Blue Rubber Bleb Nevus Syndrome.
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.
Andersen
John M.
JM
Department of Pediatrics, Division of Pediatric Gastroenterology, University of Texas Southwestern Medical School, Children's Medical Center of Dallas, 1935 Motor Street,Dallas, TX 75235, USA. janderse@childmed.dallas.tx.us