2022 Volume 50 Issue 2 Pages 88-90
Myhre syndrome (MS) is a rare genetic connective tissue disorder first reported in 1981 ; its clinical characteristics include intellectual disability, growth deficiency, muscular pseudohypertrophy, hearing loss, restricted joint mobility, laryngotracheal stenosis, choanal stenosis, and facial deformities such as short palpebral fissures, mid-face hypoplasia, prognathism, and a narrow mouth. Here, we report a difficult tracheal intubation during the induction of general anesthesia in a patient with MS.
The patient was a 17-year-old female with MS (height, 132 cm ; weight, 38 kg) who was scheduled to undergo dental treatment under general anesthesia. No cardiopulmonary complications had been diagnosed. Midazolam was administered orally as a pre-anesthetic medication because of the patient’s intellectual disability. After the rapid induction of the general anesthesia, mask ventilation was easily performed. A tracheal tube was inserted via the right nostril with no resistance, allowing nasotracheal intubation. We then attempted to perform endotracheal intubation using video laryngoscopes (GLIDESCOPE®, McGRATHTM) but failed because of trismus, macroglossia, and difficulty with neck flexion. We finally succeeded in intubating the patient using a bronchial fiberscope. General anesthesia was maintained uneventfully, and no complications were observed during the postoperative period.
The clinical manifestations mentioned above should be considered when airway management during general anesthesia is required in patients with MS. Additionally, intubation trauma can reportedly cause or exacerbate airway stenosis due to progressive fibrosis. Elective surgeries should be avoided in patients with MS whenever possible ; if unavoidable, endotracheal intubation should be approached with great caution to avoid this situation.
