Background: Traditional surgical management of multiple congenital vertebral anomalies in young children, including fusion in situ and hemiepiphyseodesis, do not promote spinal growth nor address the associated thoracic insufficiency syndrome. We hypothesize that vertical expandable prosthetic titanium rib (VEPTR) with expansion thoracoplasty may control spinal deformity, allow spinal growth, and address thoracic insufficiency syndrome in children with nonsyndromic complex congenital spinal deformities.
Methods: Eight pediatric spine centers prospectively entered clinical and radiographic data into a database on every congenital spinal deformity treated with VEPTR as part of an Food and Drug Administration study. We retrospectively reviewed these data and excluded patients with spina bifida, Jarcho-Levin, or other syndromes. Data analysis focused on surgical technique and expansion frequency, change in Cobb angle and thoracic heights, and adverse events for a consecutive series of patients with at least 2 years of follow-up.
Results: Twenty-four children with an average age at surgery of 3.3 years (range, 1.0 to 12.5 y) were treated with VEPTR insertion and expansion thoracostomy and were followed for an average of 40.7 months (range, 25 to 78 mo). Twenty-three (95.8%) had associated rib fusions. All patients had subsequent expansion surgery; 50% had 5 or more expansions. Twenty patients (83.3%) had an improvement in Cobb angle during treatment with an average improvement of 8.9 degrees. All had an increase in thoracic height, with a mean increase of 3.41 cm. The most common adverse events were device migration in 7 patients and infection or skin problems in 6 patients.
Conclusions: VEPTR insertion with expansion thoracoplasty represents a successful treatment paradigm for nonsyndromic congenital spinal deformities. We report multicenter data with midterm follow-up of children without syndromic diagnoses, in which the vast majority had an improvement in Cobb angle and thoracic height over the treatment period. Challenges include the demands of multiple procedures, skin problems, and device migration.
Level of Evidence: Level IV—prognostic study.
*Division of Orthopaedic Surgery, Children’s Hospital of Philadelphia
§Shriners Hospital for Children, Philadelphia
∥Children’s Hospital of Pittsburgh, Pittsburgh, PA
†Children’s Hospital Boston, Boston, MA
‡Primary Children’s Medical Center, Salt Lake City, UT
¶NYU Hospital for Joint Diseases, New York, NY
The authors declare no conflicts of interest.
Reprints: John M. Flynn, MD, Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Wood Building, 2nd Floor, Philadelphia, PA 19104. E-mail: firstname.lastname@example.org.