腹膜后纖維化培訓(xùn)課件

腹膜后纖維化Introduction

腹膜后纖維化retroperitonealfibrosis（RPF）少見的纖維化反應(yīng)（膠原血管?。?905，由法國泌尿科醫(yī)生Albarran首次介紹腹膜后纖維化反應(yīng)繼發(fā)輸尿管狹窄1948，Ormond首次提出RPF的概念慢性主動脈周炎、輸尿管周炎、硬化性腹膜后肉芽腫因輸尿管梗阻是常見的后遺癥，排泄性尿路造影、逆行性腎盂造影可用于發(fā)現(xiàn)梗阻的部位及程度CT、MR成為診斷該病及評價繼發(fā)表現(xiàn)的主力18F-FDGPET核素顯像2腹膜后纖維化Introduction

惡性腹膜后纖維化占8%，預(yù)后差熟悉其潛在的臨床表現(xiàn)以及典型或不典型的影像學(xué)特征是關(guān)鍵3腹膜后纖維化Epidemiology發(fā)病率為1/200，000好發(fā)年齡：40-60；男女比例（2-3）：1絕大部分病例（>70%）為特發(fā)性腹膜后纖維化其余常與感染、惡性腫瘤以及藥物介導(dǎo)伴發(fā)罕有兒童及家族遺傳病例報道4腹膜后纖維化Epidemiology尿路梗阻性疾病

全身系統(tǒng)性疾病常與各種免疫紊亂伴發(fā)以及對免疫抑制劑有效

自身免疫病因假說腹膜后纖維化是對進展期動脈粥樣硬化的嚴(yán)重炎癥反應(yīng)假定的過敏原為粥樣斑塊產(chǎn)生的臘樣色素5腹膜后纖維化與腹膜后纖維化相關(guān)的免疫疾病原發(fā)性膽管硬化纖維化縱膈炎腎小球腎炎風(fēng)濕性關(guān)節(jié)炎系統(tǒng)性紅斑狼瘡結(jié)節(jié)性動脈周圍炎強直性脊柱炎橋本甲狀腺炎全垂體機能減退硬化性腸系膜炎6腹膜后纖維化Clinicalpresentation早期癥狀腹部或腰部不適下肢水腫或不適（淋巴管閉塞）晚期癥狀深靜脈血栓少尿、無尿惡心、嘔吐、尿毒癥表現(xiàn)高血壓腸系膜缺血腸梗阻7腹膜后纖維化惡性淋巴瘤（Hodgkin’s淋巴瘤）轉(zhuǎn)移（結(jié)腸、乳腺、肺、泌尿系統(tǒng)、甲狀腺原發(fā)瘤）腹膜后肉瘤類癌出血主動脈瘤動脈周圍血腫創(chuàng)傷或手術(shù)炎癥Crohn’s病放化療后感染結(jié)核、尿路感染腎臟創(chuàng)傷8腹膜后纖維化Pathology大體蒼白、血栓樣腫塊；邊界不清；累及鄰近器官，如輸尿管和下腔靜脈鏡下纖維增生伴多種炎細(xì)胞浸潤，如淋巴細(xì)胞、巨噬細(xì)胞及血管內(nèi)皮細(xì)胞9腹膜后纖維化ImagefeaturesConventionalradiographyExcretoryurographysonographyCTMRIScintigraphy10腹膜后纖維化Sonography低回聲或無回聲、邊界清楚、形態(tài)不規(guī)則腹膜后腫塊可伴腎積水、輸尿管積水尾側(cè)超出骶骨岬，且不呈分葉狀----良性敏感性低，25%原發(fā)性膽管硬化膽管擴張（膽總管狹窄）門脈高壓（門靜脈受壓）胰管局部或彌漫擴張（硬化性胰腺炎）11腹膜后纖維化Sonography60-year-oldmanwithbiopsy-provenidiopathicretroperitonealfibrosis.A,Transversesonogramatlevelofmidaortarevealspresenceofparaaorticandpreaortichypoechoicsofttissuemass(arrows).Rightureteralandpelvicalycealdilatationwerefoundtocoexist.B,CorrelatingCTimagealsoshowsobstructiveuropathy(arrowheads)resultingfromureteralinvolvementthatprecludedcontrastadministration.Notethatcalcifiedabdominalaortaisnotelevatedfromunderlyinglumbarspineandrelativelysmoothperipheralmarginsofabnormalsofttissue(arrows).

12腹膜后纖維化CT病變定位、范圍、鄰近器官及血管有利于顯示病因腹主動脈瘤胰腺炎、腸系膜腺病腹膜后腫塊、位于脊柱旁、邊界清楚、形態(tài)不規(guī)則、呈等密度13腹膜后纖維化CT腹主動脈或髂動脈周圍輸尿管后腹膜腹主動脈分叉處中心向前十二指腸胰腺脾臟縱隔骶骨頭尾側(cè)一般不會發(fā)生骨破壞，但惡性疾病可繼發(fā)一般良性病變中腹主動脈和下腔靜脈不會發(fā)生移位，但也有例外14腹膜后纖維化CT強化強化程度與纖維化進展相關(guān)顯著強化---急性期；低強化或無強化---進展期或慢性疾病Brun等發(fā)現(xiàn)約1/3經(jīng)手術(shù)病理證實的RPF患者無CT異常表現(xiàn)15腹膜后纖維化CT55-year-oldmanwithretroperitonealfibrosis.AandB,AxialoralandIVcontrast-enhancedCTimagesshowpresenceoflow-attenuationmassanteriorandlateraltoaortaandiliacvessels,withoutanteriordisplacementofeitheraortaorinferiorvenacava.Retroperitonealmassobliteratesfatplanebetweenvesselsandpsoasmuscle(arrows,A).Plaquebifurcatesandfollowscommoniliacarteries(arrowhead,B).16腹膜后纖維化CT55-year-oldmanwithinflammatoryabdominalaorticaneurysm.AandB,OralandIVcontrast-enhancedaxial(A)andcoronal(B)CTimagesshowill-definedmassofsoft-tissueattenuationsurroundingatheromatousaneurysm.Bilateralnephrostomytubeshavebeenplacedforobstructiveuropathy.17腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用比較困難惡性征象體積巨大，占位效應(yīng)，主動脈及下腔靜脈移位（原因可能是血管后方的淋巴結(jié)腫大）易形成結(jié)節(jié)，呈分葉狀良性征象“tethering”血栓密度，向周圍浸潤、蔓延但是敏感性和特異性都較低18腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用64-year-oldwomanwithabdominalpain.Contrast-enhancedabdominalCTscanrevealspresenceofretroperitonealmass.Aortaisminimallyelevatedfromunderlyingspine,raisingconcernforunderlyingneoplasia.CT-guidedbiopsyandsubsequentcystoscopyconfirmedpresenceofmetastatictransitionalcellcarcinomaofurinarybladder.20腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用35-year-oldmanwithHIVwhopresentedwithabdominalpainandfever.Sputumcultureandchestradiographysuggestedtuberculosis.IVcontrast-enhancedCTscanofabdomenshowsnonlobulatedretroperitonealparaaorticmassofsofttissueattenuation.Biopsyconfirmedbenigninfectivelymphadenopathy.21腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用35-year-oldwomanwithendometrialadenocarcinoma.Confluentlow-attenuationretroperitonealmetastaticdeposits(arrow)haveappearancesimilartothatofretroperitonealfibrosis.CTscanshowsthissofttissueiscenteredonlowerinfrarenalaorta,hasrelativelysmoothmargins,anddoesnotelevateaortafromspine—featuresthatmayallowdifferentiationofmalignantfrombenignretroperitonealfibrosis.Noteassociatedlefthydronephrosis(arrowhead).22腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用47year-oldmanshowsretroperitonealmassthatfailstoelevateaortafromspine.However,thismasshassuspiciouslobulatedanteriormargin.Uppergastrointestinalendoscopy(notshown)revealedpresenceofgastricadenocarcinoma,withsubsequentbiopsy-provenretroperitonealmetastasis.23腹膜后纖維化CT在鑒別良惡性病變中的應(yīng)用50-year-oldwomanwithnewdiagnosisofpancreaticadenocarcinoma.CTscanshowsparaaorticretroperitonealsoft-tissuemassbutnoelevationofaortafromspine,whichsuggestsbenigncause.However,thismasshaslobulatedanteriormargin,whichraisesconcernformetastaticdisease.Subsequentbiopsyconfirmedmalignantnatureofthisparaaorticsofttissue.24腹膜后纖維化MRI腹膜后組織結(jié)構(gòu)顯影、病因以及并發(fā)癥的顯示信號特征與其他纖維變性相似彌漫T1WI低信號增強可反映水腫程度慢性、非活動期的纖維組織T1、T2WI均低信號可用于評價患者對治療的反應(yīng)----水腫減輕，提示療效好強化程度減輕也提示好轉(zhuǎn)25腹膜后纖維化26腹膜后纖維化MRI50-year-oldmanwithretroperitonealfibrosis.AandB,Inflammatoryabdominalaneurysmandinflammatoryretroperitonealfibrosisareseenonfat-saturatedaxialT1gradient-recalledechoimage(A).MRimagesshownear-circumferentialparaaorticsoft-tissuemasswithoutelevationofaortafromunderlyingspine.Righthydronephrosisandrightrenalatrophyhaveresulted.Contrast-enhancedimage(B)showsintenseenhancementofretroperitonealfibrosis,consistentwithactiveinflammation.27腹膜后纖維化MRI60-year-oldmanwithidiopathicretroperitonealfibrosis.Arrowsindicateretroperito