Poster 91: A Rare Case of Intermediate Uveitis Secondary to Multiple Sclerosis
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years of age and has been followed for 6 years, during which time comprehensive examinations, visual fields, fun- dus photographs, and electrodiagnostic testing have been done to confirm and monitor a diagnosis of SCRA. Conclusions: Recent developments in both genomics and electrophysiologic/psychophysical testing have made the diagnosis of SCRA more certain. Genetically, its inheri- tance has been linked to a mutation of the TEAD1 gene on chromosome 11p15 that is transmitted in an autosomal dominant fashion. To date, ancestry has been most exten- sively found to originate directly from certain indigenous populations native to Northern Europe and North America. Repeated electrophysiologic testing has revealed a tendency for normal or near-normal ERGs with abnormal EOGs. This indicates the predilection for focal damage to the retinal pigment epithelium (RPE) to occur before any alterations in sensory retinal function. Visual-field testing tends to be variable according to the area of RPE affected, color vision is characteristically normal, and visual acuity is unaffected until later in life. Unfortunately, the disorder tends to spread slowly, but inexorably, from the peripapillary area to the macula, with subsequent reduction in central visual acuity and inevitable reduction in color sensitivity. This disorder represents an important example of how applied research technology can support clinical diagnosis and care in op- tometry. Poster 90 Sickle-cell Retinopathy Michelle Steenbakkers, O.D., and Justin Barnett (O.D. ’06), University of Houston, 2525 Lucas Street, RET Building 3, Dallas, Texas 75219 Background: Sickle-cell hemoglobinopathies are caused by a genetic mutation of the beta-globin subunit of erythro- cytes. Red blood cells become crescent-shaped and lacking the ability to travel through peripheral, small-caliber vascu- lature. The decreased pliability leads to increased blood viscosity and risk of vascular occlusion. In the eye, neovas- cularization, retinal detachment, and vision loss can occur. The following case report discusses the evaluation and management of a patient with advanced sickle-cell retinop- athy. Case Report: A 46-year-old woman came to us with reports of decreased near vision. Her medical history included depression, an unknown psychiatric disorder, and benign breast fibrocystoma. The patient was unsure of her sickle- cell status, had no history of infection or trauma, and no family history of retinal disease. BCVAs were 20/20 in each eye. Biomicroscopy and intraocular pressures were unre- markable in each eye. Internal evaluation revealed well- perfused optic nerves. In the right eye, a peripheral anasto- mosis located inferior-temporal was partially obstructed by an old, dense vitreous hemorrhage. In the left eye, a black sunburst lesion was noted superior to the optic nerve. In the superior-temporal peripheral region, an operculated retinal hole was visible, with overlying fibrosis in the vitreous. Areas of peripheral gliotic seafan neovascularization were present in each eye. The patient was diagnosed with a retinal hole O.S. and suspected sickle-cell retinopathy in both eyes. She was referred for laser treatment of the retinal hole and testing for definitive diagnosis of sickle-cell anemia. Hemo- globin electrophoresis revealed the patient was positive for HbSC disease. Conclusion: Early diagnosis of sickle-cell hemoglobinopa- thy is paramount in prevention of end-stage multiple organ disease. This poster will outline the variant mutations of sickle-cell disease, as well as the diagnosis and management of sickle-cell retinopathy. Poster 91 A Rare Case of Intermediate Uveitis Secondary to Multiple Sclerosis Laura Young, O.D., M.S., Jeffrey Joy, O.D., Chandra Altemare, M.D., and Robert Morris, O.D., W.G. “Bill” Hefner VAMC, 1601 Brenner Avenue, Salisbury, North Carolina 28144 Background: Intermediate uveitis is an intraocular inflamma- tion that involves the anterior vitreous, peripheral retina, and pars plana. It is usually found in persons 15 to 40 years old, with no gender or racial preference, and bilateral in presenta- tion. It is often called pars planitis; however, pars planitis is actually an isolated ocular subset of intermediate uveitis. The etiology of intermediate uveitis is unknown, but there have been some systemic associations, including multiple sclerosis (MS), sarcoidosis, and inflammatory bowel disease. Purpose: In this poster, we will be focusing on the association with multiple sclerosis. Ocular involvement is common in MS and often may be the presenting manifestation of the disease. Optic neuritis is the most common ocular condition associated with MS. In contrast, inflammation of the uveal tract is a much less common and less well-known manifestation of MS. Interme- diate uveitis is reported in 1% of MS patients, whereas, up to15% of intermediate uveitis cases are associated with MS. Multiple sclerosis is a chronic inflammatory demyelinating disease of the central nervous system (CNS) with an autoim- mune origin. It is believed there are antigens co-expressed in the CNS and in the retina and the uvea that might be of pathogenetic relevance to MS and intermediate uveitis. Fur- thermore, a common immunogenetic predisposition to MS and intermediate uvetitis is the HLA–DR15 allele. Case Report: A case of a 33-year-old man with intermediate uveitis secondary to a known diagnosis of multiple sclerosis will be presented. Color photographs of the anterior seg- ment and posterior segment will be included. Conclusion: The poster will also include a discussion of the signs and symptoms, differential diagnoses, laboratory tests, and treatment recommendations for intermediate uveitis. Additionally, a brief literature review of the associations between MS and intermediate uveitis and new treatment options will be incorporated into the poster. 301 Poster Presentations
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Transcript of Poster 91: A Rare Case of Intermediate Uveitis Secondary to Multiple Sclerosis
years of age and has been followed for 6 years, duringwhich time comprehensive examinations, visual fields, fun-dus photographs, and electrodiagnostic testing have beendone to confirm and monitor a diagnosis of SCRA.Conclusions: Recent developments in both genomics andelectrophysiologic/psychophysical testing have made thediagnosis of SCRA more certain. Genetically, its inheri-tance has been linked to a mutation of the TEAD1 gene onchromosome 11p15 that is transmitted in an autosomaldominant fashion. To date, ancestry has been most exten-sively found to originate directly from certain indigenouspopulations native to Northern Europe and North America.Repeated electrophysiologic testing has revealed a tendencyfor normal or near-normal ERGs with abnormal EOGs. Thisindicates the predilection for focal damage to the retinalpigment epithelium (RPE) to occur before any alterations insensory retinal function. Visual-field testing tends to bevariable according to the area of RPE affected, color visionis characteristically normal, and visual acuity is unaffecteduntil later in life. Unfortunately, the disorder tends to spreadslowly, but inexorably, from the peripapillary area to themacula, with subsequent reduction in central visual acuityand inevitable reduction in color sensitivity. This disorderrepresents an important example of how applied researchtechnology can support clinical diagnosis and care in op-tometry.
Poster 90
Sickle-cell RetinopathyMichelle Steenbakkers, O.D., andJustin Barnett (O.D. ’06), University of Houston, 2525Lucas Street, RET Building 3, Dallas, Texas 75219
Background: Sickle-cell hemoglobinopathies are caused bya genetic mutation of the beta-globin subunit of erythro-cytes. Red blood cells become crescent-shaped and lackingthe ability to travel through peripheral, small-caliber vascu-lature. The decreased pliability leads to increased bloodviscosity and risk of vascular occlusion. In the eye, neovas-cularization, retinal detachment, and vision loss can occur.The following case report discusses the evaluation andmanagement of a patient with advanced sickle-cell retinop-athy.Case Report: A 46-year-old woman came to us with reportsof decreased near vision. Her medical history includeddepression, an unknown psychiatric disorder, and benignbreast fibrocystoma. The patient was unsure of her sickle-cell status, had no history of infection or trauma, and nofamily history of retinal disease. BCVAs were 20/20 in eacheye. Biomicroscopy and intraocular pressures were unre-markable in each eye. Internal evaluation revealed well-perfused optic nerves. In the right eye, a peripheral anasto-mosis located inferior-temporal was partially obstructed byan old, dense vitreous hemorrhage. In the left eye, a blacksunburst lesion was noted superior to the optic nerve. In thesuperior-temporal peripheral region, an operculated retinal
hole was visible, with overlying fibrosis in the vitreous.Areas of peripheral gliotic seafan neovascularization werepresent in each eye. The patient was diagnosed with a retinalhole O.S. and suspected sickle-cell retinopathy in both eyes.She was referred for laser treatment of the retinal hole andtesting for definitive diagnosis of sickle-cell anemia. Hemo-globin electrophoresis revealed the patient was positive forHbSC disease.Conclusion: Early diagnosis of sickle-cell hemoglobinopa-thy is paramount in prevention of end-stage multiple organdisease. This poster will outline the variant mutations ofsickle-cell disease, as well as the diagnosis and managementof sickle-cell retinopathy.
Poster 91
A Rare Case of Intermediate Uveitis Secondary toMultiple SclerosisLaura Young, O.D., M.S., Jeffrey Joy, O.D.,Chandra Altemare, M.D., and Robert Morris, O.D., W.G.“Bill” Hefner VAMC, 1601 Brenner Avenue, Salisbury,North Carolina 28144
Background: Intermediate uveitis is an intraocular inflamma-tion that involves the anterior vitreous, peripheral retina, andpars plana. It is usually found in persons 15 to 40 years old,with no gender or racial preference, and bilateral in presenta-tion. It is often called pars planitis; however, pars planitis isactually an isolated ocular subset of intermediate uveitis. Theetiology of intermediate uveitis is unknown, but there havebeen some systemic associations, including multiple sclerosis(MS), sarcoidosis, and inflammatory bowel disease. Purpose:In this poster, we will be focusing on the association withmultiple sclerosis. Ocular involvement is common in MS andoften may be the presenting manifestation of the disease. Opticneuritis is the most common ocular condition associated withMS. In contrast, inflammation of the uveal tract is a much lesscommon and less well-known manifestation of MS. Interme-diate uveitis is reported in 1% of MS patients, whereas, upto15% of intermediate uveitis cases are associated with MS.Multiple sclerosis is a chronic inflammatory demyelinatingdisease of the central nervous system (CNS) with an autoim-mune origin. It is believed there are antigens co-expressed inthe CNS and in the retina and the uvea that might be ofpathogenetic relevance to MS and intermediate uveitis. Fur-thermore, a common immunogenetic predisposition to MS andintermediate uvetitis is the HLA–DR15 allele.Case Report: A case of a 33-year-old man with intermediateuveitis secondary to a known diagnosis of multiple sclerosiswill be presented. Color photographs of the anterior seg-ment and posterior segment will be included.Conclusion: The poster will also include a discussion of thesigns and symptoms, differential diagnoses, laboratory tests,and treatment recommendations for intermediate uveitis.Additionally, a brief literature review of the associationsbetween MS and intermediate uveitis and new treatmentoptions will be incorporated into the poster.
301Poster Presentations
