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1/4. Total intravenous anaesthesia without muscle relaxants in a child with diagnosed Duchenne muscular dystrophy.

    The case of a 3 year old child, affected by Duchenne muscular dystrophy, who underwent adenoidectomy and bilateral myringotomy, is reported. Total intravenous anaesthesia (propofol 1% infusion (160 micrograms kg-1min-1) and remifentanil (0.55 microgram kg-1min-1) without any muscle relaxants was used. The postoperative period was uneventful.
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keywords = anaesthesia
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2/4. Acute heart failure during spinal surgery in a boy with Duchenne muscular dystrophy.

    patients with Duchenne muscular dystrophy (DMD) are at high risk of perioperative complications. DMD may be accompanied by heart failure resulting from dystrophic involvement of the myocardium, which can be subclinical in the early stages of the disease. This case demonstrates that a normal preoperative ECG and echocardiograph cannot exclude the development of heart failure during anaesthesia in DMD patients undergoing major surgery.
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keywords = anaesthesia
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3/4. Tracheocoele in a Duchenne muscular dystrophy patient. Case report.

    Tracheocoele, a congenital or acquired lesion, is rarely detected radiologically and even more rarely diagnosed clinically. This tracheal lesion is characterised by the presence of a single cystic lesion filled with air or a mixture of liquid and air, of extremely variable size, occurring in almost all cases, in a locus minoris resistentiae situated in the right posterolateral portion of the trachea. The rare case is described of a voluminous tracheocoele located in the left paratracheal region, extending from the cricoid to sternal notch, manifesting clinically, 3 months prior to evaluation in our hospital in a 27-year-old male suffering from Duchenne muscular dystrophy since the age of 5 years. For 10 years, the patient had been treated with intermittent positive pressure ventilation via nasal mask, due to progressive deterioration of respiratory function. diagnosis of tracheocoele, initially made at computed axial tomography scan, was confirmed by flexible laryngotracheoscopy under local anaesthesia. Due to severe comorbidity associated with the clinical picture described, the absence of a significant set of symptoms, and the problems concerning anaesthesiological management of the patient, palliative treatment was the only choice. This consisted in cervical compression bandaging during assisted nasal ventilation. Close follow-up was performed in order to monitor any progression of the lesion or onset of related complications. This is the second case of tracheocoele originating in the left paratracheal region reported in the literature, and the first associated with Duchenne muscular dystrophy and prolonged use of a positive pressure respirator. The aetiopathogenic mechanisms that may have determined the formation of this rare lesion are then taken into consideration.
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ranking = 0.2
keywords = anaesthesia
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4/4. Anaesthetic management during labour of a manifesting carrier of Duchenne muscular dystrophy.

    We describe the peripartum anaesthetic management of a 36-year-old woman who was a manifesting carrier of Duchenne muscular dystrophy. Duchenne muscular dystrophy is an X-linked recessive disorder affecting young males associated with severe complications during anaesthesia if depolarising neuromuscular blocking drugs and volatile agents are used. A manifesting carrier is a heterozygous female who demonstrates the disease in a milder form than in males. This probably occurs because of skewed X-inactivation. We planned to establish regional anaesthesia should an operation be necessary during labour or delivery and to use propofol total intravenous anaesthesia and rocuronium if general anaesthesia became unavoidable. At 37 weeks, the woman went into spontaneous labour, but fetal distress necessitated caesarean section for which combined spinal-epidural anaesthesia was used.
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keywords = anaesthesia
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