Scoliosis in Patients with Charcot-Marie-Tooth Disease

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The Journal of Bone and Joint Surgery (American). 2007;89:1504-1510.

doi:10.2106/JBJS.F.01161 2007

Scoliosis in Patients with Charcot-Marie-Tooth Disease

http://www.ejbjs.org/cgi/content/abstract/89/7/1504

Lori A. Karol, MD1 and Elerson, RN1

1 Texas ish Rite Hospital, 2222 Welborn Street, Dallas, TX

75219.

Investigation performed at the Texas ish Rite Hospital for

Children, Dallas, Texas

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°. 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.