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先天性脑积水分流治疗后患者的成人长期预后。

Adult long-term outcome of patients after congenital hydrocephalus shunt therapy.

作者信息

Preuss M, Kutscher A, Wachowiak R, Merkenschlager A, Bernhard M K, Reiss-Zimmermann M, Meixensberger J, Nestler U

机构信息

Department of Neurosurgery, Pediatric Neurosurgery, University Hospital Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany,

出版信息

Childs Nerv Syst. 2015 Jan;31(1):49-56. doi: 10.1007/s00381-014-2571-8. Epub 2014 Oct 17.

DOI:10.1007/s00381-014-2571-8
PMID:25323796
Abstract

INTRODUCTION

Even though shunt surgery has been an established and widely accepted treatment for congenital hydrocephalus for five decades, long-term follow-up and functional outcome data is rare.

PATIENTS AND METHODS

Sixty-nine patients were examined after congenital hydrocephalus had been treated between 1971 and 1987 at the Department of Pediatric Surgery (University of Leipzig) within the first 12 months of life. Median age of patients was 32 years (range 25-42 years). Patients agreed to undergo a contemporary hydrocephalus assessment.

RESULTS

Seven patients (10.1%) became shunt-independent before adulthood. By the age of 20, 82% of the patients had needed at least one shunt revision, 100% by the age of 30. 21.7% of the primary valves (Spitz-Holter) remained intact without revision until today up to 35 years (mean functional intactness 23 years). Shunt infections occurred in 4.3% of primary implantations. 48% of the patients had a good functional outcome (mRS = 0-1). 40.9% of the patients attended regular school. In summary, 75% patients work on a daily basis. 44.6% live an independent life, 35.4% rely on parental supervision, and 20% depend on professional care institutions.

CONCLUSION

Compared to the pre-shunt era, surgery within the first year of life is advantageous regarding visual function, educational progress, and social results. The outcome achieved throughout childhood remains stable during adult life as long as catastrophic events of shunt malfunction can be prevented. Epilepsy, motor deficits, acute shunt dysfunction, and problems of social integration as well as aging parental caregivers seem to be prominent factors of morbidity in adulthood.

摘要

引言

尽管分流手术作为先天性脑积水的一种既定且被广泛接受的治疗方法已有五十年,但长期随访和功能结局数据却很少见。

患者与方法

对1971年至1987年间在莱比锡大学儿外科接受先天性脑积水治疗的69例患者在出生后的头12个月内进行了检查。患者的中位年龄为32岁(范围25 - 42岁)。患者同意接受当代脑积水评估。

结果

7例患者(10.1%)在成年前无需分流。到20岁时,82%的患者至少需要进行一次分流修复,到30岁时这一比例为100%。21.7%的初级瓣膜(斯皮茨 - 霍尔特瓣膜)至今保持完整未进行修复,长达35年(平均功能完好时长23年)。初次植入时分流感染发生率为4.3%。48%的患者功能结局良好(改良Rankin量表评分为0 - 1)。40.9%的患者正常上学。总体而言,75%的患者能够正常工作。44.6%的患者独立生活,35.4%的患者依赖父母监管,20%的患者依赖专业护理机构。

结论

与分流手术前的时代相比,出生后第一年内进行手术在视觉功能、教育进展和社会成果方面具有优势。只要能预防分流故障的灾难性事件,儿童期取得的结局在成年期会保持稳定。癫痫、运动功能障碍、急性分流功能障碍、社会融入问题以及父母照顾者老龄化似乎是成年期发病的突出因素。

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