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

Spine
Anthony M Sestero, Joseph H Perra

Abstract

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. 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. 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. 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. The spinal deformities associated with central core disease in these 2 cases were severe. The posterior elements underwent autofusion n...Continue Reading

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Related Concepts

Teens
Tumoral Calcinosis
Kyphosis Deformity of Spine
Malignant Hyperpyrexia due to Anesthesia
Scoliosis, Unspecified
Spondylosyndesis
Bone Structure of Spine
Structure of Ligamentum Flavum
Central Core Myopathy (Disorder)
Facet Joint Structure

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