Coats’-like Response Associated with Linear Scleroderma


Purpose: To present a case of linear scleroderma known as “en coup de sabre” associated with Coats’- like response.

Case Report: A 12-year-old boy presented with subacute painless vision loss in the ipsilateral side of the patient’s en coup de sabre lesion. Ocular examination revealed vitreous hemorrhage with severe exudation of the posterior pole and telangiectatic vessels. Fundus fluorescein angiography indicated multiple vascular beadings and fusiform aneurysms with leakage which was consistent with a Coats’-like response. The patient was subsequently treated with intravitreal bevacizumab and targeted retinal photocoagulation. Twelve months’ follow-up showed marked resolution of macular exudation with significant visual improvement.

Conclusion: Physicians should be aware of the possible ophthalmic disorders accompanying en coup de sabre and careful ophthalmologic examinations should be performed in these patients. As presented in the current case, treatment with intravitreal anti-VEGF agents and laser photocoagulation may be a beneficial option for patients with coats’-like response.


Bevcizumab, Coat’s Disease, Craniofacial, En Coup de Sabre, Scleroderma

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