Retroperitoneal Fibrosis - Urology · PDF fileRetroperitoneal Fibrosis Grand Rounds VGH ... • Review of RP Fibrosis – Def’n, Etio, Epid, Pathology, Pathophysiology, Staging

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Retroperitoneal FibrosisRetroperitoneal Fibrosis

Grand Rounds VGH Grand Rounds VGH Sept 14, 2005Sept 14, 2005Omar NazifOmar Nazif

OutlineOutline

•• Case PresentationCase Presentation–– Hx, Px, ImagingHx, Px, Imaging

•• Review of RP FibrosisReview of RP Fibrosis–– DefDef’’n, Etio, Epid, Pathology, Pathophysiology, n, Etio, Epid, Pathology, Pathophysiology,

Staging, Imaging, Dx, Medical and Surg MgtStaging, Imaging, Dx, Medical and Surg Mgt•• Case PresentationCase Presentation

–– Mgt, follow up imagesMgt, follow up images•• SummarySummary

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Case PresentationCase Presentation

•• IDID–– 63 yo F, HTN, dyslipidemia, no previous 63 yo F, HTN, dyslipidemia, no previous

surgery, otherwise healthysurgery, otherwise healthy

•• CCCC–– Non specific back painNon specific back pain


•• HPIHPI–– Non specific flank pain L > R for 6 mosNon specific flank pain L > R for 6 mos–– Gradual onset of SxGradual onset of Sx–– AnorexiaAnorexia–– No LUTSNo LUTS–– No hematuriaNo hematuria–– No migrainesNo migraines

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•• Physical examPhysical exam–– Mild L CVA tendernessMild L CVA tenderness–– Abd exam negativeAbd exam negative–– Pelvic exam negativePelvic exam negative

•• MedsMeds–– AltaceAltace–– LipitorLipitor

•• AllergiesAllergies–– nonenone


•• TestsTests–– Cr 115Cr 115–– Lytes NLytes N–– CBC NCBC N–– Incr ESRIncr ESR–– U/A & U C&S U/A & U C&S ––veve

•• ImagingImaging

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Definition Retroperitoneal Definition Retroperitoneal Fibrosis (RPF)Fibrosis (RPF)

•• Fibrotic process involving RP encircling and Fibrotic process involving RP encircling and strangulating the organs & vessels in the RP strangulating the organs & vessels in the RP spacespace

•• Dense woody hard mass in center of RP typ L45 Dense woody hard mass in center of RP typ L45 vertebravertebra

•• Envelopes IVC and AOEnvelopes IVC and AO–– Extends from AO bifurcation to renal pedicleExtends from AO bifurcation to renal pedicle–– Extends laterally to outer edge of psoas enveloping Extends laterally to outer edge of psoas enveloping

the uretersthe ureters

RP Fibrosis AKARP Fibrosis AKA

•• OrmondOrmond’’s Ds D•• Periureteritis fibrosaPeriureteritis fibrosa•• Periureteritis plasticaPeriureteritis plastica•• Chronic periureteritisChronic periureteritis•• Sclerosing RP granulomaSclerosing RP granuloma•• Fibrous retroperitonitisFibrous retroperitonitis

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EpidEpid

•• PtPt’’s 40 s 40 –– 60 yo 60 yo •• Children rareChildren rare•• M:F 2.5:1M:F 2.5:1

EtioEtio

•• Idiopathic RP fibrosis 30%Idiopathic RP fibrosis 30%•• Secondary 70%Secondary 70%

Secondary Causes of RPFSecondary Causes of RPF

•• ChemicalsChemicals–– AsbestosAsbestos–– Talcum powderTalcum powder–– AviteneAvitene–– Methyl methacrylateMethyl methacrylate

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•• MalignancyMalignancy–– Primary RP tumorsPrimary RP tumors

•• LymphomaLymphoma, sarcoma, multiple myeloma, sarcoma, multiple myeloma

–– Metastatic Periureteral diseaseMetastatic Periureteral disease•• Rectal, colon, breast, pancreatic, prostatic, gastric Rectal, colon, breast, pancreatic, prostatic, gastric

–– Carcinoid RP tumorsCarcinoid RP tumors


•• RP Inflammatory ProcessesRP Inflammatory Processes–– Collagen vascular DCollagen vascular D

•• Mesenteric panniculitisMesenteric panniculitis•• AO or iliac aneurysmAO or iliac aneurysm

–– CT DCT D•• SLE, RASLE, RA

–– Pancreatitis, enteritis, diverticulitisPancreatitis, enteritis, diverticulitis–– SarcoidosisSarcoidosis–– endometriosisendometriosis

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•• MedicationsMedications–– Beta blockersBeta blockers–– MethyldopaMethyldopa–– HydralazineHydralazine–– Ergotamine alkaloids Ergotamine alkaloids LLSD, MethysergideSD, Methysergide

–– Dopaminergic agonists Dopaminergic agonists pergolide, pramipexolpergolide, pramipexol

–– HaloperidolHaloperidol–– AmphetaminesAmphetamines–– PhenacitinPhenacitin


•• RP InjuryRP Injury–– TraumaTrauma

•• HemorrhageHemorrhage•• Urinary extravasationUrinary extravasation•• Rectal perforatRectal perforat’’n after Ba eneman after Ba enema

–– XRTXRT–– Previous operationPrevious operation

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•• InfectionInfection–– Biliary tract infectionsBiliary tract infections–– GonorrheaGonorrhea–– Ruptured liver echinococcal cystRuptured liver echinococcal cyst–– TBTB–– Chronic UTIChronic UTI–– SyphilisSyphilis

Primary RPFPrimary RPF

•• Typ occurs as an isolated entityTyp occurs as an isolated entity•• 15% of idiopathic cases are assoc w 15% of idiopathic cases are assoc w

fibrotic processes elsewhere in bodyfibrotic processes elsewhere in body

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Multifocal FibrosclerosisMultifocal Fibrosclerosis

•• Rare syndromes involving multiple organ Rare syndromes involving multiple organ systemssystems–– Sclerosing mediastinitisSclerosing mediastinitis–– Sclerosing cholangitisSclerosing cholangitis–– Orbital pseudotumorOrbital pseudotumor–– RiedelRiedel’’s thyroiditiss thyroiditis

PathogenesisPathogenesis

•• Early in D Early in D inflammationinflammation•• Late in D Late in D fibrosisfibrosis

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Pathology iRPFPathology iRPF

•• GrossGross–– Flat, grayFlat, gray--white, hard fibrous plaquewhite, hard fibrous plaque

•• MicroscopicMicroscopic–– Densely fibrotic collagenDensely fibrotic collagen–– Non specific areas of inflammationNon specific areas of inflammation–– Macrophages, lymphocytes, plasma cellsMacrophages, lymphocytes, plasma cells–– Occasional eosinophilsOccasional eosinophils

PathologyPathology

•• Often lateral margins of mass demonstrate Often lateral margins of mass demonstrate more inflammatory process than central more inflammatory process than central portionportion

•• Depending on stage of D, pathology can Depending on stage of D, pathology can vary from an active inflammatory infiltrate vary from an active inflammatory infiltrate or can be bland with hypocellular collagen or can be bland with hypocellular collagen and minimal cellularityand minimal cellularity

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SSxSSx

•• Presents insidiouslyPresents insidiously•• Vague abd and flank painVague abd and flank pain•• Nonspecific systemic complaintsNonspecific systemic complaints

–– AnorexiaAnorexia–– Wt lossWt loss–– Moderate pyrexiaModerate pyrexia–– N & VN & V

•• Most Sx occur from envelopment of uretersMost Sx occur from envelopment of ureters

SSxSSx

•• Dull non colicky pain in girdle distributionDull non colicky pain in girdle distribution•• Discomfort unaffected byDiscomfort unaffected by

–– PositionPosition–– ActivityActivity–– Defecation or micturitionDefecation or micturition

•• Discomfort worsens with timeDiscomfort worsens with time•• L/E edema L/E edema •• DVTDVT•• HTNHTN

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InvInv’’nn

•• USUS–– HydronephrosisHydronephrosis–– Hypoechoic periaortic massHypoechoic periaortic mass

IVPIVP

•• Classic TriadClassic Triad–– Proximal hydroureteronephrosisProximal hydroureteronephrosis–– Medial deviation of uretersMedial deviation of ureters–– Extrinsic compression of uretersExtrinsic compression of ureters

•• Bilateral hydronephrosisBilateral hydronephrosis•• Encasement of ureters prevents dilation of Encasement of ureters prevents dilation of

middle and distal ureteral segmentsmiddle and distal ureteral segments

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CTCT

•• Exam of choice to visualize Exam of choice to visualize –– extent of fibrosisextent of fibrosis–– Presence of LADPresence of LAD–– Primary malignancyPrimary malignancy

•• Fibrotic plaque exhibits similar attenuation Fibrotic plaque exhibits similar attenuation to muscleto muscle

•• Contrast enhancement of massContrast enhancement of mass

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MRMR

•• Findings are comparable to CTFindings are comparable to CT•• Contrast study with impaired renal fnContrast study with impaired renal fn•• Fibrotic plaques present w a characteristic Fibrotic plaques present w a characteristic

T1 and T2 weighted imageT1 and T2 weighted image

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PyelographyPyelography

•• Not primary studyNot primary study–– antegrade or retrogradeantegrade or retrograde

•• Pyelography may be performed during Tx Pyelography may be performed during Tx –– Perc NT placedPerc NT placed–– Ureteral stentsUreteral stents

•• Studies can be useful to determine extent Studies can be useful to determine extent and length of ureteral involvementand length of ureteral involvement

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Nuclear RenographyNuclear Renography

•• May assist in determining cause of renal May assist in determining cause of renal insufficiencyinsufficiency

•• Will provide differential renal fn in ptWill provide differential renal fn in pt’’s w s w non functioning kidneynon functioning kidney

BiopsyBiopsy

•• Once Dx is suggested by imaging, Bx is reqOnce Dx is suggested by imaging, Bx is req’’d to d to confirm Dxconfirm Dx–– Exclude maligExclude malig

•• CT guided needle BxCT guided needle Bx–– Minimally invasiveMinimally invasive

•• FNAFNA–– Appearance is often non specificAppearance is often non specific

•• Open vs Laparoscopic surgeryOpen vs Laparoscopic surgery–– For larger Bx specimensFor larger Bx specimens

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Laboratory InvLaboratory Inv’’nn

•• Incr ESRIncr ESR•• Incr gamma globulin levelIncr gamma globulin level•• Mod leukocytosisMod leukocytosis•• Renal insufficiencyRenal insufficiency•• AnemiaAnemia•• Lyte abNlitiesLyte abNlities•• Consider tumor markers if indicatedConsider tumor markers if indicated

Acute MgtAcute Mgt

•• Prompt DxPrompt Dx•• Preservation & correction of renal fnPreservation & correction of renal fn

–– Perc NTPerc NT–– JJ ureteral stentsJJ ureteral stents

•• Post obstructive diuresisPost obstructive diuresis•• Assess ptAssess pt’’s for DVT and L/E edemas for DVT and L/E edema•• Stop offending RxStop offending Rx

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Mgt Mgt -- MedicalMedical

•• CorticosteroidsCorticosteroids•• ImmunosuppressantsImmunosuppressants•• Combination TxCombination Tx

CorticosteroidsCorticosteroids

•• Primary Tx for RPFPrimary Tx for RPF•• Historically used ptHistorically used pt’’s unfit for surg or w s unfit for surg or w

extensive involvement of main vesselsextensive involvement of main vessels•• Administered postAdministered post--op w good long term op w good long term

successsuccess

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•• Evidence for EfficacyEvidence for Efficacy–– KardarKardar study is largest clinical study, n=11study is largest clinical study, n=11–– Numerous case reports confirming efficacyNumerous case reports confirming efficacy

•• Success determined by regression of mass Success determined by regression of mass & resolut& resolut’’n of ureteral obstn of ureteral obst’’nn

•• Complete disappearance of mass @ 6Complete disappearance of mass @ 6--20 20 mosmos

Kardar et al. Steroid Tx for RPF Dose and Duration of Tx. J Urol 2002


•• Patient SelectionPatient Selection–– More likely to benefit are ptMore likely to benefit are pt’’s w evidence of s w evidence of

inflammatinflammat’’nn•• LeukocytosisLeukocytosis•• +ve ANA titer+ve ANA titer

•• In early stage when fibrotic tissue is rich In early stage when fibrotic tissue is rich in inflammat cells in inflammat cells better steroid better steroid responseresponse

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•• No agreement on duratNo agreement on durat’’n of Txn of Tx•• Prednisolone 20Prednisolone 20--60 mg po eod for 2 mos60 mg po eod for 2 mos

–– taper to 5 mg po od for w 2 yrs then stoptaper to 5 mg po od for w 2 yrs then stop

•• Administer H2 blockers, Ca Administer H2 blockers, Ca supplementatsupplementat’’n to minimize side effectsn to minimize side effects

Other AgentsOther Agents

•• Use of several immunosuppressants alone Use of several immunosuppressants alone or in combination w glucocorticoids has or in combination w glucocorticoids has been reportedbeen reported

•• AzathioprineAzathioprine•• Oral cyclophosphamideOral cyclophosphamide•• Mycophenolate mofetilMycophenolate mofetil

Marcolongo R et al. Immunosuppressive Tx for iRPF. Am J Med 2004Grotz et al. Tx of RPF by mycophenolate and steroids. Lancet 1998

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SeRMsSeRMs

•• TamoxifenTamoxifen–– Effective in regression of desmoid tumorsEffective in regression of desmoid tumors–– Both desmoid tumors and RPF are charact by Both desmoid tumors and RPF are charact by

locally invasive fibrous tissuelocally invasive fibrous tissue–– MOA unknownMOA unknown

•• Tamoxifen is an alternative if corticoid Tx Tamoxifen is an alternative if corticoid Tx failsfails

Clark et al. Response of RPF to tamoxifen. Surgery 1991Ozener et al. Effects of tamoxifen in RPF. Nephrol Dial Transplant 1997

Bourouma et al. Tx of iRPF with Tamoxifen. Nephrol Dial Transplant 1997Tziomalos et al. Tx of iRPF w combined steroids & tamoxifen. Clin Nephrol 2004

Surgical MgtSurgical Mgt

•• Traditional approach to RPFTraditional approach to RPF•• Presently, surgical exploration w Presently, surgical exploration w

ureterolysis after failure of medical Rxureterolysis after failure of medical Rx•• Excludes RP malignancyExcludes RP malignancy•• Principle of surgeryPrinciple of surgery

–– Ureterolysis w manipulation to prevent Ureterolysis w manipulation to prevent recurrent obstrecurrent obst’’nn

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•• ApproachApproach–– Midline incision xyphoid to pubic symphysisMidline incision xyphoid to pubic symphysis–– White line of ToldtWhite line of Toldt–– Reflect L & R colon mediallyReflect L & R colon medially–– Deep Bx of mass for frozen & permanent Deep Bx of mass for frozen & permanent –– Commence dissection @ non dilated ureter to avoid Commence dissection @ non dilated ureter to avoid

injury to thin dilated proximal segmentinjury to thin dilated proximal segment

•• Consider Tx of both ureters even if only 1 ureter Consider Tx of both ureters even if only 1 ureter is involvedis involved


•• Reposition ureters & protect them from further Reposition ureters & protect them from further entrapmententrapment–– Retract ureters laterallyRetract ureters laterally

•• Secure peritoneum medially to psoas to maintain ureters in Secure peritoneum medially to psoas to maintain ureters in this positionthis position

–– Displace ureters into peritoneal cavity with closure of Displace ureters into peritoneal cavity with closure of peritoneum behind themperitoneum behind them

–– Omental wrapOmental wrap–– Ureteral wrap in polytetrafluoroethylene vascular graftUreteral wrap in polytetrafluoroethylene vascular graft

Loh A et al. iRPF Tx w PTFE vascular graft. Br J Urol 1991

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•• Remove plaque around renal arteries and Remove plaque around renal arteries and iliac vesselsiliac vessels–– restore renal arterial Prestore renal arterial P–– prevent L/E claudicationprevent L/E claudication

Post Op CarePost Op Care

•• Remove ureteral stents 6Remove ureteral stents 6--8 wks after 8 wks after surgery after obstsurgery after obst’’n has resolvedn has resolved

•• Consider post op steroidsConsider post op steroids•• Recurrence has been seen 9 yrs post opRecurrence has been seen 9 yrs post op

–– Long term f/u reqLong term f/u req’’dd

Cerfolio RJ et al. iRPF: is there a role for post op steroids? Curr Surg 1990

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LaparoscopyLaparoscopy

•• Lap ureterolysis has been performedLap ureterolysis has been performed–– Kavoussi et al 1992Kavoussi et al 1992

•• 33--4 trocars are placed along the midline4 trocars are placed along the midline–– UmbilicusUmbilicus–– BetwBetw’’n umbilicus and pubisn umbilicus and pubis–– BetwBetw’’n umbilicus and xyphoid processn umbilicus and xyphoid process

Fugita, Kavoussi et al. Lap Tx of RPF. J Endorul 2002Kavoussi et al. Lap ureterolysis J Urol 1992

LaparoscopyLaparoscopy

•• Difficult procedureDifficult procedure•• Approach has been proven less morbid w Approach has been proven less morbid w

all advantages of laparoscopyall advantages of laparoscopy•• Open conversion ~15%Open conversion ~15%•• 90% long term success rate90% long term success rate

Elashry et al. Lap vs Open Ureterolysis for RPF. J Urol 1996

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Technical ConsiderationsTechnical Considerations

•• if ureterolysis is impossible d/t ++ fibrosis and if ureterolysis is impossible d/t ++ fibrosis and ureteral strictureureteral stricture–– AutotransplantationAutotransplantation–– Ileal substitutionIleal substitution–– Appendix substitutionAppendix substitution

•• If lower ureter is involvedIf lower ureter is involved–– Boari flapBoari flap

•• If poor kidney fn and N contralateral kidney If poor kidney fn and N contralateral kidney consider Nxconsider Nx

Surgical ComplicationsSurgical Complications

•• Ureteral injury m/cUreteral injury m/c–– Urine leakUrine leak

•• SBOSBO

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AAA ConsiderationsAAA Considerations

•• Aneurysmectomy and ureterolysisAneurysmectomy and ureterolysis–– Excellent resultsExcellent results–– ImprovImprov’’t of renal fn > 75% ptt of renal fn > 75% pt’’ss

•• Endovascular graft repairEndovascular graft repair–– Resolution of obst'n of both uretersResolution of obst'n of both ureters


•• The pt is started on glucocorticoidsThe pt is started on glucocorticoids•• Her back pain and symptoms begin to Her back pain and symptoms begin to

improveimprove

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SummarySummary

•• RPFRPF–– Idiopathic 30%Idiopathic 30%–– Secondary causes 70%Secondary causes 70%

•• Identify and correctIdentify and correct

•• Non specific SSxNon specific SSx•• CT and MR gold std imagingCT and MR gold std imaging•• Bx mass to r/o malignancyBx mass to r/o malignancy•• Medical Tx w steroids, immunosuppressants, Medical Tx w steroids, immunosuppressants,

TamoxifenTamoxifen•• Surgical Tx for failed medical TxSurgical Tx for failed medical Tx

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