Scoliosis in Goldenhar syndrome with curve reversal during brace treatment: a case report

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(2020) 21:685

CASE REPORT

Open Access

Scoliosis in Goldenhar syndrome with curve reversal during brace treatment: a case report Masashi Uehara* , Shugo Kuraishi, Shota Ikegami, Hiroki Oba, Takashi Takizawa, Ryo Munakata, Terue Hatakenaka, Tetsuhiko Mimura and Jun Takahashi

Abstract Background: Goldenhar syndrome sometimes displays progressive scoliosis and other spinal deformities that require treatment. However, few reports exist on scoliosis correction in Goldenhar syndrome. We described the rare radiological outcomes of a patient with Goldenhar syndrome who received brace treatment for scoliosis. Case presentation: A 4-year-old boy was diagnosed as having Goldenhar syndrome and referred to our hospital for scoliosis treatment. The deformity deteriorated gradually, and left convex scoliotic angle was 26 degrees (T3-L2) at 11 years of age. Unexpectedly during treatment with an orthopedic brace, the curve had reversed to 21 degrees (T5-L2) at 7 months of therapy. After another adjustment of the brace, his right convex scoliotic angle improved to 13 degrees (T4-L2) at 15 months of treatment. Conclusions: Curve reversal may occur during brace treatment for scoliosis in Goldenhar syndrome. Clinicians may opt to periodically check curve correction despite the risk of increased radiation exposure. Keywords: Scoliosis, Goldenhar syndrome, Brace, Radiological findings, Overcorrection, Curve reversal

Background Goldenhar syndrome is a hereditary disease first reported in 1952 as a disorder with facio-auriculovertebral dysplasia [1]. The severity of dysplasia varies by case [2, 3]. The frequency of Goldenhar syndrome is estimated at 1 in 3500 to 5600 live births [4, 5]. The male: female ratio of patients is approximately 3:2 [4, 5]. Scoliosis and other spinal deformities have been associated with this syndrome and can sometimes be progressive and require treatment [6]. Vertebral abnormalities and congenital spinal deformities are also common in Goldenhar syndrome [7–9].

* Correspondence: [email protected] Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan

To date, there are a few reports on the treatment of scoliosis in Goldenhar syndrome. We herein describe the rare radiological outcomes of an afflicted patient who received a brace for scoliosis correction.

Case presentation This study was approved by the institutional ethical review board of Shinshu University School of Medicine (No. 4847) prior to its start and was conducted in accordance with the ethical standards set forth in the 2013 Declaration of Helsinki for research involving human subjects. Written informed consent was obtained from the patient’s parent for publication of this Case report and any accompanying images. A 4-year-old boy was diagnosed as having Goldenhar syndrome and referred to our hospital for

© The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distrib