Coats Disease in 9 Patients: A Hispanic Case Series

Abstract

Background/Aim To describe demographic and clinical characteristics, treatment, and visual prognosis of Coats disease in Hispanic patients.

Methods A retrospective chart review was performed on nine patients (ten eyes) diagnosed with Coats disease in our two clinical centrers from 2004 – 2017.

Results Mean age at diagnosis was 5.5 years (range 1 – 12 years) and mean follow-up time was 48 months (range 9 – 108 months). Eight patients (89%) were male and had unilateral disease and one (11%) female patient had bilateral disease. In 40% of the cases, patients were asymptomatic. Visual acuity at first presentation was worse than hand motion in 60% of the eyes. Half of the eyes (5/10 eyes, 50%) had exudative retinal detachment (≥ stage IIIA). Vascular ablation with cryotherapy combined with retinal photocoagulation was the most frequent therapeutic approach (40%). Despite anatomical success at 6 months in 100% of the treated eyes, visual outcome at 1 year of treatment was poor (worse than 20/200) in 70% of the cases.

Conclusions In our case series, patients were mostly asymptomatic on presentation, with severe stages of Coats disease. Even with anatomical success after surgical treatment in all treated cases, long-term visual prognosis remained very limited.

Zusammenfassung

Hintergrund/Ziel Beschreibung der demografischen und klinischen Charakteristika, der Behandlung und Prognose bei Morbus Coats in hispanischen Patienten.

Methoden Retrospektive Observationsstudie von Patienten mit Morbus Coats, die zwischen 2004 und 2017 in unseren 2 spezialisierten Augenkliniken vorstellig waren.

Ergebnisse Das mittlere Alter bei Erstdiagnose betrug 5,5 Jahre (Range: 1 – 12 Jahre) und die mittlere Nachbeobachtungszeit betrug 48 Monate (Range: 9 – 108 Monate). Acht Patienten (89%) waren männlich und hatten eine einseitige Erkrankung. Eine weibliche Patientin (11%) präsentierte eine bilaterale Erkrankung. In 40% der Fälle waren die Patienten asymptomatisch. Der Visus beim ersten Besuch war bei 60% der Augen eine Handbewegung oder schlechter. Die Hälfte der Patienten (5/10 Augen, 50%) zeigten bei Erstdiagnose bereits eine exsudative Netzhautablösung (≥ Stadium IIIA). Die Kryotherapie in Kombination mit einer Photokoagulation der Netzhaut war der häufigste therapeutische Ansatz (40%). Trotz eines anatomischen Erfolgs nach 6 Monaten in 100% der behandelten Augen blieb der Visus in 70% der behandelten Augen auch nach 1 Jahr weiterhin schlecht (< 20/200 Snellen = 0,1 Dezimal).

Fazit In unserer Fallserie waren die meisten Patienten trotz bereits fortgeschrittenem Erkrankungsstadium bei Erstdiagnose asymptomatisch. Auch mit erfolgreicher chirurgischer Intervention blieb die langfristige Visusprognose in vielen Fällen sehr reduziert.

Key words

Coats disease - cryotherapy - exudative retinal detachment - Hispanics - subretinal fluid drainage - vascular ablation

Schlüsselwörter

Morbus Coats - Kryotherapie - exsudative Netzhautablösung - Hispanics - subretinale Flüssigkeitsdrainage - Gefäßablation

Publication History

Received: 02 January 2021

Accepted: 27 September 2021

Article published online:
15 December 2021

• References

• 1 Kivela T. George coats and his disease. Ophthalmologica 2012; 228: 194-195
• 2 Shields JA, Reichstein D, Mashayekhi A. et al. Retinal vasoproliferative tumors in ocular conditions of childhood. J AAPOS 2012; 16: 6-9
• 3 Meier P. [Retinal detachment in children: differential diagnosis and current therapy]. Klin Monbl Augenheilkd 2008; 225: 779-790
• 4 Joussen AM, Gordes RS, Heussen FA. et al. [Retinal exudative disease in childhood: Coatsʼ disease and familial exudative vitreoretinopathy (FEVR)]. Klin Monbl Augenheilkd 2013; 230: 902-913
• 5 Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina 2002; 22: 80-91
• 6 Shields JA, Shields CL, Honavar SG. et al. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol 2001; 131: 561-571
• 7 Rishi P, Rishi E, Uparkar M. et al. Coatsʼ disease: an Indian perspective. Indian J Ophthalmol 2010; 58: 119-124
• 8 Morris B, Foot B, Mulvihill A. A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis. Eye (Lond) 2010; 24: 1797-1801
• 9 Al-Qahtani AA, Almasaud JM, Ghazi NG. Clinical Characteristics and Treatment Outcomes of Coats Disease in a Saudi Arabian Population. Retina 2015; 35: 2091-2099
• 10 Cebeci Z, Bayraktar S, Yilmaz YC. et al. Evaluation of Follow-Up and Treatment Results in Coatsʼ Disease. Turk J Ophthalmol 2016; 46: 226-231
• 11 Mulvihill A, Morris B. A population-based study of Coats disease in the United Kingdom II: investigation, treatment, and outcomes. Eye (Lond) 2010; 24: 1802-1807
• 12 Shields CL, Udyaver S, Dalvin LA. et al. Coats disease in 351 eyes: Analysis of features and outcomes over 45 years (by decade) at a single center. Indian J Ophthalmol 2019; 67: 772-783
• 13 Singh SR, Jayakumar K, Jain S. et al. Diagnosis and treatment of bilateral Coats disease in a 5-year-old girl. J AAPOS 2019; 23: 243-245
• 14 Rishi E, Rishi P, Appukuttan B. et al. Coatsʼ disease of adult-onset in 48 eyes. Indian J Ophthalmol 2016; 64: 518-523
• 15 Smithen LM, Brown GC, Brucker AJ. et al. Coatsʼ disease diagnosed in adulthood. Ophthalmology 2005; 112: 1072-1078
• 16 Ong SS, Buckley EG, McCuen 2nd BW. et al. Comparison of Visual Outcomes in Coatsʼ Disease: A 20-Year Experience. Ophthalmology 2017; 124: 1368-1376
• 17 Daruich A, Matet A, Munier FL. Younger Age at Presentation in Children with Coats Disease Is Associated with More Advanced Stage and Worse Visual Prognosis: A Retrospective Study. Retina 2018; 38: 2239-2246
• 18 Brockmann C, Lowen J, Schonfeld S. et al. Vascular findings in primarily affected and fellow eyes of middle-aged patients with Coatsʼ disease using multimodal imaging. Br J Ophthalmol 2021; 105: 1444-1453
• 19 Resnikoff S, Lansingh VC, Washburn L. et al. Estimated number of ophthalmologists worldwide (International Council of Ophthalmology update): will we meet the needs?. Br J Ophthalmol 2020; 104: 588-592
• 20 Sigler EJ, Randolph JC, Calzada JI. et al. Current management of Coats disease. Surv Ophthalmol 2014; 59: 30-46
• 21 Han ES, Choung HK, Heo JW. et al. The effects of external subretinal fluid drainage on secondary glaucoma in Coatsʼ disease. J AAPOS 2006; 10: 155-158
• 22 Lin CJ, Hwang JF, Chen YT. et al. The effect of intravitreal bevacizumab in the treatment of Coats disease in children. Retina 2010; 30: 617-622
• 23 Enders P, Schaub F, Fauser S. [Indications for Retinal Laser Therapy Revisited]. Klin Monbl Augenheilkd 2018; 235: 1383-1392
• 24 Ramasubramanian A, Shields CL. Bevacizumab for Coatsʼ disease with exudative retinal detachment and risk of vitreoretinal traction. Br J Ophthalmol 2012; 96: 356-359
• 25 Shields JA, Shields CL, Honavar SG. et al. Classification and management of Coats disease: the 2000 Proctor Lecture. Am J Ophthalmol 2001; 131: 572-583