Anterior segment OCT imaging in mucopoly-saccharidoses type I, II, and VI

作者：Ahmed T Y^*; Turnbull A M J; Attridge N F; Biswas S; Lloyd I C; Au L; Ashworth J L

来源：Eye, 2014, 28(3): 327-336.

DOI：10.1038/eye.2013.281

• mucopolysaccharidosis
• hurler
• hunter
• Maroteaux-Lamy
• anterior segment OCT
• lysosomal storage disorders

Purpose To 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). %26lt;br%26gt;Methods Prospective, 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. %26lt;br%26gt;Results Ocular 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 0-2mm zone was greater in MPS VI than in MPS I, approaching but not attaining statistical significance (P = 0.07). The 2-5 and 5-7mm 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 7-10mm zone (P = 0.57). Measurements of the patient with MPS II resembled the mean values of the MPS I group. %26lt;br%26gt;Conclusion AS-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.

• 出版日期2014-3