Simultaneous progression patterns of scoliosis, pelvic obliquity, and hip subluxation/dislocation in non-ambulatory neuromuscular patients: an approach to deformity documentation.

backgroundA triad of deformities-thoracolumbar scoliosis, pelvic obliquity, and femoral head (hip) subluxation/dislocation-occurs frequently in non-ambulatory neuromuscular patients, but their close inter-relationship is infrequently appreciated or quantified. We propose a deformity documentation approach to assess each component simultaneously.

methodsThe documentation assesses each component for maximal functional level, deformity, and flexibility/rigidity: deformity from antero-posterior radiographs (scoliosis-maximal functional position, pelvic obliquity-sitting, hip position-supine) and flexibility/rigidity from extent of repositioning on supine (spine, pelvis) and frog lateral (hip) radiographs. The approach was applied in 211 patients: Duchenne muscular dystrophy (110), spinal muscular atrophy (49), cerebral palsy (26), and other neuromuscular disorders (26).

resultsMeasurement of 2124 radiological data points allowed for deformity (mild to moderate to severe) and flexibility/rigidity (fully reducible to partially to non-reducible) gradations for scoliosis, pelvic obliquity, and hip subluxation/dislocation. The charting documented: (1) numerical deformity and flexibility/rigidity changes [x-axis: age; y-axis: angulation (scoliosis and pelvic obliquity) and percent coverage (hip subluxation or dislocation) from 0-120]; and (2) grade deformity and flexibility/rigidity changes [x-axis: age; y-axis: deformity and flexibility/rigidity, following conversion of numerical measurements to a 1-5 grade scale]. In subgroups with the most extensive documentation, thoracolumbar and lumbar scoliosis extended into the sacrum with 98 % (114/116) accompanied by pelvic obliquity; and scoliosis developed more rapidly than hip deformity in 44 % (28/63), scoliosis and hip deformity developed at the same time in 40 % (25/63), and hip deformity developed more rapidly than scoliosis in 16 % (10/63) (Pearson's chi-squared test p = 0.0501, almost significant). CONCLUSION AND SIGNIFICANCE: Documentation of the triad of neuromuscular deformities is applicable to all diagnoses; it outlines maximal functional level, deformity, and flexibility/rigidity at each site; and it shows the relationship between spine, pelvic, and hip deformation. Prospective charting will enhance both clinical management and clinical research into neuromuscular deformity.