罕见的脑干血管母细胞瘤髓内出血。

Y-M Ryang, M F Oertel, A Thron, J Gilsbach, V Rohde
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引用次数: 7

摘要

目的:血管母细胞瘤引起的出血非常罕见,且多发生在蛛网膜下腔。由血管母细胞瘤引起的实质内出血更少见,这些出血几乎全部位于幕上脑、小脑和脊髓。我们报告第一例脑干出血由于延髓血管母细胞瘤。病例报告:一名47岁女性,表现为急性头痛、无音、吞咽困难、左侧偏瘫和感觉迟钝,伴有不同的警觉状态,最终发展为急性呼吸衰竭。颅脑计算机断层扫描(CT)和磁共振成像(MRI)显示一个小的延髓后血管母细胞瘤,引起脑实质内出血和急性闭塞性脑积水,由于脑室内血肿延伸。结果:脑室外置管治疗急性脑积水后,经显微外科后正中枕下入路,枕骨大孔扩大最小,可将成血管细胞瘤及相关出血全部切除。术后随访6个月,患者恢复良好,仅残留少量神经功能缺损。结论:脑干出血最常见的原因是动脉高血压和海绵状血管瘤。然而,血管母细胞瘤作为脑实质内脑干出血的可能鉴别诊断不应被忽视。虽然高血压脑干出血的预后大多是灾难性的,很少有手术指征,但对于由潜在肿瘤引起的出血,应考虑手术治疗。特别是在血管母细胞瘤的治疗中,手术治疗策略对成功的结果至关重要。因此,作者建议将血管母细胞瘤纳入脑干出血患者的诊断检查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Rare intramedullary hemorrhage of a brainstem hemangioblastoma.

Object: Hemorrhages caused by hemangioblastomas are very rare and mostly located in the subarachnoid space. Intraparenchymal bleedings due to hemangioblastomas are even less frequent, and these hemorrhages are almost exclusively located in the supratentorial brain, cerebellum and spinal cord. We report the first case of a brainstem hemorrhage due to a hemangioblastoma of the medulla oblongata.

Case report: A 47-year-old woman presented with acute onset of headache, anarthria, inability to swallow, left-sided hemiparesis and hemidysesthesia with varying states of vigilance, finally developing acute respiratory failure. Cranial computed tomography (CT) scanning and magnetic resonance imaging (MRI) revealed a small hemangioblastoma of the posterior medulla oblongata causing intraparenchymal hemorrhage and acute occlusive hydrocephalus due to intraventricular hematoma extension.

Result: After implantation of an external ventricular catheter to treat acute hydrocephalus, the hemangioblastoma as well as its associated hemorrhage could be removed in toto via a microsurgical posterior median suboccipital approach with minimal foramen magnum enlargement. During the follow-up period of six months postoperatively the patient showed good recovery with only slight residual neurological deficits.

Conclusion: The most common causes of brainstem hemorrhages are arterial hypertension and cavernous hemangiomas. However, hemangioblastomas should not be ignored as a possible differential diagnosis for intraparenchymal brainstem hemorrhage. While the prognosis in hypertensive brainstem bleedings is mostly disastrous and surgery rarely indicated, an operative therapy should be considered in cases of hemorrhages caused by underlying tumors. Especially in the treatment of hemangioblastoma, the surgical management strategy is crucial for a successful result. Therefore, the authors recommend including the search for hemangioblastomas into the diagnostic workup in patients with brainstem hemorrhages.

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Zentralblatt Fur Neurochirurgie
Zentralblatt Fur Neurochirurgie 医学-神经科学
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