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Vertical Expandable Prosthetic Titanium Rib as Treatment of Thoracic Insufficiency Syndrome in Spondylocostal Dysplasia

Ramirez, Norman MD*; Flynn, John M. MD*; Emans, John B. MD; Betz, Randal MD; Smith, John T. MD§; Price, Nigel MD; St. Hilaire, Tricia BS; Joshi, Ajeya P. MD; Campbell, Robert M. MD**

Journal of Pediatric Orthopaedics: September 2010 - Volume 30 - Issue 6 - p 521–526
doi: 10.1097/BPO.0b013e3181e78e6c

Background Spondylocostal dysplasia (SCD) constitutes a heterogeneous patient group with multiple vertebral formations and segmentation defects of the entire spine, with asymmetric rib malformations. Respiratory failure has been reported in spondylocostal dysplasia secondary to thoracic insufficiency syndrome. The vertical expandable prosthetic titanium rib (VEPTR) reconstructs the chest wall to address the thoracic insufficiency seen in this patient population. The purpose of this study is to evaluate spinal deformity correction and respiratory function outcomes in a spondylocostal dysplasia population treated with VEPTR.

Methods A cohort of 20 patients with spondylocostal dysplasia and 2-year follow-up were evaluated from a multicenter IDE study of 214 patients who had surgery with the VEPTR device. Data collected included gender, nonskeletal malformations, age at surgery, number of procedures, estimated blood loss, length of stay, and surgical time. Clinical and radiographic parameters were collected, and respiratory function was assessed.

Results In 14 of 20 patients (70%), spinal deformity was controlled evidenced by a decrease of the initial Cobb coronal angle at last follow-up. Fourteen patients (70%) maintained their oxygen level throughout treatment. At preoperative and last evaluation, assisted ventilation rating (AVR) scores showed that 5 patients improved their level of ventilation and 14 patients maintained their AVR level at room air. One patient decreased his level from supplemental oxygen to night ventilation. Mean thoracic spinal length (growth) by year was 0.82 cm. No mortality occurred in this group of patients.

Conclusions VEPTR implantation in SCD allows continued thoracic spine growth while controlling progressive spine deformity. The improved AVR ratings after surgery suggest a beneficial effect on the natural history of TIS in this population. Mortality and complication rate seem acceptable in this high-risk population of SCD patients.

Level of Evidence Therapeutic study, Level IV, (case series, no comparison group).

*Hospital De La Concepción, San Germán, Puerto Rico

Orthopaedic Surgery, Children's Hospital, Boston, MA

Shriner's Hospital for Children

Chest Wall and Spine Deformity Study Group

**Children's Hospital of Philadelphia, Philadelphia, PA

§Department of Orthopaedics, University of Utah and Primary Children's Medical Center, Salt Lake City, UT

Chidren‘s Mercy Hospital, Kansas City, MO

San Antonio Orthopedic Group, San Antonio, TX

Supported by: the Chest Wall and Spine Deformity Study Group.

Reprints: Norman Ramirez, MD, Pediatric Orthopaedic Department, Hospital La Concepcion, Edificio San Vicente de Paul, Carr# 2 Km 173.4, Oficina 501-502, San German, Puerto Rico 00683. Email:

© 2010 Lippincott Williams & Wilkins, Inc.