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Anterior Chamber Angles in Different Types of Mucopolysaccharidoses

Jia-Rong Zhang1, Jen-Hung Wang2, Hong-Zin Lin3

  • 1Department of Ophthalmology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan.

American Journal of Ophthalmology
|January 17, 2020
PubMed
Abstract

Insights

Mucopolysaccharidoses (MPS) patients often have narrow anterior chamber angles and elevated intraocular pressure (IOP), increasing glaucoma risk. Swept-source OCT aids in understanding these angle structures and MPS-related glaucoma.

Area of Science:

  • Ophthalmology
  • Genetics
  • Medical Imaging

Background:

  • Mucopolysaccharidoses (MPS) are rare genetic disorders affecting multiple organs, including the eyes.
  • Ocular complications in MPS can lead to vision impairment, with anterior segment abnormalities being a significant concern.

Purpose of the Study:

  • To evaluate anterior chamber angle status and estimate intraocular pressure (IOP) in patients with mucopolysaccharidoses (MPS) types I, II, IV, and VI.
  • To investigate the relationship between MPS types and specific anterior segment and IOP characteristics.

Main Methods:

  • Prospective cross-sectional study involving 27 MPS patients (types I, II, IV, VI) and 20 controls.
  • Anterior chamber angle assessment using swept-source optical coherence tomography (SS-OCT).
  • Intraocular pressure (IOP) estimation using Corvis ST tonometer.

Main Results:

  • MPS I patients showed narrow angles/peripheral iridocorneal touches with elevated IOP (80%).
  • MPS II patients exhibited plateau iris but normal IOP.
  • MPS IV patients had normal angles but elevated IOP (44.4%), while MPS VI patients had shallow angles (81.8%) with some elevated IOP.
  • MPS types I, II, and VI demonstrated smaller trabecular iris angles compared to MPS IV and controls.

Conclusions:

  • MPS type I patients are prone to glaucoma with narrow/closed angles; MPS II to plateau iris; MPS IV to open-angle glaucoma; MPS VI to narrow angles.
  • MPS types I, IV, and VI showed higher IOP estimates than controls.
  • SS-OCT is valuable for investigating angle structure and glaucoma pathophysiology in MPS.

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