Diagnosis and treatment of gastrointestinal bleeding in Bean syndrome

Blue Rubber-Bleb Nevus Syndrome (BRBNS, Bean Syndrome) is a rare disorder characterized by the presence of cutaneous and visceral vascular malformations. Although it may affect several organs, the gastrointestinal tract is one of the most frequently involved sites. It can cause gastrointestinal bleeding and chronic anemia. We report an 8-years-old girl who was admitted because of cutaneous malformations and anemia suspected to be caused by gastrointestinal bleeding. Cutaneous lesions were compatible with Blue Rubber-Bleb Nevus Syndrome. Vascular malformations were confirmed by diagnostic gastroduodenoscopy and colonoscopy and further therapeutical intervention was planned. We performed banding of 2 lesions located in the stomach and sclerotherapy of a duodenal lesion. Six colonic lesions were treated with banding and three other polypoid lesions were endoscopically resected A combined laparoscopic and endoscopic approach was performed for evaluation and treatment of small-bowel lesions. Eighteen lesions were found. We endoscopically resected 8 polipoid lesions. Eight other large transmural lesions were removed by wedge resection. Only one lesion required termino-terminal anastomosis. We consider that endoscopic treatment and laparoscopically assisted enteroscopy are safe and effective options in the treatment of gastrointestinal bleeding caused by vascular malformations.