761333

research-article2018
EJO0010.1177/1120672118761333European Journal of OphthalmologySalchow and Gehle

EJO European
Journal of
Ophthalmology
Original Research Article

European Journal of Ophthalmology

Ocular manifestations of Marfan

1­–6
© The Author(s) 2018
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https://doi.org/10.1177/1120672118761333
DOI: 10.1177/1120672118761333
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Daniel J Salchow1 and Petra Gehle2

Abstract
Purpose: To study ocular manifestations of Marfan syndrome in children and adolescents.
Methods: Retrospective comparative cohort study on consecutive patients up to age 17 years, presenting to the
interdisciplinary Marfan clinic of Charité-University Medicine Berlin over a period of 4 years.
Results: A total of 52 Marfan syndrome patients and 73 controls were enrolled. In Marfan syndrome eyes, the cornea
was flatter (mean keratometry, 40.86 ± 2.13 vs 42.55 ± 1.55 diopters in control eyes, p < .001) and corneal astigmatism
was greater (1.50 ± 1.22 vs 0.88 ± 0.49 diopters in control eyes, p < .001). The central cornea was thinner in Marfan
syndrome eyes (537.35 ± 40.64 vs 552.95 ± 38.57 μm, p = 0.007) and Marfan syndrome eyes were more myopic than
control eyes (spherical equivalent, –2.77 ± 4.77 vs −0.64 ± 1.92 diopters, p < .001). Visual acuity was reduced (logMAR
0.11 ± 0.17 vs 0.04 ± 0.26, p = 0.014) and intraocular pressure was lower in Marfan syndrome eyes. Iris transillumination
defects were more common in Marfan syndrome eyes (19.6% vs 4.3% in control eyes, odds ratio for Marfan syndrome
in the presence of iris transillumination defects = 7.2). Ectopia lentis was only found in Marfan syndrome eyes (25 Marfan
syndrome patients, 49% with available data, bilateral in 68%).
Conclusion: Iris transillumination defects and ectopia lentis are characteristic ocular findings in children and adolescents
with Marfan syndrome. Myopia is more common and corneal curvature, central corneal thickness, and visual acuity are
reduced in Marfan syndrome eyes. Children with Marfan syndrome need regular comprehensive eye examinations to
identify potential complications.

Keywords
Marfan syndrome, children, adolescents, ocular manifestations, ocular complications

Date received: 11 December 2017; accepted: 5 February 2018

Introduction
Marfan syndrome (MFS) is an autosomal dominant con-
nective tissue disorder, caused by mutations in the fibrillin
gene (FBN1).1,2
Ocular manifestations of MFS are common, including
transillumination defects, increased axial length (AL),
decreased corneal curvature, greater corneal astigmatism,
and myopia. Potentially severe ocular complications of
MFS are ectopia lentis (EL) and retinal detachments, and
average visual acuity (VA) is decreased in patients with
MFS.3 The diagnosis of MFS is clinical, but genetic testing
is available. Diagnostic criteria for MFS were updated in
2010 (Ghent 2 criteria) and include EL as a major
criterion.4,5
There is limited information on the ocular manifesta-
tions and complications of MFS on children. We compare
biometric and structural properties of the eyes of children
with MFS to a control group.
Methods
Study population
Patients were recruited from the interdisciplinary Marfan
clinic at Charité-University Medicine Berlin. Medical
records of patients presenting from December 2009 to
December 2013 were reviewed. Children and adolescents
up to 16 years of age (“children”) were included in the
1Department of Ophthalmology, Charité—University Medicine Berlin,
Berlin, Germany
2Department of Cardiology, Charité—University Medicine Berlin,
Berlin, Germany
Corresponding author:
Daniel J Salchow, Department of Ophthalmology, Charité—University
Medicine Berlin, Campus Virchow Klinikum, Augustenburger Platz 1,
Berlin 13353, Germany.
Email: daniel.salchow@charite.de
2 European Journal of Ophthalmology 00(0)
Table 1.  Biometry, keratometry, and refractive error of eyes of children with Marfan syndrome and control eyes.
Marfan
AL (mm) 23.69 ± 1.76 (87)
AD (mm)a 2.91 ± 0.26 (18)
ACD (mm)a 3.44 ± 0.30 (51)
LT (mm)a 3.75 ± 0.24 (23)
Kmed (D) 40.86 ± 2.13 (88)
AST (D) 1.50 ± 1.22 (87)
CCT (µm) 537.35 ± 40.64 (78)
SE (D)a –2.77 ± 4.77 (78)
AL: axial length; AD: aqueous depth; ACD: anterior chamber depth; LT: lens thickness; Kmed (D): mean corneal curvature in diopters; AST: corneal
astigmatism; CCT: central corneal thickness; SE: spherical equivalent in diopters.
Values are mean ± standard deviation, number of eyes with available data in parentheses.
aOnly phakic eyes included.
analysis. The study was approved by the ethics committee
of Charité-University Medicine Berlin.
Diagnosis of Marfan syndrome
MFS was diagnosed according to the Ghent 2 criteria.5
Children with MFS were included in the study group, chil-
dren in whom a connective tissue disorder was ruled out
served as controls.
Ophthalmological examination
Best-corrected VA, cycloplegic refraction (Humphrey
Automatic Refractor 599, Carl Zeiss Meditec Inc.,
Dublin, CA, USA) or retinoscopy, and manifest refrac-
tion if necessary were measured. Intraocular pressure
(IOP) was measured using noncontact tonometry (NCT,
Nidek NT-530, Nidek Co. Ltd., Aichi, Japan) and con-
firmed with Goldmann applanation tonometry if required.
The anterior segment was examined using slit lamp
biomicroscopy before and after pupil dilation (neosyn-
ephrine 2.5% and tropicamide 1% eyedrops). EL was
diagnosed if the edge of the lens was visible in mydriasis.
The fundus was examined using indirect ophthalmoscopy
in mydriasis.
Biometry
AL, central corneal thickness (CCT), aqueous depth (AD,
distance between posterior corneal surface and anterior
lens vertex), anterior chamber depth (ACD, distance
between the anterior corneal vertex and the anterior lens
vertex), and lens thickness (LT) were measured. Using
keratometry, average corneal curvature (Kmed) was meas-
ured. Biometry was performed with the IOL Master™
(software version V.3.01, Carl Zeiss AG, Oberkochen,
Germany) to measure AL, ACD, and keratometry, or using
the LENSTAR LS 900® (Haag-Streit Holding AG, Könitz,
Switzerland) to measure CCT, AL, AD, ACD, and LT. In
some patients, the ORBSCAN II® (Bausch & Lomb,
Control p value
23.61 ± 1.02 (130) 0.647
3.04 ± 0.27 (27) 0.12
3.56 ± 0.40 (95) 0.61
3.53 ± 0.23 (53) <0.001
42.55 ± 1.55 (141) <0.001
0.88 ± 0.49 (141) <0.001
552.95 ± 39.57 (125) 0.007
–0.64 ± 1.92 (120) <0.001
Rochester, NY, USA) or the GALILEI™ V5.2.1 (Ziemer
Ophthalmic Systems AG, Port, Switzerland) were used to
measure CCT and to perform keratometry.
Statistical analysis
Statistics were performed using SPSS Statistics 22 (IBM,
Armonk, NY, USA). Chi-square test or Fisher’s exact test
were used to evaluate categorical variables, the t-test for
continuous variables if normally distributed, and Mann–
Whitney U-test for those without normal distribution.
Results
Study population
A total of 52 (28 males) children with MFS and 73 (51
males) controls fulfilled the age criterion. MFS patients
were younger (11.1 ± 4.2 years, mean ± standard deviation,
range: 1–17 years) than controls (13.5 ± 3.1 years, range:
3–17 years, p > 0.001).
Biometry
AL was comparable in MFS and control eyes (Table 1).
MFS eyes with EL tended to be longer than those without
(24.04 ± 1.38 vs 23.48 ± 1.99 mm, p = 0.147). There was a
trend toward shallower ACD in MFS eyes, though the
difference was not statistically significant (Table 1). In
MFS eyes with EL (n = 13) and without (n = 37), ACD
was similar (AD, 3.08 ± 0.16 vs 2.87 ± 0.28 mm, p = 0.233;
ACD, 3.43 ± 0.29 vs 3.45 ± 0.32 mm, p = 0.906). The lens
was thicker in MFS eyes, and MFS eyes had a flatter cor-
nea and higher corneal astigmatism than control eyes
(Table 1). In 60.2% of MFS eyes, Kmed was <41.5 D,
compared with 27% of control eyes (p < 0.001).
Kmed < 41.5 D in at least one eye was present in 63.6%
of MFS patients and 29.6% of controls ((odds ratio (OR)
4.2, 95% confidence interval (CI), 1.9–9.3). In MFS and
control eyes, a steeper cornea was associated with shorter
Salchow and Gehle 3

Table 2.  Frequencies and odds ratio for different degrees of myopia in phakic eyes as a diagnostic criterion for Marfan syndrome.

MFS patients Control patients OR 95% CI

Myopia >3 D 15 (34.9%) 8 (12.9%) 3.6a 1.4–9.6
Myopia >0.75 D 18 (41.9%) 8 (12.9%) 4.9a 1.9–12.7

MFS: Marfan syndrome; OR: odds ratio; CI: confidence interval; D: diopters.
Odds ratios are adjusted for gender and age. If only one eye of a patient fulfilled the criterion, the subject was included in the respective category.
aStatistically significant differences.

Table 3.  Structural ocular findings in children with Marfan syndrome (MFS) and controls.

Ocular structure MFS eyes Control eyes Pathology (MFS patients/controls)

Normal/Total Normal/Total
External/eyelids  97/100 138/140 Blepharospasm 0/1 (bilat.)
Ptosis 1/0 (unilat.)
Milia 1/0 (bilat.)
Conjunctiva 100/100 136/140 Injection 0/1a (bilat.)
Melanosis 0/1(bilat.)
Cornea 102/102 140/140 n/a
Anterior Chamber 102/102 138/138 n/a
Iris  80/102 131/138 Transillumination defects 11/3 (bilat. 9/3)
Iridodonesis 1/0 (bilat.)
Iris synechia 0/1 (unilat.)
Pupil 100/100 140/140 n/a
Optic disc  94/102 132/138 Blurred margins 2/5 (bilat. 2/1)
Tilted disc 2/0 (bilat.)
Macula 100/102 136/138 Blunt macular reflex 0/2 (unilat.)
Pigmentary changes 1/0 (bilat.)
Retina/ocular fundus 100/102 136/138 Degenerative/myopic changes 2/0 (bilat.)
Tortuous retinal vessels 0/1 (bilat.)

Total: number of patients with documented findings for the respective category; bilat.: bilateral; unilat.: unilateral.
aNo documented cause or reason for this finding.

AL (Pearson corre­lation coefficient, –0.239, p = 0.05 and Visual acuity

−0.496, p < 0.001, respectively).
MFS eyes with EL had a flatter cornea than eyes with-
out (Kmed 39.88 ± 1.71 D vs 41.59 ± 2.17 D, p = 0.0001).
These eyes also had higher corneal astigmatism (1.91 ± 1.16
vs 1.18 ± 1.19 D, p = 0.005).
The central cornea was thinner in MFS eyes (Table 1).
It was thicker in MFS eyes with EL than in those without
(556 ± 33.34 vs 524.02 ± 40.84 µm, p < 0.001).
Refractive error
MFS eyes were more myopic than control eyes (Table 1).
MFS eyes with EL tended to be more myopic than those
without; though this was not statistically significant
(spherical equivalent, SE–3.69 ± 5.75 D vs −2.16 ± 3.96 D,
p = 0.172).
More MFS patients and eyes were myopic compared with
controls and control eyes. Subjects with myopia were more
likely to have MFS (Table 2). EL tended to be more common
in patients with myopia >3 D in at least one eye (53.3% vs
22.2% in patients not meeting this criterion, p = 0.085).
Best-corrected VA was reduced in MFS eyes (logMAR
0.11 ± 0.17 vs 0.04 ± 0.26 in control eyes, p = 0.014). MFS
eyes with EL had significantly lower VA than those with-
out (logMAR 0.19 ± 0.29 vs 0.06 ± 0.10, p < 0.001). Mean
VA was further reduced in aphakic (logMAR 0.22, n = 2)
and pseudophakic MFS eyes (0.21 ± 0.14, n = 5).
Intraocular pressure
IOP was lower in MFS eyes (14.41 ± 3.51 vs 15.97 ± 3.02 
mmHg in control eyes, p < 0.001). IOP was higher in MFS
eyes with EL (15.74 ± 3.51 vs 13.71 ± 3.28 mmHg in those
without EL, p = 0.006).
Structural findings
Structural findings are listed in Table 3. ITDs were more
common in MFS eyes (20/102 eyes, 19.6%) than in control
eyes (6/138 eyes, 4.3%; p < 0.001; OR for MFS in subjects
with ITD, 7.2, 95% CI 1.5–34.9). ITDs were also more
4 European Journal of Ophthalmology 00(0)
Table 4.  Direction of lens subluxation in eyes of children
with Marfan syndrome patients with ectopia lentis and available
information.
Direction of lens subluxation Number (%)
Superior 20 (47.6)
Superonasal 3 (7.1)
Nasal 2 (4.8)
Superotemporal 1 (2.4)
Inferior 1 (2.4)
Not specified 15 (35.7)
Total 42 (100)
common in MFS eyes without EL (9/59 eyes, 15.3%) than
in control eyes (p = 0.017). ITDs were bilateral in 9/11
MFS patients and in 2/4 controls. EL was present in 11/20
(55%) MFS eyes with ITD compared with 30/82 (36.6%)
MFS eyes without ITD (p = 0.279). Of the 11 MFS patients
with ITD, one had unilateral pseudophakia. None of the
patients with ITD had associated ocular disorders. Of four
controls with ITD, one had a history of retinal detachment
repair in that eye.
Lens.  Bilaterally normal structural (not counting EL, see
below) lens findings were documented in 45/51 MFS
patients and 69/70 controls. One MFS patient was bilat-
erally aphakic and three were pseudophakic (two bilater-
ally, one unilaterally). All aphakic and pseudophakic
patients had a history of EL. One MFS patient had an
“anterior peripheral cataract.” One control was unilaterally
pseudophakic.
Information on lens position was not available for 2 MFS
eyes and 10 control eyes. Only children with MFS had EL.
Of 25 MFS patients (49% of MFS patients with available
information, 12 males) with EL, it was bilateral in 17. Thus,
EL was noted in 42/102 MFS eyes (47.1%). Age and gender
were not associated with EL. The lens was decentered supe-
riorly in the majority of eyes (Table 4). One MFS patient
had bilateral phacodonesis (not counted as EL).
Ocular Fundus, Retina, and Vitreous.  100/102 MFS eyes and
136/138 control eyes had normal findings. Two control
eyes had a “blunt” macular reflex (one had a history of
retinal detachment) and the macula of one MFS eye had
“pigmentary changes.” Degenerative/myopic changes
were noted in both eyes of one MFS patient with a refrac-
tive error of error (SE) right eye −18.50 D, left eye −18.75
D. Tortuous retinal vessels were noted bilaterally in one
control. Information on the vitreous was available for four
MFS eyes (all normal) and six control eyes (four normal,
persisting hyaloid artery in both eyes of one control). No
MFS patient had a retinal detachment or a history thereof.
One control had a history of unilateral retinal detachment
of unknown cause and poor VA in that eye (less than 1.00
logMAR).
Glaucoma. There were no cases of glaucoma. One MFS
patient had suspected glaucoma (emmetropic, IOP 18 mmHg,
cup/disc ratio 0.4 bilaterally).
Discussion
To our knowledge, this is the first comprehensive report on
ocular manifestations of MFS in children and adolescence
that includes a control group. It is a retrospective study,
with its inherent limitations. Also, the control group was
composed of patients evaluated for a hereditary connective
tissue disorder, and does not represent the general popula-
tion. Different technology was used for biometry, reflect-
ing technological progress during the study period, but
possibly limiting the uniformity of the data. Diagnostic
criteria for MFS evolve, affecting study group composi-
tion and limiting comparability across studies.
While MFS eyes are longer than control eyes in
adults,3,6–9 this was not the case in children. Compared
with a recent report,10 MFS eyes in our study were shorter.
This may be due to different measuring techniques or due
to differences in study group composition. AL > 23.5 mm
used to be a diagnostic criterion for MFS,4 but our data
suggest that it would not be useful in children. Furthermore,
AL was comparable in MFS eyes with and without EL.
This is in contrast to adults, in whom EL is associated with
increased AL.3,6,9,11 Possibly, differences in AL become
more pronounced as axial growth of the eye continues
throughout adolescence.
In adults, Gehle et al.3 found a significantly shallower
anterior chamber in MFS eyes, while others did not,10,12
which agrees with our findings in children. Similar to
adults with MFS,3,6,12 EL did not affect ACD in children
with MFS, which is a novel finding.
The eyes of children with MFS had a thicker crystalline
lens than control eyes, paralleling findings in adults.3,6,12
There were too few eyes with EL and data on AD and LT
to study whether these parameters are correlated. In adult
MFS patients, LT does not seem to be a reliable marker for
EL,3,6 but there are no reports on the relationship of these
parameters in children.
Although not a diagnostic criterion for MFS, reduced
corneal curvature is characteristic for MFS in adults.3,13–15
In our children with MFS, Kmed was significantly reduced
compared with controls, confirming a smaller study on
children with MFS.10 Kmed < 41.5 D in one or both eyes
increased the likelihood for MFS fourfold. We recommend
to consider corneal curvature as a diagnostic criterion for
MFS in children.
Corneal astigmatism in children with MFS was compa-
rable with that reported by others.10 It was significantly
higher than in controls, which has been reported for
adults.3,14 Increased corneal astigmatism in MFS eyes was
associated with EL, supporting studies on adults3,10,14 and
children with MFS.10
Salchow and Gehle 5
Decreased CCT is commonly reported in adult MFS
patients3,11,13–15 and our study is the first to report this in chil-
dren. Although the cornea was not pathologically thin in
MFS eyes, reduced CCT has clinical relevance as a risk fac-
tor for primary open-angle glaucoma.16 Despite decreased
CCT, keratoconus does not appear to be more common in
MFS.8,15
Although not specific for MFS, myopia >3 D is part of the
diagnostic systemic score for MFS.5 Children with MFS were
more myopic than control eyes and had a higher prevalence
of myopia (34.9% had myopia >3 D in at least one eye).
Myopia was less severe in our children with MFS (–2.77 D)
than in a recent study (–7.85 D) by Kinori et al.10 The reason
for this is unclear. Possibly, the degree of EL (which can
induce myopia) was more pronounced in their cohort.
The prevalence of myopia varies between races,17,18 and
with age and myopia >3 D is not a valid criterion in all
populations. However, higher myopia in MFS patients
should raise the suspicion for EL (present in 53.3% of our
children with MFS and myopia >3 D in at least one eye).
Average VA was reduced in MFS eyes, which to our
knowledge has not been reported for children. It is, how-
ever, in agreement with recent studies on adults with
MFS.6,7,11 Potential causes include EL, myopia, aphakia,
amblyopia, and others. As in adults with MFS,3,6,11 we found
that EL was associated with reduced VA in children with
MFS, because EL can induce myopia, irregular astigma-
tism, and high hyperopia (if the lens is completely luxated).
Children with MFS had lower IOP than controls, which
is a novel finding for this age group, although it has been
reported in adults.3,6,11 To some degree, the IOP may have
been underestimated in MFS eyes because of reduced
CCT. EL was associated with a higher IOP in MFS eyes,
which has been found in adults.6,11 Therefore, the IOP
should be monitored more closely in children with EL.
ITD and iridodonesis were more common in children
with MFS than in controls. Compared with adults with
MFS, the frequency of ITD was higher than that reported
by Maumenee8 (approximately 10%), but lower than in the
study by Gehle et al.3 (27.4%). Subtle ITD may be missed
in uncooperative children. Although ITD may occur in
pseudoexfoliation syndrome, pigment dispersion syn-
drome, after ocular surgery and trauma, and in association
with intraocular inflammation, these diagnoses were
absent in subjects with ITD.
EL is a major diagnostic criterion for MFS and was
exclusive to children with MFS, highlighting its signifi-
cance. However, EL is not pathognomonic for MFS and
may also be caused by ocular trauma, infection, inflamma-
tion and tumors. Homocystinuria,19 dominant familial EL,
and other genetic causes should also be considered.20,21 EL
was considerably more common in our children with MFS
(49%) than in a recent report on children and adults
(36.4%).3 It is possible that EL prompts children and their
families to seek medical care early on (e.g. because of
decreased VA), causing a higher prevalence of EL in the
pediatric age group.
Peripheral retinal degenerations were related to high
myopia. Retinal detachment (RD) was not observed in
children with MFS, but adults with MFS are at increased
risk for RD.22–26 We recommend regular ocular fundus
examinations to identify risk factors for RD such as periph-
eral myopic degenerations and atrophic holes. Vitreous
findings were infrequently documented in both groups
limiting our ability to draw any conclusions.
While the frequency of glaucoma in children with MFS
is not known, Izquierdo et al.27 reported a prevalence of
23.7/1000 in MFS patients younger than 40 years. Although
we did not find glaucoma in children with MFS, they
should be monitored for it, because EL is a known risk fac-
tor for glaucoma.19
In summary, children with MFS are at increased risk for
EL, myopia, and reduced VA. ITD and decreased corneal
curvature should be considered and validated as diagnostic
criteria for MFS in children.
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
References
1. Dietz HC, Cutting GR, Pyeritz RE, et al. Marfan syndrome
caused by a recurrent de novo missense mutation in the
fibrillin gene. Nature 1991; 352(6333): 337–339.
2. Dietz HC, Pyeritz RE, Hall BD, et al. The Marfan syndrome
locus: confirmation of assignment to chromosome 15 and
identification of tightly linked markers at 15q15-q21.3.
Genomics 1991; 9(2): 355–361.
3. Gehle P, Goergen B, Pilger D, et al. Ocular manifestations
of Marfan syndrome. PLoS ONE 2017; 12(9): e0183370.
4. De Paepe A, Devereux RB, Dietz HC, et al. Revised diag-
nostic criteria for the Marfan syndrome. Am J Med Genet
1996; 62(4): 417–426.
5. Loeys BL, Dietz HC, Braverman AC, et al. The revised
Ghent nosology for the Marfan syndrome. J Med Genet
2010; 47(7): 476–485.
6. Drolsum L, Rand-Hendriksen S, Paus B, et al. Ocular find-
ings in 87 adults with Ghent-1 verified Marfan syndrome.
Acta Ophthalmol 2015; 93(1): 46–53.
7. Konradsen TR and Zetterstrom C. A descriptive study of
ocular characteristics in Marfan syndrome. Acta Ophthalmol
2013; 91(8): 751–755.
8. Maumenee IH. The eye in the Marfan syndrome. Trans Am
Ophthalmol Soc 1981; 79: 684–733.
9. Setälä K, Ruusuvaara P and Karjalainen K. Corneal
endothelium in Marfan syndrome: a clinical and specular
microscopic study. Acta Ophthalmol 1988; 66(3): 334–340.
6 European Journal of Ophthalmology 00(0)
10. Kinori M, Wehrli S, Kassem IS, et al. Biometry characteris-
tics in adults and children with Marfan syndrome: from the
Marfan eye consortium of Chicago. Am J Ophthalmol 2017;
177: 144–149.
11. Kara N, Bozkurt E, Baz O, et al. Corneal biomechanical
properties and intraocular pressure measurement in Marfan
patients. J Cataract Refract Surg 2012; 38(2): 309–314.
12. Konradsen TR, Koivula A, Kugelberg M, et al.
Accommodation measured with optical coherence tomog-
raphy in patients with Marfan’s syndrome. Ophthalmology
2009; 116(7): 1343–1348.
13. Heur M, Costin B, Crowe S, et al. The value of keratometry
and central corneal thickness measurements in the clinical
diagnosis of Marfan syndrome. Am J Ophthalmol 2008;
145(6): 997–1001.
14. Konradsen TR, Koivula A, Kugelberg M, et al. Corneal cur-
vature, pachymetry, and endothelial cell density in Marfan
syndrome. Acta Ophthalmol 2012; 90(4): 375–379.
15. Sultan G, Baudouin C, Auzerie O, et al. Cornea in Marfan
disease: Orbscan and in vivo confocal microscopy analysis.
Invest Ophthalmol Vis Sci 2002; 43(6): 1757–1764.
16. Gordon MO, Beiser JA, Brandt JD, et al. The ocular hyper-
tension treatment study: baseline factors that predict the
onset of primary open-angle glaucoma. Arch Ophthalmol
2002; 120(6): 714–720.
17. Foster PJ and Jiang Y. Epidemiology of myopia. Eye 2014;
28(2): 202–208.
18. Jobke S, Kasten E and Vorwerk C. The prevalence rates of
refractive errors among children, adolescents, and adults in
Germany. Clin Ophthalmol 2008; 2(3): 601–607.
19. Cross HE and Jensen AD. Ocular manifestations in the
Marfan syndrome and homocystinuria. Am J Ophthalmol
1973; 75(3): 405–420.
20. Hayward C and Brock DJ. Fibrillin-1 mutations in Marfan
syndrome and other type-1 fibrillinopathies. Hum Mutat
1997; 10(6): 415–423.
21. Kainulainen K, Karttunen L, Puhakka L, et al. Mutations in
the fibrillin gene responsible for dominant ectopia lentis and
neonatal Marfan syndrome. Nat Genet 1994; 6(1): 64–69.
22. Dotrelova D, Karel I and Clupkova E. Retinal detachment
in Marfan’s syndrome: characteristics and surgical results.
Retina 1997; 17(5): 390–396.
23. Lee SY and Ang CL. Results of retinal detachment surgery
in Marfan syndrome in Asians. Retina 2003; 23(1): 24–29.
24. Loewenstein A, Barequet IS, De Juan E, et al. Retinal detach-
ment in Marfan syndrome. Retina 2000; 20(4): 358–363.
25. Remulla JF and Tolentino FI. Retinal detachment in Marfan’s
syndrome. Int Ophthalmol Clin 2001; 41(4): 235–240.
26. Sharma T, Gopal L, Shanmugam MP, et al. Retinal detach-
ment in Marfan syndrome: clinical characteristics and surgi-
cal outcome. Retina 202; 22(4): 423–428.
27. Izquierdo NJ, Traboulsi EI, Enger C, et al. Glaucoma in
the Marfan syndrome. Trans Am Ophthalmol Soc 1992; 90:
111–117.