Visual outcome and surgical results in children with Marfan syndrome.
Marfan syndrome
ectopia lentis
lensectomy
visual and surgical result
Journal
Clinical & experimental ophthalmology
ISSN: 1442-9071
Titre abrégé: Clin Exp Ophthalmol
Pays: Australia
ID NLM: 100896531
Informations de publication
Date de publication:
Dec 2019
Dec 2019
Historique:
received:
07
08
2018
revised:
05
07
2019
accepted:
15
07
2019
pubmed:
22
7
2019
medline:
15
12
2020
entrez:
21
7
2019
Statut:
ppublish
Résumé
To determine visual and surgical results in children with Marfan syndrome. Marfan syndrome involves ocular complications which can lead to visual disturbance and amblyopia. Data about the visual and surgical results in children with Marfan syndrome is vital for the clinical management of these patients. Retrospective data analysis. Eighty-two eyes of 41 patients with a genetically proved diagnosis of Marfan syndrome. Medical records of patients with Marfan syndrome were reviewed between 2007 and 2017. Ocular complications, visual acuity (VA) of patients with/without lensectomy and surgical method were evaluated. VA outcomes of patients with Marfan syndrome with/without surgical repair of ectopia lentis. In 27 (66%) of the 41 patients a bilateral subluxation of the lens was visible and 14 (52%) patients received lensectomy. The mean age at initial presentation was 12.3 ± 9 years and mean follow-up was 3 years (range 1-7). VA varied from 1.2 to -0.1 logMAR at first examination. At initial presentation, mean VA was 0.1 ± 0.7 logMAR in patients with a normal lens status (n = 14) and 0.3 ± 0.5 logMAR in patients with subluxation of the lens (n = 27) (P < .01). VA improved from 0.2 ± 0.5 logMAR to 0.1 ± 0.5 logMAR (P = .06) in children with subluxation of the lens who did not need a lensectomy (n = 12) and from 0.5 ± 0.5 logMAR to 0.2 ± 0.5 logMAR (P = .02) in patients who were referred to lensectomy with/without secondary lens implantation. In Marfan syndrome subluxation of the lens was found in the majority of children. Improvement of VA resulted in children with subluxation of the lens and following lensectomy.
Sections du résumé
IMPORTANCE
OBJECTIVE
To determine visual and surgical results in children with Marfan syndrome.
BACKGROUND
BACKGROUND
Marfan syndrome involves ocular complications which can lead to visual disturbance and amblyopia. Data about the visual and surgical results in children with Marfan syndrome is vital for the clinical management of these patients.
DESIGN
METHODS
Retrospective data analysis.
PARTICIPANTS
METHODS
Eighty-two eyes of 41 patients with a genetically proved diagnosis of Marfan syndrome.
METHODS
METHODS
Medical records of patients with Marfan syndrome were reviewed between 2007 and 2017. Ocular complications, visual acuity (VA) of patients with/without lensectomy and surgical method were evaluated.
MAIN OUTCOME MEASURES
METHODS
VA outcomes of patients with Marfan syndrome with/without surgical repair of ectopia lentis.
RESULTS
RESULTS
In 27 (66%) of the 41 patients a bilateral subluxation of the lens was visible and 14 (52%) patients received lensectomy. The mean age at initial presentation was 12.3 ± 9 years and mean follow-up was 3 years (range 1-7). VA varied from 1.2 to -0.1 logMAR at first examination. At initial presentation, mean VA was 0.1 ± 0.7 logMAR in patients with a normal lens status (n = 14) and 0.3 ± 0.5 logMAR in patients with subluxation of the lens (n = 27) (P < .01). VA improved from 0.2 ± 0.5 logMAR to 0.1 ± 0.5 logMAR (P = .06) in children with subluxation of the lens who did not need a lensectomy (n = 12) and from 0.5 ± 0.5 logMAR to 0.2 ± 0.5 logMAR (P = .02) in patients who were referred to lensectomy with/without secondary lens implantation.
CONCLUSIONS AND RELEVANCE
CONCLUSIONS
In Marfan syndrome subluxation of the lens was found in the majority of children. Improvement of VA resulted in children with subluxation of the lens and following lensectomy.
Identifiants
pubmed: 31325202
doi: 10.1111/ceo.13596
pmc: PMC6973035
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1138-1145Informations de copyright
© 2019 The Authors. Medical University of Vienna. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.
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