Cj. Bogdan et al., AIRWAY-OBSTRUCTION IN HEMOPHILIA (FACTOR-VIII DEFICIENCY) - A 28-YEARINSTITUTIONAL REVIEW, The Laryngoscope, 104(7), 1994, pp. 789-794
Life-threatening airway compromise is rarely reported as a major compl
ication of coagulation disorders. However, before adequate factor repl
acement 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. Fift
een episodes of airway obstruction occurred. Additionally, 6 cases of
potential airway compromise and 5 cases of airway-endangering orophary
ngeal bleeding were identified. Tracheotomy was performed in 5 patient
s; 1 fatality occurred before modern replacement products were availab
le. Patients with this disorder have a 13% chance of some form of airw
ay-endangering event with an 8% chance that it will be immediately lif
e-threatening. Tracheotomy and subsequent decannulation are safe proce
dures in these patients.