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| ORIGINAL ARTICLE |
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| Year : 2023 | Volume
: 16
| Issue : 1 | Page : 64-68 |
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Pars plana lensectomy and iris-claw Artisan intraocular lens implantation in patients with Marfan syndrome
Zahra Karjou1, Saeed Karimi2, Mohadeseh Yaghoobi1, Homayoun Nikkhah2, Sare Safi3
1 Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2 Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science; Department of Ophthalmology, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3 Ophthalmic Epidemiology Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| Date of Submission | 23-Jan-2022 |
| Date of Decision | 13-Aug-2022 |
| Date of Acceptance | 02-Dec-2022 |
| Date of Web Publication | 21-Feb-2023 |
Correspondence Address:
Zahra Karjou
Department of Ophthalmology, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Ibn Sina St., Baharestan Sq., Tehran 11498
Iran
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ojo.ojo_25_22
 Abstract | | |
RATIONAL: The rationale of this study was to evaluate the visual and anatomical outcomes of pars plana lensectomy and iris-claw Artisan intraocular lens (IOL) implantation in patients with subluxated crystalline lenses secondary to Marfan syndrome.
MATERIALS AND METHODS: In this retrospective case series, we evaluate the records of 21 eyes of 15 patients with Marfan syndrome and moderate-to-severe crystalline lens subluxation who underwent pars plana lensectomy/anterior vitrectomy and implantation of iris-claw Artisan IOL at referral hospital from September 2015 to October 2019.
RESULTS: Twenty-one eyes of 15 patients (10 males and five females) with a mean age of 24.47 ± 19.14 years were included. Mean best-corrected visual acuity was improved from 1.17 ± 0.55 logMAR to 0.64 ± 0.71 logMAR at the final follow-up visit (P < 0.001). The mean intraocular pressure did not change significantly (P = 0.971). The final refraction showed a mean sphere of 0.54 ± 2.46 D and a mean cylinder of 0.81 ± 1.03 at the mean axis of 57.92 ± 58.33 degrees. One eye developed rhegmatogenous retinal detachment 2 months after surgery.
CONCLUSIONS: Pars plana lensectomy and iris-claw Artisan IOL implantation seem to be a useful, impressive, and safe procedure with a low rate of complications in Marfan patients with moderate-to-severe crystalline lens subluxation. Visual acuity was significantly improved with acceptable anatomical and refractive outcomes.
Keywords: Crystalline lens subluxation, iris-claw Artisan intraocular lens, Marfan syndrome, pars plana lensectomy
How to cite this article:
Karjou Z, Karimi S, Yaghoobi M, Nikkhah H, Safi S. Pars plana lensectomy and iris-claw Artisan intraocular lens implantation in patients with Marfan syndrome. Oman J Ophthalmol 2023;16:64-8 |
How to cite this URL:
Karjou Z, Karimi S, Yaghoobi M, Nikkhah H, Safi S. Pars plana lensectomy and iris-claw Artisan intraocular lens implantation in patients with Marfan syndrome. Oman J Ophthalmol [serial online] 2023 [cited 2023 Mar 31];16:64-8. Available from: https://www.ojoonline.org/text.asp?2023/16/1/64/370042 |
| Introduction | |  |
Marfan syndrome is a genetic disorder that affects the connective tissue and is caused by a mutation in the fibrillin-1 (FBN1) gene on chromosome 15. The FBN1 gene encodes an FBN1 protein which is a principal component of the zonules, and its defect results in ectopia lentis (50%–80% cases).[1],[2] Marfan syndrome is a systemic disease that classically affects the cardiovascular, musculoskeletal, and ocular systems. For ocular diagnosis of Marfan syndrome, one major or two minor criteria should exist.[3] Lens subluxation is a major ocular criterion; flat cornea (as measured by keratometry),[4] increased axial length of globe, and hypoplastic ciliary muscle or iris are minor criteria. Other diagnostic ocular findings include retinal detachment, high myopia, cataract, glaucoma, strabismus, and amblyopia,[5],[6],[7],[8],[9] Ectopia lentis may result in refractive errors such as astigmatism, myopia, or aphakic hyperopia. Patients may experience diplopia if the equator of the lens crosses the visual axis.[2] Initial management is conservative with refractive correction and amblyopia therapy. However, surgery is recommended when it is difficult to obtain functional visual acuity with a nonsurgical approach, or when severe lens subluxation or posterior dislocation causes unstable refractive status, or if lens dislocation to the anterior chamber (AC) causes secondary intraocular pressure (IOP) rise or endothelial compromise.[2],[10],[11] The best surgical approach is still controversial. The limbal approach or pars plana approach may be chosen. The surgeon's comfort level can be an important factor in any method.[12] In the presence of large lens subluxation, it is very difficult to place a posterior chamber intraocular lens (PC-IOL) even with the support of a capsule tension ring (CTR) or Cionni ring, although there is the possibility of ring and IOL dislocation.[12],[13] For aphakia correction, various IOLs can be used, such as angle-supported AC-IOLs, scleral fixation PC-IOLs, and, more recently, retropupillary iris-claw IOLs.[14] These cases have a tendency to avoid angle-supported AC, due to the potential risk of secondary glaucoma and long-term endothelial damage.[13],[15] Iris-claw Artisan implantation is the optimal choice for aphakia correction with several advantages such as good visual outcome, fewer complications, and easy placement.[14],[16],[17]
We evaluated the visual and anatomical outcomes of pars plana lensectomy with implantation of Artisan IOL in eyes with subluxated lenses in Marfan syndrome.
| Materials and Methods | | |
In this retrospective noncomparative case series, we evaluate the records of all patients with Marfan syndrome and subluxated crystalline lens who needed pars plana lensectomy with the implantation of iris-claw Artisan IOL at a referral hospital from September 2015 to October 2019. The Marfan syndrome was diagnosed based on Ghent criteria ocular features such as ectopic lentils, flat cornea, increased axial length, positive family history, and systemic involvement confirmed by internal medicine and cardiologists.[18] The study was performed based on the Declaration of Helsinki and was approved by the institutional ethics committee. We included only patients with at least 3 months of follow-up. The patients with a history of previous intraocular surgery, missing complete pre- and postoperative records, and lens dislocation into the vitreous cavity were excluded from the study. Preoperative data included the demographic details, main preexisting ocular pathology, Snellen visual acuity, refraction, and IOP. Surgical technique, intraoperative problems, and postoperative information, including final best-corrected visual acuity (BCVA), IOP, refraction, and incidence of complications, were obtained. IOL power was measured using laser interferometry (IOL Master 500; Zeiss, Jena, Germany). Based on the axial length, IOL power calculation was done using the SRK/T, Hoffer, or Holladay formula. In all cases, our target refraction was emmetropia. All procedures were performed by one surgeon under general anesthesia. Lensectomy was performed using the pars plana approach. In this approach, 3 mm from the limbus and two sclerotomies were made. A vitreous cutter probe was used to perform a complete lensectomy to ensure that all capsular residues were removed. Subsequently, anterior vitrectomy and peripheral retinal examination were done to discover any unintentional retinal breaks. The sclerotomies were secured with 8–0 Vicryl suture. Finally, pupil constriction was achieved by carbachol 0.01%, and peripheral iridectomy was performed. A limbal incision was made at the 12 o'clock position and two ports at the 10 and 2 o'clock positions. The aphakic Artisan IOL (Ophtec, Groningen, the Netherlands) was inserted into the AC and fixed to the iris with an enclavation needle (Ophtec, Groningen, the Netherlands). At the end of the surgery, AC washing was done, and the wound was sutured with 10–0 nylon. Postoperatively, topical antibiotics and steroid drops were prescribed and tapered for 4 weeks. Selective corneal suture removal according to corneal astigmatism was performed in 6–8 postoperative weeks. BCVA, anterior and posterior segment examinations, and IOP were documented on follow-up visits.
Statistical analysis
A commercially available statistical software package (SPSS version 23; IBM Corp., New York, NY, USA) was used to perform univariate analyses using parametric and nonparametric methods. For qualitative variables, frequencies and proportions (as percentages) were calculated. For normally distributed quantitative variables, the mean and standard deviation were calculated. For quantitative variables which were not normally distributed, the median and 25% quartile were calculated. Statistical significance was defined as P < 0.05.
| Results | | |
Twenty-one eyes of 15 patients (10 males and five females) with Marfan syndrome and severe crystalline lens subluxation were included. Thirteen eyes were from a male patient, and eight eyes were from a female patient. The mean age of patients was 24.47 ± 19.14 years. Mean BCVA significantly improved from 1.17 ± 0.55 logMAR preoperatively to 0.64 ± 0.71 logMAR at the final follow-up visit (P < 0.001). The mean follow-up duration was 14.7 ± 5.2 months.
The mean IOP before the surgery was 15.15 ± 7.4. The mean IOP at the last follow-up after the surgery was 13.5 ± 2.7 mmHg, respectively (P = 0.971).
Preoperative refractive error was unstable and invaluable. The mean final postoperative spherical equivalent was + 0.14 ± 2.37 diopters (D). The constituent components of refraction were evaluated using power vector analysis that showed a mean sphere of 0.54 ± 2.46 D and a mean cylinder of 0.81 ± 1.03 at a mean axis of 57.92 ± 58.33°. There was no significant early postoperative complication. Only one case developed rhegmatogenous retinal detachment (RRD) 2 postoperative months and was approached by standard three-port pars plana deep vitrectomy and silicone oil tamponade. Visual acuity improvement occurred in all eyes except three which seems to be caused by preoperative amblyopia. We did not observe clinically significant IOP elevation or other complications such as pupillary block, uveitis, IOL dislocation, endophthalmitis, macular edema, or endothelial decompensation in follow-up visits.
| Discussion | | |
The present study showed the feasibility, effectiveness, and safety of pars plana lensectomy and anterior iris-claw IOL implantation in patients with moderate-to-severe crystalline lens subluxation in Marfan syndrome. Our results showed significant improvement in visual acuity and acceptable refractive outcomes postoperatively. This improvement was achieved in the 1st postoperative weeks and then remained almost stable throughout the follow-up visits. Comparing pre- and postoperative values, IOP did not change significantly. The development of amblyopia in children with large lens subluxations can be a factor limiting postoperative visual gain when the surgery was performed after childhood. Three of our patients did not show significant improvement in visual acuity due to amblyopia.
The management of ectopia lentis differs based on the preoperative and intraoperative findings. The lens subluxation grade plays an important role. In cases of mild lens subluxation, phacoemulsification and CTR supported, in the bag IOL implantation can be performed. In all of our cases, the grade of the lens subluxation was moderate or severe. All patients were managed with pars plana lensectomy/anterior vitrectomy followed by anterior iris-claw Artisan IOL implantation. This approach has advantages over phacoemulsification, particularly in complex eyes with specific challenges including difficult capsulorhexis, phacoemulsification in the context of an unstable and floating lens and its capsule, and also insertion of CTR or fixation of the Cionni ring. Furthermore, pars plana lensectomy and anterior vitrectomy can avoid intraoperative complications of phacoemulsification technique such as lens capsular bag compromise or vitreous traction and postoperative in-bag IOL dislocation, especially in severe cases.[19],[20]
Artisan implantation is an effective method to manage the aphakia in the absence of capsular support. Although AC Artisan IOL implants were associated with some complications such as iritis, IOL disenclavation, and endothelial cell compromise; numerous studies indicated that despite some endothelial cell loss in the long term, IOL and corneal endothelium keep a safe distance from each other (ranging from 2.99 to 3.01 ± 0.56 mm).[21],[22],[23],[24],[25] Several studies have shown that Artisan IOL had several advantages and fewer complications when compared with the transscleral sutured PC-IOLs and angle-supported AC-IOLs.[11] Insertion and implantation of Artisan IOL are easy and visual outcomes are good. Being fixated on the mid-peripheral iris in a location that is away from the AC angle, Artisan IOL is less likely to damage the angle structures.[26],[27] The scleral-fixated IOL implantation technique may increase the occurrence of postoperative retinal detachment in patients with Marfan syndrome because these patients commonly have some peripheral retinal changes predisposing them to an RRD.[28] Cleary et al. believe that the main advantage of iris-claw ACIOL over scleral-fixated IOL is that manipulation of the vitreous not occurred, and the posterior segment remains intact.[29] Suture erosion and IOL subluxation or dislocation may occur in IOL suturing to the sclera or iris and sutured Cionni. Such a way that IOL dislocation has been reported in up to 24% of pediatric cases.[30] Other complications include intraoperative hemorrhages, cystoid macular edema, IOL tilting, decentration or iris capture, and increased retinal detachment incidence.[31],[32],[33] Posteriorly enclavated iris-claw IOL may potentially lower endothelial cell loss, but some recent studies showed that anterior and posterior iris-claw IOL proved almost identical effectiveness and safety for aphakic correction. Surgical technique choice depends on the surgeon's trend.[34],[35]
In our study, only one eye developed RRD 2 months postoperatively. Even without surgical intervention, in eyes with Marfan syndrome, the retinal detachment rate is higher than in the normal population, perhaps due to high axial myopia and lens subluxation.[1],[5],[36],[37] Zonular deficiency and subsequent lens subluxation in Marfan syndrome are assumed to produce a persistent pull on the ora serrata. Increased axial length, liquefied vitreous gel, and traction on vitreoretinal adhesion are other predisposing factors, leading to retinal thinning and eventually retinal detachment.[36],[37] Chandra et al. showed that 21% of Marfan patients with retinal detachment had previous subluxated lens surgery history. The authors concluded that lens subluxation surgery could be a risk factor for retinal detachment occurrence.[38] A more severe grade of lens subluxation and high axial myopia was seen in the patients who developed retinal detachment.
Limitations of our study are retrospective design and a relatively small sample size. Moreover, the endothelial cell damage was not evaluated which is a concern in Artisan implantation. Since Marfan syndrome is a rare condition, multicenter studies with larger sample sizes and longer follow-up visits are recommended.
| Conclusions | | |
Our study showed that pars plana lensectomy and iris-claw Artisan IOL implantation seems to be a safe and effective procedure with a low rate of complication in Marfan patients with moderate-to-severe crystalline lens subluxation. Visual acuity significantly improved, and the anatomical and refractive outcomes were acceptable.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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