The effect of mid-thoracic VEPTR opening wedge thoracostomy on cervical tilt associated with congenital thoracic scoliosis in patients with thoracic insufficiency syndrome

Abstract

Study design: Prospective clinical trial of vertical expandable prosthetic titanium rib (VEPTR) surgery in patients with congenital thoracic scoliosis with thoracic insufficiency syndrome and cervical tilt.

Objective: Report efficacy and safety of VEPTR mid-thoracic opening wedge thoracostomy in treatment of cervical tilt and head/truncal decompensation in children with thoracic insufficiency syndrome associated with thoracic congenital scoliosis.

Summary of background data: Cervical tilt in children is a rare problem, usually associated with congenital cervical or cervical-thoracic scoliosis, and in progressive deformity, surgical fusion or hemi-vertebrectomy risks neurologic injury, loss of motion of the cervical spine, and unknown effect on pulmonary function. VEPTR patients with congenital scoliosis can have severe neck tilt with poor cosmesis, with the risk of neck pain in adulthood. VEPTR mid-thoracic opening wedge thoracostomy treats thoracic insufficiency syndrome, and paradoxically also appears to improve the neck alignment cosmesis.

Materials methods: Patients with fused ribs and congenital scoliosis, thoracic insufficiency syndrome, and cervical tilt were treated with VEPTR mid-thoracic thoracostomy. Complication rates, respiratory outcome, pulmonary function tests in older children, Cobb angles, head/truncal decompensation, T1 oblique take-off, space available for lung, height of the thoracic spine, and cervical tilt angle were measured.

Results: Fourteen patients with cervical tilt, fused ribs, progressive congenital scoliosis, and TIS were treated. The primary thoracic scoliosis and space available for lung improved, cervical tilt stabilized, and head and truncal decompensation improved. One child was weaned off a ventilator. Complications included device migration, infection, and transient brachial palsy.

Conclusion: Mid-thoracic VEPTR opening wedge thoracostomy can stabilize cervical tilt associated with thoracic congenital scoliosis and fused ribs and may be an alternative to cervicothoracic spine fusion or hemi-vertebrectomy in some young children.