Prevalence and patterns of scoliosis in children with multiple pterygium syndrome.

BACKGROUND: Although scoliosis is a predominant feature of multiple pterygium syndrome (MPS), the pattern of deformity and the progression of the curvature have not been well described. The purpose of this study was to assess the prevalence of scoliosis among patients with MPS, and to characterize the abnormalities of the vertebrae and to assess the progression of the scoliosis. METHODS: From 1969 to 2008, we identified 19 patients with MPS but only 16 patients (8 boys and 8 girls) had complete data. Medical records and radiographs of these 16 patients were reviewed. Magnetic resonance imaging was performed in 8 patients to evaluate intraspinal anomalies. Functional mobility score was used to assess the ambulatory ability. RESULTS: Of 16 patients, 13 patients (81.3%) had scoliosis. The mean age when the scoliosis was first noticed was 3.3±2.6 years (range, 1 mo to 8.2 y). The mean Cobb angle at first visit was 37.4±18.1 degrees (range, 14.0 to 75.0 degrees). With a mean follow-up of 4.0±4.9 years, the Cobb angle at the last visit was 43.3±19.1 degrees (range, 20.0 to 72.0 degrees). Congenital scoliosis was observed in 7 patients (3 unilateral unsegmented bar, 3 fusion of the cervical spine, 1 block vertebrae), whereas neuromuscular scoliosis was observed in 1 patient. A common radiographic finding was narrowing of the intervertebral disc space with decreased height of vertebrae in the thoracic area. Intraspinal anomalies were seen in 4 patients (3 tethered spinal cords, 1 syrinx). At the last follow-up, 5 of 13 patients who had scoliosis were able to walk at school without assistance (Functional mobility scale-500 ≥5). CONCLUSIONS: Scoliosis is common among children with MPS. It is frequently accompanied by fusion of the cervical area. Intraspinal anomalies such as tethered cord syndrome and syringomyelia are common associated anomalies. Therefore, it is important to look for intraspinal anomalies. Closed monitoring of the patient's ambulatory ability and bowel and bladder continence is also needed. LEVEL OF EVIDENCE: Level IV, Diagnostic Study.

Congenital vertical talus in multiple pterygium syndrome.

BACKGROUND: Congenital vertical talus (CVT) is a rare foot deformity, but it is a commonly associated anomaly in patients with multiple pterygium syndrome (MPS). If left untreated, it can cause pain and morbidity, which will affect the patient's ambulation and quality of life. The aim of this study was to assess the prevalence of CVT among patients with MPS, to characterize the clinical and radiological features and examine the outcome of treatment. METHODS: We reviewed the medical records from 1969 to 2009, and detected 14 patients with a diagnosis of MPS. Data regarding clinical findings, radiographs, associated anomalies, and treatment were collected and analyzed. RESULTS: CVT was seen in 10 of 14 patients (71%). All of them had bilateral involvement. Eight of the 10 (80%) were girls, and 3 of these 10 (30%) were nonambulatory patients. All 7 ambulatory patients had manipulation and casting, followed by a single-stage surgical release. The mean age at surgery was 3.0 ± 3.7 years (range, 3 mo-9 y 2 mo). At the last follow-up, all of the 7 patients (100%) had painless plantigrade feet and a reduced talonavicular joint, and none had recurrence of the deformity. The overall mean follow-up was 6 years (range, 2-19 y) and the mean age at the last follow-up was 9 years (range, 2-23 y). The commonly associated anomalies were scoliosis (93%), tethered cord (14%), hip dislocation (43%), cardiac (29%), respiratory (43%), and gastrointestinal anomalies (29%). CONCLUSIONS: CVT is common in MPS. The other common anomalies included scoliosis, hip dislocation, and respiratory problems. Treatment with manipulation and casting followed by, a single-stage surgical release resulted in a good outcome.

Anterior distal femoral stapling for correcting knee flexion contracture in children with arthrogryposis--preliminary results.

BACKGROUND: Fixed flexion contractures of the knee are more common and disabling than extension contractures in children with arthrogryposis. For correcting these deformities, there are various surgical options such as soft tissue release, distal femoral osteotomy, and frame distraction. We sought in this study to examine the effectiveness of anterior distal femoral stapling using 8-plates for correcting knee flexion contracture in children with arthrogryposis. METHODS: We retrospectively assessed 16 knees in 10 children using clinical and radiographic measures. To determine the outcome, we assessed the Functional Mobility Scale (FMS) as well. Statistically, a paired t test, independent t test, and Wilcoxon signed-rank test were used to analyze the results. RESULTS: After anterior distal femoral stapling, there was a reduction in the flexion deformity of the knee in children with arthrogryposis, P<0.05. There was an estimated 18-degree correction comparing the mean preoperative flexion deformity and the mean postoperative flexion deformity. This correction was significant in children when the knee flexion deformity was less than 45 degrees. The FMS also improved in those patients where the residual flexion contracture was less than 30 degrees at follow-up, suggesting an improvement in their ambulatory capacity, P<0.05. CONCLUSION: Among children with arthrogryposis who present with knee flexion contractures, anterior distal femoral stapling with 8-plates improved their flexion deformity and ambulatory capacity. This technique is less invasive than soft tissue releases, distal femoral osteotomy, or frame distraction and is most rewarding in children with arthrogryposis whose flexion contractures is less than 45 degrees.

Passive exoskeletons for assisting limb movement.

This article presents the state of the art in passive devices for enhancing limb movement in people with neuromuscular disabilities. Both upper- and lower-limb projects and devices are described. Special emphasis is placed on a passive functional upper-limb orthosis called the Wilmington Robotic Exoskeleton (WREX). The development and testing of the WREX with children with limited arm strength are described. The exoskeleton has two links and 4 degrees of freedom. It uses linear elastic elements that balance the effects of gravity in three dimensions. The experiences of five children with arthrogryposis who used the WREX are described.