Outcomes of Iris-Claw IOL Implantation in Patients with Marfan’s Syndrome in Jordan

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Abstract

Objective

The management of ocular complications of Marfan’s syndrome, especially ectopia lentis, is challenging. In this study, we present the effectiveness and the safety of iris-claw intraocular lens (IOL) implantation along with lensectomy for those patients. Also, we compare the practice of implanting these IOLs either in the anterior chamber of retropupillary.

Methods

Retrospectively, we included all patients with Marfan’s syndrome who underwent lensectomy with iris-claw IOL implantation as a result of ectopia lentis. The patients were categorized into two groups: anterior chamber iris claw IOL and retropupillary iris-claw IOL. The clinical and demographic data, the visual outcome and postoperative complications were compared.

Results

Eighteen eyes of 10 patients were included in the study. The mean age of the patients was 19.1 years. Six patients were males. The iris-claw IOL was implanted anteriorly in 13 eyes. The visual outcome was comparable between both groups and most patients achieved improvement in the visual acuity. In addition, the postoperative complications developed similarly in both groups. However, all cases of IOL disenclavation (6 cases) developed in the anterior group. It is revealed that the age of the patient was the most significant factor affecting the occurrence of IOL disenclavation.

Conclusion

Iris-claw IOL (either anteriorly or retropupillary) is an effective and relatively safe method in treating ectopia lentis in patients with Marfan’s syndrome. In younger patients, anterior iris-claw IOL is safer than retropupillary iris-claw IOL as the risk of disenclavation is higher in younger patients.

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Most cited references 31

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The revised Ghent nosology for the Marfan syndrome.

B Loeys, H Dietz, A Braverman … (2010)

The diagnosis of Marfan syndrome (MFS) relies on defined clinical criteria (Ghent nosology), outlined by international expert opinion to facilitate accurate recognition of this genetic aneurysm syndrome and to improve patient management and counselling. These Ghent criteria, comprising a set of major and minor manifestations in different body systems, have proven to work well since with improving molecular techniques, confirmation of the diagnosis is possible in over 95% of patients. However, concerns with the current nosology are that some of the diagnostic criteria have not been sufficiently validated, are not applicable in children or necessitate expensive and specialised investigations. The recognition of variable clinical expression and the recently extended differential diagnosis further confound accurate diagnostic decision making. Moreover, the diagnosis of MFS--whether or not established correctly--can be stigmatising, hamper career aspirations, restrict life insurance opportunities, and cause psychosocial burden. An international expert panel has established a revised Ghent nosology, which puts more weight on the cardiovascular manifestations and in which aortic root aneurysm and ectopia lentis are the cardinal clinical features. In the absence of any family history, the presence of these two manifestations is sufficient for the unequivocal diagnosis of MFS. In absence of either of these two, the presence of a bonafide FBN1 mutation or a combination of systemic manifestations is required. For the latter a new scoring system has been designed. In this revised nosology, FBN1 testing, although not mandatory, has greater weight in the diagnostic assessment. Special considerations are given to the diagnosis of MFS in children and alternative diagnoses in adults. We anticipate that these new guidelines may delay a definitive diagnosis of MFS but will decrease the risk of premature or misdiagnosis and facilitate worldwide discussion of risk and follow-up/management guidelines.

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Visual acuities "hand motion" and "counting fingers" can be quantified with the freiburg visual acuity test.

Lutz Hansen, Nicolas Feltgen, Stefan K Schulze … (2006)

The visual acuity (VA) of patients with very low vision is classified using the semiquantitative scale "counting fingers" (CF), "hand motion" (HM), "light perception" (LP), and "no light perception." More quantitative measures would be desirable, especially for clinical studies. The results of clinical VA measurements, Early Treatment Diabetic Retinopathy Study (ETDRS) charts, and the Freiburg Visual Acuity Test (FrACT) were compared. The FrACT is a computerized visual acuity test that can present very large Landolt C optotypes when necessary. Examined were 100 eyes of 100 patients with various eye diseases (e.g., diabetic retinopathy, ARMD), covering a range of VAs from LP to decimal 0.32. The FrACT optotypes were presented on a 17-inch LCD monitor with random orientation. After extensive training, two ETDRS and FrACT measurements were obtained. The testing distance was 50 or 100 cm. ETDRS and FrACT coincided closely for VA > or = 0.02 (n = 80). ETDRS measures were successfully obtainable down to CF (at 30 cm; test-retest averaged over all patients, coefficient of variation [CV](ETDRS) = 9% +/- 8%), and FrACT provided reproducible measurements down to HM (test-retest CV(FrACT) =12% +/- 11%). For CF (n = 6), both ETDRS and FrACT resulted in a mean VA of 0.014 +/- 0.003 (range, 0.01-0.02). The VA results of FrACT for HM (n = 12) were 0.005 +/- 0.002 (range, 0.003-0.009); the individual values were highly reproducible. No results were obtainable for LP (n = 2). The three acuity procedures concur above a VA of 0.02. The results suggest that the category CF at 30 cm can be replaced by 0.014, using ETDRS or FrACT. Using FrACT, one can even reproducibly quantify VA in the HM-range, yielding a mean VA of 0.005.

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Revised diagnostic criteria for the Marfan syndrome.

A. De Paepe, R Devereux, H Dietz … (1996)

In 1986, the diagnosis of the Marfan syndrome was codified on the basis of clinical criteria in the Berlin nosology [Beighton et al., 1988]. Over time, weaknesses have emerged in these criteria, a problem accentuated by the advent of molecular testing. In this paper, we propose a revision of diagnostic criteria for Marfan syndrome and related conditions. Most notable are: more stringent requirements for diagnosis of the Marfan syndrome in relatives of an unequivocally affected individual; skeletal involvement as a major criterion if at least 4 of 8 typical skeletal manifestations are present; potential contribution of molecular analysis to the diagnosis of Marfan syndrome; and delineation of initial criteria for diagnosis of other heritable conditions with partially overlapping phenotypes.

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Author and article information

Journal

Journal ID (nlm-ta): Clin Ophthalmol

Journal ID (iso-abbrev): Clin Ophthalmol

Journal ID (publisher-id): opth

Title: Clinical Ophthalmology (Auckland, N.Z.)

Publisher: Dove

ISSN (Print): 1177-5467

ISSN (Electronic): 1177-5483

Publication date (Electronic): 17 November 2022

Publication date Collection: 2022

Volume: 16

Pages: 3811-3819

Affiliations

[1 ]Department of Special Surgery, Division of Ophthalmology, Faculty of Medicine, Jordan University of Science & Technology , Irbid, 22110, Jordan

[2 ]Department of Ophthalmology, King Abdullah University Hospital , Irbid, 22110, Jordan

Author notes

Correspondence: Rami A Al-Dwairi; Abdelwahab Aleshawi, Department of Special Surgery, Division of Ophthalmology, Faculty of Medicine Jordan University of Science and Technology , Irbid, 22110, Jordan, Tel +962795355056; +962799273019, Fax +962 2 7201064, Email ramialdwairi@yahoo.com; abdelwahhabjamal@yahoo.com

Author information

Rami A Al-Dwairi http://orcid.org/0000-0002-4187-4527

Asem A Alqudah http://orcid.org/0000-0002-8321-2572

Noor M Alqudah http://orcid.org/0000-0001-5341-9137

Firas Alrazem http://orcid.org/0000-0001-9996-4042

Zaki Shannak http://orcid.org/0000-0003-3774-4022

Article

Publisher ID: 385352

DOI: 10.2147/OPTH.S385352

PMC ID: 9677929

PubMed ID: 36419564

SO-VID: ccdc2df5-6e68-43e6-99bb-1fd6c68fe95c

License:

This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms ( https://www.dovepress.com/terms.php).

History

Date received : 07 August 2022

Date accepted : 27 October 2022

Page count

Figures: 0, Tables: 3, References: 32, Pages: 9

Funding

Funded by: have not declared any grant for this work;

The authors have not declared any grant for this work from any funding authority.

Outcomes of Iris-Claw IOL Implantation in Patients with Marfan’s Syndrome in Jordan

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Abstract

Objective

Methods

Results

Conclusion

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Karger: Ophthalmology

Most cited references 31

The revised Ghent nosology for the Marfan syndrome.

Visual acuities "hand motion" and "counting fingers" can be quantified with the freiburg visual acuity test.

Revised diagnostic criteria for the Marfan syndrome.

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