22 Nonoperative Treatment of Syndromic Scoliosis
Syndromic scoliosis occurs in a broad group of conditions of varying etiology:
Neurofibromatosis
Heritable disorders of connective tissue: osteogenesis imperfecta, Marfan syndrome, Ehlers–Danlos syndrome
Mucopolysaccharidoses
Skeletal dysplasias
Metabolic bone disease: rickets
Endocrine disorders
Down syndrome
The options for the nonoperative treatment of spine deformity, from the treating surgeon’s perspective, are more limited than the options that patients may find available to them during a brief Internet search:
Serial casting: Cotrel (elongation, derotation, flexion); Risser
Bracing
Traction: halo–gravity, halo–femoral, halo–pelvic
Exercise
Serial casting is often used to treat early onset scoliosis (developing before the age of 5 years), and general anesthesia is usually required every 3 or 4 months to refit the cast. Although an advantage of casting is that the cast cannot be removed by the patient or family, tolerance is an issue; however, many younger children function well with a cast on.
The efficacy of bracing is widely debated in the more common condition of adolescent or late onset idiopathic scoliosis, and it is therefore not surprising that there is a relative paucity of literature regarding the role of bracing in the treatment of syndromic scoliosis. Patients and their surgeons may, however, seek what are considered to be “less invasive” treatments for their condition in an attempt to avoid or delay surgery. Patient compliance is considered to be an important issue in brace effectiveness. In adolescent idiopathic scoliosis, the use of a brace during the premenarchal growth spurt (peak height velocity) may be of importance, whereas in syndromic scoliosis, patterns of curve progression are not so well defined, and the effects of the condition may extend beyond the usual peak growth periods. Many brace types exist, further complicating the interpretation of data.
Traction, in its various forms, is often used as a preoperative intervention in an attempt to improve the flexibility of a severe scoliosis and thereby the correction obtained.
Exercise therapy has been proposed in the treatment of idiopathic scoliosis, and research continues. At present, there is no evidence for its efficacy in syndromic scoliosis.
22.1 Casting
Mehta 1 reported the outcome of Cotrel (elongation, derotation, flexion) casting in 136 patients with early onset scoliosis. Treatment started before the age of 4 years, and follow-up was for 9 years. The majority of the cases had idiopathic-type curves, but a group of 36 patients had defined or undefined syndromes. Some of the patients in the group had neurologic disorders, but these did not specifically include neurofibromatosis or Marfan syndrome. The results suggest that some cases of syndromic scoliosis have the potential to respond to casting. The main factors affecting response to treatment were age and Cobb angle at presentation. Delays in referral and subsequent rapid Cobb angle progression were felt to militate against a good outcome. Some patients were subsequently managed in a brace.
22.2 Bracing
22.2.1 Neurofibromatosis
Winter et al 2 reviewed the natural history, associated anomalies, and response to nonoperative and operative treatment in 102 patients with scoliosis and neurofibromatosis. Of these, 80 patients had features of dystrophic scoliosis, with rib penciling, vertebral scalloping, and foraminal enlargement. Milwaukee braces were used to treat 10 patients in this group, and in none of them was curve progression arrested. The average curve at the start of brace treatment was 53 degrees, and at the end it was 80 degrees. The curves of the 22 patients without dystrophic features behaved quite differently, and in the four documented cases in the paper treated with a Milwaukee brace for scoliosis or kyphosis, progression to surgery did not occur. The authors concluded that brace treatment was not indicated for dystrophic curves but might be effective for nondystrophic scoliosis.
22.2.2 Marfan Syndrome
Sponseller et al 3 investigated the effectiveness of bracing in Marfan syndrome. The study group included 22 patients with curves of 45 degrees or less and a Risser grade of 2 or lower. Bracing was recommended for 18 hours or more per day, and follow-up continued until maturity or surgery (minimum, 2 years). The average age at the initiation of bracing was 8.7 years (range, 4–12). The initial correction of the curve in the brace was significant, at 45%. Progression was prevented in four patients, but in 20 of 24 patients, treatment was considered a failure. The mean progression was +6 degrees (mean rate of progression, +8 degrees per year), and the average final curve measurement was 49 degrees. Sixteen of the patients underwent or were advised to undergo surgical correction of their scoliosis. The difference between patient age and degree of curvature in the successful group and the unsuccessful group was not statistically significant.
The authors concluded that the majority patients who have Marfan syndrome with a curve of 25 degrees or more and a Risser grade of 2 or lower will continue to have curve progression despite brace treatment, and that consideration of surgical correction is required (Fig. 22.1).
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