Congenital Cervicothoracic Scoliosis Treated with Hemiepiphysiodesis and Placement of Distraction-Based Instrumentation: A Case Report.

Abstract

Early-onset scoliosis has many diverse causes1. The management of early-onset scoliosis is complicated by the need to control progression of spinal deformity without negatively impacting pulmonary function with early spinal fusion2,3. This has led to the development of “growth-friendly” instrumentation that allows growth of the thorax and increases the space for lung development4. Surgical options for management of congenital cervicothoracic scoliosis have included posterior fusion in situ or hemivertebra resection 5-8. Fusion alone does not allow for large correction, and it can result in an asymmetric head tilt. Hemivertebra resection allows for correction of the deformity but carries increased risk of iatrogenic neurologic injury8. In this case report, we present the application of growth-friendly instrumentation (developed for correction of scoliosis in the thoracic spine) to perform a gradual correction of deformity in the cervicothoracic spine. Hemiepiphysiodesis of the convex side was combined with distraction-based instrumentation on the concave side to improve head position without the neurologic risk posed by acute correction of the deformity. The patient’s parents were informed that data concerning the case would be submitted for publication, and they provided consent. A two-year-old girl with congenital cervicothoracic scoliosis presented for evaluation (Fig. 1). The patient had a 70° curve with an apex at T2 and a T1 tilt measuring 45°. T1 tilt was measured as the angle from the superior endplate of T1 to the horizontal reference line on a standing radiograph. There were seven ribs at the most cephalad left side of the spine compared with four ribs on the right side, the equivalent of three hemivertebrae. On the concave side, there was a unilateral bar from T2 to T6 (Fig. 2). She had a severe head tilt and minimal range of neck motion. …