Two cases of Hunter's syndrome—The anaesthetic and operative difficulties in oral surgery

Narrow trachea in mucopolysaccharidoses

“…Hopkins, et al, reported an MPS II patient, age 20 years, who died because of respiratory complications after oral surgery for a hyperplastic coronoid process which had limited the patient's jaw motion. At autopsy, the trachea was small and flattened anteroposteriorly; microscopic findings were not given [5]. Murray reported a 43-year-old "Hurler" patient (by clinical description, the patient most likely had MPS II) who required a tracheostomy because of obstruction related to a "small, irregular larynx with thickened cords" [9].…”

Otolaryngological findings in mucopolysaccharidosis

“…[16][17][18] Recent studies from Germany and the Netherlands report an incidence of one case in 140,000-330,000 live births, and 1.3 cases per 100,000 male births. [19,20] The severe form of Hunter syndrome is typically diagnosed in children aged between 2-4 years. The attenuated form of Hunter syndrome may not be diagnosed until the teenage years or well into adulthood.…”

“…• The enlarged tongue, hypertrophic adenoids and tonsils, and skeletal changes in the jaw and neck that limit the opening of the mouth [7,20,21] all contribute to respiratory problems, as noted in "Respiratory System".…”

“…[7,20,21] • Respiratory obstruction is secondary to the accumulation of glycosaminoglycans in the cells of the trachea.…”

Anesthetic Management in Mucopolysaccharidoses

“…Postoperative edema is possible until 27 hours after anesthesia [71] with acute respiratory obstruction requiring urgent tracheotomy which could end in the death of the patient. Bredenkamp et al, in 1992, highlighted a significant upper airway obstruction in 17 out of 45 (38%) patients and 7 patients (16%) needed tracheotomy [72].…”