- Email: [email protected]
Best's vitelliform macular dystrophy with pseudohypopyon: an optical coherence tomography study
and diabetes (17%). Hearing problems were present in 6%. Fifty-eight percent of patients had two or more comorbidities. Adjustment for medical conditions did not have an effect on treatment arm comparisons of 24-month NEIVFQ scores (Table 1 and Table 2). Similarly, there was no effect when treatment arm comparisons were adjusted for presence of multiple conditions (0 or 1 versus 2 or more conditions; data not shown). However, 3 point or greater treatment differences in the NEI-VFQ scores unadjusted and adjusted for SF-36 PCS and MCS scores were observed for ocular pain (4.3 points), color vision (3.8 points), mental health (3.3 points), dependency (3.1 points), and social functioning (2.7 points) subscales (Table 1 and Table 2). As much as a 4.3 point change in treatment effect was observed when NEI-VFQ scores were adjusted for SF-36 summary scores compared with unadjusted analysis. Differences of this magnitude may yield a clinically meaningful finding from the adjusted analysis that may have not been detected in the unadjusted analysis or vice versa. These results suggest that adjustment of NEI-VFQ scores for general health should be considered, even in randomized trials. The SF-36 summary scales may provide a better measure of general health status than individual medical conditions. REFERENCES
1. Mangione CM, Berry S, Spritzer K, et al. Identifying the content area for the 51-item National Eye Institute Visual Function Questionnaire: results from focus groups with visually impaired persons. Arch Ophthalmol 1998;116:227–233. 2. Mangione CM, Lee PP, Gutierrez PR, et al. Development of the 25-item National Eye Institute Visual Function Questionnaire. Arch Ophthalmol 2001;119:1050 –1058. 3. Miskala PH, Bressler NM, Meinert CL. Relative contributions of reduced vision and general health to NEI-VFQ scores in patients with neovascular age-related macular degeneration. Arch Ophthalmol. Forthcoming. 4. Submacular Surgery Trials Research Group. Submacular Surgery Trials (SST) manual of procedures. Springfield, VA: US Department of Commerce, National Technical Information Service accession no. PB98 –166648 1998. 5. Ware JE, Kosinski M, Keller SD. SF-36 physical and mental health summary scales: a user’s manual. Boston: Health Institute, New England Medical Center, 1994.
Best’s Vitelliform Macular Dystrophy With Pseudohypopyon: An Optical Coherence Tomography Study Gamze Men, MD, Figen Batıog˘ lu, MD, ¨ zkan, MD, Huban Atilla, MD, Seyhan Sonar O ¨ ¨ zlem Aslan, MD Yasemin Ozdamar, MD, and O VOL. 137, NO. 5
To report optical coherence tomography (OCT) changes in Best’s vitelliform macular dystrophy (BVMD) with pseudohypopyon. DESIGN: Observational case report. METHODS: Both eyes of a patient with BVMD showing pseudohypopyon were examined with OCT. RESULTS: OCT demonstrated the presence of serous retinal elevation with normal appearing retinal pigment epithelium (RPE) superiorly, and broadening of the outer-retina-choroid-complex signal under retinal elevation, inferiorly. CONCLUSIONS: OCT findings in our study suggest the accumulation of material under neurosensory retina in BVMD with pseudohypopyon. (Am J Ophthalmol 2004;137:963–965. © 2004 by Elsevier Inc. All rights reserved.) PURPOSE:
B
EST’S VITELLIFORM MACULAR DYSTROPHY (BVMD) IS
an autosomal dominant disorder of the retina that presents with variable clinical appearance. Pseudohypopyon is the typical lesion of stage III BVMD. Few studies1–3 on BVMD have employed optical coherence tomography (OCT) as the main observation procedure. We analyzed cross-sectional OCT images of a macular lesion in a case with stage III BVMD. A 51-year-old woman presented with decreased vision in both eyes. Her best-corrected visual acuity was RE, 20/200 and LE, 20/100. Symmetrical retinal elevations with a size of three disk-diameters were observed in the macula of both eyes. The subretinal fluid seemed relatively clear in the superior part of the lesions, whereas in the inferior part, a yellow-white subretinal material accumulation caused a level margin and created the appearance of a pseudohypopyon (Figure 1). Fluorescein angiography revealed hyperfluorescent defects superiorly, and blockage of the fluorescence corresponding to the location of pseudohypopyon inferiorly. Electro-oculography demonstrated an abnormal pattern and very low dark trough values in both eyes (30.5 V OD, 5.86 V OS), despite normal Arden ratios. A serous retinal detachment with normal appearing RPE was revealed in the superior part of the lesions on OCT (OCT Scanner, Model 1999, Humphrey-Zeiss Medical Systems, California). In the inferior part, however, broadening of the outer-retina-choroid-complex (ORCC) signal under retinal elevation was observed (Figure 2).
Accepted for publication Nov 3, 2003. From the Department of Ophthalmology, Social Insurance Eye Hos¨ ., Y.O ¨ ., O ¨ .,A.) and from the Departpital, Ankara, Turkey (G.M., S.S.O ment of Ophthalmology, Ankara University, Ankara, Turkey (F.B., H.A.). Inquiries to Gamze Men, MD, Mahatma Gandhi Caddesi, Mesa Ufuk 1 Sitesi 51/17, 06700 GOP, Ankara, Turkey; fax: (90) 312 468 00 94; e-mail: [email protected]
BRIEF REPORTS
963
FIGURE 2. Vertical central macular OCT scans of right (top), and left (bottom) eyes. Note normal appearing RPE epithelium at the bottom (arrowheads), serous retinal elevation, and broadened signal from ORCC (star) corresponding to the subretinal material accumulation.
FIGURE 1. Ophthalmoscopic appearance of the right (top), and left (bottom) eyes showing pseudohypopyon consistent with BVMD. Note the central irregularity of pigmentation and the accumulation of the material under the retina.
in relation to the RPE were not specified. RPE elevation above a moderately reflective region on OCT was reported by Andrade2 in an uncomplicated fellow eye with a disruptive egg-yolk-like lesion. These OCT findings indicate sub-RPE material accumulation. Pianta1 observed a spectrum of OCT changes in BVMD, including serous retinal detachment and accumulation of material in subretinal and in sub-RPE space. The presence of similar alterations in various retinal disorders such as central serous chorioretinopathy and age-related macular degeneration was stated by the authors, and the OCT changes mentioned above were not found unique for BVMD. We are unaware of previous reports of serous retinal detachment and accompanying accumulation of material under neurosensory retina in BVMD with pseudohypopyon and could not find any reference to it in a computerized search using Medline. Because we did not find any other study reporting similar OCT changes in other retinal diseases, our findings may be considered unique for this stage of BVMD.
The characteristic ophthalmoscopic and fluorescein angiographic findings observed in our patient were consistent with stage III BVMD. The serous retinal detachment that we detected on OCT has also been reported as a typical feature for BVMD.3 OCT changes in our study suggested accumulation of material between neurosensory retina and RPE. The site of the material accumulation in BVMD is a subject of debate. Based on histopathology, sub-RPE4 and subretinal5 material accumulation was described. In addition to a serous retinal detachment, Pierro3 reported broadening of the ORCC signal on OCT consistent with accumulation of lipofuscina substance in BVMD. However, the stages of the lesions and the location of the material accumulation 964
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
MAY 2004
REFERENCES
1. Pianta MJ, Aleman TS, Cideciyan AV, et al. In vivo micropathology of Best macular dystrophy with optical coherence tomography. Exp Eye Res 2003;76:203–211. 2. Andrade RE, Farah ME, Cardillo JA, Hofling-Lima AL, Uno F, Costa RA. Optical coherence tomography in choroidal neovascular membrane associated with Best’s vitelliform dystrophy. Acta Ophthalmol Scand 2002;80:216 –218. 3. Pierro L, Tremolada G, Introini U, Calori G, Brancato R. Optical coherence tomography findings in adult-onset foveomacular vitelliform dystrophy. Am J Ophthalmol 2002;134: 675–680. 4. O’Gorman S, Flaherty WA, Fishman GA, Berson EL. Histopathologic findings in Best’s vitelliform macular dystrophy. Arch Ophthalmol 1988;106:1261–1268. 5. Weingeist TA, Kobrin JL, Watzke RC. Histopathology of Best’s macular dystrophy. Arch Ophthalmol 1982;100:1108 – 1114.
Topical Prostaglandin F2␣ Analog Induced Poliosis Celia S. Chen, MPHC, Jane Wells, BMBS, and Jamie E. Craig, DPhil, FRANZCO To report poliosis as a side effect associated with topical prostaglandin F2␣ (PGF2␣) analogs. METHOD: Case series. RESULTS: Seven patients treated with different topical PGF2␣ analogs for primary open angle glaucoma developed bilateral poliosis, either alone or in combination with other adverse effects of PGF2␣ analog therapy. CONCLUSION: Poliosis is a possible adverse effect of topical PGF2␣ analog therapy which is previously unreported. Topical PGF2␣ analog therapy should be included in the differential diagnosis of patients with poliosis. (Am J Ophthalmol 2004;137:965–966. © 2004 by Elsevier Inc. All rights reserved.) PURPOSE:
T
OPICAL PROSTAGLANDIN F2〈 (PGF2〈) ANALOGS ARE
used in the treatment of primary open-angle glaucoma. Adverse effects associated with PGF2␣ analog therapy include eyelash hypertrichosis and increased pigmentation.1 Poliosis has not been reported previously as an adverse effect of PGF2␣ analog therapy. We report seven cases of poliosis secondary to topical PGF2␣ analog therapy Accepted for publication Nov 3, 2003. From the Department of Ophthalmology, Flinders Medical Centre, Bedford Drive, Bedford Park, Adelaide, Australia. Inquiries to Jamie E. Craig, Department of Ophthalmology, Flinders Medical Centre, Bedford Drive, Bedford Park, Adelaide, Australia, SA 5042; fax: 61 8 8404 –2040; e-mail: [email protected]
VOL. 137, NO. 5
and discuss the clinical implications and proposed pathogenesis of PGF2␣ analog induced poliosis. Seven patients, ages 59 to 78, were observed to develop poliosis after PGF2␣ analog treatment for primary open angle glaucoma or normal tension glaucoma over a sixmonth period (Table 1). Poliosis was noted as early as six weeks after starting treatment (Figure 1). Other known PGF2␣ analog side effects such as conjunctival hyperemia and hypertrichosis were noted in six of the seven patients. Poliosis was noted to increase at subsequent reviews in two patients (Patients 1 and 4). One patient ceased PGF2␣ analog treatment because of an increase in poliosis (Patient 4). Five patients elected to continue with PGF2␣ analog treatment despite the side effect. In one patient, PGF2␣ analog treatment was discontinued after selective laser trabeculoplasty. Poliosis and hypertrichosis persisted, at the time of last review, two months after cessation of treatment. Poliosis occurs in several hereditary syndromes or is acquired after inflammation, irradiation, or infection. Differential diagnosis of poliosis include Vogt-KoyanagiHarada disease,2 sympathetic ophthalmia,2 Alezzandrini’s disease, tuberous sclerosis, Waardenberg syndrome, and cyclosporin A or chloroquine phosphate drug use.3 We report the observation of poliosis with topical PGF2␣ analog use. A previous report noted ocular inflammation with poliosis associated with latanoprost use.4 We have observed this side-effect with all three currently available PGF2␣ analog agents (latanoprost, travaprost, and bimatoprost) despite being in different subclasses. Poliosis may occur singly or in combination with other reported adverse effects of PGF2␣ analog use and affected lashes are typically interspersed with normally pigmented lashes. The pathogenesis of poliosis secondary to PGF2␣ analog treatment is not yet known. Melanogenesis only occurs during the anagen phase of the hair follicle cell cycle. Prostaglandin can induce and prolong this phase, resulting in either new follicle growth or hypertrophy of existing hair follicles.5 During anagen, genetically predetermined differences in follicular response results in some dermal papillae secreting mitogens, whereas others synthesize inhibitory factors.5 This may in part account for the varying response of hair follicles to PGF2␣. We observed on several occasions that the white lashes were new. This implies that the side effect may result from failure of pigmentation in newly stimulated eyelash growth or a stimulated growth of previously inconspicuous white lashes. Our observations in seven patients over a short time period (six months) imply that this side-effect is relatively common. Further prospective study with careful pretreatment and post-treatment photographs would help demonstrate the cause and increase understanding of this side effect.
BRIEF REPORTS
965
1. Mangione CM, Berry S, Spritzer K, et al. Identifying the content area for the 51-item National Eye Institute Visual Function Questionnaire: results from focus groups with visually impaired persons. Arch Ophthalmol 1998;116:227–233. 2. Mangione CM, Lee PP, Gutierrez PR, et al. Development of the 25-item National Eye Institute Visual Function Questionnaire. Arch Ophthalmol 2001;119:1050 –1058. 3. Miskala PH, Bressler NM, Meinert CL. Relative contributions of reduced vision and general health to NEI-VFQ scores in patients with neovascular age-related macular degeneration. Arch Ophthalmol. Forthcoming. 4. Submacular Surgery Trials Research Group. Submacular Surgery Trials (SST) manual of procedures. Springfield, VA: US Department of Commerce, National Technical Information Service accession no. PB98 –166648 1998. 5. Ware JE, Kosinski M, Keller SD. SF-36 physical and mental health summary scales: a user’s manual. Boston: Health Institute, New England Medical Center, 1994.
Best’s Vitelliform Macular Dystrophy With Pseudohypopyon: An Optical Coherence Tomography Study Gamze Men, MD, Figen Batıog˘ lu, MD, ¨ zkan, MD, Huban Atilla, MD, Seyhan Sonar O ¨ ¨ zlem Aslan, MD Yasemin Ozdamar, MD, and O VOL. 137, NO. 5
To report optical coherence tomography (OCT) changes in Best’s vitelliform macular dystrophy (BVMD) with pseudohypopyon. DESIGN: Observational case report. METHODS: Both eyes of a patient with BVMD showing pseudohypopyon were examined with OCT. RESULTS: OCT demonstrated the presence of serous retinal elevation with normal appearing retinal pigment epithelium (RPE) superiorly, and broadening of the outer-retina-choroid-complex signal under retinal elevation, inferiorly. CONCLUSIONS: OCT findings in our study suggest the accumulation of material under neurosensory retina in BVMD with pseudohypopyon. (Am J Ophthalmol 2004;137:963–965. © 2004 by Elsevier Inc. All rights reserved.) PURPOSE:
B
EST’S VITELLIFORM MACULAR DYSTROPHY (BVMD) IS
an autosomal dominant disorder of the retina that presents with variable clinical appearance. Pseudohypopyon is the typical lesion of stage III BVMD. Few studies1–3 on BVMD have employed optical coherence tomography (OCT) as the main observation procedure. We analyzed cross-sectional OCT images of a macular lesion in a case with stage III BVMD. A 51-year-old woman presented with decreased vision in both eyes. Her best-corrected visual acuity was RE, 20/200 and LE, 20/100. Symmetrical retinal elevations with a size of three disk-diameters were observed in the macula of both eyes. The subretinal fluid seemed relatively clear in the superior part of the lesions, whereas in the inferior part, a yellow-white subretinal material accumulation caused a level margin and created the appearance of a pseudohypopyon (Figure 1). Fluorescein angiography revealed hyperfluorescent defects superiorly, and blockage of the fluorescence corresponding to the location of pseudohypopyon inferiorly. Electro-oculography demonstrated an abnormal pattern and very low dark trough values in both eyes (30.5 V OD, 5.86 V OS), despite normal Arden ratios. A serous retinal detachment with normal appearing RPE was revealed in the superior part of the lesions on OCT (OCT Scanner, Model 1999, Humphrey-Zeiss Medical Systems, California). In the inferior part, however, broadening of the outer-retina-choroid-complex (ORCC) signal under retinal elevation was observed (Figure 2).
Accepted for publication Nov 3, 2003. From the Department of Ophthalmology, Social Insurance Eye Hos¨ ., Y.O ¨ ., O ¨ .,A.) and from the Departpital, Ankara, Turkey (G.M., S.S.O ment of Ophthalmology, Ankara University, Ankara, Turkey (F.B., H.A.). Inquiries to Gamze Men, MD, Mahatma Gandhi Caddesi, Mesa Ufuk 1 Sitesi 51/17, 06700 GOP, Ankara, Turkey; fax: (90) 312 468 00 94; e-mail: [email protected]
BRIEF REPORTS
963
FIGURE 2. Vertical central macular OCT scans of right (top), and left (bottom) eyes. Note normal appearing RPE epithelium at the bottom (arrowheads), serous retinal elevation, and broadened signal from ORCC (star) corresponding to the subretinal material accumulation.
FIGURE 1. Ophthalmoscopic appearance of the right (top), and left (bottom) eyes showing pseudohypopyon consistent with BVMD. Note the central irregularity of pigmentation and the accumulation of the material under the retina.
in relation to the RPE were not specified. RPE elevation above a moderately reflective region on OCT was reported by Andrade2 in an uncomplicated fellow eye with a disruptive egg-yolk-like lesion. These OCT findings indicate sub-RPE material accumulation. Pianta1 observed a spectrum of OCT changes in BVMD, including serous retinal detachment and accumulation of material in subretinal and in sub-RPE space. The presence of similar alterations in various retinal disorders such as central serous chorioretinopathy and age-related macular degeneration was stated by the authors, and the OCT changes mentioned above were not found unique for BVMD. We are unaware of previous reports of serous retinal detachment and accompanying accumulation of material under neurosensory retina in BVMD with pseudohypopyon and could not find any reference to it in a computerized search using Medline. Because we did not find any other study reporting similar OCT changes in other retinal diseases, our findings may be considered unique for this stage of BVMD.
The characteristic ophthalmoscopic and fluorescein angiographic findings observed in our patient were consistent with stage III BVMD. The serous retinal detachment that we detected on OCT has also been reported as a typical feature for BVMD.3 OCT changes in our study suggested accumulation of material between neurosensory retina and RPE. The site of the material accumulation in BVMD is a subject of debate. Based on histopathology, sub-RPE4 and subretinal5 material accumulation was described. In addition to a serous retinal detachment, Pierro3 reported broadening of the ORCC signal on OCT consistent with accumulation of lipofuscina substance in BVMD. However, the stages of the lesions and the location of the material accumulation 964
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
MAY 2004
REFERENCES
1. Pianta MJ, Aleman TS, Cideciyan AV, et al. In vivo micropathology of Best macular dystrophy with optical coherence tomography. Exp Eye Res 2003;76:203–211. 2. Andrade RE, Farah ME, Cardillo JA, Hofling-Lima AL, Uno F, Costa RA. Optical coherence tomography in choroidal neovascular membrane associated with Best’s vitelliform dystrophy. Acta Ophthalmol Scand 2002;80:216 –218. 3. Pierro L, Tremolada G, Introini U, Calori G, Brancato R. Optical coherence tomography findings in adult-onset foveomacular vitelliform dystrophy. Am J Ophthalmol 2002;134: 675–680. 4. O’Gorman S, Flaherty WA, Fishman GA, Berson EL. Histopathologic findings in Best’s vitelliform macular dystrophy. Arch Ophthalmol 1988;106:1261–1268. 5. Weingeist TA, Kobrin JL, Watzke RC. Histopathology of Best’s macular dystrophy. Arch Ophthalmol 1982;100:1108 – 1114.
Topical Prostaglandin F2␣ Analog Induced Poliosis Celia S. Chen, MPHC, Jane Wells, BMBS, and Jamie E. Craig, DPhil, FRANZCO To report poliosis as a side effect associated with topical prostaglandin F2␣ (PGF2␣) analogs. METHOD: Case series. RESULTS: Seven patients treated with different topical PGF2␣ analogs for primary open angle glaucoma developed bilateral poliosis, either alone or in combination with other adverse effects of PGF2␣ analog therapy. CONCLUSION: Poliosis is a possible adverse effect of topical PGF2␣ analog therapy which is previously unreported. Topical PGF2␣ analog therapy should be included in the differential diagnosis of patients with poliosis. (Am J Ophthalmol 2004;137:965–966. © 2004 by Elsevier Inc. All rights reserved.) PURPOSE:
T
OPICAL PROSTAGLANDIN F2〈 (PGF2〈) ANALOGS ARE
used in the treatment of primary open-angle glaucoma. Adverse effects associated with PGF2␣ analog therapy include eyelash hypertrichosis and increased pigmentation.1 Poliosis has not been reported previously as an adverse effect of PGF2␣ analog therapy. We report seven cases of poliosis secondary to topical PGF2␣ analog therapy Accepted for publication Nov 3, 2003. From the Department of Ophthalmology, Flinders Medical Centre, Bedford Drive, Bedford Park, Adelaide, Australia. Inquiries to Jamie E. Craig, Department of Ophthalmology, Flinders Medical Centre, Bedford Drive, Bedford Park, Adelaide, Australia, SA 5042; fax: 61 8 8404 –2040; e-mail: [email protected]
VOL. 137, NO. 5
and discuss the clinical implications and proposed pathogenesis of PGF2␣ analog induced poliosis. Seven patients, ages 59 to 78, were observed to develop poliosis after PGF2␣ analog treatment for primary open angle glaucoma or normal tension glaucoma over a sixmonth period (Table 1). Poliosis was noted as early as six weeks after starting treatment (Figure 1). Other known PGF2␣ analog side effects such as conjunctival hyperemia and hypertrichosis were noted in six of the seven patients. Poliosis was noted to increase at subsequent reviews in two patients (Patients 1 and 4). One patient ceased PGF2␣ analog treatment because of an increase in poliosis (Patient 4). Five patients elected to continue with PGF2␣ analog treatment despite the side effect. In one patient, PGF2␣ analog treatment was discontinued after selective laser trabeculoplasty. Poliosis and hypertrichosis persisted, at the time of last review, two months after cessation of treatment. Poliosis occurs in several hereditary syndromes or is acquired after inflammation, irradiation, or infection. Differential diagnosis of poliosis include Vogt-KoyanagiHarada disease,2 sympathetic ophthalmia,2 Alezzandrini’s disease, tuberous sclerosis, Waardenberg syndrome, and cyclosporin A or chloroquine phosphate drug use.3 We report the observation of poliosis with topical PGF2␣ analog use. A previous report noted ocular inflammation with poliosis associated with latanoprost use.4 We have observed this side-effect with all three currently available PGF2␣ analog agents (latanoprost, travaprost, and bimatoprost) despite being in different subclasses. Poliosis may occur singly or in combination with other reported adverse effects of PGF2␣ analog use and affected lashes are typically interspersed with normally pigmented lashes. The pathogenesis of poliosis secondary to PGF2␣ analog treatment is not yet known. Melanogenesis only occurs during the anagen phase of the hair follicle cell cycle. Prostaglandin can induce and prolong this phase, resulting in either new follicle growth or hypertrophy of existing hair follicles.5 During anagen, genetically predetermined differences in follicular response results in some dermal papillae secreting mitogens, whereas others synthesize inhibitory factors.5 This may in part account for the varying response of hair follicles to PGF2␣. We observed on several occasions that the white lashes were new. This implies that the side effect may result from failure of pigmentation in newly stimulated eyelash growth or a stimulated growth of previously inconspicuous white lashes. Our observations in seven patients over a short time period (six months) imply that this side-effect is relatively common. Further prospective study with careful pretreatment and post-treatment photographs would help demonstrate the cause and increase understanding of this side effect.
BRIEF REPORTS
965