Clinical response to surgical decompression in atypical pediatric Bow Hunter's syndrome suggesting alternative pathophysiology: Case report.

Bow Hunter's syndrome is a rare cause of posterior circulation ischemia, produced by the mechanical and reversible occlusion of the vertebral artery during cephalic rotation. Diagnosis requires clinical suspicion and careful inspection of images with three-dimensional reconstruction. The study of choice is dynamic digital subtraction angiography (DSA). Treatment alternatives are: medical, surgical or endovascular. We report the case of an 8-year-old boy with recurrent infarctions of the posterior circulation secondary to the dissection of the vertebral artery, in association with an occipital bone spur. Dynamic DSA was negative. Conservative initial management was elected with cervical immobilization and anticoagulation, but due to persistence of symptoms, surgical decompression was decided. The patient did not repeat symptoms postoperatively and returned to his usual life. This is the first case reported to our knowledge of a surgical pediatric patient with asymptomatic atypical compression of VA secondary to BHS, whose dynamic angiography was negative, suggesting an alternative mechanism of the syndrome.

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.