Anthropometric data from three groups of adolescent girls - preoperative adolescent idiopathic scoliosis (AIS), screened for scoliosis and normals were analysed by comparing skeletal data between higher and lower body mass index subsets. Unexpected findings for each of skeletal maturation, asymmetries and overgrowth are not explained by prevailing theories of AIS pathogenesis. A speculative pathogenetic theory for girls is formulated after surveying evidence including: (1) the thoracospinal concept for right thoracic AIS in girls; (2) the new neuroskeletal biology relating the sympathetic nervous system to bone formation/resorption and bone growth; (3) white adipose tissue storing triglycerides and the adiposity hormone leptin which functions as satiety hormone and sentinel of energy balance to the hypothalamus for long-term adiposity; and (4) central leptin resistance in obesity and possibly in healthy females. The new theory states that AIS in girls results from developmental disharmony expressed in spine and trunk between autonomic and somatic nervous systems. The autonomic component of this double neuro-osseous theory for AIS pathogenesis in girls involves selectively increased sensitivity of the hypothalamus to circulating leptin (genetically-determined up-regulation possibly involving inhibitory or sensitizing intracellular molecules, such as SOC3, PTP-1B and SH2B1 respectively), with asymmetry as an adverse response (hormesis); this asymmetry is routed bilaterally via the sympathetic nervous system to the growing axial skeleton where it may initiate the scoliosis deformity (leptin-hypothalamic-sympathetic nervous system concept = LHS concept). In some younger preoperative AIS girls, the hypothalamic up-regulation to circulating leptin also involves the somatotropic (growth hormone/IGF) axis which exaggerates the sympathetically-induced asymmetric skeletal effects and contributes to curve progression, a concept with therapeutic implications. In the somatic nervous system, dysfunction of a postural mechanism involving the CNS body schema fails to control, or may induce, the spinal deformity of AIS in girls (escalator concept). Biomechanical factors affecting ribs and/or vertebrae and spinal cord during growth may localize AIS to the thoracic spine and contribute to sagittal spinal shape alterations. The developmental disharmony in spine and trunk is compounded by any osteopenia, biomechanical spinal growth modulation, disc degeneration and platelet calmodulin dysfunction. Methods for testing the theory are outlined. Implications are discussed for neuroendocrine dysfunctions, osteopontin, sympathoactivation, medical therapy, Rett and Prader-Willi syndromes, infantile idiopathic scoliosis, and human evolution. AIS pathogenesis in girls is predicated on two putative normal mechanisms involved in trunk growth, each acquired in evolution and unique to humans.

Patients with quadriplegic spastic cerebral palsy with and without ITB pumps showed significant curve progression over time. ITB pumps do not appear to alter the natural history of curve progression in this population.

In this paper, we introduce the concept of functional anteversion of the femur and its positional change. It emerged from studies using B-mode and real-time ultrasound to determine femoral anteversion in two knee positions. Twenty healthy children were scanned at the hips whilst lying supine with knees extended and then with the knees flexed to about 90". The "anteversion" angle between the head-trochanter line and the horizontal measured with knees extended and feet together is determined by three factors: 1) anatomical femoral anteversion, 2 ) femoro-tibia1 rotation at the knee, and 3 ) tibial torsion. This is one type of "functional anteversion" of the femur.The best reproducibility for functional femoral anteversion was obtained by scanning with real-time ultrasound and the knees extended (95% confidence limits within 5 2.8"); the use of B-mode ultrasound in the knees-extended position is significantly less reproducible (95% confidence limits within 5 6.1"). In the flexed-knee position, the reproducibility is similar with both B-mode and real-time ultrasound ( F 2.6-3.5"). Both positions have limitations in attempts to measure anatomical femoral anteversion by ultrasound.The functional femoral anteversion angle measured by B-mode and real-time ultrasound is significantly larger (by 9-10') with the knees flexed than with the knees extended. This positional change of measured femoral anteversion involving axial rotation at the hip is attributed mainly to 1) lateral rotation (unlocking) of the femur at the knee during flexion from the fully extended position and 2) any lateral tibial torsion which rotates the femur medially in the knees-extended position with the feet vertical. The considerable individual variation and asymmetry of this axial rotational change may have relevance to the etiology of certain clinical disorders of the spine, hip, and knee joints. 0 1993 Wiley-Liss, Inc.

Introduction Intrathecal baclofen pumps are an effective treatment for spasticity in patients with cerebral palsy. Recently, concerns have been raised regarding the potential for intrathecal baclofen pumps to accelerate scoliosis progression and increase the need for spinal fusion surgery. To date the literature remains unclear, with studies often failing to account for the natural history of scoliosis progression in this population when assessing the influence of baclofen pumps. We aim to clarify matters by comparing progression of scoliosis in quadriplegic spastic cerebral palsy with and without intrathecal baclofen pumps. Material and Methods: Design: Retrospective matched cohort. Patients: Inclusion criteria: Quadripegic spastic cerebral palsy, GMFCS level 5, follow up > 1 year, adequate radiographic/clinical data. Methods: Suitable patients who underwent intrathecal baclofen pump insertion were matched to cases with the same diagnosis and function level with comparable age and baseline Cobb angle but without intrathecal baclofen pumps. Outcomes: Primary: Sequential radiographs measured to assess annual coronal curve progression and peak curve progression. Secondary: Need for spinal fusion for curve progression. Analysis: Between group comparisons made with paired t-tests and chi square tests. Results Baclofen group: 17 patients (7 female), mean age at insertion of pump 9.4 (3.6), 10 cases with a scoliosis > 10° at baseline. Initial Cobb angle 21° (21) and Risser grade 0.8. Twelve cases had hip subluxation/dislocation at baseline. Mean follow up 4.7 (1.3–8.8) years. Mean Cobb angle at final follow up was 77° (27). Non-baclofen group: 17 patients (10 female), mean age at baseline 9.5 (3.7), 12 cases with a scoliosis at baseline. Initial Cobb angle 25° (20) and Risser grade 0.9. Three cases had hip subluxation/dislocation at baseline. Mean follow up 3.6 (1–7.5) years. Mean Cobb angle at final follow up was 67° (30). The two groups were statistically similar in terms of age, initial Cobb angle, Risser grade and presence/absence of scoliosis at baseline. The baclofen group had a higher proportion of hip subluxation/dislocation at baseline (p = 0.006). Mean annual curve progression was 13.4° (7.2) for the baclofen group vs 13.6° (9.1) for the non-baclofen group (p = 0.8). Mean peak curve progression was 31.7 (34) for the baclofen group and 27.1 (18) for the non-baclofen group (p = 0.95). 4 patients with baclofen pumps and 5 without required spinal fusion for curve progression during follow up. All patients, aside one non-baclofen case, had developed a scoliosis by follow up; mainly thoracolumbar/lumbar collapsing C curves. Conclusion Patients with quadriplegic spastic cerebral palsy with and without baclofen pumps showed significant curve progression over time. Intrathecal baclofen pumps do not appear to alter the natural history of curve progression in this population.