1. LA+IOL SF without vitrectomy in Marfan syndrome
<Case conference>
LA+IOL SF without vitrectomy in Marfan syndrome
서울성모병원
안과 R3 정수경/pf.주천기

2. 최 O 희 M/15 C/C Dec VA(OU) P/Hx P/Ex F/Hx: mother- lens subluxation
Tall statue
High arched palate

3. Ocular exam VA OD 0.16(n-c) OS 0.2(n-c)
Ref) OD Ds =-3.5Dc Ax40 OS error
Ker) OD /38.25(WTR) OS /38.00(WTR)
AL OD mm OS mm
IOP OD 10 mmHg OS 11 mmHg
EOM straight at 1 position by H-test, no LOM
Orbit OU no exophthalmos
Lid OU no swelling
Conj. OD not injected OS not injected
Cornea OD clear OS clear
AC OD deep & cell(-) OS deep & cell(-)
Pupil OD round & nl. sized, LR(+) OS round & nl sized, LR(+)

4. Stretched Zonule

5. Lens OD subluxated lens OS subluxated lens
Fundus OD CDR 0.6/0.6 c flat macula
OS CDR 0.6/0.6 c flat macula
OD OS

6. Imp. Plan Lens subluxation(OU) Marfan syndrome(OU)
Lens aspiration +IOL S-F (OS)
Pediatrics consult for Marfan syndrome

7. 동영상 삽입

8. Progress note <POD #1d> VA 0.5 (ref. error) IOP 7 mmHg
Cornea mild edematous
AC deep & cell(++)
Pupil round & nl. Sized
Lens IOL fixated state/OS
<POD#7d>
VA 0.4(0.5x-1.75Ds = -2.50DcAx180)
Cornea clear
AC deep & cell(rare)

9. LA+IOL SF without vitrectomy in Marfan syndrome

10. Introduction Marfan syndrome
Chromosome # 15 long arm에 있는 fibrillin gene 결함
심혈관계, 골격계, 눈의 이상이 특징적으로 나타나는 결체조직 질환
AD or sporadic
10,000명당 1명의 빈도
인종이나 성별 간에 발생률의 차이는 없다.
Ophthalmic features
Ectopia lentis (80%) ; bilat./ symmetric
Angle anomaly (75%)
Retinal detachment ; lattice degeneration, high myopia
Etc.. – hypoplasia of dilator pupillae, pph. Iris transillumination,
strabismus, flat cornea, blue sclera
크고 마른 체격, scoliosis, sternal deformity 발생
팔 다리 길고, 손가락 발가락 길다.
Aortic dissection 및 mitral valve disease

11. Introduction
J Cataract Refract Surg 2000; 26:781–784

12. Introduction Lens subluxation Supero-temporal (m/c)
May be Microspherophakic
Treatment
Glasses prescription
Operation Indication
No correction with glasses
Irregular astigmatism and glare
BCVA <20/70
Posterior dislocation into vitreous
Secondary glaucoma (anterior dislocation) or corneal endothelial damage
Uveitis or monocular diplopia
Ectopia lentis중에서도 lens subluxation이 많다.
사진 추가 할 것!

13. Surgical strategies for the management of zonular compromise

14. Capsular tension ring
open, compressible, polymethylmethacrylate (PMMA) ring with a rounded eyelet at each end
it exerts a centrifugal force at the capsular equator, expands the capsular bag and redistributes tension from the weakened zonules to stronger, intact zonules
indicated in the presence of mild segmental zonulysis (<4 clock hours) or mild generalized weakness as indicated by slight lens movement upon making a rhexis or mild ovalization/decentration of the completed rhexis
contraindicated in the presence of an anterior radial or posterior capsule tear ; further radial or posterior extension

15. Modified capsular tension rings
Cionni and Osher modified the standard CTR in 1998
open, PMMA ring similar to the standard CTR, except one (model 1-L) or two (model 2-L) fixation eyelets where attached that extended anterior to the anterior capsule ; sutured to the scleral wall
If the degree of zonular compromise is more severe (moderate to advanced) or progressive, the standard CTR in not sufficient for capsular stabilization and a M-CTR or CTS should be utilized
contraindicated in the presence of an anterior radial or posterior capsule tear

16. Capsular tension segments
In 2002, Ahmed designed the CTS (Morcher), intended for use in cases of severe zonular compromise
Providing intraoperative capsular stability and postoperative centration of the capsular bag and IOL
partial open ring, PMMA segment (120 degree) with an anteriorly placed eyelet that allows suture fixation to the scleral wall
Can be used in the presence of anterior or posterior capsular tear

17. Iris retractors and capsular support hooks
5-0 prolene iris hooks to tent the capsule opposite the phaco incision
Iris hooks + CTR placement in eyes with 3~6 clock hours of zonular compromise
5-0 flexible nylon hook ending in a bifurcated T-shaped foot pad that contacts the capsular equator
4-0 nylon with two angulations ; 160 ° bend for iris and limbus,
30 ° for the capsulorhexis margin

18. When to place capsular support devices
At any point after capsulorhexis and hydrodissection
Early placement ; Prior to phacoemulsification
Prevent damage to the bag during phaco and I/A
Make cortex removal difficult
Further iatrogenic zonular weakening during insertion
CTS may allow easier cortex removal

19. Pars plana approach
Severe lens instability with greater than 180 ° of zonular dialysis or Complete lens dislocation
Complete vitectomy eliminates future vitreoretinal traction
However it is difficult to induce a posterior vitreous separation in young patients

20. IOL placement Mild to moderate capsular instability AC IOL
CTR or CTS allows in the bag placement of a PC IOL
IOL haptics be positioned in the area of zonular compromise
Sulcus placement; if the anterior capsular rim is intact and the extent of lens subluxation is less than 3mm
AC IOL
Technically less challenging and less time consuming
Corneal endothelial cell loss
Uveitis glaucoma hyphema syndrome
Iris chaging with persistent inflammation
Erosion of the haptic into the ciliary body

21. IOL placement Suture fixation of the lens in the PC
IOL closer to the nodal point of the eye
Physiologic location
Lower rate of corneal compromise
Technically challenging
Iris fixated PCIOL
Iris chafe, chronic inflammation, irregular pupil, iridodialysis
Appropriate placement of sutures is the key
Scleral fixated PCIOL
Suture erosion
Intraocular hemorrhage
IOL decentration and tilt

22. Endocapsular tension ring implantation

23. Endocapsular tension ring implantation

24. Cionni endocapsular ring implantation in Marfan’s Syndrome
15 eyes of 12 patients with a subluxated lens secondary to Marfan’s syndrome
A 2-eyelet Cionni ring and an AcrySofH (Alcon) foldable intraocular lens (IOL) were implanted.
Cionni ring implantation is an effective procedure to correct partial lens subluxation and has few complications (during 14 months of follow-up) in patients with Marfan’s Syndrome.
Cionni ring: sclera에 fixation 되는 lens
(그림 설명)
Visante OCT
photograph of the anterior segment of the same patient demonstrating the
cionni ring eyelet anterior to the IOL, at the posterior chamber.
Br J Ophthalmol 2007;91:1477–1480

25. Pediatric Secondary Lens Implantation in the Absence of Capsular Support
18 eyes with posterior chamber intraocular lenses (PCIOLs)
10 eyes implanted with anterior chamber intraocular lenses (ACIOLs)
Conclusion; PCIOLs sutured to the ciliary sulcus offer a superior option to ACIOLs for correction of childhood aphakia in children lacking capsular support.
J AAPOS 2001;5:301-6

26. Current Opinion in Ophthalmology 2001, 12:47–57

27. Iris fixated PCIOL; 16 eyes, Iris-craw ACIOL; 15 eyes Conclusion;
Outcomes of Iris-Claw Anterior Chamber (Artisan) versus Iris-Fixated Foldable Intraocular Lens (AcrySof MA60AC) in Subluxated Lens Secondary to Marfan Syndrome
31 eyes of 16 patients
Iris fixated PCIOL; 16 eyes, Iris-craw ACIOL; 15 eyes
Conclusion;
The iris-sutured PCIOL and iris-claw ACIOL produced comparable improvements.
Although IOL dislocation tended to occur more frequently in the iris-fixated PCIOL group, the difference was not significant.
Ophthalmology 2010;117:1479–1485

29. Artisan Aphakic Intraocular Lens implantation in Cases of Subluxated Crystalline Lenses Due to Marfan Syndrome
Two children and three adults 를 대상으로 시행함
No complications occurred during surgery
Visual acuity was improved by 4 Snellen lines
Endothelial cell status remained constant
Possible complications (1-2%)
irregular pupil, transient corneal edema, transient intraocular pressure elevation, and IOL decentration
Artisan : iris clawed IOL lens
Journal of Refractive Surgery
Vol. 22 January/February 2006

30. Transscleral IOL Fixation With Preserved Anterior Vitreous Face
in Marfan Syndrome With Ectopia Lentis
6명, 12안을 대상으로 함
모든 환자에서 2줄 이상의 시력 향상 보임
술후 합병증- 2안에서 동공물림; medical treatment로 해결됨 한계
오랜기간 subluxation된 환자에서는 부적합함
(ant. Hyaloid membrane 이 보존될 가능성이 적음)
Intraocular lens implantation using the scleral fixation
technique is the first choice in patients with Marfan's
syndrome because it reduces the complications of IOL
decentration.
J Korean Ophthalmol Soc 2009;50(8):
J Pediatr Ophthalmol Strabismus2000;37:206-8

31. Summary…..
Br J Ophthalmol 2007;91:1477–1480