Can inhalation agents be used in the presence of a child with myopathy?

PURPOSE OF REVIEW: Anaesthesia for a child with a muscle disease is always challenging because there is a risk of malignant hyperthermia, rhabdomyolysis or hypermetabolic reaction if a halogenated agent is used. Ongoing progress in genetics helps in clarifying the link between malignant hyperthermia (a calcium channelopathy) and muscle diseases. RECENT FINDINGS: We provide a summary of the most recent clinical, pathophysiological and genetic information on those risks when the diagnosis is known or suspected preoperatively. Some simple clues are also given to help make a decision in the presence of an infant or child with hypotonia or motor delay but no diagnosis. SUMMARY: Only a few muscle diseases are really associated with a risk of malignant hyperthermia. The risk of rhabdomyolysis is more difficult to clarify and a multicentric database would be useful to evaluate the risk/benefit ratio of all anaesthetic drugs in patients with muscle diseases.

Mortality and morbidity associated with correction of severe cervical hyperextension.

STUDY DESIGN: Three case reports of patients with treatment of severe cervical hyperextension. OBJECTIVE: Cervical hyperextension is a rare spine deformity that is associated with myopathies. Previous reports of surgical correction have reported no major operative complications. This report outlines our experience with 3 patients who experienced significant complications. SUMMARY OF BACKGROUND DATA: The limited literature on the treatment of cervical hyperextension has good to excellent outcomes. METHODS: Three case reports are presented. RESULTS: Three cases with severe cervical hyperextension with intraoperative correction had associated morbidity and mortality. One case had a failed intubation requiring tracheotomy. This was followed by a successful posterior release with halo traction for 2 weeks and then an instrumented posterior cervical fusion. This patient died at home 2 weeks after surgery. The second and third cases had an intraoperative spinal cord injury during a posterior release for cervical hyperextension. CONCLUSION: Patients with severe cervical hyperextension have high neurologic perioperative risk.

A case report of severe kyphoscoliosis and autofusion of the posterior elements in two siblings with central core disease.

STUDY DESIGN: A case report of two siblings (ages 14 and 17 years) with central core disease and prior malignant hyperthermia successfully treated with spinal fusion surgery for severe kyphoscoliosis. OBJECTIVES: Our objectives were as follows: to describe the previously unreported findings of posterior element autofusion and ligamentum flavum ossification; to increase surgeon awareness to the nature of this condition and associated findings; and to prepare the surgeons for the possibility of autofusion and the required surgical modifications, including extensive osteotomies at the time of spinal fusion surgery to achieve correction based on these findings. SUMMARY OF BACKGROUND DATA: Central core disease is a rare congenital myopathy with a reported association with kyphoscoliosis. Spinal deformity of this severity in central core disease has not previously been reported in the literature. METHODS: Two siblings with central core disease, history of malignant hyperthermia, and severe kyphosing scoliosis (187 degrees and 108 degrees) underwent correction of deformity and spinal fusion surgery. The clinical, operative, and radiographic features are detailed. RESULTS: The spinal deformities associated with central core disease in these 2 cases were severe. The posterior elements underwent autofusion necessitating alteration in surgical technique to correct the deformity. Despite the risks of malignant hyperthermia and the difficulty of surgical correction, good clinical improvements can be achieved even in cases of severe deformity. CONCLUSIONS: A diagnosis of central core disease must be considered in patients presenting with severe spinal deformity and myopathic symptoms. This spinal deformity may be progressive and become severe. Surgical intervention in these cases may be complicated by posterior element autofusion necessitating alteration in surgical technique to correct the deformity. Despite the risk of malignant hyperthermia, surgery may be performed safely.