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1 대한안과학회지 2015 년제 56 권제 4 호 J Korean Ophthalmol Soc 2015;56(4): ISSN (Print) ISSN (Online) Case Report 호모시스틴뇨증환자에서변형된수정체낭팽창고리공막고정과난시교정인공수정체낭내삽입술 Modified Capsular Tension Ring Scleral Fixation and Toric Intraocular Lens Implantation in a Patient with Homocystinuria 박대현 정진권 Dae Hyun Park, MD, Jin Kwon Chung, MD 순천향대학교의과대학안과학교실 Department of Ophthalmology, Soonchunhyang University College of Medicine, Cheonan, Korea Purpose: To report a case of modified capsular tension ring scleral fixation and in-the-bag toric intraocular lens (IOL) implantation in a pediatric patient with severe crystalline lens subluxation due to homocystinuria. Case summary: A 9-year-old male diagnosed with homocystinuria and crystalline lens subluxation presented with progressive decrease of visual acuity. Uncorrected distant visual acuity (UDVA) and corrected distant visual acuity were 0.03 and 0.6 in the right eye and 0.01 and 0.5 in the left eye, respectively. Slit-lamp examination showed severe crystalline lens subluxation toward the inferiomedial side in both eyes. Corneal astigmatism in the right eye and left eye was 2.75 diopters (D) and 3.00 D, respectively based on keratometry. A combination of subluxated crystalline lens aspiration, scleral-fixated modified capsular tension ring insertion and in-the-bag toric IOL implantation were performed in both eyes. After continuous curvilinear capsulorhexis, nucleus and cortex of the crystalline lens were removed by irrigation and aspiration. A modified capsular tension ring with 2 fixation hooks (Model 2-L) was inserted into the capsular bag and fixed at the scleral wall. Next, toric IOL was inserted into the capsular bag. UDVA was 0.8 in the right eye and 0.9 in the left eye and 3 months postoperatively, the IOL rotation was less than 3 degrees from intended axis in both eyes. Conclusions: In a patient with severe congenital crystalline lens subluxation and moderate to severe corneal astigmatism, scleral fixation of modified capsular tension ring and in-the-bag toric IOL implantation is a possible surgical option. J Korean Ophthalmol Soc 2015;56(4): Key Words: Crystalline lens subluxation, Homocystinuria, Modified capsular tension ring, Toric intraocular lens Received: Revised: Accepted: Address reprint requests to Jin Kwon Chung, MD Department of Ophthalmology, Soonchunhyang University Hospital, #59 Daesagwan-ro, Yongsan-gu, Seoul , Korea Tel: , Fax: schcornea@schmc.ac.kr * This study was presented as a video at the 112th Annual Meeting of the Korean Ophthalmological Society 호모시스틴뇨증은시스타티오닌베타합성효소결핍에의한선천성대사이상으로혈장, 소변및뇌척수액에호모시스테인, 호모시스틴, 메티오닌의증가와시스테인, 시스틴감소가특징이며상염색체열성으로유전된다. 1 임상적으로조기수정체탈구와동반된심한근시진행 (80%) 이가장흔하고 2,3 대부분의경우에서정신지체, 마르팡증후군같은골격이상, 골다공증이나뇌혈관, 심혈관또는말초혈관폐쇄가동반된다. 4,5 호모시스틴뇨증에서수정체부분이탈은보통조기에나타나며양안대칭적, 그리고하비측으로생긴다. 소아에서발생한양안수정체부분이탈은굴절 c2015 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 614

- 박대현 정진권 : 선천성수정체부분이탈환자의수술방법 - 이상약시를초래하고특히시축의 1.3 mm 이내에수정체경계가있는경우더심한약시가생긴다. 6 심한수정체부분이탈에서안경과콘택트렌즈를조기부터시도하지만불규칙적인난시와근시진행으로치료가힘들어일반적으로수술이필요하다.

2 - 박대현 정진권 : 선천성수정체부분이탈환자의수술방법 - 이상약시를초래하고특히시축의 1.3 mm 이내에수정체경계가있는경우더심한약시가생긴다. 6 심한수정체부분이탈에서안경과콘택트렌즈를조기부터시도하지만불규칙적인난시와근시진행으로치료가힘들어일반적으로수술이필요하다. 수술방법으로수정체절제술과전방유리체절제술후인공수정체공막고정술이주로시행되며추후 Nd:YAG 레이저를이용해서후낭혼탁을제거하는것이가능한경우변형된수정체낭팽창고리 (modified capsular tension ring, MCTR) 공막고정을이용한인공수정체낭내삽입술을고려해볼수있다. 7-9 국내에서호모시스틴뇨증으로인한수정체탈구는 1991 년보고된바가있으나 10 호모시스틴뇨증으로인한수정체탈구환자에서인공수정체낭내삽입술에대한보고는없다. 저자들은호모시스틴뇨증으로인한심한수정체부분이탈소아환자에서 2개의고리가있는 MCTR을이용하여난시교정인공수정체낭내삽입술을시행한 1예를보고하고자한다. 증례보고 2년전타병원에서호모시스틴뇨증과수정체부분이탈을진단받은 9세남아가수년전부터점점진행되는시력저하를주소로내원하였다. 내원당시나안시력은우안 0.03, 좌안 0.01이었고최대교정시력우안 (0.6) x Ds Dc A x 20º, 좌안 (0.5) x Ds Dc A x 180º 로측정되었다. 세극등현미경검사상전반적인수정체소대약화와중력으로인해하비측으로심한수정체부분이탈소견을보였고 (Fig. 1) 안저검사상이상소견은없었다. 각막곡률검사상 90도경선이가파른우안 2.75D, 좌안 3.00D의 난시가있었고환아가수술후 Nd:YAG 레이저를이용한후낭절개가가능한나이로판단되어수정체낭을보존하고난시교정용인공수정체를삽입하는것을계획하였다. 전신마취하에양안 MCTR 공막고정술과수정체낭내난시교정인공수정체삽입술을시행하였다. 먼저수정체소대약화가가장심한부분인상이측각막윤부 2 mm 뒤쪽에 MCTR 공막고정을위한공막피판을만들고수정체소대약화가가장심한부위에서 90º 이상떨어진곳에 2.8 mm 투명각막절개술을시행한후점탄물질을주입하면서전방내로유리체탈출이있는지확인하였으나유리체탈출은없었다. 우안은 26 Gauge bent needle (Sungshim medical, Bucheon, Korea) 을이용하여수정체중심부위의전낭에작은구멍을만들고수정체낭겸자 (capsulorrhexis forceps) 를이용하여수정체낭원형절개술을시행하였으며좌안은수정체소대의약화로인해 bent needle로전낭에작은구멍이만들어지지않아 2개의 needle을전방에넣고 needle이서로맞물리게하여전낭에작은구멍을만들고 (Fig. 2A) 3개의윤부절개부위를통해홍채훅을삽입하여수정체낭원형절개의경계부에위치시킨후부드럽게당겨수정체낭을지지하고시야확보를한후 (Fig. 2B) 수정체낭원형절개술을시행하였다. 평형염액 (BSS Plus, Alcon, Fort Worth, TX, USA) 을주입하여수력분리술과수력분층술을시행한후관류및흡인 (Irrigation & Aspiration) 으로수정체핵과피질을제거하였다. 수정체낭의허탈을예방하기위해관류및흡인기를제거하기전에수정체낭을점탄물질로채운후관류및흡인기를제거하였다. 공막고정을위해 MCTR의 2개의고정고리에각각 Double-armed 10-0 polypropylene을통과시킨후투명각막절개창을통해수정체낭내로 MCTR을삽입하였다. 먼저수정체소대가가장약한부분에미리만들 A B Figure 1. Slit-lamp photographs showing bilateral inferomedial crystalline lens subluxation through the dilated pupil. (A) Inferomedial crystalline lens subluxation in the right eye. (B) Inferomedial crystalline lens subluxation in the left eye. OD = oculus dexter; OS = oculus sinister. 615

3 - 대한안과학회지 2015 년제 56 권제 4 호 - A B C D Figure 2. Intraoperative photographs. (A) Making anterior capsule tear using two 26-gauge needles. (B) Supporting anterior capsule using 3 microhook iris retractors. (C) Fixation of modified capsular tension ring (MCTR) to the sclera. Note that two hooks are not perpendicular. The meridian of second hook is determined by the diameters of MCTR and sulcus, individually. (D) A complex of toric intraocular lens and scleral fixated MCTR is well centered along the intended axis. Table 1. Postoperative clinical results POD # 1 day POD # 1 week UDVA CDVA MR SE IOP UDVA CDVA MR SE IOP OD , º OS , º POD # 1 month POD # 3 months UDVA CDVA MR SE IOP UDVA CDVA MR SE IOP OD , º , º OS , º , º POD = postoperative day; UDVA = uncorrected distant visual acuity; CDVA = corrected distant visual acuity; MR = manifest refraction; SE = spherical equivalent; IOP = intraocular pressure. 어놓았던공막피판으로 MCTR을임시고정한후두번째고정고리가위치하는곳에공막피판을만들고 MCTR을임시고정하였다 (Fig 2C). 투명각막절개창을통해난시교정인공수정체 ( 양안 : 23.5D AcrySof SN6AT6 Toric IOL, Alcon, Fort Worth, TX, USA) 를삽입한후각막의가파른난시축 (90 ) 에맞춰서인공수정체의위치를조정하였다. 삽입된 인공수정체와수정체낭복합체의중심화를위해임시로고정했던 MCTR 공막고정봉합사의긴장상태를조정한후공막에영구고정하였다 (Fig. 2D). 각막, 공막, 그리고결막절개창을 10-0 Nylon으로봉합하고수술을마쳤다. 수술후 1일, 1주, 1개월, 3개월째추적관찰결과는 Table 1과같다. 수술 3개월째나안시력우안 0.8, 좌안

- 박대현 정진권 : 선천성수정체부분이탈환자의수술방법 - A B C Figure 3. Postoperative photohgraphs. (A, B) External photographs shows clear cornea, conjunctiva and round pupil 3 months after operation.

4 - 박대현 정진권 : 선천성수정체부분이탈환자의수술방법 - A B C Figure 3. Postoperative photohgraphs. (A, B) External photographs shows clear cornea, conjunctiva and round pupil 3 months after operation. (C) Three degrees counterclockwise axis rotation is found in a postoperative 3 months slit-lamp photograph in the right eye. (D) Two degrees clockwise axis rotation is found in a postoperative 3 months slit-lamp photograph in the left eye. OD = oculus dexter; OS = oculus sinister. D Table 2. The distribution of manifest refractive errors and Fourier transformed J 0 and J 45 values before and after surgery (spectacle plane) Before surgery After surgery (POD # 3 months) M J 0 J 45 B M J 0 J 45 B OD OS POD = postoperative day; M = S + C/2; J 0 = (-C/2)cos(2α); J 45 = (-C/2)sin(2α); B = (S = spherical diopter; C = cylindrical diopter; α = cylindrical axis; B = blurring strength). 로측정되었으며세극등검사와안저검사상이상소견은없었다 (Fig. 3A, B). 수술후 3개월째산동후시행한세극등검사상난시교정용인공수정체는우안에서반시계방향으로 3º, 좌안에서시계방향으로 2º 의축회전 (Axis roation) 을보였으며유의한중심이탈은관찰되지않았다 (Fig. 3C, D). 수술전과후의난시를비교하기위한벡터분석에서우안은 J 0 와 J 45 가각각 2.42 Ds, 1.23 Ds 감소하였으며좌안은 J 0 가 1.91 Ds 감소하였고 J 45 는 0.21 Ds 증가하였다. 굴절력과난시벡터를모두고려한 Blurring strength는우안에서 Ds 좌안에서 Ds 감소하였다 (Table 2). 고찰 하측으로진행된수정체부분이탈에서원인이외상인경우상측의국소적인수정체소대약화를의미하지만호모시스틴뇨증이나마르팡증후군과같이선천성원인인경우전반적인수정체소대약화를의미하며이로인해치료하기어렵다. 11 소아에서선천성수정체부분이탈은과거에는주 617

5 - 대한안과학회지 2015 년제 56 권제 4 호 - 로수정체절제술과전방유리체절제술시행후안경이나콘택트렌즈를사용하여굴절이상을교정하거나 12 수정체절제술및전방유리체절제술후인공수정체공막고정술을시행하였다. 7,8,13,14 그러나수정체절제술및전방유리체절제술을시행할경우안내출혈, 포도막염, 낭포성황반부종, 유리체탈출및견인에의한이차성녹내장, 망막박리와같은합병증이발생할확률이높아지므로 Nd:YAG 레이저가가능한소아에서는 MCTR을이용하여수정체낭을보존하는수술을시행하고추후발생한후낭혼탁은 Nd:YAG 레이저로치료하는것이바람직할것으로생각한다. 9,11,17 Acrysof SA series (Alcon, Fort Worth, TX, USA) 는일체형인공수정체로수정체낭내에서오랫동안중심화를이루고염증반응이적으며지지부가부드러워삽입하기쉽고변형이적은장점이있다. 인공수정체를조작하기쉽고작은수정체낭에서도탈중심화가잘일어나지않으므로소아에서수정체낭내인공수정체삽입시가장선호되며안정적인인공수정체이다 난시교정인공수정체인 AcrySof SN6AT (Alcon, Fort Worth, TX, USA) 또한광학부가 acrylate, 지지부는 methacrylate copolymer로구성된일체형인공수정체로 Acrysof SA series와구성이같아소아에서안정성이우수할것으로생각한다. 난시교정인공수정체의수정체낭내삽입은난시를교정하여우수한시력예후를보이고술후 6개월동안축회전이평균 4도이하로높은회전안정성을가졌다 년이상장기추적관찰한결과 378안중 6안에서만 20 이상의축회전이있었고모두술후 10일이내에발생하여난시교정인공수정체는장기추적관찰에서도높은회전안정성을보여주었다. 24 본증례의환자에서도수술후 3개월까지양안모두난시교정인공수정체의축회전이 3 미만으로안정적이었으며벡터분석을통한난시의변화와 blurring strength변화또한난시교정인공수정체를사용하여효과적으로교정된것을알수있었다. 25 소아의선천성수정체부분이탈에서 MCTR을이용하여수정체낭내인공수정체를삽입한연구들에따르면약 90% 의환자에서시력호전을보여모양체고랑인공수정체삽입술이나인공수정체공막고정을한경우와유사하거나더좋은결과를보였고 5-9% 에서만수술후인공수정체탈구를보여선천성수정체부분이탈환자에서 MCTR을사용하여낭내인공수정체를삽입하는것이안전하고효과적인수술이라보고하였다 전반적인수정체소대약화로인한심한선천성수정체부분이탈환자에서중등도이상의각막난시가있는경우 2 개의고정고리가있는 MCTR을사용하여수정체낭을중심안정화하고난시교정인공수정체를낭내에삽입하여우 수한시력교정효과를볼수있었다. 수술후 Nd:YAG 레이저로후낭절개술을시행할수있는환아에게수술적치료의좋은방법중하나가될수있을것으로생각하며공막고정된고리와수정체낭복합체그리고인공수정체의눈속안정성에대해서는보다많은증례와장기간의추적관찰이필요할것으로생각한다. REFERENCES 1) Schimke RN, McKusick VA, Huang T, Pollack AD. Homocystinuria. studies of 20 families with 38 affected members. JAMA 1965;193: ) Mulvihill A, Yap S, O'Keefe M, et al. Ocular findings among patients with late-diagnosed or poorly controlled homocystinuria compared with a screened, well-controlled population. J AAPOS 2001;5: ) Mudd SH, Skovby F, Levy HL, et al. The natural history of homocystinuria due to cystathionine beta-synthase deficiency. Am J Hum Genet 1985;37: ) Yap S. Classical homocystinuria: vascular risk and its prevention. J Inherit Metab Dis 2003;26: ) Bilwani F, Syed NA, Usman M, Khurshid M. Familial homocystinuria. J Coll Physicians Surg Pak 2005;15: ) Romano PE, Kerr NC, Hope GM. Bilateral ametropic functional amblyopia in genetic ectopia lentis: its relation to the amount of subluxation, an indicator for early surgical management. Binocul Vis Strabismus Q 2002;17: ) Wu-Chen WY, Letson RD, Summers CG. Functional and structural outcomes following lensectomy for ectopia lentis. J AAPOS 2005; 9: ) Asadi R, Kheirkhah A. Long-term results of scleral fixation of posterior chamber intraocular lenses in children. Ophthalmology 2008;115: ) Pfeifer V, Morela K. Ectopic lens extraction in children. Coll Antropol 2001;25 Suppl: ) Chung DY, Chung YT. A case of homocystinuria with ectopia lentis. J Korean Ophthalmol Soc 1991;32: ) Hoffman RS, Snyder ME, Devgan U, et al. Management of the subluxated crystalline lens. J Cataract Refract Surg 2013;39: ) Halpert M, BenEzra D. Surgery of the hereditary subluxated lens in children. Ophthalmology 1996;103: ) Lam DS, Ng JS, Fan DS, et al. Short-term results of scleral intraocular lens fixation in children. J Cataract Refract Surg 1998; 24: ) Bardorf CM, Epley KD, Lueder GT, Tychsen L. Pediatric transscleral sutured intraocular lenses: efficacy and safety in 43 eyes followed an average of 3 years. J AAPOS 2004;8: ) Hoyt CS, Nickel B. Aphakic cystoid macular edema: occurrence in infants and children after transpupillary lensectomy and anterior vitrectomy. Arch Ophthalmol 1982;100: ) Koenig SB, Ruttum MS, Lewandowski MF, Schultz RO. Pseudophakia for traumatic cataracts in children. Ophthalmology 1993; 100: ) Gimbel HV, Camoriano GD, Aman-Ullah M. Bilateral Implantation of Scleral-Fixated Cionni Endocapsular Rings and Toric Intraocular 618

6 - 박대현 정진권 : 선천성수정체부분이탈환자의수술방법 - Lenses in a Pediatric Patient with Marfan's Syndrome. Case Rep Ophthalmol 2012;3: ) Trivedi RH, Wilson ME Jr. Single-piece acrylic intraocular lens implantation in children. J Cataract Refract Surg 2003;29: ) Trivedi RH, Wilson ME Jr, Bartholomew LR, et al. Opacification of the visual axis after cataract surgery and single acrylic intraocular lens implantation in the first year of life. J AAPOS 2004; 8: ) Kugelberg M, Kugelberg U, Bobrova N, et al. After-cataract in children having cataract surgery with or without anterior vitrectomy implanted with a single-piece AcrySof IOL. J Cataract Refract Surg 2005;31: ) Sun XY, Vicary D, Montgomery P, Griffiths M. Toric intraocular lenses for correcting astigmatism in 130 eyes. Ophthalmology 2000;107: ; discussion ) Horn JD. Status of toric intraocular lenses. Curr Opin Ophthalmol 2007;18: ) Bauer NJ, de Vries NE, Webers CA, et al. Astigmatism management in cataract surgery with the AcrySof toric intraocular lens. J Cataract Refract Surg 2008;34: ) Miyake T, Kamiya K, Amano R, et al. Long-term clinical outcomes of toric intraocular lens implantation in cataract cases with preexisting astigmatism. J Cataract Refract Surg 2014;40: ) Thibos LN, Horner D. Power vector analysis of the optical outcome of refractive surgery. J Cataract Refract Surg 2001;27: ) Cionni RJ, Osher RH, Marques DM, et al. Modified capsular tension ring for patients with congenital loss of zonular support. J Cataract Refract Surg 2003;29: ) Vasavada V, Vasavada VA, Hoffman RO, et al. Intraoperative performance and postoperative outcomes of endocapsular ring implantation in pediatric eyes. J Cataract Refract Surg 2008;34: ) Kim EJ, Berg JP, Weikert MP, et al. Scleral-fixated capsular tension rings and segments for ectopia lentis in children. Am J Ophthalmol 2014;158: = 국문초록 = 호모시스틴뇨증환자에서변형된수정체낭팽창고리공막고정과난시교정인공수정체낭내삽입술 목적 : 호모시스틴뇨증으로인한양안수정체부분이탈소아환자에서변형된수정체낭팽창고리를이용한난시교정인공수정체낭내삽입술증례를보고하고자한다. 증례요약 : 호모시스틴뇨증과양안수정체부분이탈로진단받은 9 세남자환아가지속적인시력저하를주소로내원하였다. 나안시력은우안 0.03, 좌안 0.01 그리고최대교정시력은우안 0.6, 좌안 0.5 로측정되었다. 세극등검사상양안모두하비측으로심한수정체부분이탈이관찰되었으며각막곡률검사상우안 2.75D, 좌안 3.00D 의난시가있어변형된수정체낭팽창고리를이용한난시교정인공수정체낭내삽입술을시행하였다. 수술은수정체낭원형절개후관류및흡인기를사용하여수정체핵과피질을제거하였고두개의고리가있는수정체낭팽창고리를이용하여수정체낭을공막에고정한후난시교정인공수정체를삽입하였다. 수술후 3 개월째나안시력우안 0.8, 좌안 0.9 로측정되었으며양안모두 3 도이내의인공수정체회전을보였다. 결론 : 심한선천성부분이탈환자에서중등도이상의각막난시가있는경우변형된수정체낭팽창고리를사용하여난시교정인공수정체낭내삽입술이가능할수있다. < 대한안과학회지 2015;56(4): > 619

<283732372D3733312920B4D9C3CAC1A120BCD2C7C1C6AEC4DCC5C3C6AEB7BBC1EEC0C720B3EBBEC8C0C720BDC3B7C2BAB8C1A4BFA120B4EBC7D120C0AFBFEBBCBA20C6F2B0A1283035292E687770>

<283732372D3733312920B4D9C3CAC1A120BCD2C7C1C6AEC4DCC5C3C6AEB7BBC1EEC0C720B3EBBEC8C0C720BDC3B7C2BAB8C1A4BFA120B4EBC7D120C0AFBFEBBCBA20C6F2B0A1283035292E687770> 대한안과학회지 제 49 권 제 5 호 2008 J Korean Ophthalmol Soc 49(5):727-731, 2008 DOI : 10.3341/jkos.2008.49.5.727 다초점 소프트콘택트렌즈의 노안의 시력보정에 대한 유용성 평가 김현경 1 김효명 2 정성근 1 가톨릭대학교 의과대학 성모병원 안과학교실 1, 고려대학교 의과대학 안암병원 안과학교실