Background Data: Radiography has been the mainstay of patient monitoring in scoliosis, but there is an increasing demand for its reduction to specific situations where treatment is to be decided or modified. There is concern that substitution of traditional methods with clinical impression and surface topography might not be feasible or safe.

Study Design: An outcome study of a year’s intake of new patients with adolescent idiopathic scoliosis using a protocol derived from experience with surface topography.

Method: Tolerance limits for observer and subject variation and observed changes over time were established and correlated with recorded Cobb angle changes. A “derived Cobb angle” was calculated from topographic spinal angles and radiographs of 75 patients with non-congenital scoliosis and tested against 141 similar patients. Cobb angle = 15.3 + 1.22* topographic spinal angle. A protocol was adopted with topography at every clinic visit, radiography reserved for cases with severe deformity, additional symptoms or where surgical intervention was considered. This protocol was tested on new adolescent idiopathic scoliosis (AIS) presentations to the general clinic in a single year (2001) with regard to status at presentation and outcome (n=49).

Results: Measurement error, on the average of four repositioned scans on 105 consecutive patients rounded up to 10 units on all parameters. In 75 patients with non-congenital scoliosis, change ≥10° in Cobb angle was always accompanied by a similar change on at least one topographic parameter. The mean difference was −3.9°, SD 14.7, and was greater in very small, larger or double curves and in obese patients. There was significant correlation (p< 0.01) between changes in the Cobb angle over time and that derived from the spinal angle. 49 girls presenting with a presumptive diagnosis of AIS were diagnosed thus: Normal, n=8, 4 after radiograph, all now discharged; Asymmetry, n=24, no radiographs, 11 discharged immediately, 10 after 0.5 – 1.5 years, 3 lost; AIS, n=17, Cobb angle 10–93°, 5 surgery, 6 discharged, 4 currently followed, 2 non-attendees.

Discussion: The incomplete correlation is acceptable, since within-subject variation of the Cobb angle is unknown but the observer variation was shown by Carman et al (JBJS 72(A):328–333) to be over 8°. The discrepancy between actual and derived Cobb angles at the extremes is understandable as small curves are inflated by the obligatory constant, while increased subcutaneous tissue smoothes the surface, and both double and large curves show more rotation of vertebral bodies than of spinous processes. This can be tolerated because in small curves, prediction is made on maturity indicators rather than Cobb angle, while at higher values, cosmesis is the issue, small changes in Cobb angle are less relevant, and pubertal status determines progression potential more effectively than radiographic measures.

Conclusion: Topography and reduced use of radiography allows safe monitoring of adolescent idiopathic scoliosis. It provides a validated cosmetic score which documents deformity progression, is an adjunct to clinical decision making and is mathematically related to the Cobb angle. Basic clinical modalities and careful consideration of every patient on an individual basis are still essential.

Introduction: Hurler syndrome (mucopolysaccharidosis type I) is an autosomal recessive disorder with characteristic progressive musculoskeletal manifestations termed dysostosis multiplex. These include dorsolumbar kyphosis with gibbus deformity, deficient femoral head ossification and acetabular dysplasia, valgus deformity of the knee, broad hands prone to carpal tunnel syndrome, pes planus and generalised joint contracture. Untreated, death occurs early in childhood. Therapeutic bone marrow transplantation (BMT) has improved life expectancy and now patients attain skeletal maturity. BMT, however, appears to have little influence on skeletal outcome, and optimum orthopaedic management is as yet uncertain.

Methods: Nineteen patients treated by BMT at a mean of 10 months (range 3–19 months), have been followed over a mean of 93 months (range 3–196 months). Five patients are now adolescent. We describe the clinical and radiological features of these patients and detail orthopaedic procedures and their outcome. Descriptive statistical analysis is used, displaying mean values and standard deviation where appropriate.

Results: All patients demonstrate characteristic acetabular dysplasia and failure of ossification of the superolateral femoral head. Nine have undergone bilateral pelvic and femoral derotation osteotomies at a mean of 48 months (range 24–105 months); mean acetabular angle preoperatively was 33 ± 3.8°. Five patients had pelvic osteotomy only. IN the remaining five patients, mean acetabular angle is 25 ± 3.3°. Genu Valgum of variable severity due to failure of ossification of the lateral aspect of the proximal tibial metaphysis is observed early. Four patients underwent medial epiphyseal stapling at a mean of 105 months. While most patients demonstrate thoracolumbar vertebral anomalies peculiar to Hurler syndrome, only five had had progressive scoliosis, two having had anterior spinal fusion at 37 and 72 months. Most patients experience symptoms of carpal tunnel syndrome from about 48 months. Only one patient has significant difficulty mobilising independently.

Conclusions: Our findings reflect the variability of musculoskeletal problems seen in Hurler syndrome. This cohort represents one of the largest available for study, and ongoing review will clarify the natural outcome of the disease and help determine if and when orthopaedic intervention is appropriate.

Objective: Determination of the height at maturity of patients with congenital vertebral anomalies and analysis of the effect of spinal surgery.

Design: Review of clinical measurements collected prospectively.

Subjects: Patients with congenital vertebral anomaly who had passed their sixteenth birthdays.

Outcome measures: Stature compared to normal centiles for age and gender, with consideration of the type of abnormality, age at surgery and co-existing abnormalities.

Results: Thirty-eight boys, mean age 16.41 years, SD 0.31, and 79 girls, mean age 16.76 yrs, SD 1.3 were identified. Growth rate over two years after age16 years was slow (boys: 1.0 cm, SD 3.05; girls: 0.33 cms, SD 1.98). At maturity, mean height for boys was just above the third centile while girls were on theirs. Height was significantly lower for patients with prior spinal surgery and with a diagnosis of VACTERL association. In boys, there was no correlation between age at surgery and final height, but in girls final height correlated significantly with age at diagnosis and at surgery and negatively with age at menarche.

Conclusion: Stature at maturity is reduced and this is exacerbated by associated abnormalities and by spinal surgery. However, those with more severe abnormalities are more likely to have had surgery, and these two effects were not differentiated in this study.

Objective: Examination of treatment outcome in a mixed group with early onset scoliosis.

Design: Retrospective review of patient data in order to plan a meaningful prospective study of treatment protocols.

Background: Scoliosis presenting before age 10 years, whatever the classification, has potentially significant consequences for morbidity and mortality, yet is discussed less than the more benign adolescent form.

Subjects: Patients with early onset idiopathic or syndromic scoliosis who were at least 15 years old at last review.

Outcome measures: Cobb angle change and the incidence of non-operative treatment and of surgery.

Results: There were 44 male and 74 female patients (19 IIS, 44 JIS and 55 syndromic) with follow-up 12.3 years SD 4.35 and mean Cobb angle progression from 26.6° to 47.21°. Non-operative treatment did not reduce the incidence of surgery either over-all (66.1%) or in individual groups (84.2% IIS, 63.6% JIS and 61.8% syndromic). Surgery in conservatively treated patients was at a later age (11.8 years SD 3.72) in comparison to those without (9.1 years SD 4.53), suggesting some advantage.

Conclusion: While orthotic treatment cannot be shown to prevent surgery in this series, achievement of the more modest aim of delay until nearer the pubertal growth spurt would be an undoubted benefit. Further study is necessary to determine whether this is a therapeutic effect or artefact.

Objective: Assessment of the outcome for spinal surgery in early onset deformity.

Design: Prospectively collected data on surface topography and respiratory function was analysed.

Subjects: Patients who underwent anterior and posterior spinal surgery before the age of 10 years for non-congenital deformity with special regard to cosmesis and respiratory function.

Outcome measures: Surface topography and respiratory function.

Results: In 10 children with pre-operative surface topography, surgery produced only an apparent reduction in the spinal angle. In the larger group, N=25, who have serial topography post-operatively (mean topographic follow-up 3.5 yrs, SD 1.55), the Suzuki hump sum (a measure of rib hump and back asymmetry) had increased significantly by a mean of 6.9 (SD 14.3, t=2.424, P=0.023). Respiratory function declined. Cobb angles varied in stability but usually increased initially. Trunk balance was maintained.

Conclusion: Surgery in early onset spinal deformity offers mechanical correction of deformity and a promise of no further progression, while also intending to preserve respiratory function into adult life, thus reducing morbidity and early mortality. Initial findings suggest that this is not so: while Cobb angle and trunk balance may be stabilised, the rib hump continues to increase and respiratory function to decline.

Objective: Adolescent idiopathic scoliosis (AIS) has been intensively studied for many years but conclusions on prognosis and best treatment are hampered by the shortage of untreated controls.

Design: Analysis of patient data in a prospectively maintained database.

Subjects: Patients with AIS (42 male, 458 female), at least 15 years old at last review.

Outcome Measures: Cobb angle change, growth, and the incidence of surgical treatment.

Results: Of 500 patients, 36 (7.2%) were braced and 138 (27.6%) had surgery. At diagnosis mean age was 13.8 yrs. SD 1.55, Cobb angle 30°, SD 17.85. Change over a mean of 3.5 years averaged 5.94°, SD 11.61, range −23° to +55°. There was no association between brace treatment and outcome. Below 50° at presentation, there was a significant association between age at diagnosis and curve progression. The change in Cobb angle correlated significantly with both absolute increase in height and in growth rate.

Conclusion: These results can be repeated in any scoliosis centre in the world, regardless of treatment policy. About one quarter will develop a significant cosmetic deformity. The remainder will stabilise their scoliosis without treatment. Deformity progresses with growth and correlates with growth rate, so is intrinsic to the growth process.