[Anesthesia in a patient with Scheie syndrome].

Mucopolysaccharidosis (MPS) is a rare metabolic disease characterized by abnormal accumulation and excretion of mucopolysaccharides. Patients with MPS have many anesthetic problems including ischemic heart disease, valve insufficiency, difficult intubation, joint stiffness and mental retardation. We report a case of Scheie syndrome, a mild type of MPS, which presented a unique problem of difficult intubation. The patient was a 35 year old woman, scheduled for anterior fixation of the 4th lumbar vertebra. Her manifestations included low height, corneal clouding and systemic joint stiffness. Coronary artery disease was suspected from ECG. There was no mental retardation. Mouth opening was possible for about 4.5 cm. After induction with fentanyl, thiopental and vecuronium, intubation under laryngoscopy was tried but impossible because mouth opening was restricted to only 2 cm. Therefore muscle relaxant was reversed with neostigmine and atropine. Then under spontaneous respiration, she was intubated using a bronchofiberscope. It is difficult to explain why her mouth opening was more restricted after induction. We suspect mechanical change of temporomandibular joint or low compliance of the muscles. In conclusion, as reported previously, spontaneous respiration should be maintained until intubation. Especially in a patient with Scheie syndrome, whose mental development is normal, fiberoptic intubation should prove to be useful.