阿博特综合征和克鲁宗综合征的异常静脉袢及其与脑室大小和颅内压增高的关系。

IF 2.1 3区 医学 Q3 CLINICAL NEUROLOGY
Iris E Cuperus, Jip Y Mulders, Marjolein H G Dremmen, Catherine A de Planque, Irene M J Mathijssen, Marie-Lise C Van Veelen
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引用次数: 0

摘要

目的:阿博特综合征(Apert Syndrome)和克鲁宗综合征(Crouzon Syndrome)患儿经常出现的颅内静脉血管异常、脑室袢、脑室肥大与颅内压(ICP)升高之间的确切关系仍然是一个谜。本研究旨在评估异常静脉系统与脑室大小和ICP增高之间的关系,并评估静脉结构随时间的发展变化:这项回顾性队列研究纳入了所有阿博特综合征或克鲁宗综合征患者,这些患者均有脑部 CT 静脉造影(CTV)扫描结果。异常静脉血管通过总侧支评分(TCS)进行评估,该评分对 9 个颅内和颅外静脉结构进行评分(TCS 范围为 0-16)。脑室大小(前枕骨角比 [FOHR])在同一扫描中测量。ICP增高、分流、扁桃体疝和头围均从电子病历中提取,作为次要协变量。如果有后续的 CTV 扫描结果,则对其进行评分:本研究共纳入 90 名患者。TCS的平均值为7.5 ± 2.5,阿博特综合征和克鲁宗综合征患者的TCS值相当(平均值分别为8.0和7.3)。额外异常静脉结构的存在与 FOHR 增加 3.2% 相关(p < 0.01)。将静脉结构分为颅内静脉和颅外静脉后,发现两者与 FOHR 的关系相似(分别为 4.1% 和 2.3%;p < 0.01)。扫描时ICP正常和增高的患者的TCS相似。16 名患者接受了两次 CTV 扫描。两次扫描的中位间隔时间为 3.2 年。随着时间的推移,袢的存在保持稳定(中位数 ΔTCS = 0.3)。7名有功能分流的患者也保持了较高的TCS(中位数TCS = 9):结论:在阿博特综合征和克鲁宗综合征患者中,观察到静脉袢与脑室大小之间存在密切关系,其中颅内和颅外静脉引流模式异常与脑室较大有关。初步的纵向数据表明,尽管对 ICP 增高进行了有效治疗,但静脉袢的存在随着时间的推移保持不变,这表明即使 ICP 正常,也需要依赖这些静脉袢。作者假设,这种对侧支引流的依赖是其内部静脉解剖异常的结果,并使患者容易出现 ICP 增高。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anomalous venous collaterals in Apert and Crouzon syndromes and their relationship to ventricle size and increased intracranial pressure.

Objective: The exact association between the frequently present anomalous intracranial venous vasculature, emissary collaterals, ventriculomegaly, and increased intracranial pressure (ICP) in children with Apert and Crouzon syndromes remains an enigma. This study aimed to evaluate the association between the aberrant venous system and ventricle size and increased ICP, and to assess the development of the venous structures over time.

Methods: This retrospective cohort study included all patients with Apert or Crouzon syndrome with available CT venography (CTV) scans of the brain. Anomalous venous vasculature was assessed by the total collateral score (TCS), which scored 9 intra- and extracranial venous structures (TCS range 0-16). Ventricle size (fronto-occipital horn ratio [FOHR]) was measured on the same scan. The presence of increased ICP, a shunt, tonsillar herniation, and head circumference were extracted from electronic patient records and were used as secondary covariates. Subsequent CTV scans were scored when available.

Results: Ninety patients were included in this study. The mean TCS was 7.5 ± 2.5, and was comparable for patients with Apert and Crouzon syndromes (mean 8.0 and 7.3, respectively). The presence of an extra abnormal venous structure was associated with an increase of the FOHR of 3.2% (p < 0.01). After dividing the venous structures into intra- and extracranial, a similar association between both and the FOHR was found (4.1% and 2.3%, respectively; p < 0.01). The TCS was similar for patients with normal and increased ICP at the time of the scan. Sixteen patients had dual CTV scans. The median time between both scans was 3.2 years. The presence of collaterals remained stable over time (median ΔTCS = 0.3). Seven patients with functioning shunts also maintained high TCSs (median TCS = 9).

Conclusions: In patients with Apert and Crouzon syndromes, a close relationship between venous collaterals and ventricle size was observed, in which a more extensive aberrant venous drainage pattern, both intra- and extracranial, was associated with larger ventricles. Preliminary longitudinal data suggested that the presence of venous collaterals remained constant over time despite effective treatment of increased ICP, indicating reliance on these collaterals even in cases of normal ICP. The authors hypothesize that this dependence on collateral drainage is the result of their aberrant internal venous anatomy and predisposes individuals to increased ICP.

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来源期刊
Journal of neurosurgery. Pediatrics
Journal of neurosurgery. Pediatrics 医学-临床神经学
CiteScore
3.40
自引率
10.50%
发文量
307
审稿时长
2 months
期刊介绍: Information not localiced
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