What are the therapeutic options for angiodysplasia lesions in Coats plus (Cerebroretinal microangiopathy with calcifications and cysts) syndrome?

From the Research

Therapeutic Options for Angiodysplasia Lesions in Coats Plus Syndrome

The therapeutic options for angiodysplasia lesions in Coats plus syndrome are limited and primarily focused on managing symptoms and preventing complications. Some of the options include:

• Monthly octreotide treatment to control recurrent gastrointestinal bleeding, as reported in a patient with Coats plus syndrome 1
• Laser photocoagulation and intravitreal bevacizumab to improve retinal vascular and exudative changes, as used in a case of two brothers with Coats Plus syndrome 2
• Palliative care and intravenous octreotide to stop bleeding, as used in a 6-year-old girl with Coats plus syndrome 3
• Genetic testing to confirm diagnosis, as genetic testing found 2 pathological mutations in the conserved telomere maintenance component 1 (CTC1) gene, diagnostic of Coats plus syndrome 4

Gastrointestinal Bleeding Management

Gastrointestinal bleeding is a common complication in Coats plus syndrome, and management options include:

• Octreotide treatment to control bleeding, as reported in a patient with Coats plus syndrome 1
• Endoscopy to diagnose and treat vascular telangiectasia, as used in a 6-year-old girl with Coats plus syndrome 3

Retinal Vascular Disease Management

Retinal vascular disease is a key feature of Coats plus syndrome, and management options include:

• Laser photocoagulation and intravitreal bevacizumab to improve retinal vascular and exudative changes, as used in a case of two brothers with Coats Plus syndrome 2
• Fluorescein angiography to diagnose retinal capillary nonperfusion, as used in a 38-year-old woman with Coats plus syndrome 4