Rates of Bone Spicule Pigment Appearance in patients with Retinitis Pigmentosa Sine Pigmento.

PURPOSE: To determine rate of bone spicule pigmentation appearance in patients with Retinitis Pigmentosa (RP) DESIGN: Retrospective, observational case series.

PARTICIPANTS: A total of 240 patients were analyzed for this study METHODS: A retrospective analysis was conducted at the Electrodiagnostic Clinic at Columbia University Medical Center of all patients' medical records with a diagnosis of RP between July 2017 and January 2018. The medical records of these patients were analyzed to determine whether the patients presented with pigment migration on their first and last visit to our clinic. Among those who did not have bone spicule at first visit, we examined the time to appearance of newly formed bone spicule. The survival distribution was then estimated using the Kaplan-Meier estimator, where the event is bone spicule and time starts at first visit.

RESULTS: From the 240 patients analyzed, 213 patients presented with intraretinal pigmentation on the first visit to our clinic, and 27 patients presented without intraretinal pigmentation. Of these 27 patients, 10 patients developed pigmentation by their follow-up, with a median time to appearance of bone spicule of 5.4 years from first visit, according to the Kaplan-Meier estimates.

CONCLUSIONS: The timeline of bone spicule pigment appearance in RP has important implications in the natural history characterization of disease progression and application as a biomarker for interventional trials.