Sequential Customized Therapeutic Keratectomy for Reis-Bücklers' Corneal Dystrophy: Long-term Follow-up

Abstract

R eis-Bücklers' corneal dystrophy, an anterior stromal dystrophy caused by mutations in the transforming growth factor beta-induced gene, typically presents in childhood with confluent, early, irregular geographic-like opacities varying in density and developing at the level of Bowman's layer and superficial stroma. 1 Opacities are initially discrete, but subsequently extend to the limbus and deeper stroma. Corneal optical coherence tomography (OCT) shows a homogenous, confluent, membrane-like layer of hyperreflective deposits at the level of Bowman's layer and anterior stroma. 2 Reis-Bücklers' corneal dystrophy progressively hinders visual acuity and causes recurrent erosions. Conservative therapy includes gels and ointments, but conventional treatment for extreme cases includes corneal transplantation. 3 Femtosecond laser-assisted lamellar keratectomy, featuring a 110-to 140-µm corneal free cap, has been recently reported. 4 Phototherapeutic keratectomy (PTK) using a 193-nm excimer laser has been used to treat vision-hampering corneal dystrophies causing recurring corneal erosion for the past 25 years. 5-8 PTK appears to be the less invasive surgical solution, and it can be repeated, postponing for a long time partial-or full-thickness corneal transplantation (deep anterior lamellar keratoplasty or penetrating keratoplasty). 9

Corneal histomorphology and electron microscopic observation of R124L mutated corneal dystrophy in a relapsed pedigree

“…Nevertheless, the disease often relapses within several years after the operation. When there is CD recurrence, PTK or keratoplasty can be performed again [12,29] . Therefore, the patients with CD recurrence in this study underwent PKP or LKP again.…”

“…The diagnostic basis of CD mainly includes clinical observation of lesion location and morphology, histopathological changes, and genetic testing and typing results [8][9][10] . Current treatments for CD include phototherapeutic keratectomy (PTK) and keratoplasty [11][12][13][14] . After surgery, the symptoms could be improved or relieved and vision could be improved.…”

Management of Stromal Corneal Dystrophies; Review of the Literature with a Focus on Phototherapeutic Keratectomy and Keratoplasty

“…Vinciguerra and colleagues showed a variation of a technique of sequential customised therapeutic keratectomy, which achieved a significant visual improvement in their cases from an average VA of 20/50 preoperatively to post-op average VA of 20/25 ( p < 0.01) [ 16 ]. They showed a significant reduction in the coma aberration and no change in the spherical and trefoil as well as total higher-order aberrations.…”

“…Another strategy to enhance visual outcomes and reduce recurrence rates was described by Vinciguerra and colleagues [ 16 ], who reported good outcomes from sequential customised therapeutic keratectomy in 14 eyes of eight patients with RBCD with a mean follow-up of approximately 5 years. The surgical technique involved peeling the epithelium and the subepithelial membrane in patients with RBCD with a surgical spatula and subsequent multiple customised sequential keratectomies with repeated excimer laser photoablations guided by multiple intraoperative topographies to target irregularities.…”