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1 대한안과학회지 2014 년제 55 권제 9 호 J Korean Ophthalmol Soc 2014;55(9): pissn: eissn: Case Report 단안의비측반맹으로발현된내경동맥류 Internal Carotid Artery Aneurysm Presenting with Unilateral Nasal Hemianopsia 김경남 김창식 이연희 이성복 Kyoung Nam Kim, MD, Chang Sik Kim, MD, PhD, Yeon Hee Lee, MD, PhD, Sung Bok Lee, MD, PhD 충남대학교의학전문대학원안과학교실 Department of Ophthalmology, Chungnam National University School of Medicine, Daejeon, Korea Purpose: To report a case of unilateral nasal hemianopsia caused by a large internal carotid artery aneurysm. Case summary: A 56-year-old female presented with large cupping in the left optic nerve head detected incidentally during a routine check-up. She had no underlying systemic disease except hypertension. The best corrected visual acuity was 20/20 in both eyes and a slit-lamp examination showed no abnormal findings. Ophthalmoscopy showed cup/disc ratios of 0.6 in the right eye and 0.75 in the left eye. Relative afferent papillary defect or color vision defect was not observed. A Humphrey visual-field test indicated unilateral nasal hemianopsia in the left eye. Brain CT and angiography revealed a large 2.2-cm aneurysm on the left internal carotid artery. Conclusions: Internal carotid artery aneurysm should be considered as a possible cause of unilateral nasal hemianopsia in patients without intraocular lesion. J Korean Ophthalmol Soc 2014;55(9): Key Words: Glaucoma suspect, Internal carotid artery aneurysm, Optic disc cupping, Relative afferent pupillary defect, Unilateral nasal hemianopsia 수직또는수평경선을기준으로시야의절반이소실된경우를반맹 (hemianopsia) 이라고하며안구이후의병변에의한반맹은수직경선을따른다. 양안에서수직경선을따르는반맹이나타나는경우에는주로양이측반맹 (bitemporal hemianopsia) 이나동측반맹 (homonymous hemianopsia) 의형 Received: Revised: Accepted: Address reprint requests to Sung Bok Lee, MD, PhD Department of Ophthalmology, Chungnam National University Hospital, #282 Munhwa-ro, Jung-gu, Daejeon , Korea Tel: , Fax: sblee@cnu.ac.kr * This study was presented as a poster at the 111th Annual Meeting of the Korean Ophthalmological Society 태로나타나며, 양이측반맹은주로시신경교차부의뇌하수체선종, 두개인두종, 수막종등에의해발생하고동측반맹은시신경교차부이후의시삭, 가쪽무릎체, 시방선, 대뇌후두엽에병변이있는경우발생한다. 시신경교차전이나앞시신경교차에병변이있는경우에는단안에서수직경선을따르는반맹이나타날수있는데, 1 단안의비측반맹은매우드물게보고되어있으며, 1-5 특히양안의시력이정상인경우에는환자가단안의비측시야손상을인지하기어려워진단되기가더욱힘들것으로예상할수있다. 1-3 저자들은신경학적증상이동반되지않고시력이보존되어있는단안의비측반맹환자에서내경동맥류를진단하였으며, 국내에는아직까지단안의비측반맹으로발현된병증이보고된바없어이를보고하고자한다. c2014 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. 1406

2 - 김경남 외 : 단안 비측 반맹으로 발현된 내경동맥류 - A B Figure 1. Fundus photographs show cup/disc ratios of 0.6 in the right eye (A) and 0.75 in the left eye (B). A B Figure 2. Humphrey visual field test demonstrating normal visual field in the right eye (A) and nasal hemianopsia on pattern-deviation plot in the left eye (B). 른 병력은 없었으며 안과적, 신경학적 증상도 없었다. 양안 증례보고 의 최대교정시력은 20/20이었고, 안압은 16 mmhg였다. 세 극등현미경검사에서 양안 모두 특이소견은 없었으며 안저 56세 여자환자가 건강검진에서 우연히 좌안의 시신경유 검사에서 시신경유두함몰비는 우안이 0.6, 좌안이 0.75였다 두함몰비 증가가 발견되어 녹내장의증으로 전원되었다. 10 (Fig. 1). 양안 모두 상대구심동공운동장애는 없었고 이시하 년 전부터 고혈압에 대한 약물치료를 받고 있었고 이외 다 라 색각검사에서는 오답 없이 정상이었다. 험프리시야검사 1407

3 - 대한안과학회지 2014년 제 55 권 제 9 호 - Figure 3. Optical coherence tomography demonstrating a slight but significant superotemporal and inferotemporal retinal nerve fiber layer thinning in the left eye relative to the right eye. ONH = optic nerve head; RNFL = retinal nerve fiber layer; OD = right; OS = left; C/D = cup/disc ratio; TEM P (T) = temporal; SUP (S) = superior; NAS (N) = nasal; INF (I) = inferior. 에서 우안은 시야결손이 없었고 좌안은 전반적인 감도저하 보였다(Fig. 2). 빛간섭단층촬영검사에서 상이측과 하이측 가 있으면서 불완전하지만 수직경선을 따르는 비측 반맹을 망막신경섬유층의 두께는 좌안이 우안보다 얇았으나 양안 1408

4 - 김경남외 : 단안비측반맹으로발현된내경동맥류 - A B C Figure 4. Brain CT images of axial section (A) and coronal section (B) shows 2.2-cm large aneurysm on the left internal carotid artery (black arrow) compressing the lateral aspect of the left optic nerve in front of the optic chiasm. Brain CT angiography (C) demonstrates large aneurysm located on the paraclinoid portion of the left internal carotid artery (white arrow). 모두정상범위안에있었고이측망막신경섬유층의두께는좌안이유의하게얇아진것으로나타났다 (Fig. 3). 다시시행한시야검사에서동일한양상의시야결손이확인되었고, 뇌컴퓨터단층촬영및혈관조영술을시행하였다. 지름 2.2 cm 크기의좌측침상돌기주변내경동맥류가시신경교차부바로앞쪽에서좌측시신경을압박하고있었다 (Fig. 4). 신경외과로치료를의뢰하였으며뇌동맥류결찰술을받았다. 고찰 단안의비측시야손상은임상에서흔히접할수있는데대부분이반맹은아니며주로안내병변에의해발생한다. 1,6,7 안구이후의원인에의한단안의비측반맹은매우드물며나비뼈날개의수막종, 2 시신경관내수막종, 4 뇌하수체종양, 5 침상돌기주위내경동맥류 1,3 에의한경우가보고된바있다. Cox et al 1 과 Stacy et al 2 의보고에서는본증례와유사하게환자의시력은보존되어있었으나상대동공구심운동장애가동반되어있었다. 시야결손이있는환자에서망막의광범위한이상이없으면서상대동공구심운동장애가있으면기능성시야장애를배제할수있고시신경염, 압박시신경병증, 시신경허혈, 녹내장등의시신경병증을예상할수있다. 8 Levin et al 9 은두개강내종괴에인한압박시신경병증이있었던 3명의환자를대상으로사후에조직학적연구를시행한결과반대편시신경에비해축삭이 1/2-1/3로감소하면상대동공구심운동장애를일으킬수있다는결과를얻었다. 최근 Chew et al 10 과 Tatham et al 11 은빛간섭단층촬영검사에서측정된망막신경섬유층의두께가양안사이에각각 14.6 μm, 20.0 μm 이상차이가있으면상대동공구심운동장애를일으킬수있 다고하였다. 본증례의환자는반복된교대불빛검사에서상대동공구심운동장애를보이지않았는데양안사이의미미한차이를발견하지못했을가능성을완전히배제할수는없으나, 빛간섭단층촬영검사에서양안의망막신경섬유층의두께차이가 13.0 μm인점을고려하면상대동공구심운동장애가실제로없었다고판단된다. 시신경유두함몰비의증가는녹내장의대표적인소견중하나이며이에상응하는비측시야손상이동반된다면더욱녹내장을의심할수있으나, 시신경을압박하는안와나두개내의병변또한시신경유두함몰비의증가를유발할수있다. 12,13 본증례의환자는좌측의시신경유두함몰비가우측보다증가되어있으면서상이측과하이측의망막신경섬유층이반대안보다상대적으로얇았고이위치에상응하는비측시야손상을보였다. 또한중심시력이보존되어있어녹내장을의심할수도있었다. 하지만시야장애의정도가시신경과망막신경섬유층의손상정도보다훨씬더심각했으며불완전하지만수직경선을따르는양상을보여안구뒤쪽의병변을의심할수있었다. 최근빛간섭단층촬영검사를이용하여시행된연구들에의하면녹내장환자에서시야손상이시작되는망막신경섬유층두께의티핑포인트 (tipping point) 는평균두께는 μm, 상측두께는 μm, 하측두께는 μm이다. 14,15 본증례의환자는좌안의망막신경섬유층이우안에비해상대적으로얇았으나티핑포인트에는미치지않았다. 뇌동맥류는내경동맥과척추기저동맥에서주로발생하며아직까지정확한원인은뚜렷하게밝혀지지않았으나동맥벽의변성, 탄력섬유의결손, 고혈압등이영향을미치는것으로알려졌다. 16,17 30대에서 60대사이에호발하며남성보다여성에서 1.5배발생빈도가높다. 18 또한크기에 1409

5 - 대한안과학회지 2014 년제 55 권제 9 호 - 따라차이가있으나매년 % 정도에서파열될가능성이있고, 크기가클수록그위험이더높아지며파열시사망률이 60% 에달한다. 19 뇌동맥류는대개지주막하출혈에의한두통, 구역, 구토, 의식저하나혼수또는동안신경압박에의한눈꺼풀처짐, 복시등의증상으로발현되지만일부에서는파열되지않고서서히자라시각경로를압박함으로써발현되는시력저하와시야결손이환자의첫증상으로나타날수있다. 20 대부분직경이 10 mm 이하이지만 15 mm 이상의대형동맥류도발생할수있으며, 그중에서도직경이 25 mm 이상인거대동맥류는뇌종양과같은압박증상을잘동반한다. 20 본증례에서는지름 22 mm의대형동맥류가좌측침상돌기주변내경동맥에서발생하여시신경교차부바로앞쪽에서좌측시신경의바깥쪽을압박하여좌안의비측반맹을유발하였다. 이부위는시신경교차부에인접해있는특성으로인해내경동맥류의크기와모양에따라다양한정도의시야결손및시력저하의증상을유발할수있으며, 21,22 증례의환자에서내경동맥류가파열되지않고더진행하여시신경교차부를누를정도까지커지면좌안의시력저하와전체시야손상을유발하고우안에도시야결손이나타날수있다. 결론적으로단안의비측시야장애가있는환자에서시력이보존되어있고상대동공구심운동장애나색각이상등의소견이동반되어있지않더라도면밀한안과적검사를시행하여비측시야장애의더흔한원인인안내질환이배제되면뇌경동맥류의초기증상일수있음을주지하여조기에진단할수있어야할것으로생각한다. REFERENCES 1) Cox TA, Corbett JJ, Thompson HS, Kassell NF. Unilateral nasal hemianopia as a sign of intracranial optic nerve compression. Am J Ophthalmol 1981;92: ) Stacy RC, Jakobiec FA, Lessell S, Cestari DM. Monocular nasal hemianopia from atypical sphenoid wing meningioma. J Neuroophthalmol 2010;30: ) AMYOT R. [Rupture of an aneurysm of the internal carotid; unilateral nasal hemianopsia; crossed hemiplegia caused by carotid spasm]. Union Med Can 1959;88: ) Huber A. Eye signs and symptoms in brain tumors. St. Louis: C. V. Mosby Co., 1976;266. 5) Meadows SP. Unusual clinical features and modes of presentation in pituitary adenoma, including pituitary apoplexy. In: Smith JL, ed. Neuroophthalmology. St. Louis: C.V. Mosby Co., 1968; v ) Rahman I, Nambiar A, Spencer AF. Unilateral nasal hemianopsia secondary to posterior subcapsular cataract. Br J Ophthalmol 2003;87: ) Karp CL, Fazio JR. Traumatic cataract presenting with unilateral nasal hemianopsia. J Cataract Refract Surg 1999;25: ) Chang BL. Neuroophthalmology. Seoul: Iljokak, 2004;128. 9) Levin PS, Newman SA, Quigley HA, Miller NR. A clinicopathologic study of optic neuropathies associated with intracranial mass lesions with quantification of remaining axons. Am J Ophthalmol 1983;95: ) Chew SS, Cunnningham WJ, Gamble GD, Danesh-Meyer HV. Retinal nerve fiber layer loss in glaucoma patients with a relative afferent pupillary defect. Invest Ophthalmol Vis Sci 2010;51: ) Tatham AJ, Meira-Freitas D, Weinreb RN, et al. Estimation of retinal ganglion cell loss in glaucomatous eyes with a relative afferent pupillary defect. Invest Ophthalmol Vis Sci 2014;55: ) Greenfield DS, Siatkowski RM, Glaser JS, et al. The cupped disc. Who needs neuroimaging? Ophthalmology 1998;105: ) Bianchi-Marzoli S, Rizzo JF 3rd, Brancato R, Lessell S. Quantitative analysis of optic disc cupping in compressive optic neuropathy. Ophthalmology 1995;102: ) Alasil T, Wang K, Yu F, et al. Correlation of retinal nerve fiber layer thickness and visual fields in glaucoma: a broken stick model. Am J Ophthalmol 2014;157: ) Wollstein G, Kagemann L, Bilonick RA, et al. Retinal nerve fibre layer and visual function loss in glaucoma: the tipping point. Br J Ophthalmol 2012;96: ) Artmann H, Vonofakos D, Müller H, Grau H. Neuroradiologic and neuropathologic findings with growing giant intracranial aneurysm. Review of the literature. Surg Neurol 1984;21: ) Horowitz MB, Yonas H, Jungreis C, Hung TK. Management of a giant middle cerebral artery fusiform serpentine aneurysm with distal clip application and retrograde thrombosis: case report and review of the literature. Surg Neurol 1994;41: ) Brisman JL, Song JK, Newell DW. Cerebral aneurysms. N Engl J Med 2006;355: ) Dominick DiMaio, Vincent JM DiMaio. Forensic Pathology, 2nd ed. Florida: CRC Press, 2001;61. 20) Besada E, Fisher JP. Absent relative afferent pupillary defect in an asymptomatic case of lateral chiasmal syndrome from cerebral aneurysm. Optom Vis Sci 2001;78: ) Peiris JB, Ross Russell RW. Giant aneurysms of the carotid system presenting as visual field defect. J Neurol Neurosurg Psychiatry 1980;43: ) Park JY, Koo NK. A giant unruptured aneurysm of distal internal carotid artery presenting with compressive optic neuropathy. J Korean Ophthalmol Soc 2012;53:

6 - 김경남외 : 단안비측반맹으로발현된내경동맥류 - = 국문초록 = 단안의비측반맹으로발현된내경동맥류 목적 : 단안의비측반맹을보인환자에서내경동맥류를진단하여이를보고하고자한다. 증례요약 : 고혈압이외의다른병력이없는 56 세여자환자가건강검진에서우연히발견된좌안의시신경유두함몰비증가를주소로내원하였다. 최대교정시력은양안 20/20 이었고, 세극등현미경검사에서특이소견은없었으며시신경유두함몰비는우안이 0.6, 좌안이 0.75 였다. 양안모두상대구심동공운동장애나색각이상은없었다. 험프리시야검사에서우안은명확한시야결손이없었고좌안은수직경선을따르는비측반맹이있었다. 뇌컴퓨터단층촬영및혈관조영술에서지름 2.2 cm 크기의내경동맥류가확인되었다. 결론 : 단안의비측시야장애가있는환자에서안내질환이배제되면내경동맥류가원인일가능성을고려해야하겠다. < 대한안과학회지 2014;55(9): > 1411

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