Outcomes after lensectomy for children with Marfan syndrome

Manning, Sonia; Lanigan, Bernadette; O’Keefe, Michael

doi:10.1016/j.jaapos.2016.02.006

Cited by 24 publications

(53 citation statements)

References 20 publications

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“…[17,18]. Patients with ectopia lentis in our study showed a good median BCVA of 6/9, which is comparable to various other studies [19][20][21][22][23].…”

Section: Discussionsupporting

confidence: 89%

Clinical Profile and Visual Outcome of Pediatric Cataract Surgeries in West Malaysia

Ting

Teh

Chan

et al. 2020

MJPCH

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Purpose: We aim to describe the clinical profile and visual outcome of paediatric patients who underwent cataract surgery in a tertiary ophthalmology referral centre in West Malaysia from 2013 to 2018. Methods: This is a retrospective review of all paediatric patients who underwent cataract surgery in our centre from 2013-2018. Results: A total of 35 eyes from 23 patients were included. There were 10 (43.5%) female and 13 (56.5%) male patients. Twelve patients (24 eyes) had bilateral cataract while 11 patients had unilateral cataract. Sixteen (45.7%) eyes had congenital cataract, followed by ectopia lentis (n=10, 28.6%), traumatic cataract (n=8, 22.9%) and steroid induced cataract (n=1, 2.8%). Three types of intraocular lens (IOL) were implanted: which were posterior chamber IOL (n=22, 62.9%), iris claw IOL (n=12, 34.3%) and scleral-fixated IOL (n=1, 2.8%). Majority of eyes (n=28, 80%) had primary IOL implantation. Twenty-five (71.4%) eyes achieved best corrected visual acuity (BCVA) of 6/12 and better at 6 months post-IOL implantation. There was no statistically significant difference in the BCVA at 6 months post-IOL implantation among the different cataract aetiology, primary or secondary IOL implantation and types of IOL implant. Eight (22.9%) eyes developed post-operative complications, which included posterior capsular opacification (PCO) (n=6, 17.1%), IOL decentration (n=4, 11.4%) and glaucoma (n=1, 2.8%). Nineteen (82.6%) patients required glasses for visual rehabilitation. Conclusion: Majority of the paediatric cataract patients achieved BCVA of 6/12 or better at 6 months post-IOL implantation. The visual outcome among the different cataract aetiology, primary or secondary IOL implantation and types of IOL implanted were similar. PCO was the most common post-operative complication.

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“…[17,18]. Patients with ectopia lentis in our study showed a good median BCVA of 6/9, which is comparable to various other studies [19][20][21][22][23].…”

Section: Discussionsupporting

confidence: 89%

Clinical Profile and Visual Outcome of Pediatric Cataract Surgeries in West Malaysia

Ting

Teh

Chan

et al. 2020

MJPCH

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“…RRD occurred intra-/post-operative in 64%, the remainder being surgically naïve eyes. The RRD rate in this pedigree is significantly higher than the reported rate of EL-related RRD, (0-25%) [6,11,[16][17][18][19][20].…”

Section: Resultsmentioning

confidence: 53%

A FBN1 variant manifesting as non-syndromic ectopia lentis with retinal detachment: clinical and genetic characteristics

Stephenson

Dockery

O’Keefe

et al. 2019

Eye

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Background/objectives Fibrillin-1 (FBN1) mutations cause connective tissue dysgenesis the main ocular manifestation being ectopia lentis (EL), which may be syndromic or non-syndromic. We describe a pedigree with a FBN1 mutation causing non-syndromic EL with retinal detachment (RRD) and their management. Subjects/methods Patients with familial EL with RRD were invited to participate (vitreoretinopathy branch of Target 5000, the Irish inherited retinal degeneration study). All patients signed full informed consent. The study was approved by the Institutional Review Board of the Mater Hospital, Dublin and abided by the Declaration of Helsinki. Results Seven adults were affected with bilateral EL. All subjects had RRD with bilateral non-synchronous RRD in 57%. Conclusions The FBN1 variant described herein confers an increased risk of both EL and RRD and can now be upgraded to 'pathogenic' ACMG status.

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“…ere are also cases of Artisan aphakic IOL implantation in children with good clinical outcomes. Artisan aphakic IOL is both feasible and effective in managing ectopia lentis in children [12][13][14]. Sminia et al also reported Artisan aphakic IOL implantation in children with traumatic pediatric cataract with a follow-up period of 11.0 years [15] and in children with partial aniridia caused by perforating ocular trauma for a follow-up period of 8.9 years [16].…”

Section: Discussionmentioning

confidence: 98%

The Influence of Decentration on Higher-Order Aberrations in Artisan Aphakic Intraocular Lens Implantation Eyes

Miao

Zhang

Chen

et al. 2020

Journal of Ophthalmology

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Purpose. To characterize the influence of decentration on higher-order aberrations of Artisan aphakic intraocular lens implantation eyes. Setting. Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai, China. Design. Retrospective case series. Methods. Twenty-three eyes of 18 patients were retrospectively examined. Location of the IOL was imaged using a slit-lamp, and decentration (the distance from the center of a pupil to the geometric center of the IOL) was measured using the AutoCAD 2007 software. Ocular and internal higher-order aberrations were measured using the wavefront analyzer KR-1W (Topcon) and their correlation with decentration were analyzed. Coma, spherical, 3rd-, 4th-order, trefoil, tetrafoil aberrations, ocular, and internal higher-order aberrations were calculated for a 4.0 mm pupil diameter. Results. The mean age of patients was 33.6 ± 21.4 years (ranging from 7 to 72 years). The mean follow-up period was 28.2 ± 10.5 months (ranging from 12 to 52 months). The mean postoperative best-corrected visual acuity (BCVA) was 0.19 ± 0.19 logMAR (range −0.18–0.52). The mean decentration was 0.57 ± 0.28 mm (range 0.15–1.21 mm). There was no correlation between Artisan IOL decentration and ocular higher-order aberrations, internal higher-order aberrations, coma, spherical, 3rd-, 4th-order, trefoil, and tetrafoil aberrations, respectively. There was a positive correlation between Artisan IOL decentration and 2nd-astig aberrations when 2nd-astig aberrations were less than 0.1. Conclusions. The position of Artisan IOLs showed slight decentration and the amounts of decentration were not large enough to influence higher-order aberrations.

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Outcomes after lensectomy for children with Marfan syndrome

Cited by 24 publications

References 20 publications

Clinical Profile and Visual Outcome of Pediatric Cataract Surgeries in West Malaysia

Clinical Profile and Visual Outcome of Pediatric Cataract Surgeries in West Malaysia

A FBN1 variant manifesting as non-syndromic ectopia lentis with retinal detachment: clinical and genetic characteristics

The Influence of Decentration on Higher-Order Aberrations in Artisan Aphakic Intraocular Lens Implantation Eyes

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