Required stage dependent therapy in coats disease

Abstract

AbstractPurposesCoats disease is a rare retinal vascular disorder characterized by telangiectasia and exudation affecting predominantly young male. Due to its rareness, analyses of large cohorts over long periods are of particular importance to give a prognosis for the usually very young patients and their parents, especially with regard to required therapy.MethodsWe retrospectively analyzed patients diagnosed with Coats disease and treated between May 1992 und March 2019. The minimum follow‐up was 12 months. We evaluated the interventional procedures with regard to the initial stage.ResultsSeventy‐seven eyes of 75 patients were included in the analyses. Eighty‐one percent were male; mean age was 17.8 ± 18.2 years (range 1–71; median 10.0). Mean follow‐up time was 7.3 ± 5.6 years (range 1.1–21.4; median 6.5). In eyes with stage 1 (n = 2) only lasercoagulation (LC) was necessary. In stage 2A 65% (11/17) of eyes required LC, 30% (5/17) cryotherapie (CT), 25% (4/17) vitrectomy (VT) and 18% (3/17) ruthenium brachytherapy (RT) due to secondary vasoproliferative retinal tumor. In stage 2B 88% (29/33) were treated with LC, 30% (10/33) with CT, 21% (7/33) needed VT and 6% (2/33) RT. In 89% (8/9) of eyes staged 3A1 LC was necessary, in 100% (9/9) CT and in 44% (4/9) VT. Three eyes were staged 3A2 and all of them required VT, two additional LC and one additional CT. Similar results were seen in stage 3B (five eyes): in all eyes VT was performed, in 80% (4/5) LC and CT was done in addition. Four of six (66%) stage 4 eyes were enucleated, the 2 remaining received CT and VT or RT. We had 2 stage 5 eyes, thereof one required enucleation.ConclusionTherapy in patients with Coats’ disease strongly depends on the initial stage with less intense procedures in early stages. While most invasive treatment was necessary in stage 3, the rate of enucleation was, as expected, highest in stage 4. Therefore early diagnosis and immediate treatment is of highest priority to preserve the eye.