대한안과학회지 2018 년제 59 권제 10 호 J Korean Ophthalmol Soc 2018;59(10):968-973 ISSN 0378-6471 (Print) ISSN 2092-9374 (Online) https://doi.org/10.3341/jkos.2018.59.10.968 Case Report 각막확장증과스티븐스존슨증후군합병증환자에서미니공막렌즈의치료효과 1 예 The Therapeutic Effect of Sclerocorneal Lens in Coexisting Corneal Ectasia and Stevens-Johnson Syndrome 백진욱 박창현 나경선 김현승 Jin Uk Baek, MD, Chang Hyun Park, MD, Kyung Sun Na, MD, PhD, Hyun Seung Kim, MD, PhD 가톨릭대학교의과대학여의도성모병원안과및시과학교실 Department of Ophthalmology and Visual Science, Yeouido St. Mary s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea Purpose: We report short-term treatment effects of a mini-scleral lens in patients with Stevens-Johnson syndrome and corneal ectasia. Case summary: A 54-year-old female who had been diagnosed with Stevens-Johnson syndrome and keratoconus presented with persistent right eye pain and decreased visual acuity. Therapeutic lenses, topical antibiotic eye drops, and topical steroid eye drops were used; however the symptoms were not controlled, then the patient was treated with a mini-scleral contact lens in the right eye. At the time of the first visit to our hospital 17 years ago, and the best-corrected visual acuity (BCVA) of the right eye was logmar 0.22. However, Stevens-Johnson syndrome and corneal ectasia were severe and cataract surgery was considered because of worsening cataracts, but the patients refused this surgery. At the time, the BCVA of the right eye was then reduced to logmar 1.10. On ophthalmic examination, there was conjunctival fibrosis and corneal neovascularization of the right eye. The symptoms were not managed with a using a therapeutic lens, and then the patient was prescribed a mini-scleral contact lens. After wearing the mini-scleral contact lens, the corneal and conjunctival neovascularization was reduced. After 6 months, the BCVA of the right eye improved to logmar 0.60. Conclusions: In patients with Stevens-Johnson syndrome and corneal ecstasia, mini-scleral contact lens can be considered as a useful treatment option for visual improvement and symptom control. J Korean Ophthalmol Soc 2018;59(10):968-973 Keywords: Contact lenses, Miniscleral contact lens, Pathologic dilatation, Stevens-Johnson syndrome 원추각막은각막확장증질환의대표질환으로각막중심 Received: 2017. 8. 31. Revised: 2018. 7. 2. Accepted: 2018. 9. 27. Address reprint requests to Hyun Seung Kim, MD, PhD Department of Ophthalmology, The Catholic University of Korea Yeouido St. Mary s Hospital, #10 63-ro, Yeoundeungpo-gu, Seoul 07345, Korea Tel: 82-2-3779-1240, Fax: 82-2-761-6869 E-mail: sara514@catholic.ac.kr * Conflicts of Interest: The authors have no conflicts to disclose. 이돌출되고얇아지면서진행하는비염증성질환으로이는불규칙난시와시력저하를야기한다. 1,2 원추각막의병인은가족력, 인종, 눈을비비거나헐거운눈꺼풀증후군과같은기계적요인, 알레르기성질환및결체조직질환등과같이다양하다. 1,3 이중스티븐스존슨증후군이있는환자에서명확한기전이밝혀지지않았지만, 눈을비비는기계적요인에의해서원추각막이동반될수있다고보고되고있다. 4,5 스티븐스존슨증후군과원추각막이동반되는환자의경우시력의저하및광시증이동반되었다. 3 c2018 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution 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. 968
- 백진욱외 : 스티븐스존슨증후군과각막확장증 - 원추각막이경도에서중등도까지진행된경우경성산소투과콘택트렌즈 (rigid gas permeable lens) 만으로도교정하여시력회복이가능하다. 2,6 그러나질병이더진행하게되면경성산소투과콘택트렌즈가중심을잡기힘들며, 각막에맞는렌즈를맞추는것이어렵다. 1,2 이경우시력저하및렌즈적응도에따라서각막콜라겐교차결합술, 각막이식술, 각막내링삽입술과같은침습적인방법을선택할수있지만, 공막콘택트렌즈착용또한대안으로고려해볼수있다. 1,2,6,7 공막콘택트렌즈는큰직경의경성산소투과공막콘택트렌즈로전체직경에따라서종류가다른데직경이 12.0-15.0 mm일경우공막윤부 / 반공막렌즈, 15.0-18.0 mm일경우미니공막렌즈, 18.0-25.0 mm일경우큰공막콘택트렌즈에해당한다. 7 공막콘택트렌즈는경성산소투과콘택트렌즈와달리직경이커서각막과의접촉을최대로피하는동시에렌즈내의눈물막을형성하면서, 원추각막과같은각막확장증및스티븐스존슨증후군과같은안구표면질환을가진환자에서시력개선효과를보였다. 7 본증례에서는각막확장질환과스티븐스존슨증후군이동반된환자에서미니공막렌즈 (Mini scleral Design [MSD]; Happy Vision Corp., Anyang, Korea) 를착용했을때단기적치료효과를확인할수있는증례가있어보고하고자한다. 증례보고 34년전설파제및피린계항생제로인한스티븐스존슨증후군을진단받고, 이후 20년전양안원추각막증을진단받은 54세여자환자는본원내원당시양안원추각막증이진행되고있었다. 우안은 17년전본원내원당시측정한 나안시력 logmar 1.0 ( 스넬렌시력 0.1), 최대교정시력은 logmar 0.22 ( 스넬렌시력 0.6) (-5.50 Dsph= -0.50 Dcyl 180 A) 까지측정되었고, 좌안은원추각막증이진행하여각막이식, 백내장수술및섬유주절제술까지시행하였으나광각무상태로시력회복이되지않았다. 이후본원에서경과관찰중우안의원추각막증의진행과백내장의악화로우안최대교정시력은 logmar 1.10 ( 스넬렌시력 0.08) (-6.00 Dsph= -0.50 Dcyl 180 A) 까지저하되었다. 통증조절을위해인공눈물을수시로점안하였고, 윤활작용을하는연고는잘때마다사용하였다. 또한치료용렌즈를 2주마다주기적으로교환하였다. 그러나지속적인점안제사용및치료용렌즈의착용은독성각결막염을야기하였고, 더이상의통증조절및시력개선이되지않았다. 안약중단후독성각결막염이안정된후증상개선을위해미니공막렌즈 (Onefit, Happy Vision Corp., Anyang, Korea) 를처방하기로하였다. 공막콘택트렌즈의재질은 Boston XO (Polymer Technology Corporation, Boston, MA, USA) 로제작되었다. 미니공막렌즈처방당시소견으로세극등현미경검사상우안결막섬유화및유두비대및여포가보이며, 각막에전반적인표층점상탈락및윤부결핍으로인한각막주변부에신생혈관이관찰되었다 (Fig. 1). 각막지형도검사는 Pentacam HR EYE scanner (Oculus Inc., Wetzlar, Germany) 로시행하였다. 우안 Sim K 값은 46.4/52.5로측정되었으며, 가장얇은부위의각막두께는 426 µm로측정되었다 (Fig. 2). Flat K를기준으로처방을시작하였으며, fluorescein을포함한인공누액을채운시험렌즈착용후세극등현미경을통해정점틈새 (apical clearance) 가충분한지확인하였다. Landing zone에윤부틈새 (limbal clearance) 는 fluorescine이얇게보였고, 공막에상하좌우과도한주변부조임이없는 A B Figure 1. Slit-lamp ophthalmoscopic findings in the patient. (A) Before wearing the miniscleral lens, conjunctiva of the right eye was moderate injected and there is a tarsus scarring. (B) It is a photograph of 10 hours after miniscleral lens wear. Conjunctival injection and corneal neovascularization of the right eye decreased significantly. 969
- 대한안과학회지 2018년 제 59 권 제 10 호 - 지 관찰하였으며 결막 혈관 압박으로 인한 탈색 소견은 발 소견이 없는지 확인 후 처방을 완료하였다. 이번 증례에서 견되지 않았다(Fig. 3). 이후 전안부 단층촬영을 TOPCON 는 기본 곡선 반경은 7.2 mm, 렌즈 직경은 14.5 mm, 도수 3D OCT-2000 OCT (Topcon Corp., Tokyo, Japan)를 이용 는 -7.00 Diopter로 하였다. 하여 중심부 및 주변부의 시상높이(sagittal height)를 확인 미니공막렌즈 착용 후 통증은 현저히 줄었으며, 렌즈 착 하였다(Fig. 4). Settle back이 150 µm 정도 될 것으로 고려 용 10시간 후 세극등현미경검사상 각막 및 결막의 신생혈 하여 정점틈새가 200-300 µm 정도(수정체의 2/3, 각막의 관이 줄어드는 양상을 보였다(Fig. 1). 미니공막렌즈 착용 1/3) 될 수 있는 시험렌즈를 임시 처방하였다. 6시간 착용 후 1개월째 자동굴절검사기(Topcon, Japan)를 통한 각막곡 후 다시 내원하도록 하여 동일한 검사를 시행하면서 이상 률값은 41.00/41.50으로 측정되었다. 또한 미니공막렌즈 착 A B Figure 2. Topography of the right eye demonstrating nasal cone, asymmetric bow-tie pattern and central thinning. (A) Anterior elevation map (top left), posterior elevation map (top right), keratometric map (bottom left), and pachymetry map (bottom right). (B) Tangential map. OD = oculus dexter. A B C D E F Figure 3. After wearing the mini-scleral contact lens, the corneal and conjunctival neovascularization was reduced. (A, B) Tear film between the cornea and the miniscleral lens is well positioned by slit-lamp biomicroscope. (C-F) Photos for cardinal directions; 6 hours after miniscleral lens wear. (C) Temporal side. (D) Nasal side. (E) Superior side. (F) Inferior side. There was no evidence of conjunctival bleaching associated with conjunctival compression by the lens. 970
- 백진욱외 : 스티븐스존슨증후군과각막확장증 - A B C Figure 4. Tear film (arrows) between the cornea and the miniscleral lens is well positioned along the cornea with clear apical corneal clearance. (A) Temporal side. (B) Nasal side. (C) Center. 용 6개월후우안나안시력 logmar 0.80 ( 스넬렌시력 0.16), 최대교정시력 logmar 0.60 ( 스넬렌시력 0.25) (-2.25 Dsph= -1.00 Dcyl 80 A) 까지개선되는양상을보였다. 환자는렌즈를평균 10시간착용중이며, 세극등현미경검사상신생혈관증가없이, 눈물층이잘생성되어유지되고있으며, 우안에인공눈물만사용하며경과관찰중이다. 고찰 경성산소투과콘택트렌즈는다양한형태의안질환에서불규칙적인각막표면을보정하여시력교정의효과를볼수 있다. 4,8,9 특히큰직경의경성산소투과콘택트렌즈는각막과의기계적인접촉을줄여주고렌즈아래쪽에눈물층을형성하면서건조감및각막형태이상으로인한불규칙난시도교정할수있는효과를나타내었다. 4,9-11 그중에서도미니공막렌즈는크기가 15.0-18.0 mm로기존의공막콘택트렌즈에비해크기가작아조금더쉽게착용할수있다는장점을가지고있다. 9 본연구에서는스티븐스존슨증후군과각막확장증이동반된 1안에서미니공막렌즈를착용했을때시력개선효과및통증호전양상을보였다. 스티븐스존슨증후군환자의경우지속되는염증반응으로인해검결막및눈꺼풀의각 971
- 대한안과학회지 2018 년제 59 권제 10 호 - 화가진행되며, 눈물막의지방성분이결핍된다. 5,12 이러한변화는눈깜박임지속시각막의미세한상처를남기게된다. 12 눈을비비는행동과같이스티븐스존슨증후군의환자의경우눈을깜박이는행동만으로도각막의미세한상처가생길수있고이는각막확장증으로진행될수도있다. 11-13 스티븐스존슨증후군및각막확장증이동반된환자에서주로시력저하와눈부심을호소하였다. 3 본연구에서도환자는시력저하와극심한통증및눈부심증상을호소하였고, 증상개선을위해치료용렌즈 silicone hydrogel soft contact lens (SHCL) 를사용했으나큰호전이없었다. 하지만미니공막렌즈착용후개선되는효과를보였다. 이번증례와같이각막확장증이진행하거나안검, 각막및결막의상태가좋지않은다양한안구표면안질환에서경성산소투과콘택트렌즈착용실패혹은처방이어려운경우미니공막렌즈를처방하여성공한다양한사례들중하나로의미있는결과라고볼수있겠다. 4,7,10 본증례는미니공막렌즈처방후관찰기간이 6개월로짧으며, 후향적으로분석한한계점이있다. 시력개선을위해우안백내장수술을고려하였으나, 환자가좌안광각무상태로우안백내장수술에대한거부감을가지고있어수술을진행하지못해시력개선에한계점도있었다. 미니공막렌즈처방방법은기존의방법을참고하였다. 14 경성산소투과콘택트렌즈는착용전에비해착용후시력개선효과가있지만불편함도수반하고있다. Yildiz et al 15 에따르면원추각막환자에서렌즈종류에따른시력이경성산소투과콘택트렌즈는 93.3%, SHCL은 66.7% 개선되는효과를보여, 경성산소투과콘택트렌즈가 SHCL에비해시력개선효과가있음을알수있었다. 하지만렌즈착용후생활의질개선정도는자가설문지를통해조사한결과두렌즈의차이는크게다르지않았는데특히경성산소투과콘택트렌즈는렌즈관리의어려움및눈꺼풀자극감과같은불편함이있었다. 앞선사례를보았을때경성산소투과콘택트렌즈와마찬가지로미니공막렌즈도착용후시력개선및통증조절이될수있지만렌즈자체의불편함, 렌즈관리의어려움으로생활의질에영향을미칠수있다. 다만환자의각막의질병상태가경미한경우보다진행할수록경성산소투과콘택트렌즈의착용으로환자가치료효과를더크게느끼는것으로보았을때환자가느끼는생활의질과치료효과에대한적절한균형이필요할것으로보인다. 15 본증례를통해환자는미니공막렌즈착용후임상적결 과호전및환자의주관적증상도호전되는양상을보였다. 이번사례를통해각막확장증과스티븐스존슨증후군이동반된환자에서미니공막렌즈착용후치료효과를보았으며, 이는앞으로다양한안질환에서미니공막렌즈의활용도가커짐을기대할수있겠다. REFERENCES 1) Andreanos KD, Hashemi K, Petrelli M, et al. Keratoconus treatment algorithm. Ophthalmol Ther 2017;6:245-62. 2) Lee JH, Park YM, Park YK, et al. Long-term effect and safety of contact lenses for keratoconus. J Korean Ophthalmol Soc 2015;56: 1006-11. 3) Rathi VM, Taneja M, Dumpati S, et al. Role of scleral contact lenses in management of coexisting keratoconus and Stevens-Johnson syndrome. Cornea 2017;36:1267-9. 4) Suh SY, Lee JH, Lee SU, et al. Fitting the miniscleral contact lens in patients with corneal abnormalities. J Korean Ophthalmol Soc 2016;57:1699-705. 5) Saeed HN, Kohanim S, Le HG, et al. Stevens-Johnson syndrome and corneal ectasia: management and a case for association. Am J Ophthalmol 2016;169:276-81. 6) Porcar E, Montalt JC, España-Gregori E, Peris-Martínez C. Corneo-scleral contact lenses in an uncommon case of keratoconus with high hyperopia and astigmatism. Cont Lens Anterior Eye 2017;40:351-6. 7) Yan P, Kapasi M, Conlon R, et al. Patient comfort and visual outcomes of mini-scleral contact lenses. Can J Ophthalmol 2017;52:69-73. 8) Schornack MM, Patel SV. Scleral lenses in the management of keratoconus. Eye Contact Lens 2010;36:39-44. 9) Kim S, Lee JS, Park YK, et al. Fitting miniscleral contact lenses in Korean patients with keratoconus. Clin Exp Optom 2017;100:375-9. 10) Carracedo G, Blanco MS, Martin-Gil A, et al. Short-term effect of scleral lens on the dry eye biomarkers in keratoconus. Optom Vis Sci 2016;93:150-7. 11) Dogru M, Karakaya H, Ozçetin H, et al. Tear function and ocular surface changes in keratoconus. Ophthalmology 2003;110:1110-8. 12) Di Pascuale MA, Espana EM, Liu DT, et al. Correlation of corneal complications with eyelid cicatricial pathologies in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis syndrome. Ophthalmology 2005;112:904-12. 13) McMonnies CW. Mechanisms of rubbing-related corneal trauma in keratoconus. Cornea 2009;28:607-15. 14) Sotozono C, Yamauchi N, Maeda S, Kinoshita S. Tear exchangeable limbal rigid contact lens for ocular sequelae resulting from Stevens-Johnson syndrome or Toxic epidermal necrolysis. Am J Ophthalmol 2014;158:983-93. 15) Yildiz EH, Erdurmus M, Elibol ES, et al. Contact lens impact on quality of life in keratoconus patients: rigid gas permeable versus soft silicone-hydrogel keratoconus lenses. Int J Ophthalmol 2015; 8:1074-7. 972
- 백진욱외 : 스티븐스존슨증후군과각막확장증 - = 국문초록 = 각막확장증과스티븐스존슨증후군합병증환자에서미니공막렌즈의치료효과 1 예 목적 : 스티븐스존슨증후군과각막확장증이동반된환자에서미니공막렌즈를사용하였을때단기간의치료효과사례를보고하고자한다. 증례요약 : 34 년전설파제및피린계항생제에의한알레르기로스티븐스존슨증후군을진단받고, 이후 20 년전원추각막증을진단받은 54 세여자환자가지속되는우안통증및시력저하를주소로내원하였다. 치료용렌즈, 항생제및국소스테로이드점안제등을사용하였으나, 증세가조절되지않아우안미니공막렌즈착용을고려하였다. 17 년전병원에처음내원할당시우안은나안시력 logmar 1.0, 최대교정시력 logmar 0.22 로측정되었다. 그러나스티븐스존슨증후군과각막확장증이심해졌고, 백내장의악화로수술을고려하였으나환자는백내장수술을거부하였다. 이후최대교정시력은 logmar 1.10 까지저하되었다. 전안부검사상우안결막섬유화, 유두비대및여포가있었으며, 각막에전반적인표층점상탈락과윤부결핍으로인한각막주변부에신생혈관이관찰되었다. 치료용렌즈를통한증상조절중치료용렌즈로인한염증악화소견을보여미니공막렌즈를처방하였다. 이후각막및결막의신생혈관이줄어드는양상을보이며미니공막렌즈착용 6 개월후우안나안시력 logmar 0.80, 최대교정시력 logmar 0.60 까지개선되었다. 결론 : 스티븐스존슨증후군과각막확장증이동반된환자에서미니공막렌즈를사용할경우시력개선및증상조절에유용한치료수단으로고려될수있겠다. < 대한안과학회지 2018;59(10):968-973> 백진욱 / Jin Uk Baek 가톨릭대학교의과대학여의도성모병원안과및시과학교실 Department of Ophthalmology and Visual Science, Yeouido St. Mary s Hospital, College of Medicine, The Catholic University of Korea 973