title: Severe blue rubber bleb nevus syndrome in a neonate.
abstract: We report on a child with blue rubber bleb nevus syndrome (BRBNS) presenting during the first days of life with severe bleeding from the upper gastrointestinal tract. Medical treatment with methylprednisolone, cyklokapron, interferon 1 alpha and numerous blood transfusions were given to control bleeding during the first 3 years of life. Afterwards repeated endoscopic electrocoagulation were performed over a period of one year resulting in a termination of bleeding episodes. At ten years of age the patient developed spastic diplegia with slight walking disabilities, coordination and fine motor problems. The case is unique because 1) it is the first neonatal case with BRBNS and severe gastrointestinal bleeding; 2) the patient was successfully treated by endoscopic electrocoagulation; and 3) the long-term follow-up. The use of electrocoagulation appears to have been effective and ablation of the stomach could be avoided until now.
authors.affiliation: Department of Paediatrics, Hvidovre University Hospital, Hvidovre, Denmark. folmer@dadlnet.dk
authors.forename: L F
authors.initials: LF
authors.lastname: Hansen
authors.forename: V
authors.initials: V
authors.lastname: Wewer
authors.forename: S A
authors.initials: SA
authors.lastname: Pedersen
authors.forename: P
authors.initials: P
authors.lastname: Matzen
authors.forename: A
authors.initials: A
authors.lastname: Paerregaard
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001165:Arteriovenous Malformations / Q000150:complications / Q000188:drug therapy / Q000601:surgery*; D004359:Drug Therapy, Combination; D004564:Electrocoagulation / Q000379:methods*; D006471:Gastrointestinal Hemorrhage / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D005773:Gastroscopy; D005938:Glucocorticoids / Q000627:therapeutic use; D006391:Hemangioma / Q000601:surgery; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007231:Infant, Newborn; D016898:Interferon-alpha / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D018329:Nevus, Blue; D012878:Skin Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*; D013577:Syndrome; D014148:Tranexamic Acid / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000933:Antifibrinolytic Agents; D005938:Glucocorticoids; D007155:Immunologic Factors; D016898:Interferon-alpha; D014148:Tranexamic Acid; D008775:Methylprednisolone