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Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome.
Spine Deform 2018 May - Jun; 6(3):327-333SD

Abstract

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.

Authors+Show Affiliations

Department of Medicine, Dalhousie University, 6299 South St, Halifax, NS B3H 4R2, Canada; IWK Health Centre, 5980 University Ave, Halifax, NS B3K 6R8, Canada. Electronic address: Braydon.connell@dal.ca.Department of Medicine, Dalhousie University, 6299 South St, Halifax, NS B3H 4R2, Canada; IWK Health Centre, 5980 University Ave, Halifax, NS B3K 6R8, Canada.Shriner's Hospital, 3551 N Broad St, Philadelphia, PA 19140, USA.Rainbow Babies and Children's Hospital, 11100 Euclid Ave, Cleveland, OH 44106, USA.Children's Spine Foundation, P.O. Box 397, Valley Forge, PA 19481, USA.Children's Spine Foundation, P.O. Box 397, Valley Forge, PA 19481, USA.Department of Medicine, Dalhousie University, 6299 South St, Halifax, NS B3H 4R2, Canada; IWK Health Centre, 5980 University Ave, Halifax, NS B3K 6R8, Canada.Children's Spine Foundation, P.O. Box 397, Valley Forge, PA 19481, USA.Growing Spine Foundation, San Diego, CA, USA.

Pub Type(s)

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

Language

eng

PubMed ID

29735145

Citation

Connell, Braydon, et al. "Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome." Spine Deformity, vol. 6, no. 3, 2018, pp. 327-333.
Connell B, Oore JJ, Pahys JM, et al. Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome. Spine Deform. 2018;6(3):327-333.
Connell, B., Oore, J. J., Pahys, J. M., Thompson, G. H., St Hilaire, T., Flynn, T., & El-Hawary, R. (2018). Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome. Spine Deformity, 6(3), pp. 327-333. doi:10.1016/j.jspd.2017.11.005.
Connell B, et al. Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome. Spine Deform. 2018;6(3):327-333. PubMed PMID: 29735145.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome. AU - Connell,Braydon, AU - Oore,Jonathan J, AU - Pahys,Joshua M, AU - Thompson,George H, AU - St Hilaire,Tricia, AU - Flynn,Tara, AU - El-Hawary,Ron, AU - ,, AU - ,, PY - 2017/10/01/received PY - 2017/11/12/accepted PY - 2018/5/9/entrez PY - 2018/5/8/pubmed PY - 2019/1/12/medline KW - Early-onset scoliosis KW - Goldenhar syndrome KW - Growth friendly KW - Oculoauriculovertebral dysplasia spectrum SP - 327 EP - 333 JF - Spine deformity JO - Spine Deform VL - 6 IS - 3 N2 - 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. SN - 2212-1358 UR - https://www.unboundmedicine.com/medline/citation/29735145/Growth_Friendly_Surgery_Is_Effective_at_Treating_Early_Onset_Scoliosis_Associated_With_Goldenhar_Syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2212-134X(17)30415-X DB - PRIME DP - Unbound Medicine ER -