【病例】颅内多发海绵状血管瘤1例CT及MR

18 3月

History: A 36-year-old man presents with new-onset seizures.

病史:36岁男性,癫痫发作。

A head CT scan was performed. 行颅脑CT平扫。

【病例】颅内多发海绵状血管瘤1例CT及MR

【病例】颅内多发海绵状血管瘤1例CT及MR

MR images

A contrast-enhanced MRI scan of the brain was performed. In order: Axial T2-weighted fluid-attenuated inversion-recovery (FLAIR), axial diffusion-weighted (DWI), two axial gradient-recalled echo (GRE), axial T1-weighted postcontrast, and coronal T1-weighted postcontrast images.

行颅脑增强MR,各序列如下面图中所示。

【病例】颅内多发海绵状血管瘤1例CT及MR

【病例】颅内多发海绵状血管瘤1例CT及MR

【病例】颅内多发海绵状血管瘤1例CT及MR

Findings

  • CT: There are multiple focal hyperdense lesions, including a large lesion with foci of calcification in the right corona radiata. There is no associated mass effect.
  • MRI: There are multiple T2 hypointense brain lesions with marked susceptibility artifact. There is no significant enhancement and no surrounding FLAIR signal. There is an abnormal draining vein from the right corona radiata lesion, consistent with a developmental venous anomaly.

影像表现:

  • CT:脑内多发局限性高密度灶,其中右侧放射冠一较大病灶可见钙化,无明显占位效应。
  • MRI:脑内多发T2低信号灶,可见明显磁敏感伪影,无明显强化及周围水肿,右侧放射冠区病灶见一异常引流静脉,符合发育性静脉异常表现。

Differential diagnosis

  • Amyloid angiopathy
  • Hypertensive bleeds
  • Hemorrhagic neoplasm
  • Cavernomas

Diagnosis: Multiple hereditary cavernomas

鉴别诊断:

  • 淀粉样血管病
  • 高血压性脑出血
  • 肿瘤合并出血
  • 海绵状血管瘤

诊断:多发性遗传性海绵状血管瘤

Key points

Cavernous malformations

海绵状血管瘤

Pathophysiology  病理生理学

  • Cavernous malformations, also termed cavernomas, are benign vascular hamartomas. 海绵状血管畸形,也称海绵状血管瘤,是良性的血管错构瘤。
  • They often hemorrhage, creating a characteristic imaging appearance. 病灶常出血,具有特征性的影像表现。
  • The most common classification system is the Zabramski classification system: 最常用的分型(Zabramski分型系统):
    • Type I: Subacute hemorrhagic 亚急性出血型
    • Type II: Intermediate age hemorrhage, most common, lobulated appearance 中间时期出血型(最常见,分叶状
    • Type III: Chronic hemorrhage 慢性出血型
    • Type IV: Multiple punctate microhemorrhages seen as foci of susceptibility 多发针尖样微出血型(表现为局灶性的磁敏感性)

Epidemiology  流行病学

  • They may be sporadic or hereditary (multiple hereditary cavernomas).  可为散发性或遗传性(多发遗传性海绵状血管瘤)。
  • There is no sex predilection.  无性别优势。
  • Typically found in patients 40 to 70 years old, possibly due to increased imaging in this population.  多见于40-70岁患者,可能由于这个阶段影像检查增加所致。
  • Multiple hereditary cavernoma syndrome is inherited in an autosomal dominant pattern. It is associated with developmental venous abnormalities, superficial siderosis, café au lait spots, and cherry angiomas. 多发遗传性海绵状血管瘤综合征是常染色体显性遗传,与发育性静脉异常、表面铁沉积、咖啡斑、樱桃血管瘤有关。

Clinical presentation 临床表现

  • Patients most often present with seizures or a focal neurologic deficit based on the location of lesions. 患者多表现为癫痫或与病灶相关的局灶性神经功能缺损。
  • They may be asymptomatic. 也可无任何症状。

【病例】颅内多发海绵状血管瘤1例CT及MR

Imaging

  • CT:On CT, they appear as hyperdense ovoid lesions representing blood products from prior hemorrhage. Lesions may be occult if small or no hemorrhage is present.
  • MRI:The most important characteristic is “blooming” artifact on susceptibility-weighted sequences, consistent with residual paramagnetic blood products from prior hemorrhage. Large lesions may have a lobulated appearance, and they often demonstrate an associated developmental venous anomaly on contrast-enhanced imaging.
  • Angiography:No findings on angiography unless accompanied by an associated developmental venous anomaly or arteriovenous malformation.

影像表现:

  • CT:多表现为高密度卵圆形病灶(由于早前的出血),如果病灶体积小或无出血,将很难被发现。
  • MRI:最重要的表现是在SWI序列上由于慢性出血(顺磁性含铁血黄素)导致的“开花”样低信号,较大病灶可呈分叶状,增强扫描常可见相关的发育性静脉异常。
  • 血管造影:无异常表现,除非合并发育性静脉异常或AVM。

Treatment 治疗

  • Treatment is through microsurgical resection or stereotactic radiosurgery. 显微手术切除或立体定向放射手术。
  • Identifying an associated developmental venous anomaly is important, as this is a contraindication for surgery due to the risk of a venous infarct. 明确有无相关的发育性静脉异常非常重要,其为手术禁忌症,因为有静脉栓塞的危险。

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