Clinical and radiological data were reviewed for all patients
with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed
non-operatively or operatively in our institution. In all 16 patients were included (eight female: eight male; 50%
male), of whom nine had Hurler, five Morquio and two Hunter syndrome.
Six patients were treated non-operatively (mean age at presentation
of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5o/year;
mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean
age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression
10.8o/year; mean follow-up of 8.2 years; 4.8 to 11.8)
by circumferential arthrodesis with posterior instrumentation in
patients with flexible deformities (n = 6).Aims
Methods
We describe 13 patients with cerebral palsy and
lordoscoliosis/hyperlordosis of the lumbar spine who underwent a posterior
spinal fusion at a mean age of 14.5 years (10.8 to 17.4) to improve
sitting posture and relieve pain. The mean follow-up was 3.3 years
(2.2 to 6.2). The mean pre-operative lumbar lordosis was 108° (80
to 150°) and was corrected to 62° (43° to
85°); the mean thoracic kyphosis from 17° (-23° to
35°) to 47° (25° to 65°);
the mean scoliosis from 82° (0° to 125°)
to 22° (0° to 40°); the mean pelvic
obliquity from 21° (0° to 38°)
to 3° (0° to 15°); the mean sacral
slope from 79° (54° to 90°) to
50° (31° to 66°). The mean pre-operative
coronal imbalance was 5 cm (0 cm to 8.9 cm) and was corrected to
0.6 cm (0 to 3.2). The mean sagittal imbalance of -8 cm (-16 cm
to 7.8 cm) was corrected to -1.6 cm
(-4 cm to 2.5 cm). The mean operating time was 250 minutes (180
to 360 minutes) and intra-operative blood loss 0.8 of estimated
blood volume (0.3 to 2 estimated blood volume). The mean intensive
care and hospital stay were 3.5 days (2 to 8) and 14.5 days (10
to 27), respectively. Three patients lost a significant amount of
blood intra-operatively and subsequently developed chest or urinary
infections and superior mesenteric artery syndrome. An increased pre-operative lumbar lordosis and sacral slope were
associated with increased peri-operative morbidity: scoliosis and
pelvic obliquity were not. A reduced lumbar lordosis and increased
thoracic kyphosis correlated with better global sagittal balance
at follow-up. All patients and their parents reported excellent
surgical outcomes. Lordoscoliosis and hyperlordosis are associated with significant
morbidity in quadriplegic patients. They are rare deformities and
their treatment is challenging. Sagittal imbalance is the major
component: it can be corrected by posterior fusion of the spine
with excellent functional results. Cite this article:
