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Management of Corneal Clouding in Patients with Mucopolysaccharidosis

Orlaith McGrath1, Leon Au1, Jane Ashworth1

  • 1Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Oxford Road, Manchester M13 9WL, UK.

Journal of Clinical Medicine
|August 7, 2021
PubMed

Insights

Mucopolysaccharidoses (MPS) cause visual loss due to corneal clouding, which current therapies like ERT and HSCT minimally improve. Corneal transplantation remains the definitive treatment for this rare lysosomal storage disorder.

Area of Science:

  • Ophthalmology
  • Genetics
  • Biochemistry

Background:

  • Mucopolysaccharidoses (MPS) are rare lysosomal storage disorders.
  • Characterized by glycosaminoglycan (GAG) accumulation in various organs, including the eye.
  • Visual impairment in MPS is primarily due to corneal clouding and retinopathy.

Purpose of the Study:

  • To provide an overview of corneal clouding in MPS.
  • To discuss current clinical and surgical management strategies.
  • To highlight significant research advancements in the field.

Main Methods:

  • Literature review of MPS and ocular manifestations.
  • Analysis of current treatment modalities for corneal clouding.
  • Summary of ongoing research and potential future therapies.

Main Results:

  • Enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) show limited efficacy for corneal clouding.
  • Corneal transplantation (penetrating or deep anterior lamellar keratoplasty) is the primary treatment.
  • Research is exploring novel therapeutic approaches for GAG-related ocular disease.

Conclusions:

  • Corneal clouding significantly impacts vision in MPS patients.
  • Current systemic therapies do not effectively resolve corneal opacity.
  • Corneal transplantation is essential, with ongoing research aiming for better MPS ocular treatments.
Keywords:
Mucopolysaccharidosiscorneal cloudingdeep anterior lamellar keratoplastypenetrating keratoplasty

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