Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition.

INTRODUCTION

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease that affects the vasculature of numerous organs. HHT is characterized by the presence of multiple arteriovenous malformations that lack intervening capillaries and result in direct connections between arteries and veins.

PRESENTATION OF CASE: A 58-year-old South Korean female was admitted presenting with severe anemia, dizziness, and intermittent hematochezia. The patient had experienced chronic severe refractory gastrointestinal bleeding for years. She had an intermittent spontaneous epistaxis, telangiectasias in the oral cavity (tongue), and hepatic arteriovenous malformations and gastrointestinal telangiectasia. She underwent a segmental resection of a segment of the ileum and cecum (about 77 cm). After surgery, the patient experienced no more drops in hemoglobin count or symptoms of anemia and melena. She is living a normal life.

CONCLUSION

The present case report is a surgical case of undiagnosed HHT with chronic refractory gastrointestinal bleeding. Surgical treatment is rarely required for gastrointestinal bleeding unless nonsurgical methods such as endoscopy/colonoscopy and angiography have failed.