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多发脊髓髓内海绵状血管畸形伴出血发作,类似于髓内肿瘤。

Multiple spinal intramedullary cavernous angiomas with bleeding episode mimicking an intramedullary tumor.

机构信息

Doctoral Program of Medical Science, Faculty of Medicine, Universitas Airlangga, Indonesia.

Radiology Department, Faculty of Medicine, Universitas Airlangga, Indonesia.

出版信息

J Radiol Case Rep. 2022 Mar 1;16(3):15-22. doi: 10.3941/jrcr.v16i3.4251. eCollection 2022 Mar.

Abstract

Intramedullary cavernous angioma is a rare vascular malformation compared to cerebral cavernous malformation. The incidence of cavernous angioma is about 3 - 5% of all central nervous system lesions, 5 - 12% of all spinal vascular lesions, and 1% of all intramedullary lesions in pediatric patients. Although intramedullary cavernous angioma has the same histological picture as cerebral cavernous angioma, the natural history, and surgical approach are different from cerebral cavernous angioma. Due to its location in the eloquent area of the spinal medulla, a slight change in the size of the lesion can affect the neurological function of the patient. We describe a case of an intramedullary cavernous angioma with hemosiderin post bleeding, located in the cervical cord which was initially misdiagnosed as hemorrhagic ependymoma. On whole spine MR imaging we also found an intramedullary cavernous angioma in the lower thoracal cord at the T12 level with mild hemorrhage. Abdominal MRI showed cavernous angiomas in both kidneys. The patient underwent surgical treatment with removal of the lesion in the cervical cord and T12 level, with histopathologic findings consistent with cavernous angioma. No malignancy was detected. Post-surgery, the symptoms gradually improved. Symptomatic intramedullary cavernous angioma tends to bleed repeatedly and being unstable. Early diagnosis and surgical treatment can prevent rebleeding and more severe symptoms.

摘要

脊髓内海绵状血管畸形与脑内海绵状血管畸形相比较为少见。海绵状血管畸形的发病率约占所有中枢神经系统病变的 3-5%,所有脊髓血管病变的 5-12%,以及所有儿童患者脊髓内病变的 1%。尽管脊髓内海绵状血管畸形在组织学上与脑内海绵状血管畸形相同,但其自然病史和手术方法与脑内海绵状血管畸形不同。由于其位于脊髓的功能区,病变大小的轻微变化可能会影响患者的神经功能。我们描述了一例位于颈髓的伴出血后含铁血黄素沉积的海绵状血管畸形,最初误诊为出血性室管膜瘤。全脊柱磁共振成像还显示 T12 水平的胸髓下段有另一例海绵状血管畸形伴轻度出血。腹部 MRI 显示双侧肾脏有海绵状血管畸形。患者接受了手术治疗,切除了颈髓和 T12 水平的病变,组织病理学检查结果符合海绵状血管畸形。未发现恶性肿瘤。术后症状逐渐改善。有症状的脊髓内海绵状血管畸形倾向于反复出血且不稳定。早期诊断和手术治疗可以防止再出血和更严重的症状。

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