Management of Ectopia Lentis in a Family With Marfan Syndrome

Koenig, Steven B.

doi:10.1001/archopht.1996.01100140260001

Cited by 35 publications

(22 citation statements)

References 13 publications

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“…In some studies (6,13) the main indication for surgery was low corrected visual acuity. Glare and monocular diplopia were also important indications as they reduce visual quality.…”

Section: Discussionmentioning

confidence: 99%

“…Various techniques have already been discussed for the purpose of reducing complications and improving surgical results (4,(6)(7)(8)(9)(10)(11)(12) . The procedures most commonly used today are phacoemulsification and limbal or pars plana lensectomy, with or without IOL implantation (6)(7)(8)(9)(13)(14) .…”

Section: Discussionmentioning

confidence: 99%

“…The procedures most commonly used today are phacoemulsification and limbal or pars plana lensectomy, with or without IOL implantation (6)(7)(8)(9)(13)(14) . Other studies (7,14) reported good results and few complications for nontraumatic subluxations using limbal and pars plana lensectomy (7) without IOL implant.…”

Section: Discussionmentioning

confidence: 99%

“…Other studies (7,14) reported good results and few complications for nontraumatic subluxations using limbal and pars plana lensectomy (7) without IOL implant. To avoid aphakia, there are some options with similar results: after lensectomy and anterior vitrectomy, anterior chamber lens can be used (6) . However, other authors (9,(15)(16) used scleral fixation of the IOL (with better results when performed in the ciliary sulcus), and more currently using the posterior chamber IOLs associated with scleral fixation of the endocapsular tension ring (8,11) .…”

Section: Discussionmentioning

confidence: 99%

“…Other ocular features include: astigmatism, axial myopia, flat cornea, glaucoma (due to incomplete development of angle structures), and retinal detachment (due to myopia and aphakia) (4)(5) . Various surgical techniques have been described for the treatment of ectopia lentis, among these are the following: sectorial iridectomy, intra-and extracapsular extraction of the lens, phacoemulsification, and limbal or pars plana lensectomy (4,(6)(7)(8)(9)(10)(11)(12) . However, there is still no consensus about which technique is the safest and most effective in these cases, especially in some countries or regions where automated equipment is scarce and phacoemulsification is not available.…”

Section: Introductionmentioning

confidence: 99%

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Monoscleral fixation of IOL after extracapsular extraction of subluxated lenses in patients with Marfan syndrome

Ghanem¹,

Ghanem²,

Ghanem³

et al. 2004

Arq. Bras. Oftalmol.

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“…In some studies (6,13) the main indication for surgery was low corrected visual acuity. Glare and monocular diplopia were also important indications as they reduce visual quality.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Monoscleral fixation of IOL after extracapsular extraction of subluxated lenses in patients with Marfan syndrome

Ghanem¹,

Ghanem²,

Ghanem³

et al. 2004

Arq. Bras. Oftalmol.

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Ectopia lentis as the presenting and primary feature in Marfan syndrome

Zadeh

Bernstein

Niemi

et al. 2011

American J of Med Genetics Pt A

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Marfan syndrome (MFS) is a multisystem connective tissue disorder with primary involvement of the ocular, cardiovascular, and skeletal systems. We report on eight patients, all presenting initially with bilateral ectopia lentis (EL) during early childhood. These individuals did not have systemic manifestations of MFS, and did not fulfill the revised Ghent diagnostic criteria. However, all patients had demonstratable, disease-causing missense mutations in the FBN1 gene. Based on molecular results, cardiovascular imaging was recommended and led to the identification of mild aortic root changes in seven of the eight patients. The remaining patient had mitral valve prolapse with a normal appearing thoracic aorta. The findings presented in this paper validate the necessity of FBN1 gene testing in all individuals presenting with isolated EL. As we observed, these individuals are at increased risk of cardiovascular complications. Furthermore, we also noted that the majority of our patient cohort's mutations occurred in the 5 0 portion of the FBN1 gene, and were found to affect highly conserved cysteine residues, which may indicate a possible genotype-phenotype correlation. We conclude that in patients with isolated features of EL, FBN1 mutation analysis is necessary to aid in providing prompt diagnosis, and to identify patients at risk for potentially life-threatening complications. Additionally, knowledge of the type and location of an FBN1 mutation may be useful in providing further clinical correlation regarding phenotypic progression and appropriate medical management.

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Ophthalmological Aspects

Rosenberg¹

2004

Marfan Syndrome: A Primer for Clinicians and Scientists

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Management of Ectopia Lentis in a Family With Marfan Syndrome

Cited by 35 publications

References 13 publications

Monoscleral fixation of IOL after extracapsular extraction of subluxated lenses in patients with Marfan syndrome

Monoscleral fixation of IOL after extracapsular extraction of subluxated lenses in patients with Marfan syndrome

Ectopia lentis as the presenting and primary feature in Marfan syndrome

Ophthalmological Aspects

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