[Anesthetic management of a patient with Dyggve-Melchior-Clausen syndrome].

The Dyggve-Melchior-Clausen syndrome (DMCS) is a rare autosomal recessive skeletal dysplasia characterized by short-trunk dwarfism and mental retardation. A 49-year-old male with DMCS underwent resection arthroplasty for contracture of the right hip joint under general anesthesia using thiamylal, nitrous oxide, sevoflurane, and vecuronium. Although he was assumed to have difficult airway due to short neck, macroglossia, and disturbance of neck flexion, tracheal intubation was not difficult. No complications including malignant hyperthermia were observed during the 95 min of the operation.

[Perioperative management of a patient with systemic lupus erythematosus, myasthenia gravis, and pemphigus foliaceous].

A 38-year-old female with systemic lupus erythematosus (SLE), myasthenia gravis (MG), and pemphigus foliaceous (PF) was scheduled to undergo total hysterectomy and lymphadenectomy. Preanesthetic examination revealed anemia, a prolonged activated partial thromboplastin time, and a reduced percent vital capacity. Antiphospholipid antibody was not positive. After treating the bullous lesions of PF and the muscle weakness due to MG (noted on admission for surgery) with oral prednisolone, the patient was scheduled for surgery. To avoid the use of a muscle relaxant and the potential complications of the airway manipulation involved in using a laryngeal mask or endotracheal tube, since the patient had MG and PF, a regional anesthetic technique was selected. This involved continuous epidural anesthesia, achieved using 1% or 2% mepivacaine, with sedation by a combination of propofol infusion (3 mg.kg-1.hr-1) and nitrous oxide (60% in oxygen). The patient breathed spontaneously under the mask throughout the 3.5-hr operation. The intraoperative surgical and anesthetic course was uneventful. After a benign postoperative course, the patient was discharged on the 16th postoperative day.

[Anesthesia for a patient with pheochromocytoma associated with chronic renal failure].

A case of a 53-year-old man with left pheochromocytoma associated with chronic renal failure is described. He had been on regular hemodialysis for 15 years. Also, because of right renal carcinoma, he underwent right nephrectomy at the same time. For preoperative preparation, we kept the patient's dry weight. Anesthesia was induced with isoflurane 1.0% in oxygen, and fentanyl 0.3 mg and midazolam 5 mg, followed by tracheal intubation facilitated with vecuronium. Anesthesia was maintained with isoflurane 0.5% in oxygen 50-100%, fentanyl and midazolam. Intraoperative hypertension during the manipulation of the tumor and hypotension after removal of the tumor were controlled by continuous or bolus infusion of phentolamine and norepinephrine respectively. For intraoperative fluid management, we administered crystalloid solution 4 ml.kg-1.hr-1. Careful preoperative management was carried out to prevent severe intraoperative and postoperative cardiovascular or respiratory complications.