Masui. The Japanese journal of anesthesiology
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We report the case of a 52-year-old woman with May-Hegglin Anomaly (MHA) and a platelet count of 1.9 x 10(4) x mm(-3). She was scheduled to undergo abdominal total hysterectomy and adnexectomy with uterine fibroids. Anesthesia was maintained by inhalation of sevoflurane (1.5%), continuous intravenous infusion of remifentanil (0.2-0.3 μg x kg(-1) x min(-1)), intermittent intravenous infusion of fentanyl (35 μg), and the transversus abdominis plane block with 20 ml of 0.375% ropivacaine. ⋯ MHA is a rare hematological disorder inherited as an autosomal dominant trait that is characterized by thrombocytopenia, giant platelets, and inclusion bodies in the granulocytes. A decrease in platelet count is detectable with a blood test But function of platelets is generally normal. Anesthesiologist should understand the characteristics of MHA, and administer anesthetics without giving platelets.
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Airway management in a patient with Forestier's disease can be challenging clinically because this disease may cause not only dysphagia but also airway obstruction due to the compression of the pharynx and esophagus caused by the ossification of anterior longitudinal ligament. We report our anesthetic management in a patient with Forestier's disease. Meanwhile, we studied the causes of difficult airway and the most suitable airway device for a patient with this disease from a standpoint of anatomy of upper airway. Our study indicated the possibility that the most suitable airway device differed depending on the actual location of the ossification of anterior longitudinal ligament in the cervical spine and that more prudent airway management would be required if its lesion location extended to upper cervical spine.