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CASE REPORT
Deep anterior lamellar keratoplasty in case of Hurler-Scheie syndrome
  1. Alok Sati1,
  2. Muralidhar Ramappa1,
  3. Sunita Chaurasia1,
  4. Sitarama M Prasad2
  1. 1Department of Cornea and Anterior Segment Services, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India
  2. 2Department of Anesthesiology, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India
  1. Correspondence to Dr Muralidhar Ramappa, dr.muralidhar{at}icloud.com

Summary

A 12-year-old boy with Hurler-Scheie syndrome (H/S syndrome) reported with reduced vision in both the eyes for past few years. Deep anterior lamellar keratoplasty (DALK) was performed for visual rehabilitation in his left eye. During surgery, the predescemet's plane was reached by meticulously dissecting the lamellar fibres using a manual technique. Histopathology of the dissected cornea showed the presence of numerous alcian blue positive deposits corroborating with the diagnosis of mucopolysaccharidosis (MPS). Postoperative course was uneventful. One year following surgery, the graft was clear and had a visual acuity of 20/50 with +1.00 170° − 0.75 refractive correction. Endothelial cell count, as measured by non-contact specular microscopy, was 2473.4 cells/mm2. This case report highlights the application of DALK in a case of MPS-related corneal stromal opacification.

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