Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective

Esfandiari, Hamed; Ansari, Shabnam; Rabie, Hossein Mohammad

doi:10.20944/preprints201805.0139.v1

Cited by 7 publications

(21 citation statements)

References 32 publications

(47 reference statements)

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“…The incidence of retinal detachment in patients with Marfan syndrome has been reported to reach up to 25.6 % [1]. History of lensectomy and aphakia have been identified as risk factors for retinal detachment in Marfan syndrome [1].…”

Section: Introductionmentioning

confidence: 99%

Simultaneous Management of Retinal Detachment and Aphakia with Pars Plana Vitrectomy, Silicone Oil Tamponade and Retropupillary Iris-Claw Intraocular Lens Implantation in Patients with Marfan Syndrome

Hsin

Stappler

Potić

et al. 2022

Klin Monbl Augenheilkd

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Background Marfan Syndrome is an autosomal dominant disease with multiple ocular abnormalities including ectopia lentis and a high incidence of rhegmatogenous retinal detachment (RRD). The management of RRD may be challenging in cases of aphakic patients with Marfan. Purpose To report on the management of four cases of simultaneous RRD and aphakia with vitrectomy, silicone oil tamponade, and retropupillary iris-claw intraocular lens (IOL) implantation in patients with Marfan that have been operated at the Jules-Gonin Eye Hospital between 2019 and 2020. History and Signs Ages at presentation were 20, 30, 32, and 31 years, respectively. All patients had a history of extraction of a dislocated lens. None of the patients had a previous posterior vitrectomy. Two patients had records of previous measurements for IOL calculation by optical biometry (IOL Master, Carl Zeiss Meditec AG, Jena, Germany) about 1 year prior to the RRD development. In two cases, measurements for IOL calculation by optical biometry were based on the contralateral eye. Therapy and Outcome All patients underwent 23 G vitrectomy, peripheral iridotomy, and retropupillary iris-claw IOL. No intraoperative complications were encountered. All patients had silicone oil tamponade, one of which required heavy silicone oil. Silicone oil was removed 3 months following primary surgery. Minimum follow-up was 1 year. The single surgery anatomic success rate was 100%. All patients had visual acuity of at least 0.8 at the last follow-up (1.25, 1.0, 0.8, and 0.8 respectively). The targeted refractive results were accurately achieved in all four cases postoperatively. One patient presented ocular hypertension 2 weeks after surgery due to presumed steroid response and was managed conservatively. None of the patients had silicone oil migration into the anterior chamber. Conclusion Retropupillary iris-claw IOL implantation in cases of RRD and aphakia creates a barrier to tamponades from the posterior segment, effectively preventing them from entering the anterior segment of the eye. Therefore, the management of aphakia and retinal detachment with simultaneous vitrectomy and a retropupillary iris-claw IOL may be a successful strategy in reducing postoperative complications in patients with Marfan syndrome.

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Section: Introductionmentioning

confidence: 99%

Simultaneous Management of Retinal Detachment and Aphakia with Pars Plana Vitrectomy, Silicone Oil Tamponade and Retropupillary Iris-Claw Intraocular Lens Implantation in Patients with Marfan Syndrome

Hsin

Stappler

Potić

et al. 2022

Klin Monbl Augenheilkd

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“…Risk factors for RD in MFS include younger age, EL and aphakia. 58 We have previously reported that RD in MFS tends to occur bilaterally (30–42% of cases), and women with MFS seem to develop RD earlier than their male counterparts. 7 This is in contrast to RD affecting those without MFS.…”

Section: Ocular Manifestationsmentioning

confidence: 85%

Marfan syndrome and the eye clinic: from diagnosis to management

Akram

Aragon-Martin

Chandra

2021

Therapeutic Advances in Rare Disease

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Marfan syndrome (MFS) is an autosomal dominantly inherited disorder affecting the cardiovascular, ocular and musculoskeletal systems. Frequently, clinical suspicion and subsequent diagnosis begins in the ophthalmology clinic. Importantly, the ophthalmologist has a responsibility to cater not only to the eye, but also to be involved in a holistic approach for these patients. In this review, we discuss how MFS may present to an eye clinic, including clinical features, ocular morbidity, genetic diagnosis and management. Although this condition is ideally managed by a multidisciplinary team, our focus will be on MFS and the eye, including other conditions which may present with similar phenotypes. The ophthalmologist’s role as the potential first contact for a patient with suspected MFS is crucial in making the proper investigations and referral, with the knowledge that not all ectopia lentis cases are MFS and vice versa. Management of ocular conditions in MFS may range from simple observation to surgical intervention; current options will be discussed. Plain Language Summary Eye problems in Marfan Syndrome – A Review Marfan syndrome (MFS) is an inherited disorder that affects many systems of the body, including the heart, joints, skeleton, skin and eyes. Although the more dangerous problems caused by this are to do with the heart and blood vessels, it is quite often that such patients are first found by eye doctors. They are either seen due to being very short-sighted or with dislocated lenses which can cause major problems in the eye. Eye problems can be managed by regular observation, although they often require surgery. Because eye doctors are often the first to see these patients, they must involve other doctors of different specialities to help in diagnosing and managing important issues these patients may have, especially affecting the heart and major blood vessels. Confirmation of diagnosis is done through genetic testing, which has advanced greatly, finding new mutations which may contribute to this disorder. Genetic counselling services can help families in understanding their diagnosis and making better informed decisions about future family planning as well as screening other family members. The eye is just one part of this complex genetic disease. We look in detail at how eye doctors can best approach such patients.

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“…Anterior dislocation of the lens could cause pupillary block, secondary angle closure, and IOP rise, but not all lens subluxation can result in secondary glaucoma. For the patients with Marfan syndrome, even subluxated lens movement anteriorly, the pupillary block, and angle closure rarely happened due to lens subluxation [ 11 ]. The shallow anterior chamber, thick lens, anterior lens position, and short AL are important anatomical features for the primary angle closure glaucoma [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Comparisons of Ocular Anatomic Differences of Lens-Subluxated Eye with or without Acute Angle Closure: A Retrospective Study

Lin

Qin

Zhang

et al. 2020

Journal of Ophthalmology

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Purpose. To compare ocular anatomy differences of lens subluxation between eyes with or without acute angle closure (AAC). Methods. This is a retrospective and case-control study. Sixty cases with mild lens subluxation were recruited. Among them, 30 eyes with acute angle closure were assigned to the AAC group and 30 eyes without AAC were assigned to the non-AAC group. The anterior segment was quantitatively evaluated by ultrasound biomicroscopy (UBM). The axial length (AL) was measured with IOL Master. All patients underwent lens extraction surgery and were followed up for six months. Results. The history of blunt trauma accounted for 22 (73.3%) cases in the AAC group and 21 (70%) cases in the non-AAC group. Fifteen (50%) patients in the AAC group had iridotomy history, and high intraocular pressure recurred. The UBM analysis showed that the average central chamber depth of the affected eyes in the AAC group was 1.82 mm, which was significantly shallower than that in the fellow eyes (2.58 mm, P<0.05) or both eyes in the non-AAC group.Both eyes in the AAC group presented a shorter AL and shallower anterior chamber than the eyes in the non-AAC group. Conclusions. An asymmetrical anterior chamber between bilateral eyes is an important feature in lens subluxation-induced AAC. The crowded anterior chamber and shorter AL might be the anatomic basis for the eye with lens subluxation-induced AAC.

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Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective

Cited by 7 publications

References 32 publications

Simultaneous Management of Retinal Detachment and Aphakia with Pars Plana Vitrectomy, Silicone Oil Tamponade and Retropupillary Iris-Claw Intraocular Lens Implantation in Patients with Marfan Syndrome

Simultaneous Management of Retinal Detachment and Aphakia with Pars Plana Vitrectomy, Silicone Oil Tamponade and Retropupillary Iris-Claw Intraocular Lens Implantation in Patients with Marfan Syndrome

Marfan syndrome and the eye clinic: from diagnosis to management

Comparisons of Ocular Anatomic Differences of Lens-Subluxated Eye with or without Acute Angle Closure: A Retrospective Study

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