Case 11 - Brachycephalic Syndrome

Answers and Discussion

Case #11 -- Brachycephalic Syndrome

Answers and Discussions to the Questions:

If "Brutus" was in severe respiratory distress, what emergency treatments would you institute?

First, supplement oxygen via a mask or oxygen cage. Sedation is warranted--a low dose of acepromazine will help to decrease anxiety and allow the patient to breethe more easily. If necessary, a temporary tracheostomy should be performed.

What are the 6 components of brachycephalic syndrome and how do you confirm their presence in the patient?

1. Stenotic nares--confirmed by lack of a visible opening into the nares. Alar fold (lateral flap of each opening)will collapse during inspiration.
2. Elongated soft palate--.confirmed under anesthesia or heavy sedation. It will cover the epiglottis.

3. Laryngeal edema--confirmed under anesthesia. The mucosa is glistening or gelatinous in appearance.

4. Laryngeal collapse--confirmed under anesthesia. There is usually a smaller opening to the larynx and the structures are collapsing medially to block the opening further.

5. Everted laryngeal saccules--confirmed under anesthesia. They can be seen ventrally in the glottic lumen.

6. Tracheal hypoplasia--confirmed on radiographs.

What surgical procedures do you use to correct these problems?

Stenotic nares can be corrected by removing a small wedge-shaped portion of the alar fold. An elongated soft palate can be surgically shortened (a normal one should just cover the tip of the epiglottis). For laryngeal collapse, it's best to resect the aryepiglottic fold unilaterally. Laryngeal tie-backs do not work well in these cases because the weakened cartilage still tends to collapse medially post surgery. Everted laryngeal saccules should be surgically removed. Tracheal hypoplasia can not be corrected.

Since surgery usually results in a large amount of swelling, what procedure might you do to ensure a patent airway and what drug might you administer to decrease edema?

A temporary tracheostomy is occasionally used to ensure a patent airway for 24-48 hours post surgery. Steroids (dexamethasone sodium phosphate 0.2-2.2 mg/kg IV) will help reduce laryngeal edema and swelling.

Follow-Up: "Brutus" was discharged 48 hours after surgery. On recheck one week later, he had no signs of dyspnea or exercise intolerance.

Brachycephalic Syndrome Pearls

For brachycephalic syndrome respiratory emergencies, treat with oxygen, light sedation and tracheostomy if necessary.
Confirm the diagnosis with radiographs and a thorough laryngeal exam under anesthesia.
Most components of this syndrome can be treated surgically and should be given corticosteroids and occasionally a temporary tracheostomy post-surgery.