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

Abstract

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 significanceDocumentation 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.

Bilateral hip reconstruction improve hip stability, pelvic balance and mobility function in children with cerebral palsy

“…Progressive deformity of hip, pelvis and trunk may develop continuously and contribute to difficulty in seating ,ambulation and handling [20][21][22][23]. One of common question which parent ask a treatment team is how much mobility function of the patients would be improved after surgery?…”

Predictors for Deformity Progression in a Spinal Muscular Atrophy Cohort After Scoliosis Correction Surgery

“…The major curve of Cobb angle (MCCA), pelvic obliquity (PO), and femoral head coverage percentage (FHCP) were measured as previously described. 4 , 15 , 16 B, The patient had received segmental spinal instrumentation with pedicle screws and the Galveston pelvic fixation technique. The fusion level was from T2 to the pelvis.…”

“… 25 We followed the previous reported method to measure the PO angle. 4 , 26 An oblique line connected the most superior parts of the 2 iliac crests, and a transverse line was parallel to the lowermost exposure line of the radiograph. The angle formed between these 2 lines was defined as the PO angle (Fig.…”

“…PO and concave-side hip FHCP were converted to a scale of 1–5 as previously described. 4 A patient with a PO of 0–1.9 degrees was defined as normal (grade 1), 2–4.9 degrees was mild (grade 2), 5–8.9 degrees was moderate (grade 3), 9–11.9 degrees was severe (grade 4), and ≥12 degrees was very severe (grade 5). A patient with an FHCP ≥67% was defined as normal (grade 1), 51%–66.9% was mild (grade 2), 33%–50.9% was moderate (grade 3), <33% was severe (grade 4), and no coverage was classified as complete dislocation (grade 5).…”

Duchenne muscular dystrophy: genome editing gives new hope for treatment

“…These lead to severe muscle weakness and wasting, and cardiac and respiratory insufficiency 3. Muscle loss typically occurs first in the thighs and pelvis, followed by the forearms 4. The affected muscles might appear greater in size due to the increased lipid deposition.…”