A 19-year-old-male with no known past medical history, presents in progressive respiratory distress shortly after undergoing a tooth extraction about 6 hours prior to presentation. Symptoms began initially as “a lump in his throat” and difficulty swallowing, but eventually progressed to mild dyspnea after 1-2 hours. Denies FB or allergies. On arrival he appears anxious and is breathing at 18 breaths per minute with an O2 saturation of 100%. There is no stridor at presentation. There is no intra-oral, facial, or obvious neck swelling. There is no skin involvement or abdominal pain, nausea, vomiting or diarrhea. You suspect allergic reaction and give IM epi, IV steroids, benadryl and pepcid. ENT is consulted for scope.

His girlfriend then arrives and tells you that a few months ago patient had a “reaction to something” that caused his face and lips to swell, but never sought treatment. Meanwhile, the patient is getting worse. He now has stridor and a muffled voice, breathing at 24 breaths per minute.

Case Progression: you correctly conclude this patient requires immediate control of his airway.

  • Most experienced/available provider intubates. Consider awake intubation, if appropriate, but the set up takes about 10 minutes. Can the patient wait this long? Does your center have a fiber-optic in the ED and do you know how to use it? If  yes, use it.
  • Second most experienced/available provider at the neck. Double prep this patient for emergent cric. I would place this patient at either a CricCon 2 or 3 prior to intubation (reference: emcrit.org. See below).
  • Invite your friends. If in-house, have someone contact anesthesia for fiber-optic intubation and general surgery/ENT. If there’s time it’s best for the patient to have the most experienced provider perform the procedure in this scenario.
  • Never unnecessarily delay your airway. Sometimes you just have to go in. If the patient is progressing or decompensating and anesthesia/ENT are not immediately available, it falls on you.
Courtesy: Emcrit.org
Courtesy: Emcrit.org

Case Progression: You successfully perform RSI prior to consults arriving because your patient was crashing and you are a baller. Your colleague is at the neck, which was prepped prior with scalpel in hand at time of intubation. This patient is going to the ICU. But what happened to him? You remember seeing something like this while intubating.

Laryngeal attack in HA

Just then, his mother arrives and tells you Hereditary Angioedema runs in the family.

Hereditary Angioedema (HAE):

  • Recurrent episodes of angioedema as a result of a hereditary dysfunction/deficiency of C1 inhibitor protein in the complement system
  • Affects males/females and all races equally, approximately 40% will have first episode by age 15.
  • Angioedema usually is isolated to one system: skin, GI tract, or upper airway, but can involve a combination, and often changes from episode to episode.
  • HAE does not produce urticaria and pruritus, as in anaphylaxis
  • Triggers: physical (direct contact or trauma), medications (ACE inhibitors, estrogen-containing medications, Tamoxifen), hormonal changes in women, infection (H. pylori)
  • Angioedema is self-limiting, but can last 2-4 days without medical intervention
  • Prior to effective medical therapy, mortality was approximately 30%, almost entirely due to laryngeal involvement and subsequent airway compromise
Laryngeal HAE Attacks:
  • Typically associated with some intra-oral swelling (uvula, tongue, lips), but can occur in complete isolation
  • About 50% of patients with HAE will have laryngeal attack in their lifetime
  • Tooth extraction and oral surgery are common triggers
  • Symptoms typical progress over an average of 7 hours, but case reports exist of fulminant progression in minutes

He’s going to get some crazy recombinant C1 inhibitor. And you have some sprained ankles to see.

May 2024