Amputation Versus Staged Reconstruction for Severe Fibular Hemimelia: Assessment of Psychosocial and Quality-of-Life Status and Physical Functioning in Childhood.

BACKGROUND: Fibular hemimelia, a congenital disorder characterized by the partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency, is the most common deficiency of long bones. The purpose of the present study of children with congenital fibular hemimelia was to examine the functional and psychosocial outcomes at a minimum of 2 years after treatment either with amputation and a prosthesis or with reconstruction and lengthening. METHODS: Twenty children who were managed with primary amputation were compared with 22 children who were managed with staged limb reconstruction. The average age of the patients at the time of evaluation was 9 years (range, 5 to 15 years). Patients and parents completed psychosocial, quality-of-life, and satisfaction surveys. Patients underwent instrumented gait analysis and a timed 25 or 50-yard dash. The number and nature of surgical procedures were recorded from a retrospective chart review. RESULTS: Families of children managed with amputation had lower economic and educational levels and were more ethnically diverse compared with the families of children managed with limb reconstruction. Scores on psychosocial and quality-of-life surveys were comparable with those from healthy patient populations. Parents of males treated with amputation perceived a lower school-related quality of life for their child; socioeconomic and ethnic differences between groups might account for this finding. Statistically but not clinically significant differences were measured during instrumented gait analysis at a self-selected walking speed and during a timed 25 or 50-yard dash. The majority of patients and parents reported satisfaction with the treatment method selected and would select the same treatment method again. CONCLUSIONS: At this interim stage of growth, there were no significant functional or psychological differences between groups. Both groups were satisfied with the outcome in mid-childhood, irrespective of the selection of amputation or limb reconstruction. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Accuracy in the prediction and estimation of adherence to bracewear before and during treatment of adolescent idiopathic scoliosis.

BACKGROUND: Because bracing for scoliosis may prevent curve progression, it is important to recognize nonadherence. We used temperature sensors to determine actual bracewear and examined: (1) the ability of a new pretreatment questionnaire to predict bracewear; (2) the ability of the physician and orthotist to predict bracewear before treatment and (3) the ability of physicians, orthotists, patients, and parents to accurately estimate bracewear during the first year of treatment. METHODS: Sixteen males and 108 females with adolescent idiopathic scoliosis were fitted with a Boston brace equipped with a temperature sensor and told that investigators were examining comfort. Before treatment, each patients completed an 18-item Brace-Beliefs Questionnaire (BBQ), and physicians/orthotists rated the likelihood that their patient would be adherent. During treatment, physicians, orthotists, patients, and parents provided estimates of daily bracewear. Data obtained at 1 to 3, 4 to 7, and 9 to 12 months into treatment were analyzed. RESULTS: Scores from the BBQ were related to actual adherence (r = 0.46, P < 0.001). No patient scoring more than 1 SD below the BBQ sample mean had an adherence level more than 40%. Correlations of physician/orthotist pretreatment predictions with actual adherence were minimal. Overall, patients wore the brace 47% of the prescribed time, although they were estimated to have worn it 64%, 66%, 72%, and 75% by physicians, orthotists, parents, and patients, respectively. Physicians/orthotists incorrectly identified at least 1 of every 4 nonadherers. CONCLUSIONS: Predicting a patient's adherence before treatment is difficult, but a pretreatment questionnaire may be helpful. During treatment, all respondents overestimated adherence. Health care providers should be mindful of overreports of bracewear and skeptical of their own assessments of adherence. CLINICAL RELEVANCE: Potential nonadherence may be predicted by a brief treatment-specific questionnaire. Treatment teams should not assume that patients follow their instructions or that family members are accurate sources of adherence information during treatment. Health care providers also should not assume that they can accurately predict adherence based on subjective expectations.

The impact on health-related quality of life from non-steroidal anti-inflammatory drugs, methotrexate, or steroids in treatment for juvenile idiopathic arthritis.

OBJECTIVE: To assess and compare the impact of medication treatments on health-related quality of life (HRQOL), family function, and medical status in children with juvenile idiopathic arthritis (JIA). METHODS: Fifty-seven children diagnosed with JIA were assessed by a pediatric rheumatologist and placed into one of three treatment groups: (1) non-steroidal anti-inflammatory; (2) methotrexate; or (3) steroids via IV methylprednisolone. Questionnaires were administered at baseline and 4-month follow-up. The attending pediatric rheumatologist provided additional medical information. RESULTS: Data document the impact of JIA on HRQOL, particularly on physical and pain domains. Steroid patients experienced improved HRQOL at follow-up relative to other groups, despite reporting more problems with side effects. CONCLUSION: These results demonstrate positive benefits of steroids in treating JIA children, despite the greatest incidence of adverse side effects.

Performance on the NEPSY among children with spina bifida.

Usefulness of the NEPSY: A Developmental Neuropsychological Assessment (NEPSY) to assess attention/executive function skills was examined in a sample of 30 children with spina bifida and shunted hydrocephalus (SBSH; 20 females/10 males; age range: 6-12 years). Statistically significant moderate-to-strong correlations between the NEPSY and other measures of executive functioning were obtained. The strength of these associations decreased when controlling for intelligence as measured by the WISC-III. The Attention and Executive Function domain appeared more closely associated with tests requiring focused attention and an ability to shift set than those emphasizing more abstract reasoning, memory, or non-verbal problem-solving skills. The NEPSY appears useful in the assessment of specific executive function abilities in young individuals with SBSH. More research into the NEPSY and particularly its Attention and Executive Function domain is needed among individuals with SBSH before its use as a stand-alone tool apart from adjunctive cognitive assessment instruments.