Shriners Hospitals for Children - Greenville

Clinical decision-making for the management of foot deformities in children is primarily based upon the analysis of weight-bearing radiographs of the foot and ankle. However, a comprehensive quantitative technique for the analysis of such radiographs has not been described. Ten radiographic measurements were developed and applied to the foot and ankle radiographs of a normal foot and ankle in 60 children (mean age 10 years, range 5-17 years). Intraobserver variability and interobserver variability were determined for 10 cases. Mean values for the 10 measurements were calculated from the entire study group. Intraobserver variability was excellent, with correlation coefficients for the 10 measurements ranging from 0.89 to 0.99. The absolute value of the mean differences in angular measurements ranged from 0.8 to 2.5 degrees. Interobserver variability was also excellent, with correlation coefficients ranging from 0.86 to 0.99. The absolute value of the mean differences in angular measurements ranged from 0.5 to 3.2 degrees. The mean values, standard deviations, and ranges for the 10 radiographic parameters from the 60 normal feet have been determined. Clinically acceptable variability of 10 selected radiographic measurements of the foot and ankle was achieved, and normal values and ranges for these measurements were determined. Quantitative segmental analysis of foot and ankle alignment, using these 10 radiographic measurements, can be used to describe common malalignment patterns, and this may assist in clinical decision-making and assessment of outcome.

BACKGROUND: The Shriners Hospital for Children Upper Extremity Evaluation (SHUEE) is a video-based tool for the assessment of upper extremity function in children with hemiplegic cerebral palsy. This tool includes spontaneous functional analysis and dynamic positional analysis and assesses the ability to perform grasp and release. The purpose of the present study was to assess the reliability, concurrent validity, and construct validity of this instrument. METHODS: The Shriners Hospital for Children Upper Extremity Evaluation studies for eleven subjects with hemiplegic cerebral palsy were selected for the evaluation of intraobserver and interobserver reliability. Concurrent validity was determined through analysis of the Shriners Hospital for Children Upper Extremity Evaluation, Pediatric Evaluation of Disability Inventory, and Jebson-Taylor Test of Hand Function scores for twenty children. Construct validity was determined through analysis of Shriners Hospital for Children Upper Extremity Evaluation scores for eighteen children before and after flexor carpi ulnaris to extensor carpi radialis brevis tendon transfer. RESULTS: The absolute mean differences between the two scoring sessions for three raters were 1.2 and 1.0 for the spontaneous functional analysis and the dynamic positional analysis, respectively. Although the mean differences were significantly different from 0 (p < 0.001 and p = 0.003), the differences were small and not clinically important with regard to the total possible score. There was excellent intraobserver reliability between the two sessions with regard to both spontaneous functional analysis (r = 0.99) and dynamic positional analysis (r = 0.98). Assessment of interobserver reliability revealed absolute mean differences between four raters of 3.8 and 3.7 for the spontaneous functional analysis and the dynamic positional analysis, respectively. These differences were significantly different from 0 (p < 0.001); however, the magnitudes of these differences were not important with regard to total score or clinical interpretation. There was excellent interobserver reliability for both the spontaneous functional analysis (r = 0.90) and the dynamic positional analysis (r = 0.89). There was 100% agreement within and between examiners for the grasp-and-release section. The Shriners Hospital for Children Upper Extremity Evaluation showed fair correlation with the self-care scaled score from the Pediatric Evaluation of Disability Inventory (r = 0.47) and good inverse correlation with the non-dominant total time section of the Jebson-Taylor test (r = -0.76). The Shriners Hospital for Children Upper Extremity Evaluation wrist score improved for all eighteen subjects after the flexor tendon transfer, and the mean improvement was significant (p < 0.001). CONCLUSIONS: The present study establishes the clinical reliability, concurrent validity, and construct validity of the Shriners Hospital for Children Upper Extremity Evaluation for the assessment of upper extremity function in children with hemiplegic cerebral palsy.

A new paradigm based on an appreciation of the biomechanics of normal and pathologic gait and a better understanding of muscle-tendon unit anatomy and physiology has emerged for orthopaedic clinical decision making to optimize the ambulatory abilities of children with cerebral palsy. This quantitative, biomechanically based approach has been accepted as a research and teaching tool and as an instrument of outcome assessment; however, controversy remains concerning the expense of using this approach and about its accuracy and repeatability. This paradigm is used within a diagnostic matrix consisting of five data sources. Members of the clinical and technical teams from the motion analysis laboratory interpret data from the clinical history, physical examination, diagnostic imaging, quantitative gait analysis, and examination under anesthesia. The certainty of intervention selection is proportional to the consistency of the data within the diagnostic matrix. When inconsistencies in the data exist, input from both the clinical and technical teams is needed to resolve discrepancies. Working within the framework of the diagnostic matrix, it is possible to identify the indications used in the selection and recommendation of musculoskeletal surgical interventions to optimize gait in children with cerebral palsy. It is important to examine indications and controversies for surgical intervention related to iliopsoas recession, femoral rotational osteotomy, medial hamstring lengthening, rectus femoris transfer, and gastrocnemius recession.

Background: The use of magnetic resonance imaging has led to the diagnosis of abnormalities of the central nervous system associated with apparent idiopathic scoliosis. The indications for magnetic resonance imaging for presumed adolescent idiopathic scoliosis have not been established. Methods: One thousand, two hundred and eighty children with presumed adolescent idiopathic scoliosis were evaluated over a ten-year period. Magnetic resonance imaging of the central nervous system (brainstem and spinal cord) was performed for specific patients, on the basis of the presence of selected indicators determined from the clinical history, physical examination, and plain radiographic examination of the spine. The medical records were reviewed to determine the specific indicators, the results of the imaging studies, and the subsequent treatment. Results: Magnetic resonance imaging was ordered for 274 (21%) of the 1280 children who were evaluated. Abnormal findings were seen in twenty-seven (10%) of the 274 patients who underwent imaging, or 2% of the entire cohort. The most valuable single indicator of an abnormal finding on magnetic resonance imaging was absence of thoracic apical segment lordosis: eight of thirty-nine patients with that indicator had an abnormal finding on magnetic resonance imaging. The optimal diagnostic yield for a single category of indicators occurred when an atypical curve pattern was the only indicator: six of fifty-eight patients in whom this was the case had an abnormal finding on magnetic resonance imaging. None of the twenty children in whom pain was the only indicator category had an abnormal imaging study. The optimal diagnostic yield occurred when both an atypical curve pattern and neurological indicators were present: thirteen (25%) of fifty-three patients in whom this was the case had an abnormal finding on magnetic resonance imaging. Thirteen of the twenty-seven patients received surgical treatment for the abnormality of the central nervous system revealed by the imaging. Conclusions: The correct use of diagnostic tests is an important component of effective medical practice. An abnormality of the central nervous system is present in approximately 10% of patients with presumed adolescent idiopathic scoliosis in whom only subtle abnormalities are identified on the basis of the clinical history, physical examination, or radiographic examination. Knowledge of the diagnostic value of the specific clinical indicators, considered individually and in combination, can help the clinician to determine more effectively when advanced imaging of the central nervous system should be performed. Level of Evidence: Diagnostic study, Level III-1 (study of nonconsecutive patients [no consistently applied reference “gold” standard]). See Instructions to Authors for a complete description of levels of evidence.

Tendon adhesion is acknowledged to be a function of both an overwhelming inflammatory response at the surgical site and the loss of physical separation that is normally present between the tendons and the synovial sheath. Adhesions bind the flexor tendons to each other and to surrounding structures, interfering with their normal gliding function. The clinical result of adhesion formation following flexor tendon surgery is poor digital function. This study investigated the effect of intraoperative treatments of high viscosity absorbable gels made of various combinations of hyaluronic acid and nonsteroidal anti-inflammatory drugs, on adhesion formation in a leghorn chicken flexor tendon model. Forty-eight mature, white leghorn chickens were used to verify the surgical model and to test five different gel treatments. The gels were formed from: 2% sodium hyaluronate in phosphate buffered saline alone or combined with 1 mg/mL tolmetin sodium; 1 mg/mL naproxen sodium; 0.216 g/mL calcium acetate; or 0.216 g/mL calcium acetate plus 1 mg/mL naproxen sodium. The gels were applied by injecting 0.2 mL of the specified composition into the intrasheath space near the conclusion of the surgical procedure. Gross and histological evaluations were conducted to analyze the efficacy. All of the treatments significant reduced the extent and severity of postsurgical tendon adhesion in this animal model as compared with the control (no gel treatment) (p < 0.05). The combination of naproxen sodium and calcium acetate in a high viscosity sodium hyaluronate carrier was the most effective composition. The combination of a high viscosity gel and nonsteroidal anti-inflammatory drugs appears to maintain the natural separation between the tendons and their sheaths and decrease the tissue inflammatory response through mediating two of the major stimuli in adhesion formation.

BACKGROUND: According to the most recent data, an estimated 17.1% of children in the United States are obese. We found no published studies documenting the prevalence of obesity in ambulatory children with cerebral palsy. The purpose of this study was to document the prevalence of obesity in ambulatory children with cerebral palsy and examine the trend in this measure over the last decade. METHODS: A retrospective review was performed to analyze the age, gender, height, weight, physical classification of the cerebral palsy, and functional level as determined with the Gross Motor Function Classification System (GMFCS) of all children with cerebral palsy who had a gait analysis performed in the Motion Analysis Laboratory of our institution between January 1994 and December 2004. This information was used to determine the prevalence of obesity (a body mass index in or above the 95th percentile of the sex-specific body mass index-for-age growth chart) in this population and its relationship to age, gender, the physical classification of the cerebral palsy, and the GMFCS level. RESULTS: When the data were grouped into three time periods (1994 to 1997, 1998 to 2002, and 2003 to 2004), a significant increase in obesity over time was noted (p = 0.017). The prevalences increased from 7.7% to 14% to 16.5% in the respective time periods. The prevalence increased over time in both males and females, those with hemiplegia and those with diplegia, and those with level-I function and those with level-II function according to the GMFCS. The association between obesity and time was significant in the female (p = 0.015), hemiplegic (p = 0.049), less than eight-year-old (p = 0.020), and GMFCS level-II (p = 0.003) groups. We found that the time period was independently associated with obesity when we controlled for age, type of cerebral palsy, and GMFCS level (p = 0.014). Children with a lesser degree of involvement (GMFCS level II) had twice the odds of becoming obese than did children with greater involvement (GMFCS level III). CONCLUSIONS: The prevalence of obesity in ambulatory children with cerebral palsy has risen over the last decade from 7.7% to 16.5%, an increase that is similar to that seen in the general pediatric population in the United States. This finding may have a major impact on the general health and functional abilities of these children as they reach adult life.

The remodeling potential of angular deformities in the coronal and sagittal planes in children is widely appreciated, and has been studied extensively. The short-term clinical consequences and remodeling potential of torsional deformity after fracture of the femur are less well understood. Computerized axial tomography (CT) was used to identify and follow children with torsional malunion after standard treatment (traction/spica cast) of a closed femur fracture. Seven fracture patients with torsional differences greater than 10° were selected and observed. Computed axial tomography scans were performed at the time of spica cast removal and at the one-year follow-up examination. Four cases showed diminished femoral torsion (one case retroversion), and three cases showed increased femoral torsion. The deformity ranged from 13° to 38°. Follow-up CTs one year later showed no significant correction of deformity. Clinically, deformity of up to 25° was well tolerated. The only symptomatic patient (38° malunion) had a third CT done 28 months after injury, which showed no further correction of deformity. This study confirms the poor remodeling potential of significant posttraumatic torsional deformity of the femur in children.

PURPOSE: The differential diagnosis in children who walk on their toes includes mild spastic diplegia and idiopathic toe walking (ITW). A diagnosis of ITW is often one of exclusion. To better characterize the diagnosis of ITW, quantitative gait analysis was utilized in a series of patients with an established diagnosis of ITW. STUDY DESIGN: Patients with an established diagnosis of ITW were analyzed by quantitative gait analysis. Data were recorded as each subject walked in a self-selected toe-walking pattern. The subject was then asked to ambulate making every effort to walk in a normal heel-toe reciprocating fashion. Data were collected to determine if this group of idiopathic toe walkers was able to normalize their gait. Datasets were compared with each other and with historical normal controls. RESULTS: Fifty-one neurologically normal children (102 extremities) with ITW were studied in the Motion Analysis Laboratory at a mean age of 9.3 years. In the self-selected trials, significant deviations in both kinematics and kinetics at the level of the ankle were identified. Disruption of all 3 ankle rockers and a plantar flexion bias of the ankle throughout the gait cycle were most commonly seen. When asked to attempt a normal heel-toe gait, 17% of the children were able to normalize both stance and swing variables. In addition, 70% were able to normalize some but not all of the stance and swing variables. CONCLUSION: Quantitative gait analysis is an effective tool for differentiating mild cerebral palsy from ITW. Kinematic and kinetic distinctions between the diagnoses are evident at the knee and ankle. The ability to normalize on demand at least some of the kinematic and kinetic variables associated with toe walking is seen in most children with ITW.

STUDY DESIGN: A retrospective radiographic review was performed on 41 patients with scoliosis associated with a Chiari I malformation and/or syringomyelia. OBJECTIVES: To characterize curve patterns and curve features in this population and possibly refine the radiographic indications for magnetic resonance imaging in patients with a normal history and physical examination. SUMMARY OF BACKGROUND DATA: A subset of patients with "idiopathic" scoliosis may have an underlying neurologic abnormality. The radiographic indications for magnetic resonance imaging in asymptomatic patients with a normal clinical examination are not well defined. METHODS: Data were collected from standing posteroanterior and lateral radiographs. The curve pattern and specific curve features were recorded and compared with historic controls. Thoracic kyphosis and total lumbar lordosis were also measured. RESULTS: Fifty-one percent of patients were male. Ten curve patterns were identified, and, based on our criteria, approximately 50% of patients had an "atypical" pattern (left thoracic, double thoracic, triple, long right thoracic). A subset of those with "typical" patterns (right thoracic, right thoracic/left lumbar) had atypical features including a superior or inferior shift of the apex and/or the upper or lower end vertebrae. The mean kyphosis (T3-T12) was 41.8 degrees. CONCLUSIONS: Although the decision to obtain magnetic resonance imaging in a patient with scoliosis should be based on both clinical and radiographic criteria, we suggest that a heightened index of suspicion is warranted with certain curve patterns (left thoracic, double thoracic, triple, and a long right thoracic curve with end vertebra caudal to T12), and with a high or low apex and/or end vertebra, especially in males and patients with a normal to hyperkyphotic thoracic spine.

BACKGROUND: Cubitus varus deformity is the most common complication after the treatment of displaced supracondylar humerus fractures. METHODS: A retrospective analysis was performed evaluating patients who had undergone a translation step-cut osteotomy for correction of cubitus varus deformity between 1993 and 2008. Postoperative radiographs to union were reviewed for all patients. Subjective information and range of motion measures were documented from the medical records. Patients and their families were also contacted through telephone and administered a questionnaire as well as the QuickDash functional outcome measure to assess their function and satisfaction. RESULTS: Thirty-seven participants were identified and reviewed. Average age at the time of surgery was 8 years and 10 months, with an average follow-up of 2 years and 4 months. Outcomes were assessed in terms of technical, functional, and satisfaction domains. Technical Domain: Average humeroulnar angle (radiographic carrying angle) correction was 26 degrees. Bauman's angle improved 21 degrees on average in patients who were skeletally immature. Functional Domain: Elbow range of motion was maintained in all planes with few exceptions. The clinical carrying angle improved 26 degrees on average, and was symmetric with the unaffected side in 22 of 25 cases in which it was documented. The QuickDash was applied to 15 participants. Eight participants had perfect scores for the symptoms/disability section, and 7 had scores in the top 20% for function. Satisfaction Domain: A study-specific questionnaire was applied to 16 participants. All patients and parents questioned would recommend the procedure to other parents/patients. CONCLUSION: The translation step-cut osteotomy reliably corrects the coronal plane angular deformity associated with cubitus varus, resulting in excellent outcomes in technical, functional, and satisfaction domains. The osteotomy is relatively simple to perform, and is inherently more stable than other proposed methods. It also minimizes the prominence of the lateral condyle associated with simple lateral closing wedge osteotomy.

Seventy-nine consecutive children with cerebral palsy who underwent osteotomies about the hip for subluxation or dislocation were studied retrospectively to determine risk factors that would correlate with postoperative complications of death, fracture, or decubitus ulcer. Except for the three patients who died, all of the children had > or = 1 year of follow-up. Twenty (25%) patients had at least one complication. Three children died; one at 1 week, one at 2 weeks, and one at 5 months after surgery. Sixteen patients sustained 25 fractures. All were managed with cast or splint immobilization in the clinic. Five patients developed decubitus ulcers requiring > or = 2 weeks of local care, but none required skin grafts or flaps. Complications occurred in 13 (68%) of 19 children with gastrostomies or tracheostomies but in only seven (12%) of the remaining 60 children. Only one (8%) of 13 ambulatory patients had a complication compared with 19 (29%) of 66 nonambulatory patients. In conclusion, ambulatory function correlates well with the risk of complications after osteotomies. A nonambulatory patient with a gastrostomy or tracheostomy is at even greater risk. Fortunately the fractures and ulcers observed in this series healed uneventfully with no operative intervention.

BACKGROUND: The floor-reaction ankle-foot orthosis is commonly prescribed for children with cerebral palsy who walk with excessive ankle dorsiflexion and excessive knee flexion during the stance phase of gait. The purposes of this study were to evaluate the efficacy of this orthosis objectively and to identify clinical parameters that may compromise its function. METHODS: All children with cerebral palsy who had comprehensive gait analyses in both barefoot and braced walking conditions during a single visit to our Motion Analysis Laboratory between January 2001 and August 2007 were identified. Kinematic study parameters included mean sagittal dynamic range of motion of the ankle in stance, peak ankle dorsiflexion in stance, peak knee extension in midstance, and mean foot progression angle in stance. The minimum sagittal knee moment in midstance was also examined in this study for subjects who walked without assistive devices. Range-of-motion and skeletal alignment data obtained from the physical examination record of each subject included knee flexion contracture, popliteal angle, hip flexion contracture, and thigh-foot angle. RESULTS: Twenty-seven children had quantitative gait analyses (barefoot and with the orthoses in the same visit). The mean sagittal plane dynamic range of motion of the ankle in stance was reduced from 23 degrees +/- 9 degrees when walking barefoot to 10 degrees +/- 3 degrees when the orthosis was worn (p < 0.001), and the mean peak knee extension in midstance improved from 29 degrees +/- 14 degrees of flexion to 18 degrees +/- 14 degrees of flexion (p = 0.013). Strong negative linear correlations were found between the magnitude of knee and hip flexion contractures on physical examination and the amount of peak knee extension in midstance (r = -0.784 and r = -0.705, respectively). A strong positive correlation was found between the mean minimum sagittal knee moment in midstance and the amount of peak knee extension in midstance (r = 0.820). Our investigation did not provide evidence of a correlation between peak knee extension in midstance and any of the following parameters in the orthosis: clinical examination measurements of the thigh-foot angle (r = 0.120), the popliteal angle (r = -0.300), or the mean foot progression angle in the stance phase of gait (r = -0.188). CONCLUSIONS: The floor-reaction ankle-foot orthosis is effective in restricting sagittal plane ankle motion during the stance phase of gait in patients with cerebral palsy. As a result, improvements in knee extension and the sagittal plane knee extensor moment in stance phase are achieved. The best outcomes with this orthosis, as determined by peak knee extension in midstance, were seen in the subjects with knee and hip flexion contracture of < or =10 degrees . Knee and hip flexion contractures of > or =15 degrees were found to limit the efficacy of the orthosis in controlling knee extension in midstance. Such contractures should be considered as contraindications to the prescription of this orthosis or should be addressed (surgically or otherwise) prior to the application of a floor-reaction ankle-foot orthosis in these patients.

Clinical assessment of femoral anteversion (FA) in children with cerebral palsy (CP) is frequently determined by the trochanteric prominence angle test (TPAT). Limited three-dimensional volumetric imaging by axial tomography of the femur was performed before surgery for 35 hips in 20 children with CP. The TPAT was performed before the imaging study for 31 hips in 18 children. The TPAT angle was within 10 degrees of the FA as determined from the computed tomography scans (Murphy technique) for 17 femurs (55%). The most prominent portion of the greater trochanter was located anterior to the femoral neck axis (mean 27 degrees, range 0 degrees-52 degrees) on the three-dimensional images in 34 of 35 hips. A simulated TPAT, measured from the imaging studies, consistently underestimated the FA as determined by the Murphy technique (mean 10 degrees, range 0 degrees-18 degrees). Accurate clinical assessment of FA by the TPAT in children with CP presumes that the prominence of the greater trochanter lies perpendicular to the axis of the femoral neck. Three-dimensional imaging showed the prominence to be anterior, to a variable degree, to the femoral neck axis, which in addition to clinical factors such as obesity compromises the accuracy of this clinical maneuver.

BACKGROUND: Clinicians interested in assessment and outcome measurement of upper extremity (UE) function and performance in children with cerebral palsy (CP) must choose from a wide range of tools. QUESTIONS/PURPOSES: We systematically reviewed the literature for UE assessment and classification tools for children with CP to compare instrument content, methodology, and clinical use. METHODS: We searched Health and Psychosocial Instruments (HaPI), US National Library of Medicine (PubMed), and Cumulative Index to Nursing and Allied Health Literature (CINAHL Plus) databases (1937 to the present) to identify UE assessment and outcomes tools. We identified 21 tools for further analysis and searched HaPI, PubMed, CINAHL Plus, and Google Scholar ( http://scholar.google.com/schhp?tab=ws ) databases to identify all validity and reliability studies, systematic reviews, and original references for each of the 21 tools. RESULTS: The tools identified covered ages birth to adulthood. International Classification of Functioning, Disability and Health domains addressed by these tools included body function, body structure, activities and participation, and environmental factors. Eleven of the tools were patient or family report, seven were clinician-based observations, and three tools could be used in either fashion. All of the tools had published evidence of validity. Nine of the tools were specifically designed for use in subjects with CP. Two of the tools required formal certification before use. Ten of the tools were provided free of charge by the investigators or institution who developed them. CONCLUSIONS: Familiarity with the psychometric and clinometric properties of assessment and classification tools for the UE in children with CP greatly enhances a clinician's ability to select and use these tools in daily clinical practice for both clinical decision-making and assessment of outcome.

Seventy-nine consecutive children with cerebral palsy who underwent osteotomies about the hip for subluxation or dislocation were studied retrospectively to determine risk factors that would correlate with postoperative complications of death, fracture, or decubitus ulcer. Except for the three patients who died, all of the children had ≥1 year of follow-up. Twenty (25%) patients had at least one complication. Three children died; one at 1 week, one at 2 weeks, and one at 5 months after surgery. Sixteen patients sustained 25 fractures. All were managed with cast or splint immobilization in the clinic. Five patients developed decubitus ulcers requiring ≥2 weeks of local care, but none required skin grafts or flaps. Complications occurred in 13 (68%) of 19 children with gastrostomies or tracheostomies but in only seven (12%) of the remaining 60 children. Only one (8%) of 13 ambulatory patients had a complication compared with 19 (29%) of 66 nonambulatory patients. In conclusion, ambulatory function correlates well with the risk of complications after osteotomies. A nonambulatory patient with a gastrostomy or tracheostomy is at even greater risk. Fortunately the fractures and ulcers observed in this series healed uneventfully with no operative intervention.

A multicenter study on fibrous dysplasia of bone (FD) was promoted by the European Pediatric Orthopaedic Society in 1999 in order to gain insight into the natural history of the disease and to evaluate current diagnostic and therapeutic approaches. We collected and reviewed clinical, radiographic, pathological, and molecular genetic data when possible, from a total of 64 cases diagnosed as either monostotic FD (MFD), polyostotic FD (PFD), or McCune–Albright syndrome (MAS), evaluated or treated in 11 participating centers. Results from the initial analysis of the series indicate five main points: (1) Significant diagnostic pitfalls affect the diagnosis of MFD and, to a lesser extent, PFD in orthopedic centers and allied radiology and pathology facilities, which may be circumvented by the adoption of stringent diagnostic criteria, and in some cases by the analysis of FD-associated GNAS1 mutations. (2) MFD carries a significant risk for fracture in the face of limited disease in the proximal femur, whereas its tendency to progress is restricted to a minority of cases, and long-term outcome is usually satisfactory, regardless of treatment, in non-progressive cases. (3) The profile of tibial disease, both in MFD and in PFD, is markedly different from that of femoral disease. (4) As expected, MAS patients have the most extensive disease and the most complicated course, regularly experience multiple fractures, and require adequate surgical treatment. It appears that conservative treatment of femoral fracture, or curettage and cancellous bone grafting, or fixation with screws and plates are not indicated for the treatment of femoral fractures in these patients and should all be discouraged. Internal fixation with intramedullary nails provides stabilization of extensively affected bones, and prevents further fractures and major deformities, and thus providing a better option both for acute and elective surgery in patients with extensive involvement of the femur or of other limb long bones. (5) Evaluation of patients with FD at orthopedic centers should include, but rarely does, a thorough evaluation of endocrine profile and phosphate metabolism, and proper pathological and radiographic assessment.

Surgical management of toe-walking gait in children with cerebral palsy currently favors simultaneous, multilevel soft-tissue and bony interventions. Formulation of such a surgical plan is based on our ability to determine which of the gait deviations present are primary and which are secondary or compensatory. To evaluate this issue further, 32 normal children, walking normally and voluntarily toe-walking, were compared to 15 children with cerebral palsy walking in an obligatory toe-walking gait pattern. Computer-based analysis of gait was performed for each child, including time-distance, kinematic, kinetic, and electromyographic analyses. Significant deviations common to both normal and cerebral palsy toe-walking groups were determined to be due, at least in part, to the biomechanical constraints associated with a toe-walking gait pattern. Deviations unique to the cerebral palsy group were thought to represent primary gait deviations related to the underlying injury to the central nervous system. This study identifies the need to develop more sophisticated techniques of data collection and analysis and supports the inclusion of more varied and demanding functional activities for distinguishing between primary and secondary gait deviations in children with cerebral palsy.

Background: Assessment of femoral anteversion in children with cerebral palsy with two or three-dimensional computed tomography scans may be limited by both positional and anatomic variables. Three-dimensional computed tomography techniques are considered to be more accurate than two-dimensional imaging when the femur is not optimally positioned in the gantry or when the neck-shaft angle is increased. Methods: Computed tomography scanning was performed on a series of nine model femora with anteversion ranging from 20° to 60° and neck-shaft angles ranging from 120° to 160°. Each femoral model was scanned in two holding devices, the first of which held the femur in optimal alignment (normal model) and the second of which held the femur in flexion, adduction, and internal rotation (cerebral palsy model) relative to the gantry. Femoral anteversion was calculated for each model from two and three-dimensional computed tomography scans by four examiners on two separate occasions. The intraobserver and interobserver reliability, the accuracy, and the effect of increasing the neck-shaft angle on the accuracy of the measurements made on the two and three-dimensional scans of the normal and cerebral palsy models were then examined. Results: The mean differences in the measurements of femoral anteversion made by the same examiner (intraobserver reliability) were <2° for the two-dimensional scans of the normal and cerebral palsy models and the three-dimensional scans of the normal models, and the mean difference was <4° for the three-dimensional scans of the cerebral palsy models. The mean differences among examiners (interobserver reliability) were <3° for the two-dimensional scans of the normal and cerebral palsy models and the three-dimensional scans of the normal models, and the mean difference was <6° for the three-dimensional scans of the cerebral palsy models. The accuracy of the assessments of femoral anteversion of the normally aligned models was comparable between the two and three-dimensional scans. However, the three-dimensional assessment was significantly more accurate than the two-dimensional assessment for measurement of anteversion of the cerebral palsy models (p = 0.003). Accuracy within 5° was comparable between the two and three-dimensional scans for measurement of the normally aligned models, with 86% of the two-dimensional measurements and 78% of the three-dimensional measurements falling within 5° of the actual measurements. However, the accuracy within 5° was significantly compromised when the models were placed in cerebral palsy alignment. Only 3% of the two-dimensional measurements and 14% of the three-dimensional measurements fell within 5° of the actual measurements, with three-dimensional assessment being significantly better than two-dimensional assessment (p = 0.006). Increasing the neck-shaft angle did not significantly compromise the accuracy of measurement of femoral anteversion with either the two-dimensional or the three-dimensional technique (p > 0.05 for all comparisons). Conclusions: When adequate alignment of the femur in the computed tomography scanner was possible, a simple two-dimensional technique exhibited excellent intraobserver and interobserver reliability and clinically acceptable accuracy within the relevant ranges of anatomic variability tested (neck-shaft angles of 120° to 160° and femoral anteversion of 20° to 60°). When optimal alignment of the femur in the scanner was not possible, neither two-dimensional nor three-dimensional techniques exhibited clinically acceptable accuracy for the measurement of femoral anteversion.

STUDY DESIGN: A retrospective review of 15 patients with Down syndrome who had undergone arthrodesis of the upper cervical spine for instability. OBJECTIVES: To determine the complication rate and long-term outcome after posterior cervical arthrodesis for upper cervical instability in patients with Down syndrome. SUMMARY OF BACKGROUND DATA: Atlantoaxial instability is common in patients with Down syndrome, and fusion of the upper cervical spine has been recommended for patients who have instability, with or without myelopathy. Unfortunately, the results of posterior cervical arthrodesis are not well reported, and the natural history of this condition is unknown. METHODS: Fifteen patients with an average follow-up period of 74.6 months (range, 24-142 months) were reviewed after posterior arthrodesis of the upper cervical spine. Twelve patients were reexamined by the investigators specifically for the purpose of this study, and three patients had long-term follow-up results available from chart review. RESULTS: Eleven of 15 patients (73%) sustained 23 major complications including nonunion, loss of reduction, neurologic deterioration, late subaxial instability, infection, and wound dehiscence. Six patients (40%) required seven reoperations to address a complication. Ultimately, 12 patients (80%) obtained osseous union, but a definite clinical improvement was identifiable in only three patients, whereas two others had worsened neurologically at latest follow-up evaluation. CONCLUSIONS: A high complication rate should be anticipated after posterior arthrodesis of the upper cervical spine in patients with Down syndrome. A cautious approach to asymptomatic instability in this condition is advocated.

Surgical management of toe-walking gait in children with cerebral palsy currently favors simultaneous, multilevel soft-tissue and bony interventions. Formulation of such a surgical plan is based on our ability to determine which of the gait deviations present are primary and which are secondary or compensatory. To evaluate this issue further, 32 normal children, walking normally and voluntarily toe-walking, were compared to 15 children with cerebral palsy walking in an obligatory toe-walking gait pattern. Computer-based analysis of gait was performed for each child, including time-distance, kinematic, kinetic, and electromyographic analyses. Significant deviations common to both normal and cerebral palsy toe-walking groups were determined to be due, at least in part, to the biomechanical constraints associated with a toe-walking gait pattern. Deviations unique to the cerebral palsy group were thought to represent primary gait deviations related to the underlying injury to the central nervous system. This study identifies the need to develop more sophisticated techniques of data collection and analysis and supports the inclusion of more varied and demanding functional activities for distinguishing between primary and secondary gait deviations in children with cerebral palsy.