1/17. Antenatal and preoperative genetic and clinical assessment in myotonic dystrophy.The antenatal investigation of an obstetric patient with a history of myotonia is described. The smooth and striated muscle dysfunction in myotonic dystrophy renders these patients, as a group, liable to surgical correction and exposure to anaesthesia. A caesarean section is reported to illustrate the preferred timing of diagnosis and peripartum management. While regional anaesthesia is preferred, myotonic dystrophy is not a contraindication to general anaesthesia, provided risks are anticipated and steps taken to minimize complications.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/17. Anaesthetic management of a patient with myotonic dystrophy.A 13-year-old boy with myotonic dystrophy underwent insertion of a percutaneous gastrostomy feeding tube under general anaesthesia. We used a laryngeal mask airway and a spontaneously breathing technique with propofol total intravenous anaesthesia. Postoperative vomiting and aspiration, 12 h after the procedure, subsequently required intubation and ventilation. We discuss the anaesthetic management of this case and review the features of the disease to be considered when contemplating anaesthesia in such patients.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
3/17. A combined technique utilising regional anaesthesia and target-controlled sedation in a patient with myotonic dystrophy.myotonic dystrophy presents several problems to the anaesthetist. We describe what we believe to be the first report of target-controlled sedation combined with regional anaesthesia in a patient with myotonic dystrophy. Precise control of propofol levels and titration to patient satisfaction avoided the problem of delayed recovery which has been described with propofol anaesthesia.- - - - - - - - - - ranking = 2keywords = anaesthesia (Clic here for more details about this article) |
4/17. Effective doses of vecuronium in a patient with myotonic dystrophy.Of the forms of muscular dystrophy, myotonic dystrophy has the greatest systemic involvement. Although most patients with myotonic dystrophy show normal sensitivity to non-depolarising neuromuscular blocking drugs, some have been reported to show greatly increased sensitivity to these drugs, and little is known about the sensitivity of different muscles. We compared effective doses of vecuronium in a patient with myotonic dystrophy at the orbicularis oculi, adductor pollicis and flexor hallucis brevis muscles during total intravenous anaesthesia. The calculated ED50 for the orbicularis oculi (7.77 microg x kg(-1) (95% CI 3.10-16.8 microg x kg(-1))) was lower than for the adductor pollicis (25.3 microg x kg(-1) (95% CI 20.7-43.3 microg x kg(-1))) and flexor hallucis brevis muscles (29.5 microg x kg(-1) (95% CI 11.0-85.6 microg x kg(-1); p < 0.01)). The ED90 was also lower for the orbicularis oculi (35.7 microg x kg(-1) (95% CI 14.8-66.5 microg x kg(-1))) than for the other muscles (51.8 microg x kg(-1) (95% CI 29.3-145.0 microg x kg(-1)) and 50.6 microg x kg(-1) (95% CI 5.29-642.0 microg x kg(-1)), respectively) (p < 0.01)).- - - - - - - - - - ranking = 0.33333333333333keywords = anaesthesia (Clic here for more details about this article) |
5/17. scoliosis in Steinert syndrome: a case report.BACKGROUND CONTEXT: Steinert syndrome is described as an autosomal dominant condition characterized by progressive muscular wasting, myotonia, musculoskeletal manifestations and rare spinal defects. Little is reported about spinal deformity associated with this syndrome. PURPOSE: We present a patient with Steinert syndrome complicated by scoliosis. In the literature on muscular dystrophy, other than Duchenne, little mention is given to the problem of scoliosis in general and its treatment in particular. STUDY DESIGN: A case report of a patient with Steinert syndrome associated with thoracic scoliosis and hypokyphosis is presented. methods: A 17-year-old boy presented with King type II right thoracic scoliosis (T5-T11, Cobb angle of 40 degrees) and hypokyphosis--10 degrees. He was treated with posterior stabilization and instrumentation at level T3-L2 with a postoperative correction of the scoliotic curve to 20 degrees. Histopathologic examination of the muscles confirmed the diagnosis of Steinert myotonic dystrophy. RESULTS: At 30-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. CONCLUSIONS: scoliosis in Steinert syndrome shares the characteristic of an arthrogrypotic neuromuscular curve and demands the extensive soft tissue release for optimal surgical correction. Intraoperative observations included profound tissue bleeding, abnormally tough soft tissues and a difficult recovery from anaesthesia.- - - - - - - - - - ranking = 0.33333333333333keywords = anaesthesia (Clic here for more details about this article) |
6/17. Obstetric anaesthesia in dystrophia myotonica.Two patients with dystrophia myotonica presented for urgent Caesarean section. Their per- and postoperative courses illustrate the anaesthetic problems posed by this disease. Respiratory difficulties are compounded by pregnancy and there is increased susceptibility to uterine haemorrhage. Choice of anaesthetic agent is discussed. Both had general anaesthetics; muscle relaxation was achieved with vecuronium.- - - - - - - - - - ranking = 1.3333333333333keywords = anaesthesia (Clic here for more details about this article) |
7/17. Cardiac anaesthesia in a patient with myotonic dystrophy.We describe a patient with myotonic dystrophy who required open-heart surgery for an atrial septal defect. He also had a sick sinus syndrome and an abnormal myocardium on histological examination. Anaesthesia using fentanyl, droperidol, nitrous oxide and a low concentration of enflurane was uneventful. Atelectasis of the left lung developed on the first postoperative day after removal of the tracheal tube. This was successfully treated by fibreoptic bronchoscopy.- - - - - - - - - - ranking = 1.3333333333333keywords = anaesthesia (Clic here for more details about this article) |
8/17. Anesthetic management of a parturient with myotonia dystrophica: a case report.We report the case of a 22-year-old parturient with myotonia dystrophica. She underwent two separate intraabdominal surgical procedures in one day, both under lumbar epidural anaesthesia. Management was directed toward prevention of shivering, a known trigger of myotonic crises. Measures used included warm ambient atmosphere, warmed IV fluids, warming blankets and administration of epidural sufentanil, an opioid recently ascribed as an inhibitor of shivering in parturients.- - - - - - - - - - ranking = 0.33333333333333keywords = anaesthesia (Clic here for more details about this article) |
9/17. Continuous infusion of propofol in dystrophia myotonica.The use of intravenous infusions of propofol and atracurium in a 41-year-old woman, weighing 64 kg, with dystrophia myotonica during major oral surgery is described. propofol and atracurium, in total doses of 2488 mg and 75 mg respectively, were used during the four-hour procedure. There were no intraoperative problems and operating conditions were excellent. Emergence was rapid and there were no postoperative complications. This technique offers a safe alternative to inhalational anaesthesia for patients with dystrophia myotonica.- - - - - - - - - - ranking = 0.33333333333333keywords = anaesthesia (Clic here for more details about this article) |
10/17. postoperative complications of dystrophia myotonica.A 44-year-old man with known mitral stenosis presented for minor surgery. Anaesthesia was induced with fentanyl, droperidol and etomidate and maintained with nitrous oxide, oxygen and vecuronium. Surgery and anaesthesia were uneventful but the postoperative course was stormy, with respiratory arrest, haemophilus pneumonia, refractory cardiac dysrhythmias and gastrointestinal atony. A diagnosis of dystrophia myotonica was made after 3 weeks in the intensive care unit, and he spent 6 weeks in hospital. A high index of suspicion for this disease must be maintained and intensive care facilities and monitoring should be available for all procedures.- - - - - - - - - - ranking = 0.33333333333333keywords = anaesthesia (Clic here for more details about this article) |
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