Autologous Full Thickness RPE-Choroid Transplantation in Inherited Macular Dystrophy

Abstract

Purpose:: To describe 4 patients with inherited macular dystrophy who had undergone an autologous RPE-choroid transplantation

Methods:: 4 patients (1 with RDS R172W mutation and 3 with un-characterised bulls eye maculopathy) were included. Autologous RPE grafts were performed only if patients had less than 6 month history of subjective loss of reading ability. Each patient had pre-operative and 6 month post-operative distance acuity (EDTRS) and reading ability (MNRead) assessments, colour fundus photography, angiography, autofluorescence imaging, OCT, multifocal ERG, automated microperimetry and fixation analysis. Preoperative distance logMAR acuity ranged from 0.32 to 0.82.

Results:: At 6 - 9 month post-operatively, all grafts were perfused with viable RPE based on high-speed ICG angiography and autofluorescence imaging. In one of four patients, foveal fixation was restored and reading speed increased from zero to 58 words per minutes for logMAR 0.6 text. Multifocal ERG also demonstrated improved function of the neural retina over the RPE graft compared to pre-operative measurement. Despite preservation of retinal sensitivity (measured by Nidek MP1) over the graft in all patients, none had improvement in distance visual acuity. Post-operatively, one patient had a macular-on retinal detachment which was successfully reattached and another had a minor vitreous haemorrhage which spontaneously resolved.

Conclusions:: This is the first demonstration of restored foveal fixation and improved reading speed following autologous RPE-choroid graft in progressive inherited macular dystrophy.

Keywords: transplantation • retinal pigment epithelium • retinal degenerations: hereditary