Pycnodysostosis is autosonal recessive inheritance. It feature makes low height, sluggish growth of body, short finger, flat fingernails, and easy fracture, etc. We experienced general anesthesia of the patient who complicated mandibular osteomyelitis and pathological fracture to Pycnodysostosis patient. Patient: 40 years old and male. (
Fig. 1-6) Pathological fracture was pointed out by image inspection. Clinical diagnosis: Right mandibular osteomyelitis and mandibular pathological fracture. Operation proposed: Resection of low jaw, and re constructive operation by plate. This time, when general anesthesia was done to Pycnodysostosis, we should have examined it. The danger of airway obstruction is pointed out in Pycnodysostosis. The anatomical features of down the uvula, tongue subsidence, and mandibular retreat, etc. is given as a cause. Therefore, the possibility of easily falling into hypoxemia and hypercapnemia is reported. It is possible for the pulmonary vascular resistance to increase if hypoxemia continues for a long time, and to cause pulmonary hypertention and the right heart failure. Therefore, it is thought that upper airway management of after operation is necessary. NLA and Propofol is selected intravenous anesthesia. (
Fig. 7) There is a possibility of the fracture by backing etc. Extubation is done at the early stage of after operation and we ventilate with mask. There is a report that you should awake after that. However, we thought that upper airway management was important. And, we judged that it was safer to do extubation after it had awoken enough.
We experienced Pycnodysostosis patient's general anesthesia. The necessity for doing the anesthesia preparation of understood the feature to manage this disease anesthesia before operation, and assumed all situations was suggested.
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