A descriptive study of ocular characteristics in Marfan syndrome

Konradsen, Tiina Rysä; Zetterström, Charlotta

doi:10.1111/aos.12068

Cited by 73 publications

(79 citation statements)

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“…Previous studies on patients with MFS showed an average AL of 24.0–25.0 mm. 3,5,7,11,12 This AL is relatively longer compared to population studies on healthy patients, which suggest a normal AL of approximately 23.5 mm. 13,14 Our study confirms these previous reports, with an average AL of 25.25 mm in adults and 24.24 mm in children.…”

Section: Discussionmentioning

confidence: 72%

“…7 According to previous reports, myopia >3 D is a nonspecific criterion for MFS, with a prevalence of about 30–40% in patients with MFS. 3,5,7 However, the refractive error of all MFS subjects <5 years of age (Table 3), when it was known, showed high to very severe myopia (range −3.75 to −26 D). This might strengthen the conclusion that MFS should be considered among other etiologies in young children with high myopia.…”

Section: Discussionmentioning

confidence: 99%

“…3,5–12 However, most studies were performed on a defined European population, mainly from Scandinavia, 5–8,11 and those studies used the Ghent 1 or older criteria for the diagnosis of MFS. To the best of our knowledge, other than the seminal paper by Maumenee in 1981, 3 the only descriptive biometric study on an American population—also using older criteria for the diagnosis of MFS—was a retrospective study that focused specifically on keratometry and central corneal thickness (CCT).…”

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confidence: 99%

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Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Kinori

Wehrli

Kassem

et al. 2017

American Journal of Ophthalmology

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PURPOSE To report on the biometric findings of adults and children with Marfan syndrome (MFS) recruited from 2 annual National Marfan Foundation conferences (2012 and 2015). DESIGN Cross-sectional study. METHODS Subjects diagnosed with MFS by Ghent 2 nosology were included for analysis. Subjects were divided into “adults” (≥16 years of age) and “children” (5–15 years of age). Biometric data included values for refractive error, axial length (AL), corneal curvature, anterior chamber depth, lens thickness, and central corneal thickness. RESULTS Of the 117 subjects evaluated, 74 (35 adults, 32 children, and 7 children <5 years of age) had a definite diagnosis of MFS and were included in the study. The AL was longer (25.25 ± 0.32 mm vs 24.24 ± 0.33 mm, P [ .03) and the lens was thicker (3.94 ± 0.09 mm vs 3.62 ± 0.10 mm, P [ .03) in adults. Both groups had flat corneas (average keratometry [Kmed] of 41.59 ± 0.35 diopters [D] in adults vs 40.89 ± 0.36 D in children, P [ .17). A negative correlation was found between AL and Kmed (L0.33, P < .001). The corneas of patients with MFS with ectopia lentis (EL) were significantly flatter and with higher degree of corneal astigmatism compared to patients without EL (Kmed of 40.68 ± 0.31 D vs 41.75 ± 0.28 D, P < .01 and corneal astigmatism of 1.68 ± 0.16 D vs 1.13 ± 0.14 D, P =.01). CONCLUSIONS Children with established MFS have flat corneas at least to the same degree as adults. Corneas of patients with MFS with EL are flatter and have a higher degree of corneal astigmatism. We strongly suggest that corneal parameters should be measured if MFS is suspected, especially in children that may not yet have developed EL.

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Kinori

Wehrli

Kassem

et al. 2017

American Journal of Ophthalmology

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“…In these diagnostic criteria, aortic root-aneurysm/dissection and EL are the crucial features of Marfan syndrome [10]. The previous studies showed that 37% to 87% Marfan syndrome patients had EL [13, 18–20], with other ocular characters in Marfan syndrome bilaterally and symmetrically, including flatted corneas and increased AL [14]. Previous study suggested that expression of abnormal fibrillin in Marfan syndrome may lead to enlargement of the eyeball, which may be the cause of a longer AL in Marfan syndrome [5].…”

Section: Discussionmentioning

confidence: 99%

Distribution of axial length in Chinese congenital ectopia lentis patients: a retrospective study

et al. 2017

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BackgroundCongenital ectopia lentis (CEL) usually leads to refractive error and may influence the axial length development. But few investigations have reported patient demographics and the distribution of axial length (AL) before surgery in Chinese pediatric patients with CEL. To describe the distribution of AL before surgery in CEL patients and its relationship with patients’ demographics, such as age, Marfan syndrome, sex, and laterality.MethodsThis retrospective study reviewed 306 CEL patients from January 1, 2006 to December 31, 2015. One eye was randomly selected from each patient if both eyes were EL. The influences of Marfan syndrome, sex, and laterality to AL in different age subgroups were evaluated and compared. The differences of the AL between groups were assessed using the student t test or paired t-test. P-values less than 0.05 were considered statistically significant.ResultsTwo hundred forty-seven eyes were enrolled. 58.3% of all the patients had binoculus EL, 70% of all the patients were male and 36% of all the patients were diagnosed with Marfan syndrome. The mean AL of EL patients was 25.1 ± 2.5 mm. There was no statistical difference in the AL between patients with and without Marfan syndrome, and in the AL between male and female patients. There was statistical difference in AL between the EL-affected eye and the unaffected eye in monocular EL patients younger than 12 years old.ConclusionsThis study suggests that AL can be influenced by CEL, but the influence of CEL may be reduced after the age of 12 years old, which will likely provide a useful reference when considering the most appropriate time of surgery.

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“…While cardiovascular complications, such as aortic aneurysm and mitral valve prolapse, are life threatening, abnormalities in the skeletal system, such as long and thin extremities with spidery fingers and toes, account for the typical clinical picture of MFS [2, 7]. Concerning the ocular system, typical clinical findings include an increased axial length of the eye bulb, a flattened corneal curvature, iris transillumination, early cataract, glaucoma, amblyopia, retinal detachment, and ectopia lentis or lens dislocation [8–10]. The latter occurs in about 60% of patients suffering from MFS [11, 12].…”

Section: Introductionmentioning

confidence: 99%

Long-term follow-up after scleral lens fixation in patients with Marfan syndrome

et al. 2017

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BackgroundThe dislocation of the crystalline lens is a common finding in patients with Marfan syndrome (MFS). Scleral intraocular lens (IOL) fixation is an accepted treatment method of this complication. To now, no long-term data on scleral IOL fixation in MFS exist.MethodsWe present a retrospective study of 27 eyes of 17 MFS patients that underwent scleral lens fixation at our clinic between 1999 and 2012. These patients are compared to an age- and surgeon-matched group of 31 eyes of 27 patients who underwent the same procedure for reasons other than MFS.ResultsThe median age in the MFS group was 35.4 years versus 35.6 years in the non-MFS group. The median follow-up was 4 years for MFS and 3 years for non-MFS. In the MFS group, significantly more IOL-dislocations occurred than compared to the non-MFS group (30% vs. 6.5%, p = 0.02). Retinal detachment occurred in four MFS-eyes compared to three eyes in the non-MFS group. Biometry prediction error was 1.11 diopters (D) for MFS and 1.33 D for non-MFS (p = 0.11). Median BCVA (best-corrected visual acuity, logMAR) was 0.1 in the MFS group versus 0.3 in non-MFS patients.ConclusionScleral lens fixation in MFS patients achieves satisfying visual and refractive outcomes. Our data shows a significantly higher rate of IOL dislocations in patients with MFS. We therefore recommend addressing this complication preoperatively.Electronic supplementary materialThe online version of this article (10.1186/s12886-017-0625-x) contains supplementary material, which is available to authorized users.

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A descriptive study of ocular characteristics in Marfan syndrome

Abstract: Despite increased AL, high myopia is not as common in MFS as expected because of a flat cornea. Biometry should be considered in suspected cases of MFS when EL is not present.

Cited by 73 publications

References 18 publications

Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Distribution of axial length in Chinese congenital ectopia lentis patients: a retrospective study

Long-term follow-up after scleral lens fixation in patients with Marfan syndrome

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