title: [Bean or blue rubber blue nevus syndrome. Presentation of 6 patients].
abstract: OBJECTIVE
  Bean or Blue Rubber Bleb Nevus Syndrome (BRBNS) is an uncommon disease characterized by multifocal venous malformations that predominantly affect the skin, soft tissues and gastrointestinal tract. Our objective is to familiarize the pediatric surgeon with this condition, promoting early diagnosis and seriated surgical treatment as therapeutic options. We have reviewed the diagnosis and treatment of 6 patients treated from 1993 to 2009.
  
  
  METHODS
  All the patients had a venous malformation (> 10 cm) that acted as a marker. After, characteristic skin lesions appeared as well as chronic anemia due to digestive bleeding associated to hypofibrinogenemia. Age of appearance of the anemic picture varied from 6 months to 7 years, it being more aggressive the earlier its appearance. The diagnosis of the disease was made by endoscopically. All the patients required transfusion with blood derivatives 5 to 25 times a year. Treatment was multidisciplinary: periodic transfusions in every case, endoscopic sclerosis in lesions that affect the colon or stomach and surgical treatment for lesions of the jejunum and ileum. In two of these patients, 23 and 46 lesions were excised, respectively. In the last year, the tendency has been to use enterotomy and fulguration with Argon.
  
  
  RESULTS
  The patients who have undergone excision of the digestive malformations have experienced a significant decrease in their transfusion needs. One female patient with miliary dissemination died.
  
  
  CONCLUSIONS
  BRBNS is a difficult to diagnose due to its low frequency. Treatment of gastrointestinal tract lesions is multidisciplinary, combining endoscopic and surgical techniques that decrease transfusions and improve the quality of life of the patients.
authors.affiliation: Departamento de Cirugía Pediátrica, Hospital Universitario La Paz, Madrid. ramip.maria@gmail.com
authors.forename: M
authors.initials: M
authors.lastname: Ramírez
authors.forename: J C
authors.initials: JC
authors.lastname: López Gutiérrez
authors.forename: M
authors.initials: M
authors.lastname: Diaz
authors.forename: C
authors.initials: C
authors.lastname: Soto
authors.forename: M
authors.initials: M
authors.lastname: Miguel
authors.forename: C A
authors.initials: CA
authors.lastname: de la Torre Ramos
authors.forename: S
authors.initials: S
authors.lastname: Barrena
authors.forename: Z
authors.initials: Z
authors.lastname: Ros
authors.forename: J A
authors.initials: JA
authors.lastname: Tovar
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D018329:Nevus, Blue / Q000175:diagnosis / Q000628:therapy; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis / Q000628:therapy