Danielsson Aina J, Bartonek Asa, Levey Eric, McHale Kathleen, Sponseller Paul, Saraste Helena
Department of Orthopaedics, Sahlgrenska University Hospital, 413 45, Göteborg, Sweden,
J Child Orthop. 2008 Feb;2(1):45-54. doi: 10.1007/s11832-007-0069-6. Epub 2007 Dec 15.
Modern principles for treatment of patients with myelomeningocele include early closure of the neural tube defect, neurosurgical treatment of hydrocephalus and treatment aimed at minimizing contractures and joint dislocations. The aim is to achieve a better survival rate and a better quality of life (QOL). Better ambulatory function is thought to improve the management of activities of daily living. This study focused on evaluating which factors might affect ambulation, function and health-related QOL in children with myelomeningocele.
Thirty-eight patients with neurological deficit from myelomeningocele were examined in an unbiased follow-up. This included a physical examination using validated methods for ambulatory function and neuromuscular status, chart reviews and evaluation of radiographs in terms of hip dislocation and spine deformity. The Pediatric Evaluation of Disability Inventory (PEDI) was used to measure mobility, self-care and social function, and the Child Health Questionnaire (CHQ-PF50) was used to measure QOL.
Muscle function class, quadriceps strength, spasticity in hip and/or knee joint muscles and hip flexion contracture as well as the ambulatory level all affected functional mobility as well as self-care/PEDI. Patients with hip dislocation, spinal deformity or those who were mentally retarded also had significantly worse functional mobility. Besides being affected by the severity of the neurological lesion, self-care/PEDI was significantly impaired by hip flexion contracture and absence of functional ambulation. General health-related QOL was significantly lower in this patient group than for US norms. Nonambulatory and mentally retarded patients had a significantly lower physical function of their QOL (CHQ).
The severity of the disease, i.e. reduced muscle strength and occurrence of spasticity around hip/knee, affected ambulation, functional mobility and self-care. Acquired deformities (hip dislocation and spine deformity) affected functional ambulation only. Patients with reduced functional mobility and self-care experienced lower physical QOL. Children with myelomeningocele had significantly reduced QOL compared to healthy individuals.
现代治疗脊髓脊膜膨出患者的原则包括早期闭合神经管缺陷、对脑积水进行神经外科治疗以及旨在尽量减少挛缩和关节脱位的治疗。目标是实现更高的生存率和更好的生活质量（QOL）。更好的行走功能被认为可改善日常生活活动的管理。本研究着重评估哪些因素可能影响脊髓脊膜膨出患儿的行走、功能及与健康相关的生活质量。
对38例患有脊髓脊膜膨出所致神经功能缺损的患者进行了无偏倚随访。这包括使用经过验证的方法对行走功能和神经肌肉状况进行体格检查、病历审查以及对髋关节脱位和脊柱畸形的X线片评估。使用儿童残疾评估量表（PEDI）来测量活动能力、自我护理和社会功能，使用儿童健康问卷（CHQ-PF50）来测量生活质量。
肌肉功能分级、股四头肌力量、髋关节和/或膝关节肌肉的痉挛以及髋关节屈曲挛缩以及行走水平均影响功能活动能力以及自我护理/PEDI。患有髋关节脱位、脊柱畸形或智力发育迟缓的患者功能活动能力也明显较差。除了受神经病变严重程度影响外，自我护理/PEDI还因髋关节屈曲挛缩和缺乏功能性行走而明显受损。该患者组与健康相关的总体生活质量明显低于美国标准。不能行走和智力发育迟缓的患者其生活质量的身体功能明显较低（CHQ）。
疾病的严重程度，即肌肉力量减弱以及髋/膝关节周围痉挛的发生，影响行走、功能活动能力和自我护理。后天性畸形（髋关节脱位和脊柱畸形）仅影响功能性行走。功能活动能力和自我护理能力下降的患者身体生活质量较低。与健康个体相比，脊髓脊膜膨出患儿的生活质量明显降低。
