Bilateral Macular Halo and Full-Thickness Macular Hole Repair in Niemann-Pick Disease Type B

Purpose: To report the results of surgery in a patient with Niemann-Pick disease type B, bilateral macular halos, and a full-thickness macular hole (FTMH) in the right eye. Methods: A case was evaluated. Results: A 72-year-old man with Niemann-Pick disease type B presented with an FTMH in the right eye. On examination, the visual acuity (VA) was 20/120 OD and 20/16 OS. Bilateral, symmetric, circular yellow–white deposits encircled the fovea. Optical coherence tomography showed focal parafoveal inner retinal hyperreflectivity bilaterally and an FTMH in the right eye. The patient had a vitrectomy with inner limiting membrane (ILM) peeling; the peeled membrane was unremarkable on cytopathology. Six weeks postoperatively, the MH was closed and the VA had improved to 20/40. Conclusions: Successful MH closure is possible in the presence of macular halos secondary to Niemann-Pick disease type B. Cytopathology of the ILM suggests the ILM is not involved in the pathogenesis of macular halos.