We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

We have investigated whether patients with adolescent-onset idiopathic scoliosis (AIS) are more likely to have a low body-weight. Measurements of weight, height and body mass index (BMI) were made in 44 young women with AIS and compared with age- and gender-adjusted normative data. The body mass criteria of the International Classification of Diseases for eating disorders was used to determine how many patients were within the range considered to be ‘eating disordered’. Compared with the normative data, the AIS group did not differ significantly in terms of height, (p = 0.646), but they were significantly lighter (p < 0.001) and had significantly lower BMI scores (p < 0.001); 25% of the series had BMI scores which were within the range considered to be anorexic.

The relationship between a diagnosis of AIS and low body-weight may indicate disordered eating and is thus a cause for concern, particularly in the light of the well-established relationship between eating psychopathology and osteoporosis. Aspects of organic health may need to be considered in addition to the cosmetic deformity.

We present a study of ten consecutive patients who underwent excision of thoracic or thoracolumbar hemivertebrae for either angular deformity in the coronal plane, or both coronal and sagittal deformity. Vertebral excision was carried out anteriorly alone in two patients. Seven patients had undergone previous posterior spinal fusion. Their mean age at surgery was 13.4 years (6 to 19). The mean follow-up was 78.5 months (20 to 180). The results were evaluated by radiological review of the preoperative, postoperative and most recent follow-up films.

The mean preoperative coronal curve was 78.2° (30 to 115) and was corrected to 33.9° (7 to 58) postoperatively, a mean correction of 59%. Preoperative coronal decompensation of 35 mm was improved to 11 mm postoperatively. Seven patients had significant coronal decompensation preoperatively, which was corrected to a physiological range postoperatively. There were no major complications and no neurological damage.

We have shown that resection of thoracic and thoracolumbar hemivertebrae can be performed safely, without undue risk of neurological compromise, in experienced hands.