Visual outcomes of scleral-fixated posterior chamber intraocular lenses in children with genetic ectopia lentis: a 6-year retrospective study

Visual outcomes of scleral-fixated posterior chamber intraocular lenses in children with genetic ectopia lentis: a 6-year retrospective study | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Visual outcomes of scleral-fixated posterior chamber intraocular lenses in children with genetic ectopia lentis: a 6-year retrospective study Kevin Omry Vallotton, Oussama Habra, Beatrice Frueh This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8096373/v1 This work is licensed under a CC BY 4.0 License Status: Under Revision Version 1 posted 15 You are reading this latest preprint version Abstract Background To assess the feasibility and outcomes of lensectomy with sulcus-sutured intraocular lens (IOL) implantation in pediatric patients with atraumatic ectopia lentis. Methods Pediatric patients with atraumatic (sub-)luxated crystalline lenses that underwent lensectomy, anterior vitrectomy and sclera-fixated implantation of a polymethylmethacrylate (PMMA) IOL in the sulcus were included in this retrospective study. Outcome measures included corrected distance and near visual acuities (LogMar), refractive outcomes (spherical equivalent, prediction error), and complication rates. Follow-up averaged 6.14 ± 3.1 years with a minimal follow-up of two years and a maximal follow-up of 11 years. Results A total of 19 children (36 eyes; 55% male; mean age 5.11 ± 1.9 years) were included. Preoperative mean best corrected visual acuity (BCVA) improved from 0.72 ± 0.3 (near) and 0.48 ± 0.2 (distance) to 0.14 ± 0.3 and − 0.01 ± 0.1 postoperatively, respectively. Spherical equivalent changed from − 6.28 ± 4.6 diopters (D) preoperatively to + 1.41 ± 1.4D at one month, to -0.26 ± 3.6D at 6-year. The difference between spherical equivalent and targeted refraction was 0.59 ± 1.01D at 1-month and − 1.69 ± 2.3D at 6-year. Complications included 5 cases of IOL subluxation (4 spontaneous and one traumatic). Conclusions Lensectomy with scleral-fixated sulcus IOL implantation is an option for managing pediatric patients with ectopia lentis, yielding significant visual improvement and manageable refractive outcomes. Ectopia lentis Marfan syndrome sclera-fixated intraocular lenses Figures Figure 1 Figure 2 Figure 3 Background Ectopia lentis is a condition marked by the dislocation of the crystalline lens from its normal position, which can result in significant visual impairment. It usually manifests in the context of systemic disorders, though it can also arise due to trauma or idiopathic causes. Among the systemic diseases associated with ectopia lentis, Marfan syndrome is the most common( 1 ). followed by conditions such as Weill-Marchesani syndrome( 2 ), and homocystinuria( 3 ). The condition can also occur in an isolated form due to mutations in the ADAMTSL4 gene on chromosome 1, although this is less frequent. Marfan syndrome is an autosomal dominant disorder caused by mutations in the FBN1 gene located on chromosome 15 affecting the synthesis of fibrillin-1, a glycoprotein essential for the formation of elastic fibers in connective tissue. Children with Marfan syndrome have a higher risk of developing amblyopia due to high refractive error, anisometropia and ectopia lentis, retinal pathology or strabismus( 4 ). Since in children ectopia lentis is usually severe, preserving the capsular bag is less likely, so that leaving children aphakic with a later correction with glasses or contact lenses is common. Nevertheless, this has notable limitations like visual field narrowing, contact-lens related complications and discomfort and are associated with a high incidence of amblyopia( 5 ). Different surgical methods are being used depending on the severity of the lens subluxation and capsular bag integrity( 6 ). Surgical options that do not let the children aphakic but do not need capsular support have been increasingly favored, including scleral-fixated intraocular lenses (IOLs). These IOLs are fixated to the sclera with sutures or suture-less, providing a stable optical correction that can significantly improve visual acuity and reduce the risk of amblyopia. This approach usually involves either a limbal or pars plana incision, which depends on various factors, including the degree of lens subluxation and the presence of any additional ocular pathologies. Despite the widespread adoption of surgical treatment of lens subluxation in children, long-term data on the ocular outcomes in this population are relatively scarce and there is today still no surgical approach defined as the gold standard for ectopia lentis in children( 7 ). One of the critical considerations in pediatric IOL implantation is the anticipated growth of the eye, which typically involves an increase in axial length and a potential shift in refractive error over time. In patients with Marfan syndrome, this issue is even more pronounced due to their tendency to develop high axial lengths and significant myopia. A broadly chosen approach in younger children is to aim for a slightly hyperopic correction, allowing for the natural elongation of the eye over time, with the expectation that the patient will achieve emmetropia or a slight myopia in later years. However, the degree of hyperopia must be tailored to the patient’s age and expected ocular growth since excessive hyperopic correction is problematic in pseudophakic patients without accommodative power. This study retrospectively examines the long-term outcomes of scleral-fixated sulcus IOLs in pediatric patients with ectopia lentis, reporting an evaluation of the safety profile of the procedure, the stability of visual outcomes over time, and the effectiveness of the selected refractive targets in managing the anticipated growth of the eye. Given the challenges associated with this condition, particularly in young patients, the insights of this study might help refine surgical techniques and improve the overall management strategy for achieving optimal visual outcomes in this population. Methods The aim of this study was to evaluate the long-term visual outcomes after scleral-fixated IOL implantation in children with ectopia lentis in order to assess if this procedure represents a viable and effective long-term solution for these patients. We retrospectively analyzed all pediatric patients who underwent lensectomy, anterior vitrectomy and scleral-fixated sulcus intraocular lenses for the treatment of ectopia lentis from January 2012 to November 2024. Inclusion criteria were a diagnosis of non-traumatic ectopia lentis, age below 12 years, a minimal follow-up of two years and indication for surgery such as significant dislocation of the crystalline lens with lack of improvement in best corrected visual acuity with conservative management. Parents were informed about the possibility to leave the eye aphakic. They were informed about the advantages (no need for aphakic correction) and the disadvantages of the procedure (possible late-onset suture breaks with need to re-suture, lack of long-term results in children, etc.). All patients underwent genetic testing for diagnostic purposes. Patients with Marfan syndrome were specifically tested for mutations in the FBN1 gene. In cases where another disease was suspected as the underlying cause of ectopia lentis, an extended genetic panel was conducted. Patients underwent optical biometry preoperatively with IOL master (Zeiss) and corneal topography with Pentacam (Oculus) and TMS (Tomey). In accordance to the axial lengths measured preoperatively with caution given to standardizing the procedure for all patients, the SRK-T formula, adequate for the pediatric population( 8 , 9 ), was used in all patients to calculate the IOL power. The surgical procedure consisted of a corneoscleral tunnel and the preparation of two scleral pockets at 3’ and 9’ o’clock, manual capsulorhexis, then lens aspiration with bimanual I/A using hooks to stabilize the capsular bag followed by capsule removal and anterior vitrectomy and the implantation through a 6.5mm corneoscleral tunnel of a PMMA intraocular lens (IOL) in the sulcus. The lens was sutured to the sclera at the 3’ and 9’ clock hours using a double-armed 10 − 0 prolene suture using a curved needle. The scleral flaps were then sutured with 10 − 0 nylon. The two one-piece PMMA lenses used were PC 279Y IOL (Ophtec BV), which has 1 positioning hole per haptic and 81B IOL (Morcher), which has 3 positioning holes per haptic. The optics were 6.5mm. All eyes underwent this technique. The target refraction, whether hyperopic or emmetropic, was selected based on the patient's age at the time of surgery. In general, +2D was targeted for patients under 3 years old, +1D under 4 years old, and emmetropia from 4 years old on. Despite emmetropization is usually reached by age of 9 years old, patients with Marfan syndrome undergo further axial length growth through life( 10 , 11 ), which is why a moderate hypermetropia was targeted in order to minimize undesired myopic shift in adulthood. Visual and refractive examinations Patients underwent refractive examinations both preoperatively and postoperatively, with long-term follow-up over several years. The two reported time points are the initial postoperative refraction testing one month after surgery, and the last postoperative consultation by November 2024. All visual acuities were recorded using the LogMAR scale. Refractive examinations were performed by experienced orthoptists using either retinoscopy or subjective refraction, depending on the patient’s age. In addition, patients underwent corneal topography either with Pentacam (Oculus Gmbh) oder with TMS-IV (Tomey), and the differences in cylinder power and axis between corneal topography and manifest refraction was investigated. Amblyopia was defined as an interocular difference of ≥ 2 lines in visual acuity (VA), or a VA ≤ 0.2 logMar, that could not be attributed to any ocular or visual system abnormality or to refractive error. Statistical analysis The statistical analysis involved solely descriptive statistics. Results are reported in percentages and, when applicable, in means and standard deviations. All descriptive statistical analyses were performed using R version 2023.09.1 (Posit Software, PBC). RESULTS Patients demographics and characteristics A total of 19 patients (36 eyes) were included in this study. The mean age of the patients at the time of surgery was 5.1 ± 1.9 years and at last follow-up was 10.1 ± 3.2 years. Eleven (58%) patients were < 4 years old at time of surgery. Seventeen patients (89%) had bilateral ectopia lentis, resulting in a total of 34 eyes affected bilaterally, while 2 patients had only one eye treated. Of the 19 patients, 18 (95%) were diagnosed with Marfan syndrome. Genetic testing confirmed FBN1 gene mutations in all patients with Marfan syndrome, including various missense mutations such as p.Arg122Cys, p.Cys1361Arg, and p.Cys781Tyr, as well as frameshift mutations like 3688delA. One patient was diagnosed with Weill-Marchesani syndrome and exhibited a de novo heterozygous missense variant in the FBN1 gene. The mean follow-up duration for all patients was 6.1 ± 3.1 years. Visual and refractive outcome The PC 279Y IOL was implanted in 19 eyes (53%), while the 81B IOL was implanted in 18 eyes (47%). The selection of the IOL type was based on surgeon preference and specific anatomical considerations for each case. The mean axial length preoperatively was 22.7 ± 1.3 mm. The average keratometry was 42.7 ± 2.3 when measured with the Pentacam and 41.7 ± 1.9D when measured with the TMS-3, revealing flat corneas. Preoperatively, the visual acuities were as follows: the mean best-corrected near monocular visual acuity (NBCVA) was 0.72 ± 0.31 on the LogMAR scale, and the mean distance monocular best-corrected visual acuity (DBCVA) was 0.54 ± 0.27. Following surgery, the monocular NBCVA improved to 0.19 ± 0.28, while the DBCVA reached 0.05 ± 0.22 at the latest follow-up. Boxplots of the pre- and postoperative DBCVA are reported (Fig. 1 ). Linear regression found statistically significant association between preoperative DBCVA and DBCVA at last follow-up (R 2 = 0.537, p < 0.0001), as well as between preoperative NBCVA and NBCVA at last follow-up (R 2 = 0.208, p < 0.01). The preoperative mean spherical equivalent was − 6.3 ± 4.6 diopters (D) with a mean cylindrical power of 5.0 ± 2.9D. The mean target refraction for postoperative correction was set at + 0.88 ± 1.3D. At one-month post-surgery, the mean spherical equivalent was + 1.4 ± 1.4D and the mean cylindrical power had reduced to 2.7 ± 2.1D. At 6-year, a myopic shift was observed. The difference between spherical equivalents at 6-year and at 1-month was − 2.26 ± 2.11D, and the spherical equivalent was − 0.7 ± 2.6D at 6-year. The mean cylindrical power further reduced to 2.2 ± 1.4D at 6-year. Boxplots of target refraction and postoperative spherical equivalents are shown (Fig. 2 ). Refractive prediction error (PE) was determined as the difference between first post-operative refraction at 1-month and the target refraction. The PE was 0.59 ± 1.01D. As shown (Fig. 3 ), 56% of eyes had a SE within 1D of the target refraction, 36% within 0.5D and 17% within 0.25D. In addition, the difference in cylinder power between corneal astigmatism in topography and and manifest astigmatism at 6-year was 0.11 ± 1.75D and in cylinder axis was 18.83 ± 16.81 degrees. Orthoptic examinations identified various strabismic deviations. Eleven eyes (31%) were orthophoric. Amblyopia was identified in 3 eyes (8%), while 27 eyes (92%) showed no signs of amblyopia. The three eyes were diagnosed with deprivation amblyopia. Eleven eyes were operated on when patients were less than 4 years of age thus especially at risk for amblyopia. Complications and comorbidities No intraoperative complications occurred. Postoperatively, complications included visual relevant IOL subluxation in 14% of eyes (in 21% of patients), 4 out of 5 were spontaneous subluxations and one was traumatic. The 4 spontaneous IOL subluxations happened 8.5 ± 2.2 years post-operative (range 5.4 to 10.1 years). Three eyes required pars plana vitrectomy and IOL refixation using Gore-Tex sutures and one IOL-refixation with prolene-suture without pars plana vitrectomy. One eye experienced a subluxation without visual symptoms and did not require surgical intervention. Six percents of eyes showed strong suture-induced astigmatism, necessitating suture lysis to improve refractive outcomes. Discussion In this study, we present the refractive and visual outcomes over several years following lensectomy combined with scleral-fixated intraocular sulcus lenses in the management of ectopia lentis in pediatric patients. Additionally, we examine the associated orthoptic and systemic comorbidities in this patient population, which is critical for understanding the broader implications of the condition and its treatment. This study addresses the fact that crystalline lens subluxation in the context of genetic syndromes poses significant challenges in pediatric ophthalmology due to the need to balance immediate visual correction with the refractive changes that occur over the years as the child grows. Some studies that investigated the outcomes of aphakic children have shown that it is problematic due to the limitations of glasses and contact lenses, as well as the increased risk of amblyopia and suboptimal visual development. Better visual improvement was reported when lensectomy is followed by IOL implantation in comparison to aphakia( 12 ), better meeting the visual and developmental needs of pediatric patients. Intraocular lens implantation after lensectomy is possible by different means in those patients, but scleral-fixated intraocular lenses (IOLs) seem to offer the dual benefits of stabilizing the lens position and providing sustained visual improvement. The best timepoint for surgery in children with ectopia lentis still remains controversial, with early surgery as a potential benefit in regard to preventing amblyopia and potentially minimizing further axial lengthening( 5 ). Our study highlights the safety profile of this surgical technique. Potential vision threatening complications of scleral-fixated IOLs include retinal detachment, vitreous hemorrhage, choroidal hemorrhage, IOL subluxation( 13 ). In our cohort the perioperative and postoperative complication rates were remarkably low. Notably, there were no intraoperative complications reported, underscoring the reliability of the procedure when performed by an experienced surgeon. The postoperative vision- threatening complications were rare and readily manageable. We believe the low rate of dislocations were due to the lens choice (PMMA lens with adapted loops for fixation) and the covering of the prolene sutures with a scleral flap, preventing knot erosion through conjunctiva. Nevertheless, it should be kept in mind that patients with Marfan syndrome are at higher risk of scleral melting after any surgical procedure( 12 , 13 ). IOL subluxations usually happen through suture insufficiency or break, which can be provoked by either direct cutting by the haptic or by degradation of the polypropylene material itself( 14 ). The rate of IOL sublu xation in our study (14%) falls in the typical range of 7 to 15% reported in different studies( 12 , 13 , 15 , 16 ). Nervertheless, it is to report that even if 14% of eyes sustained an IOL subluxation, 21% of patients were affected and had to undergo an extra surgery for refixation, so that children and their parents have to be well informed preoperatively about the relative high risk of undergoing multiple surgeries, each of them carrying their own risks. Most data on this topic are nevertheless based on adult populations without Marfan syndrome or with shorter post-operative follow-up than our study. IOLs that can be fixated to the sclera without sutures such as the Carlevale, where haptics are secured within scleral pockets or under the conjunctiva, or with the flanged intrascleral fixation technique (Yamane), where the haptics are flanged with cautery and tucked under the conjunctiva, avoid suture-related complications. But the Carlevale IOL is currently not available in the USA and was reported in Marfan syndrome patients to lead to a higher rate of intra- and post-operative complications, including possible rupture of the T-shaped IOL harpoon( 11 ), and the rate of suture-related complications was very low in our cohort (2 patients). The Yamane technique was shown applicable in pediatric population if slightly modified( 17 ), but caution should be given to intrascleral haptics in Marfan patients that are prone to scleral melting. A further method usable in young children is the bag-in-the-lens technique but it necessitates enough remaining capsule which is often not the case in the Marfan population, and would need an adapted technique for these patients like the one presented by Van OS et al. with additional bean-shaped segments sutures to the sclera in order to ensure stability of the IOL over time( 16 ). As a further option, anterior chamber lenses have become safer due to innovative lens designs( 16 , 18 ) and may also provide good visual outcomes in children with ectopia lentis but complications such as IOL instability and corneal decompensation can be expected, especially in this young population with a long life expectation post-operative. An additional problem is the presence of iridodonesis with potential disabling visual symptoms. Iris-enclavated IOls like the artisan IOL also have good reported outcomes( 19 ) but further long-term data in children are needed. In terms of visual outcomes, our findings show stable vision correction over many years, providing both near and distance vision improvements, which have been maintained throughout the follow-up period. Given that the predictive error in IOL power calculation might be greater than in adults, careful consideration was given to the target refraction. Our results show first post-operative refractionvery close to the target refraction with expected slight hyperopia and myopization on the long term. The long-term visual acuity was very high compared to reported visual acuities after both lensectomy without IOL-implantation( 20 ) and after iris-fixated artisan-lens implantation( 21 ) and the incidence of amblyopia was low in our cohort (8%). Especially, 58% of the patients were less than 4 years old at time of surgery, thus possibly particularly profiting from IOL-implantation for amblyopia prevention. The refractive outcomes observed in our study underscore the importance of anticipating axial elongation over time, which led to a shift from hyperopia towards emmetropia or slight myopia in our study population, a desirable outcome in pseudophakic patients. Given the mean age at surgery and the extended follow-up in our cohort, we believe that the final refractive outcome greatly mirrors the expected refraction in adulthood of these patients, since axial length growth in pseudophakic eyes in Marfan syndrome usually ceases around 15 years of age( 22 ). A limitation of our study is its relatively small number of participants, which stems from the low prevalence of the conditions under investigation. The rarity of severe ectopia lentis in pediatric populations inherently limits the size of the study cohort, resulting in a broad range of ages at the time of surgery. This variability in patient age introduces challenges in standardizing outcomes, as the progression of axial length and refractive changes can differ based on the timing of the intervention and the underlying condition. It is worth noting that axial length in our cohort was within the normal range for age, which may indicate a lower risk of excessive axial elongation or unexpected myopic shift over time—introducing a potential selection bias. However, despite being in the normal range, the axial lengths were not far from those reported in 5-year-old Marfan patients in other studies, suggesting that progressive axial elongation may still occur, as typically observed in the Marfan population( 22 ). Additionally, a mean follow-up of 6 years may be insufficient to fully assess the extent of long-term axial growth and its associated retinal complications. The cohort size limits the generalizability of the findings. On the other hand, our patients had very classical findings of Marfan syndrome with high myopia despite flat corneas. The refractive results were acceptable despite the use of the SRK-T formula and the flat corneas of our patients. We believe that the use of new generation, multivariable formulas may further refine the refractive outcomes of those patients. Conclusions Scleral-fixated sulcus intraocular lenses seem to be a viable treatment option for pediatric ectopia lentis with acceptable long-term refractive results and incidence of amblyopia. Larger, prospective studies are warranted to refine our understanding of the optimal refractive targets for long-term visual outcomes in this patient population. Abbreviations IOL intraocular lens PMMA polymethylmethacrylate Declarations Ethics approval and consent to participate This study was performed in accordance with International Conference on Harmonization Good Clinical Practice guidelines and the tenets of the Declaration of Helsinki. Ethics approval was obtained by the ethical committee commission of Bern (ID 2021-01280). Ethics approval was obtained and written informed consent was collected from all parents. The trial number of this study is DLF 5322 and is registered under the BASEC number 2022-01557. Consent for publication Not applicable. Availability of data and materials The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. Competing interests The authors (K.V., O.H. and B.F.) declare that they have no competing interests. Funding No funding was received for this study. Authors' contributions K.V. contributed to the acquisition, analysis, and interpretation of data, and drafted or substantially revised the manuscript. O.B. contributed to the acquisition of the manuscript. F.B. contributed to the conception of the article and substantively revised it. All authors read and approved the final manuscript Acknowledgments Not applicable References Zadeh N, Bernstein JA, Niemi AK, Dugan S, Kwan A, Liang D, et al. Ectopia lentis as the presenting and primary feature in Marfan syndrome. 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Dev Ophthalmol. 2016;57:49–68. van Zeeburg EJT, Sminia ML, Schalij-Delfos NE. Long-term results of anterior chamber iris claw intraocular lens implantation in children with ectopia lentis in Marfan syndrome. J AAPOS Off Publ Am Assoc Pediatr Ophthalmol Strabismus. 2024 June;28(3):103922. Chen ZX, Jia WN, Ma Y, Chen TH, Hong JH, Sun Y, et al. Predicting axial length in patients with Marfan syndrome and ectopia lentis after modified capsular tension ring and intraocular lens implantation. J Cataract Refract Surg. 2023 June;49(6):571–7. Additional Declarations No competing interests reported. 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12:33:01","extension":"xml","order_by":12,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":64840,"visible":true,"origin":"","legend":"","description":"","filename":"a7382d28fe92484caed7328a7c5873171structuring.xml","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/fb61eeee4d8af3d111675224.xml"},{"id":97793975,"identity":"432d7df8-8e57-453a-b438-78d7afaf7c2e","added_by":"auto","created_at":"2025-12-09 12:33:01","extension":"html","order_by":13,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":71629,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/1722253974c478faa64828e9.html"},{"id":97793968,"identity":"52dd9a9a-8e53-40b3-ac44-0e43b16d8060","added_by":"auto","created_at":"2025-12-09 12:33:00","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":232079,"visible":true,"origin":"","legend":"\u003cp\u003eDistribution of preoperative and postoperative best-corrected distance visual acuity (BCDVA).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eLegend:\u003c/strong\u003e The boxplots show the best-corrected distance visual acuity preoperatively (left) and post-operatively at last follow-up (right) in LogMar scale.\u003c/p\u003e","description":"","filename":"figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/0a5e728d9abde51939bad539.png"},{"id":97896114,"identity":"0160c677-fab4-428d-833d-15378b6c61e8","added_by":"auto","created_at":"2025-12-10 15:35:54","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":214482,"visible":true,"origin":"","legend":"\u003cp\u003eDistribution of preoperative target refraction and postoperative spherical equivalents.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eLegend:\u003c/strong\u003e The boxplots show the target refraction (left) and the post-operative spherical equivalents at 1-month (middle) and at 6-year (right) in diopters.\u003c/p\u003e","description":"","filename":"figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/c599cbe879c12360942d4545.png"},{"id":97897358,"identity":"a9745536-3827-4a25-90b4-7ee5c3fc6c4c","added_by":"auto","created_at":"2025-12-10 15:37:46","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":191264,"visible":true,"origin":"","legend":"\u003cp\u003eDistribution of the refractive prediction error at 1-month.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eLegend:\u003c/strong\u003e The bar charts show the following distribution: 44% of spherical equivalents were more than 1D from the preoperative target refraction, 56% within 1D, 36% within 0.5D and 17% within 0.25D.\u003c/p\u003e","description":"","filename":"figure3.png","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/807b4d59381d6587b67b7f30.png"},{"id":98622653,"identity":"245528c7-0019-458e-853f-4ca4e77400a5","added_by":"auto","created_at":"2025-12-19 16:59:52","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1140298,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8096373/v1/04ffbe11-4da4-4a80-82fd-39aba9fc8855.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Visual outcomes of scleral-fixated posterior chamber intraocular lenses in children with genetic ectopia lentis: a 6-year retrospective study","fulltext":[{"header":"Background","content":"\u003cp\u003eEctopia lentis is a condition marked by the dislocation of the crystalline lens from its normal position, which can result in significant visual impairment. It usually manifests in the context of systemic disorders, though it can also arise due to trauma or idiopathic causes. Among the systemic diseases associated with ectopia lentis, Marfan syndrome is the most common(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). followed by conditions such as Weill-Marchesani syndrome(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e), and homocystinuria(\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). The condition can also occur in an isolated form due to mutations in the ADAMTSL4 gene on chromosome 1, although this is less frequent.\u003c/p\u003e\u003cp\u003eMarfan syndrome is an autosomal dominant disorder caused by mutations in the FBN1 gene located on chromosome 15 affecting the synthesis of fibrillin-1, a glycoprotein essential for the formation of elastic fibers in connective tissue. Children with Marfan syndrome have a higher risk of developing amblyopia due to high refractive error, anisometropia and ectopia lentis, retinal pathology or strabismus(\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eSince in children ectopia lentis is usually severe, preserving the capsular bag is less likely, so that leaving children aphakic with a later correction with glasses or contact lenses is common. Nevertheless, this has notable limitations like visual field narrowing, contact-lens related complications and discomfort and are associated with a high incidence of amblyopia(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). Different surgical methods are being used depending on the severity of the lens subluxation and capsular bag integrity(\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). Surgical options that do not let the children aphakic but do not need capsular support have been increasingly favored, including scleral-fixated intraocular lenses (IOLs). These IOLs are fixated to the sclera with sutures or suture-less, providing a stable optical correction that can significantly improve visual acuity and reduce the risk of amblyopia. This approach usually involves either a limbal or pars plana incision, which depends on various factors, including the degree of lens subluxation and the presence of any additional ocular pathologies. Despite the widespread adoption of surgical treatment of lens subluxation in children, long-term data on the ocular outcomes in this population are relatively scarce and there is today still no surgical approach defined as the gold standard for ectopia lentis in children(\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eOne of the critical considerations in pediatric IOL implantation is the anticipated growth of the eye, which typically involves an increase in axial length and a potential shift in refractive error over time. In patients with Marfan syndrome, this issue is even more pronounced due to their tendency to develop high axial lengths and significant myopia. A broadly chosen approach in younger children is to aim for a slightly hyperopic correction, allowing for the natural elongation of the eye over time, with the expectation that the patient will achieve emmetropia or a slight myopia in later years. However, the degree of hyperopia must be tailored to the patient\u0026rsquo;s age and expected ocular growth since excessive hyperopic correction is problematic in pseudophakic patients without accommodative power.\u003c/p\u003e\u003cp\u003eThis study retrospectively examines the long-term outcomes of scleral-fixated sulcus IOLs in pediatric patients with ectopia lentis, reporting an evaluation of the safety profile of the procedure, the stability of visual outcomes over time, and the effectiveness of the selected refractive targets in managing the anticipated growth of the eye.\u003c/p\u003e\u003cp\u003eGiven the challenges associated with this condition, particularly in young patients, the insights of this study might help refine surgical techniques and improve the overall management strategy for achieving optimal visual outcomes in this population.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003eThe aim of this study was to evaluate the long-term visual outcomes after scleral-fixated IOL implantation in children with ectopia lentis in order to assess if this procedure represents a viable and effective long-term solution for these patients.\u003c/p\u003e\u003cp\u003eWe retrospectively analyzed all pediatric patients who underwent lensectomy, anterior vitrectomy and scleral-fixated sulcus intraocular lenses for the treatment of ectopia lentis from January 2012 to November 2024. Inclusion criteria were a diagnosis of non-traumatic ectopia lentis, age below 12 years, a minimal follow-up of two years and indication for surgery such as significant dislocation of the crystalline lens with lack of improvement in best corrected visual acuity with conservative management. Parents were informed about the possibility to leave the eye aphakic. They were informed about the advantages (no need for aphakic correction) and the disadvantages of the procedure (possible late-onset suture breaks with need to re-suture, lack of long-term results in children, etc.).\u003c/p\u003e\u003cp\u003eAll patients underwent genetic testing for diagnostic purposes. Patients with Marfan syndrome were specifically tested for mutations in the FBN1 gene. In cases where another disease was suspected as the underlying cause of ectopia lentis, an extended genetic panel was conducted.\u003c/p\u003e\u003cp\u003ePatients underwent optical biometry preoperatively with IOL master (Zeiss) and corneal topography with Pentacam (Oculus) and TMS (Tomey). In accordance to the axial lengths measured preoperatively with caution given to standardizing the procedure for all patients, the SRK-T formula, adequate for the pediatric population(\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e), was used in all patients to calculate the IOL power. The surgical procedure consisted of a corneoscleral tunnel and the preparation of two scleral pockets at 3\u0026rsquo; and 9\u0026rsquo; o\u0026rsquo;clock, manual capsulorhexis, then lens aspiration with bimanual I/A using hooks to stabilize the capsular bag followed by capsule removal and anterior vitrectomy and the implantation through a 6.5mm corneoscleral tunnel of a PMMA intraocular lens (IOL) in the sulcus. The lens was sutured to the sclera at the 3\u0026rsquo; and 9\u0026rsquo; clock hours using a double-armed 10\u0026thinsp;\u0026minus;\u0026thinsp;0 prolene suture using a curved needle. The scleral flaps were then sutured with 10\u0026thinsp;\u0026minus;\u0026thinsp;0 nylon. The two one-piece PMMA lenses used were PC 279Y IOL (Ophtec BV), which has 1 positioning hole per haptic and 81B IOL (Morcher), which has 3 positioning holes per haptic. The optics were 6.5mm.\u003c/p\u003e\u003cp\u003eAll eyes underwent this technique. The target refraction, whether hyperopic or emmetropic, was selected based on the patient's age at the time of surgery. In general, +2D was targeted for patients under 3 years old, +1D under 4 years old, and emmetropia from 4 years old on. Despite emmetropization is usually reached by age of 9 years old, patients with Marfan syndrome undergo further axial length growth through life(\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e), which is why a moderate hypermetropia was targeted in order to minimize undesired myopic shift in adulthood.\u003c/p\u003e\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eVisual and refractive examinations\u003c/h2\u003e\u003cp\u003ePatients underwent refractive examinations both preoperatively and postoperatively, with long-term follow-up over several years. The two reported time points are the initial postoperative refraction testing one month after surgery, and the last postoperative consultation by November 2024. All visual acuities were recorded using the LogMAR scale. Refractive examinations were performed by experienced orthoptists using either retinoscopy or subjective refraction, depending on the patient\u0026rsquo;s age.\u003c/p\u003e\u003cp\u003eIn addition, patients underwent corneal topography either with Pentacam (Oculus Gmbh) oder with TMS-IV (Tomey), and the differences in cylinder power and axis between corneal topography and manifest refraction was investigated.\u003c/p\u003e\u003cp\u003eAmblyopia was defined as an interocular difference of \u0026ge;\u0026thinsp;2 lines in visual acuity (VA), or a VA\u0026thinsp;\u0026le;\u0026thinsp;0.2 logMar, that could not be attributed to any ocular or visual system abnormality or to refractive error.\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e\u003ch2\u003eStatistical analysis\u003c/h2\u003e\u003cp\u003eThe statistical analysis involved solely descriptive statistics. Results are reported in percentages and, when applicable, in means and standard deviations. All descriptive statistical analyses were performed using \u003cb\u003eR\u003c/b\u003e version 2023.09.1 (Posit Software, PBC).\u003c/p\u003e\u003c/div\u003e"},{"header":"RESULTS","content":"\u003cdiv id=\"Sec6\" class=\"Section2\"\u003e\u003ch2\u003ePatients demographics and characteristics\u003c/h2\u003e\u003cp\u003eA total of 19 patients (36 eyes) were included in this study. The mean age of the patients at the time of surgery was 5.1\u0026thinsp;\u0026plusmn;\u0026thinsp;1.9 years and at last follow-up was 10.1\u0026thinsp;\u0026plusmn;\u0026thinsp;3.2 years. Eleven (58%) patients were \u0026lt;\u0026thinsp;4 years old at time of surgery. Seventeen patients (89%) had bilateral ectopia lentis, resulting in a total of 34 eyes affected bilaterally, while 2 patients had only one eye treated. Of the 19 patients, 18 (95%) were diagnosed with Marfan syndrome. Genetic testing confirmed FBN1 gene mutations in all patients with Marfan syndrome, including various missense mutations such as p.Arg122Cys, p.Cys1361Arg, and p.Cys781Tyr, as well as frameshift mutations like 3688delA. One patient was diagnosed with Weill-Marchesani syndrome and exhibited a de novo heterozygous missense variant in the FBN1 gene. The mean follow-up duration for all patients was 6.1\u0026thinsp;\u0026plusmn;\u0026thinsp;3.1 years.\u003c/p\u003e\u003c/div\u003e\n\u003ch3\u003eVisual and refractive outcome\u003c/h3\u003e\n\u003cp\u003eThe PC 279Y IOL was implanted in 19 eyes (53%), while the 81B IOL was implanted in 18 eyes (47%). The selection of the IOL type was based on surgeon preference and specific anatomical considerations for each case. The mean axial length preoperatively was 22.7\u0026thinsp;\u0026plusmn;\u0026thinsp;1.3 mm. The average keratometry was 42.7\u0026thinsp;\u0026plusmn;\u0026thinsp;2.3 when measured with the Pentacam and 41.7\u0026thinsp;\u0026plusmn;\u0026thinsp;1.9D when measured with the TMS-3, revealing flat corneas.\u003c/p\u003e\u003cp\u003ePreoperatively, the visual acuities were as follows: the mean best-corrected near monocular visual acuity (NBCVA) was 0.72\u0026thinsp;\u0026plusmn;\u0026thinsp;0.31 on the LogMAR scale, and the mean distance monocular best-corrected visual acuity (DBCVA) was 0.54\u0026thinsp;\u0026plusmn;\u0026thinsp;0.27.\u003c/p\u003e\u003cp\u003eFollowing surgery, the monocular NBCVA improved to 0.19\u0026thinsp;\u0026plusmn;\u0026thinsp;0.28, while the DBCVA reached 0.05\u0026thinsp;\u0026plusmn;\u0026thinsp;0.22 at the latest follow-up. Boxplots of the pre- and postoperative DBCVA are reported (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eLinear regression found statistically significant association between preoperative DBCVA and DBCVA at last follow-up (R\u003csup\u003e2\u003c/sup\u003e\u0026thinsp;=\u0026thinsp;0.537, p\u0026thinsp;\u0026lt;\u0026thinsp;0.0001), as well as between preoperative NBCVA and NBCVA at last follow-up (R\u003csup\u003e2\u003c/sup\u003e\u0026thinsp;=\u0026thinsp;0.208, p\u0026thinsp;\u0026lt;\u0026thinsp;0.01).\u003c/p\u003e\u003cp\u003eThe preoperative mean spherical equivalent was \u0026minus;\u0026thinsp;6.3\u0026thinsp;\u0026plusmn;\u0026thinsp;4.6 diopters (D) with a mean cylindrical power of 5.0\u0026thinsp;\u0026plusmn;\u0026thinsp;2.9D. The mean target refraction for postoperative correction was set at +\u0026thinsp;0.88\u0026thinsp;\u0026plusmn;\u0026thinsp;1.3D. At one-month post-surgery, the mean spherical equivalent was +\u0026thinsp;1.4\u0026thinsp;\u0026plusmn;\u0026thinsp;1.4D and the mean cylindrical power had reduced to 2.7\u0026thinsp;\u0026plusmn;\u0026thinsp;2.1D. At 6-year, a myopic shift was observed. The difference between spherical equivalents at 6-year and at 1-month was \u0026minus;\u0026thinsp;2.26\u0026thinsp;\u0026plusmn;\u0026thinsp;2.11D, and the spherical equivalent was \u0026minus;\u0026thinsp;0.7\u0026thinsp;\u0026plusmn;\u0026thinsp;2.6D at 6-year. The mean cylindrical power further reduced to 2.2\u0026thinsp;\u0026plusmn;\u0026thinsp;1.4D at 6-year. Boxplots of target refraction and postoperative spherical equivalents are shown (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eRefractive prediction error (PE) was determined as the difference between first post-operative refraction at 1-month and the target refraction. The PE was 0.59\u0026thinsp;\u0026plusmn;\u0026thinsp;1.01D. As shown (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e), 56% of eyes had a SE within 1D of the target refraction, 36% within 0.5D and 17% within 0.25D.\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eIn addition, the difference in cylinder power between corneal astigmatism in topography and and manifest astigmatism at 6-year was 0.11\u0026thinsp;\u0026plusmn;\u0026thinsp;1.75D and in cylinder axis was 18.83\u0026thinsp;\u0026plusmn;\u0026thinsp;16.81 degrees.\u003c/p\u003e\u003cp\u003eOrthoptic examinations identified various strabismic deviations. Eleven eyes (31%) were orthophoric. Amblyopia was identified in 3 eyes (8%), while 27 eyes (92%) showed no signs of amblyopia. The three eyes were diagnosed with deprivation amblyopia. Eleven eyes were operated on when patients were less than 4 years of age thus especially at risk for amblyopia.\u003c/p\u003e\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e\u003ch2\u003eComplications and comorbidities\u003c/h2\u003e\u003cp\u003eNo intraoperative complications occurred. Postoperatively, complications included visual relevant IOL subluxation in 14% of eyes (in 21% of patients), 4 out of 5 were spontaneous subluxations and one was traumatic. The 4 spontaneous IOL subluxations happened 8.5\u0026thinsp;\u0026plusmn;\u0026thinsp;2.2 years post-operative (range 5.4 to 10.1 years). Three eyes required pars plana vitrectomy and IOL refixation using Gore-Tex sutures and one IOL-refixation with prolene-suture without pars plana vitrectomy. One eye experienced a subluxation without visual symptoms and did not require surgical intervention. Six percents of eyes showed strong suture-induced astigmatism, necessitating suture lysis to improve refractive outcomes.\u003c/p\u003e\u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn this study, we present the refractive and visual outcomes over several years following lensectomy combined with scleral-fixated intraocular sulcus lenses in the management of ectopia lentis in pediatric patients. Additionally, we examine the associated orthoptic and systemic comorbidities in this patient population, which is critical for understanding the broader implications of the condition and its treatment.\u003c/p\u003e\u003cp\u003eThis study addresses the fact that crystalline lens subluxation in the context of genetic syndromes poses significant challenges in pediatric ophthalmology due to the need to balance immediate visual correction with the refractive changes that occur over the years as the child grows. Some studies that investigated the outcomes of aphakic children have shown that it is problematic due to the limitations of glasses and contact lenses, as well as the increased risk of amblyopia and suboptimal visual development. Better visual improvement was reported when lensectomy is followed by IOL implantation in comparison to aphakia(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e), better meeting the visual and developmental needs of pediatric patients. Intraocular lens implantation after lensectomy is possible by different means in those patients, but scleral-fixated intraocular lenses (IOLs) seem to offer the dual benefits of stabilizing the lens position and providing sustained visual improvement. The best timepoint for surgery in children with ectopia lentis still remains controversial, with early surgery as a potential benefit in regard to preventing amblyopia and potentially minimizing further axial lengthening(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eOur study highlights the safety profile of this surgical technique. Potential vision threatening complications of scleral-fixated IOLs include retinal detachment, vitreous hemorrhage, choroidal hemorrhage, IOL subluxation(\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). In our cohort the perioperative and postoperative complication rates were remarkably low. Notably, there were no intraoperative complications reported, underscoring the reliability of the procedure when performed by an experienced surgeon. The postoperative vision- threatening complications were rare and readily manageable. We believe the low rate of dislocations were due to the lens choice (PMMA lens with adapted loops for fixation) and the covering of the prolene sutures with a scleral flap, preventing knot erosion through conjunctiva. Nevertheless, it should be kept in mind that patients with Marfan syndrome are at higher risk of scleral melting after any surgical procedure(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). IOL subluxations usually happen through suture insufficiency or break, which can be provoked by either direct cutting by the haptic or by degradation of the polypropylene material itself(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). The rate of IOL sublu xation in our study (14%) falls in the typical range of 7 to 15% reported in different studies(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e). Nervertheless, it is to report that even if 14% of eyes sustained an IOL subluxation, 21% of patients were affected and had to undergo an extra surgery for refixation, so that children and their parents have to be well informed preoperatively about the relative high risk of undergoing multiple surgeries, each of them carrying their own risks. Most data on this topic are nevertheless based on adult populations without Marfan syndrome or with shorter post-operative follow-up than our study. IOLs that can be fixated to the sclera without sutures such as the Carlevale, where haptics are secured within scleral pockets or under the conjunctiva, or with the flanged intrascleral fixation technique (Yamane), where the haptics are flanged with cautery and tucked under the conjunctiva, avoid suture-related complications. But the Carlevale IOL is currently not available in the USA and was reported in Marfan syndrome patients to lead to a higher rate of intra- and post-operative complications, including possible rupture of the T-shaped IOL harpoon(\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e), and the rate of suture-related complications was very low in our cohort (2 patients). The Yamane technique was shown applicable in pediatric population if slightly modified(\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e), but caution should be given to intrascleral haptics in Marfan patients that are prone to scleral melting. A further method usable in young children is the bag-in-the-lens technique but it necessitates enough remaining capsule which is often not the case in the Marfan population, and would need an adapted technique for these patients like the one presented by Van OS et al. with additional bean-shaped segments sutures to the sclera in order to ensure stability of the IOL over time(\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e). As a further option, anterior chamber lenses have become safer due to innovative lens designs(\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e) and may also provide good visual outcomes in children with ectopia lentis but complications such as IOL instability and corneal decompensation can be expected, especially in this young population with a long life expectation post-operative. An additional problem is the presence of iridodonesis with potential disabling visual symptoms. Iris-enclavated IOls like the artisan IOL also have good reported outcomes(\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e) but further long-term data in children are needed.\u003c/p\u003e\u003cp\u003eIn terms of visual outcomes, our findings show stable vision correction over many years, providing both near and distance vision improvements, which have been maintained throughout the follow-up period. Given that the predictive error in IOL power calculation might be greater than in adults, careful consideration was given to the target refraction. Our results show first post-operative refractionvery close to the target refraction with expected slight hyperopia and myopization on the long term. The long-term visual acuity was very high compared to reported visual acuities after both lensectomy without IOL-implantation(\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e) and after iris-fixated artisan-lens implantation(\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e) and the incidence of amblyopia was low in our cohort (8%). Especially, 58% of the patients were less than 4 years old at time of surgery, thus possibly particularly profiting from IOL-implantation for amblyopia prevention.\u003c/p\u003e\u003cp\u003eThe refractive outcomes observed in our study underscore the importance of anticipating axial elongation over time, which led to a shift from hyperopia towards emmetropia or slight myopia in our study population, a desirable outcome in pseudophakic patients. Given the mean age at surgery and the extended follow-up in our cohort, we believe that the final refractive outcome greatly mirrors the expected refraction in adulthood of these patients, since axial length growth in pseudophakic eyes in Marfan syndrome usually ceases around 15 years of age(\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eA limitation of our study is its relatively small number of participants, which stems from the low prevalence of the conditions under investigation. The rarity of severe ectopia lentis in pediatric populations inherently limits the size of the study cohort, resulting in a broad range of ages at the time of surgery. This variability in patient age introduces challenges in standardizing outcomes, as the progression of axial length and refractive changes can differ based on the timing of the intervention and the underlying condition. It is worth noting that axial length in our cohort was within the normal range for age, which may indicate a lower risk of excessive axial elongation or unexpected myopic shift over time\u0026mdash;introducing a potential selection bias. However, despite being in the normal range, the axial lengths were not far from those reported in 5-year-old Marfan patients in other studies, suggesting that progressive axial elongation may still occur, as typically observed in the Marfan population(\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e). Additionally, a mean follow-up of 6 years may be insufficient to fully assess the extent of long-term axial growth and its associated retinal complications.\u003c/p\u003e\u003cp\u003eThe cohort size limits the generalizability of the findings. On the other hand, our patients had very classical findings of Marfan syndrome with high myopia despite flat corneas. The refractive results were acceptable despite the use of the SRK-T formula and the flat corneas of our patients. We believe that the use of new generation, multivariable formulas may further refine the refractive outcomes of those patients.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eScleral-fixated sulcus intraocular lenses seem to be a viable treatment option for pediatric ectopia lentis with acceptable long-term refractive results and incidence of amblyopia. Larger, prospective studies are warranted to refine our understanding of the optimal refractive targets for long-term visual outcomes in this patient population.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eIOL\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eintraocular lens\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003ePMMA\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003epolymethylmethacrylate\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was performed in accordance with International Conference on Harmonization Good Clinical Practice guidelines and the tenets of the Declaration of Helsinki. Ethics approval was obtained by the ethical committee commission of Bern (ID 2021-01280). Ethics approval was obtained and written informed consent was collected from all parents. The trial number of this study is DLF 5322 and is registered under the BASEC number 2022-01557.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors (K.V., O.H. and B.F.) declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNo funding was received for this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eK.V. \u0026nbsp;contributed to the acquisition, analysis, and interpretation of data, and drafted or substantially revised the manuscript.\u003c/p\u003e\n\u003cp\u003eO.B. contributed \u0026nbsp;to the acquisition of the manuscript.\u003c/p\u003e\n\u003cp\u003eF.B. contributed to the conception of the article and \u0026nbsp;substantively revised it.\u003c/p\u003e\n\u003cp\u003eAll authors read and approved the final manuscript\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgments\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable \u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eZadeh N, Bernstein JA, Niemi AK, Dugan S, Kwan A, Liang D, et al. Ectopia lentis as the presenting and primary feature in Marfan syndrome. Am J Med Genet A. 2011 Nov;155A(11):2661\u0026ndash;8. \u003c/li\u003e\n\u003cli\u003eChu BS. Weill-Marchesani syndrome and secondary glaucoma associated with ectopia lentis. Clin Exp Optom. 2006 Mar;89(2):95\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eSabrane I, Saoudi S, El Ikhloufi M, Elkaissoumi L, Taouri N, Amazouzi A, et al. Ectopia lentis in homocystinuria. J Fr Ophtalmol. 2019 Feb;42(2):219\u0026ndash;20. \u003c/li\u003e\n\u003cli\u003eEsfandiari H, Ansari S, Mohammad-Rabei H, Mets M. Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective. J Ophthalmic Vis Res. 2019 Jan;14(1):71\u0026ndash;7. \u003c/li\u003e\n\u003cli\u003eRomano P, Kerr N, 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; \u003c/li\u003e\n\u003cli\u003eDogaroiu AC, Dudau M, Dogaroiu C, Tataru CP. Visual Outcomes in Ectopia Lentis in Marfan Syndrome: A Study of Four Surgical Techniques in Children and Adults. Med 2024 Vol 60 Page 1098. 2024 July;60(7):1098\u0026ndash;1098. \u003c/li\u003e\n\u003cli\u003eMandal S, Singhal D, Saluja G, Nagpal R, Tripathy K, Tripathi M, et al. Management of ectopia lentis in children. Saudi J Ophthalmol. 2024 July;38(3):226\u0026ndash;226. \u003c/li\u003e\n\u003cli\u003eVasavada V, Shah SK, Vasavada VA, Vasavada AR, Trivedi RH, Srivastava S, et al. Comparison of IOL power calculation formulae for pediatric eyes. Eye Lond Engl. 2016 Sept;30(9):1242\u0026ndash;50. \u003c/li\u003e\n\u003cli\u003eZhang M, Chen T, Deng M, Chen Z, Jiang Y. Accuracy of IOL power calculation formulas in Marfan lens subluxation patients with in-the-bag IOLs and implantation of scleral-sutured single-eyelet modified capsular tension rings. J Cataract Refract Surg. 2021 Nov;47(11):1423\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eDahan E, Ophth M, Drusedau MUH. Choice of lens and dioptric power in pediatric pseudophakia. J Cataract Refract Surg. 1997;23 Suppl 1(5):618\u0026ndash;23. \u003c/li\u003e\n\u003cli\u003eEnyedi LB, Peterseim MW, Freedman SF, Buckley EG. Refractive changes after pediatric intraocular lens implantation. Am J Ophthalmol. 1998 Dec;126(6):772\u0026ndash;81. \u003c/li\u003e\n\u003cli\u003eRezar-Dreindl S, Stifter E, Neumayer T, Papp A, Gschliesser A, Schmidt-Erfurth U. Visual outcome and surgical results in children with Marfan syndrome. Clin Experiment Ophthalmol. 2019 Dec;47(9):1138\u0026ndash;1138. \u003c/li\u003e\n\u003cli\u003eNg J, Behshad S, Farid M. Review of Surgical Techniques for Posterior Chamber Intraocular Lens Fixation in the Absence of Capsular Lens Support. US Ophthalmic Rev. 2015;08(02):86\u0026ndash;86. \u003c/li\u003e\n\u003cli\u003eSen P, Attiku Y, Bhende P, Rishi E, Ratra D, Sreelakshmi K. Outcome of sutured scleral fixated intraocular lens in Marfan syndrome in pediatric eyes. Int Ophthalmol. 2020 June;40(6):1531\u0026ndash;8. \u003c/li\u003e\n\u003cli\u003eKim Y, Choi EY, Lee CS, Kim SS, Byeon SH. Clinical Characteristics of recurrent intraocular lens dislocation after scleral-fixated sutured intraocular lens and long-term outcomes of intraocular lens re-fixation. Graefes Arch Clin Exp Ophthalmol Albrecht Von Graefes Arch Klin Exp Ophthalmol. 2022 Oct;260(10):3267\u0026ndash;73. \u003c/li\u003e\n\u003cli\u003eLee J, Lee J, Lee CS, Kim M, Byeon SH, Kim SS, et al. Factors predicting redislocation or suture-break in eyes after scleral-fixated intraocular lens. J Cataract Refract Surg [Internet]. 2024 Oct; Available from: https://journals.lww.com/jcrs/fulltext/2024/10000/factors_predicting_redislocation_or_suture_break.9.aspx\u003c/li\u003e\n\u003cli\u003eSternfeld A, Taranum Basith SS, Kurup SP, Basti S. Secondary intraocular lens implantation using the flanged intrascleral fixation technique in pediatric aphakia: case series and review of literature. J Am Assoc Pediatr Ophthalmol Strabismus. 2020 Oct 1;24(5):286.e1-286.e6. \u003c/li\u003e\n\u003cli\u003eVan Os L, Hildebrand GD, Tassignon MJ. Adapted Bag-in-the-Lens Implantation Technique in Children with Congenital Ectopia Lentis. Klin Monatsbl Augenheilkd. 2021 Oct;238(10):1058\u0026ndash;64. \u003c/li\u003e\n\u003cli\u003eDonaldson KE, Gorscak JJ, Budenz DL, Feuer WJ, Benz MS, Forster RK. Anterior chamber and sutured posterior chamber intraocular lenses in eyes with poor capsular support. J Cataract Refract Surg. 2005 May;31(5):903\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eRepka MX. Visual Rehabilitation in Pediatric Aphakia. Dev Ophthalmol. 2016;57:49\u0026ndash;68. \u003c/li\u003e\n\u003cli\u003evan Zeeburg EJT, Sminia ML, Schalij-Delfos NE. Long-term results of anterior chamber iris claw intraocular lens implantation in children with ectopia lentis in Marfan syndrome. J AAPOS Off Publ Am Assoc Pediatr Ophthalmol Strabismus. 2024 June;28(3):103922. \u003c/li\u003e\n\u003cli\u003eChen ZX, Jia WN, Ma Y, Chen TH, Hong JH, Sun Y, et al. Predicting axial length in patients with Marfan syndrome and ectopia lentis after modified capsular tension ring and intraocular lens implantation. J Cataract Refract Surg. 2023 June;49(6):571\u0026ndash;7. \u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-ophthalmology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"boph","sideBox":"Learn more about [BMC Ophthalmology](http://bmcophthalmol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/boph","title":"BMC Ophthalmology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Ectopia lentis, Marfan syndrome, sclera-fixated intraocular lenses","lastPublishedDoi":"10.21203/rs.3.rs-8096373/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8096373/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e\u003cp\u003eTo assess the feasibility and outcomes of lensectomy with sulcus-sutured intraocular lens (IOL) implantation in pediatric patients with atraumatic ectopia lentis.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003ePediatric patients with atraumatic (sub-)luxated crystalline lenses that underwent lensectomy, anterior vitrectomy and sclera-fixated implantation of a polymethylmethacrylate (PMMA) IOL in the sulcus were included in this retrospective study. Outcome measures included corrected distance and near visual acuities (LogMar), refractive outcomes (spherical equivalent, prediction error), and complication rates. Follow-up averaged 6.14\u0026thinsp;\u0026plusmn;\u0026thinsp;3.1 years with a minimal follow-up of two years and a maximal follow-up of 11 years.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003eA total of 19 children (36 eyes; 55% male; mean age 5.11\u0026thinsp;\u0026plusmn;\u0026thinsp;1.9 years) were included. Preoperative mean best corrected visual acuity (BCVA) improved from 0.72\u0026thinsp;\u0026plusmn;\u0026thinsp;0.3 (near) and 0.48\u0026thinsp;\u0026plusmn;\u0026thinsp;0.2 (distance) to 0.14\u0026thinsp;\u0026plusmn;\u0026thinsp;0.3 and \u0026minus;\u0026thinsp;0.01\u0026thinsp;\u0026plusmn;\u0026thinsp;0.1 postoperatively, respectively. Spherical equivalent changed from \u0026minus;\u0026thinsp;6.28\u0026thinsp;\u0026plusmn;\u0026thinsp;4.6 diopters (D) preoperatively to +\u0026thinsp;1.41\u0026thinsp;\u0026plusmn;\u0026thinsp;1.4D at one month, to -0.26\u0026thinsp;\u0026plusmn;\u0026thinsp;3.6D at 6-year. The difference between spherical equivalent and targeted refraction was 0.59\u0026thinsp;\u0026plusmn;\u0026thinsp;1.01D at 1-month and \u0026minus;\u0026thinsp;1.69\u0026thinsp;\u0026plusmn;\u0026thinsp;2.3D at 6-year. Complications included 5 cases of IOL subluxation (4 spontaneous and one traumatic).\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e\u003cp\u003eLensectomy with scleral-fixated sulcus IOL implantation is an option for managing pediatric patients with ectopia lentis, yielding significant visual improvement and manageable refractive outcomes.\u003c/p\u003e","manuscriptTitle":"Visual outcomes of scleral-fixated posterior chamber intraocular lenses in children with genetic ectopia lentis: a 6-year retrospective study","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-12-09 12:32:56","doi":"10.21203/rs.3.rs-8096373/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2026-01-12T02:17:43+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-01-03T07:03:56+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-01-02T09:44:09+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-12-24T16:34:44+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"190850317210162766643083274448542502176","date":"2025-12-23T19:39:34+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"41854311346786455743824472694292957369","date":"2025-12-20T11:41:55+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-12-15T11:01:24+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"167305650285336909811336126823449719546","date":"2025-12-13T02:40:24+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"27749312529279297325385652758759856360","date":"2025-12-09T05:44:23+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"235562611374211263411361019624975742396","date":"2025-12-08T09:33:35+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-12-05T10:10:44+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2025-11-13T11:40:47+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-11-13T11:10:42+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-11-13T11:10:18+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Ophthalmology","date":"2025-11-12T12:32:17+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-ophthalmology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"boph","sideBox":"Learn more about [BMC Ophthalmology](http://bmcophthalmol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/boph","title":"BMC Ophthalmology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"e88afd83-dad6-4b7d-8014-fa3db88b7e61","owner":[],"postedDate":"December 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"in-revision","subjectAreas":[],"tags":[],"updatedAt":"2026-05-19T07:39:21+00:00","versionOfRecord":[],"versionCreatedAt":"2025-12-09 12:32:56","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8096373","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8096373","identity":"rs-8096373","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}