Study design: Prospective clinical trial of vertical expandable prosthetic titanium rib (VEPTR) in patients with combined spine and chest wall deformity with scoliosis and fused ribs.
Objective: Report the efficacy and safety of expansion thoracostomy and VEPTR surgery in the treatment of thoracic insufficiency syndrome (TIS) associated with fused ribs.
Summary of background data: Traditional attitudes toward early-onset combined chest and spine deformity assume that thoracic deformity is best controlled by treatment directed at spine deformity, often involving early spinal arthrodesis. Campbell and others have heightened awareness of the interrelationship between lung, chest, and spine development during growth and characterized TIS as the inability of the thorax to support normal respiration or lung growth. Expansion thoracostomy and VEPTR insertion was developed to directly control both spine and chest wall deformity during growth, while permitting continued vertebral column and chest growth at an early stage.
Methods: Multidisciplinary evaluation of children with combined spine and chest wall deformity included pediatric pulmonologist, thoracic, and orthopedic surgeon evaluations. One or more opening wedge expansion thoracostomies and placement of VEPTR devices were performed as described by Campbell, with repeated device lengthenings during growth. Parameters measured included Cobb angle, length of thoracic spine, CT-derived lung volumes, and in older children pulmonary function tests.
Results: Thirty-one patients with fused ribs and TIS were treated, 4 of whom had undergone prior spinal arthrodesis at other institutions with continued progression of deformity. In 30 patients, the spinal deformity was controlled and growth continued in the thoracic spine during treatment at rates similar to normals. Increased volume of the constricted hemithorax and total lung volumes obtained during expansion thoracostomy were maintained at follow-up. Complications included device migration, infection, and brachial plexus palsy.
Conclusions: Expansion thoracostomy and VEPTR insertion with serial lengthening may be the preferred treatment for young children with chest wall deformity and scoliosis associated with fused ribs but requires multidisciplinary care and attention to details of soft tissue management. When indicated, surgical intervention with VEPTR can be considered early in growth, before deformity is severe, since spinal growth will continue with treatment.