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.
It is uncertain whether instrumented spinal fixation in nonambulatory children with neuromuscular scoliosis should finish at L5 or be extended to the pelvis. Pelvic fixation has been shown to be associated with up to 30% complication rates, but is regarded by some as the standard for correction of deformity in these conditions. The incidence of failure when comparing the most caudal level of instrumentation, either L5 or the pelvis, using all-pedicle screw instrumentation has not previously been reported. In this retrospective study, we compared nonambulatory patients undergoing surgery at two centres: one that routinely instrumented to L5 and the other to the pelvis. In all, 91 nonambulatory patients with neuromuscular scoliosis were included. All underwent surgery using bilateral, segmental, pedicle screw instrumentation. A total of 40 patients underwent fusion to L5 and 51 had their fixation extended to the pelvis. The two groups were assessed for differences in terms of clinical and radiological findings, as well as complications.Aims
Methods
We report the incidence of and risk factors for
complications after scoliosis surgery in patients with Duchenne muscular
dystrophy (DMD) and compare them with those of other neuromuscular
conditions. We identified 110 (64 males, 46 females) consecutive patients
with a neuromuscular disorder who underwent correction of the scoliosis
at a mean age of 14 years (7 to 19) and had a minimum two-year follow-up.
We recorded demographic and peri-operative data, including complications
and re-operations. There were 60 patients with cerebral palsy (54.5%) and 26 with
DMD (23.6%). The overall complication rate was 22% (24 patients),
the most common of which were deep wound infection (9, 8.1%), gastrointestinal
complications (5, 4.5%) and hepatotoxicity (4, 3.6%). The complication
rate was higher in patients with DMD (10/26, 38.5%) than in those
with other neuromuscular conditions (14/84, 16.7% (p = 0.019). All
hepatotoxicity occurred in patients with DMD (p = 0.003), who also
had an increased rate of deep wound infection (19% In our series, correction of a neuromuscular scoliosis had an
acceptable rate of complications: patients with DMD had an increased
overall rate compared with those with other neuromuscular conditions.
These included deep wound infection and hepatotoxicity. Hepatotoxicity
was unique to DMD patients, and we recommend peri-operative vigilance
after correction of a scoliosis in this group. Cite this article:
Technological advances and shorter rescue times have allowed early and effective resuscitation after trauma and brought attention to the host response to injury. Trauma patients are at risk of progressive organ dysfunction from what appears to be an uncontrolled immune response. The availability of improved techniques of molecular diagnosis has allowed investigation of the role of genetic variations in the inflammatory response to post-traumatic complications and particularly to sepsis. This review examines the current evidence for the genetic predisposition to adverse outcome after trauma. While there is evidence supporting the involvement of different polymorphic variants of genes in determining the post-traumatic course and the development of complications, larger-scale studies are needed to improve the understanding of how genetic variability influences the responses to post-traumatic complications and pharmacotherapy.