Deep anterior lamellar keratoplasty in case of Hurler-Scheie syndrome.
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/mm(2). This case report highlights the application of DALK in a case of MPS-related corneal stromal opacification.
Department of Cornea and Anterior Segment Services, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
Alok
A
Sati
Muralidhar
M
Ramappa
Sunita
S
Chaurasia
Sitarama M
SM
Prasad
D002648:Child; D003316:Corneal Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D003319:Corneal Stroma / Q000473:pathology / Q000601:surgery*; D004728:Endothelium, Corneal / Q000473:pathology / Q000601:surgery*; D006801:Humans; D015948:Keratoplasty, Penetrating; D008297:Male; D008059:Mucopolysaccharidosis I / Q000150:complications / Q000473:pathology / Q000601:surgery*; D014792:Visual Acuity