KISEP KOR J CEREBROVASCULAR SURGERY March 2003 Vol. 5, No 1, page 5-11 무증후성혈관기형의처치 * 계명대학교의과대학신경외과학교실, 뇌연구소 임만빈 Management of Asymptomatic Vascular Ma

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1 KISEP KOR J CEREBROVASCULAR SURGERY March 2003 Vol. 5, No 1, page 5-11 무증후성혈관기형의처치 * 계명대학교의과대학신경외과학교실, 뇌연구소 임만빈 Management of Asymptomatic Vascular Malformation Man-Bin Yim, MD Department of Neurosurgery and Brain Research Institute, Keimyung University School of Medicine, Daegu, Korea ABSTRACT Appropriate clinical decision making for the management of any asymptomatic vascular malformations requires an accurate assessment of the natural history of the lesions and the risks inherent in the treatment of the condition. To obtain the more information about decision making for the management of asymptomatic vascular malformation, this article discusses about guideline of the management of those lesions based on the author s experiences and review of the literatures. (Kor J Cerebrovascular Surgery 5:5-11, 2003) KEY WORDS:Vascular malformation Asymptomatic Management. 서 계명대학교의과대학신경외과학교실, 뇌연구소 전화 :(053) , 7356 전송 :(053) y760111@dsmc.or.kr 론 재료및방법 5

2 결 1. 뇌동정맥기형 1) 자연력과무증상예의치료 과 Percent symptomatic Age (years) Percentage probability of bleeding % 3% Age in years A B C Percentage probability of mortality and morbidity Morbidity 2% Mortality 2% 3% 3% Age in years Fig. 1. Graph showing the correlation of the first appearance of symptoms with the age A and the percentage probability of bleeding B and of mortality and morbidity C in patients with cerebral arteriovenous malformation, assuming bleeding and rebleeding rates of 2% to 3% yearly. 16) A B C D Fig. 2. T1W1 MRI, axial view of the 49-year old female patient, showing low signal mass at right temporal lobe A. Right internal carotid artery ICA angiogram, A-p B view, showing small arteriovenous malformation AVM at right temporal lobe. She refused treatment with surgery or radiosurgery for that lesion. She was admitted again with comatose state suddenly about 1 year after diagnosis of the AVM. Brain CT, taken at that time, showing large amount of hematoma at right temporal lobe C. Postoperative ICA angiogram, A-p D view, showing complete disappearance of the AVM. 6 Kor J Cerebrovascular Surgery 5:5-11, 2003

3 Table 1. Clinical summary of surgical AVMs cases presenting with headache or incidentally discovered No Age Sex Location Size Surgical result Abnormal gene expression and suppression 1 63 M Parietal 3 cm No neurological deficit 2 50 M Parietal 4 cm No neurological deficit 3 35 F Cerebellum 3 cm No neurological deficit One time seizure after 4* 49 F Temporal 2 cm surgery 5 46 M Occipital 6 cm No neurological deficit Venous malformation Cavernous malformation precursor Capillary telangiectasia 6 35 M Sylvian 2 cm No neurological deficit Incidentally discovered, but resected the AVM after hemorrhage * * Cavernous malformation * 2. 해면상혈관종 1) 정의, 발생및자연력 Clinical manifestation Fig. 3. Hypothetical schema regarding the genesis and proliferation of the CM. It is postulated that venous malformations and capillary malformations telangiectasia may be the result of mutation and abnormal gene expression or suppression. These lesions may subsequently predispose to the development of associated cavernous malformations, 1) hemorrhagic angiogenic proliferation. * Kor J Cerebrovascular Surgery 5:5-11,

4 2) 치료방법의선택 Slow ooze and the hemosiderin ring Brain irritation Seizure Focal neurologic deficit (?) Intralesional hemorrhage Gross hemorrhage and lesion expansion beyond lesion Lesion expansion, Increase focal turgor Focal neurologic deficit Headache (?) Seizure (?) Containe or non-contained hemorrhage into surrounding brain Focal neurologic deficit Hemorrhagic stroke Headache (acute) Seizure Fig. 4. Pathophysiologic phenomena associated with cavernous angioma. 23) A1 A2 B1 B2 Fig. 5. The T1W1 weighted MRI, axial view of the 19-year-old female patient who presented with right occulomotor nerve palsy, showing a high signal mass at interpeducular portion of the midbrain. This mass was removed through right trans-sylvian approach and diagnosed as cavernous angioma A1 & 2. The T1W1-weighted MRI axial view, taken about 2 years after operation due to sudden onset of headache, showing recurrence of cavernous angioma at the same site B1 & 2. 8 Kor J Cerebrovascular Surgery 5:5-11, 2003

5 A1 A2 B1 B2 Fig. 6. T1-weighted enhanced axial MRI A1 & A2 demonstrates a typical appearance of a cavernous angioma at right temporal Heschl s gyrus, heterogeneous central high signal intensity and peripheral hypointensity rim. Immediate postoperative follow-up CT showing disappearance of the lesion. Olive Hypoglossal nerve Pyramid Decussation of corticospinal tract C1 Cochlear nucleus Dorsal motor nocle of vagus Area of the nucleus ambiguus Medial longitudinal fasciculus Nucleus of hypoglossal nerve Tralctus solitarius A B C App III CN Fig. 7. AVentral view of brainstem. arrowparamedian oblique access throuth the anterolateral sulcus, Bdorsal view of the brain stem. The two triangle, suprafacial and infrafacial triangle indicate the safe entry zones via the floor of the fourth ventricle for pons lesions, Can oblique section throuth the midbrain, passing throuth the rostral end of one inferior colliculus and the caudal end of the contralateral superior colliculus, demonstrating the nucleus and fibers of the oculomotor nerve. arrow indicates safe entry zone. Table 2. Surgical approaches and safe entry zone for brain stem cavernous angioma 3)5)14) Location Approach Safe entry zone Medulla Ventral Far lateral app. Paramedian oblique access through the anterolateral sulcus between XII CN and C1 N root Dorsal Median suboccipital app. Through the acoustic area Pons Ventral Combined petrosal app. V CN exit zone, 1cm from the midline to 1.2 cm widening laterally Dorsal Midbrain Median suboccipital app. 1 Paramedian 5 mm from the midline at the level of the suprafacial and/or infrafacial triangle 2 Median sulcus between the VI CN nuclei and the III CN nuclei Ventral Sylvian or subtemporal Boundary zone AbovePCA, belowsca, medialiii CN exit, lateralpyramidal tract Dorsal Supracerebellar Median intercollicular entry Infratentorial app. AbbreviationApp=approach, CN=cranial nerve, N=nerve, PCA=posterior cerebral artery, SCA=superior cerebellar artery Kor J Cerebrovascular Surgery 5:5-11,

6 Table 3. The locations of author s cases of cavernous angioma Supratentorial cases Frontal Parietal Temporal Infratentorial cases Cerebellum Brain stem midbrain4pons1 Multiple case Spinal cord T21T11-L cases Table 4. The treatment modalities and outcomes of author s cases of cavernous angioma Treatment Resection cases Conservative cases Radiosurgery case Ventriculo-peritoneal shunt case Outcome Good * Poor ** 2 cases of brain stem lesion recurred after surgical resection about 5 and 4 years later in each Complete paraplegia due to hemorrhage from the cavernous angioma at T11-L1 did not improved after resection of the lesion - 10 Kor J Cerebrovascular Surgery 5:5-11, 2003

7 결 중심단어 : REFERENCES 1) Barrow DL, Awad IA. Conceptual overview and management strategies, in Awad IA, Barrow DL (eds): Cavernous malformation. Park Ridge, Ill: American Association of Neurological Surgeons, 1993, pp ) Buhl R, Hempelmann RG, Stark AM, Mehdom HM. Therapeutical consideration in patients with intracranial venous angioma. Eur J Neurol 9: 165-9, ) Cantore G, Missori P, Santoro A. Cavernous angiomas of the brain stem. Intra-axial anatomical pitfalls and surgical strategies. Surg Neurol 52:84-94, ) Curling OD, Kelly DL, Elster AD, Craven TE. An analysis of the natural history of cavernous angiomas. J Neurosurg 75:702-8, ) England MA, Wakely J. A Colour atlas of the brain & spinal cord. London: Wolfe, 1991, p 154 6) Fults D, Kelly DL. Natural history of arteriovenous malformations of the brain: A clinical study. Neurosurgery 15:658-62, ) Graf CJ, Perret GE, Torner JC. Bleeding from cerebral arteriovenous malformations as part of their natural history. J Neurosurg 58:331-7, ) Hasegawa T, Mclnerney J, Kondziolka D, Lee JYK, Flickinger JC, Lunsford LD. Long-term results after stereotactic radiosurgery for patients with cavernous malformations. Neurosurgery 50:1190-8, ) Johnson PC, Wascher TM, Golfinos J, Spetzler RF. Definition and pathologic features, in Awad IA, Barrow DL (eds): Cavernous malformation. Park Ridge, Ill: American Association of Neurological Surgeons, 1993, pp ) Kim DS, Park YG, Choi JU, Chung SS, Lee KC. An analysis of the natural history of cavernous malformations. Surg Neurol 48:9-18, 1997 론 11) Kondziolak D, Lunsford LD, Kestle RW. The natural history of cerebral cavernous malformations. J Neurosurg 83:820-4, ) Kondziolka D, Lunsford D, Flickinger JC, Kestle RW. Reduction of hemorrhage risk after stereotactic radiosurgery for cavernous malformations. J Neurosurg 83:825-31, ) Kupersmith MJ, Hadas K, Fred E, Guopei Y, Alejandro B, Henry W, et al. Natural history of brain stem cavernous malformations. Neurosurgery 48:47-54, ) Kyoshima K, Kobayashi S, Gibo H, Kuroyanagi K. A study of safe entry zones via the floor of the fourth ventricle for brain-stem lesions. Report of three cases. J Neurosurg 78:987-93, ) Labauge P, Brunereau L, Laberge S, Houtteville JP. Prospective followup of 33 asymptomatic patients with familial cerebral cavernous malformations. Neuology 57:1825-8, ) Luessenhop AJ, Rosa L. Cerebral arteriovenous malformations. Part II. Contemp Neurosurg 11:1-6, ) Michelson WJ. Conservative management in Awad IA, Barrow DL (eds): Cavernous malformations. Park Ridge, Illinois: American Association of Neurological Surgeons, 1993, pp ) Moriarity JL, Wetzel M, Clatterbuck RE, Javedan S, Sheppard JM, Rigamonti KH, et al. The natural history of cavernous malformations: A prospective study of 68 patients. Neurosurgery 44: , ) Pollock BE, Garces YI, Stafford SL, Foote RL, Schomberg PJ, Link ML. Stereotactic radiosurgery for cavernous malformations. J Neurosurg 93:987-91, ) Porter RW, Detwiler PW, Spetzler RF, Lawton MT, Baskin JJ, Derksen PT, et al. Cavernous malformations of the brainstem: experience with patients. J Neurosurg 90:50-8, ) Rigamonti D, Hadley MN, Drayer BP, Johnson PC, Hoenig-Rigamonti K, Knight JT, et al. Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med 319:343-7, ) Robinson JR, Awad IA, Little JR. Natural history of the cavernous angioma. J Neurosurg 75:709-14, ) Robinson JR, Awad IA. Clinical spectrum and natural course in Awad IA, Barrow DL (eds): Cavernous malformations. Park Ridge, Illinois: American Association of Neurological Surgeons, 1993, pp ) Sami M, Eghbal R, Carvalho GA, Matthies C. Surgical management of brainstem cavernomas. J Neurosurg 95:825-32, ) Sandalcioglu IE, Wiedemayer H, Secer S, Asgari S, Stolke D. Surgical removal of brain stem cavernous malformations: Surgical indications, technical considerations, and results: J Neurol Neurosurg Psychiatry 72:351-5, ) Shah MV, Heros RC. Microsurgical treatment of supratentorial lesions in Awad IA, Barrow DL (eds): Cavernous malformations. Park Ridge, Illinois: American Association of Neurological Surgeons, 1993, pp ) Verlaan DJ, Davenport WJ, Stefan H, Sure U, Siegel AM, Rouleau GA. Cerebral cavernous malformations: Mutations in Krit 1. Neurology 58:853-7, ) Yim MB. Surgical management of large cerebral arteriovenous malformation. Kor J Cerebrovascular Disease 1:64-74, ) Zabramski JM, Wascher TW, Spetzler RF, Johnson B, Golfinos J, Drayer BP, et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 80:422-32, 1994 Kor J Cerebrovascular Surgery 5:5-11,

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