Tratamento cirúrgico da escoliose na amiotrofia espinhal progressiva

Abstract

RESUMO -Objetivo: Descrever os resultados imediatos e tardios da cirurgia para correção de escoliose em pacientes com amiotrofia espinhal progressiva (AEP). Método: 14 pacientes com AEP submetidos a cirurgia para escoliose foram selecionados para análise retrospectiva dos prontuários e radiografias, assim como para exame radiográfico e aplicação de um questionário na consulta final. O tempo médio de seguimento foi de 22 meses. A escoliose média pré-operatória foi de 78,4° pelo método de Cobb. Todos os pacientes apresentavam obliqüidade pélvica (média de 25,5°) e 11 apresentavam cifose. A média de idade à época da cirurgia foi de 12 anos e 3 meses. Em 12 pacientes foi realizada artrodese via posterior com instrumental de Luque-Galveston, e em 2 com instrumental de Cotrel-Dubousset. Resultados: Foi obtida correção no pós-operatório imediato de 64,3% da escoliose, 36,4% da cifose e 70,9% da obliqüidade pélvica. As complicações foram fístula liquórica e infecção precocemente em um caso; e tardiamente soltura da amarria de T1, em dois casos. Houve perda média de correção no final do seguimento de 0,26° da escoliose e de 1,28° da obliqüidade pélvica. Os pais e os pacientes referiram boa melhora em relação aos aspectos estéticos, postura, equilíbrio para sentar e cuidados de higiene, assim como das intercorrências respiratórias. Conclusão: A cirurgia para escoliose na AEP tem impacto satisfatório quanto à estética, qualidade de vida e função respiratória dos pacientes, com mínima perda da correção obtida com o tempo e com poucas complicações.PALAVRAS-CHAVE: amiotrofia espinhal progressiva, escoliose, artrodese de coluna, tratamento ortopédico, doenças neuromusculares. Surgical treatment of scoliosis in spinal muscular atrophyABSTRACT -Objective: To describe the early and late postoperative data from SMA patients with surgical procedure. Method: Clinical data and radiographic imaging from 14 SMA patients with surgical treatment of scoliosis were reviewed, and all were reassessed clinically with new spinal radiographs and a questionnaire. The mean follow-up were 22 months. The mean preoperative Cobb angle was 78.4°. All patients presented pelvic obliquity (mean 25.5°) and 11 had cifosis. The mean age at time of surgery was 12 years and 3 months. All patients were treated with posterior spinal fusion with Luque-Galveston instrumentation in 12 and CotrelDubousset instrumentation in 2. Results: The average curve correction at the immediate postoperative was 64.3% for scoliosis and 36.4% for cifosis, with the pelvic obliquity correction of the 70.9%. The complications were liquoric fistula and infection earlier in one case, and lately wire looseness of T1 in 2 patients. It was detected mean lost of the correction at the final assessment of the 0.26° of the scoliosis and the 1.28° of the pelvic obliquity. Relatives and the patients related good improvement regarding to esthetic aspects, posture balance, body care, as well as respiratory problems. Conclusion: The spinal fusion for scoliosis in SMA patients has a satisfactory i...

Epidemiological Profile and Outcomes in Postoperative Neuromuscular Escoliosis

“…However, we cannot infer anything from this finding, since there are no studies that stratify outcomes by underlying diagnosis. 13,18 Immobility, although with a small overall percentage (2%) in this study, was reported as a complication because it is a factor that can impact hospital outcomes. 19,20 The 1 st postoperative was the day of least adherence to the proposed mobility goals (24%) and this should be a point of attention to be managed in the institutions, in conjunction with patient education and the active participation of the patient and family in postoperative planning, including information about daily goals.…”

“…In this study, the Cobb angle, which is an important factor when analyzing outcomes related to surgical time, for example, was not documented. 13,18 Most studies only report complications related to the intraoperative period or the most serious postoperative ones, such as infection of the surgical site and respiratory infection. In this sense, the complication rate percentages found in the literature are lower than the values observed in our study.…”

Proximal spinal muscular atrophy: current orthopedic perspective

“…Early spinal surgery tends to have better results because of milder scoliosis at the time of surgery and higher flexibility of the spine, such that better correction can be achieved 29,35. Furthermore, it improves sitting comfort as well as quality of life, including the patients’ self-confidence due to enhanced appearance 37,38. The aim for spine stabilization is to restore sitting ability without arm support and to avoid impingement of the ribs on the pelvis.…”

“…Additional advantages include immediate sitting ability postsurgery and early mobilization 36. Independent of the surgical method, complications such as delayed wound healing and respiratory and neurological complications have been described for SMA patients 30,37,38. Furthermore, the anesthetic risk due to reduced respiratory function has to be considered for the surgical and postoperative planning.…”

Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery

“…Scoliosis is a frequent manifestation in all forms of SMA, being secondary to progressive weakness of the axial muscles 10,11 . Surgical treatment is indicated in most cases, especially when the curve is greater than 45 degrees, to control curve progression and pelvic obliquity 12,13 . The surgical approach, often with the use of bone graft, makes the conventional lumbar puncture by posterior interlaminar approach a challenge in sucesso semelhante.…”