Cervical myelopathy in a child with Sprengel shoulder and Klippel-Feil syndrome.

Abstract

Sprengel shoulder, Klippel-Feil syndrome and congenital scoliosis are associated conditions. Cervical myelopathy in a child due to a concomitant omovertebral bar causing posterior cord compression and a hypermobile cervical disc protrusion adjacent to fused cervical segments causing anterior compression at the same level is very rare. We report the presentation, findings, surgical management and results of treatment in such a child. A 9-year-old girl with Sprengel shoulder presented with cervical myelopathy (Frankel D). Imaging revealed a bony omovertebral bar connected to the left scapula compressing the spinal cord posteriorly through a lamina defect at C5 resulting in significant cervical stenosis. A hypermobile disc protrusion adjacent to congenitally fused segments resulted in anterior compression at the same level. She was treated surgically with cervical laminectomy and instrumented fusion, excision of the omovertebral bar and modified Woodward procedure for the left Sprengel shoulder. At 2year follow-up, she had improved neurologically (Frankel E) and there was improved shoulder symmetry and abduction. MRI showed resolution of cervical stenosis. Although there was no significant progression of congenital scoliosis, it will need to be monitored. Cervical myelopathy due to an omovertebral bar and cervical disc protrusion in a child with Klippel-Feil syndrome and Sprengel shoulder is a rare presentation and can be treated with a single posterior approach addressing both pathologies.