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BiliaryObstruction
Updated:Nov27,2016
Author:JenniferLynnBonheur,MDChiefEditor:VinayKumarKapoor,MBBS,MS,FRCS,FAMS
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OVERVIEW

Background
Disordersofthebiliarytractaffectasignificantportionoftheworldwidepopulation,andthe
overwhelmingmajorityofcasesareattributabletocholelithiasis(gallstones).IntheUnitedStates,
20%ofpersonsolderthan65yearshavegallstonesand1millionnewlydiagnosedcasesof
gallstonesarereportedeachyear.
Tobetterunderstandthesedisorders,abriefdiscussionofthenormalstructureandfunctionofthe
biliarytreeisneeded.Bileistheexocrinesecretionoftheliverandisproducedcontinuouslyby
hepatocytes.Itcontainscholesterolandwasteproducts,suchasbilirubinandbilesalts,whichaidin
thedigestionoffats.Halfthebileproducedrunsdirectlyfromtheliverintotheduodenumviaasystem
ofducts,ultimatelydrainingintothecommonbileduct(CBD).Theremaining50%isstoredinthe
gallbladder.Inresponsetoameal,thisbileisreleasedfromthegallbladderviathecysticduct,which
joinsthehepaticductsfromthelivertoformtheCBD.TheCBDrunsinthehepatodudoenalligament
andthencoursesthroughtheheadofthepancreasforapproximately2cmitjoinstheterminalpartof
thepancreaticducttoformtheapullaofVaterbeforepassingthroughthepapillaofVaterintothe
duodenum.[1]

Pathophysiology
Biliaryobstructionreferstotheblockageofanyductthatcarriesbilefromthelivertothegallbladder
orfromthegallbladdertothesmallintestine.Thiscanoccuratvariouslevelswithinthebiliarysystem.
Themajorsignsandsymptomsofbiliaryobstructionresultdirectlyfromthefailureofbiletoreachits
properdestination.
Theclinicalsettingofcholestasisorfailureofbiliaryflowmaybeduetobiliaryobstructionby
mechanicalmeansorbymetabolicfactorsinthehepaticcells.Forthesakeofsimplicity,theprimary
focusofthisarticleismechanicalcausesofbiliaryobstruction,furtherseparatingtheminto
intrahepaticandextrahepaticcauses.Thediscussionofintracellular/metaboliccausesofcholestasis
isverycomplex,thepathogenesisofwhichisnotalwaysclearlydefined.Therefore,thesecausesare
mentionedbutarenotdiscussedindetail.
Intrahepaticcholestasisgenerallyoccursatthelevelofthehepatocyteorbiliarycanalicular
membrane.Causesincludehepatocellulardisease(eg,viralhepatitis,druginducedhepatitis),drug
inducedcholestasis,biliarycirrhosis,andalcoholicliverdisease.Inhepatocellulardisease,
interferenceinthe3majorstepsofbilirubinmetabolism,ie,uptake,conjugation,andexcretion,
usuallyoccurs.Excretionistheratelimitingstepandisusuallyimpairedtothegreatestextent.Asa
result,conjugatedbilirubinpredominatesintheserum.

Extrahepaticobstructiontotheflowofbilemayoccurwithintheductsorsecondarytoexternal
compression.Overall,gallstonesarethemostcommoncauseofbiliaryobstruction.Othercausesof
blockagewithintheductsincludemalignancy,infection,andbiliarycirrhosis.Externalcompressionof
theductsmayoccursecondarytoinflammation(eg,pancreatitis)andmalignancy.Regardlessofthe
cause,thephysicalobstructioncausesapredominantlyconjugatedhyperbilirubinemia.
Accumulationofbilirubininthebloodstreamandsubsequentdepositionintheskincausesjaundice
(icterus).Conjunctivalicterusisgenerallyamoresensitivesignofhyperbilirubinemiathangeneralized
jaundice.Totalserumbilirubinvaluesarenormally0.21.2mg/dL.Jaundicemaynotbeclinically
recognizableuntillevelsareatleast3mg/dL.[2]Urinebilirubinisnormallyabsent.Whenitispresent,
onlyconjugatedbilirubinispassedintotheurine.Thismaybeevidencedbydarkcoloredurineseen
inpatientswithobstructivejaundiceorjaundiceduetohepatocellularinjury.However,reagentstrips
areverysensitivetobilirubin,detectingaslittleas0.05mg/dL.Thus,urinebilirubinmaybefound
beforeserumbilirubinreacheslevelshighenoughtocauseclinicaljaundice.
Thelackofbilirubinintheintestinaltractisresponsibleforthepalestoolstypicallyassociatedwith
biliaryobstruction.Thecauseofitching(pruritus)associatedwithbiliaryobstructionisnotclear.Some
believeitmayberelatedtotheaccumulationofbileacidsintheskin.Otherssuggestitmayberelated
tothereleaseofendogenousopioids.

Etiology
Causesofbiliaryobstructioncanbeseparatedintointrahepaticandextrahepatic.

Intrahepaticcauses
Mechanicalorintrahepaticcausesaremostcommonlyhepatitisandcirrhosis.Drugsmayalsocause
directdamagetohepatocytesandmetabolicobstruction.Notethefollowing:
Hepatitisisinflammationofthelivercharacterizedbydiffuseorpatchynecrosis.Causesof
hepatitisincludeviruses,drugs,andalcohol.
Cirrhosisischaracterizedbygeneralizeddisorganizationofhepaticarchitecturewithnodule
formationandscarringintheparenchyma.Cirrhosisresultsfromchronic,notacute,
inflammationoftheliver.Althoughmanycausesexist,themajorityofcasesofcirrhosisinthe
UnitedStatesaresequelaeofalcoholichepatitisorchronichepatitisBandCinfections.PBCis
achronic,progressive,nonsuppurative,granulomatousdestructionoftheintrahepaticducts.
PBC,anautoimmunedestructionofsmallhepaticducts,ismorecommoninwomenthanin
men.
Drugs,suchasanabolicsteroidsandchlorpromazine,areknowntodirectlycausecholestasis
(bymechanismsnotentirelyunderstood).Thiazidediureticusemayslightlyincreasetheriskfor
developinggallstones,themostcommoncauseofbiliaryobstruction.Amoxicillin/clavulanicacid
(Augmentin)isoneofthemostfrequentcausesofacutecholestaticinjurythatcanmimicbiliary
obstruction.Otherdrugs,suchasacetaminophenorisoniazid,cancausehepatocellular
necrosis.Typically,druginducedjaundiceappearsearlywithassociatedpruritus,butthe
patient'swellbeingshowslittlealteration.Generally,symptomssubsidepromptlywhenthe
offendingdrugisremoved.

Extrahepaticcauses
Extrahepaticcausesmaybefurthersubdividedintothosethatareintraductalandthosethatare
extraductal.

Intraductalcausesincludeneoplasms,stonedisease,biliarystricture,parasites,primarysclerosing
cholangitis(PSC),AIDSrelatedcholangiopathy,andbiliarytuberculosis.
Extraductalobstructioncausedbyexternalcompressionofthebiliaryductsmaybesecondaryto
neoplasms,pancreatitis,orcysticductstoneswithsubsequentgallbladderdistension.
Neoplasmsarevarioustumorsthatmayleadtobiliaryobstruction.
Cholangiocarcinomas(raretumorsarisingfromthebiliaryepithelium),ampullarycarcinomas
(neoplasmsoftheampullaofVater),andgallbladdercarcinomas(tumorswithextensionintothe
CBD)causeobstructionwithintheducts.
Metastatictumors(usuallyfromthegastrointestinaltractorthebreast)andthesecondary
adenopathiesintheportahepatisthatmaybeassociatedwiththesetumorscancauseexternal
bileductcompression.
Ofpancreatictumors,60%occurintheheadofthepancreasandmanifestearlywithobstructive
jaundice.
Stonediseaseisthemostcommoncauseofobstructivejaundice.Gallstonesmaypassthroughthe
CBDandcauseobstructionandsymptomsofbiliarycolicandcholecystitis.Largerstonescan
becomelodgedintheCBDandcausecompleteobstruction,withincreasedintraductalpressure
throughoutthebiliarytree.Mirizzisyndromeisthepresenceofastoneimpactedinthecysticductor
thegallbladderneck,causinginflammationandexternalcompressionofthecommonhepaticductand
thusbiliaryobstruction.
Ofbiliarystrictures,95%areduetosurgicaltraumaand5%areduetoexternalinjurytotheabdomen
orpancreatitisorerosionoftheductbyagallstone.Stonediseaseisthemostcommoncauseof
biliarystricturesinpatientswhohavenotundergoneanoperation.Atearintheductcausesbile
leakageandpredisposesthepatienttoalocalizedinfection.Inturn,thisaccentuatesscarformation
andtheultimatedevelopmentofafibrousstricture.
Ofparasiticcauses,adultAscarislumbricoidescanmigratefromtheintestineintothebileducts,
therebyobstructingtheextrahepaticducts.Eggsofcertainliverflukes(eg,Clonorchissinensis,
Fasciolahepatica)canobstructthesmallerbileductswithintheliver,resultinginintraductal
cholestasis.ThisismorecommoninAsiancountries.[3]
PSCismostcommoninmenaged2040years,andthecauseisunknown.However,PSCis
commonlyassociatedwithinflammatoryboweldisease(IBD),mostcommonlyinpatientswith
pancolitis.IBD(thevastmajoritybeingulcerativecolitis)ispresentin6080%ofpatientswithPSC,
andPSCisfoundinapproximately3%ofpatientswithulcerativecolitis.PSCischaracterizedby
diffuseinflammationofthebiliarytract,causingfibrosisandstrictureofthebiliarysystem.Itgenerally
manifestsasaprogressiveobstructivejaundiceandismostreadilydiagnosedbasedonfindingsfrom
endoscopicretrogradecholangiopancreatography(ERCP).
AIDSrelatedcholangiopathymanifestsasabdominalpainandelevatedliverfunctiontestresults,
suggestingobstruction.TheetiologyofthisdisorderinpatientswhoareHIVpositiveisthoughttobe
infectious(cytomegalovirus,Cryptosporidiumspecies,andmicrosporidiahavebeenimplicated).
Directcholangiographyoftenrevealsabnormalfindingsintheintrahepaticandextrahepaticductsthat
maycloselyresemblePSC.
Biliarytuberculosisisextremelyrare.However,withtheresurgenceoftuberculosisandthe
emergenceofMycobacteriumtuberculosisstrainsthatareresistanttomanydrugs,biliarytuberculosis
maybeencounteredmorefrequentlyinthefuture.Histopathologicevidenceofcaseating
granulomatousinflammationwithbilecytologyrevealingMtuberculosisisconfirmatory.Polymerase
chainreactionisusefultoexpeditethediagnosisifbiliarytuberculosisisbeingconsidered.

Biliaryobstructionassociatedwithpancreatitisisobservedmostcommonlyinpatientswithdilated
pancreaticductsduetoeitherinflammationwithfibrosisofthepancreasorapseudocyst.
Notably,intravenousfeedingspredisposepatientstobilestasisandaclinicalpictureofobstructive
jaundice.Considerthisintheevaluationofbiliaryobstruction.
Sumpsyndromeisanuncommoncomplicationofasidetosidecholedochoduodenostomyinwhich
food,stones,orotherdebrisaccumulateintheCBDandtherebyobstructnormalbiliarydrainage.

Epidemiology
UnitedStatesdata
Theincidenceofbiliaryobstructionisapproximately5casesper1000people.

Racerelateddemographics
Theracialpredilectiondependsonthecauseofthebiliaryobstruction.Gallstonesarethemost
commoncauseofbiliaryobstruction.PersonsofHispanicoriginandNorthernEuropeanshavea
higherriskofgallstonescomparedtopeoplefromAsiaandAfrica.
NativeAmericans(particularlyPimaIndians)haveanincreasedincidenceofobesityanddiabetes
withintheirpopulationandareespeciallypronetodevelopinggallstones.Pimawomenhavealifetime
chanceofdevelopinggallstonesashighas80%.

Sexrelateddemographics
Thesexualpredilectionalsodependsonthespecificcauseofthebiliaryobstruction.Gallstone
diseaseisthemostcommoncauseofbiliaryobstruction.Womenaremuchmorelikelytodevelop
gallstonesthanmen.Bythesixthdecade,almost25%ofAmericanwomendevelopgallstones,with
asmanyas50%ofwomenaged75yearsdevelopinggallstones.Thisincreasedriskislikelycaused
bytheeffectofestrogenontheliver,causingittoremovemorecholesterolfromthebloodand
divertingitintothebile.
Approximately20%ofmenaged75yearshavegallstones,withmorecomplicateddiseasecourses
occurringinthosewhohavehadcholecystectomies.

Prognosis
Theprognosisaswellasthemortalityandmorbidityofbiliaryobstructiondependsonthecause.

Complications
Notethefollowing:
Thecomplicationsofcholestasisareproportionaltothedurationandintensityofthejaundice.
Highgradebiliaryobstructionbeginstocausecelldamageafterapproximately1monthand,if
unrelieved,mayleadtosecondarybiliarycirrhosis.
Acutecholangitisisanothercomplicationassociatedwithobstructionofthebiliarytractandis
themostcommoncomplicationofastricture,mostoftenattheleveloftheCBD.Bilenormallyis
sterile.Inthepresenceofobstructiontoflow,stasisfavorscolonizationandmultiplicationof
bacteriawithinthebile.Concomitantincreasedintraductalpressurecanleadtotherefluxof

biliarycontentsandbacteremia,whichcancausesepticshockanddeath.Forthisreason,
medicaltreatmentofthepatientwithcholangitisservesonlyasatemporizingmeasure.Long
termreliefofthebiliaryobstruction,whetheritbesurgical,percutaneous,orendoscopic,is
necessarytopreventanadverseoutcome.[4]
Patientswithbiliaryobstructionwhoundergobiliarytractsurgerymaydeveloppostoperative
acuteoliguricrenalfailure.Thecomplicationmaybeduetonephrotoxicbilesaltsandpigments,
endotoxins,orinflammatorymediators.Elderlypatientswhoaredeeplyjaundicedaremore
likelytodeveloppostoperativeoliguricrenalfailurethanpatientsofthesameagewithout
jaundice.
Biliarycolicthatrecursatanypointafteracholecystectomyshouldpromptevaluationfor
possiblecholedocholithiasis.
Failureofbilesaltstoreachtheintestineresultsinfatmalabsorptionwithsteatorrhea.In
addition,thefatsolublevitaminsA,D,E,andKarenotabsorbed,resultinginvitamin
deficiencies.DisorderedhemostasiswithanabnormallyprolongedPTmayfurthercomplicate
thecourseofthesepatients.Cholestyramineandcolestipol,usedtotreatpruritus,bindtobile
saltsandcanexacerbatethesevitamindeficiencies.
Persistentcholestasisfromanycausemaybeassociatedwithdepositsofcholesterolintheskin
(cutaneousxanthomatosis)and,occasionally,inbonesandperipheralnerves.
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