Objective
To describe the occurrence of fatal laryngeal edema in patients with hereditary angioedema
due to C1 esterase inhibitor deficiency.
Patients and Methods
We describe 6 patients from various regions of Germany who died from laryngeal edema
within the last 10 years. Furthermore, we conducted a retrospective survey of 58 patients
with hereditary angioedema, originating from 46 affected families. The data were obtained
from the attending physicians and from the patients' relatives.
Results
Among the 6 reported patients, aged 9 to 78 years, hereditary angioedema had been
diagnosed in 3 and was undiagnosed in 3. None of them had an emergency cricothyrotomy
or received C1 inhibitor concentrate. The interval between onset of the laryngeal
edema and asphyxiation was 20 minutes in a 9-year-old boy, and in the other patients,
the interval was 1 to 14 hours (mean for all, 7 hours). The retrospective survey of
58 patients with hereditary angioedema revealed 23 deaths by asphyxiation (40%). The
average age of all 29 patients at the time of asphyxiation was 39 years.
Conclusion
Laryngeal edema in hereditary angioedema may be fatal. Most of the patients asphyxiated
between their 20th and 50th years of life, but asphyxiation can occur even in children.
The possibility that the first episode of laryngeal edema may be fatal must be emphasized
to the relatives, and attending physicians must have a high degree of awareness.
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© 2000 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
