Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome

Xu, Wan Wan; Kurup, Sudhi P.; Fawzi, Amani A.; Durbin, Mary K; Maumenee, Irene H.; Mets, Marilyn B.

doi:10.1080/13816810.2016.1275017

Cited by 11 publications

(15 citation statements)

References 8 publications

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“…However, to exclude any possible bias caused by differences in eye conformation in myopic patients, we selected only patients with a refractive error of less than 6 diopters and analyzed the choroidal thickness and central retinal thickness of both groups, which was not statistically different. Recently, Xu et al 3 (2017) investigated the retinal nerve fiber layer thickness and central retinal thickness in a large cohort of patients with MfS using spectral domain OCT, demonstrating that 18% of patients with MfS presented a decrease in retinal nerve fiber layer thickness. These data could be related to abnormalities of the inner retinal layers, as a result of decreased blood flow.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, Xu et al 3 (2017) investigated the retinal nerve fiber layer thickness and central retinal thickness in a large cohort of patients with MfS using spectral domain OCT, demonstrating that 18% of patients with MfS presented a decrease in retinal nerve fiber layer thickness. These data could be related to abnormalities of the inner retinal layers, as a result of decreased blood flow.…”

Section: Discussionmentioning

confidence: 99%

“…Other ocular features included an axial myopia of more than 3 diopters, microspherophakia, cataract, glaucoma, iris transillumination defects, and a flattened cornea. 3 − 8 …”

Section: Introductionmentioning

confidence: 99%

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Retinal and Choroidal Vasculature in Patients with Marfan Syndrome

Marino

Cesareo

Aloe

et al. 2020

Trans. Vis. Sci. Tech.

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To assess the retinal and choroidal vasculature in patients with genetically confirmed Marfan syndrome (MfS). Methods: This prospective, case-control, observational study included 48 eyes of 24 patients with a genetic diagnosis of MfS and compared them with 52 eyes of 26 healthy controls. Best-corrected visual acuity, choroidal and retinal thickness measured by spectral domain-optical coherence tomography, retinal and choroidal vasculature characterized by optical coherence tomography angiography, were collected. A genetic counseling was carried out. A transthoracic echocardiogram was performed to evaluate the dimension of the aortic root, the ascending aorta and the left ventricle function and dimensions. Results: A significant decrease in the superficial and deep retinal capillary plexi vessel density (VD) was evident, such as a decrease in the choriocapillaris plexus VD. In patients with MfS, a negative correlation between left ventricular diameter and the VD of the superficial and deep plexi was observed. Patients with MfS with greater posterior wall and interventricular septum dimensions had lower VD in both plexi (P < 0.05). Moreover, there was a negative correlation between the dimension of the ascending aorta and foveal choriocapillary VD. In patients with MfS, increasing diameter of the ascending aorta was associated with a lower foveal choriocapillary VD (P < 0.05). Conclusions: The severity of MfS correlates with the impairment of the retinal and choroidal vasculature. Translational Relevance: Optical coherence tomography angiography may be a reproducible and noninvasive tool to study retinal blood flow in patients with MfS, with potential diagnostic and prognostic value.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Retinal and Choroidal Vasculature in Patients with Marfan Syndrome

Marino

Cesareo

Aloe

et al. 2020

Trans. Vis. Sci. Tech.

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“…About 30% of eyes had relatively long AL (Z-AL score ≥ 3), and the largest had a Z-score of 10.68. A long AL has been proven to be a risk factor for retinal detachment 26 and open-angle glaucoma 27 in the eyes of MFS patients.…”

Section: Discussionmentioning

confidence: 99%

Analysis of axial Length of Marfan Syndrome with Bilateral Ectopia Lentis by Z-scores for Chinese Patients under 20 years Old

Chen¹,

Chen²,

Zhang³

et al. 2020

Preprint

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Purpose: To analyze the axial length (AL) in young patients with Marfan syndrome (MFS) and bilateral ectopia lentis (EL) before the lens surgery. Methods: This study reviewed MFS patients under 20 with bilateral EL from January 2015 to October 2020. The Z-scores were introduced in terms of the number of standard deviations (SD) from the mean of age-matched normative data. Using Z-scores, the distribution of AL and influence factors were evaluated. The correlations between AL and other biometrics were analyzed. Results: We reviewed 183 patients, and enrolled both eyes. The mean age was 8.44 ± 4.69 years old. The mean AL increased from 23.41 ± 1.73 mm under 5 years old to 26.40 ± 2.28 mm in the 16-20 age group, and when plotted, the trend presented a logarithmic curvature. The mean Z-AL score was 1.74 ± 2.68. One-third of eyes had Z-score < 0. The right eye and the eye complicated with megalocornea had larger Z-AL scores. A positive correlation was found between Z-AL and Z-Corneal Curvature Radius (r = 0.265, P < 0.001).Conclusion: Young patents with bilateral EL but small AL should not be excluded from MFS without systematic examination. The age-adjusted Z-score will facilitate further study of the individual variations in AL across different ages.

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“…Some data on younger generations have been reported in hospital-based clinical studies with small sample size and thus were susceptible to selection bias [13–15]. In fact, the assessment of pRNFL thickness also has important implications in the diagnosis of several pediatric nerve disorders such as congenital glaucoma [16] and Marfan syndrome [17].…”

Section: Introductionmentioning

confidence: 99%

Distribution and Determinants of Peripapillary Retinal Nerve Fiber Layer Thickness and Its Association with Sleep Quality in Chinese Teenagers

Pan

Qian

Zhong

et al. 2019

Journal of Ophthalmology

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Purpose. We aimed to evaluate the distribution and determinants of peripapillary retinal nerve fiber layer (pRNFL) thickness and its associations with general sleep quality in Chinese school students. Methods. 1063 grade 7 students aged 13 to 14 years with pRNFL thickness data from a school-based study on grade 7 students in Southwestern China participated in the study. The pRNFL thickness was measured on the optical coherence tomography images of a circular scan centered on the optic disc. Refractive error was measured after cycloplegia using an autorefractor and biometric parameters including axial length (AL) were measured by an IOLMaster. Participants’ sleep quality was measured by the Children’s Sleep Habits Questionnaire (CSHQ). Results. The mean pRNFL thickness was 106.8 ± 10.7 μm among the 1063 participants. There was an increasing trend of spherical equivalent and a decreasing trend of AL with RNFL thickness. In multivariate analysis, each diopter of spherical equivalent increase was associated with 0.64 μm increase in pRNFL thickness. Girls had an increased mean pRNFL thickness compared with boys with a mean difference of 1.65 μm. Per 10 μm increase in pRNFL thickness was significantly associated with a 0.5 reduction in CSHQ score (better sleep quality). Conclusions. More myopic refractive error was the major ocular determinant of decreased pRNFL thickness. In addition, students with thinner pRNFL tended to have a worse sleep quality.

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Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome

Abstract: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18% of eyes with Marfan syndrome had RNFL thickness < 5% of the population.

Cited by 11 publications

References 8 publications

Retinal and Choroidal Vasculature in Patients with Marfan Syndrome

Retinal and Choroidal Vasculature in Patients with Marfan Syndrome

Analysis of axial Length of Marfan Syndrome with Bilateral Ectopia Lentis by Z-scores for Chinese Patients under 20 years Old

Distribution and Determinants of Peripapillary Retinal Nerve Fiber Layer Thickness and Its Association with Sleep Quality in Chinese Teenagers

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