검안및콘택트렌즈학회지 2019 년제 18 권제 2 호 Ann Optom Contact Lens 2019;18(2):61-65 ISSN 2384-0919 (Print) ISSN 2384-0927 (Online) Case Report 두단계수술적치료로교정된큰각의외편위를동반한양측성핵간안근마비 Two-staged Surgical Correction for Large Angle Exotropia in Wall-eyed Bilateral Internuclear Ophthalmoplegia 박선경 신희종 윤준명 임혜빈 Sun Kyoung Park, MD, Hee Jong Shin, MD, Jun Myeong Yun, MD, Hye Bin Yim, MD, PhD 가톨릭대학교의과대학인천성모병원안과학교실 Department of Ophthalmology, Incheon St. Mary s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea Purpose: We report a case of two-staged adjustment surgery in wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) with severe bilateral lateral deviation after attack of cerebrovascular infarction. Case summary: A 67-year-old male patient diagnosed with acute intracranial hemorrhage was referred to department of ophthalmology due to severe diplopia that worsened during horizontal gaze of both eyes. On the alternate prism cover test, he had a severe exotropia of about 90 prism diopters (PD) at primary position, and -4 degrees of eye movement disorder were identified on both eyes. Brain magnetic resonance imaging showed a high-signal ischemic infarction on midbrain and pons. Nine months after the initial examination, we performed two-staged adjustment surgery of recession of lateral rectus muscle with resection of medial rectus muscle in both eyes. After 3 months of the two-staged adjustment surgery, he had an exotropia of about 2 PD at primary position on far alternate prism cover test and straight at primary position on near alternate prism cover test. Conclusions: We report a case of two-staged adjustment surgery in WEBINO with severe bilateral lateral deviation showed good results. Ann Optom Contact Lens 2019;18(2):61-65 Key Words: Diplopia; Wall-eyed bilateral internuclear ophthalmoplegia 핵간안근마비는안쪽세로다발의일측혹은양측의손상으로측면주시시내전장애와반대측눈의정상적인외전및해리성안진과눈모음은비교적보존되는점을특징으로하는임상증후군이다. 1 일측성의경우대부분안쪽세로다발의허혈에의해발생하고, 양측성의경우다발성경화증과같은탈수초성질환에서흔히보고되었다. 2,3 그외에 Received: 2019. 4. 8. Revised: 2019. 5. 23. Accepted: 2019. 6. 3. Address reprint requests to Hye Bin Yim, MD, PhD Department of Ophthalmology, Incheon St. Mary s Hospital, #56 Dongsu-ro, Bupyeong-gu, Incheon 21431, Korea Tel: 82-32-280-5110, Fax: 82-32-510-5833 E-mail: yiimhb@naver.com 보고된원인으로는뇌간및제4뇌실종양, Arnold-Chiari 기형, 감염성뇌염, 수두증, 경막하출혈, 뇌혈관기형, Wernicke 뇌병증, 약물, 두부외상등이있다. 4,5 대부분의핵간안근마비는제1안위에서정위이며, 양측성인경우에도심한외편위는동반하지않지만양측성핵간안근마비에서드물게양안의심한외편위가동반되기도하는데, 이경우를 wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) 라고한다. 6,7 국내에서는 Lee et al 8 이두부외상으로인한 WEBINO와그에대해양눈의내직근절제및외직근후전술을통한조정수술을시행하여호전된증례 1예를보고한바가전부이다. 저자들은기존보고와다른뇌혈관경색으로발생한심한양안외편위를보인 WEBINO에서한쪽 Copyright 2019, The Korean Optometry Society The Korean Contact Lens Study Society Annals of Optometry and Contact Lens is an Open Access Journal. All articles are 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. 61
- 검안및콘택트렌즈학회지 2019 년제 18 권제 2 호 - 눈씩두단계의조정수술을시행하여호전된증례를경험하였기에문헌고찰과함께보고하는바이다. 증례 기저질환으로당뇨및고혈압이있던 67세남자환자가급성뇌출혈로진단을받고본원신경외과에입원하여보존적치료및재활치료를시행받았다. 의식회복후 1개월뒤, 양측의수평주시시악화되는심한복시증상을주소로안과에의뢰되었다. 외래내원시에시행한안과검사소견으로는최대교정시력은우안 0.9, 좌안 0.6이었으며, 안압은우안 10 mmhg, 좌안 10 mmhg였고, 세극등현미경검사상전안부및안저에서이상소견은관찰되지않았다. 교대프리즘가림검사상제1안위에서약 90프리즘디옵터의심한외사시를보였고, 안구운동검사에서는내전시우안 -4, 좌안 -4 정도의안구운동장애가확인되었다. 양안모두외전및하전시에는정상적인안구운동을보였으며, 상전시에는 -1 정도의경한안구운동장애가동반되었으나임상적인의의는없었다. 내전시반대측눈의외전과동반 된안진이관찰되었으며, 눈모음 (convergence) 기능은비교적보존되어있었다 (Fig. 1). 신경외과입원당시에시행하였던뇌자기공명영상 (brain magnetic resonance imaging) 강조영상에서중뇌및뇌교에고신호를보이는허혈성경색병변이관찰되었다 (Fig. 2). 초진이후 9개월의경과관찰기간동안호전없는 90프리즘디옵터의외사시및양안의안구운동제한으로이에대해조정수술을진행하기로계획하였다. 환자의연령및외사시정도와술후교정가능정도를종합적으로고려하여외직근후전술및내직근절제술을한쪽눈씩두단계에걸쳐시행하기로하였다. 일차적으로좌안의외직근에대해 10.0 mm 널치기후전술및내직근에대해 7.0 mm 절제술을시행하였고, 술후 2개월째에좌안의외전정도는 -2에서 -1 정도로감소된소견이관찰되었다. 첫번째수술후 4개월째에우안의외직근에대해 8.0 mm, 널치기후전술및내직근에대해 6.0 mm 절제술을시행하였으며, 술후 3개월째는교대프리즘가림검사상원거리제1안위에서약 2프리즘디옵터의외사시, 근거리제1안위에서정위상태로관찰되었고, 복시도사라 Figure 1. Pre-operative extraocular movements in 9 cardinal gazes. The pictures show bilateral adduction limitation associated with large angle exotropia. 62
- 박선경외 : 두단계로수술한양측성핵간안근마비 - Figure 2. Brain magnetic resonance imaging. The images show infarction in midbrain level (white arrowheads). Figure 3. Post-operative extraocular movements in 9 cardinal gazes. The pictures show improvement of bilateral adduction limitation associated with large angle exotropia after surgery. 63
- 검안및콘택트렌즈학회지 2019 년제 18 권제 2 호 - 졌다. 내전장애는우안 -1, 좌안 -1 정도로수술전에비해크게호전된소견이관찰되었다 (Fig. 3). 고찰 안쪽세로다발은교뇌중심부에근접하여외향신경핵사이와활차신경핵그리고동안신경핵의외하방을지나면서안구운동신경핵들을연결한다. 9 동측으로의수평주시운동중추는뇌교방내측망상체 (pontine paramedian reticular formation) 로, 중추에서시작한신호는동측의외향신경핵과반대측의안쪽세로다발을따라동안신경핵중내직근신경핵으로가서동측으로의수평주시운동을조절한다. 1 핵간안근마비는이과정에서안쪽세로다발의병변으로인해내직근신경핵으로가는신호가방해를받아동측의눈에서내전장애가발생할수있다. 1,2 하지만 Gonyea 3 는내직근신경핵의마비없이도외사시를보이는경우를보고하였으며, 이에대한원인으로핵간, 핵상, 핵하등부위에관계없이내전근의이향운동기능장애 (dysconjugate impairment) 때문으로생각하였다. 본증례와같이핵간안근마비에서는눈모음 (convergence) 이유지되는것을관찰할수있고, 이와관련된중추는시피질과뇌교방내측망상체로생각되나아직확립되어있지않다. 안쪽세로다발의병변이동안신경핵을포함하는입쪽의 (rostral) 병변일경우, 눈모음의장애가발생하기도한다. 10 단안운동및이향운동에서헤링법칙을따르는동향운동에비해내전정도가크지않기때문에, 핵간안근마비에서작은내전의움직임이관찰되는것도눈모음이보존되는것이라고하였다. 7 양안외사시가큰상태에서눈모음을유발하는것은어려우므로반대측눈을가린상태에서코쪽을향해주시물체를접근시키는방법을사용하여눈모음을유발시킬수있다. 8 내전시반대측눈의외전과연관된안진의원인에대해서는명확하게밝혀지지않았다. 11,12 내전근의약화를극복하기위한일종의정상적반응으로보기도하며, 교뇌와연수에있는정중앙섬유로 (midline fiber tract) 를따라흩어져있는신경세포의집합체정중으로세포군에서주시유발안진을일으킬수있는것으로보고되어있다. 13 WEBINO 환자의수술방법에따른수술적결과를보고한바는많지않다. Roper-Hall et al 14 은총 8명의 WEBINO 환자를대상으로 4예에대해서는한눈의외직근후전술및내직근절제술을, 3예에대해서는한눈의외직근후전술및내직근절제술을시행한후반대쪽안의외직근후전술과내직근절제술을두단계로시행하였다. 또한 1예에대해서는양안의외직근후전술및내직근절제술을동시에시행하여 전체적으로환자군의 85% 가량에서외편위감소와 87.5% 에서융합반응이관찰되었음을보고하였으며, 더불어수술이후내전의제한및외전과동반된안진도유의하게호전되는비교적좋은결과를보고하였다. 여러저자들은수술에앞서양안의외직근후전술혹은양안의내직근절제술과같이두눈의대칭적인수술은효과적이지않으며, 수술과정에내직근을강화시키는방법이포함되어야함을강조하였다. 그리고외편위의양이크지않을경우에는한눈에대한외직근후전술및내직근절제술로도표준적인수술의교정량으로예상되는정도보다좋은결과를기대할수있음을보고하였다. 14,15 Buckley and Elston 15 은 WEBINO 환자에대해성공적인조정봉합을동반한양안의외직근후전술및내직근절제술을동시에시행한사례를보고하기도하였으나, 매우큰정도의편위를갖는젊은환자에서만고려되어야할것이다. 편위의정도가매우크고, 수술에의한교정정도가예측하기어려운경우에는본증례와같이한쪽눈의외직근후전술및내직근절제술을시행한후반대쪽안의외직근후전술과내직근절제술을두단계로시행할수있으며, Roper-Hall et al 14 은두단계수술로수술전외편위가 84% 가량호전되고기타동반된안과적소견의호전을보고하였다. 핵간안근마비는정상적인안구운동조절력과융합반응이떨어져일반적인사시교정술로는그결과를예측하기어려우며외편위정도가클수록더욱큰어려움이존재한다. 본증례의한계점은수술후 3개월이후의장기적인추적관찰결과를얻지못하였다는점으로, 수술전의외사시각및안구운동장애정도를고려하였을때추후재발할가능성을배제할수없으나큰외사시각을가진양측성핵간안근마비에서한쪽눈씩, 두단계의외직근후전술및내직근절제술을시행하여만족할만한수술직후의외사시교정을경험하였으며수술후 3개월째까지단기경과의좋은결과를보았기에문헌고찰과함께보고하는바이다. REFERENCES 1) Cogan DG, Kubik CS, Smith WL. Unilateral internuclear ophthalmoplegia; report of 8 clinical cases with one postmortem study. Arch Ophthalmol 1950;44:783-96. 2) Cogan DG. Internuclear ophthalmoplegia, typical and atypical. Arch Ophthalmol 1970;84:583-9. 3) Gonyea EF. Bilateral internuclear ophthalmoplegia: association with occlusive cerebrovascular disease. Arch Neurol 1974;31:168-73. 4) Jacobs DA, Galetta SL. Multiple sclerosis and the visual system. Ophthalmol Clin North Am 2004;17:265-73. 5) Baker RS. Internuclear ophthalmoplegia following head injury. J Neurosurg 1979;51:552-5. 64
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