Blue Rubber Bleb Nevus Syndrome

Opinion statement  

–	Venous malformations of blue rubber bleb nevus syndrome (BRBNS) may involve any area of the gastrointestinal tract.
–	Gastrointestinal blood loss and anemia brings these patients to the attention of gastroenterologists.
–	Effective treatment of these malformations throughout the gastrointestinal tract requires aggressive management to ultimately decrease blood loss and restore the patient’s hemoglobin to a near-normal level.
–	Treatment of patients with BRBNS includes supportive measures, endoscopic ablation, and surgery.
–	Supportive therapy consists of proton pump inhibitors and octreotide to decrease blood loss, iron replacement, and blood transfusions.
–	The effective management of patients with anemia demands aggressive treatment of venous malformations in the small bowel. This requires a collaboration between the surgeon and the therapeutic endoscopist, ie, laparotomy and excision of larger lesions with surgically assisted enteroscopy and thermal ablation of smaller lesions via enterotomy.
–	There is no effective systemic therapy for treatment of the vascular malformations in patients with BRBNS.