Deep Anterior Lamellar Keratoplasty for Maroteaux-Lamy Syndrome

Abstract

Given systemic problems in patients with MLS and less postoperative complications of DALK in comparison with penetrating keratoplasty, it seems DALK is the better choice for these patients.

Indications for and Outcomes of Deep Anterior Lamellar Keratoplasty in Mucopolysaccharidoses

“…2,3,14,15 Some authors prefer this technique because signs of degeneration and decompensation could develop in the endothelium over the course of disease. 16 However, replacement of all layers of the cornea may not be needed in these cases, where the disease is mostly limited to stromal and epithelial layers. 16 Table 2 shows 11 cases (including our cases) of patients with DALK and MPS I.…”

“…16 However, replacement of all layers of the cornea may not be needed in these cases, where the disease is mostly limited to stromal and epithelial layers. 16 Table 2 shows 11 cases (including our cases) of patients with DALK and MPS I. Ten of 11 eyes had successful "big-bubble" technique.…”

Clinical guidelines for diagnosing and managing ocular manifestations in children with mucopolysaccharidosis

“…Recent reports have also shown successful outcomes with deep anterior lamellar keratoplasty in children with MPS (Harding et al. 2010; Rahmati‐Kamel et al. 2010).…”

Outcomes of keratoplasty in the mucopolysaccharidoses: an international perspective

“…There are some reports of recurrent corneal opacification following keratoplasty in patients with MPS IV,17 while others show excellent outcomes 6. Results for keratoplasty in patients with MPS VI are mixed,15 16 18 with one report of a patient who had previously been treated with HSCT maintaining a clear corneal graft for 13 years 4. Most reported cases of keratoplasty for MPS experienced some benefit, with no reported endophthalmitis and few complications.…”

“…Careful consideration of DALK versus full-thickness grafts for MPS must be made on a case-by-case basis as many patients with MPS may be ineligible for DALK due to excessive GAG accumulation in the stroma or if corneal clouding obscures assessment of endothelial cell status 18. Others have reported success with DALK in MPS 18 20 21. In our patient with full-thickness PK in one eye and DALK in the second eye, the BCVA outcome for each eye was identical.…”