Growth Friendly Surgery is Effective at Treating Scoliosis Associated with Goldenhar Syndrome Braydon Connell, Jonathan Oore, Joshua Pahys, George Thompson,

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Growth Friendly Surgery is Effective at Treating Scoliosis Associated with Goldenhar Syndrome Braydon Connell, Jonathan Oore, Joshua Pahys, George Thompson, Tricia St. Hilaire, Tara Flynn, Ron El-Hawary, Growing Spine and Children’s Spine Study Groups

Introduction Goldenhar Syndrome or Oculoauriculovertebral Dysplasia Spectrum (OVAS) has been associated with the development of spinal deformity, which may or may not have associated vertebral anomalies. Spinal and rib anomalies abnormalities are most prevalent in the cervical and thoracic spine (Anderson P, & David D., 2005).

Purpose/Hypothesis The purpose is to evaluate the results of Growth Friendly (GF) surgical treatment in patients with Goldenhar Syndrome. The hypothesis is that scoliosis and spine height will improve for patients with Goldenhar Syndrome who undergo Growth Friendly treatment for early onset scoliosis.

Inclusion Criteria Patients consented to GSSG/CSSG database Diagnosed with Goldenhar syndrome at less than 10 years of age Growth Friendly surgical treatment Follow-up >2 years

Methods Scoliosis, kyphosis, spine height, and hemithoracic height/width were determined pre-implant, immediately post-op and at 2 year follow. Severity of complications (SV) were recorded (Smith et al. JPO 2015).

Results 10 patients Mean age of 4.6±2.5 year at implantation 1 Traditional Growing Rod, 1 Growing Rods (rib- based) and 8 Rib-Based Distraction. Mean age of 4.6±2.5 year at implantation Three groups were compared: Pre-operative Post-implantation Two year follow up

Results Pre-Implant Post-op 2 yrs f/u Cobb angle 64º 52.3º 50.2º Kyphosis 35.8 º 37.9 º 42.4 º T1-S1 Height 23.5 cm 23.6 cm 27.3 cm Right hemithoracic height 8.8 cm 9.6 cm 10.7 cm Left hemithoracic height 9.9 cm 10.2 cm 12.3 cm Right hemithoracic width 8.0 cm 7.2 cm 7.9 cm Left hemithoracic width 8.6 cm Complication Eight patients had ≥1 complication with SV I (n=7), SV II (n=2), and SV IIA (n=7). These included infection (n=4), migration (n=3), pneumonia (n=2), and instrumentation failure (n=2).

Results The difference approaches statistical significance (p=0.06)

Results The difference approaches statistical significance (p=0.07)

Results The difference approaches statistical significance (p=0.07)

Conclusion At two year follow up, Growth Friendly surgical intervention in treating EOS associated with OVAS trended towards improvements in scoliosis, spine height and hemithoracic height/width with the majority of patients experiencing severity grade I or II complications.

References Anderson, P. J., & David, D. J. (2005). Spinal anomalies in Goldenhar syndrome. The Cleft palate-craniofacial journal, 42(5), 477-480. El-Hawary, R., Chukwunyerenwa, C. Update on Evaluation and Treatment of Scoliosis. Pediatr Clin North Am 61, 1223-41 (2014). El-Hawary, R., Heflin, J., Joukhadar, N., Yasin, M., Skaggs, D. Superior Extension of Upper Instrumented Level in Distraction-Based Surgery: A Surrogate for Clinically Significant PJK. Spine Deformity 2, 505 (2014). Gibson JNA, Sillence DO, Taylor TKF. Abnormalities of the spine in Goldenhar’s Syndrome. J Pediatr Orthop 1996; 16L: 344-9. Tsirikos AI, McMaster MJ. Goldenhar-Associated conditions (hemifacial microsomia) and congenital deformities of the spine. Spine 2006; 31(13): E400-407.