The Effect of Expansion Thoracoplasty on Thoracic Insufficiency Syndrome Associated with Spondylocostal Dysostosis (Variant of Jarcho-Levin Syndrome)**

• a – NORD, FDA Orpan Product Office; d - Synthes Spine Co.

Background: Severe foreshortening of the thorax due to congenital spinal deformity and rib anomalies in Spondylocostal dysostosis (Jarcho-Levin Syndrome variants) is associated with thoracic insufficiency syndrome. Deformity may be addressed by fusion, but spinal growth is subsequently inhibited with the risk of development of progressive thoracic insufficiency syndrome because of unclear effect on growth of the thorax and the underlying lungs. Treatment for this should correct deformity of the thoracic spine, while allowing growth in length by distracting the thorax bilaterally. With the vertical expandable titanium rib prosthesis (VEPTR) , it is possible to correct the congenital scoliosis in these patients with growth of the spine.

Method and Materials: Ten patients with spondylocostal dyostosis were treated with opening wedge thoracostomies with VEPTR. The implanted devices were periodically lengthened in out-patient surgery during follow-up.

Results: Seven were female and three male. Mean age at surgery was 3.3 yrs. Mean EBL was 65 cc’s for implantations. Nine patients had bilateral implants. Mean number of procedures per patient was 15. Mean f/u was 4.25 yrs ( 2- 7.25 yrs) Mean respiratory rate went from 31 to 26 per minute. Mean CBG pO2 was stable at 76 mm Hg. Mean pCO2 went from 35 to 39 mm Hg. Eight patients were on room air preop and stayed so at f/u. Two had respiratory insufficiency preop: one on full-time ventilator was weaned to oxygen support improved, the other stayed stable on oxygen. PFT’s: preop % nl FVC ( N=5) was a mean of 52% (35-73% ), post op 46% (32-54%), total f/u VC (N=7) 44% (34-54%) . Seven patients had scoliosis. Mean Cobb angle at preop was 27º, at f/u 16º. Kyphosis went from 19 º to 30º at f/u. SAL went from 80% to 90%. Mean T1-12 height was 9.5 cm at preop, at f/u 12.9 cm. Mean T1-12 length was 9.6 cm at preop, and 13.2 at f/u. Thoracic width went from 14.3 cm at preop to 17.7 cm. at f/u. Depth increased from 10.7 to 15.1 cm. IPLR was 1.3 at preop and 1.4 at f/u. Transverse deformity: TR went from 16º to 13º, SR from 14º to 9º, and PHTR from 1.7 to 1.6. Lung volume preop was 470cc, increasing to 672 cc. Lung density went from -547 HU to -580 HU. Complications: Six patients had no complications. Two had device migrations and one had rib fx. One had wound infection with three episodes of skin slough, and later expired of respiratory insufficiency with cor pulmonale.

Discussion and Conclusions: Congenital scolioisis in these patients is controlled with VEPTR treatment without the need of spine fusion with beneficial growth of the thoracic spine with increase in thoracic volume needed for lung growth. Additional long term follow-up will be needed to define treatment efficacy.

**The FDA has not cleared a drug and/or medical device the use described in this presentation (i.e., the drug or medical device is being discussed in an “off-label use).

• If noted the author indicates something of value received. The codes are identified as: a-research or institutional support; b-miscellaneous funding; c-stock or stock options; d-royalties; e-other financial or material support including consulting.