Scoliosis in patients with Charcot-Marie-Tooth disease

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J Bone Joint Surg Am. 2007 Jul;89(7):1504-10.

Scoliosis in patients with Charcot-Marie-Tooth disease.

Karol LA, Elerson E.

Texas ish Rite Hospital for Children, 2222 Welborn Street,

Dallas, TX 75219, USA.

BACKGROUND: Scoliosis appears to occur in approximately one-third of

patients with Charcot-Marie-Tooth disease. Little is known about the

response of these curves to treatment. The purpose of this study was

to establish the prevalence of scoliosis in a large population of

children and adolescents with Charcot-Marie-Tooth disease, to

evaluate factors linked with curve progression, and to assess the

response to orthotic and surgical treatment.

METHODS: The medical records of 298 patients were retrospectively

reviewed. Radiographs were reviewed for patients identified as

having spinal deformity. The type, size, and progression of the

scoliotic curve were measured, and the effectiveness of bracing and

surgical treatment was assessed.

RESULTS: Forty-five patients with scoliosis associated with Charcot-

Marie-Tooth disease were identified. The average age at the

diagnosis of the spinal deformity was 12.9 years, and the average

curve magnitude at the time of diagnosis was 27.6 degrees. One-third

of the curves were left thoracic, and 49% were associated with

increased thoracic kyphosis. Twenty-four of the thirty-four curves

that were followed for more than one year progressed. Brace

treatment was successful in only three of sixteen patients. Surgery

was performed in fourteen of the forty-five patients. Long posterior

spinal fusions were performed most often, with an average of 13.1

spinal segments fused. Instrumentation was used in all posterior

fusions. Intraoperative neurologic monitoring was possible for only

three of the twelve patients for whom it was attempted during

surgery, but there were no intraoperative neurologic complications.

CONCLUSIONS: Scoliosis in patients with Charcot-Marie-Tooth disease

differs from that in patients with idiopathic scoliosis. Thoracic

hyperkyphosis is common, and bracing is usually unsuccessful.

Surgical fusion does not appear to be associated with a high rate of

complications, although it is often impossible to perform

intraoperative neurologic monitoring.