We report the results of vertebral column resection
(VCR) for paediatric patients with spinal deformity. A total of
49 VCRs in paediatric patients from four university hospitals between
2005 and 2009 with a minimum two-year follow-up were retrospectively
identified. After excluding single hemivertebral resections (n =
25) and VCRs performed for patients with myelomeningocele (n = 6),
as well as spondylectomies performed for tumour (n = 4), there were
14 patients who had undergone full VCR at a mean age of 12.3 years
(6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular
scoliosis in three, congenital kyphosis in two, global kyphosis
in two, adolescent idiopathic scoliosis in one and secondary scoliosis
in one. A total of seven anteroposterior and seven posterolateral approaches
were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively
and 37° (17° to 80°) at the two-year follow-up; correction was a
mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to
70%) in the posterolateral group at the two-year follow-up (p =
0.53). The mean Scoliosis Research Society-24 total scores were
100 (92 to 108) for the anteroposterior and 102 (95 to 105) for
the posterolateral group. There was one paraparesis in the anteroposterior
group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful
procedure.
We have treated 175 patients with a chordoma over a ten-year period. Only two had a family history of the condition and we describe these in this paper. In one patient the tumour was at the craniocervical junction and in the other the lesion affected the sacrum. We have undertaken a literature review of familial chordoma and have identified chromosomal abnormalities associated with the condition.