Pathma Thiagasorupan1, Emmanuel Barreau1, Gaël Gendron1,2, Eric Frau1,3, Cécile Cauquil4, David Adams4, Marc Labetoulle1, Antoine Rousseau1. 1. Department of Ophthalmology, Bicêtre Hospital, Assistance Publique - Hôpitaux de Paris, Paris-Saclay University, French Reference Center for Hereditary Transthyretin Amyloidosis (NNERF), French Reference Network for Rare Ophthalmic diseases (OPHTARA), Le Kremlin-Bicêtre, France. 2. Department of Ophthalmology, Centre Hospitalier National Ophtalmologique des XV-XX, Paris, France. 3. Centre Ophtalmologique Saint-Sulpice, Paris, France. 4. Department of Neurology, Bicêtre Hospital, Assistance Publique - Hôpitaux de Paris, Paris-Saclay University, French Reference Center for Hereditary Transthyretin Amyloidosis (NNERF), Le Kremlin-Bicêtre, France.
Abstract
INTRODUCTION: Vitrectomy may improve visual acuity of hereditary transthyretin amyloidosis (ATTRv) patients presenting with vitreous opacities but is associated with severe complications. The objective of this study is to report visual outcomes, early and late complications of a series of ATTRv patients who underwent vitrectomy in the French ATTRv reference center. METHODS: This retrospective, single-center study, included all ATTRv patients who underwent vitrectomy between 2002 and 2017. Data were collected on pre and postoperative best corrected visual acuity (BCVA) and early and late postoperative complications. RESULTS: A total of 21 eyes from 15 patients were included. The mean postoperative follow-up was 40 ± 20 months (6-160 months). BCVA increased from 0.7 ± 0.4 LogMAR preoperatively to 0.3 ± 0.4 LogMAR (p = 0.003) at last postoperative visit. During follow-up, all initially glaucomatous eyes worsened, with three eyes (37%) requiring filtering surgery and two eyes (25%) had further vision loss. Among non-glaucomatous patients, four eyes (31%) developed glaucoma with two requiring trabeculectomy and one eye (8%) had further vision loss. Three eyes (three patients) presented with complications of amyloid angiopathy. Three eyes (three patients) experienced recurrence of vitreous deposits requiring surgical revision. CONCLUSION: Due to the potential complications, vitrectomy in ATTRv requires specific perioperative management and life-long postoperative monitoring.
INTRODUCTION: Vitrectomy may improve visual acuity of hereditary transthyretin amyloidosis (ATTRv) patients presenting with vitreous opacities but is associated with severe complications. The objective of this study is to report visual outcomes, early and late complications of a series of ATTRv patients who underwent vitrectomy in the French ATTRv reference center. METHODS: This retrospective, single-center study, included all ATTRv patients who underwent vitrectomy between 2002 and 2017. Data were collected on pre and postoperative best corrected visual acuity (BCVA) and early and late postoperative complications. RESULTS: A total of 21 eyes from 15 patients were included. The mean postoperative follow-up was 40 ± 20 months (6-160 months). BCVA increased from 0.7 ± 0.4 LogMAR preoperatively to 0.3 ± 0.4 LogMAR (p = 0.003) at last postoperative visit. During follow-up, all initially glaucomatous eyes worsened, with three eyes (37%) requiring filtering surgery and two eyes (25%) had further vision loss. Among non-glaucomatous patients, four eyes (31%) developed glaucoma with two requiring trabeculectomy and one eye (8%) had further vision loss. Three eyes (three patients) presented with complications of amyloid angiopathy. Three eyes (three patients) experienced recurrence of vitreous deposits requiring surgical revision. CONCLUSION: Due to the potential complications, vitrectomy in ATTRv requires specific perioperative management and life-long postoperative monitoring.