Pediatric emergency medicine trisk 1679 1679
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Potential triggers include direct mechanical friction or trauma as well as viral
infections, and thus affected children should avoid contact sports and mitigate
exposure to ill children (i.e., early daycare). The transition to puberty may
increase the frequency, severity, and duration of acute episodes. Other reported
triggers include stress, dental manipulation, and medications including estrogen
therapies and angiotensin-converting enzyme inhibitors.
Goals of Treatment
Treating acute attacks of swelling includes rapid, controlled airway management
in cases of laryngeal edema, and prompt administration of C1-esterase inhibitor
concentrate (or alternative therapy) to reduce morbidity and mortality.
Clinical Considerations
Clinical Recognition
Diagnosis of hereditary angioedema early in life is critical but often delayed. A
complete history and physical examination should be performed including a
detailed assessment of the airway, posterior pharynx, and abdomen. Abdominal
ultrasonography may be useful in discriminating angioedema from a surgical
process. Ultrasound findings consistent with an acute attack include bowel
hyperemia and ascites. Patients may report a prodrome of paresthesias at the
location of developing edema. Episodes are self-limited, but may last for 2 to 4
days.
Laboratory Assessment
The diagnosis is suspected with normal C3 and low C4 levels secondary to
increased conversion of C4 to C1. Functional and antigenic C1-inhibitor levels
should be obtained but may be falsely low in children younger than 2 years of
age.
Management
Initial management of acute attacks includes airway evaluation and management,
fluid resuscitation, and pain control. Intubation should be considered for patients
with dysphagia, stridor, voice changes, or dyspnea. Because of the potential for
significant airway edema with landmark distortion, laryngoscopy should ideally
be performed in a setting equipped to perform a surgical airway as indicated.
Unfortunately, unlike anaphylaxis, epinephrine (IM or nebulized), corticosteroids,
and antihistamines are usually ineffective in the management of hereditary
angioedema. Therefore, use of these therapies should never precede definitive
airway management.
