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1 대한안과학회지 2017 년제 58 권제 12 호 J Korean Ophthalmol Soc 2017;58(12): ISSN (Print) ISSN (Online) Case Report 원발개방각녹내장환자에서발생한시신경유두부종 1 예 A Case of Optic Nerve Head Swelling in a Patient with Primary Open-angle Glaucoma 김동근 1,2 김정림 1,2 Dong Geun Kim, MD 1,2, Jung Lim Kim, MD, PhD 1,2 인제대학교의과대학부산백병원안과학교실 1, 인제대학교의과대학부산백병원안과질환 T2B 기반구축센터 2 Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine 1, Busan, Korea Therapeutics Center for Ocular Neovascular Disease, Busan Paik Hospital, Inje University College of Medicine 2, Busan, Korea Purpose: To report a case of masked glaucomatous optic nerve head damage due to acute swelling in a primary open-angle glaucoma patient. Case Summary: A healthy 21-year-old male visited our clinic complaining of blurred vision in the right eye for 1 week. The intraocular pressure (IOP) was 60 mmhg, as measured by a Goldmann applanation tonometer. No specific anterior segment finding other than severe corneal edema was found on slit lamp examination. Maximum tolerated medical therapy was performed, and a further examination was done 1 day after the IOP lowering. No glaucomatous change in the optic disc or retinal nerve fiber layer was observed on fundus examination and optical coherence tomography (OCT), but the optic disc of the right eye was more hyperemic than that of the left eye. A superonasal visual field defect was also observed using automated perimetry. After treatment, the IOP was kept within the normal range using IOP-lowering eye drops. However, an inferonasal retinal nerve fiber layer defect was observed on fundus examination and OCT, and a superonasal scotoma was detected by perimetry. J Korean Ophthalmol Soc 2017;58(12): Keywords: Optic nerve head swelling, Primary open-angle glaucoma 안과영역에서빛간섭단층촬영 (optical coherence tomography, OCT) 이대중화되면서녹내장의진단과치료에널리이용되고있다. 망막신경섬유층과시신경유두신경테의두 Received: Revised: Accepted: Address reprint requests to Jung Lim Kim, MD, PhD Department of Ophthalmology, Inje University Busan Paik Hospital, #75 Bokji-ro, Busanjin-gu, Busan 47392, Korea Tel: , Fax: pcheck@hanmail.net * This study was supported by the Korea Healthcare Technology Research and Development (R&D) Project of the Ministry of Health and Welfare Affairs grant HI15C1142. 께감소는녹내장성변화를시사하는대표적인소견중하나로, 빛간섭단층촬영을통한망막신경섬유층과시신경유두의분석은녹내장의초기진단뿐아니라녹내장성진행을좀더민감하게정량적으로평가할수있게해주었다. 1 급성폐쇄각녹내장에서망막신경섬유층의두께변화에대한이전연구에서급성기에망막신경섬유층두께의일시적증가를보였던경우도보고되었으며, 2,3 포도막염에의한녹내장에서도이러한역설적변화가보고된바있다. 4 저자들은최근의한증례에서개방각녹내장환자에서고안압상태에서망막신경섬유층두께의증가와이로인한녹내장성시신경유두손상의차폐를경험하여이를보고하고자한다. * Conflicts of Interest: The authors have no conflicts to disclose. c2017 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 1425

- 대한안과학회지 2017 년제 58 권제 12 호 - 증례보고 고혈압, 당뇨등의특이병력및가족력이없던 21세남자가일주일전부터발생한우안의시야흐림을주소로내원하였다. 안과적검사에서교정시력우안 0.5, 좌안 1.0으로측정되었으며, 골드만압평안압계로측정한안압이우안 60 mmhg, 좌안 15 mmhg로측정되었다.

5% timolol maleate fixed combination (Cosopt, Merck & Co, Inc., Whitehouse Station, NJ, USA), 0.15% brimonidine (Alphagan-P, Allergan Pharmaceuticals, Irvine, CA, USA), 0.

2 - 대한안과학회지 2017 년제 58 권제 12 호 - 증례보고 고혈압, 당뇨등의특이병력및가족력이없던 21세남자가일주일전부터발생한우안의시야흐림을주소로내원하였다. 안과적검사에서교정시력우안 0.5, 좌안 1.0으로측정되었으며, 골드만압평안압계로측정한안압이우안 60 mmhg, 좌안 15 mmhg로측정되었다. 세극등검사에서우안각막부종이관찰되었으나 Van Herick 법을이용한전방깊이측정상양안의중심전방깊이는중심각막두께의 4배, 주변부전방깊이는각막두께의 1/2로전방깊이는정상이었으며전방내염증을포함한이상소견은관찰되지않았다. 고안압에대해일차적으로 2% dorzolamide hydrochloride/0.5% timolol maleate fixed combination (Cosopt, Merck & Co, Inc., Whitehouse Station, NJ, USA), 0.15% brimonidine (Alphagan-P, Allergan Pharmaceuticals, Irvine, CA, USA), 0.005% latanoprost (Xalatan, Pfizer Inc., New York, NY, USA) 를점안하였고, 15% mannitol 정맥혈관주사및경구용탄산탈수효소억제제 (Acetazol, Hanlim Pharm Co., Seoul, Korea) 를복용하였다. 다음날시행한검사에서안압은 18 mmhg로측정되며각막부종이호전된모습을보였으며전방내염증이나각막후면침착물등의소견은없었고, 전방각경검사에서개방각상태이며특이소견이관찰되지않았다. 중심각막두께 (DGH 55 Pachymeter; DGH Technology Inc., Exton, PA, USA) 는우안 569 μm, 좌안 585 μm로측정되었다. 안저검사에서시신경유두및망막신경섬유층의녹내장성변화는관찰되지않았으나좌안에비해우안의시신경유두가충혈된모습을보였으며 (Fig. 1A, C), 자동시야검사 (Humphrey Field Analyzer II; Carl Zeiss A B C D Figure 1. Color and red-free fundus photographs of 21-year-old male who visited our clinic with ocular hypertension. On the initial fundus photographs, there was no prominent glaucomatous change of optic nerve head, butthe optic nerve was slightly congested (A, C). For the next two months, intraocular pressure (IOP) was maintained within normal range with IOP-lowering medication but fundus examination after two months showed increased cupping of the optic disc, superotemporal and inferotemporal side retinal nerve fiber layer defect (B, D, white arrows). 1426

GHT = glaucoma hemifield test; VFI = visual field index; M D = mean deviation; PSD = pattern standard deviation. A B Figure 3. Optical coherence tomography (OCT) of 21-year old male.

3 - 김동근 김정림 : 원발개방각녹내장에서 시신경유두부종 - A B Figure 2. Automated perimetery of the patient. At the initial visit, superonasal defect was observed without typical glaucomatous damage on the fundus examination (A). Such visual field defect persisted after 2 months (B). GHT = glaucoma hemifield test; VFI = visual field index; M D = mean deviation; PSD = pattern standard deviation. A B Figure 3. Optical coherence tomography (OCT) of 21-year old male. Initial OCT showed no neuroretinal rim thinning or retinal nerve fiber layer defect (A). However, inferotemporal rim thinning and retinal nerve fiber layer defect was observed after 2 months (B). ONH = optic nerve head; RNFL = retinal nerve fiber layer; OU = oculus unitas; OD = oculus dexter; OS = oculus sinister; C/D = cup/disc; TEMP = temporal; SUP = superior; NAS = nasal; INF = inferior; S = superior; N = nasal; I = inferior; T = temporal. 1427

4 - 대한안과학회지 2017 년제 58 권제 12 호 - Meditec, Dublin, CA, USA) 에서우안상비측의시야결손이관찰되었다 (Fig. 2A). 빛간섭단층촬영 (Cirrus OCT, Carl Zeiss Meditec, Dublin, CA, USA) 검사에서우안망막신경섬유층두께는모든영역에서정상범위내로측정되었으나좌안보다전반적으로두꺼운양상을보였고, 시야결손과대응되는망막신경섬유층두께감소는관찰되지않았다 (Fig. 3A). 원발개방각녹내장진단하 Dorzolamide/timolol fixed combination drug, brimonidine, latanoprost를지속적으로점안하며경과관찰하였으며안압하강제사용 4일후검사에서우안교정시력 1.0, 안압은 15 mmhg로측정되었고, 자동시야검사에서상비측시야결손이첫검사와유사하게관찰되었다. 2주, 1개월후의경과관찰에서도안압은 15 mmhg 이하로유지되었으며, 2개월후경과관찰에서우안교정시력은 0.8, 안압은 14 mmhg로측정되었으나자동시야검사에서상비측시야결손이지속적으로관찰되었다 (Fig. 2B). 또한안저검사에서우안시신경유두의충혈이감소되며함몰이증가된모습과상이측, 하이측의망막신경섬유층결손이관찰되었고 (Fig. 1B, D), 빛간섭단층촬영에서하이측망막신경섬유층의결손이관찰되었다 (Fig. 3B). 고찰 망막신경섬유층과시신경유두에대한평가는녹내장의진단과치료에있어가장중요한부분중하나이다. 공초점주사레이저검안경검사, 주사레이저편광측정법, 빛간섭단층촬영등의검사는이러한망막신경섬유층과시신경유두에대해좀더객관적이고정량적인평가를가능하게해주었다. 1 하지만이러한망막신경섬유층에대한정량적검사들의해석에있어서유의해야할점중하나로급성안압상승상태에서망막신경섬유층의두께증가가보고된바있다. Tsai et al 2 은단안급성폐쇄각녹내장환자를대상으로한전향적연구에서빛간섭단층촬영을이용하여망막신경섬유층두께변화를측정하였으며, 급성폐쇄각녹내장발생 1 주후반대안에비해평균및각사분면의망막신경섬유층두께가모두유의하게증가하였음을관찰하였다. 저자들은가능한원인으로급격한안압상승으로인한축삭운반차단으로시신경유두의부종이발생하고이로인해시신경유두주변망막신경섬유층의두께가증가한다는것을제시하였다. 5,6 Liu et al 3 은단안의급성폐쇄각녹내장환자와만성폐쇄각녹내장환자를대상으로한전향적연구에서 3일내, 2주일, 1개월, 3개월, 6개월의망막신경섬유층두께를비교하였다. 급성폐쇄각녹내장안의망막신경섬유층두께가 3일내에는반대안보다두꺼웠으며이후유의하게감소하는양 상을보이고, 2주일에서 1개월사이에반대안에비해얇아지며이후지속적으로얇아지는양상이관찰되었다. 하지만만성폐쇄각녹내장안에서는이러한시간에따른두께변화가관찰되지않았다. 이러한기전을급격한안압상승으로인한심한혈류감소, 허혈손상, 재관류손상, 축삭운반차단으로설명하였으며만성폐쇄각녹내장의경우중등도의안압상승이점진적으로일어나기때문에허혈손상을유발할정도가아닌관류압저하와그로인한축삭운반저하로급성폐쇄각녹내장과는다른변화양상을보인다고하였다. 안압상승과관련된동물실험들을살펴보면망막과망막신경절은시신경에가까울수록안압의영향을많이받고 7 안압상승으로인한시신경유두의초기변화소견으로사상판의변형과함께사상판앞신경조직의두께증가가보고된것이있으며 8 이는망막신경절세포내축삭운반차단과신경조직의부종때문으로설명하였다 원발개방각녹내장은병변의진행이서서히일어나며병의말기까지증상이없기때문에환자가발병을느끼지못하는것이일반적이나예외적으로각막부종, 눈의불편등을유발하는심한안압상승을경험하는젊은환자의경우도보고되고있다. 12,13 본증례의경우 1주일전시야흐림증상이있었고초진시전방각은열려있었으며안압은 60 mmhg로측정되었다. 다음날안압이정상화된후시행한안저검사및빛간섭단층촬영에서망막신경섬유층이나시신경유두의녹내장성변화가관찰되지않았지만상비측의시야결손이관찰되었던것이특이할점이다. 4일후시행한시야검사에서도상비측의시야결손이보였고, 녹내장성손상이외에시야장애를일으킬수있는다른원인을찾을수없었다. 2 개월후초진시관찰된우안시신경유두의비대칭적충혈과부종이감소되었고상비측의시야결손에상응하는하이측의망막신경섬유층결손이나타났다. 저자들은초진시시야결손과일치하지않는이러한해부학적소견을고안압으로인한축삭운반차단으로유발된시신경유두와망막신경섬유층의부종으로이미존재하던녹내장성시신경유두손상이차폐된결과로판단하였다. Aung et al 14 과 Chew et al 15 은단안의급성폐쇄각녹내장환자를대상으로한전향적연구에서급성폐쇄각녹내장발생후첫방문시에는양안의평균망막신경섬유층두께차이를보이지않았고, 2-4개월후급성폐쇄각녹내장안의평균망막신경섬유층두께가반대안에비해유의한감소를보인다고보고하였다. 급성기망막섬유층두께에있어서위의연구결과와차이가있어보이지만망막신경섬유층두께측정시기가다르므로결과해석시이점을고려해야한다. 또한본증례에서 2개월후관찰된망막신경섬유층결손은급격한안압상승후발생하는망막신경섬유층두 1428

5 - 김동근 김정림 : 원발개방각녹내장에서시신경유두부종 - 께감소와도관련이있을것으로생각된다. Asrani et al 4 은 3명의포도막염성녹내장환자에서역설적인망막신경섬유층두께변화를보고하였다. 양안의베쳇범포도막염환자 1명과단안의특발성앞포도막염환자 2명에서포도막염의활성과함께 32 mmhg에서 60 mmhg의고안압이동반되었으며, 당시시행한빛간섭단층촬영에서정상범위내의망막신경섬유층두께가관찰되었다. 하지만이후포도막염과고안압이지속적으로조절되었음에도 6개월에서 1년후시행한빛간섭단층촬영에서망막신경섬유층이얇아지는것이관찰되었고, 저자들은이러한변화의원인을녹내장의진행에의한것이아니라포도막염으로발생한염증성변화에의한초기의망막신경섬유층부종이감소한것으로설명하였다. 본증례에서각막부종감소후각막후면침착물등이관찰되지않은것으로보아단안에발생한이차녹내장은아니라고생각되지만 2개월후안압이조절됨에도망막신경섬유층결손이발생한것은급격한안압상승으로인한녹내장성손상과함께망막신경섬유층부종이감소하면서기존의시야결손에상응하는녹내장성손상이나타난것으로생각된다. 본증례처럼폐쇄각이나포도막염의소견이관찰되지않은원발개방각녹내장에서도축삭운반차단이일어날정도의심한고안압소견이있었던경우에서는시신경유두와망막신경섬유층의부종이발생할가능성이있으며이로인하여녹내장성시신경손상이차폐될가능성또한염두에두고안압하강치료와함께잦은경과관찰이필요할것으로판단된다. 또한안압조절이잘되고있음에도망막신경섬유층두께의감소와시신경유두함몰이증가하는경우는이전에급격한안압상승으로발생한망막신경섬유층의부종이감소하면서이러한현상이생길가능성도있으므로감별이필요할것으로사료된다. REFERENCES 1) Anton A, Moreno-Montañes J, Blázquez F, et al. Usefulness of optical coherence tomography parameters of the optic disc and the retinal nerve fiber layer to differentiate glaucomatous, ocular hypertensive, and normal eyes. J Glaucoma 2007;16:1-8. 2) Tsai JC, Lin PW, Teng MC, Lai IC. Longitudinal changes in retinal nerve fiber layer thickness after acute primary angle closure measured with optical coherence tomography. Invest Ophthalmol Vis Sci 2007;48: ) Liu X, Li M, Zhong YM, et al. Damage patterns of retinal nerve fiber layer in acute and chronic intraocular pressure elevation in primary angle closure glaucoma. Int J Ophthalmol 2010;3: ) Asrani S, Moore DB, Jaffe GJ. Paradoxical changes of retinal nerve fiber layer thickness in uveitic glaucoma. JAMA Ophthalmol 2014;132: ) Quigley HA, Guy J, Anderson DR. Blockade of rapid axonal transport. Effect of intraocular pressure elevation in primate optic nerve. Arch Ophthalmol 1979;97: ) Tso MO, Fine BS. Electron microscopic study of human papilledema. Am J Ophthalmol 1976;82: ) Fortune B, Yang H, Strouthidis NG, et al. The effect of acute intraocular pressure elevation on peripapillary retinal thickness, retinal nerve fiber layer thickness, and retardance. Invest Ophthalmol Vis Sci 2009;50: ) Yang H, Downs JC, Bellezza A, et al. 3-D histomorphometry of the normal and early glaucomatous monkey optic nerve head: prelaminar neural tissues and cupping. Invest Ophthalmol Vis Sci 2007;48: ) Anderson DR, Hendrickson A. Effect of intraocular pressure on rapid axoplasmic transport in monkey optic nerve. Invest Ophthalmol 1974;13: ) Quigley HA, Addicks EM. Chronic experimental glaucoma in primates. II. Effect of extended intraocular pressure elevation on optic nerve head and axonal transport. Invest Ophthalmol Vis Sci 1980;19: ) Minckler DS, Bunt AH, Klock IB. Radioautographic and cytochemical ultrastructural studies of axoplasmic transport in the monkey optic nerve head. Invest Ophthalmol Vis Sci 1978;17: ) Gupta V, Gupta S, Dhawan M, et al. Extent of asymmetry and unilaterality among juvenile onset primary open angle glaucoma patients. Clin Exp Ophthalmol 2011;39: ) Stanmper RL, Drake MV. Becker-Shaffer's Diagnosis and Therapy of the Glaucomas, 7th ed. St Louis: The CV Mosby Company, 1999; ) Aung T, Husain R, Gazzard G, et al. Changes in retinal nerve fiber layer thickness after acute primary angle closure. Ophthalmology 2004;111: ) Chew SS, Vasudevan S, Patel HY, et al. Acute primary angle closure attack does not cause an increased cup-to-disc ratio. Ophthalmology 2011;118:

6 - 대한안과학회지 2017 년제 58 권제 12 호 - = 국문초록 = 원발개방각녹내장환자에서발생한시신경유두부종 1 예 목적 : 원발개방각녹내장환자에서급성기시신경유두의부종으로인해녹내장성시신경유두손상이차폐되었던증례를경험하여보고하고자한다. 증례요약 : 특이병력및가족력이없던 21 세남자가일주일전부터발생한우안의시야흐림을주소로내원하였다. 골드만압평안압계로측정한우안안압은 60 mmhg 로측정되었으며세극등검사에서심한각막부종이외의전안부특이소견은관찰되지않았다. 최대약물요법을시행하여안압을하강시킨다음날추가적검사를시행하였고, 안저검사및빛간섭단층촬영에서시신경유두및망막신경섬유층의녹내장성변화는관찰되지않았으나좌안에비해우안의시신경유두가충혈된모습을보였고, 자동시야검사에서우안상비측의시야결손이관찰되었다. 이후점안안압하강제를사용하며경과관찰하여안압은정상범위내로유지되었으나 2 개월후시행한자동시야검사에서우안상비측시야결손은지속되었으며, 안저검사및빛간섭단층촬영에서하비측의망막신경섬유층결손이관찰되었다. < 대한안과학회지 2017;58(12): > 1430

< D BAFBB0A3BCB7B4DCC3FEC3D4BFB5C0BB20C0CCBFEBC7D120C1A4BBF32C20B3ECB3BBC0E5C0C7C1F52C20C3CAB1E220B3ECB3BBC0E5BFA1BCADC0C720BAAFB

< D BAFBB0A3BCB7B4DCC3FEC3D4BFB5C0BB20C0CCBFEBC7D120C1A4BBF32C20B3ECB3BBC0E5C0C7C1F52C20C3CAB1E220B3ECB3BBC0E5BFA1BCADC0C720BAAFB 빛간섭단층촬영을이용한정상, 녹내장의증, 초기녹내장에서의변수비교분석 1379 1380 Table 1. Criteria for each subject Enrolled group Criteria All subjects Best corrected visual acuity >0.5 Refractive error