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Show PHOTO ESSAY Magnetic Resonance Imaging of Choroidal Inflammation in Vogt- Koyanagi- Harada Disease Michael S. Vaphiades, DO and Russell W. Read, MD iO r B * m * - FIG. 1. T1- weighted fat- saturated axial MRI in a patient with Vogt- Koyanagi- Harada syndrome. Axial ( A) and coronal ( B) precontrast and axial ( C) and coronal ( D) postcontrast scans show bilateral diffuse choroidal thickening and enhancement. A serous retinal detachment is present OS. Abstract: Acute binocular visual loss, photophobia, headache, and pulsatile tinnitus developed in a 51- year- old woman. Ophthalmologic examination showed bilateral optic disc edema with peripapillary nerve fiber layer hemorrhages. Lumbar puncture disclosed a monocytic pleocyto-sis. A diagnosis of Vogt- Koyanagi- Harada disease was made. Magnetic resonance imaging showed striking enhancement and thickening of the posterior ocular wall. A macular star figure appeared several days after prednisone treatment was begun. Laboratory evaluation was entirely negative. Within weeks, the clinical manifestations had resolved except for retinal striae. This is the third report Departments of Ophthalmology ( MSV, RWR), Neurology ( MSV), Neurosurgery ( MSV), and Pathology ( RWR), University of Alabama at Birmingham, Birmingham, Alabama. This work was supported in part by unrestricted grants from Research to Prevent Blindness, Inc., New York, New York, and the EyeSight Foundation of Alabama, Inc., Birmingham, Alabama. Address correspondence to Michael S. Vaphiades, DO, Department of Ophthalmology, University of Alabama, Birmingham, 700 South 18th Street, Suite 601, Birmingham, AL 35233; E- mail: vaph@ uab. edu of the magnetic resonance imaging visualization of choroidal inflammation in Vogt- Koyanagi- Harada disease and shows the imaging abnormalities in finer detail than earlier reports. ( JNeuro- Ophthalmol 2004 ; 24: 295- 296) A51 - year- old woman presented with a 6- week history of bilateral visual loss accompanied by photophobia, severe headaches, and pulsatile tinnitus. Blood pressure was normal. Best- corrected visual acuities were 20/ 400 OD and 20/ 70 OS. Automated perimetry showed nonspecifically constricted visual fields OU. Pupils were equal in size and reacted briskly to direct light without a relative afferent pupillary defect. Ocular motility, trigeminal and facial nerve function, palpebral fissures, and exophthalmometry were normal. Slit- lamp biomicroscopy revealed normal anterior segments bilaterally. Tonometry was normal OU. Dilated fundus examination showed swollen optic nerves OU and low- lying serous J Neuro- Ophthalmol, Vol. 24, No. 4, 2004 295 JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 Vaphiades and Read FIG. 2. Fundus photography shows optic disc edema OU with splinter disc hemorrhages, a macular star figure OD ( A), and macular exudates OS ( B). retinal detachments OU. There were peripapillary nerve fiber layer hemorrhages OD. Cranial and orbital magnetic resonance imaging ( MRI) showed diffuse bilateral enhancement of the posterior ocular wall and serous retinal detachment OS ( Fig. 1). Ultrasonography revealed diffuse chorioretinal thickening without evidence of scleritis. A lumbar puncture showed a normal opening pressure with a leukocyte count of 57/ mm3 ( 100% monocytes), no red cells, normal protein, glucose, cryptococcal antigen, and luetic titers. A diagnosis of Vogt- Koyanagi- Harada ( VKH) disease was made and the patient was treated with prednisone 60 mg/ d. The patient experienced relief of her headache. Seven days after institution of oral prednisone, the exudative detachments were decreased in size and macular exudates appeared as a partial macular star figure. ( Fig. 2). Fluorescein angiography at this time showed continued disc hyperemia and peripapillary leakage. Laboratory evaluation for causes of Leber stellate neuroretinitis was negative, including serologies for syphilis, Bartonella henselae and Bartonella quintana, Borrelia burgdorferi, Ehrlichia species, and Rocky Mountain spotted fever. Over the course of 10 weeks, the patient's visual acuity improved to 20/ 20 OU, with complete resolution of the serous retinal detachments and macular exudates. Macular striae persist, but the patient has no metamorphopsia. VKH disease consists of bilateral diffuse choroiditis with exudative retinal detachments and papillitis ( 1). A macular star figure has apparently not been previously reported in this condition. Auditory and central nervous system involvement in VKH disease usually precedes ocular findings and is typified by hearing loss, tinnitus, and men-ingismus with associated cerebrospinal fluid pleocytosis. Involvement of the integumentary system occurs later in the disease course and may include alopecia, poliosis, or vitiligo ( 1,2). The posterior ocular wall inflammation consists of choroiditis without scleritis ( 2). This manifestation was first imaged in 1990 ( 3) with a sagittal MRI using a surface coil. The scan showed choroidal thickening, a finding that was also appreciated on CT ( 3). In 1994, Ibanez et al ( 4) demonstrated intense enhancement and thickening of the choroid on axial Tl- weighted orbital MRI. Our case illustrates these findings in even higher resolution. REFERENCES Read RW. Vogt- Koyanagi- Harada disease. Ophthalmol Clin North A « 2002; 15: 333^ U. Read RW, Holland GN, Rao NA, et al. Revised diagnostic criteria for Vogt- Koyanagi- Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 2001; 131: 647- 52. Johnston CA, Teitelbaum CS. Magnetic resonance imaging in Vogt- Koyanagi- Harada syndrome. Arch Ophthalmol 1990; 108: 783- 84. Ibanez HE, Grand MG, Meredith TA, et al. Magnetic resonance imaging findings in Vogt- Koyanagi- Harada syndrome. Retina 1994; 14: 164- 8. 296 © 2004 Lippincott Williams & Wilkins |
