Abstract
Purpose:
To report the utility of PROSE prosthetic devices in patients with Familial Dysautonomia. PROSE therapy is used in patients with ocular surface disease. Patients with Familial Dysautonomia suffer from neurotrophic keratoconjunctivitis and alacrima secondary to familial dysautonomia.
Methods:
Retrospective case series of five consecutive patients with Familial Dysautonomia who were fit with PROSE prosthetic devices by a PROSE-trained optometrist at our institution and who received care by one of our cornea specialists with at least two years of follow-up. Outcome measures studied included visual acuity and ocular surface status (at time of device fitting, three months, and most recent visit), time to full time wear, and complications. Visual acuity improvement at 3 months and most recent exam compared to initial acuity was analyzed by the Student’s paired t-test.
Results:
Nine eyes of five FD subjects, aged 5 to 23 years, underwent fitting with the PROSE prosthetic device and received subsequent follow-up care at our institution for an average of 30 months (24-40 months). Prior to fitting, all patients exhibited corneal scarring and/or punctate epithelial erosions (PEE) on slit-lamp exam and the average visual acuity was 0.801 ± 0.29 logMAR. All patient caregivers were able to learn device insertion and removal techniques within the first two visit. At the three month time point, all patients showed improvement of PEE, though scarring remained. Three month average visual acuity was 0.576 ± 0.17 logMAR (p=0.08). At the most recent visit, average visual acuity improved to 0.504 ± 0.12 logMAR (p=0.01), and all eyes showed improvement of their ocular surface.
Conclusions:
Because of its long term positive effects on visual acuity and ocular surface health, PROSE therapy is a valuable treatment option for patients with familial dysautonomia who suffer from corneal disease.