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.
Diastematomyelia is a rare congenital abnormality
of the spinal cord. This paper summarises more than 30 years’ experience
of treating this condition. Data were collected retrospectively
on 138 patients with diastematomyelia (34 males, 104 females) who
were treated at our hospital from May 1978 to April 2010. A total
of 106 patients had double dural tubes (type 1 diastematomyelia),
and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs,
CT myelography, and MRI showed characteristic kyphoscoliosis, widening
of the interpedicle distance, and bony, cartilaginous, and fibrous
septum. The incidences of symptoms including characteristic changes
of the dorsal skin, neurological disorders, and congenital spinal
or