Scoliosis in Proteus syndrome: case report.

STUDY

DESIGN

The case of patient with scoliosis based on a rare hamartomatous, Proteus syndrome, is reported.

OBJECTIVES

To present the characteristics of scoliosis associated with Proteus syndrome, and to investigate the mechanisms that cause it.

SUMMARY OF BACKGROUND DATA: Proteus syndrome, a rare hamartomatous disorder first coined by Wiedemann, manifests many clinical morphologic abnormalities including scoliosis. The characteristics and cause of scoliosis in this syndrome are fully unknown.

METHODS

A patient with Proteus syndrome was followed from the age of 3 months to the age of 21 years. This patient received spinal corrective surgery for severe scoliosis. Detailed investigations of the scoliosis as well as the physical and imaging examinations were performed to characterize the scoliosis.

RESULTS

Computed tomography showed exclusive asymmetric appearance of lumbar spine, hypertrophy of the only right facet joints, and pedicles at L1-L4, which accorded with the right-side hemihypertrophy of the patient's extremities.

CONCLUSIONS

Scoliosis with Proteus syndrome seems to be based on hemihypertrophy, with no influence of mechanical stress.