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脊髓脊膜膨出患儿Chiari II型畸形的一期和二期治疗

Primary and secondary management of the Chiari II malformation in children with myelomeningocele.

作者信息

Messing-Jünger Martina, Röhrig Andreas

机构信息

Neurosurgical Department, Asklepios Children's Hospital, Arnold-Janssen Strasse 29, 53757, Sankt Augustin, Germany.

出版信息

Childs Nerv Syst. 2013 Sep;29(9):1553-62. doi: 10.1007/s00381-013-2134-4. Epub 2013 Sep 7.

Abstract

OBJECT

Chiari malformation type II is almost exclusively found in patients with open spinal dysraphism. Etiology and pathophysiology are not yet completely understood, and management guidelines regarding the best follow-up and treatment of this pathological entity do not exist. In order to assess essential management aspects, literature and a series of secondary neurosurgical interventions in Chiari II patients have been reviewed.

METHODS

A literature review regarding etiology, diagnostics, pathophysiology, and management of Chiari malformation type II (CMII) and a retrospective evaluation of a series (2009-2012) of secondary interventions in Chiari II patients have been performed. Inclusion criteria were ICD for myelomeningocele with or without hydrocephalus and ICD for Chiari malformation and neurosurgical OR procedure. Evaluated parameters were: patient demographics, primary management, secondary neurosurgical operations (cranio-cervical decompression, shunt revision, myelolysis) as well as specific findings pre- and postoperatively. Essential results from literature review and patients' series are compiled in order to define management recommendations.

RESULTS

Fifty patients (28 f, 22 m; mean age, 7.1 years (range, 0.5-26 years)) with myelomeningocele-associated Chiari malformation type II were operated on between 2009 and 2012. Twenty-four patients had syringomyelia and scoliosis each, and 12 suffered from both. Orthopedic surgery for scoliosis or kyphosis had been performed in 13 cases. Shunt revision was performed in 38 cases, myelolysis in 17, and decompression of the foramen magnum in 14 (28 %). After a mean follow-up of 1.9 years, syringomyelia decreased from 24 to 16 cases. There was a postoperative reduction of neck pain (one third), sensorimotor (two fifths), and cranial nerve deficits (one half). CSF flow at the foramen magnum did not change visibly after surgery. Ventricular size improved in about half of the patients. Slit-like ventricles were found in nine (6 pre-surgical) and enlarged ventricles in nine (23 pre-surgical). Complication rate was 6 % (3/50) per cases, and no patient died or deteriorated neurologically after surgery.

CONCLUSION

CMII-related management guidelines are not well defined, since clinical constellations and presentations are varying. Often associated findings are syringomyelia, hydrocephalus, and scoliosis, and symptomatic CMII may be triggered by more than one underlying condition. According to literature and clinical experience, management recommendations can be defined. The most important finding is that hydrocephalus is often involved in symptomatic CMII and must always be considered first in any symptomatic patient. Intrinsic brain stem dysfunctions cannot be treated surgically, and monitoring of vital functions is sometimes the only clinical means that can be offered to the patient. Knowledge of the complex background has led to improved follow-up programs for the affected children and thus also improved longtime survival.

摘要

目的

II型Chiari畸形几乎仅见于开放性脊柱裂患者。其病因和病理生理学尚未完全明确,关于这一病理实体最佳随访和治疗的管理指南也不存在。为了评估基本的管理方面,我们回顾了相关文献以及一系列针对II型Chiari畸形患者的二次神经外科干预措施。

方法

我们进行了一项关于II型Chiari畸形(CMII)病因、诊断、病理生理学和管理的文献综述,并对2009年至2012年期间一系列II型Chiari畸形患者的二次干预措施进行了回顾性评估。纳入标准为患有或不患有脑积水的脊髓脊膜膨出的国际疾病分类(ICD)以及Chiari畸形和神经外科手术的ICD。评估参数包括:患者人口统计学特征、初始管理、二次神经外科手术(颅颈减压、分流术修正、脊髓空洞切开术)以及术前和术后的具体发现。汇总文献综述和患者系列的基本结果以确定管理建议。

结果

2009年至2012年期间,对50例患有脊髓脊膜膨出相关II型Chiari畸形的患者(28例女性,22例男性;平均年龄7.1岁(范围0.5 - 26岁))进行了手术。24例患者患有脊髓空洞症,24例患有脊柱侧弯,12例同时患有这两种疾病。13例患者接受了脊柱侧弯或后凸畸形的矫形手术。38例患者进行了分流术修正,17例进行了脊髓空洞切开术,14例(28%)进行了枕骨大孔减压术。平均随访1.9年后,脊髓空洞症患者从24例减少至16例。术后颈部疼痛(减少三分之一)、感觉运动障碍(减少五分之二)和颅神经功能缺损(减少一半)的情况有所改善。手术后枕骨大孔处的脑脊液流动没有明显变化。约一半的患者脑室大小有所改善。发现9例患者存在裂隙样脑室(术前6例),9例患者存在脑室扩大(术前23例)。并发症发生率为6%(3/50),术后无患者死亡或神经功能恶化。

结论

由于临床情况和表现各异,CMII相关的管理指南尚未明确界定。常见的相关表现包括脊髓空洞症、脑积水和脊柱侧弯,有症状的CMII可能由多种潜在疾病引发。根据文献和临床经验,可以确定管理建议。最重要的发现是脑积水常与有症状的CMII相关,在任何有症状的患者中都必须首先考虑。脑干内在功能障碍无法通过手术治疗,有时对重要功能的监测是能为患者提供的唯一临床手段。对复杂背景的了解促使针对受影响儿童的随访计划得到改进,从而也提高了长期生存率。

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