성균관대학교의과대학강북삼성병원이비인후과학교실이용우, 장선오, 김민범 J Korean Skull Base Society 12 권 2 호 : 47~52, 2017 경유양동상미로접근법을이용한안면신경감압술로회복된 Ramsay Hunt syndrome 1 례 A case of

성균관대학교의과대학강북삼성병원이비인후과학교실이용우, 장선오, 김민범 J Korean Skull Base Society 12 권 2 호 : 47~52, 2017 경유양동상미로접근법을이용한안면신경감압술로회복된 Ramsay Hunt syndrome 1 례 A case of Ramsay Hunt syndrome treated by facial nerve decompression via transmastoid/supralabyrinthine approach 종설1 종설2 원저1 원저2 증례1 증례2 증례3 증례4 증례5 증례6 증례7 증례8 증례9 Department of Otolaryngology-Head and Neck surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea Yong Woo Lee, Sun O Chang, Min-Beom Kim Ramsay Hunt syndrome (RHS) is a disease that shows a triad of facial palsy, vesicles on auricle, and hearing symptoms. This syndrome is characteristic of herpetic inflammation activation on the 교신저자 Min-Beom Kim 논문접수일 : 2017 년 8 월 5 일논문완료일 : 2017 년 8 월 25 일주소 : Department of Otolaryngology-Head and Neck surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 29, Saemunan-ro, Jongno-gu, Seoul 03181, Korea Tel : +82-2-2001-2264 Fax : +82-2-2001-2273 E-mail : minbeom.kim@gmail.com geniculate ganglion due to reactivation of varicella-zoster virus. Based on the pathogenesis of disease, high dose corticosteroids with antiviral agents are recommended as first line treatment. However, for those who are anticipated with unfavorable prognosis, surgical decompression of facial nerve is considered as an appropriate option. Currently, surgical treatment is recommended to be performed within 14 days, and electrophysiologic tests such as electroneurography, electromyography are used for selection of adequate subjects. A 56 year-old male diagnosed of RHS was treated with Facial nerve decompression via transmastoid/supra-labyrinthe approach and showed favorable prognosis. We reviewed this case with relevant publishings in medical literature. Key Words Electromyography Facial paralysis Geniculate ganglion Herpes zoster oticus 경유양동상미로접근법을이용한안면신경감압술로회복된 Ramsay Hunt syndrome 1 례 47

INTRODUCTION Ramsay Hunt 증후군 (Ramsay Hunt syndrome) 은슬신경절상에서잠재해있던대상포진바이러스 (varicella-zoster virus) 의재활성화로인한질환으로알려져있다. [1, 2] 내이도내에서안면신경과전정와우신경의해부학적근접성으로인해이명, 청력저하, 현훈과안진등이동반될수있다. [3] 본저자들은좌측의안면마비와청력저하가발생하여내원한 56세남자 Ramsay Hunt 증후군환자를치험하였다. 보존적치료에도신경전도검사 (electroneuronography) 상불량한예후가예상되어안면신경감압술을경유양동상미로접근법 (transmastoid/ supra-labyrinthe approach) 을통해시행하였으며이를문헌고찰과함께증례보고하는바이다. 한자기공명영상 (magnetic resonance imaging) 에서는내이도에서부터안면신경의슬신경절에이르기까지조영증강이관찰되는것외에는특이병변은관찰되지않았고측두골단층촬영상에서도병변은관찰되지않았으며상반고리관 (superior semicircular canal) 과고실개 (tegmen tympani) 사이에공간이있는것을확인할수있었다 (Fig. 3). 안면마비가발생한지 3일째와 5일째에시행한신경전도검사상좌측의 orbicularis oculi, orbicularis oris muscle 에 Fig. 2 CASE REPORT 56세남자환자가내원 6일전부터발생한좌측이개의부종과내원전날발견된좌측의안면마비를주소로외래로내원하였다. 내원당시좌측이개와외이도의부종, 발적및수포소견과함께통증을호소하고있었으나청력저하는호소하지않았고좌측안면부의 House-Brackmann grade VI에해당하는마비가관찰되어 (Fig. 1) 하루 methylprednisolone 48 mg의고용량스테로이드요법과함께항바이러스제인 acyclovir 투여를시작하였다. 환자는고혈압과간세포암으로 3년전간부분절제술을시행받은병력이있었다. 입원당시혈액및감염성질환에대한혈청학적검사상특이소견은관찰되지않았다. 입원 2일째, 환자가좌측의청력저하를호소하여시행한순음청력검사상좌이의 41.7 db의감각신경성난청소견관찰되었고 (Fig. 2), 중추및후미로성병변의확인을위해시행 Initial pure tone audiometry. Ipsilateral sensorineural hearing loss, dominant in high frequency was observed. Rt.: right, Lt.: Left, AC: air conduction, BC: bone conduction, SRT: speech recognition threshold, MCL: most comfortable level, UCL: uncomfortable listening level, DR: dynamic range. Fig. 1 Initial clinical photo of the patient. House-Brackmann grade VI was observed. 48 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2

자극에대한반응이전혀관찰되지않았고 (Fig. 4), 근전도검사에서도자발전위와운동단위전위가감지되지않는모습이었다. 이에, 조기고용량의스테로이드치료에도불구하고연속적으로시행한신경전도검사상 90% 이상의신경변성이관찰되었고임상적으로나전기생리적으로회복의증거가보이지않아, 마비발생 7 일째경유양동상미로접근법을통해안면신경감압술을시행하게되었다. 수술은후이개를통한접근을하여유양돌기삭개술을시행하였고, 구불정맥동에서중두개와의경막까지넓게노출시키고이소골중침골을제거하였다. 안면신경골관감압을위해경유돌공에서유돌분절을따라골관을제거하였으며, 고실분절을확인하기위해안면신경의슬신경절까지노출시켰다. 그이후, 고실천장 (tegmen tymapani) 을 drilling하면서상반고리관과고실개사이의공간을통하여미로분절에접근하여감압을시행하였고내이관공 (meatal foramen) 을개방하였다. 수술당시신경의종창이전체적으로관찰되었고노출된미로분절서부터유돌분절에이르기까지창백하면서홍반성소견이관찰되었으며 (Fig. 5), 수술중시행한 nerve monitoring에대해서도반응을보이지않았다. 수술중제거한침골을대체하여부분이소골대치술시행하였으며, 수술은합병증없이마무리되었다. 수술후 8일째까지특이합병증은관찰되지않고환자는퇴원하였다. 수술시행 2달째근전도검사상운동단위전위와자발전위가관찰되었고경미하게주관적인호전을호소하였지만임상적으로는여전히완전마비소견관찰되었다. 수술시행 5개월째근전도검사상다형성파형의운동단위전위가관찰되었고 (Fig. 6) 임상적으로 House-Brackmann grade III으로회복소견관찰되었다. 이후 1년이지난시점에서시행한순음청력검사상좌측 24.2dB 으로회복되어일상생활에불편이없는수준이며 (Fig. 7), 좌측안면마비도 House-Brackmann grade II 로회복소견이관찰되며 (Fig. 8), 기 타합병증없이추적관찰중이다. DISCUSSION 이개대상포진이라고도알려진 Ramsay Hunt 증후군의일차적 인치료는고용량의스테로이드와항바이러스제의병합요법이사 용되고있으며, 이러한약제는급성대상포진증상의지속기간을 단축하고장기적인신경손상을감소시키는효과를보인다. [1] 기존 의문헌들은급성안면마비가발생하고 3 일이내치료를시작할시 [4, 5] 에전반적인예후를향상시킨다고밝히고있다. 위와같은보존적치료법외에, 안면신경마비의치료에대해서 Fig. 4 Initial electroneurography, showing nearly absent voluntary potential on left (Lt.) facial muscle. Rt.: right. Fig. 3 Magnetic resonance imaging showing enhancement on internal auditory canal to geniculate ganglion (above), space between superior semicircular canal and tegmen tympani is observed on computed tomography imaging (below). 경유양동상미로접근법을이용한안면신경감압술로회복된 Ramsay Hunt syndrome 1 례 49

논란이되어왔던것은, 수술적치료가환자의예후를개선하는지, 그리고어떤환자들에게, 어느시점에수술적치료를시행하는것이이상적인가에대한논의였다기존의문헌상에서는신경전도검사상증상발병 6일이내 90% 이상혹은 14일내 95% 이상의신경변성소견이관찰되는경우를나쁜예후의지표로보고수술적치료를권장하였고, 14일이내에외과적치료가행해질때그치료결과가좀더좋았다고보고되고있다. [6, 7] 본증례의경우, House- Brackmann grade VI에해당하는완전마비소견이관찰되며증상발생 4일째, 6일째두차례시행한신경전도검사상모두환측의 95% 이상의신경변성소견, 근전도검사상자발전위와운동단위전위가감지되지않아기존보고들을통해서권장되는안면신경감압술의적응증에해당되는사례였다. 급성의안면마비를유발하는질환의외과적치료에있어서, trauma 등에의한신경이절단된경우를제외하고는, 안면신경감압술이주된치료법으로받아들여지고있다. 감압술을시행할때, 흔히사용되는접근법으로는경유양동접근법과중두개와접근법, 그리고앞의두가지접근을혼합한방법이있다. 어떤방법을선택하는지에앞서, 환자의연령과과거병력, 잔존청력, 손상된신경의분절, 환자의기대정도와위험감내도를종합적으로고려하여야한다. Bell 마비나 Ramsay Hunt 증후군과같은급성말초성안면마비서는내이관공이포함된미로분절을주로침범하는것으로알려져있으며, 따라서안면신경감압술을시행할시에미로분절에접근할수있는중두개와접근이권장되고있다. 중두개와접근은많은장점에도불구하고개두술 (craniotomy) 을시행함으로써뇌실질과주변의중요혈관, 신경들의손상에대한위험성이존재한다. 하지만, 고실천장과상반고리관사이에공간이확보될수있는경우에는경유양동상미로접근법을통해서개두술로인한위험은피하면서안면신경에의미로분절에의감압을시행할수있다. [8-10] 본증 례에서는청력과평형기능이잔존해있고, 자기공명영상의 T1 강조 영상에서병변측의슬신경절과고실분절의근위부에서고음영의 신호가관찰되어, 해당부위의안면신경손상이의심되고있어경 유양동상미로접근법을통해안면신경감압술을시행하게되었고, Fig. 6 Electromyography (EMG) of postoperative 5 month, polyphasic potential observed on all left (Lt.) facial muscles. MUAPs: motor unit action potentials. Fig. 5 Surgical photo after decompression of facial nerve bony canal. Nerve swelling throughout all segments exposed is observed. 50 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2

슬신경절과미로분절일부를확인하기위해유양돌기삭개술과후 고실개방술을시행한이후, 침골과추골일부를제거한후고실개 를 drilling 하여일부제거하여제한적으로중두개경막을노출시켜 수술적치료를시행하였다. 본증례는, 순음청력검사상의고음역난청에더불어온도안진검 사좌측의 79% 반고리관마비와전정유발근전위가좌측서관찰되 Fig. 7 Pure tone audiometry of postoperative 1 year. Improvement of left (Lt.) hearing to serviceable range. Rt.: right, AC: air conduction, BC: bone conduction, SRT: speech recognition threshold, MCL: most comfortable level, UCL: uncomfortable listening level, DR: dynamic range. 지않아, 대상포진바이러스가전정신경과와우신경을모두침범하였음을짐작할수있었다. 기존연구를통해, 이처럼전정신경에침범이있는경우, 안면마비의회복에있어나쁜예후인자로작용할수있다는것이밝혀지기도하였다. [3] 저자들은증상발생하루만에고용량의스테로이드와항바이러스제를시작하고 7일째에, 수술을시행하여기존연구들에서권장하는적절한시기에약물치료와감압술을진행하였고, 수술시행 50일째에이르러근전도검사와신경전도검사에서뚜렷한신경재생의증거가관찰되었으며, 임상적으로는수술 5개월째에이르러회복되는모습을보였다. 수술후 1년째에는 House-Brackmann grade II로임상적으로 complete recovery 하였음을확인하여여러가지불량한예후인자들에도불구하고적절한시기에적극적으로치료를시행후 functional gain 4.0을보인증례에해당하겠다. 외과적감압술을시행하는최적의시기에대한이론적인배경은월러변성 (Wallerian degeneration) 의시기에기초하고있다. Gantz 등 [10] 의연구에의해기존까지증상발생 14일이내에감압술을시행한경우에효과가있다고알려져있었지만, 일부저자들은수술전고용량의스테로이드요법을충분히시행한경우에, 증상발생 2 달까지도수술적치료에의한효과가있다고보고하여시행시기에따라차이는있지만, 비수술군과비교하여좋은치료성적이보고되고있다. [11, 12] 따라서, 저자들은적절한적응증에따라외과적감압술을시행하여우수한치료결과를보인증례를보고하는바이며, 급성의안면신경손상을보이는환자들에있어, 신경생리검사상수술적치료의적응증을만족하는경우에는적극적으로안면신경감압술을시행하는것이예후에좋을것으로생각된다. Fig. 8 Clinical photo of postoperative 1 year. Left facial palsy nearly completely recovered with House-Brackmann grade II observed. 경유양동상미로접근법을이용한안면신경감압술로회복된 Ramsay Hunt syndrome 1 례 51

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