Vertical expandable prosthetic titanium rib as treatment of thoracic insufficiency syndrome in spondylocostal dysplasia.
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.


METHODS
Therapeutic study, Level IV, (case series, no comparison group).
Ramirez
Norman
N
Hospital De La Concepción, San Germán, Puerto Rico. normanpipe@aol.com
Flynn
John M
JM
Emans
John B
JB
Betz
Randal
R
Smith
John T
JT
Price
Nigel
N
St Hilaire
Tricia
T
Joshi
Ajeya P
AP
Campbell
Robert M
RM
D000015:Abnormalities, Multiple / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D010100:Oxygen / Q000378:metabolism; D019736:Prostheses and Implants; D011474:Prosthesis Design; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D012272:Ribs / Q000002:abnormalities / Q000601:surgery*; D013577:Syndrome; D014025:Titanium; D016896:Treatment Outcome
D014025:Titanium; D010100:Oxygen