Pathogenesis of adolescent idiopathic scoliosis in girls - a double neuro-osseous theory involving disharmony between two nervous systems, somatic and autonomic expressed in the spine and trunk: possible dependency on sympathetic nervous system and hormones with implications for medical therapy

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

Left convex thoracic scoliosis: retrospective analysis of 25 patients after surgical treatment

OBJECTIVE: A retrospective analysis of clinical and radiological data was conducted, with an emphasis on perioperative complications and risk factors and a minimum follow-up period of two years. The postoperative quality of life was assessed using the SRS-22 questionnaire. METHODS: Between 1999 and 2009, 25 patients (nine male, 16 female) with LCTS, with a mean age of 13.7 years (2.3-29.8 years), were treated with correction and instrumented fusion at a single institution. Seven patients had congenital scoliosis and 18 patients had noncongenital scoliosis (idiopathic, n = 5; neuropathic, n = 4; neoplasm-associated/iatrogenic, n = 3; secondary to other conditions, n = 6). The average preoperative Cobb angle was 74° (49-102°). RESULTS: A mean correction of 51% was achieved postoperatively. The mean Cobb angle at the final follow-up examination was 45° (19-85°), with a significant loss of correction of 8.8° on average. Major complications affected five patients (20%): respiratory insufficiency requiring prolonged intubation, intraoperative cardiac arrest with resuscitation being necessary twice in one patient, persistent clonus, low-grade infection, implant-based complications requiring revision surgery, and adding-on. Minor complications were observed in 22 patients (88%), mainly gastrointestinal and pulmonary. No cases of paraplegia or death occurred. A noncongenital etiology had been diagnosed before the age of 10 years in all of the patients who had major complications. The best score on the SRS-22 questionnaire was achieved in the domain of pain (87%), while the poorest was in the domain of self-image (68%). CONCLUSIONS: The results of this study emphasize an increased complication rate in patients with LCTS scheduled for scoliosis surgery. Additional preoperative examinations (MRI, paediatric consultation, cardiologic consultation, pulmonary function test) are mandatory in patients with LCTS. Preoperatively, patients should be informed about the increased cardiopulmonary and neurological risk which may be associated with scoliosis surgery.OBJETIVO: Fue realizado un análisis retrospectivo de datos clínicos y radiológicos, con énfasis en complicaciones perioperatorias y factores de riesgos, y un período mínimo de seguimiento de 2 años. La calidad de vida posoperatoria fue evaluada usándose el cuestionario SRS-22. MÉTODOS: entre 1999 y 2009, 25 pacientes (nueve del sexo masculino, 16 del sexo femenino) con ETCI, con edad promedio de 13,7 años (2,3 - 29,8 años) fueron tratados con corrección y fusión instrumentada, en una única institución. Siete pacientes tenían escoliosis congénita y 18 pacientes tenían escoliosis no congénita (idiopática, n = 5; neuropática, n = 4; asociada a neoplasma/iatrogénica, n = 3; secundaria de otras condiciones, n = 6). El ángulo de Cobb preoperativo promedio fue 74º (49-102º). RESULTADOS: una corrección promedio de 51% fue alcanzada después de la operación. El ángulo de Cobb promedio, en el examen final de seguimiento, fue 45º (19-85º), con una pérdida significativa de corrección de 8,8º en promedio. Complicaciones graves afectaron a cinco pacientes (20%): insuficiencia respiratoria que requirió intubación prolongada, paro cardíaco intraoperativo con resucitación siendo necesaria dos veces para un paciente, clonus persistente, infección leve, complicaciones con implantes que precisaron de otra cirugía, y accesorios. Complicaciones de menor importancia fueron observadas en 22 pacientes (88%), principalmente gastrointestinales y pulmonares. No ocurrieron casos de paraplejía ni de muerte. Una etiología no congénita fue diagnosticada, antes de la edad de 10 años, en todos los pacientes que tuvieron complicaciones graves. La mejor puntuación en el cuestionario SRS-22 fue obtenida en el dominio de dolor (87%), mientras que la peor se registró en el dominio de la autoimagen (68%). CONCLUSIONES: Los resultados de este estudio enfatizan una tasa de complicación aumentada en pacientes portadores de ETCI con programación de cirugía de escoliosis. Exámenes preoperatorios adicionales (IRM, consulta pediátrica, consulta cardiológica, prueba de función pulmonar) son obligatorios en pacientes con ETCI. Antes de la operación, los pacientes deben ser informados sobre los mayores riesgos cardiopulmonar y neurológico que pueden estar vinculados a la cirugía de escoliosis.OBJETIVO: Foi realizado estudo retrospectivo baseado em parâmetros clínicos e radiológicos, com ênfase nas complicações, fatores de risco e seguimento mínimo de dois anos. A qualidade de vida pós-operatória foi avaliada por meio do questionário SRS-22. MÉTODOS: No período de 1999 a 2009, 25 pacientes (nove do sexo masculino e 16 do sexo feminino) com idade variando de 2,3 a 29,8 anos (média de idade de 13,7 anos) foram submetidos a tratamento cirúrgico por meio de instrumentação e artrodese. Sete pacientes apresentavam escoliose congenital, cinco apresentavam escoliose idiopática, quatro neuropática, três associada à neoplasia ou iatrogenia, e seis associada a outras doenças. A média dos valores pré-operatórios do ângulo de Cobb foi 74° (49-102°). RESULTADOS: A média de correção no pós-operatório foi de 51%. Na avaliação de seguimento final, o valor médio do ângulo de Cobb foi 45° com variação de 19 a 85°, tendo ocorrido significante perda da correção com o valor médio de 8.8°. Complicações maiores ocorreram em cinco pacientes (20%): insuficiência respiratória requerendo entubação prolongada, parada cardíaca intraoperatória com ressuscitação, clônus persistente, infecção de baixo grau, complicações com implantes requerendo revisão cirúrgica, e descompensação do tronco. Complicações menores foram observadas em 22 pacientes (88%), sendo principalmente gastrointestinais e pulmonares. Nenhum caso de paraplegia ou morte ocorreu na série de pacientes estudados. As curvas de etiologia não congênita foram diagnosticadas antes dos 10 anos de idade em todos os pacientes que apresentaram complicações maiores. O melhor escore do questionário SRS-22 foi observado no domínio da dor (87%), e o pior no domínio da autoimagem (68%). CONCLUSÕES: O resultado do estudo enfatiza as altas taxas de complicação nos pacientes portadores de escoliose torácica sinistro-convexa que são submetidos ao tratamento cirúrgico. Avaliações pré-operatórias adicionais (RNM, avaliação pediátrica, avaliação cardiológica, teste de função pulmonar) devem ser realizadas nesse grupo de pacientes. Antes do procedimento, os pacientes devem ser informados acerca do alto risco de complicações cardiopulmonares e neurológicas associadas ao tratamento cirúrgico da deformidade