Maculopathy in sickle cell disease

Bachmeier, Isabel and Blecha, Christiane and Foell, Juergen and Wolff, Daniel and Jaegle, Herbert (2021) Maculopathy in sickle cell disease. OPHTHALMOLOGE, 118 (10). pp. 1013-1023. ISSN 0941-293X, 1433-0423

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Abstract

Background Sickle cell disease (SCD) is a hereditary hemoglobinopathy, which leads to microcirculatory disturbances of various organ systems through recurrent vaso-occlusive episodes, with a possibly fatal outcome. Sickle cell retinopathy (SCR) is the best described ocular manifestation of SCD. Irrespective of the presence of peripheral SCR, sickle cell maculopathy (SCM) can occur early in the course of the disease. Methods Review of the international and German literature on ocular involvement in SCD with a focus on SCR and SCM and an overview of current systemic therapeutic approaches in SCD on the occasion of the presentation of two patients with HbSS SCD. Results and conclusion In contrast to SCR, SCM with temporal thinning of the inner retinal layers has only been increasingly described in the literature in the last 5 years, with the advent of SD-OCT and OCTA. Irrespective of the presence of SCR, as many as about half of the patients may develop SCM early in the course of the disease. As a result of progress in systemic therapeutic options and due to migration, the clinical picture will occur more often also in Germany. By knowing about this complication of SCD an early diagnosis can be made and unnecessary diagnostics can be avoided.

Item Type: Article
Uncontrolled Keywords: ; Sickle cell maculopathy; Macular thinning; Retinopathy; Optic neuropathy; OCT angiography
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Augenheilkunde
Medicine > Lehrstuhl für Innere Medizin III (Hämatologie und Internistische Onkologie)
Medicine > Abteilung für Pädiatrische Hämatologie, Onkologie und Stammzelltransplantation
Depositing User: Dr. Gernot Deinzer
Date Deposited: 02 Aug 2022 06:21
Last Modified: 02 Aug 2022 06:21
URI: https://pred.uni-regensburg.de/id/eprint/45959

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