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1 online ML Comm 0CASE REPORT0 J Kor Neurotraumatol Soc 2009;5: ISSN 고립성제 4 뇌실수두증환자에서항법장치유도로시행한측부소뇌경유뇌실 - 복강단락술 : 증례보고 고려대학교의과대학구로병원신경외과학교실 정규하 김종현 김주한 박윤관 정흥섭 권택현 Navigation Assisted Lateral Transcerebellar Ventriculoperitoneal Shunt for Treatment of Trapped 4 th Ventricle Hydrocephalus Patient Kyuha Chong, MD, Jong Hyun Kim, MD, PhD, Joo Han Kim, MD, PhD, Youn Kwan Park, MD, PhD, Hung Seob Chung, MD, PhD and Taek Hyun Kwon, MD, PhD Department of Neurosurgery, Korea University College of Medicine, Guro Hospital, Seoul, Korea Trapped 4 th ventricle hydrocephalus (TFVH) is occasionally presented as a series of sequel which came after inflammatory condition of central nervous system (CNS). Treatment of TFVH with intervention may aid preservation of life and neurological function. This is a report of TFVH patient who underwent improvement of neurology without complication, after navigation assisted lateral transcerebellar ventriculo-peritoneal (V-P) shunt system application. A Thirty-four years-old, female came to hospital with gait disturbance and dizziness. She had history of V-P shunt operation at lateral ventricle because of hydrocephalus due to listeria meningitis. General work-up and imaging study performed, and no other specific new finding except enlargement of 4 th ventricle. The cause of dizziness and syncope was deduced as TFVH. With Stealth navigation system s assistance, lateral transcerebellar (LTC) V-P shunt had planned. With navigation system s guidance, proximal catheter insertion was performed with confirmation of clear cerebrospinal fluid (CSF) drainage. Then, using 3-way connector, proximal catheters located at lateral and 4 th ventricle was connected to form a one way out system. Two proximal catheters and one valve system finally set. Patient s symptoms and hydrocephalus were gradually improved with shunt pressure management without specific complications. Complication of brainstem penetration with LTC V-P shunt for the treatment of TFVH may be reduced with navigation assistance technique. So, we are expecting more easy concern of LTC approach for the treatment of TFVH, by this technique. (J Kor Neurotraumatol Soc 2009;5: ) KEY WORDS: Hydrocephalus Fourth ventricle Ventriculoperitoneal shunt Stereotactic technique. 서 론 고립성제 4뇌실수두증 (Trapped 4 th ventricle hydrocephalus: TFVH) 은루시카공 (foramina of Luschka), 마겐디공 (foramina of Magendie) 및중뇌수도관 (cerebral aqueduct) 의차단 (blockage) 에의해발생한다. TFVH 의 Received: July 13, 2009 / Revised: July 15, 2009 Accepted: September 23, 2009 Address for correspondence: Taek Hyun Kwon, MD, PhD Department of Neurosurgery, Korea University College of Medicine, Guro Hospital, 97 Gurodong-gil, Guro-gu, Seoul , Korea Tel: , Fax: 원인으로는감염및출혈등의중추신경계의염증상태가발생한이후, 합병증의일환으로때때로나타나는것으로알려져있다. TFVH 는루시카공, 마겐디공및중뇌수도관의차단발생이후제 4뇌실내의뇌척수액 (cerebrospinal fluid: CSF) 이정상적으로지속, 생산되면서제 4뇌실의낭성확장이진행되고, 이로인해후두와공간을점유하는기타질환과같은증상들이나타나게된다. 25) TFVH 는드문질환이나, 염증성질환유병및수두증치료를위한측뇌실뇌실-복강 (ventriculo-peritoneal: V-P) 단락술시행이후발생할수있는질환으로, 생존과신경학적기능의보존및호전을위해 TFVH 에대한다양 Copyright c 2009 Journal of Korean Neurotraumatology Society 109

2 Navigation Assisted V-P Shunt for Trapped 4 th Ventricle Hydrocephalus 한치료들이시도되고있다. 13,25) 저자들은항법유도장치를이용하여측부소뇌경유 (lateral transcerebellar: L- TC) V-P 단락술을통해증상의호전및기존 LTC 방법의단점을보완할수있었던 TFVH 환자의증례를경험하였기에문헌고찰과함께보고하는바이다. 증례 34세여자환자로한달전부터발생한어지럼증과보행장애를주소로내원하였다. 과거력상, 환자는내원 10개월전급성두통을주소로응급실을통해내원하여리스 테리아뇌수막염 (listerial meningitis) 진단하에치료를받았으며, 합병증으로발생한뇌수두증에대하여 V-P 단락술을시행받은병력이있었다. 당시뇌자기공명영상 (magnetic resonance imaging: MRI) 에서두개강내에특이소견은보이지않았으나 (Figure 1), CSF 검사결과백혈구 470/μL, 단백질 142 mg/dl, 포도당 38 mg/dl 소견을보였고, 이에따라경험적항생제를선투여하였다. 치료 2일째, 대발작 (generalized seizure) 을일으킨후글라스고우혼수척도 (Glasgow Coma Scale: GCS) 5점으로의식저하소견을보여시행한뇌확산자기공명영상 (diffusion MRI) 에서뇌수두증소견이관찰되었고 (Figure 2), FIGURE 1. Initial magnetic resonance imaging before 1 st ventriculoperitoneal shunt operation. There were no specific intracranial findings, except subgaleal hematoma at right parietal area, extracranial. The patient had minimal head trauma history, at about 1 month before imaging study. FIGURE 2. Diffusion magnetic resonance image after seizure and mental change. Diffuse enlargements of both lateral ventricle and 3 rd ventricle enlargement were identified, which were compatible with acute hydrocephalus. FIGURE 3. Computed tomography scan after 1 st external ventricular drainage procedure. Hydrocephalus was improved with procedure. 110 J Kor Neurotraumatol Soc 2009;5:

3 Kyuha Chong, et al. FIGURE 4. Computed tomography scan after 1 st ventriculoperitoneal shunt operation at right lateral ventricle. Hydrocephalus improvement was maintained after operation. A FIGURE 5. Imaging study at 2 nd admission. A: Computed tomography scan. 4 th ventricle enlargement was obviously identified. B: Magnetic resonance imaging after modulation of shunt system s valve pressure. The size of both lateral ventricles was improved, but 4 th ventricle was still enlarged with T2 FLAIR s high signal change around 4 th ventricle. FLAIR: fluid-attenuated inversion recovery. B 뇌실외배액술 (external ventricular drainage: EVD) 시행후 GCS 11점으로호전되었다 (Figure 3). EVD 시행및항생제치료 4주째 CSF 균동정 3회음성확인후 120 mmh 2 O의압력으로설정한 Codman adult type Hakim programmable valve system (Jonhson & Jonhson Co., Raynham, MA, USA) 을이용하여우측측뇌실 V-P 단락술을시행하였고, 수술후의식 (GCS 14점 ) 및방사선소견상뇌수두증소견이호전되어퇴원하였다 (Figure 4). 재내원후시행한이학적검사소견상, 안구진탕소견은관찰되지않았으나지속적으로어지럼증을호소하고있었고, 양측하지의조정 (coordination) 에있어보행시약한정도의장애와이전퇴원시와같은정도의양하지의위약 (Grade IV+) 소견이외에는의식상태 (GSC 15 점 ) 와체온은정상이었다. 내원후시행한뇌전산화단층촬영 (computed tomography: CT) 소견상이전입원및경과관찰 상관찰되지않았던최대직경 33 mm 의제 4뇌실확장소견이관찰되었다 (Figure 5A). 단락체계 (shunt system) 압력조절을 50 mmh 2 O까지시행하였으나, 지속적인제 4뇌실확장소견과함께증상호전이관찰되지않아뇌 MRI 를시행한결과 T2 fluid-attenuated inversion recovery (FLAIR) 영상에서제 4뇌실확장과주위의신호증강이관찰되었고, T1 영상소견에서중뇌수도관의확장없이양측뇌실과제 4뇌실의확장소견이관찰되어 (Figure 5B), TFVH 진단하에항법유도장치를이용하여우측측부소뇌경유 V-P 단락술을시행하였다. 본원에서는 Stealth Station TM Treon (Medtronics, Sofamor Danek, Memphis, TN, USA) 뇌항법유도장치 system 을사용하였다. 수술전환자의두피에표지자 (fiducial marker) 를붙인후 CT 촬영을시행하고, 전신마취하에좌측와위상태에서환자의두부를 Mayfield system 을이용하여고정하였다. 항법유도장치와환자간의 111

4 Navigation Assisted V-P Shunt for Trapped 4 th Ventricle Hydrocephalus 정위적위치보정및계산을시행한후, 제 4뇌실내목표위치를계산하였다. 중앙에서우측으로 3 cm, 뒤통수점 (inion) 에서하방 2 cm 위치가뒤통수정맥동 (occipital sinus) 과가로정맥동 (transverse sinus) 을피하고제 4뇌실내에잘고정될수있는적합한천두공위치로항법유도장치상계산되었다. 근위도관삽입은, 항법유도장치를이용하여, 이전우측측뇌실 V-P 단락술에서시행했던것과동일한도관을삽입하였다. 목표경로선상의후두부두개골외피질로부터총 45 mm ( 제 4뇌실내 1 cm) 를항법유도하에삽입한후, 유도철사 (guide wire) 를제거하자투명한무색의 CSF 가높은압력으로배액되었다. 소뇌부의근위도관을겸자를이용하여고정한뒤, 이전 우측부근위도관과밸브를분리하고, 우측부근위도관에서의투명한 CSF 배액을확인하였다. 이후 Y 자모양의연결관을이용하여연결관근위부의 2개의관과우측부와소뇌부의근위도관을각각연결하고, 연결관원위부의 1개의관과밸브를연결하여 2개의근위도관-1개의밸브 단락체계를 120 mmh 2 O 만들고수술을마쳤다 (Figure 6). 수술시배액된 CSF 검사상특이소견은관찰되지않았다. 수술후부터환자의어지럼증은점차적으로호전되었고밸브압력을조절 (100 mmh 2 O) 하여수술후약 6주에어지럼증은완전히없어졌고, 8주째보행장애도호전되었다. 8주째시행한 CT 소견에서도확장되었던제 4뇌실, 양측측뇌실과제 3뇌실등이모두정상범위로되었 FIGURE 6. Skull simple X-ray after 2 nd shunt operation. Using 3-way connector, proximal catheters located at lateral and 4 th ventricle was connected to form a one way out system. Two proximal catheters and one valve system finally set. A B FIGURE 7. Post 2 nd shunt operation computed tomography scan. The trapped 4 th ventricle was improved without penetration of brain stem, and hydrocephalus was successfully improved. A: 6 weeks later. B: 8 weeks later. 112 J Kor Neurotraumatol Soc 2009;5:

5 Kyuha Chong, et al. 다. 제 4뇌실에삽입하였던도관은제 4뇌실내에뇌간 (brain stem) 에특별한손상을주거나관통소견없이잘위치하고있었고, 출혈등의다른특이합병증소견은관찰되지않았다 (Figure 7). 고찰 루시카공과마겐디공폐쇄의임상 병리학적소견및양상은 Dandy에의해 1921년처음보고되었다. Dandy는선천성 (congenital) 과염증후 (post inflammatory) 유형으로구분하였으며, 소뇌에공간을점유하는병변중하나로기술하였다. 종양이아닌제 4뇌실의확장으로두통, 오심, 구토및유두부종과같은두개내압력증상과함께, 후두와종양에서와같은조화운동불능 (ataxia), 안구진탕, 변화운동곤란증 (dysdiadochokinesis) 및운동거리조절이상 (dysmetria) 등의소뇌증상이관찰되었고, 후두와제 4뇌실의감압후증상이소실되었음을보고하였다. 8,25) CSF 는뇌실계전반의맥락총 (choroid plexus) 에서형성되어루시카공과마겐디공을경유하여제 4뇌실을통해빠져나간다. 마겐디공의등부 (dorsal) 는소뇌와충부 (vermis) 의미부확장 (caudal extension) 으로형성되고, 배부 (ventral) 와측부는연수 (medullar) 의빗장 (obex) 으로이루어져있다. 마겐디공은정상적인구조에서대수조 (cistern magna) 로연결되는데, 일부에서는이경로가폐쇄되어있기도한다. 22) Chiari 제 1형기형의경우제 4뇌실의특발성완전출구폐쇄 (complete idiopathic outlet obstruction) 로수두증을유발하게되는데, 이경우에는중뇌수도관의병변은동반하지않는다. 16) 하지만 TFVH 의경우근위부로는중뇌수도관, 원위부로는마겐디공과루시카공의폐쇄로제 4뇌실의출구폐쇄가형성된다. TFVH 에서중뇌수도관의폐쇄를유발하는임상적기전은대부분감염이나출혈같은뇌실계의심각한염증반응을포함하는데, 염증반응에대한치료후전반적인뇌실계의감압이이루어지면서중뇌수도관의벽이나란히붙게되고뇌실막의염증이남아있는상태에서유착및협착이발생하게된다. 제 4뇌실의맥락총에서지속적으로 CSF 가생성되고, 구상밸브 (ball valve) 와같이중뇌수도관을통해단일방향으로 CSF 가유입, 축적되어점진적인제 4뇌실의확장을일으키게된다. 13) TFVH를일으킬수있는염증요인으로감염과뇌실내출혈이그주된요인이나, 1) 선천성요인-Dandy-Walker 기형, 2) 출혈후상태 -뇌실내출혈, 뇌지주막하출혈, 3) 감염그리 고감염후상태 -세균성뇌막염과뇌실염, 항산균성결핵, 콕시디오이데스 (coccidiomycosis) 와캔디다 (candida) 등의진균증, 낭미충증 (cysticercosis) 등의기생충감염, V-P 단락술이후감염, 4) 염증후상태-사코이드증 (sarcoidosis), 5) 종양 -암종수막염(carcinomatous meningitis) 등의다양한원발요인들이보고되고있다. 6,10,12-14,18) TFVH 의치료로다양한방법들이제시되고있으나, 높은합병증발생률과실패율로인해많은고려가지속되고있다. 이상적인치료목표로영구적이식물을되도록피하면서도낮은합병증발생률과높은성공률을유지할수있는방법들이강구되고있으나, 실제임상에서이같은목표를획득하기는어려운상태이다. 따라서주요합병증, 중복시술및복합단락체계형성등을피하는것을실질적목표로하고있으며, 이를위해환자에따른단락술, 내시경적시술, 뇌실경및개두술등의병합치료가제시되고있다. 2,4,5,13,17,19,24) 각각의장점도있으나 TFVH 치료에있어, 대부분의 TFVH 환자들의경우교통성또는폐쇄성의수두증병력으로단락술을시행받은과거력을가지고, 내시경과개두술의경우단락술과같은 CSF 전환술에비해현재까지높은이환율과불확실한효용성을보이며, 시술의간편정도를이유로단락술이많이고려되고있다. 2,15,5,13,20) CSF 전환을목적으로시행하는단락술의방법으로는 1) 소뇌경유 (transcerebellar), 2) 중뇌수도관경유 (transaqueductal), 3) 마겐디공경유 (transforaminal, Magendie), 4) 피질경유 (transcortical) 등의방법이있다. 이들방법중소뇌경유단락술의경우, 제 4뇌실을직접적인목표로하여다른종류의단락술에비해적용이용이하고단락술도관삽입의길이가다른종류의단락술에비해짧은장점을가지나, 삽입경로가제 4뇌실바닥과비교적수직에가까운관계로단락술이후감압에의한소뇌와뇌간의팽창및제 4뇌실의축소로인해삽입한도관의뇌간관통이일어날확률이다른종류의단락술에비해높은단점을가지고있다. 7,17,23) 뇌항법유도장치체계는수술중에실시간화상정보를제공하고, 기존의틀을이용한정위적시술이가지는수술가능부위의공간적제한성을극복할수있다는장점을가지고있어신경외과분야에서사용이증가하고있는장비체계이다. 1,3,9) 조작이간단하고비교적안전하며, 수술중공간적제약없이정확한해부학적정보를제공하여, 심부를포함한두개강내종양수술및, 조직생검, 뇌실조루술 (venticulostomy), 농양및혈종배액술등을비롯하여 V-P 단락술에도적용되고있다. 3,9,11) 저자들은환자의 113

6 Navigation Assisted V-P Shunt for Trapped 4 th Ventricle Hydrocephalus 경우우측측뇌실에 V-P 단락술을시행한환자로, 시술의용이성과뇌항법유도장치의정확성을고려하여항법장치유도하에측부소뇌경유 V-P 단락술및단락조정 (shunt modification) 을시행하였다. 측뇌실또는제 4뇌실에단일단락술을시행할경우양뇌실간의압력차이및상방또는하방탈출 (herniation) 가능성증가등의이유로, 2) Raimondi 등 21) 은방사선, 압력, 병리등의다방면에걸친연구를통해측뇌실과제 4 뇌실단락술을동시에시행할것을제안하였다. 이에따라 TFVH 발생이전환자의소뇌및제 4뇌실크기를참고하여제 4뇌실내근위도관삽입길이및위치를계산하였으며, 단락술시행후양측뇌실과제 4뇌실간의압력차이로상방또는하방탈출가능성을보정하기위해이전에시행한우측뇌실근위단락관과제 4뇌실근위단락관을하나의밸브에 Y 자형태의연결관을이용하여단일밸브체계를형성하였고, 뇌간관통의합병증없이성공적인단락술결과및증상호전결과를얻을수있었다. 결 론 현재까지다양한 TFVH 환자의치료방법이제시되고있다. 측부소뇌관통접근을통한단락술은효과적이면서도비교적간단한술기로시행할수있으나, 삽입한도관이뇌간을관통할수있는단점을가지고있었다. 저자들은 TFVH 치료를위하여, 항법유도장치를이용한계산및유도로단락술시행후뇌간관통및특별한합병증없이성공적인제 4뇌실의수두증회복을경험하였다. 이에항법유도장치를이용한측부소뇌관통단락술을 TFVH 치료에있어용이하게시행할수있는치료방법으로고려할것을제안하는바이다. 중심단어 : 뇌수종 제 4뇌실 뇌실복강단락술 정위수술방법. REFERENCES 1) Alberti O, Dorward NL, Kitchen ND, Thomas DG. Neuronavigation--impact on operating time. Stereotact Funct Neurosurg 68:44-48, ) Asai A, Hoffman HJ, Hendrick EB, Humphreys RP. Dandy-Walker syndrome: experience at the Hospital for Sick Children, Toronto. Pediatr Neurosci 15:66-73, ) Barnett GH, Kormos DW, Steiner CP, Weisenberger J. Use of a frameless, armless stereotactic wand for brain tumor localization with two-dimensional and three-dimensional neuroimaging. Neurosurgery 33: , ) Chai WX. Long-term results of fourth ventriculo-cisternostomy in complex versus simplex atresias of the fourth ventricle outlets. Acta Neurochir (Wien) 134:27-34, ) Coker SB, Anderson CL. Occluded fourth ventricle after multiple shunt revisions for hydrocephalus. Pediatrics 83: , ) Colli BO, Pereira CU, Assirati Júnior JA, Machado HR. Isolated fourth ventricle in neurocysticercosis: pathophysiology, diagnosis, and treatment. Surg Neurol 39: , ) Colpan ME, Savas A, Egemen N, Kanpolat Y. Stereotactically-guided fourth ventriculo-peritoneal shunting for the isolated fourth ventricle. Minim Invasive Neurosurg 46:57-60, ) Dandy WE. The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynecol Obstet 32: , ) Dorward NL, Paleologos TS, Alberti O, Thomas DG. The advantages of frameless stereotactic biopsy over frame-based biopsy. Br J Neurosurg 16: , ) Eller TW, Pasternak JF. Isolated ventricles following intraventricular hemorrhage. J Neurosurg 62: , ) Hall WA. The safety and efficacy of stereotactic biopsy for intracranial lesions. Cancer 82: , ) Harrison HR, Reynolds AF. Trapped fourth ventricle in coccidioidal meningitis. Surg Neurol 17: , ) Harter DH. Management strategies for treatment of the trapped fourth ventricle. Childs Nerv Syst 20: , ) Hesselmann V, Wedekind C, Terstegge K, Schulte O, Voges J, Krug B, et al. An isolated fourth ventricle in neurosarcoidosis: MRI findings. Eur Radiol 12 Suppl 3:S1-S3, ) Hubbard JL, Houser OW, Laws ER Jr. Trapped fourth ventricle in an adult: radiographic findings and surgical treatment. Surg Neurol 28: , ) Karachi C, Le Guérinel C, Brugiéres P, Melon E, Decq P. Hydrocephalus due to idiopathic stenosis of the foramina of Magendie and Luschka. Report of three cases. J Neurosurg 98: , ) Lee M, Leahu D, Weiner HL, Abbott R, Wisoff JH, Epstein FJ. Complications of fourth-ventricular shunts. Pediatr Neurosurg 22: , ) Lourie H, Shende MC, Krawchenko J, Stewart DH Jr. Trapped fourth ventricle: a report of two unusual cases. Neurosurgery 7: , ) Mohanty A. Endoscopic third ventriculostomy with cystoventricular stent placement in the management of dandy-walker malformation: technical case report of three patients. Neurosurgery 53: , ) Montgomery CT, Winfield JA. Fourth ventricular entrapment caused by rostrocaudal herniation following shunt malfunction. Pediatr Neurosurg 19: , ) Raimondi AJ, Samuelson G, Yarzagaray L, Norton T. Atresia of the foramina of Luschka and Magendie: the Dandy-Walker cyst. J Neurosurg 31: , ) Rakate HL. Hydrocephalus classification and pathophysiology, in McLone DG (ed): Pediatric neurosurgery: surgery of the developing nervous system. Philadelphia: Saunders, pp , ) Sharma RR, Pawar SJ, Devadas RV, Dev EJ. CT stereotaxy guided lateral trans-cerebellar programmable fourth ventriculo-peritoneal shunting for symptomatic trapped fourth ventricle. Clin Neurol Neurosurg 103: , ) Villavicencio AT, Wellons JC 3rd, George TM. Avoiding complicated shunt systems by open fenestration of symptomatic fourth ventricular cysts associated with hydrocephalus. Pediatr Neurosurg 29: , ) Zimmerman RA, Bilaniuk LT, Gallo E. Computed tomography of the trapped fourth ventricle. AJR Am J Roentgenol 130: , J Kor Neurotraumatol Soc 2009;5: