A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

Mullender, Margriet G.; Blom, NA; Kleuver, Marinus de; Fock, Johanna M.; Hitters, W. M. G. C.; Horemans, Anja M.C.; Kalkman, Cor J.; Pruijs, J. E. H.; Timmer, R. R.; Titarsolej, P. J.; Haasteren, N. C. van; Jäger, Manuela; Vught, A. J. van; Royen, Barend J. van

doi:10.1186/1748-7161-3-14

Cited by 63 publications

(47 citation statements)

References 74 publications

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“…This pattern of scoliosis progression was also pointed out by Nadeau et al 13. Development of scoliosis in Duchenne muscular dystrophy, on the other hand, is strongly related to the loss of walking ability 18. In Duchenne muscular dystrophy, typically, scoliosis is not evident in ambulatory patients and starts after patients become wheelchair dependent.…”

Section: Discussionmentioning

confidence: 52%

Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy

Yonekawa

Komaki

Okada

et al. 2013

Journal of Neurology, Neurosurgery & Psychiatry

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Section: Discussionmentioning

confidence: 52%

Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy

Yonekawa

Komaki

Okada

et al. 2013

Journal of Neurology, Neurosurgery & Psychiatry

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“…They advocated the use of a sitting type orthosis for patients who reject surgery for improvement of sitting comfort. 1 In contrast, Kotwicki et al (2008) observed that a correctly designed orthosis was able to partially correct the spinal curvature and pelvic obliquity, provide a stable sitting position, balance the trunk, facilitate breathing, discharge the abdomen, and unload soft tissues of the buttocks. 12 In the present study, an improvement of 5 degrees in Cobb angle and 3 degree in Ferguson angle was observed in A-P radiogram analysis for brace on and brace off conditions.…”

Section: Discussionmentioning

confidence: 99%

The effect of a spinal orthosis on posture and cardio-respiratory functions in progressive myopathic scoliosis

Mohanty

Tripathi

Lenka

et al. 2012

Prosthetics &Amp; Orthotics International

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Background: Historically, the orthotic treatment in progressive myopathic scoliosis has not been as effective as expected. The purpose of this study was to investigate the effect of a spinal orthosis on scoliotic curve correction, alignment of altered posture and cardio-respiratory functions. Case Description and Methods: An 11 year-old girl diagnosed with myopathy was fitted with a custom molded thoracolumbo-sacral orthosis to enhance the sitting ability. The cardio-respiratory tests were performed by a COSMED K4 b² metabolic analyzer. The anterior posterior radiographs were analyzed for measuring Cobb angle and Ferguson angle. A plumb line test was used to assess the postural improvements. Findings and Outcomes: No significant difference was observed for variables such as O 2 consumption level, PaO 2 , tidal volume, heart rate and energy expenditure/min. The improved posture was evident by shifting of the plumb line 18 mm towards the mid line with the use of orthosis. Very minor improvements in both angles were observed with the use of the brace. Conclusion:The brace treatment cannot be expected to have a lasting corrective effect although it can be used as a sitting support and for maintaining posture Clinical relevanceThis case study provides an objective prescription of thoraco-lumbo-sacral orthosis as a sitting support and for improving posture in progressive myopathic scoliosis if suitably designed and properly fitted.

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“…Havia um limiar baixo para a solicitação de raio X da coluna ou encaminhamento para um cirurgião de coluna vertebral para uma avaliação inicial. O tratamento conservador da escoliose incluía uso de colete, e, em casos graves, cirurgias de fixação e de fusão da coluna eram realizadas [17][18][19] . A osteopenia era comum nos meninos com DMD, e o efeito adicional dos esteroides orais aumentava a incidência de osteoporose e de fraturas da coluna vertebral, assim como de outras fraturas 20,21 .…”

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Long-term management of children with neuromuscular disorders

Strehle

2009

J Pediatr (Rio J)

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Objective: Duchenne muscular dystrophy is the commonest genetic myopathy but there exist a large number of inherited neuromuscular diseases which individually are very rare and where clinical information is not widely available. This review is based on the author's experience in a pediatric muscle clinic and provides practical guidance and treatment plans for frequently encountered problems. Sources:A MEDLINE search was conducted to retrieve recent articles relevant to the management of children with inherited myopathies and neuropathies. A patient cohort (n = 200) was evaluated using descriptive statistics. Summary of the findings:Duchenne muscular dystrophy accounted for almost half of the diagnoses, followed by spinal muscular atrophy (12%), Becker muscular dystrophy and myotonic dystrophy (7% each). Sixteen patients (9%) had an unknown myopathy. Conclusions:As with other chronic illnesses, these patients should be regularly reviewed by health professionals from an early age to increase life expectancy and improve quality of life. It is useful for physicians to take a structured approach when looking after children with neuromuscular disorders and to monitor all affected organ systems. ResumoObjetivo: A distrofia muscular de Duchenne é o tipo mais comum de miopatia genética. Contudo, existe um grande número de doenças neuromusculares hereditárias que são individualmente muito raras e sobre as quais não há muita informação clínica disponível. Este artigo de revisão baseia-se na experiência do autor em uma clínica pediátrica para tratamento de doenças musculares e apresenta orientação prática e planos terapêuticos para os problemas frequentemente encontrados. Fontes dos dados:O banco de dados da MEDLINE foi pesquisado com o objetivo de localizar artigos recentes e relevantes para o manejo de crianças com miopatias e neuropatias hereditárias. Uma coorte de 200 pacientes foi avaliada através de análise estatística descritiva. Síntese dos dados:A distrofia muscular de Duchenne representou quase metade dos diagnósticos, seguida da atrofia muscular espinhal (12%), da distrofia muscular de Becker e da distrofia miotônica (7% cada). Dezesseis pacientes (9%) apresentaram miopatia de origem desconhecida. Conclusões:Assim como ocorre com outras doenças crônicas, esses pacientes devem passar por acompanhamento periódico realizado por profissionais de saúde desde cedo para aumentar sua expectativa de vida e melhorar sua qualidade de vida. É útil para os médicos adotarem uma abordagem estruturada ao atender crianças com transtornos neuromusculares e monitorar todos os sistemas de órgãos afetados.J Pediatr (Rio J). 2009;85(5):379-384

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A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

Cited by 63 publications

References 74 publications

Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy

Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy

The effect of a spinal orthosis on posture and cardio-respiratory functions in progressive myopathic scoliosis

Long-term management of children with neuromuscular disorders

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