Jenkinson Michael D, Campbell Sophie, Hayhurst Caroline, Clark Simon, Kandasamy Jothy, Lee Maggie K, Flynn Ann, Murphy Peter, Mallucci Conor L
Department of Neurosurgery, The Walton Centre Foundation NHS Trust, Lower Lane, Liverpool, L9 7LJ, UK.
Childs Nerv Syst. 2011 Jun;27(6):967-74. doi: 10.1007/s00381-010-1368-7. Epub 2010 Dec 31.
The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.
Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated.
Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social-emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function.
Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.
脊柱裂- Chiari II型畸形-脑积水综合征的长期预后尚不清楚。传统的神经外科预后指标较为粗略。神经心理学测试在预后评估中越来越重要。我们调查了出生时行脊髓脊膜膨出修补术的成年人的健康状况、残疾情况、生活方式和认知功能。
在神经外科/神经心理学联合门诊对常规随访的成年患者进行评估。患者完成生活方式问卷、脑积水预后问卷（HOQ）并接受认知测试。研究了可能影响预后的临床变量，包括分流管修订次数、分流管感染和枕骨大孔减压术。
对21名中位年龄为35岁的成年人进行了调查。所有患者均接受过脑积水治疗，8例因头痛或进行性脑干症状接受了枕骨大孔减压术，其中7例症状稳定，1例症状改善。只有8名患者独立生活，5名有带薪工作，5名自愿工作。HOQ认知功能评分（0.56±0.20；平均值±标准差[SD]）低于身体（0.64±0.15）和社会情感（0.65±0.17）健康评分。整个队列的认知功能各不相同，注意力受影响最严重（73.9±17.0；平均值±SD）。分流管故障或枕骨大孔减压术的反复发生与较差的认知功能无关。
尽管在儿童期进行了干预且脑脊液分流充分，但成年后独立生活的预后仍然很差。所有患者都有认知障碍的因素。脑结构异常可能比分流管故障更重要地决定认知预后。
