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1 대한안과학회지 2017 년제 58 권제 7 호 J Korean Ophthalmol Soc 2017;58(7): ISSN (Print) ISSN (Online) Case Report 성인코우츠병에서유리체강내라니비주맙주입술을시행한 1 예 Intravitreal Ranibizumab Injection in dult-onset Coats Disease: Case Report 김한얼 신성환 박성표 Han Ul Kim, MD, Seong Hwan Shin, MD, Sung Pyo Park, MD, PhD 한림대학교강동성심병원안과 Department of Ophthalmology, Hallym University Kangdong Sacred Heart Hospital, Seoul, Korea Purpose: To report a case of adult-onset Coats disease which showed decreased retinal edema and improved visual acuity following intravitreal ranibizumab injection. Case summary: 21-year-old woman visited our hospital for decreased visual acuity in left eye from 3 months ago. Her best corrected visual acuity was 1.0 in the right eye, and 0.4 in the left eye. The intraocular pressure was 19 mmhg in the right eye and 16 mmhg in the left eye. t anterior segment examination, no abnormal findings were found. On fundus examination, subretinal exudates, superior retinal edema at posterior pole, and telangiectasia along superortemporal vascular arcade were observed in the left eye. Upon diagnosis as Coats disease, intravitreal ranibizumab was performed, and laser photocoagulation was done around the retinal telangiectasia and nonperfusion area. Then, the second and third intravitreal ranibizumab injections were performed by a month, and her best visual acuity was improved to 0.8 and optical coherence tomography revealed decreased retinal edema. Conclusions: We report a case of adult-onset Coats disease. Intravitreal ranibizumab injection is effective in rapid visual improvement and decrease of retinal edema as combination therapy with laser photocoagulation which was a generalized treatment of choice in Coats disease. J Korean Ophthalmol Soc 2017;58(7): Keywords: Coats disease, Intravitreal ranibizumab injection, Macular edema 코우츠병은어린나이에진단되는질환으로, 인종및유전적차이를보이지않는다. 병의초기에는망막모세혈관의확장이관찰되고, 이로인하여망막내와망막하삼출물이발생되고, 이로인해삼출성망막박리및신생혈관녹내장으로이어지는특발성의발육이상망막혈관질환이다. 진 Received: Revised: ccepted: ddress reprint requests to Sung Pyo Park, MD, PhD Department of Ophthalmology, Hallym University Kangdong Sacred Heart Hospital, #150 Seongan-ro, Gangdong-gu, Seoul 05355, Korea Tel: , Fax: sungpyo@hanafos.com * Conflicts of Interest: The authors have no conflicts to disclose. 단나이는평균 5세로알려져있고 75% 에서남성에발생하는것으로알려져있으며, 주로단안에발생하는질환이다. 1 치료는광응고술이가장효과적인치료법으로알려져있다. 2,3 최근에는주로당뇨망막병증, 맥락막신생혈관, 그리고다양한망막질환에의한황반부종등에서치료로이용되는유리체강내혈관내피성장인자항체가코우츠병에서도효과적이라는보고가많이되고, 실제로임상적으로사용되고있다. 4-6 국내에서는본증례에서처럼성인코우츠병에서유리체강내라니비주맙주입술후망막부종의호전및시력호전을보인증례는이전에보고된바없어보고하고자한다. 증례보고 기저질환이없는 21 세여자환자가 3 개월전부터지속되 c2017 The Korean Ophthalmological Society This is an Open ccess article distributed under the terms of the Creative Commons ttribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 870

() No definite improvement was observed at a month after first intravitreal ranibizumab injection and photocoagulation. C Figure 2. Optical coherence tomographs of the left eye.

2 - 김한얼외 : 코우츠병및라니비주맙 - Figure 1. Fundus photographs of left eye. () Fundus photograph of left eye at initial visit reveals subretinal exudates at posterior pole, and telangiectasia along superortemporal vascular arcade. () No definite improvement was observed at a month after first intravitreal ranibizumab injection and photocoagulation. C Figure 2. Optical coherence tomographs of the left eye. () The optical coherence tomography at the first visit shows subretinal exudates and superior macular edema. () month after intravitreal ranibizumab injection, macular edema decreased. (C) month after second intravitreal ranibizumab injection, macular edema more decreased. 는좌안의시력저하를주소로내원하였다. 내원시최대교정시력은우안 1.0, 좌안 0.4였고, 안압은우안 19 mmhg, 좌안 16 mmhg로측정되었다. 세극등검사상전안부에서 특이소견은관찰되지않았다. 안저검사상좌안에망막하삼출물및후극부상측망막부종, 그리고상이측혈관궁을따라분포된모세혈관확장증소견을보였다 (Fig. 1). 빛간 871

3 - 대한안과학회지 2017 년제 58 권제 7 호 - Figure 3. Fluorescein angiographs of the left eye. () Fluorescein angiographs of the left eye at initial visit shows retinal telangiectasia at posterior pole, and late leakage, nonperfusion area at peripheral retina. () Late leakage was shown at similar area at a month after third intravitreal ranibizumab injection. 섭단층촬영 (Spectralis Optical Coherence Tomography: OCT, Heidelberg, Heidelberg Engineering) 에서도좌안후극부상측망막부종및망막하삼출물이관찰되었고 (Fig. 2), 형광안저촬영에서는좌안주변부에서비관류영역및모세혈관확장증과함께조영후기로갈수록누출이발생하는소견이관찰되었다 (Fig. 3). 이에코우츠병으로진단하였고좌안유리체강내라니비주맙 (1 mg/0.1 ml) 주입술을시행하였다. 그리고이때좌안망막주변부의조영제누출부위및비관류영역에망막레이저광응고술을시행하였다. 한달후좌안상측망막부종은감소되었고시력은 0.63으로증가되었다 (Fig. 2). 라니비주맙주입술에대한반응이있다는판단하에좌안유리체강내라니비주맙 2차주입술을시행하였다. 한달후좌안의망막부종은더욱감소되었고시력또한 0.8로호전되었다 (Fig. 2C). 그리고유리체강내라니비주맙 3차주입술을시행하였다. 이후 1달뒤시행한안저검사상좌안망막의삼출물의뚜렷한감소는보이지 않았지만 (Fig. 1), 빛간섭단층촬영검사상망막부종의호전을관찰할수있었다. 또한이때시행한형광안저촬영검사상에서여전히동일부위에후기누출이관찰되어 (Fig. 3), 레이저광응고술을추가로시행하였다. 이후 3개월간망막부종의재발없이잘유지되었다. 고찰 코우츠병은주로어린나이의남자에서단안에발생하는질환으로, 크게두가지의병리학적기전을가진다. 첫번째는망막혈관내피세포및주위세포의소실에의한혈액망막장벽의파괴로인해혈액성분이망막조직및망막하공간으로누출되는것이다. 또다른하나는비정상적인주위세포및내피세포의존재로인해미세동맥류의형성및혈관폐쇄등이일어나고이로인해망막허혈로이어지는것이다. 7-9 유전학적으로는 CR1 유전자돌연변이와의연 872

4 - 김한얼외 : 코우츠병및라니비주맙 - 관성에대한주장도있었고, 10 NDP 유전자의돌연변이와의연관성을시사하는연구도있었다. 11 형광안저조영소견으로는모세혈관비관류지역, 모세혈관확장증등이관찰되고초기부터지속적인형광누출이망막조직및망막하공간에고이는소견이관찰된다. Shields et al 12,13 은코우츠병을모세혈관확장증, 삼출물, 삼출망막박리, 녹내장등의유무에따라크게 5단계로구분하였고이에따라단계에맞는치료를권장하였다. 모세혈관확장증과삼출물이발생한초기단계에서는광응고술이나확장된혈관에냉동응고술을시행한다. 그리고삼출성망막박리가관찰되는심한코우츠병에서는망막박리의정도에따라망막하액배출술, 공막두르기또는유리체절제술등의수술적치료를고려할수있다. 하지만, 코우츠병에서본증례처럼넓은범위의망막하삼출물및망막부종이발생되어있는경우에는레이저치료의효과가제한적일수있다. Sigler et al 14 은황반부종이나삼출물또는장액성망막박리등이관찰되는코우츠병에서는부종이나삼출물에의해레이저의효과가떨어질수있으므로유리체강내항혈관내피성장인자주입술후에광응고술을시행하거나둘을동시에시행하는병합치료를일차치료로추천하였다. 최근에는코우츠병에서유리체강내항혈관내피성장인자주입술을통해혈관누출을억제하는데효과가있다는보고도있었다. 15 실제로외국에서유리체강내베바시주맙주입술후황반부종이호전된증례도적지않게보고되었다. 4,5,16,17 국내에도유리체강내베바시주맙주입술과광역학치료또는유리체강내베바시주맙과트리암시놀론주입술을병합한치료로망막부종및삼출물이호전되어레이저광응고술의효과적인반응을유도하여병합치료로서좋은결과를얻은증례들이보고된바있다. 18,19 또한 Gaillard et al 6 은주로황반변성이나당뇨황반부종에사용되는라니비주맙을유리체강내주사하여혈액망막장벽의일시적인회복및신생혈관의억제효과를보았다고발표하였다. 이렇게최근에국내외에서레이저광응고술에대한병합치료로유리체강내항혈관내피성장인자를이용한치료가코우츠병에서적지않게이루어지고있다. 유리체강내항혈관내피성장인자치료가코우츠병에서효과가있는기전으로는코우츠병환자의안구내혈관내피성장인자의증가와관련되어있다고생각되고있다. 실제로정상안에비해안구내혈관내피성장인자의수치가유의하게상승되어있었고, 유리체강내베바시주맙주입술후안구내혈관내피성장인자의수치가크게감소하였다는보고가있었다. 20 물론코우츠병에서유리체강내항혈관내피성장인자주입술의기전에대해서는더많은연구가필요할것으로보 이지만, 황반의부종을감소시키는데효과가있는것으로보이며, 이는기존의치료인레이저광응고술의치료효과를높이는데도움이될수있다는점, 그리고기존에국내에서보고되었던베바시주맙주입뿐만아니라본증례에서처럼라니비주맙또한황반부종의감소에효과가있음은코우츠병의치료방침에있어충분한가치가있을것으로사료된다. REFERENCES 1) Egerer I, Tasman W, Tomer TT. Coats disease. rch Ophthalmol 1974;92: ) Ridley ME, Shields J, rown GC, Tasman W. Coats' disease. Evaluation of management. Ophthalmology 1982;89: ) Schefler C, errocal M, Murray TG. dvanced Coats' disease. Management with repetitive aggressive laser ablation therapy. Retina 2008;28:S ) Lin CJ, Hwang JF, Chen YT, Chen SN. The effect of intravitreal bevacizumab in the treatment of coats disease in children. Retina 2010;30: ) Ray R, arañano DE, Hubbard G. Treatment of coats' disease with intravitreal bevacizumab. r J Ophthalmol 2013;97: ) Gaillard MC, Mataftsi, almer, et al. Ranibizumab in the management of advanced coats disease stages 3 and 4: long-term outcomes. Retina 2014;34: ) Chang MM, McLean IW, Merritt JC. Coats' disease: a study of 62 histologically confirmed cases. J Pediatr Ophthalmol Strabismus 1984;21: ) Fernandes F, Odashiro N, Maloney S, et al. Clinical-histopathological correlation in a case of Coats' disease. Diagn Pathol 2006; 1:24. 9) Ghorbanian S, Jaulim, Chatziralli IP. Diagnosis and treatment of coats' disease: a review of the literature. Ophthalmologica 2012; 227: ) Cremers FP, Maugeri, den Hollander I, Hoyng C. The expanding roles of C4 and CR1 in inherited blindness. Novartis Found Symp 2004;255:68-79; discussion 79-84, ) Dickinson JL, Sale MM, Passmore, et al. Mutations in the NDP gene: contribution to norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity. Clin Exp Ophthalmol 2006; 34: ) Shields J, Shields CL, Honavar SG, et al. Classification and management of coats disease: the 2000 proctor lecture. m J Ophthalmol 2001;131: ) Shields J, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina 2002;22: ) Sigler EJ, Randolph JC, Calzada JI, et al. Current management of coats disease. Surv Ophthalmol 2014;59: ) Smithen LM, rown GC, rucker J, et al. Coats' disease diagnosed in adulthood. Ophthalmology 2005;112: ) lvarez-rivera LG, braham-marín ML, Flores-Orta HJ, et al. Coat's disease treated with bevacizumab (vastin). rch Soc Esp Oftalmol 2008;83: ) Entezari M, Ramezani, Safavizadeh L, assirnia N. Resolution 873

5 - 대한안과학회지 2017 년제 58 권제 7 호 - of macular edema in coats' disease with intravitreal bevacizumab. Indian J Ophthalmol 2010;58: ) Jun JH, Kim YC, Kim KS. Resolution of severe macular edema in adult coats' disease with intravitreal triamcinolone and bevacizumab injection. Korean J Ophthalmol 2008;22: ) Kim J, Park KH, Woo SJ. Combined photodynamic therapy and intravitreal bevacizumab injection for the treatment of adult coats' disease: a case report. Korean J Ophthalmol 2010;24: ) He YG, Wang H, Zhao, et al. Elevated vascular endothelial growth factor level in coats' disease and possible therapeutic role of bevacizumab. Graefes rch Clin Exp Ophthalmol 2010;248: = 국문초록 = 성인코우츠병에서유리체강내라니비주맙주입술을시행한 1 예 목적 : 성인코우츠병환자에서유리체강내라니비주맙주입술시행후망막부종감소및시력호전을보인증례를경험하여이를보고하고자한다. 증례요약 : 기저질환이없는 21 세여자가 3 개월전부터지속되는좌안의시력저하를주소로내원하였다. 최대교정시력우안 1.0, 좌안 0.4, 안압은우안 19 mmhg, 좌안 16 mmhg 로측정되었다. 세극등검사상전안부에서특이소견은관찰되지않았다. 안저검사상좌안에망막하삼출물, 후극부상측망막부종및상이측혈관궁을따라분포된모세혈관확장증소견을보였다. 코우츠병진단하에유리체강내라니비주맙주입술을시행하였고모세혈관확장증및모세혈관비관류지역에대하여레이저광응고술을시행하였고 1 개월후망막부종감소를보였다. 이후 1 개월간격으로유리체강내라니비주맙 2 차, 3 차주입술을추가시행하였고최대교정시력좌안 0.8 로개선되었으며빛간섭단층촬영상상측망막부종감소를보였다. 결론 : 성인코우츠병환자에서유리체강내라니비주맙주입술이코우츠병의일반적인치료로이용되는레이저광응고술과병합치료로서망막부종감소및시력회복에효과적임을보고하는바이다. < 대한안과학회지 2017;58(7): > 874

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(유영석-마대중)( ).hwp 대한안과학회지 2011 년제 52 권제 1 호 J Korean Ophthalmol Soc 2011;52(1):112-116 pissn: 0378-6471 eissn: 2092-9374 DOI : 10.3341/jkos.2011.52.1.112 = 증례보고 = 양안에발생한코우츠병 1 예 마대중 1 최진 1 장지웅 1 김정훈 1,2 김성준 1,2 유영석 1,2