Abstract

The authors present what appears to be the first case of congenital kyphosis due to a T12 hemivertebra in a four-year-old boy with endochondral gigantism syndrome of unknown origin. Because of his overgrowth, the patient had severe medical and orthopaedic problems and was almost immobile. Prior to surgery, he experienced a rapidly progressive thoracolumbar kyphosis to 60° (T10-L2). MRI of the brain and spine showed critical protraction of the spinal cord and myelopathy from compression at T12. Single-stage posterior resection of the hemivertebra with spinal shortening and dorsal transpedicular instrumentation of T10-L2 was performed. Although the bone tissue was cartilaginous and dysplastic, 42° (30%) correction was achieved along with decompression of the spinal canal. The patient experienced no neurological impairment post-operatively. At follow-up examination 1.5 year after surgery, the patient's movement disorder had improved markedly and he was able to stand and walk. This very rare case demonstrates that single-stage posterior hemivertebra resection and transpedicular instrumentation for correction of congenital kyphosis can be a safe and effective procedure even in a very challenging case.