title: Life-threatening pharyngeal edema after sclerotherapy of oral venous malformations in a patient with blue rubber bleb nevus syndrome.
abstract: BACKGROUND
  Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular disorder characterized by multiple venous malformations of the skin and internal organs. Oral lesions are very common and occur in over half of the patients with this condition. Sclerotherapy is currently the first-line treatment modality of symptomatic cases due to its high efficacy and low rate of complications.
  
  
  METHODS
  We report the case of a 68-year-old male with BRBNS who presented with dysphagia and difficulty with speech due to prominent oral venous malformations. After the use of sclerotherapy with ethanolamine oleate to control his symptoms, the patient exhibited severe edema of the tongue and posterior pharyngeal wall which caused constriction of his airway. The patient was intubated, and remained so for 72 hours until his edema resolved. In addition to his oral lesions, the patient also exhibited other features of BRBNS including cutaneous, soft-tissue, gastrointestinal, and neurological manifestations of disease.
  
  
  CONCLUSIONS
  Physicians should be aware of the potentially life-threatening complication of severe tongue and pharyngeal edema when using sclerotherapy for the treatment of oral vascular malformations. Additionally, dermatologists should be familiar with the many systemic manifestations which can be present in patients with BRBNS, as illustrated in this case.
authors.affiliation: University of Miami Miller School of Medicine, Department of Dermatology, Miami, FL 33136, USA;
authors.forename: Scott F
authors.initials: SF
authors.lastname: Lindsey
authors.forename: Brandon
authors.initials: B
authors.lastname: Reiders
authors.forename: Hilit F
authors.initials: HF
authors.lastname: Mechaber
keywords: Bean syndrome; blue rubber bleb nevus syndrome; oral mucous membranes; oral vascular malformation; sclerotherapy; venous malformation