Effectiveness of the Rigo Chêneau versus Boston-style orthoses for adolescent idiopathic scoliosis: a retrospective study.

BACKGROUND: Bracing can effectively treat adolescent idiopathic scoliosis (AIS), but patient outcomes have not been compared by brace type. We compared outcomes of AIS patients treated with Rigo Chêneau orthoses (RCOs) or custom-molded Boston-style thoracolumbosacral orthoses (TLSOs). METHODS: We retrospectively reviewed patient records from one scoliosis center from 1999 through 2014. Patients were studied from initial treatment until skeletal maturity or surgery. Inclusion criteria were a diagnosis of AIS, initial major curve between 25° and 40°, use of an RCO or TLSO, and no previous scoliosis treatment. RESULTS: The study included 108 patients (93 girls) with a mean (±standard deviation) age at brace initiation of 12.5 ± 1.3 years. Thirteen patients wore an RCO, and 95 wore a TLSO. Mean pre-bracing major curves were 32.7° ± 4.8° in the RCO group and 31.4° ± 4.4° in the TLSO group (p = 0.387). Mean brace wear time was similar between groups. Mean differences in major curve from baseline to follow-up were -0.4° ± 9.9° in the RCO group and 6.9° ± 12.1° in the TLSO group (p = 0.028). Percent changes in major curve from baseline to follow-up were 0.0% ± 30.5% for the RCO group and 21.3% ± 38.8% for the TLSO group (p = 0.030). No RCO patients and 34% of TLSO patients progressed to spinal surgery (p = 0.019). At follow-up, major curves improved by 6° or more in 31% of the RCO group and 13% of the TLSO group (p = 0.100). CONCLUSIONS: Patients treated with RCOs compared with Boston-style TLSOs had similar baseline characteristics and brace wear time yet significantly lower rates of spinal surgery. Patients with RCOs also had lower mean and percent major curve progression versus those with TLSOs.

Quantifying Health Status and Function in Marfan Syndrome.

Two hundred thirty patients were prospectively enrolled in this study and completed various portions of the Short Form 36 and a study-specific questionnaire (visual analog scale 1 to 10, comprising three separate questionnaires) to evaluate quality of life and function in patients with Marfan syndrome. The greatest health concern was cardiac problems (high in 70% of patients), followed by spine issues and generalized fatigue (both high, in 53%). The most severe reported pain involved the back: 105 patients (46%) rated pain as 6 to 10 on the visual analog scale. Among the 72 patients who responded to work life questions, work hours were reduced because of treatment in 59 (82%) or directly because of Marfan syndrome in 29 (40%). Across all Short Form 36 domains, patients scored significantly lower than United States population norms (p<.05); physical health scores were considerably lower than mental health scores.

Improving clinical recognition of Marfan syndrome.

BACKGROUND: Marfan syndrome is a potentially fatal disorder with cardiovascular, skeletal, and other manifestations that may also be seen in individuals without Marfan syndrome, making diagnosis difficult. Our goals were (1) to examine the ways in which patients have been recognized as having Marfan syndrome, (2) to examine the prevalence of current diagnostic findings, and (3) to determine which physically evident features are most sensitive and specific for referral to confirm a diagnosis of Marfan syndrome. METHODS: Between 2005 and 2007, we prospectively studied 183 consecutive patients with identified Marfan syndrome (Marfan group) and 1257 orthopaedic patients and family members (non-Marfan group). For the Marfan group, we recorded age at the time of recognition and the methods by which the syndrome was recognized; we used Ghent criteria to identify physically and radiographically evident features. For the non-Marfan group, we examined for Ghent criteria that could be noted on the basis of a routine history, physical examination, or radiographs. We used means, odds ratios, and frequencies to analyze the diagnostic use of each finding (alpha = 0.05). RESULTS: According to the Ghent criteria, 27% of patients in the Marfan group (mean age at the time of diagnosis, 7.3 years) had major skeletal involvement whereas 19% had zero or one skeletal feature. The most common physical features were craniofacial characteristics, high-arched palate, positive thumb and wrist signs, and scoliosis. In the non-Marfan group, 83% had one skeletal feature, 13% had two skeletal features, and 4% had three skeletal features or more. The physical features with the highest diagnostic yield were craniofacial characteristics, thumb and wrist signs, pectus excavatum, and severe hindfoot valgus. CONCLUSIONS: Musculoskeletal clinicians should be aware of the diagnostic features of Marfan syndrome. Patients with three to four physically evident features, or two highly specific features (e.g., thumb and wrist signs, craniofacial features, dural ectasia, or protrusio), should be carefully reexamined and possibly referred for an echocardiogram or a genetics consultation. LEVEL OF EVIDENCE: Diagnostic Level II. See Instructions to Authors for a complete description of levels of evidence.