Vascular findings in primarily affected and fellow eyes of middle-aged patients with Coats’ disease using multimodal imaging

Abstract

AimsTo investigate the retinal vascular structure and capillary anomalies of affected and fellow eyes of patients with unilateral Coats’ disease using multimodal imaging.MethodsClinical investigation of both eyes of each patient with diagnosed Coats’ disease using ultra-widefield (UWF) fundus imaging, including UWF fluorescein angiography (UWFFA), spectral domain optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A).ResultsWe analysed 38 eyes of 19 patients with unilateral Coats’ disease and found that all fellow eyes (19/19; 100%) revealed vascular alterations, detected by UWFFA, predominantly located in the temporal periphery. Thereby, 89% of the fellow eyes (17/19) presented capillary bed abnormalities, that did not exceed the capillary level; 58% (11/19) presented tortuous abnormalities and 26% (5/19) presented microaneurysmatic abnormalities, exceeding the capillary level. If primarily affected eyes presented central Coats’ specific vascular abnormalities, fellow eyes revealed tortuous vascular abnormalities twice as often (78% (7/9) vs 40% (4/10); P=0.096). In primarily affected eyes, a tendency towards larger foveal avascular zones was revealed, compared to fellow eyes (0.28±0.16 mm2 vs 0.20±0.10 mm2; P=0.123).ConclusionThe use of modern multimodal imaging allows the detection of even subtle vascular changes in fellow eyes of patients with Coats’ disease. Coats’ disease appears to be a bilateral ocular disease with a predominant manifestation in one eye of the affected patients.

Updating Understanding of Macular Microvascular Abnormalities and Their Correlations With the Characteristics and Progression of Macular Edema or Exudation in Coats' Disease

“…Shields et al described retinal capillary dropout adjacent to telangiectasia in the peripheral regions on fundus FFA images, ( 2 ) but the existence of macular capillary dropout was unknown. More recently, Brockmann et al reported an enlarged foveal avascular zone in eyes with stage 1–2 Coats' disease (19 eyes) compared with the contralateral eyes, but the difference was not significant ( 12 ). Schwartz et al reported that the parafoveal VD was decreased in eyes with Coats' disease (13 eyes) compared with unaffected contralateral eyes, ( 13 ) but the sample was relatively small and the clinical significance of the decreased VD was not determined.…”

“…To achieve this, we enrolled a relatively large number of patients with eyes displaying macular edema or exudation secondary to Coats' disease and followed them for over 18 months. Considering that widefield imaging revealed abnormal peripheral vessels in the unaffected eyes of patients with Coats' disease in prior studies ( 8 , 12 ), widefield imaging of the unaffected eyes was performed in our study at baseline and during the follow-up period to ensure they did not have abnormal vessels or exudation. This allowed us to use the unaffected eyes as a control group.…”

Longitudinal characterization and treatment response of retinal arterial macroaneurysms in adult-onset coats disease

“… 2 , 4 Some studies have suggested bilateral involvement in adult-onset disease. 4 , 5 Somatic mutations of implicated genes, such as NDP and ABCA4, have been associated with some cases of pediatric Coats disease. 6 , 7 …”

“… 3 , 10 , 11 Laser photocoagulation, cryotherapy, intravitreal anti-vascular endothelial growth factor (anti-VEGF), drainage of subretinal fluid, and vitrectomy have been used in the treatment of Coats disease. 3 , 5 , 12 If left untreated, Coats disease can lead to secondary glaucoma and total retinal detachment. 1 , 2 , 13 …”

Experience with Intravitreal Ranibizumab as an Adjunct to Ablation Therapy in Eyes with Exudative Coats’ Disease

“… 3 The disease is believed to be congenital but non-familial with a classic predilection towards unilateral presentation in young males, 3 although the bilaterality of the condition has recently been suggested with the imaging of subtle vascular abnormalities in the fellow eye. 4 Classification is based on morphology, with Shields’ classification 5 being the most commonly utilized.…”