종설1 종설2 원저1 원저2 증례1 증례2 증례3 증례4 증례5 증례6 증례7 증례8 증례9 J Korean Skull Base Society 12 권 2 호 : 18~24, 2017 1 연세대학교의과대학세브란스병원신경외과학교실, 2 CHA 의과대학교분당차병원신경외과학교실, 3 연세대학교의과대학세브란스병원이비인후과학교실 홍제범 1, 2, 김한규 2, 김주평 2, 장종희 1, 문인석 3 Surgical approach for jugular foramen tumors 1 Department of Neurosurgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea 2 Department of Neurosurgery, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea 3 Department of Otorhinolaryngology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea Je Beom Hong 1, 2, Han Kyu Kim 2, Joo Pyung Kim 2, Jong Hee Chang 1, In Seok Moon 3 Background: The infratemporal fossa approach type A (ITFA-A) is a good approach for small jugular foramen tumors with a small extraforaminal cervical extension. On the other hand, the posterolateral approach to jugular foramen has been adopted to see the posterior aspect of the jugular foramen. For large tumors, we combined posterolateral approach with various otologic operations including ITFA-A, transcochlear approach and fallopian bridge technique. The purpose of this study is to evaluate the surgical adequacy of our approaches including postoperative complications and outcomes. Methods: From January 2014 to January 2017, we operated total 14 cases of jugular foramen tumors. We chose the surgical approach to these tumors based on their location and extent. And the facial nerve manipulation was added in combined approach in which facial nerve was dissected and transpositioned (3 cases) or remained in fallopian canal (fallopian bridge technique, 3 cases). Results: Grossly total resection was achieved in 12 patients (85.7%). Immediate postoperative 교신저자 Joo Pyung Kim 논문접수일 : 2017 년 8 월 5 일논문완료일 : 2017 년 8 월 25 일주소 : Department of Neurosurgery, CHA Bundang Medical Center, CHA University School of Medicine, 59, Yatap-ro, Bundang-gu, Seongnam 13496, Korea Tel : +82-31-780-5688 Fax : +82-31-780-5269 E-mail : jpkim@cha.ac.kr lower cranial nerve deficit occurred in 10 patients (71.4%). Postoperative facial nerve paralysis and hearing impairment occurred in 4 patients (28.6%) and 6 patients (42.9%) respectively. Two-thirds of the jugular foramen could be exposed in the combined approach which enabled the complete removal of tumors regardless of the size at this area. Conclusion: Using skull base technique with thorough understanding of surrounding anatomic structures followed in wider exposure, gross total removal can be achieved by multidirectional approach under relative safety. Key Words approach, jugular foramen, skull base, tumor 18 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2
INTRODUCTION METHODS 현대의학의눈부신발전에도불구하고경정맥공종양 (jugular foramen tumors) 은도전적수술적치료를요하는힘든종양이다. 이들종양에는부신경절종 (paraganglioma), 신경초종 (schwannoma), 수막종 (meningioma), 척삭종 (chordoma), 연골육종 (chondrosarcoma), 전이성암 (metastatic carcinoma) 등이있다. [1] 이종양들이수술하기어려운이유는종양의위치가혈관및각종뇌신경과복잡하게연관되어있어수술후기능, 미용적합병증이발생할경우심리적합병증까지동반할정도로중요한구조물이많기때문이다. 경정맥구 (jugular bulb) 뿐만아니라제 9, 10 및 11번뇌신경과연관이있고, [2] 종양이큰경우내경동맥 (internal carotid artery) 및제 6, 7 및 8번뇌신경, 12번뇌신경과도관련이있을수있다. 종양의크기가더커질경우, 해면정맥동, 사대, 뇌간까지연관되어종양제거가어려운경우도발생할수있다. 그래서최근에는방사선수술등의다양한치료법을이용하여고식적수술과병행하여환자에게적합한치료를적용하고있다. [3-5] 하지만경정맥공종양의치료에서최대한의안정적수술적제거가기본적으로요구되어이에저자는경정맥공종양의수술적치료에서여러과와의협력수술 ( 다학제적접근 ) 을통한종양의안전한제거에대해연구해보고자하였다. 1. Patients and Methods 최근 2014 년 1 월부터 2017 년 1 월까지총 14 명의경정맥공종양 환자에대해수술적치료를시행하였다. 모든환자는수술전두부 컴퓨터단층촬영 (computed tomography), 자기공명영상 (magnetic resonance imaging) 및뇌혈관조영술 (cerebral angiography) 을시 행받았다. 모든환자의의무기록과영상의학검사를검토하여나 이, 성별, 수술전증상, 수술전후신경학적검사결과, 병변의위치 Fig. 1 Patient s position and skin incision. The patient is placed in the supine position with head held in clamp and turned 60 degree to the opposite site of the operation. Table 1. Patients and disease characteristics Patient no. Sex Age (years) Type a) Approach Pathology Follow-up (months) 1 M 40 B3 Combined (ITF-A) Paraganglioma 30 2 F 43 A Far lateral Meningioma 31 3 F 53 D Combined (ITF-A) Meningioma 22 4 F 63 D Combined (Transcochlear) Chondrosarcoma 20 5 M 44 D Combined (Fallopian bridge) Schwannoma 19 6 M 61 A Far lateral Schwannoma 19 7 F 52 B2 Far lateral Schwannoma 16 8 F 46 D Combined (Fallopian bridge) Meningioma 12 9 M 45 B2 Posterolateral Schwannoma 12 10 F 54 D Combined (Fallopian bridge) Schwannoma 8 11 M 56 B1 Far lateral Metastatic carcinoma 12 12 M 48 A Far lateral Schwannoma 7 13 F 40 B2 Posterolateral Paraganglioma 30 14 M 41 A Far lateral Schwannoma 39 M: male, F: female, ITF-A: infratemporal fossa approach type A a)type was determined by jugular foramen schwannoma classification. 19
Fig. 2 및수술적접근법, 절제정도를조사하였다. Suboccipital triangle exposure. Fig. 3 Occipital condyle and jugular process. After the suboccipital craniotomy, atlantooccipital joint and jugular process can be exposed. Fig. 4 Jugular process drilling. The hatched area indicates the extent of bone removal. 2. Surgical Procedure 수술적접근법은 6례에서극외측접근법 (far lateral approach), 2례에서후외측접근법 (posterolateral approach) 이시도되었으며, 나머지 6례에서타과와공동으로복합접근법 (combined approach) 이시도되었다 (Table 1). 모든수술에서체성감각유발전위 (somatosensory evoked potentials), 운동유발전위 (motor evoked potential) 신경감시를준비하였으며, 필요에따라뇌간청각유발전위 (brainstem auditory evoked potentials) 나안면신경감시장치 (facial nerve monitoring) 를통하여청신경및안면신경기능감시를하였다. 수술적접근법의선택은환자들병변의위치에따라결정되었으며, 다학제적접근에포함된팀들은신경외과, 두경부외과, 이과, 신경영상의학과였다. 환자의자세는앙와위 (supine position) 에서고개를수술부위반대방향으로 60도회전시키고진행하였다. 이후수술진행시필요에따라침대의각도를조절하였다 (Fig. 1). 1) Far lateral approach 피부절개를가한후두경부근육들을구분하여절개하였다. 후두하삼각 (suboccipital triangle) 보다위에있는근육은한층으로박리하여수술시간을단축시켰다 (Fig. 2). 후두하삼각을파악하여추골동맥 (vertebral artery) 의주행을확보하고수술을진행하였다. 이후후두개개두술을시행하고추골동맥을아래쪽내측으로전위시키면환추-후두관절 (atlanto-occipital joint) 과후두관절구 (occipital condyle) 가노출된다 (Fig. 3). 다음단계로후두관절구를일부제거한후경막을절개하고종양의제거를시작하게된다. 2) Posterolateral approach 극외측접근법만으로종양을충분히노출시킬수없는경우경정맥돌기 (jugular process) 를드릴하고 (Fig. 4), 미로하유양돌기절제술 (infralabyrinthine mastoidectomy) 을시행하여경정맥공을더넓게노출시킬수있다. 이경우안면신경은안면신경관 (fallopian canal) 속에그대로둔체로유양돌기절제술을시행한다. 경정맥돌기를점차드릴하면설하신경관 (hypoglossal canal) 을확보하게되고설하신경관과경정맥공사이의경정맥결절 (jugular tubercle) 을드릴하여경정맥공후, 내측으로더노출시킬수있다. 3) Neck dissection 내경정맥 (internal jugular vein) 의노출이필요한경우에는경부절제술을시행하게된다. 이를통해내경정맥, 미주신경 (vagus nerve), 척수부신경 (spinal accessory nerve), 설하신경 20 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2
(hypoglossal nerve) 을노출시키게되고내경정맥의원위부를확보하여필요시결찰할수있도록하였다. 4) Combined approach 상기접근법만으로종양의충분한노출이부족한경우이과의사 (otologist) 의도움을받아복합접근법을시행하였다. 이는A형측두하와접근법 (infratemporal fossa approach type A), [6, 7] 경와우접근법 (transcochlear approach) [8] 혹은안면신경관브릿지기술 (fallopian bridge technique) 이었다. A형측두하와접근법시행시에는안면신경을전방으로전위시켰으며 (Fig. 5), 경와우접근법시행시에는안면신경을후방으로전위시켰다. 안면신경관브릿지기술 (fallopian bridge technique) 을병행할때는안면신경의수직분절 (vertical segment) 을골격화 (skeletonization) 한채미로하함기세포 (infralabyrinthine air cells) 및안면신경후함함기세포 (retrofacial air cells) 를제거하였다. 안면신경조작 (facial nerve manipulation) 에서유양돌기분절 (mastoid segment) 만을재라우팅 (rerouting) 하는단편전방재라우팅 (short anterior rerouting) 을시행한경우는없었다. 복합접근법각각의경우에서병변을충분히노출시킬수있도록유양돌기절제술을시행한후종양을제거하였다. 5) Tumor removal 경정맥구를종양이침범한경우상추체정맥동 (superior petrosal sinus) 원위부에서 S상정맥동 (sigmoid sinus) 을결찰및절단하였다. 내경정맥또한결찰및절단하고종양을제거하였다. 내경정맥을먼저결찰하고종양의제거를시작함으로써심장으로공기가들어가생길수있는공기색전증 (air embolism) 을막을수있었고모든례에서공기색전증의합병증은발생하지않았다. 종양의제거시제일중요한요소는주변혈관이나신경의손상없이종양을제거하는것이다. 종양이크게측두하와공간으로자란경우, 내경동맥의손상에주의해야한다. 또한경정맥구의안쪽면을최대한보존하면서종양을제거하여하위뇌신경을보호하도록해야한다. 하추체정맥동 (inferior petrosal sinus) 로부터유입되는혈액에대한지혈작업시에도경정맥구바깥으로나가는하위뇌신경들에대해서특히주의하여야한다. 경막내 (intradural) 종양을제거할때는종양과주변신경, 혈관과절개면 (dissection plane) 을최대한찾으려고하였고유착관계가너무심한경우는일부종양을남기도록하였다. 6) Skull base reconstruction 경정맥공종양의수술후재건에서, 경막봉합이쉽지않고경막의결손이있는경우가많아뇌척수액누수가발생할확률이높 다. 우리는수술후뇌척수액의유출을막기위해미세수술기법 을이용하여최대한경막을빈틈없이 (watertight) 닫았다. 경막에 종양이침범하여경막을제거한경우에는인공경막이나자가근막 (autologous fascia) 을이용하여재건하였다. 결손부위가큰경우 는복부지방 (abdominal fat) 이나측두근 (temporalis muscle) 을 이용하여결손부위를채워주었다. 9) 뇌척수액누수가발생한경우 Fig. 5 Facial nerve anterior transposition. In the infratemporal fossa approach type A procedure, the facial nerve is rerouted anteriorly. Fig. 6 The 40 years old male patient, main symptom was tinnitus and a glomus jugulare tumor was found on magnetic resonance imaging. The surgical approach was a combined approach (infratemporal fossa approach type A+far lateral+neck dissection). Postoperative facial paralysis and dysphagia were present, but much recovered after rehabilitation. 21
는요추천자를통한일시적뇌척수액배액을시행하여해결할수있었다. RESULTS 총 14명의수술받은환자중남자는 7명, 여자는 7명이었으며평균나이는 49.0세 (40-63세) 이었다. 평균추적관찰기간은 19.8 개월 (7-39개월) 이었다 (Table 1). 12례 (85.7%) 에서전절제 (gross total resection) 를시행하였고, 2례에서는주변신경조직과유착관계가심하여아전절제술 (subtotal resection) 을시행하였다. 수술전종양으로의혈액공급과정맥유출을잘파악하고필요시색전술이나혈관중재시술을고려해야하고종양제거후혈관문합술이필요할경우도고려해야한다. [10, 11] 이번연구에서는총 6례에서수술전색전술이시행되었고혈관문합술이시행된경우는없었다. 병리조직결과는 2례에서부신경절종, 1례에서연골육종, 7례에서신경초종, 3례에서수막종, 1례에서전이성암이나왔다 (Table 1). 수술전증상으로는청력저하 (hearing impairment) 가 6례, 어지러움이 5례로가장많았으며, 연하곤란, 두통, 구음장애, 안면마 Table 2. Symptoms and signs at presentation No. of patients Symptom Hearing impairment 6 Dizziness 5 Dysphagia 3 Headache 4 Speech disturbance 2 Sign Facial palsy 2 Diplopia 3 Dysarthria 3 Other cranial nerve deficit 6 Table 3. Postoperative complications Deficit No. of patients (%) Lower cranial nerve palsy 10 (71.4) Improved lower cranial nerve palsy 4 (28.6) Facial palsy 4 (28.6) Improved facial palsy 3 (21.4) Hearing impairment 6 (42.9) CSF leakage 2 (14.3) 비, 복시등의증상이있었다 (Table 2). 수술후합병증으로는하위뇌신경마비 (lower cranial nerve palsy) 가 10 례, 청력저하가 6 례, 안면마비증세가 4 례에서발생하 였다 (Table 3). 수술후하위뇌신경마비가발생한환자중 4 례, 안 면마비가발생한환자중한 3 례에서는빠른호전을보였고나머지 환자들도호전되는양상을보여추적관찰중이다. 뇌척수액누출은 2 례에서발생하였으며 2 환자모두요추천자술을열흘가량유지함 으로써뇌척수액누수의문제를해결할수있었다. 사망환자는없 었다. 1. Case 1 40 세남환, 이명을주소로시행한검사상경정맥사구종 (glomus jugulare tumor) 이발견되었다 (Fig. 6). 수술적접근법은 A 형측 두하와접근법 + 극외측접근법 + 경부절제술 (neck dissection) 의 복합접근법을통해잘제거되었다. 수술후하우스 - 브랙만등급 (House-Brackmann grade) IV 의안면마비가발생하였지만, 수술 12 개월후추적검사상하우스 - 브랙만등급 II 로호전되었다. 수술 후발생한연하곤란은수술 12 개월후일반식이가가능한정도로 회복되었다. 2. Case 2 45 세남자환자로청력저하와안면마비가발생하여시행한검 사에서 B2 형 (Type B2) 의경정맥공신경초종 (jugular foramen Table 4. Classification of JF schwannomas Tumor classification A B C D B1 B2 B3 JF: jugular foramen. Definition Tumor arising from cisternal part of the nerves, without significant extension into the JF Intraosseous tumor inside the JF Intraosseous tumor with significant extension into the cisternal space Intraosseous tumor with significant extension into the infratemporal fossa Tumor arising from the peripheral part of the nerve (extracranial type) Triple dumbbell-shaped tumor with intracranial, intraosseous and extracranial parts [Reprinted from "Surgical treatment of jugular foramen schwannoma: surgical treatment based on a new classification", by Samii M, Alimohamadi M, Gerganov V, 2015, Neurosurgery, 77, pp.424-32. Copyright 2015 by the Oxford University Press. Reprinted with permission]. 22 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2
schwannoma) 이진단되었다 (Fig. 7). 후외측접근법을통하여종양제거를시도하였으며종양은전절제가되었다. 수술후하우스- 브랙만등급 IV의안면마비가발생하였지만, 수술 6개월후하우스-브랙만등급 II로호전되었다. DISCUSSION 경정맥공종양의분류는각각의병리 ( 신경초종, 곁신경절종, 수막종 ) 에대해다른분류법이존재한다. [12] 우리는경정맥공신경초종을나누는기준을전체종양에적용하였다. [13] 해부학적위치관계를파악하고수술접근법을고려하는데그분류가가장직관적이고타당하다고판단되어그분류를따랐다 (Table 4). 경정맥공종양의접근법은크게세가지로나누어볼수있다. [5,14-16] 전방접근법은일반적으로많이쓰이지않는다. 이전방접근법에는내시경경비적 / 경상악동경익상골접근법 (endoscopic transnasal/transmaxillary transpterygoid approach), [17] 경관골-경하악골접근법 (zygomatic-transmandibular approach), [18] B형, C형측두하와접근법 (infratemporal fossa approaches type B, C) [14] 등이있다. 측방접근법에는 A형측두하와접근법, 경정맥접근법 (transjugular approach) [19] 및여러가지변형된경추체접근법들이있다. 후방접근법에는후S상정맥동및극외측경후두과접근법 (retrosigmoid and extreme lateral transcondylar approach) 이있다. 그외에복합접근법으로추체후두경S상정맥동접근법 (petrooccipital transsigmoid approach), [20] 복합경유양돌기후미로및하미로경정맥경후두과경결절상위경부접근법 (combined transmastoid retrolabyrinthine and infralabyrinthine transjugular transcondylar transtubercular high cervical approach)[21] 등아주다양한복합접근법들이있다. 본연구진들은경정맥공종양에대한접근법을극외측접근법, 후외측접근법, A형측두하와접근법및복합접근법으로나누었고종양의위치및크기에따라접근법을결정하였다. A형종양은극외측경후두과접근법 (far lateral transcondylar approach) 으로접근하였고, B2형은후외측접근법및극외측경후두과접근법로접근하여제거할수있었다. B3형과 D형은복합접근법이필요하였다. 이번연구에서 B1형과 C형은없었으나, B1형과 C형의경우 A 형측두하와접근법을이용할수있을것으로판단된다. 복합접근법을선택할때내경동맥과닿아있는경우는 A형측두하와접근법을조합하였고내경동맥을 90도이상둘러싸면서종양이자란경우안면신경브릿지기술 (fallopian bridge technique) 을 이용하였다. 추체사대부위 (petroclival area) 까지종양이자란경우 는경와우접근법을조합하였다. 후외측접근법에 A 형측두하와접근법등을결합했을경우경정 맥공을더광범위하게노출시킬수있었고 (Fig. 8), 그결과좀더 안전하게신경및혈관구조물, 주변구조물을살리면서종양을최 Fig. 7 The 45 years old male patient presented with hearing loss and facial paralysis, a type B2 jugular foramen schwannoma was found on magnetic resonance imaging. The tumor was removed through a posterolateral approach. The tumor was well removed and the patient was undergoing rehabilitation because of the worsening facial palsy after surgery. Fig. 8 Jugular foramen exposure by combined approach. The arrow indicates the postero-medial side of jugular foramen exposed by posterolateral approach. The dotted arrow indicates the superolateral side of jugular foramen exposed by infratemporal fossa approach type A. The double-line arrow indicates the area exposed by the combined approaches. 23
대한제거할수있었다. 신경외과와이비인후과의협업을통한복합접근법을이용하면경정맥공을 2/3이상넓게노출시킬수있고보다안전한종양의제거를가져올수있음을이번연구를통해서알수있었다. CONCLUSION 경정맥공종양의성공적인제거를위해서는종양의임상적, 영상의학적, 해부학적특징에대한철저한이해가필요하다. 신경을보전하면서종양을최대한제거하기위해서는다양한두개저기법의활용이필요하며이를위해서여러과의협동수술및미세수술기법의끊임없는숙련이요구된다. 우리는두개저미세수술기법및여러과의협동수술을통해서성공적으로경정맥종양이제거될수있음을알수있었다. References 1. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, Pfeilsticker LN, Coelho Neto M. Tumors of the jugular foramen: diagnosis and management. Neurosurgery 2005;57:59-68. 2. Inserra MM, Pfister M, Jackler RK. Anatomy involved in the jugular foramen approach for jugulotympanic paraganglioma resection. Neurosurg Focus 2004;17:E6. 3. Lim M, Gibbs IC, Adler JR, Jr., Chang SD. Efficacy and safety of stereotactic radiosurgery for glomus jugulare tumors. Neurosurg Focus 2004;17:E11. 4. Tomasello F, Conti A. Judicious management of jugular foramen tumors. World Neurosurg 2015;83:756-7. 5. Patel SJ, Sekhar LN, Cass SP, Hirsch BE. Combined approaches for resection of extensive glomus jugulare tumors. A review of 12 cases. J Neurosurg 1994;80:1026-38. 6. Sanna M, Shin SH, Piazza P, Pasanisi E, Vitullo F, Di Lella F, et al. Infratemporal fossa approach type a with transcondylar-transtubercular extension for Fisch type C2 to C4 tympanojugular paragangliomas. Head Neck 2014;36:1581-8. 7. Fisch U. Infratemporal fossa approach to tumours of the temporal bone and base of the skull. J Laryngol Otol 1978;92:949-67. 8. Sanna M, Mazzoni A, Saleh EA, Taibah AK, Russo A. Lateral approaches to the median skull base through the petrous bone: the system of the modified transcochlear approach. J Laryngol Otol 1994;108:1036-44. 9. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, Pfeilsticker LN, Neto MC, et al. Jugular foramen tumors: diagnosis and treatment. Neurosurg Focus 2004;17:E5. 10. Sanna M, Khrais T, Menozi R, Piaza P. Surgical removal of jugular paragangliomas after stenting of the intratemporal internal carotid artery: a preliminary report. Laryngoscope 2006;116:742-6. 11. Sekhar LN, Tzortzidis FN, Bejjani GK, Schessel DA. Saphenous vein graft bypass of the sigmoid sinus and jugular bulb during the removal of glomus jugulare tumors. Report of two cases. J Neurosurg 1997;86:1036-41. 12. Makek M, Franklin DJ, Zhao JC, Fisch U. Neural infiltration of glomus temporale tumors. Am J Otol 1990;11:1-5. 13. Samii M, Alimohamadi M, Gerganov V. Surgical treatment of jugular foramen schwannoma: surgical treatment based on a new classification. Neurosurgery 2015;77:424-32. 14. David CA. Preoperative planning and surgical approaches to tumors of the jugular foramen. Oper Tech Neurosurg 2005;8:19-24. 15. Komune N, Matsushima K, Matsushima T, Komune S, Rhoton AL, Jr. Surgical approaches to jugular foramen schwannomas: An anatomic study. Head Neck 2016;38 Suppl 1:E1041-53. 16. Bruneau M, George B. The juxtacondylar approach to the jugular foramen. Neurosurgery 2008;62:75-8. 17. Dallan I, Bignami M, Battaglia P, Castelnuovo P, Tschabitscher M. Fully endoscopic transnasal approach to the jugular foramen: anatomic study and clinical considerations. Neurosurgery 2010;67:ons1-7. 18. Guinto G, Kageyama M, Trujillo-Luarca VH, Abdo M, Ruiz-Than A, Romero- Rangel A. Nonglomic tumors of the jugular foramen: differential diagnosis and prognostic implications. World Neurosurg 2014;82:1283-90. 19. Oghalai JS, Leung MK, Jackler RK, McDermott MW. Transjugular craniotomy for the management of jugular foramen tumors with intracranial extension. Otol Neurotol 2004;25:570-9. 20. Mazzoni A, Sanna M. A posterolateral approach to the skull base: the petrooccipital transsigmoid approach. Skull Base Surg 1995;5:157-67. 21. Liu JK, Sameshima T, Gottfried ON, Couldwell WT, Fukushima T. The combined transmastoid retro- and infralabyrinthine transjugular transcondylar transtubercular high cervical approach for resection of glomus jugulare tumors. Neurosurgery 2006;59:ONS115-25. 24 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2