Postoperative weight loss appears to be more important for the development of the SMAS than asthenic body type. Newer derotation/translation corrective techniques have not eliminated the SMAS. Gastrointestinal imaging is indicated when nausea and vomiting occur 6-12 days after surgery, associated with early satiety and normal bowel sounds. Decompression and nutritional support remain the mainstays of treatment.

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Van Nes rotationplasty may be used for patients with congenital proximal focal femoral deficiency (PFFD). The lower limb is rotated to use the ankle and foot as a functional knee joint within a prosthesis. A small series of cases was investigated to determine the long-term outcome. At a mean of 21.5 years (11 to 45) after their rotationplasty, a total of 12 prosthetic patients completed the Short-Form (SF)-36, Faces Pain Scale-Revised, Harris hip score, Oswestry back pain score and Prosthetic Evaluation Questionnaires, as did 12 age- and gender-matched normal control participants. A physical examination and gait analysis, computerised dynamic posturography (CDP), and timed ‘Up & Go’ testing was also completed. Wilcoxon Signed rank test was used to compare each PFFD patient with a matched control participant with false discovery rate of 5%. There were no differences between the groups in overall health and well-being on the SF-36. Significant differences were seen in gait parameters in the PFFD group. Using CDP, the PFFD group had reduced symmetry in stance, and reduced end point and maximum excursions. Patients who had undergone Van Nes rotationplasty had a high level of function and quality of life at long-term follow-up, but presented with significant differences in gait and posture compared with the control group.

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Background To provide the best health care for individuals with myelomeningocele (MM), clinicians and researchers need to understand their health and functional status as well as quality of life. The literature is mixed regarding the relationship between motor level and health-related quality of life (HRQOL) for these individuals. Questions/purposes We compared the HRQOL of children with MM at the L2 and above and L3-5 motor level and to demonstrate how having a shunt, age, and body mass index affect HRQOL instruments for these two motor level groups. Methods We recruited 50 patients with MM (24 male, 26 female) with a mean age of 11.5 years (range, 5-18 years) and motor levels of L2 and above (n = 15) and L3-5 (n = 35). Guardians were interviewed using standardized functional and HRQOL tools (the Pediatric Quality of Life and the Pediatric Outcomes Data Collection Instrument); height, weight, presence of a shunt, ambulatory level, and body mass index were also collected. Results We found a decreased HRQOL score for children with MM in the L2 and above motor level compared with those in the L3-5 motor level group. HRQOL had no correlation with body mass index and limited correlation with age.

Background Youth with disabilities are at risk for decreased participation in community activities. However, little is known about participation at different developmental periods of childhood and adolescence among youth with spina bifida (SB) or whether child, family, and SB-associated factors influence participation. Questions/purposes Our cross-sectional study examined participation among youth with SB and assessed how participation differs between youth ages 2-5, 6-12, and 13-18; how participation relates to child (gender) and family (caregiver marital status, education, and employment) characteristics; and how participation relates to SB-related factors (motor level, hydrocephalus, ambulation, medical issues, and bladder/bowel needs). Patients and Methods Sixty-three youth ages 2-18 years and/or their caregivers completed age-appropriate measures of participation for youth with disabilities. The patients had an average age of 9.52 years (SD = 5.22), 83% had a shunt, 34% had a motor level of L2 or higher, and 66% L3 or lower. Results A comparison of youth ages 2-5 (n = 19), 6-12 (n = 21), and 13-18 (n = 23) revealed older youth participated less in recreational, physical, and skill-based activities. Caregiver employment facilitated participation in social activities. Youth who did not have a shunt participated more often in physical and skill-based activities. Youth without recent major medical issues participated more often in physical and social activities. More caregivers reported bladder and bowel needs as barriers to participation for youth ages 6-12 than those ages 2-5 or 13-18. Conclusions Participation of youth with SB varies by age and across child and caregiver factors and should be understood in a developmental and situational context.

The aim of this study was to describe satisfaction and quality of life (QOL) of young adults with arthrogryposis after leaving pediatric care. Twenty-three adults with arthrogryposis multiplex congenital (AMC) followed at a single pediatric orthopedic hospital (average age 23.6 years; range 18-36 years; 9 males, 14 females) completed questionnaires related to demographics, mobility, and activities of daily living. The Patient Reported Outcomes Measure Information System and Satisfaction with LifeScale were utilized to evaluate QOL and life satisfaction. Eighty-three percent reported general health as good/excellent, 30% lived independently, 69% were ambulatory in the community, and 57% were employed. QOL scores for physical function were lower, but other QOL scores were consistent with the general U.S. population. Average pain intensity was mild at 2.6 out of 10, with pain frequently reported in the legs and feet. Fifty-six percent were satisfied to extremely satisfied with life. Five individuals who were dissatisfied with life also reported lower physical function, higher anxiety, depression and fatigue, and pain in multiple joints.Although most young adults with AMC presented with mild pain and limitations in physical function; overall, they reported good QOL. Findings from the current study will help clinicians anticipate the needs of individuals with AMC as they transition from pediatric to adult care. K E Y W O R D S arthrogryposis, patient reported outcomes, quality of life, satisfaction with life

In this multiauthored article, the management of lower limb deformities in children with arthrogryposis (specifically Amyoplasia) is discussed. Separate sections address various hip, knee, foot, and ankle issues as well as orthotic treatment and functional outcomes. The importance of very early and aggressive management of these deformities in the form of intensive physiotherapy (with its various modalities) and bracing is emphasized. Surgical techniques commonly used in the management of these conditions are outlined. The central role of a multidisciplinary approach involving all stakeholders, especially the families, is also discussed. Furthermore, the key role of functional outcome tools, specifically patient reported outcomes, in the continuous monitoring and evaluation of these deformities is addressed. Children with arthrogryposis present multiple problems that necessitate a multidisciplinary approach. Specific guidelines are necessary in order to inform patients, families, and health care givers on the best approach to address these complex conditions