Myhre Syndrome (MS) and LAPS Syndrome are two related genetic disorders associated with mutations in SMAD4 gene. Interventions to the upper respiratory tract may trigger stenosis with obstruction. Intubation should be avoided if possible.
The patient often has a difficult airway because of:
(1) obesity
(2) short neck
(3) prognathism
(4) thick skin
Intervention
Complication
tracheal intubation
glottic and/or subglottic stenosis
nasal tracheal intubation
choanal stenosis with nasal obstruction
An intubation tube may need to be left in place to protect the airway.
Interventions to correct the stenosis are usually followed by recurrence of the stenosis, often soon after the procedure.
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