Ocular findings in 87 adults with Ghent‐1 verified Marfan syndrome

Drolsum, Liv; Rand‐Hendriksen, Svend; Paus, Benedicte; Geiran, Odd; Semb, Svein Ove

doi:10.1111/aos.12448

Cited by 63 publications

(82 citation statements)

References 40 publications

(115 reference 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: 70%

“…Although it is no longer considered a criterion for MFS, it should be suspected in patients with longer AL and a flat cornea. 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).…”

Section: Discussionmentioning

confidence: 98%

“…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: 98%

See 2 more Smart Citations

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: 70%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 98%

See 1 more Smart Citation

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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“…Compared with patients with MFS, myopia in patients with LDS appears to be less frequent and less severe 4. Drolsum and colleagues12 reported a mean spherical equivalent of −4.08 dpt in patients with MFS, with myopia being higher in eyes with EL (spherical equivalent: −5.85±8.69 dpt). In that study, the mean refractive error of eyes without EL (spherical equivalent, −2.20±3.52 dpt) was comparable to that of LDS eyes in our study.…”

Section: Discussionmentioning

confidence: 97%

Ocular findings in Loeys-Dietz syndrome

et al. 2017

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“…While in the beginning, stent-grafting was advocated in patients with MFS, it soon became evident that the endoleak and secondary intervention rates were much higher than in patients without MFS 56,57. The reason is presumably the fixation of the vascular prostheses in the aortic vessel.…”

Section: Team Membersmentioning

confidence: 99%

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Kodolitsch¹,

Rybczynski²,

Vogler³

et al. 2016

JMDH

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Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS. First, we show how the initiative and collaboration of patient representatives, scientists, and physicians resulted in the foundation of Marfan centers, initially in the US and later in Germany, and how and why such centers evolved over time. Then, we elucidate the three main structural elements; a team of coordinators, core disciplines, and auxiliary disciplines of health care. Moreover, we explain how a multidisciplinary health care team integrates into many other health care structures of a university medical center, including external quality assurance; quality management system; clinical risk management; center for rare diseases; aorta center; health care teams for pregnancy, for neonates, and for rehabilitation; and in structures for patient centeredness. We provide accounts of medical goals and standards for each core discipline, including pediatricians, pediatric cardiologists, cardiologists, human geneticists, heart surgeons, vascular surgeons, vascular interventionists, orthopedic surgeons, ophthalmologists, and nurses; and of auxiliary disciplines including forensic pathologists, radiologists, rhythmologists, pulmonologists, sleep specialists, orthodontists, dentists, neurologists, obstetric surgeons, psychiatrist/psychologist, and rehabilitation specialists. We conclude that a multidisciplinary health care team is a means to maximize therapeutic success.

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Ocular findings in 87 adults with Ghent‐1 verified Marfan syndrome

Cited by 63 publications

References 40 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

Ocular findings in Loeys-Dietz syndrome

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

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