Functional Outcomes of Congenital Scoliosis at a Mean 35-Year Follow-up Post In Situ Fusion. Revisiting Patients From the 2002 Goldberg et al Study.

BACKGROUND: The management of congenital scoliosis poses a significant challenge for treating surgeons. The aim of our study was to provide insight into the long-term clinical results of spinal fusion in congenital scoliosis. METHODS: We performed a retrospective review of the scoliosis database in our institution for the period 1976 until 2002 identifying 43 patients with congenital scoliosis who underwent spinal fusion. Patient demographics, diagnosis, levels fused, and radiographs were evaluated. Patients were evaluated for unplanned return to the operating room (UPROR) via SRS 22, EQ5D-5L, and Oswestry Disability Index (ODI). RESULTS: Of the 43 patients who fulfilled the inclusion criteria, 22 patients agreed to participate, 3 patients were known to be deceased and 18 patients were lost to follow-up or declined to participate and were excluded. The mean age of the respondents was 40.7 years (range, 30 to 47 y) with a mean follow-up from index surgery of 35 years (range, 20 to 44 y). At most recent follow-up, 12 patients (54%) underwent UPROR. The mean age at diagnosis was 3.4 years (range, birth to 11.5 y), and the mean age for first surgery was 5.8 years (range, 1 to 13 y). As regards radiologic follow-up; the mean number of levels fused was 5.2 (range, 2 to 12). Thoracic fusion was performed in 17 patients (77%). The mean T1 to T12 height at index surgery and maturity was 166 mm (range, 130 to 240 mm) and 202 mm (range, 125 to 270 mm), respectively. The mean functional scores at follow-up were SRS 22: 4.5 (range, 2.4 to 5), cumulative EQ5D-5L score 7.2 (range, 5 to 15), and ODI: 8% (range, 2 to 30%). All respondents completed high school, 10 patients (45%) completed university, and 2 patients were awarded doctorates. Currently, 17 patients (77%) are in paid employment. CONCLUSIONS: This report constitutes the largest series of patients treated by spinal arthrodesis for congenital scoliosis followed into maturity. We demonstrate the thorax continues to grow after index fusion, patient-reported outcomes were satisfactory with superior educational and employment rates and unplanned return to theatre is rare in adult life. LEVEL OF EVIDENCE: Therapeutic Level IV.

Scoliosis: a review.

Scoliosis, lateral curvature of the spine, has been studied since Hippocrates' time, but remains a disputed subject in orthopaedic surgery, because of its several varieties, unknown cause and unpredictable course. A review of 30 years' experience in a paediatric orthopaedic unit was undertaken to clarify the problem. Patient records, collected prospectively, were examined to demonstrate the incidence, prevalence, extent, course and outcome of the commonest variety, adolescent idiopathic scoliosis. Reference was made to the results of previously published studies. Records from a school screening programme showed that, while slight degrees of spinal curvature were widely prevalent in the community, these were of no clinical significance and major cosmetic deformity was rare: only 8 in 10,000 adolescent girls had Cobb angles of 40 degrees or more and only half of these underwent surgical correction. Evidence to support non-operative treatment could not be demonstrated and it did not reduce the incidence of surgical intervention. Because the course of scoliosis did not seem to correspond with much published work, or with current hypotheses of aetiology, a rethinking of the whole subject is advocated. An alternative model of pathogenesis deriving from developmental biology was proposed. While advances in surgical methods have been significant, the core problems of aetiology and natural history remain. Until they are resolved, all conclusions on management must be provisional. This is where innovative thinking needs to be directed.

Long-term results from in situ fusion for congenital vertebral deformity.

STUDY DESIGN: Retrospective review of long-term outcome of fusion in situ for congenital vertebral anomaly with particular emphasis on cosmesis and the incidence of reoperation. OBJECTIVE: Examination of the success rate of this procedure and of risk factors for failure. BACKGROUND: Fusion in situ is the accepted prophylactic treatment to prevent deformity in congenital vertebral anomalies that have a high risk of progression or have been shown to be deteriorating. METHODS: Records of patients who were at least 15 years of age at last examination were reviewed retrospectively. Consideration was given to cosmetic outcome and to the incidence of reoperation. RESULTS: There were 43 patients in this category, 19 boys and 24 girls, who were at least 15 years of age when last seen. Reoperation had been performed in 11 cases (25.6%). The main finding was that, although the Cobb angle of the fused segment of spine remained constant after fusion, a curve sometimes developed in the whole spine, sometimes (but by no means always) centered on that fused segment. Cosmetic deformity continued to progress in a number of cases. CONCLUSIONS: Localized fusion, whether posterior alone or anterior and posterior combined, was effective in preventing progression of the Cobb angle of the congenitally malformed area but did not control the overall deformity that developed or progressed with growth. Current concepts of the pathomechanism of deformity do not adequately explain the observations, and a more biologic approach is suggested.

Growth patterns in children with congenital vertebral anomaly.

STUDY DESIGN: Height and weight data in children with congenital vertebral anomaly, recorded routinely during clinical follow-up, were analyzed. OBJECTIVES: To determine whether these children are significantly deficient in growth or show an abnormality of maturation. SUMMARY OF BACKGROUND DATA: Structural abnormalities in the vertebral column, and surgery to prevent or correct deformity secondary to this, clearly have the potential to affect the individual's final height. It is not clear, however, whether this is the whole problem, or whether there are other factors to be considered. METHODS: The height and weight of patients with all forms of spinal deformity are routinely recorded at outpatient visits. Data from patients with congenital vertebral abnormalities were analyzed and compared with the national standards. To test the possibility that the observed short stature might be familial, family groups (parents and siblings of patients) were invited to be measured. RESULTS: At the end of growth, patients with congenital vertebral anomalies were smaller than average and also smaller than their parents and siblings. This was more marked in girls and in the presence of associated anomalies, especially when multiple, as in VACTERL association (vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies). It was also exacerbated by surgery, which had a demonstrable stunting effect on the spine. DISCUSSION: Although shortening of the spine was demonstrated in patients with prior spinal surgery, the contributions of the fact of surgery and the presumed severity of anatomic abnormality could not be differentiated. The observations that, even without surgery, girls were generally on a lower percentile than boys and that both have a tendency to lose height at growth spurt invite further investigation. CONCLUSIONS: The shortening of the spine by surgery is not a contraindication to treatment, because a straight, balanced spine must always be preferable to a longer, crooked and unbalanced one. However, it is another facet to be remembered when considering the management plan of the whole child.