Background: A patient affected by blue rubber-bleb nevus syndrome had chronic gastrointestinal bleeding requiring weekly blood transfusions. Despite multiple surgical and endoscopic procedures to treat the venous malformations, the patient continued to bleed primarily from lesions in the small bowel. Therefore, this patient was treated with octreotide, a somatostatin analog known to decrease splanchnic blood flow and that is used for acute and chronic gastrointestinal bleeding.
Methods: Octreotide therapy, 5.7 microg/kg subcutaneously twice daily, was initiated, and the patient was followed up clinically. Complete blood counts, blood glucose concentration, pancreatic enzyme concentration, liver function tests, and growth hormone concentration were monitored during treatments.
Results: During the 4 weeks after initiation of octreotide therapy, hemoglobin concentration was maintained without the need for transfusions. Octreotide decreased the patient's monthly need for blood transfusion from 52 +/- 7 mL. kg-1. mo-1 of packed red blood cells to 23 +/- 7 mL. kg-1. mo-1. She had no detectable side effects or growth inhibition. Other medical interventions including -epsilonaminocaproic acid, nadolol, and total parenteral nutrition with bowel rest were not as effective as octreotide alone.
Conclusion: Octreotide decreased the patient's need for blood transfusions. Possible mechanisms include altering blood flow to the gastrointestinal tract and direct effects on the venous malformations.