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ORIGINAL ARTICLE
Year : 2017  |  Volume : 24  |  Issue : 4  |  Page : 167-170  

Outcomes of scleral-fixated intraocular-lens in children with idiopathic ectopia lentis


1 Department of Ophthalmology, King Faisal University, Al Ahsa, Kingdom of Saudi Arabia
2 Department of Ophthalmology, Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia
3 Division of Pediatric Ophthalmology, Dhahran Eye Specialist Hospital, Dhahran, Kingdom of Saudi Arabia
4 Department of Ophthalmology, Imam Abdulrahman Bin Faisal University, Dammam, Kingdom of Saudi Arabia

Date of Web Publication12-Jan-2018

Correspondence Address:
Abdulaziz I Al Somali
Department of Ophthalmology, King Faisal University, P.O. Box 400, Al Ahsa 31982
Kingdom of Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/meajo.MEAJO_105_16

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   Abstract 

PURPOSE: The purpose of the study was to review the safety and stability of scleral-fixated intraocular lens (IOL) 2 years after implantation in children with idiopathic ectopia lentis (EL).
METHODS: This retrospective case series enrolled children with EL managed between 2011 and 2015 at a tertiary eye hospital in the eastern of Saudi Arabia. Data were collected on age, gender, vision, isolated or syndromic pathology, intraoperative and postoperative complications, spherical equivalent refraction, and final best-corrected visual acuity (BCVA).
RESULTS: The series included 18 eyes of 11 children with EL (6 males and 5 females). There were 7 bilateral and 4 unilateral cases. The median age was 3.5 years (25% quartile 1 year; range: 1–8 years). Preoperatively, fixation was absent in 3 eyes, 8 eyes were fixating but vision could not be recorded. In the remaining seven eyes, median distant visual acuity was 0.1 (25% quartile: 0.08). The median postoperative follow-up was 24 months (25% quartile, 7 months). Complications included two eyes with iris capture and one eye with lens subluxation requiring re-implantation. Postoperative BCVA was better than 20/60 in 15 (83%) eyes. At last follow-up, 6 eyes required myopic correction, 1 eye was emmetropic, and 11 eyes were hypermetropic. One eye with glaucoma was managed medically.
CONCLUSION: Scleral fixated IOL in eyes of young children with EL had good visual outcomes and high stability. However, there is a high incidence of residual refractive error.

Keywords: Children, ectopia lentis, sceral fixated intraocular lens


How to cite this article:
Al Somali AI, Al-Dossari FN, Emara KE, Al Habash A. Outcomes of scleral-fixated intraocular-lens in children with idiopathic ectopia lentis. Middle East Afr J Ophthalmol 2017;24:167-70

How to cite this URL:
Al Somali AI, Al-Dossari FN, Emara KE, Al Habash A. Outcomes of scleral-fixated intraocular-lens in children with idiopathic ectopia lentis. Middle East Afr J Ophthalmol [serial online] 2017 [cited 2018 Jan 20];24:167-70. Available from: http://www.meajo.org/text.asp?2017/24/4/167/223102




   Introduction Top


Intraocular lens (IOL) implantation after lensectomy with ectopia lens in children has been always challenging for ophthalmic surgeons. Congenital ectopia lentis (EL) is a rare ocular disorder with a prevalence of 0.83/10,000 live births in Denmark.[1] It is often associated with Marfan syndrome, homocystinuria, Weill–Marchesani syndrome, or it can be idiopathic. Rarely, cases have been linked to mutations in ADAMTSL4.[2],[3] The most common presentation of EL is diplopia, strabismus, and impaired vision. Hence, these children require urgent intervention to mitigate the development of amblyopia.[4] Conservative management with spectacles or contact lens can be offered for patient. However, high incidence of amblyopia has been reported following such management.[5] Correction of refractive error and contact lenses are attempted in most case to restore the vision unless the lens is opaque or complications occur such as glaucoma.[6],[7]

Scleral fixated IOL is a commonly accepted procedure for EL. This surgical procedure is performed through a limbal approach or a pars plana approach.[8] In the Gulf region, the outcomes of scleral-fixated IOLs in aphakic children in Oman and for 5 cases of subluxated lens in central Saudi Arabia have reported moderate success.[9],[10]

To the best of our knowledge, the ocular and systemic characteristics of EL and treatment in eastern Saudi Arabia have not published. We present the ophthalmic and systemic characteristics of EL and the outcomes of scleral-fixated IOL at a tertiary eye unit in Eastern Saudi Arabia.


   Methods Top


This study was a retrospective case series of patients with EL who underwent scleral IOL fixation over a 4-year period at a tertiary eye care unit in Eastern Saudi Arabia. An Institutional Review Board approved this study. As this was a retrospective study, written consent of the patients was waived. Patient confidentiality was maintained throughout the study. All children diagnosed with EL who underwent scleral-fixated IOL implant between 2011 and 2015 at our institute were included in the study. Cases with EL due to trauma as an underlying cause for subluxation were excluded from the study. Three ophthalmologists and an epidemiologist were the study investigators.

All patients underwent a comprehensive eye examination under sedation or general anesthesia [Figure 1]. Data were collected on patient demographics, operative eye, age at the time of surgery, visual acuity, and presence of amblyopia. All tests were age appropriate. In infants, fixation of central gaze was assessed using penlight projection. In children who could communicate, we used the Lea symbol ETDRS chart at 3 m distance and noted visual acuity in decimal values.
Figure 1: Idiopathic ectopia lentis in a child

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After induction, intraoperative axial length was measured with a contact A-scan probe. Keratometry readings were taken using a handheld autorefractor. A superior scleral tunnel approach was used. Conjunctival peritomy was carried out superiorly and then at the 3 and 9 o'clock positions. The superior scleral tunnel wound was at 1.5–1.5 mm posterior to the limbus. Limbal-based partial thickness scleral flaps were performed at the 3 and 9 o'clock positions with the posterior edge located 3 mm behind the limbus. Two paracentesis sites were in each superior quadrant. The eye was filled with a dispersive viscoelastic. Bimanual or coaxial anterior-approach vitrectomy technique was adopted. For a puncture in the anterior lens, capsule vitrector was used and then the lens substance was removed along with the capsule and zonular substance. The eye was then reformatted with a viscoelastic. A double-armed 10-0 polypropylene suture (Prolene) with a straight needle (STC6 needle; Ethicon, Somerville, NJ, USA) was used. The suture needle was passed through the sclera approximately 2 mm posterior to the limbus and directed across the eye toward a 27-gauge needle, which was also inserted through the sclera 2 mm posterior to the limbus 180° opposite the suture needle (usually at the 3 and 9 o'clock positions). The superior sclera tunnel wound was then enlarged to approximately 7 mm and the 2 Prolene sutures crossing the anterior chamber were hooked and withdrawn through the central limbal opening. The sutures were cut, and the appropriate ends were passed through the haptic eyelet of a CZ70BD 7-mm all PMMA IOL (Alcon Laboratories, Fort Worth, Tx, USA) and tied to the lens. The lens was then guided into the eye and underneath the iris plane to rest in the sulcus plane before centration was adjusted by drawing upon the 10-0 Prolene sutures. The groove incision was closed using interrupted 10-0 nylon sutures. The vitrector was then used to remove all potential vitreous prolapse and viscoelastic. Miochol was injected into the eye. The conjunctiva was closed using buried 9-0 polyglactin (Vicryl) sutures. Subconjunctival antibiotic and steroids were injected at the end of the procedure.

The ocular status was assessed at day 1, after 1 week, 6–8 weeks, and 3, 6, and 12 months [Figure 2]. The visual and refractive status at last follow-up was used for evaluating success. The intra- and post-operative complications used to assess the safety of intervention and stability of IOL. A 2-line improvement at last postoperative vision compared to preoperative vision was considered a successful outcome.
Figure 2: Eye with idiopathic ectopia lentis first day after scleral fixated intraocular lens implantation

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Based on the manifest refraction at last follow-up, we calculated the spherical equivalent of that eye and classified eyes as hyperopic, emmetropic, mildly myopic (<2 D), moderately myopic (2–5.75 D), and severely myopic (≥6 D).

The data were entered into an Excel® spreadsheet (Microsoft Corp., Redmond, WA, USA). Univariate analysis with a parametric method was used for statistical analysis. Statistical Package for the Social Studies software (SPSS 16; IBM Corp., Armonk, NY, USA) was used to perform the analyses. For qualitative variables, the frequencies and percentage proportions were calculated. For quantitative variables, a histogram was plotted to evaluate the distribution. For variables that were normally distributed, the mean and standard deviation were calculated. For variables with nonnormal distribution, we calculated the median and 25% quartile.


   Results Top


Eighteen eyes of 11 children comprised the study sample. There were six males and five females. The median age was 3.5 years (25% quartile, 1 year; range: 1–8 years). There were nine right eyes and nine left eyes. Seven children had bilateral EL and four had unilateral EL. Preoperatively, fixation was absent in three eyes, eight eyes fixated but vision could not be recorded because children were too young and not cooperative. In the remaining seven eyes, median distance visual acuity was 0.1 (25% quartile, 0.08).

At follow-up on day 1 to 1 week, evaluation of refractive status was possible in 16 children. At this visit and it suggested that two eyes were emmetropic, 11 required +1 D to +3 D correction and 3 eyes had a myopic spherical equivalent.

The median postoperative follow-up after primary lensectomy with scleral-fixated IOL was 24 months (25% quartile, 7 months).

The best-corrected visual acuity at last follow-up was noted in 17 eyes. It was 20/20 to 20/60 in 15 (84%) eyes. Vision improved by two lines in 16 (89%) eyes (successful outcome). Five (28%) eyes required the correction of ± 1 D. Eight (44.4%) eyes were intentionally made hyperopic within targeted refraction and five eyes were myopic of >1 D.

In 15 (83.3%) eyes, the IOL was stable and in center of pupil at last follow-up.

Iris capture occurred in 2 (11%) eyes and both were managed medically. IOL subluxation occurred in one (5.5%) eye that was managed by re-implantation. One (5.5%) eye developed glaucoma that was successfully managed medically. Vitreous hemorrhage, endophthalmitis or retinal detachment during surgery or in the median postoperative follow-up of 24 months (25% quartile, 7 months) was not found in any eye.


   Discussion Top


The scleral-fixated IOL for the management of idiopathic EL in children between 1 and 8 years old offered at least a 2-line improvement in 89% of eyes. Postoperative visual acuity measured in 17 eyes was median 0.5 (25% quartile, 0.4). The main complications were iris capture (2 eyes) and subluxation (1 eye) that required re-implantation of the IOL and one eye that developed glaucoma that was medically treated.

This is perhaps the first large series of nontraumatic EL managed by scleral-fixated IOL in Arab children [Figure 1].

A study of five cases of EL in Saudi Arabia suggested better visual outcomes compared to eyes with acquired EL managed by lens aspiration.[10] In Oman, ten out of twenty eyes with subluxated lens managed by pars plana scleral lensectomy and scleral-fixated IOL had vision 6/12 and better.[9] However, the mean age of these patients was 25 years, and in our study, all the children were <10 years of age. Hence, comparison of the outcomes between studies should be interpreted with caution. Kumar et al. had noted 63% eyes with functionally acceptable vision in eyes managed by glued fixated IOL to manage natural lens without adequate support including those with EL.[11]

There was a marked improvement in best-corrected visual acuity within 1 week of surgery and after a median follow-up of 24 months. Healing of the corneal wound, reduction of corneal edema, and management of the captured IOL could explain the improved vision over time. Immediate postoperative follow-up outcome may not be predictive of final visual outcome [Figure 2].

Iris capture in our study was the main complication. Iris capture rates of up to 13% have been previously reported.[12] Close postoperative monitoring and medical management of iris capture is recommended to avoid long-term effects on visual outcome.

Glaucoma was noted in only one (5.5%) eye in our study. This was lower than 13% reported by Kanigowska et al.[13] in a study with 8-year follow-up. Long-term follow-up to monitor for glaucoma is appropriate for children with EL.

We found that, at the last follow up, 61% eyes were hyperopic (<+3 D). This was intentional as the surgeon had planned to attain target refraction for all patients to allow normal emmetropization. With time and once the eye reach adult globe size, the hyperopia will decrease. Bearing in mind the near vision of children is crucial, our surgeon aimed to provide spectacle for near vision for all children postoperatively. Nearly one-third of eyes were with myopic shift, which is a matter of concern. To avoid amblyopia, these myopic children must be offered contact lens or spectacles to correct residual refractive error. As refractive error could change as these children age periodic monitoring is recommended. Park et al.[14] had cautioned against under correction of long eyes as progressive myopia may develop despite management of EL.

Our series of EL eyes underwent one method of management. Future evaluation of other methods of IOL implantation in nontraumatic EL is required.

Though relatively short follow-up, median 24 months, stability after sutured scleral-fixated IOLs in our study were encouraging, long-term risks are not totally eliminated. Studies with longer follow-up period have reported suture-related complications in scleral-fixated IOL in children.[13]

There were some limitations to our study. The retrospective nature of data collection has the inherent limitation of loss of information, and there could be patients who did not present regularly for follow-up. In very young children, visual acuity was based on fixation of gaze, and this could differ from older children who were assessed using an ETDRS chart. Therefore, an improvement in visual acuity was estimated soon following surgery and at the last follow-up should be compared with caution.

Surgical management of EL in a tertiary eye hospital in an Eastern Province of Saudi Arabia provided promising results that were comparable to the international studies. The complications of our surgical approach were few, and most of them could be medically managed.


   Conclusion Top


Scleral fixated IOL in eyes of young children with EL had good visual outcomes and high stability. However, there is a high incidence of residual refractive error.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

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2.
Neely DE, Plager DA. Management of ectopia lentis in children. Ophthalmol Clin North Am 2001;14:493-9.  Back to cited text no. 2
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3.
Ahram D, Sato TS, Kohilan A, Tayeh M, Chen S, Leal S, et al. A homozygous mutation in ADAMTSL4 causes autosomal-recessive isolated ectopia lentis. Am J Hum Genet 2009;84:274-8.  Back to cited text no. 3
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Nelson L. Ectopia lentis in childhood. J Pediatr Ophthalmol Strabismus 2008;45:12.  Back to cited text no. 4
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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;17:235-41.  Back to cited text no. 5
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Matsuo T. How far is observation allowed in patients with ectopia lentis? Springerplus 2015;4:461.  Back to cited text no. 6
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Senthil S, Rao HL, Hoang NT, Jonnadula GB, Addepalli UK, Mandal AK, et al. Glaucoma in microspherophakia: Presenting features and treatment outcomes. J Glaucoma 2014;23:262-7.  Back to cited text no. 7
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Simon MA, Origlieri CA, Dinallo AM, Forbes BJ, Wagner RS, Guo S, et al. New management strategies for ectopia lentis. J Pediatr Ophthalmol Strabismus 2015;52:269-81.  Back to cited text no. 8
    
9.
Ganesh A, Al-Zuhaibi S, Mitra S, Sabt BI, Ganguly SS, Bialasiewicz AA, et al. Visual rehabilitation by scleral fixation of posterior chamber intraocular lenses in Omani children with aphakia. Ophthalmic Surg Lasers Imaging 2009;40:354-60.  Back to cited text no. 9
    
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Alzuhairy SA, Bosley TM, Alotaibi AG. Retrospective review of visual outcome in operated lens subluxation. Saudi Med J 2013;34:1030-4.  Back to cited text no. 10
    
11.
Kumar DA, Agarwal A, Prakash D, Prakash G, Jacob S, Agarwal A, et al. Glued intrascleral fixation of posterior chamber intraocular lens in children. Am J Ophthalmol 2012;153:594-601, 601.e1-2.  Back to cited text no. 11
    
12.
Kim WS. Transscleral intraocular lens fixation with preservation of the anterior vitreous face in patients with marfan syndrome and ectopia lentis. Cornea 2010;29 Suppl 1:S20-4.  Back to cited text no. 12
    
13.
Kanigowska K, Grałek M, Karczmarewicz B. Transsclerally fixated intraocular artificial lenses in children – Analysis of long-term postoperative complications. Klin Oczna 2007;109:283-6.  Back to cited text no. 13
    
14.
Park SC, Chung ES, Chung TY, Kim SA, Oh SY. Axial growth and binocular function following bilateral lensectomy and scleral fixation of an intraocular lens in nontraumatic ectopia lentis. Jpn J Ophthalmol 2010;54:232-8.  Back to cited text no. 14
    


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