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대한안과학회지 2018 년제 59 권제 4 호 J Korean Ophthalmol Soc 2018;59(4):388-392 ISSN 0378-6471 (Print) ISSN 2092-9374 (Online) https://doi.org/10.3341/jkos.2018.59.4.388 Case Report 눈분절속목동맥자루에의한가돌림신경마비 1 예 Abducens Nerve Palsy Caused by the Ophthalmic Segment of an Internal Carotid Artery Aneurysm 김인혜 1 김종훈 2 김원제 1 Inhye Kim, MD 1, Jong Hoon Kim, MD 2, Won Jae Kim, MD 1 영남대학교의과대학안과학교실 1, 영남대학교의과대학신경외과학교실 2 Department of Ophthalmology, Yeungnam University College of Medicine 1, Daegu, Korea Department of Neurosurgery, Yeungnam University College of Medicine 2, Daegu, Korea Purpose: To report a case of isolated abducens nerve palsy caused by the ophthalmic segment of an internal carotid artery (ICA) aneurysm which improved after endovascular coil trapping. Case summary: A 59-year-old female visited the ophthalmology department for a sudden onset of horizontal diplopia for 10 days. The best corrected visual acuity was 20/20 in both eyes. The pupils showed normal response to light and near stimulation in both eyes. The extraocular examination showed 35 prism diopters left esotropia at primary gaze and 4 abduction limitation of the left eye. The patient suffered intermittent headaches in the left temporal area and left retrobulbar pain for 1 month. Magnetic resonance imaging with magnetic resonance angiography of the brain was performed. A focal protruding lesion of the left ICA suggested an aneurysm. The patient consulted with the neurosurgery department. The left ophthalmic segment of the ICA aneurysm was confirmed by transfemoral cerebral angiography and treated with coil placement and the patient showed gradual improvement after the procedure. Three months after the procedure there was no diplopia. The patient showed orthotropia at primary gaze without abduction limitation. Conclusions: Isolated abducens nerve palsy can be caused by the ophthalmic segment of an ICA aneurysm, which should be considered in the differential diagnosis of ocular motility disorders. The disorder improved with coil replacement treatment. Differential diagnosis as a cause of abducens nerve palsy is important for prompt and appropriate treatment. Neuroimaging should be considered in patients with isolated abducens nerve palsy with a non-ischemic origin. J Korean Ophthalmol Soc 2018;59(4):388-392 Keywords: Abducens nerve palsy, Aneurysm, Diplopia, Internal carotid artery 가돌림신경 (abducens nerve) 은뇌줄기 (brain stem) 에서거미막밑공간 (subarachnoid space) 으로나와해면정맥굴 (cavernous Received: 2017. 11. 9. Revised: 2017. 12. 5. Accepted: 2018. 3. 22. Address reprint requests to Won Jae Kim, MD Department of Ophthalmology, Yeungnam University Hospital, #170 Hyeonchung-ro, Nam-gu, Daegu 42415, Korea Tel: 82-53-620-4191, Fax: 82-53-626-5936 E-mail: eyekwj@ynu.ac.kr * Conflicts of Interest: The authors have no conflicts to disclose. sinus) 을통과하여눈확 (orbit) 으로들어간다. 1 뇌줄기에서눈확으로이어지는가돌림신경의주행경로중경색 (infarction), 외상 (trauma), 출혈 (hemorrhage), 압박 (compression), 염증 (inflammation) 등의여러원인에의해가돌림신경마비가발생할수있다. 1-3 그중속목동맥 (internal carotid artery) 의눈분절동맥자루 (ophthalmic segment internal carotid artery aneurysm) 는가돌림신경마비를일으키는흔한원인은아니다. 3 저자들은갑자기발생한가돌림신경마비에의한수평복시환자에서속목동맥의눈분절동맥자루를진단하였고, 코일색전술 c2018 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 388

- 김인혜외 : 눈분절동맥자루에의한가돌림신경마비 - 을통한좋은경과를확인하였기에이를보고하고자한다. 증례보고 59세여자가 10일전갑자기발생한수평두눈복시로내원하였다. 이전에당뇨, 고혈압, 고지질혈증등의전신질환또는안과질환의과거력은없었다. 복시증상은갑자기시작되었으며, 일중변화를동반하지않았다. 결막부종이나시력저하는없었다. 1달전부터이전에없었던좌측측두부의간헐적인두통이있었고, 눈뒤통증은있으나눈움직임으로증상이심해지지는않았다. 시력은양안 20/20이었다. 얼굴이상감각이나통증은없었다. 눈꺼풀처짐이나뒤당김등의눈꺼풀이상소견은없었다. 동공반응검사에서빛과근접반사모두정상소견을보였다. 안구운동검사에서정면주시시 35프리즘디옵터 (prism diopters, PD) 의내사시와좌안 -4 의가쪽운동장애소견을보였다 (Fig. 1). 세극등검사와안저검사에서이상소견은없었다. 타의료기관에서뇌자기공명영상 (magnetic resonance imaging, MRI) 과자기공명혈관조영술 (magnetic resonance angiography) 을시행받았고, 속목동맥의눈분절에서돌출병변이의심되었다. 속목동맥의눈분 절동맥자루가의심되어, 환자는확진과치료를위해신경외과로의뢰되었다. 신경외과에서뇌혈관조영술을통하여속목동맥의눈분절동맥자루를진단받았다. 동맥자루는주머니모양 (saccular aneurysm) 이었으며동맥자루의목은 2.80 mm, 높이는 3.33 mm, 넓이는 3.31 mm이고, 방향은뒤쪽, 아래쪽으로해면정맥굴위에서압박하는양상이었다 (Fig. 2A). 오른쪽넙다리동맥 (femoral artery) 을경유하여왼쪽속목동맥에가이드도관을거치한후, 두개의미세도관기술법 (double catheter technique) 으로코일색전술을시행하였다 (Fig. 2B). 혈관내수술후동맥자루안으로조영제가주입되지않음을확인하였으며, 환자에게특별한신경학적증상이없음을확인한후혈관내수술은종료하였다 (Fig. 2C, 2D). 시술후두통과눈뒤통증은완화되었다. 시술 3개월후, 환자의복시는호전을보였다. 안구운동검사에서정면주시시정위및가쪽운동제한소견은없었다 (Fig. 3). 고찰 성인에서가돌림신경마비의가장흔한원인은허혈이며, 속목동맥의눈분절동맥자루는가돌림신경마비의흔한원 Figure 1. Images of the patient in nine diagnostic position of gaze at initial visit. The patient showed esotropia at primary gaze with abduction limitation of the left eye. A B C D Figure 2. Images of cerebral digital subtraction angiography (DSA) during treatment. (A) Cerebral DSA showed ophthalmic segment aneurysm of left internal carotid artery (neck: 2.80 mm, height: 3.33 mm, width: 3.31 mm, posterior-inferior direction). (B) Cerebral DSA showed two microcatheters were located in the aneurysm. (C, D) Cerebral DSA showed coil embolization was finished with no abnormal findings. 389

- 대한안과학회지 2018 년제 59 권제 4 호 - Figure 3. Images of the patient in nine diagnostic position of gaze at 3 months after the treatment. The patient showed stable ocular alignment without diplopia. 인은아니다. 3 속목동맥자루는그위치에따라시신경이나눈돌림신경 (oculomotor nerve), 도르래신경 (trochlear nerve), 가돌림신경에영향을주어시력저하나안구운동마비에의한복시등의안과적증상을유발할수있다. 4-7 해면정맥굴은다양한크기의정맥이분할과융합된얼기 (plexus) 공간내에속목동맥, 눈돌림신경, 도르래신경, 가돌림신경, 교감신경 (sympathetic carotid plexus), 삼차신경 (trigeminal nerve) 이밀집해있는해부학적특징을가지고있다. 1 속목동맥의눈분절동맥자루가해면정맥굴을압박할때뇌신경마비가발생할가능성이있고, 해면정맥굴의구조적인특징으로인해속목동맥자루의대표적인증상인복시, 눈주위통증, 두통등이발생하는것이다. 4,8,9 가돌림신경은뇌줄기에서나와서눈확으로들어가는주행경로가길며해면정맥굴을통과할때다른뇌신경과달리속목동맥가까이위치하고뇌막가쪽에부착되어있지않다. 1,3 이러한이유로가돌림신경은목동맥해면굴샛길 (carotid cavernous fistula), 속목동맥박리, 속목동맥자루등의속목동맥혈관질환에의한영향을쉽게받을수있다. 1,3 하지만이전에국내에서속목동맥의눈분절동맥자루에의해갑자기발생한가돌림신경마비와이를코일색전술을통해좋은치료경과를얻었던보고는없었다. 또한이증례에서는속목동맥의눈분절동맥자루에의한가돌림신경마비가급성으로통증을동반하여발생한것이흥미롭다. 동맥자루는대개서서히진행하는안구운동마비를유발하고, 통증을동반할수있다. 3 본증례와같이급성으로통증을동반하는경우는이전에있던동맥자루의급격한팽창으로인한뇌신경의직접적인압박기전외에도, 동맥자루가주위혈관을압박하여발생하는이차적인허혈기전에의한발생가능성도생각할수있을것이다. 10 Nguyen et al 11 은가돌림신경마비의자연회복과재발의형태로나타난해면정맥굴속목동맥자루를보고하기도했는데, 이증례도뇌동맥자루의직접적인압박외에추가적인허혈기전에의한 뇌신경마비와회복을생각할수있다. 이증례와같이가돌림신경마비환자의치료방향을결정하기위해서는정확한원인진단이중요하며, 감별진단을위한검사에서 MRI 등의뇌영상검사를언제시행할지결정하는것이중요할것이다. 일반적으로허혈외의원인에의한마비가의심되는경우에는바로 MRI를시행하고, 허혈에의한것으로생각되면 3개월까지회복여부를확인후검사를시행하는것이제시되고있다. 1,12,13 허혈에의한가돌림신경마비를진단할임상진단기준으로는다음의항목을모두만족하는것이제시되고있다. 13 첫째, 발생나이에서는 40대나그이상의경우. 둘째, 과거력에서당뇨나고혈압, 흡연등혈관병증의위험요소를가지며, 암, 혈관염또는자가면역질환의과거력은없을것. 셋째, 갑자기발생한복시이며, 하루변이 (diurnal variation) 를동반하지않고, 자연회복이발생할때까지안정적인경과를보일것. 넷째, 지속적인눈통증, 반얼굴 (hemifacial) 통증, 반머리 (hemicranial) 통증을동반하지않을것. 다섯째, 원인불명의난청, 귀울림 (tinnitus), 가돌림신경마비동측의얼굴쇠약 (facial weakness) 이없을것. 여섯째, 다른신경학적증상을동반하지않을것. 일곱째, 50대이상에서거대세포동맥염 (giant cell arteritis) 의증상이없을것. 하지만저자들은이러한임상지침외에 MRI 시행여부는환자진료환경이나의료진의임상경험에따라서달라질수있다고생각되며, 이증례의경우가돌림신경마비외에다른신경학적증상으로동반하지않았고, 나이가고령이아니며, 과거력에서당뇨, 고혈압등의혈관병증의위험요소를가지고있지않았던경우로바로 MRI 검사를시행한것은적절하였다고생각된다. 눈분절속목동맥자루는해면정맥굴속목동맥자루와달리파열되면거미막밑출혈로진행할수있다. 14 뇌동맥자루의치료는보존적치료, 혈관내치료, 수술적치료로나누어볼수있다. 9,14,15 이중혈관내치료의경우, 여러연구들에서치료받은환자들에서이전의통증이나복시증상의 390

- 김인혜외 : 눈분절동맥자루에의한가돌림신경마비 - 호전을확인할수있다. 9,15 하지만 Stiebel-Kalish et al 8 은치료후초기통증은호전을보였지만, 복시는치료후에통계적으로의미있는호전을보이지는않았으며, 치료받은군에서신경학적또는시각적합병증이치료받지않은군보다높았다고보고하기도했다. 눈분절속목동맥자루는해부학적위치상, 혈관내치료로접근과치료가어려울수있다. 14 하지만이에대한혈관내치료와수술적치료의결과와임상증상의호전에대한전향적인연구는없어, 앞으로뇌동맥자루의형태와임상양상, 치료방법에따른결과에대한추가적인연구가필요할것으로생각된다. 결론적으로급성으로발생한수평복시환자에서속목동맥의눈분절동맥자루는가돌림신경마비의원인으로고려하여야한다. 그리고코일색전술을통해좋은결과를얻을수있음을확인하였다. 가돌림신경마비의치료방향을결정하기위해서는정확한원인감별이중요하며, 허혈이외의원인이의심되는경우는감별진단을위해적극적인뇌영상검사의시행을고려해야할것이다. REFERENCES 1) Miller NR, Subramanian PS, Patel VR. Walsh and Hoyt's Clinical Neuro-Ophthalmology: the essentials, 3rd ed. Philadelphia: Wolters Kluwer, 2016; 361-9. 2) Jeon C, Sa HS, Oh SY. Causes and natural course of the sixth cranial nerve palsy. J Korean Ophthalmol Soc 2006;47:1776-80. 3) Kline LB, Foroozan R. Neuro-Ophthalmology Review Manual, 7th ed. Thorofare: SLACK, 2013; 83-122. 4) Hahn CD, Nicolle DA, Lownie SP, Drake CG. Giant cavernous carotid aneurysms: clinical presentation in fifty-seven cases. J Neuroophthalmol 2000;20:253-8. 5) Mangat SS, Nayak H, Chandna A. Horner's syndrome and sixth nerve paresis secondary to a petrous internal carotid artery aneurysm. Semin Ophthalmol 2011;26:23-4. 6) Oh MJ, Shin DS, Se R, Kim BT. Serpentine cavernous aneurysm presented with visual symptoms improved by endovascular coil trapping. J Cerebrovasc Endovasc Neurosurg 2016;18:379-84. 7) Park JY, Koo NK. A giant unruptured aneurysm of distal internal carotid artery presenting with compressice optic neuropathy. J Korean Ophthalmol Soc 2012;53:1368-71. 8) Stiebel-Kalish H, Kalish Y, Bar-On RH, et al. Presentation, natural history, and management of carotid cavernous aneurysms. Neurosurgery 2005;57:850-7; discussion 850-7. 9) Penchet G, Mourier K. Collaborative retrospective multicentre series of giant intracavernous carotid aneurysms. Neurochirurgie 2015;61:366-70. 10) Markwalder TM, Meienberg O. Acute painful cavernous sinus syndrome in unruptured intracavernous aneurysms of the internal carotid artery. Possible pathogenetic mechanisms. J Clin Neuroophthalmol 1983;3:31-5. 11) Nguyen DQ, Perera L, Kyle G. Recurrent isolated sixth nerve palsy secondary to an intracavernous carotid artery aneurysm. Eye (Lond) 2006;20:1416-7. 12) Biousse V, Newman NJ. Neuro-Ophthalmology Illustrated, 2nd ed. New York: Thieme, 2016; 321-465. 13) Pane A, Miller NR, Burdon M. The Neuro-Ophthalmology Survival Guide, 2nd ed. China: Elsevier, 2017; 169-239. 14) Patel BM, Ahmed A, Niemann D. Endovascular treatment of supraclinoid internal carotid artery aneurysms. Neurosurg Clin N Am 2014;25:425-35. 15) van Rooij WJ, Sluzewski M. Unruptured large and giant carotid artery aneurysms presenting with cranial nerve palsy: comparison of clinical recovery after selective aneurysm coiling and therapeutic carotid artery occlusion. AJNR Am J Neuroradiol 2008;29:997-1002. 391

- 대한안과학회지 2018 년제 59 권제 4 호 - = 국문초록 = 눈분절속목동맥자루에의한가돌림신경마비 1 예 목적 : 급성으로발생한수평복시환자에서눈분절 (ophthalmic segment) 속목동맥자루 (internal carotid artery aneurysm) 에의한가돌림신경마비 (abducens nerve palsy) 를진단하고, 혈관내코일색전술로좋은치료결과를얻었기에이를보고하고자한다. 증례요약 : 이전의특이과거력이없는 59 세여자가 10 일전갑자기발생한수평복시로내원하였다. 시력은양안 20/20 이었고, 동공반응검사에서는정상소견을보였다. 안구운동검사에서정면주시시 35 프리즘디옵터 (prism diopters, PD) 의내사시와좌안 -4 의가쪽운동장애소견을보였다. 1 달전부터이전에없었던측두부의간헐적인두통과좌안눈뒤통증이있었다. 뇌자기공명혈관조영술에서좌측속목동맥의눈분절 (ophthalmic segment) 의돌출병변을확인하였고, 동맥자루로의심되어환자를신경외과로의뢰하였다. 환자는뇌혈관조영술을통해좌측속목동맥의눈분절동맥자루로진단되었고, 코일색전술을시행받았다. 시술후 3 개월째환자의복시는호전되었고, 정면주시에서정위및가쪽안구운동의제한소견도없었다. 결론 : 수평복시를호소하는환자에서눈분절속목동맥자루는가돌림신경마비의원인으로고려될수있고, 혈관내코일색전술을통해서좋은결과를얻을수있음을확인하였다. 가돌림신경마비의적절한치료를위해원인감별이중요하며, 허혈이외의원인이의심되는경우는적극적인뇌영상검사의시행을고려해야한다. < 대한안과학회지 2018;59(4):388-392> 392