Transjugular Intrahepatic Portosystemic Shunt for Recurrent Gastrointestinal Bleeding in Coats Plus Syndrome: A Case Report

Coats plus syndrome is a rare autosomal-recessive telomeropathy occurring from mutations in the CTC1 gene, and only a few cases have been reported worldwide. It is characterized by multisystem involvement, particularly affecting the retina, brain, liver, and bone marrow. Current management is largely supportive, highlighting the need for targeted therapies. We report a 25-year-old patient with a history of aplastic anemia who presented with ascites and variceal bleeding. Clinical suspicion prompted evaluation for Coats plus syndrome, which was confirmed through genetic testing. Recurrent gastrointestinal bleeding was successfully managed with transjugular intrahepatic portosystemic shunt, representing, to our knowledge, its first reported use in such patients.