What is the role of the VEPTR procedure in congenital spinal deformity

The vertical expandable prosthetic titanium rib instrumentation (VEPTR) was developed to treat the thoracic insufficiency syndrome (defined as the inability of the thorax to support normal respiration and lung growth) associated with fused ribs. Following thoracoplasty of the fused ribs, the device lengthens and expands the hypoplastic hemithorax. When used in congenital scoliosis associated with fused ribs, growth of the concave and convex sides of the spine, including growth through unilateral unsegmented bars, may occur in addition to hemithorax enlargement.

Key Points

1. The prognosis for a congenital spinal deformity depends on three factors: type of anomaly, patient age, and location of the defect.

2. A wide range of intraspinal and extraspinal anomalies is associated with congenital spinal deformities, and thorough workup for associated abnormalities is critical.

3. MRI of the spine is an integral part of the evaluation of a patient with congenital spinal deformity.

4. Orthoses have little effect on progression of congenital spinal deformities.

5. Early surgical intervention is advised for progressive congenital spinal deformities to balance spinal growth and avoid development of rigid deformity and secondary structural curvatures.

Websites

Classification of congenital scoliosis and kyphosis: http://www.medscape.com/viewarticle/707687 Congenital scoliosis: http://www.srs.org/professionals/education/congenital Congenital spinal deformity: http://members.medscape.com/article/1260442-overview

BiBLiOGRAPHY

1. Andrew T, Piggot H. Growth arrest for progressive scoliosis: Combined anterior and posterior fusion of the convexity. J Bone Joint Surg 1985;67B:193-7.

2. Campbell RM Jr, Smith MD, Mayes TC, et al. The effect of opening wedge thoracoplasty on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg 2004;86A:1659-74.

3. Campos MA, Fernandes P, Dolan LA, et al. Infantile thoracolumbar kyphosis secondary to lumbar hypoplasia. J Bone Joint Surg 2008;90A:1726-9.

4. Hedequist DJ. Instrumentation and fusion for congenital spine deformities. Spine 2009;34:1783-90.

5. Hughes LO, McCarthy RE, Glasier CM. Segmental spinal dysgenesis: A report of three cases. J Pediatr Orthop 1998;18(2):227-32.

6. Keller PM, Lindseth Re, DeRosa P. Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphyseodesis and hemiarthrodesis: A preliminary report. Spine 1994;19:1933-9.

7. Lazar RD, Hall JE. Simultaneous anterior and posterior hemivertebra excision. Clin Orthop Rel Res 1999;364:76-84.

8. McMaster MJ, Singh H. The surgical management of congenital kyphosis and kyphoscoliosis. Spine 2001;26:2146-54.

9. Ruf M, Jensen R, Letko L, et al. Hemivertebra resection and osteotomies in congenital spine deformity. Spine 2009;34:1791-9.

10. Terek RM, Wehner J, Lubicky JP. Crankshaft phenomenon in congenital scoliosis: A preliminary report. J Pediatr Orthop 1991;11:527-32.

11. Yazici M, Emans J. Fusionless instrumentation systems for congenital scoliosis. Expandable spinal rods and vertical expandable prosthetic titanium ribs in the management of congenital spine deformities in the growing child. Spine 2007;34:1800-7.

12. Zeller RD, Ghanem I, Dubousset J. The congenital dislocated spine. Spine 1996;21:1235-40.

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