Deep anterior lamellar keratoplasty in case of Hurler-Scheie syndrome

Abstract

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.

Figure 1

(A–F) Typical patient of mucopolysaccharidosis showing marked growth retardation, joint stiffness, enlarged head, coarse facial features, short neck, low set ears, short stubby fingers, bloated abdomen, umbilical hernia and hallux valgus.

Figure 2

(A and B) Slit-lamp biomicroscopy showing diffuse and full-thickness stromal opacity in the right and the left eye, respectively.

Figure 3

(A–C) Histopathological slide showing interlamellar deposits, stained positive with alcian blue (A), fundus examination showing optic nerve involvement in the left eye in postoperative period (B) which was delineated in both the eyes (C and D) in preoperative period on ultrasonography B-scan.

Figure 4

Clear graft after surgery.