Faculty Bibliography

Flatfoot in a child may be normal before development of the arch, but the prevalence decreases with age. Treatment is indicated only in the presence of pain and should begin with nonsurgical management options such as stretching of the Achilles tendon and the use of soft shoe orthotics. If pain persists, a modified Evans procedure, together with additional procedures to address forefoot supination, can be successful in correcting deformity and addressing pain. A thorough understanding of the pathology and correction desired will help minimize complications and recurrence. If neuromuscular pathology is present, treatment principles are altered and greatly depend on the severity of the deformity, the association of tibialis posterior spasticity, and ambulatory status. In mild to moderate pathology in walking patients with cerebral palsy, osteotomies can be successful. Various forms of arthrodesis can decrease recurrence when the deformity is severe in a nonambulatory patient with cerebral palsy and a symptomatic valgus foot deformity. In cases of collagen disorders, where soft-tissue laxity complicates management, deformity correction may be of higher importance. Overall alignment always should be evaluated and corrected when necessary to optimize the outcome in patients with valgus foot deformities. The successful treatment of flexible or rigid flatfoot deformity must take into account underlying pathology to optimize outcomes.

BACKGROUND: Standardized pediatric hospitalist and orthopaedic co-management of spinal fusion patients may improve quality processes and outcomes. This approach has not been studied in a general academic center. OBJECTIVE: Estimate relative effects and feasibility of the interventions on quality outcomes, length of stay (LOS), catheter-acquired urinary tract infections (CAUTI), medica- tion errors, and pain scores. DESIGN AND SETTING: Retrospective cohort using inter- rupted time series, analyzing data from 83 patients aged 5 to 18 years admitted for posterior spinal fusion (PSF) in 2009 (N = 27), 2010 (N = 28), and 2011 (N = 28) on a children's service at a general academic tertiary care center. INTERVENTIONS: Multimodal approach to standardizing pediatric PSF postoperative care with interdepartmental development of order sets, clinical care guidelines, and routine pediatric hospitalist co-management of all pediatric PSF patients. MEASUREMENTS: Chi-square analysis of order set use, guideline use measured by proxy medication and documenta- tion data. ANOVA for comparison of CAUTI and medication error rate and multivariate linear regression of LOS and pain scores. RESULTS: Pediatric hospitalist co-management documen- tation increased from 64% to 80%. Guideline use increased from 40% to 79%, and order set use was < 15%. CAUTI and medication error ratios remained low. Adjusted mean LOS decreased by 0.8 days (p = 0.039, 95% CI 0.7, 1.1). Pain scores did not differ. CONCLUSION: Interdisciplinary, clinical guideline devel- opment and postoperative co-management significantly decreased hospital LOS in pediatric PSF patients. In a general academic medical center, this change may be at- tributed to a pediatric hospitalist academic team, a universal co-management process with well-communicated roles, and a pediatric hospital-based physician development of and adherence to standardized practice.

Abnormal hip development causes one-quarter to one-half of all hip disease. Dysplastic hips typically share characteristic anatomic abnormalities. The dysplastic acetabulum is typically shallow, lateralized, and anteverted with insufficient coverage anteriorly, superiorly, and laterally. The dysplastic proximal femur has a small femoral head with excessive femoral neck anteversion and a short neck with an increased neck shaft angle. These characteristic changes result in intraarticular pathology leading to hip arthritis. A variety of treatment options exist based on the degree of dysplasia and the amount of concomitant hip arthritis. Treatment options include hip arthroscopy, acetabular or femoral osteotomies, hip arthrodesis, and total hip arthroplasty.

The evolving knowledge on Legg-Calve-Perthes (LCP) demonstrates the utility of studying a rare disease systematically by piecing together the biology and mechanics of this condition and applying clinical observations to improve patient care. As treatments of less common diseases are hard to randomize and study in meaningful numbers, long-term study groups have been created to provide insight into this entity that remains an enigma in many aspects. These studies permit a more evidence-approached guide to prognosis and treatment. Meanwhile, basic science research contributes to our understanding of pathophysiology of the disruption and repair processes that lead to LCP, with the goal of clinical translation. This review of LCP aims to give an overview of the condition, with specific focus on recent literature.

BACKGROUND: : Hip flexion contracture (HFC) in the ambulatory child with cerebral palsy (CP) may impair function and lead to deteriorations in health-related quality of life. Furthermore, increasing HFC may lead to increasing disability. However, the association between passive range of motion and the measures of function and well-being is unclear. This study was designed to determine whether increasing HFC is associated with functional outcome. METHODS: : A total of 181 children, with an average age of 14.0+/-10.2 years, were evaluated as part of a multicenter prospective data collection of patients with ambulatory CP. Measurements of HFC were recorded, and patients were evaluated using walking score from Gillette Functional Assessment Questionnaire (FAQ), Gross Motor Function Measure (GMFM), and the Pediatric Outcome Data Collection Instrument (PODCI). Patients were grouped on the basis of severity of HFC: group A, 0 to 14 degrees; group B, 15 to 29 degrees; and group C >/=30 degrees. Associations were examined using the Spearman correlation. RESULTS: : There was an inverse association between degree of HFC and FAQ walking score (P<0.01, rho=-0.25). Similarly, there was an inverse association between the degree of HFC and GMFM parts D (P<0.001, rho=-0.31) and E (P<0.001, rho=-0.32). Lastly, the PODCI domains of global function, mobility, and physical function also showed an inverse association with degree of HFC (P<0.001, rho=-0.24). CONCLUSIONS: : As surgeons treating children with CP, we often rely on joint measurements as an indirect measure of function. This study of children with ambulatory CP suggests that increased HFC from the physician's perspective is associated with deterioration in function from a patient and a therapist's perspective. LEVEL OF EVIDENCE: : Level II, prospective study.

Computed tomography (CT) has been deemed a necessary part of management for Tillaux and triplane pediatric ankle fractures. However, no previously published study has attempted to quantify its usefulness in changing management. Six third-party, blinded orthopedic surgeons (F.A.L., E.N.K., D.M.P., K.J.K., D.S.F., K.A.E.) were randomly assigned to evaluate 24 pediatric Tillaux or triplane fractures with plain radiographs; after 6 months, they were again randomly assigned to evaluate the 24 radiographs plus CT scans, totaling 144 third-party, blinded evaluations. Intra- and interobserver agreements were assessed via correlation coefficient analysis. Evaluation of CT scans changed the original diagnosis of fracture type from Tillaux to triplane fracture in 7 (4.9%) of 144 evaluations. Inter- and intraobserver agreements regarding primary treatment plans did not significantly differ between radiographs and radiographs plus CT scans (0.5 vs 0.4, respectively; P>.05). The addition of CT did not significantly change the impression of the amount of displacement per case. By adding CT, more patients who were assigned nonoperative management were reassigned to operative treatment (P=.033). Adding CT, although it may influence the decision to operate on Tillaux and triplane fractures, may not be as useful as previously thought.

Familial dysautonomia (FD) is a rare neurological disease with autosomal recessive inheritance and is associated with severe kyphoscoliosis. Investigators have reported subjective observation of decreased cervical motion and high rates of proximal instrumentation failure in this population. A radiographic study of sagittal plane cervical spine motion was performed with 15 patients with FD. Measurements were compared with normal values. Patients with FD had decreased sagittal motion at all cervical levels (P<0.05). Intervertebral translation was also decreased significantly at C3-C6. FD is associated with decreased sagittal motion in the cervical spine

BACKGROUND: CT allows for accurate measurement of acetabular orientation and shape, but malpositioning of the pelvis may lead to measurement variance. PURPOSE: We therefore sought to determine: (1) whether acetabular anteversion measurements using the femoral head centers differed from those using the posterior ischia, and (2) the extent to which changing obliquity, rotation, and tilt of a pelvis in a CT scanner affected the measurement of acetabular variables. METHODS: A radiopaque human pelvis model with articulated hips was suspended from a plastic sheet as part of an adjustable frame. Changes in the transverse and sagittal planes created rotation and tilt, while rotating the frame in the coronal plane created obliquity. CT scans were obtained, varying the combinations of obliquity, rotation, and tilt by intervals of 5 degrees , up to 20 degrees . Acetabular anteversion (AA), anterior acetabular sector angle (AASA), posterior acetabular sector angle (PASA), and horizontal acetabular sector angle (HASA) were measured. RESULTS: The two methods for measuring AA yielded values differing by 1 degrees to 4 degrees but correlated (r = 0.981) across the spectrum of pelvis positioning. Pelvic obliquity and tilt were linearly associated with changes in the measurements. For each 1 degrees -increase in pelvic obliquity, AA changed -0.4 degrees , and AASA, PASA, and HASA changed 1.93 degrees , 0.99 degrees , and 2.80 degrees , respectively. For each 1 degrees -increase in pelvic tilt, AA changed 0.8 degrees , and AASA, PASA, and HASA changed -1.07 degrees , 0.52 degrees , and -0.51 degrees , respectively. Rotation had no affect on the variables. CONCLUSIONS: Small changes in pelvic obliquity and tilt were associated with variances in acetabular measurements. The measured changes were directly proportional to the changes in obliquity and tilt, and were additive. Pelvic rotation created no changes in measurement. CLINICAL RELEVANCE: Incorrect interpretation of acetabular anteversion and coverage may lead to unsatisfactory acetabular fragment positioning during reorientational surgery. Although intraoperative positioning of an acetabular fragment may not be as precise as the tools for preoperative planning, it is important for a surgeon to have the most precise data available for planning a procedure, and know where error can occur in collecting the data

Lysosome-associated membrane protein (LAMP2) cardiomyopathy is an X-linked and highly progressive myocardial storage disorder associated with diminished survival, which clinically resembles sarcomeric hypertrophic cardiomyopathy. As shown here in a young woman, the natural history of LAMP2 may demonstrate an extreme profile of left ventricular remodeling with regression of hypertrophy (i.e. marked wall thinning), chamber dilatation, and severe systolic dysfunction, all of which are associated with widespread transmural scarring.