Airway obstruction in hemophilia (factor VIII deficiency): a 28-year institutional review

Life-threatening airway compromise is rarely reported as a major complication of coagulation disorders. However, before adequate factor-replacement therapy became available, this complication was often fatal. A retrospective review of all patients with classic hemophilia admitted to our institution from 1964 through 1992 was performed. The records of 147 patients who had a total of 1804 admissions were examined. Fifteen episodes of airway obstruction occurred. Additionally, 6 cases of potential airway compromise and 5 cases of airway-endangering oropharyngeal bleeding were identified. Tracheotomy was performed in 5 patients; 1 fatality occurred before modern replacement products were available. Patients with this disorder have a 13% chance of some form of airway-endangering event with an 8% chance that it will be immediately life-threatening. Tracheotomy and subsequent decannulation are safe procedures in these patients.