검안및콘택트렌즈학회지 2019 년제 18 권제 1 호 nn Optom Contact Lens 2019;18(1):27-31 ISSN 2384-0919 (Print) ISSN 2384-0927 (Online) Case Report 통증을동반한외전마비로나타난경막동맥해면정맥동루 1 예 Cavernous Sinus Dural rteriovenous Fistula Presenting as Painful Isolated bducens Nerve Palsy 김다영 박유연 박신혜 Da-Yeong Kim, MD, Yooyeon Park, MD, Shin Hae Park, MD, PhD 가톨릭대학교서울성모병원안과학교실 Department of Ophthalmology, Seoul St. Mary s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea Purpose: To report a case of cavernous sinus dural arteriovenous fistula who presented with painful isolated abducens nerve palsy along with the characteristic conjunctival features. Case summary: 71-year-old female was referred to our clinic due to left eye pain and binocular diplopia. Esodeviation of 20 prism diopters was found with abduction limitation in the left eye. Slit lamp examination showed conjunctival injection, edema and corkscrew vessel dilatation in the left eye. rain magnetic resonance angiography showed rapid filling and increased blood flow in the left superior ophthalmic vein and cavernous sinus. Cerebral angiography revealed direct venous drainage from the left cavernous sinus fistula into the left superior ophthalmic vein. fter coil embolization, the left eye showed improvement in pain, conjunctival congestion and abduction limitation. Conclusions: Cavernous sinus dural arteriovenous fistula should be considered when characteristic symptoms and conjunctival signs are accompanied with painful isolated abducens nerve palsy. nn Optom Contact Lens 2019;18(1):27-31 Key Words: bducens nerve palsy; Ophthalmoplegia, painful 서 론 한쪽안구주위통증을동반한외안근마비는드물지만신속한치료가필요한질환이원인일가능성이있으므로원인질환의감별이중요하다. 1 외안근마비와함께안구통증을유발하는질환은해부학적으로동안신경과삼차신경의다섯번째분지에동시에영향을줄수있는해면정맥동이 Received: 2019. 3. 12. Revised: 2019. 3. 19. ccepted: 2019. 3. 19. ddress reprint requests to Shin Hae Park, MD, PhD Department of Ophthalmology, Seoul St. Mary s Hospital, College of Medicine, The Catholic University of Korea, #222 anpo-daero, Seocho-gu, Seoul 06591, Korea Tel: 82 2 2258 6204, Fax: 82 2 599 7405 E mail: vaccine@catholic.ac.kr 나상안와열의병변이발생하는질환을고려해야한다. 예를들면해면정맥동, 위안와틈새또는안와꼭지부위의종양이나혈관질환, 안와주위육아종염증인톨로사-헌트증후군, 감염, 당뇨혹은특발안신경마비등이있다. 2 그중에서경막동맥해면정맥동루 (cavernous sinus dural arteriovenous fistulas) 는외경동맥또는내경동맥의뇌막분지 (meningeal branch) 와해면정맥동내에서비정상적으로발생하는동정맥간의연결을보이는혈관질환으로폐경기여성에서많이발생하는것으로알려져있다. 3 경막동맥해면정맥동루의증상은결막부종 (chemosis), 안구돌출 (exophthalmos), 안구통, 시력감소등의안구및안와증상뿐만아니라안검하수, 복시등의해면정맥동을지나는뇌신경들의장애, 경련및뇌출혈등의대뇌증상까지다양하게나타난다. 4 하지만경막동맥해면정맥동루는혈류량이많지않아동정맥루부근의잡음이잘들리지않고상공막 Copyright 2019, The Korean Optometry Society The Korean Contact Lens Study Society nnals of Optometry and Contact Lens is an Open ccess Journal. ll articles are distributed under the terms of the Creative Commons ttribution 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. 27
- 검안및콘택트렌즈학회지 2019 년제 18 권제 1 호 - 혈관확장및외전장애이외의소견은뚜렷하지않기때문에의심하지않으면진단이쉽지않으며만성결막염, 외전신경마비, 녹내장, 공막염, 봉와직염, 상공막염등으로오진하여치료가지연되는경우가많다. 5 이에저자들은통증을동반한외전신경마비환자에서특징적인결막증상및소견을통해경막동맥해면정맥동루를진단하고치료하였던경험을보고하고자한다. 증례 71세여자환자가 1주전부터시작된좌안통증을동반한양안복시를주소로내원하였다. 두통이나이명증상은 동반되지않았다. 고혈압으로약물을복용하였고좌안에백내장수술을시행받았으며안구및두경부외상을비롯한기타특이한과거력은없었다. 우안나안시력 0.8, 좌안나안시력 0.5, 좌안교정시력 0.8이었으며, 안압은우안 18 mmhg, 좌안 26 mmhg로좌안의안압이다소증가되어있었다. 통증을동반한좌안의중등도결막충혈이있었는데, 세극등현미경검사에서결막부종과동반되어나선모양 (corkscrew-like appearance) 의상공막혈관들이윤부근처까지확장된소견을보였고결막유두 (papillae) 나여포 (follicle) 는발견되지않았다 (Fig. 1). 좌안에서중등도의외전장애와교대프리즘가림검사에서원거리약 20프리즘디옵터 (prism diopter, PD) 의내편위가관 Figure 1. Clinical findings at initial presentation before coiling. () Slit lamp photographs showing conjunctival swelling and dilated corkscrew conjunctival and episcleral vessels extending to limbus. () Gaze photographs show limitation in abduction of left eye. 28
- 김다영외 : 경막동맥해면정맥동루 1 예 - 찰되었다 (Fig. 1). 상대구심동공운동장애는없었고안구돌출계측치는우안 11 mm, 좌안 11 mm였다. Fig. 1에서관찰된결막소견과안운동검사에근거하여좌측의 6번뇌신경마비를동반한경막동맥해면정맥동루를의심하였고, 뇌자기공명혈관조영술에서좌측상부안와정맥및좌측해면동맥의혈류가증가되어있었으며뇌혈관조영술에서좌측해면정맥동으로루를형성하면서좌측상안정맥으로정맥류가흘러들어가는고신호강도가관찰되 었다. 좌측하추체정맥동은조영되지않았다 (Fig. 2). 이에근거하여좌측경막동맥해면정맥동루로최종진단하였고본원신경외과에서병변에대한색전술을성공적으로시행하였다. 이후경과관찰 7개월째우안나안시력 0.8, 좌안나안시력 1.0으로호전되었으며경도의좌안외전장애가관찰되었고교대프리즘가림검사에서 6-12 PD로좌측내편위감소되었다. 좌안의통증역시호소하지않았으며세극등현 Figure 2. () rain magnetic resonance angiography and () brain time-of-flight magnetic resonance image showing increased signal intensity in the left cavernous sinus, indicated by yellow arrows. Figure 3. Clinical findings after 7 months. () Slit lamp photograph showing resolution of conjunctival swelling and injection, engorged vessels. () Gaze photographs show remain of limitation in abduction of left eye. 29
- 검안및콘택트렌즈학회지 2019 년제 18 권제 1 호 - 미경검사상결막충혈및부종또한개선되었다 (Fig. 3). 고찰 저자들은통증을동반한외안근마비환자에서특징적인결막소견에근거하여경막동맥해면정맥동루를빠르게진단하고병변에대한색전술을시행하여성공적으로호전된증례를경험하였다. 본증례는중년여자환자에서통증을동반한외안근마비및결막충혈로발현한경막동맥해면정맥동맥루를진단하고병변에대한색전술을시행한후에증상이호전된경우였다. 본증례와같이단안의통증을동반한외안근마비를보일경우고려해야할원인질환으로는해면정맥동, 위안와틈새또는안와꼭지부위에발생하는종양이나혈관질환, 안와주위육아종염증인톨로사-헌트증후군, 감염, 당뇨혹은특발안신경마비, 갑상선안병증등이있으며 (Table 1) 원인질환에따라치료및질병경과가다양하기때문에각각의감별질환의특징적인임상양상을숙지하는것이진단및치료를신속하게하는데에도움이될수있다. 1-3 경막동맥해면정맥동루는주로외상에의해발생하는직접경동맥해면정맥동루와달리자발적으로발생하거나동맥경화, 고혈압, 교원성혈관질환등에의해발생하며저혈류형이많고임상증상의변화가느리고만성적이다. 5 증상은대개편측으로나타나나드물게양측으로나타나는경우도있다. 6 경막동맥해면정맥동루는임상양상이경미하면만성결막염으로오진하는경우가많으며, 그외에도공막염, 상공막염, 연조직염, 가성종양등으로오진된경우가보고되고있다. 5 경막동맥해면정맥동루와연관된주된증상및소견은외안근마비로인한안구운동제한, 결막충혈및부종, 박동성잡음, 복시가대표적이다. 그중에서상공막정맥이동맥화로인하여나선모양을이루며굵어진소견은만성결막염과감별할수있는특징적인소견이며, 이는본증례에서확인할수있었다. 7-8 경막동맥해면정맥동루는다른위치에생긴경막동정맥루에비해자연경과가양호한것으로알려져있으며자발적으로관해되는경우도상당수에도관찰되지만진행하는안압증가, 지속적인복시등의증상및신경학적장애가있는경우치료대상이되며, 주로혈관내치료가치료의근간을이루고있다. 9 경막동맥해면정맥동루의치료목표는완전한누공의폐쇄와내경동맥의혈류를보존하는것이다. 과거에는직접결찰이전형적인치료방법이었으나현재는치료방사선학의발달로혈관색전술을시행하여누공을폐쇄시키는것이널리이용되고있다. 본증례에서는일차적으로미세코일을이용하여대퇴정맥및내경정맥을 Table 1. Differential diagnosis of painful ophthalmoplegia 1 Neoplastic Primary parasellar tumors Pituitary adenomas Meningiomas Craniopharyngioma Epidermoid Chordoma Chondrosarcoma Local or distant metastases (to the orbit or cavernous sinus) Lymphoma Squamous cell carcinoma Nasopharyngeal tumors reast, prostate, melanoma, and other organ tumors Infectious Fungal (mucormycosis, actinomycosis) Mycobacterial (tuberculosis) acterial (extension from sinusitis, cellulitis, otitis, mastoiditis, and dental abscess) Vascular Carotid dissection Intracavernous carotid artery aneurysm Posterior communicating, basilar, or large posterior cerebral artery aneurysms Carotid-cavernous fistula Carotid-cavernous thrombosis Cavernous angioma Inflammatory Orbital pseudotumor Sarcoidosis Tolosa-Hunt syndrome Giant cell arteritis Thyroid ophthalmopathy Wegener s granulomatosis Idiopathic hypertrophic pachymeningitis Other Diabetic neuropathy Trauma Ophthalmoplegic migraine 통하여하추체정맥동으로접근하여색전술을시도하였다. 색전술에실패하였을경우에는누공에대한신경외과적수술이나방사선치료, 색전술과방사선치료의병용치료를시행할수있다. 10-12 통증을동반한외안근마비환자의감별진단을숙지하는것이중요하며특징적인결막충혈및부종의소견이동반된경우, 경막동맥해면정맥동루의가능성을고려해야한다. 30
- 김다영외 : 경막동맥해면정맥동루 1 예 - REFERENCES 1) Jonathan PG. n pproach to the patient with painful ophthalmoplegia, with a focus on tolosa-hunt syndrome. Curr Pain Headache Rep 2007;11:317-25. 2) Stephen DS, Frederick GF, Todd DR, et al. Headache Classification Committee of the International Headache Society. The international classification of headache disorders, 3rd ed.(beta version). Cephalalgia 2013;33:629-808. 3) Meyers PM, Halbach VV, Dowd CF, et al. Dural carotid cavernous fistula: definitive endovascular management and long-term follow -up. m J Ophthalmol 2002;134:85-92. 4) Suh DC, Lee JH, Kim SJ, et al. New concept in cavernous sinus dural arteriovenous fistula: correlation with presenting symptom and venous drainage patterns. Stroke 2005;36:1134-9. 5) Jang JW, Lee SW, Kim SJ. Clinical Characteristics of Dural Cavernous Sinus Fistula. J Korean Ophthalmol Soc 2000;41:737-43. 6) Haugen OH, Sletteberg O, Thomassen L, Krakenes J. ilateral non-traumatic carotid cavernous sinus fistula with spontaneous closure. cta Ophthalmol 1990;68:743-7. 7) Kim I, Yang S, Lee H, et al. Cavernous sinus dural arteriovenous fistula mimicking Tolosa-Hunt syndrome. J Korean Neurol ssoc 2014;32:218-21. 8) hn JH, Jung JH, Choi KD, Choi HY. The clinical characteristics and endovascular management outcomes of dural carotid cavernous fistulas. J Korean Ophthalmol Soc 2011;52:332-7. 9) Kim MS, Han DH, Kwon OK, et al. Clinical characteristics of dural arteriovenous fistula. J Clin Neurosci 2002;9:147-55. 10) Day JD, Fukushima T. Direct microsurgery of dural arteriovenous malformation type carotid-cavernous sinus fistulas: indications, technique, and results. Neurosurgery 1997;41:1129-24; discussion 1124-6. 11) Hirai T, Korogi Y, aba Y, et al. Dural carotid cavernous fistulas: role of conventional radiation therapy-long-term results with irradiation, embolization, or both. Radiology 1998;207:423-30. 12) Hasuo K, Mizushima, Matsumoto S, et al. Type D dural carotid-cavernous fistula. Results of combined treatment with irradiation and particulate embolization. cta Radiol 1996;37(3 pt 1): 294-8. 31