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Intracranial Cystic Cavernous Malformations 颅内囊性海绵状血管畸形

 panyunbo 2015-12-18

本期病例选自AJNR Case of the Week

Douglas Taylor, BS; Tyler Auschwitz, MD; L. Madison Mitchell II, MD
Department of Neurosurgery
University of Tennessee Health Sciences Center
Memphis, TN
双语学影像授权发送


Description

A 53-year-old woman in the emergency department with new-onset seizure

53岁男性,新发癫痫发作就诊。




Legends

Axial NECT head (A) shows a right frontal cystic lesion with a hyperdense solid nodule. Axial T2WI (B) shows a cystic lesion with a hypointense central solid component. Axial T2 FLAIR (C) shows signal cancellation of the cystic component. Axial T2* (D) reveals signal dephasing of the solid component in the absence of coarse calcifications (see A) and a rim of hemosiderin deposition in the cyst wall. Axial CE-T1WI (E) shows minimal enhancement of the solid component of the lesion.

CT平扫(A)示右侧额叶囊性病灶,内可见实性高密度结节。

横断位T2WI(B)示囊性病变可见中央低信号实性成分。

横断位T2-FLAIR(C)示囊性部分信号抑制,呈低信号。

横断位T2*WI示(D)病灶实性成分内没有明显的钙化。囊壁低信号提示含铁血黄素沉着。

横断位T1WI增强扫描(E)病灶内实性部分轻度强化。



Intracranial Cystic Cavernous Malformations

颅内海绵状血管畸形

Background

Intracranial cavernous malform。ations (CM) are benign vascular hamartomas consisting of irregular dilated vascular channels. Cystic cavernous malformations of the brain are relatively uncommon with only a few cases previously reported in the literature.

颅内海绵状血管畸形是一种良性血管错构瘤,由不规则扩张的血管组成。囊性海绵状血管畸形临床罕见,文献中仅有少量报道。

Clinical Presentation

Like many intracranial lesions, the presenting symptoms of cystic CMs are often related to their location. Common presenting symptoms include headache (32%), cerebellar signs (32%), papilledema (24%), hemiparesis (20%), and seizure (20%).

与大多数颅内病变类似,囊性海绵状血管畸形的症状与病灶所在的位置相关,主要症状包括头痛(32%),小脑体征(32%),视乳头水肿(24%),轻偏瘫(20%),癫痫发作(20%)。

Key Diagnostic Features

Cystic CMs have an inconsistent radiographic appearance, which can be attributed to the progression through different stages of cystic degeneration or the degree of thrombosis.

囊性海绵状血管畸形囊变或血栓形成的病变过程不同,而出现不同的影像学表现。

  • On CT, CMs appear as a heterogeneous cystic lesion with a hyperdense solid component

    CT上,海绵状血管畸形表现为囊性病变伴高密度实性部分。

  • On MRI, the ordinary findings include a mixed intensity nodule on T1- and T2-weighted images. The cysts often appear iso- to high-intensity on T1-weighted images, and hyperintense on T2-weighted images.

    MR通常表现为T1WI、T2WI混杂信号结节,囊性部分T1WI常呈等或高信号,T2WI高信号。

  • Because of their inconsistent radiographic appearance, surgery is often necessary to provide a histopathological specimen for definitive diagnosis.

    由于病变影像学表现复杂,最后确诊需要依靠手术病理。


DDx
  • Oligodendroglioma

    少突胶质细胞瘤

  • Cystic meningioma

    囊性脑膜瘤

  • Metastasis

    转移瘤

  • Hemangioblastoma

    血管母细胞瘤

  • Parasitic cyst

    寄生虫性囊肿

Treatment

Surgery is the optimal management for symptomatic cystic CMs. Considering the outcome, total removal of the nodule with partial resection of the cyst is considered the preferred strategy to avoid significant morbidity.

囊性海绵状血管畸形需要考虑手术治疗。完全切除实性结节伴囊性部分部分切除是最佳的手术策略,有助于患者的预后。


来源:双语学影像

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