2017 An Uncommon Etiology for Gastrointestinal Bleed in Elderly: Blue Rubber Bleb Nevus Syndrome

Abstract

INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital anomaly characterized by multiple venous malformations that can appear anywhere on the body, but most often involve the skin and gastrointestinal (GI) tract. GI bleeding usually presents in childhood or early adulthood as iron deficiency anemia. We report a case of BRBNS as a rare presentation of GI bleeding in an elderly patient with no prior history of cutaneous or GI tract involvement of the disease. CASE DESCRIPTION/METHODS: 87-year-old man with a past medical history of hypertension, coronary artery disease and atrial fibrillation (on Xarelto) was transferred to our hospital for evaluation of melena. He presented to an outside institution initially with complaints of dizziness and black tarry stools for 5 days prior to admission. He was hypotensive, labs were significant for hemoglobin (Hgb) of 5.5 g/dL and a positive stool occult blood test. Anticoagulation was discontinued. He underwent esophagogastroduodenoscopy (EGD), which showed friable mucosa in the duodenal bulb but no active source of bleeding. He received 8 units of packed red blood cells (PRBC), but his Hgb only improved to 7 g/dL. A repeat EGD was also unrevealing. He then underwent a nuclear scan, which showed a focus of bleed in left hemi-abdomen, but it was unclear if it was from the small or large bowel. On presentation to our hospital, his Hgb was 6.6 g/dL and he continued to have melena, requiring multiple PRBC transfusions. Push endoscopy revealed diffuse discontinuous multiple vascular blebs of the mucosa in the mid jejunum (Figure 1a), one of which was found bleeding (Figure 1b). Successful hemostasis was achieved with cauterization of the bleeding bleb with argon plasma coagulation (APC) (Figure 1c,d) and a hemoclip placement 2 cm distal to the lesion. Post-procedure, his Hgb remained stable above 8 g/dL throughout the hospitalization. DISCUSSION: Although BRBNS lesions can occur anywhere from mouth to anus, the small bowel is the most common site of GI tract involvement. Its presentation can vary from iron deficiency anemia to massive hemorrhage, intestinal torsion, and rupture. Some patients require lifelong iron replacement and repeated blood transfusions. Push endoscopy is the most important diagnostic modality for GI lesions. Treatment depends on the severity of lesions and can range from supportive therapies such as iron supplementation and blood transfusion to more invasive therapies such as mucosal resection, APC, sclerotherapy or bowel resection.