Corrección de escoliosis en adolescente con síndrome de espina dorsal rígida.(I)

Arkader A, Hosalkar H, Dormans JP.

Department of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4399, USA.

STUDY DESIGN: Details of presentation and approach to the treatment of scoliosis in a case of a Rigid Spine Syndrome (RSS).

OBJECTIVE: To report on the results of conservative and operative treatment of scoliosis associated with RSS, and, based on this, to propose an assessment and treatment protocol for this condition.

SUMMARY OF BACKGROUND DATA: Congenital muscular dystrophies (CMD) are a group of disorders marked by hypotonia at birth and a generally nonprogressive course of muscle weakness. Spinal rigidity is present in a number of patients with CMD. RSS is classified as a CMD, and is characterized by early rigidity of the spine, limb contractures, and restrictive respiratory dysfunction. An approach to the treatment of scoliosis in RSS has not been established.

METHODS: Details of history, diagnostic tests, and treatment of an adolescent with RSS associated with progressive scoliosis and cervical spine extension contracture is presented. The role of brace treatment, Botox (Allergan, Inc., Irvine, CA), and details of operative correction, including histology of back muscles, is defined.

RESULTS: In this case of RSS with rigid scoliosis nonresponsive to brace therapy and Botox injections, an anterior thoracolumbar spine fusion with instrumentation was successfully performed for correction of the scoliosis, and the cervical spine was addressed through a posterior approach and an occiput-cervical fusion. We outline the details of surgical procedure and restoration of spinal balance in both sagittal and coronal planes.

CONCLUSIONS: The chromosomal bases of CMD and characteristic features of RSS, including diagnostic tests, have been reviewed. Surgical intervention with spinal deformity fusion, correction, and instrumentation is indicated in RSS with progressive spinal imbalance not responding to conservative therapy is safe and can give promising results.