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遺傳性出血性毛細血管擴張癥繼發(fā)腦膿腫

 zskyteacher 2018-07-09

【雙語病例】遺傳性出血性毛細血管擴張癥繼發(fā)腦膿腫

來源:雙語學影像;本期病例選自AuntMinnie.com

Our appreciation is extended to Dr. Po-Hao Chen, University of Pennsylvania Department of Radiology, for contributing this case.

History and initial images

History

A 69-year-old woman with severe headache and fever presents to the emergency department. Initial unenhanced head CT was negative for intracranial bleed. After obtaining additional history, a CT angiography of the thorax was performed.

69歲女性,因嚴重頭疼、發(fā)熱來急診科就診,最初行頭顱CT平掃未見明顯出血。隨后仔細詢問其他病史,并行胸部CTA檢查。

Select images from an initial chest radiograph and chest CT are shown below.

1.What is the most prominent finding?

Pulmonary embolism 肺動脈栓塞

Pulmonary arteriovenous malformation 肺動靜脈畸形

Pulmonary nodule 肺內(nèi)結節(jié)

Cardiomegaly 心影增大


2.Which of the following is LEAST likely to be the cause of this patient's headache?

下列哪項最不可能是患者頭疼的原因?

Glioblastoma 膠質(zhì)母細胞瘤

Abscess 腦膿腫

Cysticercosis 腦囊蟲病

Metastatic disease 轉(zhuǎn)移瘤

Enhanced CT images

After initial unenhanced head CT was negative, an enhanced head CT is subsequently obtained.

頭顱CT增強掃描


The clinicians requested this patient's outside enhanced abdominal CT, for which select images are displayed below.

外院上腹部CT增強掃描



3.In the light of all imaging findings thus far, which of the following is the most likely diagnosis for the lesion in the region of the left thalamus?

根據(jù)以上影像資料,左側丘腦的病灶最有可能的診斷是什么?

Glioblastoma 膠質(zhì)母細胞瘤

Neurocysticercosis 腦囊蟲病

Brain abscess 腦膿腫

Cerebral vascular aneurysm 腦血管瘤


4.The abdominal images demonstrate hepatic arteriovenous malformations.

上腹部CT示肝動靜脈畸形。

True

False


5.In the light of all the imaging data, what is the most likely underlying diagnosis?

根據(jù)以上資料,最有可能的診斷是什么?

Hereditary hemorrhagic telangiectasia 原發(fā)性毛細血管擴張癥

Wyburn-Mason syndrome懷梅二氏綜合征(神經(jīng)視網(wǎng)膜血管瘤綜合征)

Septic embolism 膿毒性栓塞



選擇題答案:

  1. Pulmonary arteriovenous malformation

    These metallic-density objects are most likely embolization coils. This is most likely secondary to prior embolization of arteriovenous malformations.

    金屬高密度影考慮為肺動靜脈畸形栓塞的線圈,

  2. Cysticercosis

  3. Brain abscess

    Recurrent pulmonary arteriovenous malformations (as inferred by multiple, bilateral embolization coils and new AVM on the same-day CT) raises the possibility of hereditary hemorrhagic telangiectasia (HHT). A rim-enhancing brain mass in the setting of HHT puts brain abscess as the top differential diagnosis.

    反復發(fā)作的肺動靜脈畸形(由雙側多發(fā)栓塞線圈及同一天CT所見的新發(fā)AVM病灶判斷)提示遺傳性出血性毛細血管擴張癥( hereditary hemorrhagic telangiectasia,HHT)的可能。有HHT病史的腦內(nèi)環(huán)形強化灶,應首先考慮腦膿腫的可能性。

  4. True

    These lesions represent hepatic AVMs. Hepatic AVMs tend to be clinically silent so are not routinely screened.

    肝內(nèi)病灶考慮肝臟AVM。肝臟AVM通常沒有臨床癥狀,因此不用常規(guī)篩查。

  5. Hereditary hemorrhagic telangiectasia


Findings

  • Initial chest radiograph: There are nodular opacities in the bilateral lungs that, in the context of numerous embolization coils, most likely represent pulmonary arteriovenous malformations.

    最初胸片:兩肺內(nèi)結節(jié)影,結合肺內(nèi)多個栓塞線圈,考慮肺動脈畸形。

  • Chest CT: Multiple bilateral pulmonary arterial venous malformations that have increased from 1990. Status post bilateral embolization.

    胸部CT:兩肺多發(fā)AVM栓塞術后,與1990年時相比,病灶數(shù)量增加。

  • Enhanced brain CT: A 2.9-cm rim-enhancing mass of the left thalamus with mild to moderate associated mass effect. In the context of hereditary hemorrhagic talengiectasia and clinical presentation of fever, this most likely represents an abscess. Though less likely, additional differential considerations include primary glial neoplasm or metastasis.

    顱腦CT增強掃描:左側丘腦區(qū)環(huán)形強化灶,直徑約2.9cm,病灶可見輕至中度占位效應。結合患者診斷“遺傳性出血性毛細血管擴張癥”及發(fā)熱癥狀,考慮肺膿腫。其他鑒別診斷包括低級別神經(jīng)膠質(zhì)腫瘤和轉(zhuǎn)移瘤。

  • Abdominal CT: Numerous hepatic arteriovenous malformations are seen throughout the right and left hepatic lobes without active extravasation of contrast.

    上腹部CT:肝臟左右葉多發(fā)AVM,未見明顯造影劑外滲。


Diagnosis

Cerebral abscess secondary to hereditary hemorrhagic talengiectasia

遺傳性出血性毛細血管擴張癥繼發(fā)腦膿腫

Discussion

Also known by its eponym, Osler-Weber-Rendu syndrome, hereditary hemorrhagic telangiectasia is an autosomal dominant, rare condition caused by abnormal vascular structure. In telangiectasis, a postcapillary venule becomes markedly dilated and convoluted. Additionally, the connecting arterioles also become dilated and bypass the capillary bed to connect directly to the venules. There is perivascular infiltrate and thickened smooth muscle walls. The genetic predisposition has been linked to chromosome 9.

遺傳性出血性毛細血管擴張癥,根據(jù)其發(fā)現(xiàn)者的名字又稱Osler-Weber-Rendu綜合征,是一種罕見的常染色體顯性遺傳性血管異常。由于毛細血管擴張,毛細血管微靜脈明顯擴張、迂曲,小動脈也可見擴張,繞過毛細血管床直接與微靜脈相連。血管周圍滲出、血管壁平滑肌層增厚。

Clinical Presentation
  • Epistaxis 鼻出血

  • Cutaneous telangiectases 皮膚毛細血管擴張

  • Multiple and bilateral pulmonary arteriovenous malformation (AVM)兩肺多發(fā)肺動靜脈畸形

  • Gastrointestinal tract - AVM and angiodysplasias of the liver, stomach, and small and large bowels

    胃腸道:肝、胃、大腸、小腸的AVM或血管發(fā)育不良

  • Neurologic manifestations神經(jīng)形態(tài)表現(xiàn):

    • Migraine 偏頭痛

    • Brain abscess 腦膿腫

    • Transient ischemic attack 短暫性缺血發(fā)作

    • Seizures 癲癇

    • Subarachnoid hemorrhage 蛛網(wǎng)膜下腔出血

    • Paraparesis 下肢輕瘓



In patients with HHT, brain abscesses occur primarily in patients with pulmonary AVM. The pathogenesis of these brain abscesses are thought to arise from septic emboli passing through the AVM to the left-heart circulation, terminating in the small vessels of the brain.

毛細血管擴張癥的患者在,腦膿腫好發(fā)生于肺動靜脈畸形的患者。其病因主要是膿毒栓子穿過AVM、進入左心循環(huán),最后終止于腦的小血管。


Although only a small number (5% in at least one study) of patients with HHT are found to have cerebral AVMs, the presence of cerebral AVM is highly suggestive of HHT (50%).

僅有少量的HHT患者發(fā)現(xiàn)腦動靜脈畸形(5%),而腦AVM的發(fā)現(xiàn)高度提示HHT(50%).

Imaging Findings of HHT

Contrast CT or CT angiography are used for lung and liver evaluation. Brain is best evaluated with contrast-enhanced MRI for cerebral AVM.

增強CT或CTA通常用于肺部和肝臟檢查。顱腦AVM最好由MRI增強掃描評估。

Chest CT
Briskly enhancing arteriovenous malformations, arteriovenous fistulas

動靜脈畸形病灶迅速強化,可見動靜脈瘺

Brain CT
  • Serpentine vessels 血管迂曲擴張

  • Abscesses 膿腫

  • Enhancing AVM 增強掃描AVM病灶強化。

Abdominal CT
  • Arterioportal/arteriosystemic shunts

    肝動脈-門靜脈瘺/肝動脈-肝靜脈瘺

  • Hepatic talengiectasias

    肝臟毛細血管擴張

  • Large vascular masses

    大的血管性腫塊

Brain MR
  • T1-weighted imaging, T2-weighted imaging: Cerebral AVM (cAVM) with flow voids and hemorrhage

    T1WI、T2WI:腦AVM可見流空血管影及出血。

  • MR angiography: cAVMs; contrast administration enhances sensitivity for small cAVMs

    MRA:可見腦AVM;增強掃描可見小的AVM病灶強化。

  • MR venography: May demonstrate developmental venous anomalies

    MR靜脈造影:可見發(fā)育異常的靜脈血管



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