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小儿小脑巨大海绵状血管畸形:病例报告及文献复习

Pediatric cerebellar giant cavernous malformation: case report and review of literature.

作者信息

Villaseñor-Ledezma Javier, Budke Marcelo, Alvarez-Salgado Juan-Antonio, Cañizares María-Angeles, Moreno Luis, Villarejo Francisco

机构信息

Department of Neurosurgery, Complejo Hospitalario de Toledo, Ave. Barber 30, 45005, Toledo, Spain.

Department of Neurosurgery, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

出版信息

Childs Nerv Syst. 2017 Dec;33(12):2187-2191. doi: 10.1007/s00381-017-3550-7. Epub 2017 Jul 25.

Abstract

BACKGROUND AND IMPORTANCE

Giant cavernous malformations (GCM) are low flow, angiographically occult vascular lesions, with a diameter >4 cm. Cerebellar GCMs are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage, being surgery clearly recommended.

CLINICAL PRESENTATION

An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness. An MRI revealed a 57 × 46 × 42 mm multi-cystic, left cerebellar hemisphere mass, showing areas of hemorrhages and cysts with various stages of thrombus. There was no enhancement with contrast. Cerebral angiography ruled out an arteriovenous malformation. She underwent a left paramedian occipital craniotomy, and macroscopic gross total resection was accomplished. Histopathologic examination was consistent with a cavernous malformation. After surgery, the patient had no new neurological deficit and an uneventful postoperative recovery. Follow-up MRI confirmed total removal of the lesion.

CONCLUSION

Cerebellar GCMs in children are symptomatic lesions, which prompt immediate surgical treatment. These are rare lesions, which can radiologically and clinically mimic a tumor with bleed, having to be considered in the differential diagnosis of neoplastic lesions. Cerebellar GCMs might be suspected in the presence of large hemorrhagic intra-axial mass with "bubbles of blood," multi-cystic appearance, surrounded by hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect. Careful radiological study provides a preoperative diagnosis, but its confirmation requires histopathological examination. Complete surgical removal should be attempted when possible.

摘要

背景与重要性

巨大海绵状血管畸形(GCM)是低流量、血管造影隐匿性血管病变,直径>4厘米。小脑GCM极为罕见,基于英文文献仅报道了7例。这些病变最常见于儿童年龄组,已知该组出血风险增加,故明确推荐手术治疗。

临床表现

一名18个月大女孩有6个月斜颈及上肢笨拙病史。磁共振成像(MRI)显示左小脑半球有一个57×46×42毫米的多囊性肿块,伴有不同阶段血栓的出血区和囊肿。增强扫描无强化。脑血管造影排除动静脉畸形。她接受了左枕部旁正中开颅手术,并实现了肉眼下全切。组织病理学检查符合海绵状血管畸形。术后患者无新的神经功能缺损,术后恢复顺利。随访MRI证实病变已完全切除。

结论

儿童小脑GCM是有症状的病变,需立即进行手术治疗。这些是罕见病变,在影像学和临床上可模拟伴有出血的肿瘤,在肿瘤性病变的鉴别诊断中必须考虑。在出现伴有“血泡”的大的轴内出血性肿块、多囊性表现、被含铁血黄素环包绕、液-液平面以及伴有水肿-肿块效应时,可能怀疑为小脑GCM。仔细的影像学研究可提供术前诊断,但其确诊需要组织病理学检查。可能的话应尝试完整手术切除。

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