title: Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI.
abstract: OBJECTIVE
  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).
  
  
  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.
  
  
  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 0-2 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Manchester Academic Health Science Centre, Manchester Royal Eye Hospital, Central Manchester Foundation Trust, The University of Manchester, Manchester, UK.
authors.forename: T Y
authors.initials: TY
authors.lastname: Ahmed
authors.affiliation: Bournemouth Eye Unit, Royal Bournemouth Hospital, Dorset, UK.
authors.forename: A M J
authors.initials: AM
authors.lastname: Turnbull
authors.affiliation: Centre for Pain Research, University of Bath, Somerset, UK.
authors.forename: N F
authors.initials: NF
authors.lastname: Attridge
authors.affiliation: Manchester Academic Health Science Centre, Manchester Royal Eye Hospital, Central Manchester Foundation Trust, The University of Manchester, Manchester, UK.
authors.forename: S
authors.initials: S
authors.lastname: Biswas
authors.affiliation: Manchester Academic Health Science Centre, Manchester Royal Eye Hospital, Central Manchester Foundation Trust, The University of Manchester, Manchester, UK.
authors.forename: I C
authors.initials: IC
authors.lastname: Lloyd
authors.affiliation: Manchester Academic Health Science Centre, Manchester Royal Eye Hospital, Central Manchester Foundation Trust, The University of Manchester, Manchester, UK.
authors.forename: L
authors.initials: L
authors.lastname: Au
authors.affiliation: Manchester Academic Health Science Centre, Manchester Royal Eye Hospital, Central Manchester Foundation Trust, The University of Manchester, Manchester, UK.
authors.forename: J L
authors.initials: JL
authors.lastname: Ashworth
mesh_terms: D000293:Adolescent; D000869:Anterior Eye Segment / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D063171:Corneal Pachymetry; D005260:Female; D006801:Humans; D008297:Male; D008059:Mucopolysaccharidosis I / Q000175:diagnosis*; D016532:Mucopolysaccharidosis II / Q000175:diagnosis*; D009087:Mucopolysaccharidosis VI / Q000175:diagnosis*; D011446:Prospective Studies; D041623:Tomography, Optical Coherence