prednisolone or no oral treatment in preventing rebleeding among patients with traumatic hyphema.

Comparison of silicon oil removal with various viscosities after complex retinal detachment surgery

BackgroundDespite the progress in vitreoretinal surgery and the importance of silicone oil as an adjunct for the treatment of complex forms of retinal detachment, controversy still surrounds the issue of selecting the proper oil viscosity for clinical use. Herein, we evaluate the outcomes of retinal detachment (RD) surgery after removing silicone oils of different viscosities.MethodsIn this retropsective cohort study, eighty-two eyes with surgically re-attached retinas, of which 53 were filled with 5000cs silicone oil and 29 with 1000cs silicone oil were enrolled. We evaluated the outcomes and complications following silicone oil removal. Final anatomic success (stable re-attachment), final visual acuity (VA) and intraocular pressure (IOP)were recorded and analysed.ResultsOf 82 eyes, 41 had proliferative vitreoretinopathy (PVR), 24 were associated with intraocular foreign bodies, 10 had endophthalmitis and 7 had proliferative diabetic retinopathy with tractional retinal detachment. Prior to silicone oil removal, the retina was attached in all eyes, 29% had VA ≥ 6/120 and 52% had IOP ≥ 21 mmHg. After silicone oil removal, the retina remained attached in 59(72%) of the eyes, 34% had VA ≥ 6/120 and 9% had IOP ≥ 21 mmHg. Comparing 1000cs and 5000cs silicone oil filled eyes, redetachment occurred more frequently in the latter group especially in cases with associated PVR. Final VA worse than 6/120 was associated with initial VA < 6/120 (OR = 32.2 95%CI 7.4–140.2) and use of 5000cs silicone oil (OR = 7.9 95%CI 1.9–32.2). No factor was significantly associated with final IOP ≥ 21 mmHg.ConclusionIn complicated retinal detachment surgery, use of 5000cs silicone oil may be associated with a poorer anatomic and visual outcome compared with 1000cs silicone oil. However there was no difference between the two viscosities in IOP elevation. A randomized controlled study is necessary to further evaluate such a possibility.

Surgical management of cataract and posterior chamber intraocular lens implantation in Fuchs‘ heterochromic iridocyclitis

Background: To determine the inflammatory course and level of visual rehabilitation after cataract extraction and posterior chamber lens implantation in patients withFuchs‘ heterochromic iridocyclitis complicated by cataract. Methods: In a clinical trial, 32 eyes (visual acuity of 20/160 or worse) of 30 patients underwent extracapsular cataract extraction (19 eyes) or lensectomy(13 eyes) accompanied by posterior chamber intraocular lens implantation. Indirect ophthalmoscopy was performed intraoperatively prior to intraocular lens implantation and the extent of vitreous haze was assessed. If vitreous haziness was 3+ or more, core vitrectomy (two eyes) or three-port pars plana deep vitrectomy (four eyes) was performed. Results: After an average follow up of14.4 months (6 to 24 months), there was no statistically significant increase in cell and flare in the anterior chamber and vitreous or in keratic precipitates compared with the preoperative status of the eyes. However, 12%of the eyes developed synechiae (anterior and/or posterior) in comparison to preoperative condition (p< 0.05). Eighty-seven percent of the eyes gained visual acuity of 20/40 or better (P < 0.005). Using the logistic regression model, a higher level of preoperative inflammation was associated with reduced likelihood of gaining visual acuity of 20/25 or more (OR = 0.25, 95%CI 0.049, 1.255). The complications of surgery were synechiae, 12%; opaque posterior capsule, 12%; vitreous loss, 3%; chronic glaucoma, 3%; and retinal detachment,3%. Conclusions: Implantation of a posterior chamber intraocular lens can be well tolerated in patients with Fuchs‘ heterochromic iridocyclitis. Vitrectomy, whenever indicated in addition to cataract extraction, improves visual rehabilitation.

Outcomes of surgical (pars plicata and limbal lensectomy, vitrectomy) and non-surgical management of persistent fetal vasculature (PFV): an analysis of 54 eyes

Purpose To identify clinical characteristics associated with outcomes of treatment for persistent fetal vasculature (PFV) based on a modified classification. Methods In this noncomparative case series, 54 eyes of 47 consecutive patients with PFV managed from 1981 until 1998 at a tertiary eye hospital were reviewed. Pars plicata lensectomy and vitrectomy was performed in 34 eyes and translimbal lensectomy and vitrectomy in five. Fifteen eyes were managed non-surgically. The following outcomes were measured: 1) “Visual improvement”, defined as at least one Zipfs category improvement of best corrected final vision as compared with initial visual acuity, provided that final vision of CSUM (central, steady, unmaintained) equal to 20/100–20/30 was obtained. 2) “Cosmetic acceptability”, defined as the absence of the following: small orbit, externally small appearing eye with microcornea and hypotony, eye deviation more than 35 prism diopters without strabismus surgery and corneal opacity. Results Six eyes were inoperable. In 10 out of 33 operated eyes (30.3%) improvement of vision was obtained including four eyes with an anterior form and six eyes with a combined form of PFV. In univariate analysis, factors such as anterior form of PFV, surgery in the combined form and having a normal retina, had a weak association with a higher chance of visual improvement. In multivariate regression analysis the likelihood of unacceptable cosmetic appearance was higher in females than males (OR = 10.5), and in bilateral cases (OR = 7.9). Offspring of consanguineous marriages had more severe forms of PFV. Conclusions Although visual potential is limited in PFV, some eyes with an anterior or combined form and normal retina achieved improvement of visual acuity after surgery. However, statistically no single factor except sex and bilateral PFV (for cosmetic unacceptability) reliably predicted the results of treatment. Therapy should be individualized based on clinical findings. Genetic studies in offspring of consanguineous marriages with PFV may prove informative.

Multiple Retinal Holes and Peripheral Nonperfusion in Muscle-Eye-Brain Disease

M uscle-eye-brain (MEB) disease is a rare congenital autosomal recessive disorder with around 30 reported cases in the literature. It is 1 of 3 types of congenital muscular dystrophy with severe defects in organogenesis and neuronal migration, and, along with Walker-Warburg syndrome, is associated with ocular abnormalities. Usually, the retina in MEB disease and Walker-Warburg syndrome is described as dysplasic. A detailed histopathologic description has been given, but a clinical description of retinal findings, especially in early disease, is lacking. Herein, we describe detailed retinal findings in a neonate with the diagnosis of MEB disease confirmed by genetic testing.

Ocular findings in a patient with oculofaciocardiodental (OFCD) syndrome and a novel BCOR pathogenic variant

PurposeTo report a case of OFCD associated with a de novo BCOR pathogenic variant and highlight the ocular findings and possible mechanisms.MethodsA retrospective chart review of the patient’s ocular and systemic findings was performed. The patient underwent diagnostic whole exome sequencing (WES).ResultsThe patient had a comprehensive eye exam in infancy demonstrating bilateral congenital cataracts consisting of posterior lenticonus with a posterior cortical opacity. She also had blepharoptosis with a hooded appearance and retinal pigment hypertrophy of the inferior retina bilaterally. Systemic findings include atrial septal defect, patent ductus arteriosus, congenital clubfoot, syndactyly, tethered cord, and laryngeal cleft. WES identified a de novo heterozygous R1136X pathogenic variant in the BCOR gene.ConclusionThe typical ocular manifestation of OFCD syndrome is congenital cataracts, which can have a significant impact on visual development and so should be considered in patients with multiple medical issues that may fit the diagnosis. A comprehensive eye exam in these patients is thus warranted.

Unsuspected Ganglioneuroma of the Choroid Diagnosed after Enucleation

We report a case of an unsuspected ganglioneuroma of the choroid in a patient with neurofibromatosis type 1. A 5-year-old girl presented from an outside institution with right proptosis and glaucoma since birth. Magnetic resonance imaging was obtained and showed a cavernous sinus mass extending into the right orbit and multiple orbital lesions. Additionally, increased signal in the posterior globe of the right eye was noted, but its etiology was unclear at the time. She was lost to follow-up for 3 years and later returned with a blind painful eye. Enucleation was performed, and histopathology was significant for diffuse choroidal ganglioneuroma and advanced glaucoma. We report the atypical history, examination findings, and histopathology to support the diagnosis.