Gastrointestinal Blue Rubber Bleb Nevus Syndrome in Children: A Case Series Across Endoscopic, Surgical, and Medical...

Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare venous malformation disorder causing recurrent gastrointestinal bleeding and severe anemia. We report three children illustrating endoscopic, surgical, and medical management. All presented with melena and transfusion-requiring anemia. Case 1 developed gastric perforation after endoscopic band ligation and underwent emergency primary repair with bowel-preserving excision of accessible lesions, followed by recovery. Case 2 had more than 100 gastrointestinal lesions and underwent lesion-directed wedge resections, achieving transfusion independence and hemoglobin normalization at 1 year. Case 3 had idiopathic extrahepatic portal vein obstruction, portal hypertension, and diffuse mesenteric disease that was not safely resectable; sirolimus was initiated, but intermittent bleeding and transfusion dependence persisted. Management should be individualized according to lesion burden, anatomical extent, procedural risk, and feasibility of bowel-preserving treatment. Surgery remains important for complications and resectable disease, whereas systemic therapy may be required for diffuse unresectable involvement.