Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI

T.Y. Ahmed, A.M.J. Turnbull, N.F. Attridge, S. Biswas, I.C. Lloyd, L. Au, J.L. Ashworth

Research output: Contribution to journalArticle

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Abstract

PurposeTo describe the anterior segment optical coherence tomography (AS-OCT) characteristics of patients with ocular manifestations of mucopolysaccharidoses type I (Hurler), II (Hunter), and VI (Maroteaux-Lamy). MethodsProspective, observational study of nine consecutive patients with variants of mucopolysaccharidosis (MPS) attending the Paediatric Ophthalmology service at Manchester Royal Eye Hospital, UK. All patients underwent Visante AS-OCT imaging as part of their ophthalmic assessment.ResultsOcular involvement tended to be symmetrical. Angle-to-angle distance was significantly lower in MPS VI than in MPS I (P=0.04). Anterior chamber depth, angle opening distance, trabecular-iris space area, and scleral spur angle tended to be lower in MPS VI than in MPS I, but did not reach statistical significance. Corneal thickness in the central 0-2 mm zone was greater in MPS VI than in MPS I, approaching but not attaining statistical significance (P=0.07). The 2-5 and 5-7 mm zones were significantly thicker in MPS VI than MPS I (P=0.04, P=0.04). There was no difference in corneal thickness between MPS I and MPS VI in the peripheral 7-10 mm zone (P=0.57). Measurements of the patient with MPS II resembled the mean values of the MPS I group.ConclusionAS-OCT is valuable in quantifying anterior segment pathology in MPS. It suggests more crowded anterior segments and greater corneal thickness in patients with MPS VI than MPS I. AS-OCT is useful in evaluating the risk and mechanism of glaucoma in MPS patients, and may improve our assessment of the efficacy of systemic treatment.
LanguageEnglish
Pages327-336
Number of pages10
JournalEye
Volume28
Issue number3
Early online date3 Jan 2014
DOIs
StatusPublished - Mar 2014

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Mucopolysaccharidosis VI
Mucopolysaccharidosis II
Mucopolysaccharidosis I
Mucopolysaccharidoses
Optical Coherence Tomography
Eye Manifestations
Anterior Chamber
Iris
Ophthalmology
Glaucoma
Observational Studies
Pediatrics
Pathology

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Ahmed, T. Y., Turnbull, A. M. J., Attridge, N. F., Biswas, S., Lloyd, I. C., Au, L., & Ashworth, J. L. (2014). Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI. DOI: 10.1038/eye.2013.281

Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI. / Ahmed, T.Y.; Turnbull, A.M.J.; Attridge, N.F.; Biswas, S.; Lloyd, I.C.; Au, L.; Ashworth, J.L.

In: Eye, Vol. 28, No. 3, 03.2014, p. 327-336.

Research output: Contribution to journalArticle

Ahmed, TY, Turnbull, AMJ, Attridge, NF, Biswas, S, Lloyd, IC, Au, L & Ashworth, JL 2014, 'Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI' Eye, vol. 28, no. 3, pp. 327-336. DOI: 10.1038/eye.2013.281
Ahmed TY, Turnbull AMJ, Attridge NF, Biswas S, Lloyd IC, Au L et al. Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI. Eye. 2014 Mar;28(3):327-336. Available from, DOI: 10.1038/eye.2013.281
Ahmed, T.Y. ; Turnbull, A.M.J. ; Attridge, N.F. ; Biswas, S. ; Lloyd, I.C. ; Au, L. ; Ashworth, J.L./ Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI. In: Eye. 2014 ; Vol. 28, No. 3. pp. 327-336
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abstract = "PurposeTo describe the anterior segment optical coherence tomography (AS-OCT) characteristics of patients with ocular manifestations of mucopolysaccharidoses type I (Hurler), II (Hunter), and VI (Maroteaux-Lamy). MethodsProspective, observational study of nine consecutive patients with variants of mucopolysaccharidosis (MPS) attending the Paediatric Ophthalmology service at Manchester Royal Eye Hospital, UK. All patients underwent Visante AS-OCT imaging as part of their ophthalmic assessment.ResultsOcular involvement tended to be symmetrical. Angle-to-angle distance was significantly lower in MPS VI than in MPS I (P=0.04). Anterior chamber depth, angle opening distance, trabecular-iris space area, and scleral spur angle tended to be lower in MPS VI than in MPS I, but did not reach statistical significance. Corneal thickness in the central 0-2 mm zone was greater in MPS VI than in MPS I, approaching but not attaining statistical significance (P=0.07). The 2-5 and 5-7 mm zones were significantly thicker in MPS VI than MPS I (P=0.04, P=0.04). There was no difference in corneal thickness between MPS I and MPS VI in the peripheral 7-10 mm zone (P=0.57). Measurements of the patient with MPS II resembled the mean values of the MPS I group.ConclusionAS-OCT is valuable in quantifying anterior segment pathology in MPS. It suggests more crowded anterior segments and greater corneal thickness in patients with MPS VI than MPS I. AS-OCT is useful in evaluating the risk and mechanism of glaucoma in MPS patients, and may improve our assessment of the efficacy of systemic treatment.",
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N2 - PurposeTo describe the anterior segment optical coherence tomography (AS-OCT) characteristics of patients with ocular manifestations of mucopolysaccharidoses type I (Hurler), II (Hunter), and VI (Maroteaux-Lamy). MethodsProspective, observational study of nine consecutive patients with variants of mucopolysaccharidosis (MPS) attending the Paediatric Ophthalmology service at Manchester Royal Eye Hospital, UK. All patients underwent Visante AS-OCT imaging as part of their ophthalmic assessment.ResultsOcular involvement tended to be symmetrical. Angle-to-angle distance was significantly lower in MPS VI than in MPS I (P=0.04). Anterior chamber depth, angle opening distance, trabecular-iris space area, and scleral spur angle tended to be lower in MPS VI than in MPS I, but did not reach statistical significance. Corneal thickness in the central 0-2 mm zone was greater in MPS VI than in MPS I, approaching but not attaining statistical significance (P=0.07). The 2-5 and 5-7 mm zones were significantly thicker in MPS VI than MPS I (P=0.04, P=0.04). There was no difference in corneal thickness between MPS I and MPS VI in the peripheral 7-10 mm zone (P=0.57). Measurements of the patient with MPS II resembled the mean values of the MPS I group.ConclusionAS-OCT is valuable in quantifying anterior segment pathology in MPS. It suggests more crowded anterior segments and greater corneal thickness in patients with MPS VI than MPS I. AS-OCT is useful in evaluating the risk and mechanism of glaucoma in MPS patients, and may improve our assessment of the efficacy of systemic treatment.

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