Elizabeth Ashby

Characterization of Hip Morphology in Children With Mucopolysaccharidosis Types I and II.

Background: The purpose of this study is to describe the natural history of hip morphology in patients with mucopolysaccharidoses (MPS) I and MPS II. Methodology: This is a retrospective radiographic analysis of 88 hips in 44 children with MPS I and II. Radiographs were examined to determine hip migration, femoral head sphericity, and acetabular dysplasia at different ages throughout childhood. In individual hips, change in morphology and rate of change were analyzed. Results: There was a high rate of hip migration and femoral head dysplasia in both MPS I and MPS II. Progressive migration was seen in three quarters of hips and progressive femoral head deformity in over half of hips. Acetabular dysplasia was variable, ranging from normal to severely dysplastic, but did not change with time. Overall, hips were more dysplastic in MPS I than MPS II. Conclusions: Hip morphology is variable in MPS I and MPS II ranging from almost normal to severely dysplastic. Some hips do not deteriorate with time and thus surgical intervention may not be necessary in all cases. Deterioration is slow allowing time to plan a holistic approach to treatment. Level of Evidence: Level IV—case series.

Functional Outcome of Humeral Rodding in Children With Osteogenesis Imperfecta.

Background: The impact of corrective forearm surgery on functional ability in children with osteogenesis imperfecta (OI) has not previously been reported. This study addresses this issue. Methods: A retrospective chart review was conducted on 19 children with OI who underwent 22 corrective forearm procedures between 1996 and 2013. Functional ability was assessed preoperatively and every year postoperatively using the Pediatric Evaluation of Disability Inventory (PEDI). Results: The mean PEDI self-care score increased by 6.8 (P=0.017) and the mean PEDI mobility score increased by 7.2 (P=0.020) at 1-year postsurgery. Functional gains were greater in moderate OI (types IV, V, and VI) than in severe OI (type III). Improved function was maintained in the majority of cases at a mean of 8.9 years postcorrection. Conclusions: Corrective forearm surgery in children with OI leads to improved functional ability. Level of Evidence: Level IV.

Proximal Femoral Head Resection and Interpositional Arthroplasty

Despite preventative screening programmes in some countries, hip dislocation is still a common problem in many severely affected, non-ambulant patients with cerebral palsy. There are two scenarios where pain and stiffness affect all aspects of patient comfort and care and where a proximal femoral excision with an interpositional arthroplasty (myoplasty) must be considered: (1) the enlocated hip following hip reconstruction that is associated with a poor clinical outcome and (2) the dislocated hip, which due to anatomical or clinical factors is not reconstructible. Both indications are most common in patients assessed as IV or V on the Gross Motor Function Classification Scale (GMFCS).