Saito et al ⁶

This was a retrospective study in a residential unit for patients with severe psychosomatic disorders. Of the 108 residents identified, 79 were felt to have spastic quadriplegia, and 54 had a scoliotic curve with a Cobb angle of more than 10 degrees. Of the 54 with a Cobb angle of more than 10 degrees, 37 had had radiographic surveillance of their scoliosis from before 15 years of age, with a mean follow-up of 17.3 years (range, 10–25 years). Of these, six were ambulatory, 24 could sit, and seven were classified as bedridden (i.e., could not sit). The strength of this paper is that the subjects did not receive any intervention, in keeping with views at the time, and their follow-up from childhood to final radiograph was well into adulthood (mean age, 25.1 years; range, 15–36). The authors found that when scoliosis started before the age of 10 years, it progressed rapidly during the adolescent growth period, continuing to increase after this growth period ended. They published the finding that a curve with a Cobb angle of more than 40 degrees before 15 years of age was associated with a significantly worse progression over time. Of their 37 patients, 20 required increased amounts of nursing time to complete various activities of daily living. The average Cobb angle for those 20 patients was 73 degrees, where it was 34 degrees in those patients who did not require increased assistance.

Majd et al ⁷

The authors clinically assessed the 240 residents of an institution for patients with severe physical and/or learning disability, identifying 56 adult residents who had reached skeletal maturity (defined as Risser stage 5). These adults were followed both radiographically and clinically to detect functional decline in abilities over time and whether decline correlated with the progression of scoliosis. Functional deterioration was observed in 10 adults over time; the Cobb angle of curves changed from a mean of 41.1 to 80.6 degrees in the group with functional decline, as opposed to a change from 33.9 to 56.5 degrees in the group without decline. The authors then extrapolated these statistically significant data to estimate that the yearly deterioration of a curve ranged from 3 degrees (in the stable population) to 4.4 degrees (in the group with deterioration).

Subjects were also divided into groups depending on whether their curve was s-shaped, c-shaped, or kyphotic, and they further subdivided patients within these groups based on the location of the primary curve or on the presence or absence of scoliosis if the curve was kyphotic. The authors then described progression in each of these subgroups and were unable to show statistically significance differences between the groups in terms of progression rate or size of the initial curve. For instance, 40 patients had long c –shaped curves and the initial curve averaged 32 degrees (range, 15–85), with an average progression rate of 3.5 degrees (range, 0–13 degrees) per year. Given that these were adults who were skeletally mature, the range in this subset is so large as to be meaningless in trying to use the data to predict an individual patient’s likelihood of deterioration.

Thometz and Simon ⁸

In this institution-based retrospective study, 51 adults were followed for between 4 and 40 years (mean, 16 years) after skeletal maturity. They were among 900 adults in the institution, 180 of whom were felt to have CP; of these, 110 had scoliosis. Only patients for whom radiographs were available 4 years after skeletal maturity were included; 10 were excluded because an intervention (arthrodesis or brace) had been used to treat their scoliosis.

As in the sample of Majd et al, the variation between subjects was huge. ⁷ The authors reported that thoracic curves at the time of skeletal maturity were 46 degrees (range, 18–115), thoracolumbar curves were 54 degrees (range, 19–143), and lumbar curves were 63 degrees (range, 20–120). All radiographs were taken with the patients in the supine position to avoid the effect of gravity. Grouping their data together, the authors were able to show a difference in the mean annual rates of curve progression at skeletal maturity, which were 0.8 degrees if the Cobb angle was less than 50 degrees and 1.4 degrees if it was more than 50 degrees. Looking at their data retrospectively, they showed that a curve that developed before age 10 was likely to progress, but the development of a curve after 10 years did not protect against progression.

The authors provide useful information about the degree of change and range of curves in this population. However, whether their data can be used for a “degree of deterioration” measurement is of questionable relevance in predicting the clinical course of an individual child or young person.

Kalen et al ⁹

In their prospective study of institutionalized adults with learning disabilities, Kalen et al attempted to find differences between the incidence rates of decubiti, highest functional levels achieved, degrees of functional loss, levels of oxygen saturation, and pulse rates in patients who had CP with untreated scoliosis greater than 45 degrees and the incidence rates in those with mild or no curves. Their sample included 62 adult patients with a mean age of 39 years (range, 29–67) who were described as having CP: 54 with spastic quadriplegia, six with diplegia, one with hemiplegia, and one unclassified. Although the authors found no differences in the health of the two groups as described above, it is interesting to note that average oxygen saturation values of 80% (i.e., significantly low) were recorded in both groups. These levels feel uncomfortable by the standards of health one would expect even in these particularly vulnerable subjects, and such results would today raise questions about the health needs of both populations.

Senaran et al ¹⁰

This prospective study compared 23 children who had scoliosis and unilateral hip dislocation with a control group of 83 children who had scoliosis and no hip dislocation. The mean age at the diagnosis of scoliosis in the group with scoliosis and a dislocated hip was 10.4 years (range, 4–16), and the mean follow-up was 3.6 years (range, 2–8). In the group with scoliosis alone, the average age at diagnosis was 10.5 years (range, 3–18), and the mean follow-up was 4 years (range, 2–11). The aim of this study was to look at the effect of untreated unilateral hip dislocation on the emergence of scoliosis, and the authors found that pelvic obliquity was more of a marker than the hip dislocation per se. They reported pediatric progression at a mean of 12.2 degrees per year in the control group and of 12.9 degrees per year in the group with unilateral dislocation. This is the only study included in this systematic review to indicate the deterioration seen during the childhood growth spurt. Again, no clear diagnostic criteria were mentioned.

Gu et al ¹¹

In a study published after the systematic review of Loeters et al, Gu et al looked at 110 children younger than 18 years of age who were reported as having CP; all but one had a GMFCS level of 5 and resided in a pediatric nursing home. The first radiograph was taken when scoliosis became a clinical concern, and a thoracolumbosacral orthosis (TLSO) was worn by the 38 subjects for whom it was prescribed (there is no account of whether this was worn during subsequent radiographs). The authors were able to demonstrate that Cobb angles larger than 40 degrees by the age of 12 years were more likely to increase with age when taking the whole group together. The range of scoliosis evident at first assessment was large, although the quoted initial mean Cobb angle for the group was 20.7 degrees, the range was 0 to 92 degrees on radiograph. Equally, the mean Cobb angle on the last X ray was 39 degrees—the range was 4 to 120 degrees.