辛本柱，佟丹，劉彥紅，艾達(dá)爾艾力·斯拉依丁，劉學(xué)，黃如憲

XIN Ben-zhu1, TONG Dan2*, LIU Yan-hong3, AI Si-la-yi-ding4, LIU Xue1, HUANG Ru-xian5

新生兒Galen靜脈畸形合并動(dòng)脈導(dǎo)管、卵圓孔未閉1例

辛本柱1，佟丹2*，劉彥紅3，艾達(dá)爾艾力·斯拉依丁4，劉學(xué)1，黃如憲5

XIN Ben-zhu1, TONG Dan2*, LIU Yan-hong3, AI Si-la-yi-ding4, LIU Xue1, HUANG Ru-xian5

作者單位：
1.中國人民解放軍65322部隊(duì)醫(yī)院放射科，公主嶺 136100
2.吉林大學(xué)第一醫(yī)院放射科，長春 130021
3.吉林大學(xué)第一醫(yī)院超聲室，長春 130021
4.吉林大學(xué)第一醫(yī)院病理科，長春 130021
5.公主嶺462醫(yī)院超聲科，公主嶺 136100
1Department of Radiology, 65322 Hospital of PLA, Gongzhuling 136100, China
2Department of Radiology, the First Hospital Affiliated to Jilin University, Changchun 130021, China
3Department of Ultrasound, the First Hospital Affiliated to Jilin University, Changchun 130021, China
4Department of Pathology, the First Hospital Affiliated to Jilin University, Changchun 130021, China
5Department of Ultrasound, 462 Hospital of Gongzhuling, Gongzhuling 136100, China

患者，女，雙卵雙胞胎之小，8 d，主因“呼吸困難2 d”入院。Apgar評(píng)分1 min7分，5 min8分。其母診為妊娠期糖尿病，孕期應(yīng)用胰島素治療。?？茩z查：呼吸急促，65次/min，心率145次/min，心音亢進(jìn)，心前區(qū)可聞及4/6級(jí)連續(xù)性機(jī)械樣雜音。實(shí)驗(yàn)室檢查：肌酸激酶650 U/L，肌酸激酶同工酶629.3 U/L；肌鈣蛋白0.156 ng/ml，肌紅蛋白49.1 ng/ml。腦彩：考慮大腦大靜脈瘤樣擴(kuò)張。心彩：動(dòng)脈導(dǎo)管未閉、卵圓孔未閉。磁共振成像(magnetic resonance imaging，MRI)及磁共振靜脈造影(magnetic resonance venography，MRV)：大腦大靜脈、直竇、竇匯、頸內(nèi)靜脈見明顯擴(kuò)張流空信號(hào)影，其中大腦大靜脈、直竇及竇匯交通形似啞鈴狀，大腦中、后動(dòng)脈走行區(qū)見多發(fā)迂曲血管團(tuán)異常顯影，匯入擴(kuò)張大腦大靜脈。本例患兒因心衰死亡。

臨床診斷為Galen靜脈畸形合并動(dòng)脈導(dǎo)管未閉、卵圓孔未閉。

討論Galen靜脈畸形也稱為大腦大靜脈動(dòng)脈瘤樣擴(kuò)張、大腦大靜脈瘤、大腦大靜脈瘺，是一種罕見的顱內(nèi)先天血管畸形，約占所有顱內(nèi)血管畸形疾病的1%。組織胚胎學(xué)基礎(chǔ)在于胎兒孕6～11 w形成前腦中央靜脈，出生后該靜脈退化并作為Galen靜脈存留[1]。發(fā)生原因包括：(1)胚胎發(fā)育異常，前腦中央靜脈不能正常退化閉塞，多合并直竇發(fā)育不全或缺如，嬰幼兒多見；(2)Galen靜脈血流動(dòng)力學(xué)發(fā)生異常改變，刺激血管內(nèi)皮生長因子發(fā)生作用繼發(fā)瘤樣擴(kuò)張，多見于兒童或成人，血液動(dòng)力學(xué)改變可耐受[2]。主要分型包括：(1) Yasargil等[3]依據(jù)瘺口位置分為4型，Ⅰ型動(dòng)靜脈瘺口位于靜脈壁上，主要供血?jiǎng)用}包括脈絡(luò)膜動(dòng)脈、小腦上動(dòng)脈及腦膜支等；Ⅱ型瘺口位于靜脈壁上，供血?jiǎng)用}為中腦動(dòng)脈及丘腦穿支動(dòng)脈；Ⅲ型為最常見類型，結(jié)合Ⅰ型和Ⅱ型特點(diǎn)；Ⅳ型包含中腦、丘腦及Ⅰ型并存的真性動(dòng)靜脈畸形。(2) Osborn根據(jù)是否存在正常的Galen靜脈分為Galen靜脈畸形及Galen靜脈動(dòng)脈瘤樣擴(kuò)張[4]，其中前者分為“脈絡(luò)膜型”和“管壁型”：A脈絡(luò)膜型瘺口位于大腦中間帆腔，包括脈絡(luò)膜動(dòng)脈、丘腦穿支動(dòng)脈及胼周動(dòng)脈多支供血?jiǎng)用}；B管壁型瘺口位于前腦中央靜脈壁上，包含高流量血液，形成似動(dòng)脈瘤樣巨型擴(kuò)張，供血?jiǎng)用}為脈絡(luò)膜后動(dòng)脈及四疊體動(dòng)脈。影像學(xué)檢查：數(shù)字減影血管造影(digital subtraction angiography，DSA)為診斷金標(biāo)準(zhǔn)，可以準(zhǔn)確判斷瘺口部位和類型、供血?jiǎng)用}來源，并為血管栓塞治療提供通路。MRI可觀察到擴(kuò)張Galen靜脈及供血?jiǎng)用}、引流靜脈流空血管影(圖1～2)，需注意當(dāng)高速血流通過較大瘤體時(shí)產(chǎn)生渦流從而在相位編碼方向產(chǎn)生明顯偽影(圖3)。MRV可以觀察到回流靜脈血管走行情況(圖4～5)。發(fā)育正常足月兒動(dòng)脈導(dǎo)管、卵圓孔于出生后48 h內(nèi)功能性關(guān)閉，本例出生后動(dòng)脈導(dǎo)管未閉、卵圓孔未閉(圖6～7)，形成左向右分流[5]，肺循環(huán)血液量增加，又匯合Galen靜脈畸形大量血液，心室舒張末期容量增加，導(dǎo)致前負(fù)荷過重，出生即出現(xiàn)充血性心力衰竭(圖8)。周立霞等[6]采用MRI序列對(duì)胎兒進(jìn)行產(chǎn)前篩查發(fā)現(xiàn)Galen靜脈畸形，有利于及早診斷治療。本例未進(jìn)行系統(tǒng)產(chǎn)前檢查。雙胎及患兒之母糖尿病病史與本病是否相關(guān)未見報(bào)道，有待進(jìn)一步研究。臨床表現(xiàn)嬰幼兒因血管動(dòng)靜脈短路致腦缺血并后期發(fā)育遲緩、智力低下，成人多合并梗阻性腦積水及破裂后蛛網(wǎng)膜下腔出血[7]。治療多采取血管栓塞及手術(shù)治療，理論上最佳治療時(shí)間應(yīng)為出生后推遲4～5個(gè)月后進(jìn)行[8]。Galen靜脈畸形作為一種少見高危先天顱內(nèi)血管畸形，開展MRI胎兒產(chǎn)前篩查有診斷價(jià)值，MRV對(duì)腦靜脈形態(tài)及走行觀察效果明確。通過提高對(duì)本病的認(rèn)識(shí)，有助于臨床早期制訂有效的治療方案。

圖1 T2WI橫軸位：大腦中、后動(dòng)脈走行區(qū)域多發(fā)擴(kuò)張迂曲流空血管影，雙側(cè)橫竇及竇匯明顯增粗 圖2 T2WI橫軸位：大腦大靜脈、直竇、竇匯明顯擴(kuò)張呈啞鈴狀 圖3 T1WI矢狀位：大腦大靜脈相位編碼方向見血管搏動(dòng)偽影 圖4 MRV：下矢狀竇未見顯示，上矢狀竇顯影纖細(xì)、走行迂曲，擴(kuò)張大腦大靜脈、直竇、竇匯強(qiáng)化血管影 圖5 容積再現(xiàn)(volume rendering，VR)：大腦大靜脈瘤樣擴(kuò)張，大腦中后動(dòng)脈走行區(qū)多發(fā)迂曲擴(kuò)張血管異常顯影，匯入大腦大靜脈，雙側(cè)頸內(nèi)靜脈擴(kuò)張明顯 圖6 胸骨旁大動(dòng)脈短軸位顯示肺動(dòng)脈分叉與降主動(dòng)脈之間可見動(dòng)脈導(dǎo)管內(nèi)左向右分流 圖7 劍突下兩腔圖顯示卵圓孔未閉 圖8 數(shù)字X線攝影(digital radiography，DR)：胸片見心影增大，心胸比近0.8，右心緣明顯圓隆，左心腰凸出，肺紋理增粗紊亂Fig. 1 T2WI axial: The tortuosity, dialation and abnormal hyperplasia of arteries are found rich at the area of middle cerebral artery and posterior cerebral artery. Bilateral transverse sinus and torcular herophili are widely dialated. Fig.2 T2WI axial: The dialated greater cerebral vein of Galen communicating with torcular herophili through stratight sinus appears sign of dumbbell. Fig.3 T1WI sagittal: The flow artifacts is appeared in phase encoding direction.Fig.4 MRV: Inferior sagittal sinus is not totally displayed and superior sagittal sinus is stenotic and tortuous. The vessels of greater cerebral vein of Galen,straight sinus and torcular herophili are enhanced. Fig.5 VR: The tortuous vessels at area of middle cerebral artery are communicating with dialated greater cerebral vein of Galen. Bilateral internal jugular vein are dialated. Fig.6 Parasternal: Aorta short axial, there is left to right shunt of ductus arteriosus between pulmonary artery and thoracic aorta. Fig.7 Subcostal two chambers patent foramen ovale can be seen. Fig.8 DR: Cardiac shadow enlarges to both sides and cardiothoracic ratio is about 0.8. Round edge of right heart can be seen clearly. Left pulmonary artery is widened, Both sides of lung fields are not clear.

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Galen’s vein malformation with patent ductus arteriosus and patent foramen ovale in newborn: A case report

7 Feb 2017, Accepted 19 Apr 2017

Newborn; Galen's vein malformation; Great cerebral vein; Ductus arteriosus, patent; Foramen ovale,patent; Magnetic resonance imaging

新生兒；Galen靜脈畸形；大腦大靜脈；動(dòng)脈導(dǎo)管，未閉；卵圓孔，未閉；磁共振成像

佟丹，E-mail：tongdan1968@126.com

*Correspondence to: Tong D, E-mail: tongdan1968@126.com

2017-02-07接受日期：2017-04-19

R445.2；R743.4

B

10.12015/issn.1674-8034.2017.06.013