Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome

Spine Deform. May-Jun 2018;6(3):327-333. doi: 10.1016/j.jspd.2017.11.005.


Objective: To evaluate the radiographic results and complications of growth-friendly (GF) surgery in the treatment of early-onset scoliosis (EOS) associated with Goldenhar syndrome.

Background: Goldenhar syndrome has been associated with spinal deformity, which may be progressive. Efficacy and complication rate of GF treatment has not been reported for this population of patients with EOS.

Methods: Patients with Goldenhar syndrome and EOS with two years' follow-up were identified from two international multicenter EOS databases. Scoliosis, kyphosis, spine height, and hemithoracic height/width were determined preimplant, immediately postoperative, and at the two-year follow-up. Severity of complications (SV) was recorded (Smith et al. JPO 2015).

Results: Ten patients met inclusion criteria and had a mean age of 4.6 ± 2.5 years at GF implantation (one spine and nine rib-based). Mean preoperative scoliosis was 64°, postimplant 52°, and at mean follow up of 2.4 ± 0.5 years was 50° (p = .09). Preoperative kyphosis was 36°, postimplant 38°, and final 42° (p = .08). Preoperative T1-S1 height was 23.5 cm, postimplant 23.6 cm, and final 27.3 cm (p = .06). Preoperative convex hemithoracic height was 10.4 cm, postimplant 7.9 cm, and final 12.8 cm (p < .05). Preoperative concave hemithoracic height was 8.4 cm, postimplant 8.8 cm, and final 9.9 cm (p = .30). Preoperative right hemithoracic width was 8.02 cm, postimplant 7.22 cm, and final 7.86 cm (p = .07). Preoperative left hemithoracic width was 7.18 cm, postimplant 7.86 cm, and final 8.60 cm (p = .43). 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).

Conclusion: At minimum two-year follow-up, GF surgical intervention for the treatment of EOS associated with Goldenhar syndrome trended toward improvements in scoliosis and spine height, but had a significant improvement in convex hemithoracic height; however, the majority of patients experienced severity grade I or II complications.

Level of evidence: Level IV.

Keywords: Early-onset scoliosis; Goldenhar syndrome; Growth friendly; Oculoauriculovertebral dysplasia spectrum.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Goldenhar Syndrome / complications*
  • Humans
  • Infant
  • Male
  • North America / epidemiology
  • Orthopedic Procedures / adverse effects
  • Postoperative Complications / epidemiology*
  • Radiography
  • Retrospective Studies
  • Scoliosis / diagnostic imaging
  • Scoliosis / etiology
  • Scoliosis / surgery*