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In a report of a 61-year-old man with bilateral PION after cardiac bypass surgery, MRI of the orbits with diffusion-weighted and fl uid-attenuated inver-sion recovery FLAIR sequences can

Trang 1

had a fi nal visual outcome of hand motion or

worse All patients developed optic disc atrophy

Approximately 66% of patients had vascular

risk factors, such as hypertension, diabetes

mellitus, hypercholesterolemia, coronary artery

disease, congestive heart failure, cardiac

arrhythmia, obesity, and tobacco use The

remainder had no vascular risk factors

The average duration of operation was 8.7 h

The mean decrease of hematocrit was 14.4%

between the preoperative and the

periopera-tive period The mean decrease of systolic blood

pressure was 53 mmHg between the

preopera-tive and the perioperapreopera-tive period The mean

intraoperative estimated blood loss was 3.7 L

If no intraoperative hypotension is

docu-mented during the surgery, then anemia is most

likely, as evidenced by a mean hemoglobin level

that has decreased by 40% to 50% in the

peri-operative period.228

Neuroimaging of the optic nerves in the

peri-operative setting can sometimes show the

loca-tion of the lesion in PION In a report of a

61-year-old man with bilateral PION after

cardiac bypass surgery, MRI of the orbits with

diffusion-weighted and fl uid-attenuated

inver-sion recovery (FLAIR) sequences can reveal

abnormal hyperintensity in both intraorbital

optic nerves.245 Bilateral intraorbital optic nerve

enhancement was seen on MRI 8 weeks after

coronary bypass grafting in a 57-year-old

woman who had hypotensive posterior

isch-emic optic neuropathy.246

Histopathology demonstrates that infarction

occurs in the intraorbital portion of the optic

nerve in patients with perioperative PION The

central axial portion of the optic nerve is usually

infarcted, and may be hemorrhagic, with sparing

of the nerve periphery Occasionally, the

infarc-tion may extend to the periphery

circumferen-tially, especially in the midorbital section of the

optic nerve The loss of peripheral axons appears

to correspond to constricted visual fi elds.247–249

In the report by Nawa et al.248 on a 67-year-old

man with bilateral PION after radical neck

dissection complicated by intraoperative

hypo-tension and anemia, histopathology of the

optic nerve revealed acellularity of the fi

bro-vascular pial septae, swollen macrophages,

some hemorrhage, and loss of myelin The

para-central pial vessels had a few small thrombi, but

no emboli

Perioperative hemodynamic changes causing decreased oxygen delivery to the optic nerve are thought to cause PION These hemody-namic factors include hypotension, anemia, increased venous pressure, a prone position during surgery, direct ocular compression, increased cerebrospinal fl uid pressure, and embolism Another factor that may decrease oxygen delivery to the optic nerve is defective vascular autoregulation caused by vascular endothelial dysfunction.250 It has been shown that normal compensatory vasoconstriction and vasodilation during fl uctuating blood pressures does not occur in diabetic patients.251 This lack

of vascular autoregulation during perioperative hypotensive episodes would increase the risk of developing perioperative PION Anatomic variation of the intraorbital blood supply may also account for a patient’s susceptibility to perioperative PION The arterial supply of the intraorbital optic nerve derives from two sepa-rate systems, the peripheral centripetal vessels and the axial centrifugal vessels.252 The pial plexus is formed by collaterals directly from the ophthalmic artery and from collateral from other intraorbital subdivisions of the ophthal-mic artery The axial system is formed from branches of the central retinal artery after it penetrates the optic nerve sheath These branches radiate from the central optic nerve

to penetrate the parenchyma The anastomoses between the peripheral and central vascular systems may vary among patients Those who lack these anastomoses have a watershed zone that is more susceptible to ischemia during perioperative hemodynamic changes.252

Treatment for PION is limited at this time Perioperative correction of hemodynamic abnormalities may be benefi cial in certain instances In a report by Stevens et al.,242 cor-rection of anemia and hypotension led to com-plete visual recovery in one patient, who received blood transfusions to maintain a hema-tocrit above 35% and a blood pressure about 140/80 mmHg by discontinuation of antihyper-tensive medications Postoperative visual acuity was 20/70 in the right eye (OD) and 20/200 in the left eye (OS) After transfusion, visual acuity

Trang 2

52 J.W Chan

was 20/40 OD and 20/30 OS Seven months

later, his visual acuity improved to 20/20 OU

This report suggests that early transfusion for

perioperative anemia can prevent

periopera-tive PION Specifi c clinical guidelines for

trans-fusion have been controversial, and the decision

to transfuse should be based upon the patient’s

risk of developing complications of decreased

oxygenation.253

In addition, simultaneous internal jugular

vein ligation should be avoided to prevent

PION after radical neck dissection Staging of

the neck dissection does not appear to prevent

The visual prognosis for PION is usually poor

No proven effective treatment is available to

reverse visual loss.19

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138 Aiello AL, Sadun AA, Feldon SE Spontaneous

improvement of progressive anterior ischemic

optic neuropathy: report of two cases Arch

Ophthalmol 1992;110(9):1197–9.

139 Liu GT, Glaser JS, Schatz NJ, Smith JL Visual

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charac-teristics and prognosis for vision

Ophthalmol-ogy 1994;101(11):1779–85.

140 Liu NH, LaBree LD, Feldon SE, Rao NA The

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retrospective study Ophthalmology 2001;108(6):

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141 Tomsak RL Giant cell arteritis Ophthalmology

2002;109(2):219–20.

142 Salvarani C, Cantini F, Boiardi L, Hunder GG

Polymyalgia rheumatica and giant-cell arteritis

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143 Hayreh SS, Podhajsky PA, Raman R,

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144 Gonzalez-Gay MA, Garcia-Porrua C,

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2006;8(4):299–302.

145 Hayreh SS, Podhajsky PA, Zimmerman B

Occult giant cell arteritis: ocular manifestations

Am J Ophthalmol 1998;125(4):521–6.

146 Hayreh SS, Jonas JB Optic disc morphology

after arteritic anterior ischemic optic

neuropa-thy Ophthalmology 2001;108(9):1586–94.

147 Kaiser M, Younge B, Bjornsson J, Goronzy JJ,

Weyand CM Formation of new vaso vasorum

in vasculitis Production of angiogenic cytokines

by multinucleated giant cells Am J Pathol

minor-149 Brack A, Geisler A, Martinez-Taboada VM, Younge BR, Goronzy JJ, Weyand CM Giant cell vasculitis is a T cell-dependent disease Mol Med 1997;3(8):530–43.

150 Martinez-Taboada V, Hunder NN, Hunder GG, Weyand CM, Goronzy JJ Recognition of tissue residing antigen by T cells in vasculitic lesions

of giant cell arteritis J Mol Med 1996;74(11): 695–703.

151 Brack A, Rittner HL, Younge BR, Kaltschmidt

C, Weyand CM, Goronzy JJ mediated repression of cytokine gene transcrip- tion in human arteritis-SCID chimeras J Clin Invest 1997;99(12):2842–50.

Glucocorticoid-152 Rittner HL, Kaiser M, Brack A, Szweda LI, Goronzy JJ, Weyand CM Tissue-destructive macrophages in giant cell arteritis Circ Res 1999;84(9):1050–8.

153 Weyand CM, Goronzy JJ Pathogenic nisms in giant cell arteritis Cleve Clin J Med 2002;69(suppl 2):SII28–32.

mecha-154 Mitchell BM, Font RL Detection of varicella zoster virus DNA in some patients with giant cell arteritis Invest Ophthalmol Vis Sci 2001; 42(11):2572–7.

155 Rimenti G, Blasi F, Cosentini R, et al Temporal

arteritis associated with Chlamydia pneumoniae

DNA detected in an artery specimen J matol 2000;27(11):2718–20.

Rheu-156 Haugeberg G, Bie R, Nordbo SA Temporal

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DNA detected in an artery specimen J matol 2001;28(7):1738–9.

Rheu-157 Regan MJ, Wood BJ, Hsieh YH, et al Temporal

arteritis and Chlamydia pneumoniae: failure to

detect the organism by polymerase chain tion in ninety cases and ninety controls Arthri- tis Rheum 2002;46(4):1056–60.

reac-158 Helweg-Larsen J, Tarp B, Obel N, Baslund B No

evidence of parvovirus B19, Chlamydia

pneu-moniae or human herpes virus infection in

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159 Hunder GG, Bloch DA, Michel BA, et al The American College of Rheumatology 1990 crite- ria for the classifi cation of giant cell arteritis Arthritis Rheum 1990;33(8):1122–8.

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58 J.W Chan

160 Evans JM, Hunder GG Polymyalgia

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161 Jundt JW, Mock D Temporal arteritis with

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162 Salvarani C, Hunder GG Giant cell arteritis

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163 Wise CM, Agudelo CA, Chmelewski WL,

McK-night KM Temporal arteritis with low

erythro-cyte sedimentation rate: a review of fi ve cases

Arthritis Rheum 1991;34(12):1571–4.

164 Wong RL, Korn JH Temporal arteritis without

an elevated erythrocyte sedimentation rate

Case report and review of the literature Am J

Med 1986;80(5):959–64.

165 Miller A, Green M, Robinson D Simple rule for

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166 Foroozan R, Danesh-Meyer H, Savino PJ,

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167 Gonzalez-Alegre P, Ruiz-Lopez AD,

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168 Costello F, Zimmerman MB, Podhajsky PA,

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169 Salvarani C, Cantini F, Boiardi L, Hunder GG

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170 Guevara RA, Newman NJ, Grossniklaus HE

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171 Liu GT, Volpe NJ, Galetta SL

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172 Hall JK, Volpe NJ, Galetta SL, Liu GT, Syed

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173 Klein RG, Campbell RJ, Hunder GG, Carney

JA Skip lesions in temporal arteritis Mayo Clin Proc 1976;51(8):504–10.

174 Danesh-Meyer HV, Savino PJ , Eagle RC, et al Low diagnostic yield with second biopsies in suspected giant cell arteritis J Neuro-Ophthal- mol 2000;20:213–5.

175 Pless M, Rizzo JF III, Lamkin JC, Lessell S Concordance of bilateral temporal artery biopsy in giant cell arteritis J Neuro-Ophthal- mol 2000;20(3):216–8.

176 Ray-Chaudhuri N, Kine DA, Tijani SO, et al Effect of prior steroid treatment on temporal artery biopsy fi ndings in giant cell arteritis Br

J Ophthalmol 2002;86(5):530–2.

177 Lie JT Temporal artery biopsy diagnosis of giant cell arteritis: lessons from 1109 biopsies Anat Pathol 1996;1:69–97.

178 Poller DN, van Wyk Q, Jeffrey MJ The tance of skip lesions in temporal arteritis J Clin Pathol 2000;53(2):137–9.

impor-179 McDonnell PJ, Moore GW, Miller NR, Hutchins

GM, Green WR Temporal arteritis A pathologic study Ophthalmology 1986;93(4): 518–30.

clinico-180 Ghanchi FD, Dutton GN Current concepts in giant cell (temporal) arteritis Surv Ophthalmol 1997;42(2):99–123.

181 Nordborg E, Nordborg C Giant cell arteritis: strategies in diagnosis and treatment Curr Opin Rheumatol 2004;16(1):25–30.

182 Ho AC, Sergott RC, Regillo CD, et al Color Doppler hemodynamics of giant cell arteritis Arch Ophthalmol 1994;112(7):938–45.

183 Schmid R, Hermann M, Yannar A, ner RW Color duplex ultrasound of the temporal artery: replacement for biopsy in temporal arteritis Ophthalmologica 2002; 216(1):16–21.

Baumgart-184 Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle EJ Color duplex ultrasonogra- phy in the diagnosis of temporal arteritis N Engl J Med 1997;337(19):1336–42.

185 Salvarani C, Silingardi M, Ghirarduzzi A, et al

Is duplex ultrasonography useful for the nosis of giant-cell arteritis? Ann Intern Med 2002;137(4):232–8.

diag-186 Morgenstern KE, Ellis BD, Schochet SS, Linberg

JV Bilateral optic nerve sheath enhancement from giant cell arteritis J Rheumatol 2003;30(3): 625–7.

187 Lee AG, Eggenberger ER, Kaufman DI, rique C Optic nerve enhancement on magnetic resonance imaging in arteritic ischemic optic

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Man-neuropathy J Neuro-Ophthalmol 1999;19(4):

235–7.

188 Harada S, Mitsunobu F, Kodama F, et al Giant

cell arteritis associated with rheumatoid

arthri-tis monitored by magnetic resonance

angiogra-phy Intern Med 1999;38(8):675–8.

189 Mitomo T, Funyu T, Takahashi Y, Murakami K,

Koyama K, Kamio K Giant cell arteritis and

magnetic resonance angiography Arthritis

Rheum 1998;41(9):1702.

190 Stanson AW Imaging fi ndings in extracranial

(giant cell) temporal arteritis Clin Exp

Rheu-matol 2000;18(4 suppl 20):S43–8.

191 Zborowska B, Ell J, McGhee-Collett M, Scolyer

R, McCluskey PJ Progressive visual loss in a

patient with presumed temporal arteritis despite

treatment: how to make the diagnosis Clin Exp

Ophthalmol 2004;32(3):335–6.

192 Hayreh SS, Zimmerman B Visual deterioration

in giant cell arteritis patients while on high

doses of corticosteroid therapy Ophthalmology

2003;110(6):1204–15.

193 Hayreh SS, Zimmerman B Management of

giant cell arteritis Our 27-year clinical study:

new light on old controversies

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194 Foroozan R, Deramo VA, Buono LM, et al

Recovery of visual function in patients with

biopsy-proven giant cell arteritis

Ophthalmol-ogy 2003;110(3):539–42.

195 Kim N, Trobe JD, Flint A, Keoleian G

Late ipsilateral recurrence of ischemic optic

neuropathy in giant cell arteritis J

Neuro-Ophthalmol 2003;23(2):122–6.

196 Hunder GG Giant cell arteritis and

polymyal-gia rheumatica Med Clin N Am 1997;81(1):

195–219.

197 Andersson R, Malmvall BE, Bengtsson BA

Long-term corticosteroid treatment in giant

cell arteritis Acta Med Scand 1986;220(5):

465–9.

198 Bengtsson BA, Malmvall BE Prognosis of giant

cell arteritis including temporal arteritis and

polymyalgia rheumatica A follow-up study on

ninety patients treated with corticosteroids

Acta Med Scand 1981;209(5):337–45.

199 Chan CC, Paine M, O’Day J Predictors of

recurrent ischemic optic neuropathy in giant

cell arteritis J Neuro-Ophthalmol 2005;25(1):

14–7.

200 Doury P, Pattin S, Eulry F, Thabaut A The use

of dapsone in the treatment of giant cell

arteri-tis and polymyalgia rheumatica Arthriarteri-tis

Rheum 1983;26(5):689–90.

201 Krall PL, Mazanec DJ, Wilke WS Methotrexate for corticosteroid-resistant polymyalgia rheu- matica and giant cell arteritis Cleve Clin J Med 1989;56(3):253–7.

202 Spiera RF, Kupersmith M, Paget S, Spiera H Vision loss in giant cell arteritis patients treated with alternate-day corticosteroids: comment on the article by Hoffman et al Arthritis Rheum 2003;48(4):1159–60.

203 Utsinger PD Treatment of steroid sive giant cell arteritis with Cytoxan Arthritis Rheum 1982;25(suppl):S31.

nonrespon-204 Hoffman GS, Cid MC, Hellmann DB, et al.; International Network for the Study of Systemic Vasculitides A multicenter, random- ized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis Arthritis Rheum 2002;46(5): 1309–18.

205 Jover JA, Hernandez-Garcia C, Morado IC, Vargas E, Banares A, Fernandez-Gutierrez B Combined treatment of giant-cell arteritis with methotrexate and prednisone A randomized, double-blind, placebo-controlled trial Ann Intern Med 2001;134(2):106–14.

206 Spiera RF, Mitnick HJ, Kupersmith M, et al A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treat- ment of giant cell arteritis (GCA) Clin Exp Rheumatol 2001;19(5):495–501.

207 De Silva M, Hazleman BL Azathioprine in giant cell arteritis/polymyalgia rheumatica:

a double-blind study Ann Rheum Dis 1986; 45(2):136–8.

208 Schaufelberger C, Andersson R, Nordborg E

No additive effect of cyclosporin A compared with glucocorticoid treatment alone in giant cell arteritis: results of an open, controlled, randomized study Br J Rheumatol 1998; 37(4):464–5.

209 Cantini F, Niccoli L, Salvarani C, Padula A, Olivieri I Treatment of longstanding active giant cell arteritis with infl iximab: report of four cases Arthritis Rheum 2001;44(12):2933–5.

210 Airo P, Antonioli CM, Vianelli M, Toniati P Anti-tumour necrosis factor treatment with inf- liximab in a case of giant cell arteritis resistant

to steroid and immunosuppressive drugs matology (Oxf) 2002;41(3):347–9.

Rheu-211 Andonopoulos AP, Meimaris N, Daoussis D, Bounas A, Giannopoulos G Experience with infl iximab (anti-TNF alpha monoclonal anti- body) as monotherapy for giant cell arteritis Ann Rheum Dis 2003;62(11):1116.

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60 J.W Chan

212 Uthman I, Kanj N, Atweh S Infl iximab as

monotherapy in giant cell arteritis Clin

Rheu-matol 2006;25(1):109–10.

213 Tan AL, Holdsworth J, Pease C, Emery P,

McG-onagle D Successful treatment of resistant

giant cell arteritis with etanercept Ann Rheum

Dis 2003;62(4):373–4.

214 Weyand CM, Kaiser M, Yang H, Younge B,

Goronzy JJ Therapeutic effects of

acetylsali-cylic acid in giant cell arteritis Arthritis Rheum

2002;46(2):457–66.

215 Ruegg S, Engelter S, Jeanneret C, et al Bilateral

vertebral artery occlusion resulting from giant

cell arteritis: report of 3 cases and review of the

literature Medicine (Baltim) 2003;82(1):1–12.

216 Nesher G, Berkun Y, Mates M, Baras M,

Rubinow A, Sonnenblick M Low-dose aspirin

and prevention of cranial ischemic

complica-tions in giant cell arteritis Arthritis Rheum

2004;50(4):1332–7.

217 Hayreh SS, Zimmerman B, Kardon RH Visual

improvement with corticosteroid therapy in

giant cell arteritis Report of a large study and

review of literature Acta Ophthalmol Scand

2002;80(4):355–67.

218 Font C, Cid MC, Coll-Vinent B, Lopez-Soto A,

Grau JM Clinical features in patients with

permanent visual loss due to biopsy-proven

giant cell arteritis Br J Rheumatol 1997;36(2):

251–4.

219 Schmidt D, Vaith P, Hetzel A Prevention of

serious ophthalmic and cerebral complications

in temporal arteritis? Clin Exp Rheumatol

2000;18(4 suppl 20):S61–3.

220 Gonzalez-Gay MA, Blanco R,

Rodriguez-Valverde V, et al Permanent visual loss and

cerebrovascular accidents in giant cell arteritis:

predictors and response to treatment Arthritis

Rheum 1998;41(8):1497–504.

221 Aiello PD, Trautmann JC, McPhee TJ,

Kunsel-man AR, Hunder GG Visual prognosis in giant

cell arteritis Ophthalmology 1993;100(4):

550–5.

222 Hsu CT, Kerrison JB, Miller NR, Goldberg MF

Choroidal infarction, anterior ischemic optic

neuropathy, and central retinal artery occlusion

from polyarteritis nodosa Retina 2001;21(4):

348–51.

223 Duran E, Merkel PA, Sweet S, Swan N, Babikian

VL ANCA-associated small vessel vasculitis

presenting with ischemic optic neuropathy

Neurology 2004;62(1):152–3.

224 Khurma V, Appen R, Wolf MD, Hansen KE

Wegener granulomatosis presenting as bilateral

loss of vision Clin Rheumatol 2005;11(5): 267–9.

225 Hayakawa K, Akatsuka I, Matsukura S, Kawai

K, Ohkuma H, Shimamura K Case of anterior ischemic optic neuropathy accompanied by Churg–Strauss syndrome Nippon Ganka Gakkai Zasshi 2004;108(10):612–7.

226 Khairallah M, Zaouali S, Ben Yahia S, et al Anterior ischemic optic neuropathy associated

with Rickettsia conorii infection J

Neuro-Oph-thalmol 2005;25(3):212–4.

227 Sadda SR, Nee M, Miller NR, Biousse V, Newman NJ, Kouzis A Clinical spectrum of posterior ischemic optic neuropathy Am J Ophthalmol 2001;132(5):743–50.

228 Buono LM, Foroozan R Perioperative rior ischemic optic neuropathy: review of the literature Surv Ophthalmol 2005;50(1):15–26.

poste-229 Saul GV, Sarkies NJC PION due to internal carotid artery occlusion Neuro-Ophthalmol- ogy 1987;7:349–53.

230 Tsai RK, Sun CY Spontaneous dissection of internal carotid artery presenting as isolated posterior ischaemic optic neuropathy Br J Ophthalmol 1997;81(6):513.

231 Hashimoto M, Ohtsuka K, Suzuki Y, Hoyt WF

A case of posterior ischemic optic neuropathy

in a posterior-draining dural cavernous sinus fi stula J Neuro-Ophthalmol 2005;25(3): 176–9.

232 Kothe AC, Flanagan J, Trevino RC True rior ischemic optic neuropathy associated with herpes zoster ophthalmicus Optom Vis Sci 1990;67(11):845–9.

poste-233 Weinstein JM, Morris GL, ZuRhein GM, Gentry

LR Posterior ischemic optic neuropathy

due to Aspergillus fumigatus J Clin

Neuro-Ophthalmol 1989;9(1):7–13.

234 Lee AG, Brazis PW, Miller NR Posterior emic optic neuropathy associated with migraine Headache 1996;36(8):506–10.

isch-235 Claes C, Milea D, Bodaghi B, Tran TH, LeHoang

P, Blanc R Acute retrobulbar optic neuropathy due to rupture of an anterior communicating artery aneurysm Acta Ophthalmol Scand 2006; 84(1):145–6.

236 Hara N, Mukuno K, Ohtaka H, Shimizu K Ischemic optic neuropathy associated with subarachnoid hemorrhage after rupture Ophthalmologica 2003;217(1):79–84.

237 Perlman JI, Forman S, Gonzalez ER bar ischemic optic neuropathy associated with sickle cell disease J Neuro-Ophthalmol 1994; 14(1):45–8.

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Retrobul-238 Balm AJ, Brown DH, De Vries WA, Snow GB

Blindness: a potential complication of bilateral

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239 Chang SH, Miller NR The incidence of vision

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240 Maran AG, Amin M, Wilson JA Radical neck

dissection: a 19-year experience J Laryngol

Otol 1989;103(8):760–4.

241 Nuttall GA, Garrity JA, Dearani JA, Abel MD,

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242 Stevens WR, Glazer PA, Kelley SD, Lietman

TM, Bradford DS Ophthalmic complications

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243 Sweeney PJ, Breuer AC, Selhorst JB, et al

Ischemic optic neuropathy: a complication of

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244 Cheng MA, Sigurdson W, Tempelhoff R,

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245 Purvin V, Kuzma B Intraorbital optic nerve

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246 Vaphiades MS Optic nerve enhancement in

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247 Johnson MW, Kincaid MC, Trobe JD Bilateral

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loss and hypotension A clinicopathologic case study Ophthalmology 1987;94(12):1577–84.

248 Nawa Y, Jaques JD, Miller NR, Palermo RA, Green WR Bilateral posterior optic neuropa- thy after bilateral radical neck dissection and hypotension Graefes Arch Clin Exp Ophthal- mol 1992;230(4):301–8.

249 Schobel GA, Schmidbauer M, Millesi W, Undt

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253 Practice Guidelines for blood component therapy: a report by the American Society of Anesthesiologists Task Force on Blood Com- ponent Therapy Anesthesiology 1996;84(3): 732–47.

254 Kirkali P, Kansu T A case of unilateral posterior ischemic optic neuropathy after radical neck dissection Ann Ophthalmol 1990;22(8):297–8.

255 Marks SC, Jaques DA, Hirata RM, Saunders JR

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256 Milner GA A case of blindness after bilateral neck dissection J Laryngol Otol 1960;74: 880–5.

257 Pazos GA, Leonard DW, Blice J, Thompson

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3

Papilledema

Jane W Chan

Papilledema is clinically defi ned as optic disc

swelling resulting only from increased

intracra-nial pressure (ICP), as opposed to the optic disc

swelling from other etiologies, such as ischemia

or infl ammation (Table 3.1) Cerebrospinal

fl uid pressure (CSF) equal to or greater than

250 mmH2O taken in a person lying in the

lateral recumbent position is considered

abnor-mally elevated Normal CSF pressure is usually

in the range of 100 mmH2O to 250 mmH2O.1

Symptoms of Papilledema

One of the most common symptoms of increased

ICP is headache.3 It is usually a dull, pulsatile,

occipital or generalized headache It can occur

daily and last for hours The headache is not

related to the ICP changes or other associated

symptoms It may be worsened with Valsalva

maneuvers, such as coughing or straining It is

usually associated with neck stiffness, nausea,

and vomiting Occasionally, retrobulbar pain

may occur with eye movement Pain may even

radiate to facial dermatomes.3

Transient visual obscurations may involve

blurry vision to complete loss of vision and are

thought to be related to transient ischemia of

the optic nerve.4 Acute episodes of blurry vision

are the most common and usually last less than

30 s and rarely several hours.5 They may be

monocular or binocular and are not related to

the degree of intracranial pressure or to the

severity of papilledema These visual symptoms

are precipitated by postural changes.5 Positive

visual phenomena, such as photopsias and phosphenes, are also transient and are thought

to be related to traction of the retrobulbar optic nerve or retina.6

Pulsatile tinnitus is often unilateral and is eliminated temporarily by compression of the ispilateral jugular vein and by lowering CSF pressure by a lumbar puncture (LP) High-pressure vascular pulsations are thought to be transmitted by CSF to the venous sinus walls to cause this tinnitus.6

Signs of PapilledemaThe key feature of increased ICP is papilledema and may be classifi ed into the following four different stages: (1) early, (2) fully developed, (3) chronic, and (4) atrophic.7

A very early sign of papilledema is emia, dilatation of capillaries on the disc surface The retinal nerve fi ber layer also loses its super-

hyper-fi cial curvilinear light refl exes to become more red The optic disc usually is swollen, initially in the inferior pole, then at the superior pole, fol-lowed by the temporal and nasal aspects, respec-tively (Figure 3.1).8 Because of the disc swelling, the optic disc margins become blurred Rupture

of a distended capillary within or surrounding the disc may also cause peripapillary nerve

fi ber layer hemorrhages These fl ame-shaped hemorrhages appear as thin streaks of blood on

or near the margins of the optic disc Because

of the elevated intracranial pressure, ous retinal venous pulsations are absent CSF

Trang 13

spontane-pressures of 200 mmH2O or greater may even

inhibit these pulsations As 20% of persons with

normal CSF pressure have spontaneous venous

pulsations, the observation of spontaneous

venous pulsations only indicates that the CSF

pressure is below 200 mmH2O at that time and

is not always a reliable sign of papilledema.7

In more fully developed papilledema,

periph-eral retinal veins become engorged and dark

Closer to the disc, splinter hemorrhages may be

seen as edema in the retinal nerve fi ber layer

increases at or adjacent to the disc margin

More fl ame-shaped hemorrhages may appear

Table 3.1 The differential diagnosis of the swollen

disc (adapted from Miller and Newman 2 )

Not true disc swelling: congenitally anomalous, elevated

optic disc

• With and without buried drusen

• Tilted optic disc

• Hypoplastic optic disc

True disc swelling

• Elevated intracranial pressure: secondary to mass

lesion, cerebral venous thrombosis, or idiopathic

(pseudotumor cerebri)

• Infl ammatory: infections, demyelination, sarcoidosis

• Vascular: anterior ischemic optic neuropathy, central

retinal vein occlusion

• Compressive: secondary to neoplasms (meningioma) or

thyroid ophthalmopathy

• Infi ltrative: secondary to neoplasms (leukemia) or

sarcoidosis

• Toxic/metabolic/nutritional defi ciency

• Hereditary: Leber’s hereditary optic neuropathy

• Traumatic optic neuropathy

Figure 3.1 Early stages of papilledema Early nerve fi ber layer edema is fi rst seen superiorly (left), then inferiorly and nasally (right) (Reprinted from Spalton et al.,8 with permission from Elsevier.)

as a result of sudden rises of CSF pressure The surface of the disc then becomes elevated above the retinal surface, and microaneurysms and dilated capillaries appear The peripapillary surface blood vessels become obscured by more edema in the retinal nerve fi ber layer Focal retinal infarcts, or cotton wool spots and tortu-ous vessels, appear (Figure 3.2).7,8

In severely elevated ICP, circumferential choroidal folds, or Paton’s lines, may develop Choroidal folds may even be the initial present-ing sign of increased ICP, according to a study

by Griebel and Kosmorsky.9 Ten of 12 patients had ICPs of greater than 120 mmH2O, and 8 of

12 were diagnosed with idiopathic intracranial hypertension (IIH) Four patients had increased ICP and choroidal folds in the absence of pap-illedema It was hypothesized that the choroidal folds might represent enlargement of the retro-laminar optic nerve sheath in the absence of axonal swelling Alternatively, it was proposed that the choroidal folds might persist after reso-lution of papilledema The presence of choroi-dal folds in isolation might be related to the timing of the evaluation

Hard exudates and hemorrhages may occur

in the peripapillary region and in the macula

to cause decreased central vision Development

of macular edema may be a risk factor for manent visual loss in IIH In a study by Talks et al.,10 44% (21 of 48) of eyes in 24 patients who had progressive visual deteriora-tion from IIH requiring optic nerve sheath fen-estration developed macular changes, including choroidal folds, Paton’s lines, nerve fi ber layer hemorrhages, subretinal hemorrhages, macular

Trang 14

per-64 J.W Chan

stars, macular edema, and retinal pigment

epi-thelial changes These changes probably

con-tributed to the severe visual loss in 5 eyes, 3 of

which did not improve despite treatment It was

concluded that these macular changes might

not have had a signifi cant impact on optic

nerve-related visual loss in patients with IIH,

but the patients who developed macular edema

might be at greater risk for permanent visual

loss

If ICP increases abruptly, severe subhyaloid

hemorrhages may occur and occasionally bleed

and dissect into the vitreous in about 4% of

patients with papilledema.11 These intraretinal

hemorrhages are often the result of

compres-sion of the central retinal vein from the swollen optic disc and usually resolve with treatment of elevated ICP.12

In chronic papilledema, hemorrhages and exudates slowly resolve, and the optic disc cup

is gradually destroyed (Figure 3.3).8 The disc may have hard exudates mimicking disc drusen,

a sign that the papilledema has been present for several months Nerve fi ber layer atrophy may also appear as slitlike defects on red-free direct ophthalmoscopy.13 Chronic papilledema may even persist for many years without sig-nifi cant visual symptoms, especially in patients with pseudotumor cerebri or with intracranial tumors.14

Figure 3.3 Chronic papilledema Both optic discs

(left and right) are swollen with no hemorrhages or

cotton wool spots, indicative of a slow, gradual

increase in cerebrospinal fl uid (CSF) pressure (Reprinted from Spalton et al., 8 with permission from Elsevier.)

Figure 3.2 Later stages of papilledema Venous

engorgement increases as further disc swelling

extends temporally (left) Hemorrhages and cotton

wool spots also develop (right) (Reprinted from

Spalton et al., 8 with permission from Elsevier.)

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