The efficacy of neuroprosthesis in young hemiplegic patients, measured by three different gait indices: early results.

PURPOSE: To evaluate functional electrical stimulation (FES) neuroprothesis as a method to improve gait in hemiplegic patients, using three different gait scoring methods as measures. METHODS: Five hemiplegic patients (four with cerebral palsy at GMFCS I, one with diffuse pontine glioma) with a mean age of 16.5 years were given a FES neuroprosthesis (NESS(®) L300™) that was applied and calibrated individually. After an adaptation period during which the participants increased their daily use of the neuroprosthesis, gait was assessed with the stimulation off and with the FES on. Kinematic, kinetic, and temporal spatial data were determined using motion analysis and summarized by three scoring methods: Gait Profile Score (GPS), Gait Deviation Index (GDI), and Gillette Gait Index (GGI). Indices were calculated using the Gaitabase program available online. Patients were followed for a minimum of 1 year. RESULTS: When comparing gait with and without stimulation, all scoring methods showed improvement. GPS and GDI of the affected leg were significantly improved: 12.23-10.23° (p = 0.017) and 72.36-78.08 (p = 0.002), respectively. By applying the movement analysis profile, the decomposed GPS score, we found that only the ankle dorsiflexion and the foot progression angle were significantly changed. GGI of the affected leg showed improvement, but without statistical significance: 168.88-131.64 (p = 0.221). Total GPS of legs and the GPS, GDI, and GGI of the nonaffected leg showed improvement without statistical significance. At the 1-year follow-up, all patients expressed high satisfaction and continued to use the device. CONCLUSIONS: Dorsiflexion functional electrical stimulation improves gait in hemiplegic patients, as reflected by GPS, GDI, and GGI.

Intra- and interobserver reliability analysis of digital radiographic measurements for pediatric orthopedic parameters using a novel PACS integrated computer software program.

BACKGROUND: The between-observer reliability of repeated anatomic assessments in pediatric orthopedics relies on the precise definition of bony landmarks for measuring angles, indexes, and lengths of joints, limbs, and spine. We have analyzed intra- and interobserver reliability with a new digital measurement system (TraumaCad Wizard™). METHODS: Five pediatric orthopedic surgeons measured 50 digital radiographs on three separate days using the TraumaCad system. There were 10 anterior-posterior (AP) pelvic views from developmental dysplasia of the hip (DDH) patients, 10 AP pelvic views from cerebral palsy (CP) patients, 10 AP standing view of the lower limb radiographs from leg length discrepancy (LLD) patients, and 10 AP and 10 lateral spine X-rays from scoliosis patients. All standing view of the lower limb radiographs were calibrated by the software to allow for accurate length measurements, using as reference a 1-inch metal ball placed at the level of the bone. Each observer performed 540 measurements (totaling 2,700). We estimated intra- and interobserver standard deviations for measurements in all categories by specialists and nonspecialists. The intraclass correlation coefficient (ICC) summarized the overall accuracy and precision of the measurement process relative to subject variation. We examined whether the relative accuracy of a measurement is adversely affected by the number of bony landmarks required for making the measurement. RESULTS: The overall ICC was >0.74 for 13 out of 18 measurements. Accuracy of the acetabular index for DDH was greater than for CP and relatively low for the center-edge angle in CP. Accuracy for bone length was better than for joint angulations in LLD and for the Cobb angle in AP views compared to lateral views for scoliosis. There were no clinically important biases, and most of the differences between specialists and nonspecialists were nonsignificant. The correlation between the results according to the number of bony landmarks that needed to be identified was also nonsignificant. CONCLUSIONS: Digital measurements with the TraumaCad system are reliable in terms of intra- and interobserver variability, making it a useful method for the analysis of pathology on radiographs in pediatric orthopedics.

Long-term effect of repeated injections of botulinum toxin in children with cerebral palsy: a prospective study.

PURPOSE: To prospectively evaluate long-term effects of repeated botulinum toxin A (BTX-A) injections in children with cerebral palsy (CP). METHODS: Repeated injections of BTX-A were offered to children with CP, according to clinical indications, for a maximum of four injections within a period of two years. Injections were administered into lower extremity muscles of 26 consecutive children (age 3.7 +/- 1.2 years, 16 boys) with hemiplegic or diplegic CP. Clinical assessments before and one month following each injection included a gross motor function measure (GMFM), a modified Ashworth scale, and range-of-motion of knee extension and ankle dorsiflexion. RESULTS: Twelve children received two injections, six received three injections, five received one injection, and three received four injections. The most common reason for discontinuing treatment was the need for orthopedic surgery (n = 17). A long-term effect was demonstrated by a significant increase of the GMFM score before the first injection compared with the last injection for each patient (P < 0.0001). There was no comparable change in the muscle tone or range-of-motion. The mean rate of GMFM change during the study was significantly higher than literature norms for CP children (13.2 vs. 5.37 per year, respectively, P < 0.01). The increase of the GMFM score before and one-month after injection (short-term effect) was significantly higher after the first injection than after the last injection (P < 0.05). Similar results were found for the Ashworth scale and popliteal angle. CONCLUSIONS: Botulinum toxin A injections have a long-term effect on gross motor function in children with CP even though the effect on muscle tone is short-term. The effect apparently declines with repeated injections, with most children benefitting from 2 to 3 injections.

Treatment of severe late-onset Perthes' disease with soft tissue release and articulated hip distraction: revisited at skeletal maturity.

PURPOSE: Late-onset Perthes' disease is diagnosed after 9 years of age. Conservative treatment and conventional surgical techniques have limited ability to reduce the pressure in the joint or change the shape of the femoral head. We used a combination of soft tissue release and joint distraction with a hinged mono-lateral external fixator for these patients. Ten of our patients reached skeletal maturity and were evaluated. METHODS: Clinical assessment included: Harris hip score, hip range-of-motion (ROM), limb length discrepancy, and the Oxford hip questionnaire for pain and function. Radiographic assessment included: Sharp transverse acetabular inclination, the uncoverage percentage, the epiphyseal index before surgery (modified Eyre-Brook), at frame removal, and, at last follow-up, the epiphyseal quotient (of Sjovall) and the Stulberg classification. RESULTS: Our study included eight boys and two girls (mean age at surgery 12.3 years, range 9.4-15.1, mean age at last follow-up 18.1 years, range 15.2-22.8). The mean follow-up was 5.7 years (range 4.3-7.8). The mean Harris hip score was 86.3/100 (range 48.5-96); one patient had <85 points. The hip ROM was slightly limited in most patients, and seven patients had limb shortening between 1-4 cm. The mean Oxford hip questionnaire score was 17.4/60 (range 12-31). The mean Sharp transverse acetabular inclination of the affected side was 42 degrees (range 36-54) compared to 39 degrees for the unaffected side (P = 0.045). The mean uncoverage percentage was 37% (range 27-47) compared to 20% for the unaffected side (P = 0.017). The mean epiphyseal index was 0.71 (range 0.31-0.92) before surgery, 0.79 (range 0.50-0.93) at frame removal (P = 0.012), and 0.72 (range 0.51-0.89) at last follow-up (P = 0.646). The epiphyseal quotient for the eight unilateral cases was 0.72 (range 0.49-0.91), and the Stulberg classification was type III for three cases and type IV for seven. CONCLUSION: Patient satisfaction for function and pain following the combined procedure was good. Radiographic parameters did not change significantly. This should be regarded as a salvage procedure.

Asymptomatic or minimally symptomatic hyperCKemia: histopathologic correlates.

BACKGROUND: Persistent creatine kinase elevation is occasionally encountered in subjects without any clinical manifestation of a neuromuscular disorder or any condition known to be associated with increased serum CK levels. It is still unresolved whether extensive investigations and specifically a muscle biopsy should be performed in clinically normal individuals with elevated CK levels. OBJECTIVE: To study the muscle pathology of patients with asymptomatic or minimally symptomatic hyperCKemia. METHODS: The clinical and laboratory data of patients with persistent hyperCKemia and normal neurologic examination were reviewed and their muscle biopsies evaluated. RESULTS: The study group included 40 patients aged 7-67 years; the male to female ratio was 3:1. Nineteen patients were completely asymptomatic, 20 had mild non-specific myalgia, and 1 had muscle cramps. Electromyography was performed in 27 patients and showed myopathic changes in 7 (26%). Abnormal muscle biopsy findings (e.g., increased variation in fiber size, increased number of central nuclei, and occasional degenerating fibers) were detected in 22 of the 40 patients (55%). No fat or glycogen accumulation was detected. Immunohistochemistry demonstrated abnormal dystrophin staining in 3 patients (8%), resembling the pathologic changes of Becker muscular dystrophy. No abnormal findings were detected on immunohistochemical staining for merosin, dysferlin, caveolin 3, or alpha and gamma sarcoglycans. The EMG findings did not correlate with the pathologic findings. CONCLUSIONS: Abnormal muscle biopsies were found in 55% of patients with asymptomatic or minimally symptomatic hyperCKemia. Specific diagnosis of muscular dystrophy, however, was possible in only 8% of the patients.

Early experience with the Ponseti method for the treatment of congenital idiopathic clubfoot.

BACKGROUND: The preferred conservative treatment for congenital idiopathic clubfoot deformity remains a controversial issue. OBJECTIVES: To compare the outcomes of two groups of CICF patients who underwent two different treatment protocols. METHODS: The study cohort included 72 infants treated in our hospital from August 1998 to December 2002. Group 1 comprised 40 infants (61 clubfeet) who were treated by a traditional method (a modification of the Kite and Lovell technique), and group 2 consisted of 32 infants (48 clubfeet) treated with the Ponseti technique. Both groups were similar in age, gender and severity of the deformity (Dimeglio scoring system). RESULTS: After an average follow-up of 54.9 months (range 44-68), 35 (57%) clubfeet in group 1 required surgical intervention and 27 (44%) clubfeet had a residual deformity at last follow-up. In the Ponseti group, 45 (94%) clubfeet were fully corrected at last follow-up (average 29.2 months, range 16-45) while 3 (6%) clubfeet had residual deformity and required surgery. Achilles tendon tenotomy was performed with no complications in 47 clubfeet (in group 2) at an average age of 2.4 months (range 2-4 months). CONCLUSIONS: Even after a relatively short follow-up period, our success rate with the Ponseti approach already appears to be significantly higher and to bear fewer complications than the traditional treatment, in agreement with the results published by other medical centers. We now endorse the Ponseti technique of conservative manipulative treatment for congenital idiopathic clubfoot in our department.

Epiphyseal involvement of simple bone cysts.

Epiphyseal involvement of a simple bone cyst (SBC) is uncommon. Eight patients are reported in whom an SBC was found to cross the growth plate, involving the epiphysis in seven patients and the apophysis in one. All patients had more than two pathologic fractures. In seven patients growth disturbance was found. Functional impairment did not develop in any patient. Radiographically, all lesions presented a characteristic involvement of the epiphysis and metaphysis in various proportions. Only one of four cysts treated with methylprednisolone acetate injections showed incomplete healing; the others failed to respond. After percutaneous grafting of autologous bone marrow, three of seven cysts healed and the others attained incomplete healing. Epiphyseal involvement of SBC should be considered a more aggressive form of an active lesion.

Atypical mycobacterial granulomatous epiphyseal osteomyelitis in an immunocompetent child.

Osteomyelitis caused by atypical mycobacteria is rare in children. The majority of affected patients have had some kind of predisposing factor, namely a penetrating injury or surgery, or were immune-compromised. Our experience shows that this diagnosis should be considered in apparently healthy children as well. The use of polymerase chain reaction has now made it possible to identify the pathogen in cases that were previously diagnosed as granulomatous osteomyelitis. We present a case of atypical mycobacterial osteomyelitis affecting the distal femoral epiphysis in an immunocompetent 10-year-old child. The diagnosis in this case was made by the use of the polymerase chain reaction assay.

Primary subacute epiphyseal osteomyelitis: role of conservative treatment.

Primary subacute epiphyseal osteomyelitis is a rare disease. Owing to its insidious onset, mild symptoms, and inconsistent supportive laboratory data, diagnosis and treatment are usually delayed. The authors report a retrospective review of 16 patients with hematogenous osteomyelitis primarily affecting the epiphysis. In all of these patients an osteolytic lesion developed. In eight patients it was confined to the epiphysis or apophysis alone; in the other patients there was contiguous involvement of the adjacent metaphysis. Complete clinical and radiologic healing was observed in all patients after antibiotic therapy alone. Based on this experience, in view of the controversy in the literature, the authors recommend a conservative treatment policy in the management of both epiphyseal and epiphyseal-metaphyseal subacute osteomyelitis as the treatment of choice. Surgery should be reserved for persistent infection that does not respond to appropriate antibiotic therapy or when bone lesions cannot be distinguished from bone tumors by use of all available imaging modalities.

Perthes-like changes in geleophysic dysplasia.

Geleophysic dysplasia is a rare skeletal dysplasia characterised by 'happy natured' facies, short stature with short limbs, brachydactyly, and joint contractures. This report describes a case of a patient with unilateral Legg-Calvé-Perthes-like changes associated with dysplastic proximal capital femoral epiphysis, typical to geleophysic dysplasia.