Complications of anaesthesia in neuromuscular disorders

Despite recent research developments, Duchenne muscular dystrophy remains a fatal neuromuscular disease, affecting two to three boys in 10,000. It is an inherited X-linked recessive condition caused by a frame-shift mutation in the dystrophin gene at the Xp21.2 locus of the X chromosome1. Dystrophin is a large cell-membrane protein involved in calcium transport in the muscle cell. Boys with Duchenne muscular dystrophy have an absolute absence of dystrophin, leading to deterioration of the muscle cells and replacement with fibrofatty tissue2. This is in contrast to Becker muscular dystrophy, in which less disruptive mutations that do not result in a frame shift lead to production of variable amounts of a smaller, genetically abnormal dystrophin protein3,4.

Duchenne muscular dystrophy is highly suspected in boys who have a markedly elevated serum creatine phosphokinase level; in two-thirds of such patients, the diagnosis can be confirmed by genetic testing. Approximately two-thirds of affected patients have large deletions or duplications that can be detected with use of the multiplex polymerase chain reaction and Southern blot techniques5. Detection of point mutations in the remaining one-third of patients is more difficult. In patients without detectable mutations, muscle biopsy with dystrophin analysis is necessary for diagnosis6,7. Staining of muscle biopsy specimens with antidystrophin antibodies in patients with Duchenne muscular dystrophy reveals a complete lack of staining of sarcolemma, whereas specimens from patients with Becker muscular dystrophy do have enough dystrophin present so that partial staining of the sarcolemma is seen2.

The clinical course of weakness in patients with Duchenne muscular dystrophy is one of relentless progression. Death from pulmonary or cardiac compromise occurs in the second or third decade of life8.

The age of onset of scoliosis in boys with Duchenne …

Scoliosis in Patients with Duchenne Muscular Dystrophy

STUDY DESIGN Retrospective before-after comparison study. OBJECTIVE The primary aim of this study was to evaluate the effectiveness of a radiofrequency-based bipolar hemostatic sealer during surgical correction of adolescent idiopathic scoliosis (AIS). SUMMARY OF BACKGROUND DATA Spinal reconstructive surgery is commonly associated with excessive blood loss. Perioperative bleeding is of particular concern during correction of AIS, which often requires allogeneic or autologous transfusion. However, there are specific risks and limitations that often preclude the utilization of transfusions. Alternatives include the use of antifibrinolytic drugs and topical fibrin-based and thrombin-based agents, although safety and effectiveness are yet to be fully established. There is a clear need for assessing alternative methods of hemostasis. METHODS One hundred seventy-six AIS patients undergoing corrective spinal surgery were included in this study. Seventy-six consecutive patients were treated intraoperatively with a standard method of hemostasis consisting of hypotensive anesthesia, thrombin-soaked sponges, and intraoperative blood salvage (Control). Subsequently, an additional 100 consecutive patients were treated after the introduction of a bipolar sealer (Aquamantys 2.3 Bipolar Sealer, Salient Surgical Technologies, Portsmouth, NH). The outcomes of this study were estimated blood loss (total and per level) and transfusion rate. RESULTS Blood loss was reduced by 57% after the introduction of the bipolar sealer compared with the Control (bipolar sealer: 435±192 mL, Control: 1009±392 mL; P<0.001). There was a statistically significant difference between groups for blood loss per fusion level (bipolar sealer: 39±17 mL, Control: 95±33 mL; P<0.001). Five (6.6%) Control patients required blood transfusions versus none treated with the bipolar sealer (P=0.014). Complication rates were similar between the groups. CONCLUSIONS These findings suggest that the Aquamantys 2.3 bipolar sealer effectively supports hemostasis and reduces the need for transfusions during surgical correction of AIS.

Hemostasis with a bipolar sealer during surgical correction of adolescent idiopathic scoliosis.

Left ventricular assist device as destination therapy in cardiac end-stage dystrophinopathies: Midterm results.

The aim of this study was to report results of prophylactic spinal stabilization in patients with Duchenne muscular dystrophy. There is still debate regarding the ideal instrumentation. A prospective study of a consecutive series of 31 patients stabilized with the ISOLA system from D2 to S1 will be presented. The mean follow-up was 22 months (range, 1-60 months). The evaluation of the Cobb angle and pelvic obliquity revealed the following: 1) Cobb angle: preoperation, 48.6 degrees (range, 22-82 degrees); postoperation, 12.5 degrees (range, 0-30 degrees); follow-up, 12.5 degrees (range, 0-42 degrees); and 2) pelvic obliquity: preoperation, 18.2 degrees (range, 3-40 degrees); postoperation, 3.8 degrees (range, 0-13 degrees); follow-up, 5.1 degrees (range, 0-14 degrees). Spinal stabilization with the ISOLA system was found to be a suitable treatment for scoliosis owing to Duchenne muscular dystrophy. It should be carried out after loss of ambulation as soon as a progressive curve of more than 20 degrees is documented. The complication rate was found to be high.

Spinal stabilization in Duchenne muscular dystrophy: principles of treatment and record of 31 operative treated cases.

Platelet function deficiency in Duchenne muscular dystrophy.

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Platelet function deficiency in Duchenne muscular dystrophy.