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Normal-tension glaucoma with reversed ophthalmic artery flow
2. Hee MR, Baumal CR, Puliafito CA, et al. Optical coherence tomography of age-related macular degeneration and choroidal neovascularization. Ophthalmology 1996;103:1260 –1270. 3. Hee MR, Puliafito CA, Wong C, et al. Optical coherence tomography of central serous chorioretinopathy. Am J Ophthalmol 1995;120:63–74. 4. Hee MR, Puliafito CA, Wong C, et al. Quantitative assessment of macular edema with optical coherence tomography. Arch Ophthalmol 1995;113:1019 –1029. 5. Toth CA, Narayan DG, Boppart SA, et al. A comparison of retinal morphology viewed by optical coherence tomography and by light microscopy. Arch Ophthalmol 1997;115:1425– 1428.
Normal-Tension Glaucoma With Reversed Ophthalmic Artery Flow Masato Hashimoto, MD, Kenji Ohtsuka, MD, Hideo Ohtsuka, MD, and Takashi Nakagawa, MD PURPOSE:
To describe a case of normal-tension glaucoma with bilateral complete occlusion of the internal carotid artery. METHODS: A 58-year-old man who had a 2-year history of normal-tension glaucoma in the left eye presented with ocular ischemic syndrome. To evaluate the orbital circulation, cerebral angiography and color Doppler imaging were performed. RESULTS: Cerebral angiography showed bilateral complete occlusion of the internal carotid artery and collateral blood supply through the right and left ophthalmic arteries, in which the flow was reversed. Moreover, color Doppler imaging revealed lower blood flow velocities in the left central retinal and short posterior ciliary arteries, suggesting a steal phenomenon. CONCLUSION: Normal-tension glaucoma in the present case may be caused by chronic reduction of retrobulbar blood flow resulting from a steal phenomenon. (Am J Ophthalmol 2000;130:670 – 672. © 2000 by Elsevier Science Inc. All rights reserved.)
FIGURE 1. (Top) This 35-year-old patient noted a persisting decline of vision in the course of an intermediate uveitis of unknown cause. Biomicroscopy revealed no definite pathologic finding of the macula. (Bottom) Optical coherence tomography of the 35-year-old patient demonstrated a pronounced cystoid macular edema (optically clear cystoid spaces in the foveolar region), which resolved only after the initiation of a cytotoxic therapy.
Optical coherence tomography was a good tool for monitoring persistent cystoid macular edema not visible by biomicroscopy and early development of epiretinal membranes in long-standing intermediate uveitis (Figure 1). Cytotoxic treatment of the persistent intraocular inflammation (12 eyes) and vitrectomy and membrane peeling (three eyes) were thus indicated. This clinical study suggests that optical coherence tomography is a particularly useful imaging technique in selected uveitis patients to diagnose and localize developing epiretinal1 and juxtafoveolar choroidal neovascular membranes2,3 and to monitor the effectiveness of treatment regimens on cystoid macular edema4 and preretinal granulomas, particularly when an invasive angiography is prohibited and biomicroscopy alone can be used.5 Optical coherence tomography may therefore provide useful information on complications developing in atopic uveitis patients.
T
Accepted for publication May 17, 2000. From the Department of Ophthalmology, Sapporo Medical University, School of Medicine (M.H., K.O., T.N), and the Ohtsuka Ophthalmological Clinic (H.O), Sapporo, Hokkaido, Japan. Inquiries to Masato Hashimoto, MD, PhD, Department of Ophthalmology, Sapporo Medical University, School of Medicine, S-1, W-16, Chuo-ku, Sapporo 060, Japan; fax: 81-11-613-6575.
REFERENCES
1. Wilkins JR, Puliafito CA, Hee MR, et al. Characterization of epiretinal membranes using optical coherence tomography. Ophthalmology 1996;103:2142–2151.
670
AMERICAN JOURNAL
HE PATHOGENESIS OF NORMAL-TENSION GLAUCOMA
remains unknown. Recent studies suggest that in patients with normal-tension glaucoma, vasospasm, or inappropriate vasoconstriction causes inadequate perfusion, resulting in tissue death at the optic nerve head.1,2 Some authors suggest that in patients with normal-tension glaucoma, vascular resistance downstream from the central retinal and posterior ciliary arteries is increased, choroidal
OF
OPHTHALMOLOGY
NOVEMBER 2000
FIGURE 1. Funduscopic examination (Left) shows a typical glaucomatous disk; a large cup and focal rim thinning, and Goldmann perimetry (Right) shows an arcuate scotoma and a nasal visual field defect in the left eye.
FIGURE 2. Lateral view of right (A and C) and left (B and bottom D) cerebral angiograms shows bilateral complete occlusion of the internal carotid artery (large arrowheads) and collateral intracranial circuit (large arrows) through the ophthalmic artery (small arrowheads) from the external carotid artery.
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filling times are prolonged, and the area of indocyanine green hypofluorescence is increased in the peripapillary region.3,4 In the present report, we describe a case of normaltension glaucoma with bilateral reversed blood flow of the ophthalmic artery resulting from complete bilateral carotid artery occlusion disclosed by cerebral angiography and color Doppler imaging. A 58-year-old man who had a 2-year-history of normaltension glaucoma in the left eye presented with left ocular pain. In the left eye, he had a typical glaucomatous disk; a large cup and focal rim thinning and arcuate scotoma (Figure 1). His intraocular pressure had been maintained between 12 and 14 mm Hg without medication in both eyes. In February 1999, he had a vitreous hemorrhage in the left eye. In April 1999, he had conjunctival injection and hyphema in the left anterior chamber. He was referred to Sapporo Medical University in September 1999. On examination, his corrected visual acuity was 20/20 in the right and hand motion in the left eye. Slit-lamp examinations showed episcleral dilatation in the left eye and deep anterior chamber in both eyes. Intraocular pressure was 13 mm Hg in the right and 19 mm Hg in the left eye. Funduscopic examinations showed a full pale disk, narrowing of the retinal artery branch, and an old vitreous hemorrhage in the inferior periphery vitreous in the left eye. There was no proptosis of both eyes. Computed tomography of the orbit showed mild dilation of the superior ophthalmic vein in the right orbit. To evaluate the orbital congestion, cerebral angiography was performed. Cerebral angiography showed bilateral complete occlusion of the internal carotid artery and collateral blood supply through the ophthalmic artery on the right and on the left, in which flow was reversed (Figure 2). Moreover, color Doppler imaging showed that the peak systolic velocity of the left ophthalmic artery was 71.6 cm/sec, and the peak systolic velocity of the central retinal and short posterior ciliary arteries could not be measured in the left orbit because of slow velocity blood flow, suggesting a steal phenomenon. Reversed ophthalmic artery flow, which indicates the collateral intracranial circuit through the ophthalmic artery from the external carotid artery, occurs when the circle of Willis is insufficient to provide adequate cerebral blood flow in patients with severe carotid artery occlusion. In the present case, cerebral angiography and color Doppler imaging showed reversed ophthalmic artery flow resulting from bilateral complete internal carotid artery occlusion. Costa and associates suggested that reversed ophthalmic artery flow shows a steal phenomenon that is characterized by reduction of retrobulbar blood flow.5 Recent studies suggest that the hemodynamics of the orbit and globe appear to be one of the multifactorial origins of normal-tension glaucoma.1– 4 Therefore, normal-tension glaucoma in this case may be associated with chronic reduction of retrobulbar blood flow resulting from a steal phenomenon. 672
AMERICAN JOURNAL
REFERENCES
1. Hernandez MR, Pena JDO. The optic nerve head in glaucomatous optic neuropathy. Arch Ophthalmol 1997;115:389 – 395. 2. Yamazaki Y, Drance SM. The relationship between progression of visual field defects and retrobulbar circulation in patients with glaucoma. Am J Ophthalmol 1997;124:287– 295. 3. Butt Z, O’Brien C, McKillop G, Aspinall P, Allan P. Color Doppler imaging in untreated high- and normal- pressure open angle glaucoma. Invest Ophthalmol Vis Sci 1997;38:690 – 696. 4. O’Brart DPS, de Souza Lima M, Bartsch DU, Freeman W, Weinreb RN. Indocyanine green angiography of the peripapillary region in glaucomatous eyes by confocal scanning laser ophthalmoscopy. Am J Ophthalmol 1997;123:657– 666. 5. Costa VP, Kuzniec S, Molnar LJ, et al. Collateral blood supply through the ophthalmic artery. A steal phenomenon analyzed by color Doppler imaging. Ophthalmology 1998;105:689 – 693.
A Novel Compound Heterozygous Mutation in the RDH5 Gene in a Patient With Fundus Albipunctatus Sachiko Kuroiwa, MD, PhD, Takanobu Kikuchi, PhD, and Nagahisa Yoshimura, MD, PhD PURPOSE:
To report a novel compound heterozygous mutation in the 11-cis retinol dehydrogenase (RDH5) gene in a patient with fundus albipunctatus. METHOD: We examined the RDH5 gene genotype in members of a Japanese family. Clinical examination showed that the proband had fundus albipunctatus and his aunt had retinitis pigmentosa. The RDH5 gene was analyzed by direct genomic sequencing. RESULTS: The proband had a compound heterozygotic missense mutation of Val177Gly (GTC3 GGC) and Arg280His (CGC3 CAC) in his RDH5 gene. His mother had the Arg280His mutation and his father had the Val177Gly mutation, but his father’s aunt who has typical retinitis pigmentosa had the wild type RDH5 gene. The occurrence of Val177Gly has not been reported in the RDH5 gene of fundus albipunctatus. CONCLUSION: A novel compound heterozygous missense mutation in the RDH5 gene was found in a patient with
Accepted for publication Aug 29, 2000. From the Department of Ophthalmology, Shinshu University School of Medicine, Matsumoto, Japan. Inquiries to Nagahisa Yoshimura, MD, PhD, Department of Ophthalmology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan; fax: 81-263-32-9448; e-mail: nagaeye@hsp. md.shinshu-u.ac.jp OF
OPHTHALMOLOGY
NOVEMBER 2000
Normal-Tension Glaucoma With Reversed Ophthalmic Artery Flow Masato Hashimoto, MD, Kenji Ohtsuka, MD, Hideo Ohtsuka, MD, and Takashi Nakagawa, MD PURPOSE:
To describe a case of normal-tension glaucoma with bilateral complete occlusion of the internal carotid artery. METHODS: A 58-year-old man who had a 2-year history of normal-tension glaucoma in the left eye presented with ocular ischemic syndrome. To evaluate the orbital circulation, cerebral angiography and color Doppler imaging were performed. RESULTS: Cerebral angiography showed bilateral complete occlusion of the internal carotid artery and collateral blood supply through the right and left ophthalmic arteries, in which the flow was reversed. Moreover, color Doppler imaging revealed lower blood flow velocities in the left central retinal and short posterior ciliary arteries, suggesting a steal phenomenon. CONCLUSION: Normal-tension glaucoma in the present case may be caused by chronic reduction of retrobulbar blood flow resulting from a steal phenomenon. (Am J Ophthalmol 2000;130:670 – 672. © 2000 by Elsevier Science Inc. All rights reserved.)
FIGURE 1. (Top) This 35-year-old patient noted a persisting decline of vision in the course of an intermediate uveitis of unknown cause. Biomicroscopy revealed no definite pathologic finding of the macula. (Bottom) Optical coherence tomography of the 35-year-old patient demonstrated a pronounced cystoid macular edema (optically clear cystoid spaces in the foveolar region), which resolved only after the initiation of a cytotoxic therapy.
Optical coherence tomography was a good tool for monitoring persistent cystoid macular edema not visible by biomicroscopy and early development of epiretinal membranes in long-standing intermediate uveitis (Figure 1). Cytotoxic treatment of the persistent intraocular inflammation (12 eyes) and vitrectomy and membrane peeling (three eyes) were thus indicated. This clinical study suggests that optical coherence tomography is a particularly useful imaging technique in selected uveitis patients to diagnose and localize developing epiretinal1 and juxtafoveolar choroidal neovascular membranes2,3 and to monitor the effectiveness of treatment regimens on cystoid macular edema4 and preretinal granulomas, particularly when an invasive angiography is prohibited and biomicroscopy alone can be used.5 Optical coherence tomography may therefore provide useful information on complications developing in atopic uveitis patients.
T
Accepted for publication May 17, 2000. From the Department of Ophthalmology, Sapporo Medical University, School of Medicine (M.H., K.O., T.N), and the Ohtsuka Ophthalmological Clinic (H.O), Sapporo, Hokkaido, Japan. Inquiries to Masato Hashimoto, MD, PhD, Department of Ophthalmology, Sapporo Medical University, School of Medicine, S-1, W-16, Chuo-ku, Sapporo 060, Japan; fax: 81-11-613-6575.
REFERENCES
1. Wilkins JR, Puliafito CA, Hee MR, et al. Characterization of epiretinal membranes using optical coherence tomography. Ophthalmology 1996;103:2142–2151.
670
AMERICAN JOURNAL
HE PATHOGENESIS OF NORMAL-TENSION GLAUCOMA
remains unknown. Recent studies suggest that in patients with normal-tension glaucoma, vasospasm, or inappropriate vasoconstriction causes inadequate perfusion, resulting in tissue death at the optic nerve head.1,2 Some authors suggest that in patients with normal-tension glaucoma, vascular resistance downstream from the central retinal and posterior ciliary arteries is increased, choroidal
OF
OPHTHALMOLOGY
NOVEMBER 2000
FIGURE 1. Funduscopic examination (Left) shows a typical glaucomatous disk; a large cup and focal rim thinning, and Goldmann perimetry (Right) shows an arcuate scotoma and a nasal visual field defect in the left eye.
FIGURE 2. Lateral view of right (A and C) and left (B and bottom D) cerebral angiograms shows bilateral complete occlusion of the internal carotid artery (large arrowheads) and collateral intracranial circuit (large arrows) through the ophthalmic artery (small arrowheads) from the external carotid artery.
VOL. 130, NO. 5
BRIEF REPORTS
671
filling times are prolonged, and the area of indocyanine green hypofluorescence is increased in the peripapillary region.3,4 In the present report, we describe a case of normaltension glaucoma with bilateral reversed blood flow of the ophthalmic artery resulting from complete bilateral carotid artery occlusion disclosed by cerebral angiography and color Doppler imaging. A 58-year-old man who had a 2-year-history of normaltension glaucoma in the left eye presented with left ocular pain. In the left eye, he had a typical glaucomatous disk; a large cup and focal rim thinning and arcuate scotoma (Figure 1). His intraocular pressure had been maintained between 12 and 14 mm Hg without medication in both eyes. In February 1999, he had a vitreous hemorrhage in the left eye. In April 1999, he had conjunctival injection and hyphema in the left anterior chamber. He was referred to Sapporo Medical University in September 1999. On examination, his corrected visual acuity was 20/20 in the right and hand motion in the left eye. Slit-lamp examinations showed episcleral dilatation in the left eye and deep anterior chamber in both eyes. Intraocular pressure was 13 mm Hg in the right and 19 mm Hg in the left eye. Funduscopic examinations showed a full pale disk, narrowing of the retinal artery branch, and an old vitreous hemorrhage in the inferior periphery vitreous in the left eye. There was no proptosis of both eyes. Computed tomography of the orbit showed mild dilation of the superior ophthalmic vein in the right orbit. To evaluate the orbital congestion, cerebral angiography was performed. Cerebral angiography showed bilateral complete occlusion of the internal carotid artery and collateral blood supply through the ophthalmic artery on the right and on the left, in which flow was reversed (Figure 2). Moreover, color Doppler imaging showed that the peak systolic velocity of the left ophthalmic artery was 71.6 cm/sec, and the peak systolic velocity of the central retinal and short posterior ciliary arteries could not be measured in the left orbit because of slow velocity blood flow, suggesting a steal phenomenon. Reversed ophthalmic artery flow, which indicates the collateral intracranial circuit through the ophthalmic artery from the external carotid artery, occurs when the circle of Willis is insufficient to provide adequate cerebral blood flow in patients with severe carotid artery occlusion. In the present case, cerebral angiography and color Doppler imaging showed reversed ophthalmic artery flow resulting from bilateral complete internal carotid artery occlusion. Costa and associates suggested that reversed ophthalmic artery flow shows a steal phenomenon that is characterized by reduction of retrobulbar blood flow.5 Recent studies suggest that the hemodynamics of the orbit and globe appear to be one of the multifactorial origins of normal-tension glaucoma.1– 4 Therefore, normal-tension glaucoma in this case may be associated with chronic reduction of retrobulbar blood flow resulting from a steal phenomenon. 672
AMERICAN JOURNAL
REFERENCES
1. Hernandez MR, Pena JDO. The optic nerve head in glaucomatous optic neuropathy. Arch Ophthalmol 1997;115:389 – 395. 2. Yamazaki Y, Drance SM. The relationship between progression of visual field defects and retrobulbar circulation in patients with glaucoma. Am J Ophthalmol 1997;124:287– 295. 3. Butt Z, O’Brien C, McKillop G, Aspinall P, Allan P. Color Doppler imaging in untreated high- and normal- pressure open angle glaucoma. Invest Ophthalmol Vis Sci 1997;38:690 – 696. 4. O’Brart DPS, de Souza Lima M, Bartsch DU, Freeman W, Weinreb RN. Indocyanine green angiography of the peripapillary region in glaucomatous eyes by confocal scanning laser ophthalmoscopy. Am J Ophthalmol 1997;123:657– 666. 5. Costa VP, Kuzniec S, Molnar LJ, et al. Collateral blood supply through the ophthalmic artery. A steal phenomenon analyzed by color Doppler imaging. Ophthalmology 1998;105:689 – 693.
A Novel Compound Heterozygous Mutation in the RDH5 Gene in a Patient With Fundus Albipunctatus Sachiko Kuroiwa, MD, PhD, Takanobu Kikuchi, PhD, and Nagahisa Yoshimura, MD, PhD PURPOSE:
To report a novel compound heterozygous mutation in the 11-cis retinol dehydrogenase (RDH5) gene in a patient with fundus albipunctatus. METHOD: We examined the RDH5 gene genotype in members of a Japanese family. Clinical examination showed that the proband had fundus albipunctatus and his aunt had retinitis pigmentosa. The RDH5 gene was analyzed by direct genomic sequencing. RESULTS: The proband had a compound heterozygotic missense mutation of Val177Gly (GTC3 GGC) and Arg280His (CGC3 CAC) in his RDH5 gene. His mother had the Arg280His mutation and his father had the Val177Gly mutation, but his father’s aunt who has typical retinitis pigmentosa had the wild type RDH5 gene. The occurrence of Val177Gly has not been reported in the RDH5 gene of fundus albipunctatus. CONCLUSION: A novel compound heterozygous missense mutation in the RDH5 gene was found in a patient with
Accepted for publication Aug 29, 2000. From the Department of Ophthalmology, Shinshu University School of Medicine, Matsumoto, Japan. Inquiries to Nagahisa Yoshimura, MD, PhD, Department of Ophthalmology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan; fax: 81-263-32-9448; e-mail: nagaeye@hsp. md.shinshu-u.ac.jp OF
OPHTHALMOLOGY
NOVEMBER 2000