Dexmedetomidine infusion for sedation in a patient with myotonic dystrophy.

Myotonic dystrophy type 1 is the most common muscular dystrophy in adults. Anaesthetic management should take into consideration the numerous body systems affected, including the musculoskeletal; respiratory; cardiovascular; gastro-intestinal; and central nervous systems. A 42-year-old man with myotonic dystrophy presented for septoplasty and bilateral inferior turbinate reductions. He had severe upper and lower extremity myotonia and weakness, pulmonary impairment with non-obstructive patterns and first-degree atrioventricular block with reduced ejection fraction. He used bilevel positive airway pressure, a cough assist device and was paced 3% of the time with a single-chamber pacemaker. To reduce potential complications associated with opioid use and general anaesthetics, an opioid-free technique was planned using local anaesthetic infiltration and sedation with a dexmedetomidine infusion. The patient maintained spontaneous ventilation and haemodynamic stability, and had an uneventful postoperative course. Dexmedetomidine is a highly selective α2-adrenergic receptor agonist that has the ability to provide sedation, analgesia and anxiolysis with a stable haemodynamic profile. Avoiding both opioids and general anaesthetics in these patients may decrease the risk of peri-operative complications.

Clinical experience of intracranial chordoma - A systematic review and meta-analysis of the literature.

Chordomas are rare neoplasms of the axial skeleton, with a predilection for the clival region. The lack of data about intracranial chordomas has led to inconsistent reporting of their clinical behavior and management principles. We performed a systematic review and meta-analysis with the aim of reporting on clinical behavior and management in the literature. We performed an electronic search of the MEDLINE, EMBASE, LILACS, Web of Science, Cochrane, and CINAHL databases. We included studies of patients with intracranial chordoma that had undergone surgical management with 5-year data available. The weighted mean estimate of 5-year OS was 77.4% (95% CI, 72.9-81.7%) whilst the 5-year PFS estimate was 49.4% (95% CI, 41.6-57.3%). A wide variety of surgical approaches were utilised, with transcranial approaches being the commonest. Surgeons achieved gross total resection in 29.4% of cases (95% CI, 17.6-43.0%), Sub-total resection in 60.9% of cases (95% CI, 40.2-79.8%) and partial resection in 19.7% (95% CI; 11.9 to 28.9%). The total complication rate was 28.5% (95% CI, 18.4-39.8%), with the commonest being new cranial nerve palsy (weighted mean 11.1%, 95% CI, 7.11-15.7%). Our review demonstrates that a wide range of management options exist in the literature. These should be tailored to each patient based on factors including tumoral location, local involvement and capabilities of the surgical team. The outcomes of chordoma management have largely been stable with no improvement despite improvements in modern surgical techniques.