LAPORAN2015

MEDICINA VOLUME 46 NOMOR 1 JANUARI KASUS

TYPE IC CHOLEDOCHAL CYST PRESENTING AN EXTRAHEPATAL CHOLESTASIS

IN A 3 YEAR OLD BOY

Muhammad Reza1, Nyoman Metriani Nesa1, I Gusti Ngurah Sanjaya Putra1,

I Putu Gede Karyana1, Made Darmajaya2
Departments of Child Health1 and Paediatric Surgery2, Udayana University
Medical School / Sanglah Hospital Denpasar Bali

ABSTRACT

Choledochalcystisararecongenitaldilatationofthebileducts,mostlydiagnosedinchildhood.When
appropriateresectionisnotperformed,cholangiocarcinomamayoccurinahighincidencewithinthe
seconddecadeoflife.Thisreportaimstopresentararecaseinexperienceofdiagnosisandmanagement
typeICcholedochalcystinchildren.Wepresentcaseofa3-year-oldboywhocamewithjaundiceand
itchyskin,abdominalpain,brownishurine,palescoloredofstool.Abdominalultrasonographyand
computedtomographyscanrevealedtypeICcholedochalcyst.Patientunderwentcompletecystremoval
surgeryandbilioentericanastomosisthroughRoux-en-yhepaticojejunostomy.Excisionbiopsyconfirmed
thediagnosisoftypeICcholedochalcyst.Postsurgicalfollowupshowngoodphysicalandlaboratory
condition and there was no recurrence of symptoms. Early surgical procedure through Roux-en-y
hepatojejunostomy, has been performed. Long term follow up also facilities good prognostic to the
patient.[MEDICINA 2015;46:56-60].

Keywords: choledochal cyst, children, surgery

KISTA KOLEDOKAL TIPE IC PENYEBAB KOLESTASIS EKSTRAHEPATAL

PADA ANAK LAKI-LAKI UMUR 3 TAHUN

Muhammad Reza1, Nyoman Metriani Nesa1, I Gusti Ngurah Sanjaya Putra1,

I Putu Gede Karyana1, Made Darmajaya2
Bagian/SMF Ilmu Kesehatan Anak1 dan Bedah Anak2, Fakultas Kedokteran
Universitas Udayana / Rumah Sakit Umum Pusat Sanglah Denpasar Bali

ABSTRAK

Kista koledokus adalah merupakan penyakit saluran empedu bawaan yang jarang dijumpai dan
banyakterdiagnosispadasaatusiaanak-anak.Tindakanberupareseksikistaadalahyangterpenting
dilakukan, jika tidak segera dilakukan maka dapat meningkatkan resiko terjadinya
cholangiocarcinomadalamusiadekadekeduapenderitadalamkehidupan.Tujuankasusinidilaporkan
untukmenggambarkanpengalamandalammendiagnosisdantatalaksanakistakoledokaltipeIC
yangjarangpadaanak-anak.Laporankasusinipadaanaklaki-lakiberumur3tahundengankeluhan
kulittampakkuningdangatal,nyeriperut,urinberwarnakecoklatan,tinjayangpucat.Ultrasonografi
dan CT scan abdomen memperlihatkan adanya kista koledokus. Tindakan bedah eksisi kista dan
anastomosisbilioenterikdenganmenggunakantehnikhepatojejunostomiRoux-en-y.Diagnosakista
koledokus tipe IC terkonfirmasi saat tindakan eksisi biopsi. Evaluasi setelah dilakukan tindakan
bedahmemperlihatkanhasilyangbagus,baikdaripemeriksaanfisikmaupunpemeriksaanpenunjang
danhilangnyakeluhanyangadasebelumnya.WalaupunprosedurtindakanhepatojejunustomiRoux-
en-ysecaradinitelahdilakukan,penderitamasihmembutuhkanevaluasidalamjangkawaktuyang
lama.[MEDICINA 2015;46:56-60].

Kata kunci: kista koledokus, anak-anak, bedah

INTRODUCTION 1000inAsianpopulation,ofwhich as4:1to3:1.Choledochalcystare

holedochalcystisarare
C congenitaldilatationof
the bile ducts. The estimated
incidence in Western countries
variesbetween1in100,000and1
in150,000.Theincidenceis1in
abouttwothirdcasesarereported
fromJapan.1Choledochalcystsare
usuallydiagnosedinchildhoodand
about25%aredetectedinadult
life.2Choledochalcystsalsohave
anunexplainedfemaleandmale
preponderance,commonlyreported
classifiedbasedonthelocationof
biliaryductdilationasdescribed
andmodifiedbyTodani,etal.3
Theclassictriadofsymptoms
includesabdominalpain,palpable
abdominalmass,andjaundice,are
seeninlessthan20%ofcases.An

56 JURNAL ILMIAH KEDOKTERAN

 Type IC Choledochal Cyst Presenting An Extrahepatal Cholestasis In A 3 Year Old Boy | Muhammad Reza., dkk.
85% of children have at least 2
featuresofclassictriad,whereas
only25%ofadultspresentwithat
least2featuresoftheclassictriad.
Neonateswhohasbeendetected
antenatally are usually
asymptomaticatbirthbutneedto
beintervenedearlybeforetheonset
ofcomplications.1,2Complications
of choledochal cyst include
pancreatitis, cholangitis,
secondary biliary cirrhosis,
spontaneousruptureofcyst,and
cholangiocarcinoma. Improved
imagingmodalitieshavefacilitated
the diagnosis at any time from
antenataltoadultlife.4,5
Surgical management has
evolved from cystenterostomy,
which was associated with
recurrence of symptoms and
malignancy to primary cyst
excision with Roux-en-Y
bilioentericdrainageeitheropenor
laparoscopic.Inafewtypesuchas
inIVAandVtypecholedochalcyst
patients may need hepatic
resection or liver transplan-
tation.4,5
Thepurposeofthisreportwas
to present our diagnosis and
managementexperienceinarare
caseoftypeICcholedochalcystin
children,attemptingtoelucidate
asurgicaltreatmentisthemain
theraphy.
CASE ILLUSTRATION
A3yearoldboywasbrought
by parents to Sanglah Hospital
OutpatientclinicsonApril4th2014
with chief complaint yellowish
color of skin which started five
days before admission to the
hospital.Itstartedfrompalmsand
extendedtowholebodyfollowedby
itchyskin,inthenexttwodays,
eyes of this patient were also
getting jaundice. Patient also
experienced abdominal pain
especiallyonupperpart.Although
thepainwasnotalwaysperceived
butitwassubsequentiallygetting
worse especially after meal. He
alsocomplainedbrownishurine
appearanceandpalecolorofstool
since three days before
hospitalization. No fever noted.
Therewerenohistoryofprevious
hepatobiliary disorders, routine
medicationorhospitalizationsince
birth. There was no familial
history of recurrent cholestasis
norjaundice.
The patient was the second
childinthefamily.Hewasborn
full term at hospital,
spontaneously, birth weight of
3.100gramsandhealthycondition
and breast fed for a year. The
immunizationrecordwascomplete
according to the government
recommendation.Hisfoodrecall
wasinaccordancetorecommended
dailyallowance(RDA).Thepatient
never had a blood transfusion
history.
Duringphysicalexamination,
thepatientwasalertandthevital
sign was normal. According to
WHO 2006 growth chart, the
patient was well nourished.
Developmental stages was also
normal.Therewasnodysmorphic
face,nopaleconjunctivas.Sclera
were yellowish. Ear, nose, and
throataswellasneckexamination
withinnormallimits.Theheart
and lung sounds were normal.
Bowel sound was normal. The
abdomenwastenderatrightupper
quadrant,nodistention,nosignof
generalizedperitonitis,nopalpable
liver,spleen,normass.Therewas
noshiftingdullness,norsignof
livercirrhosis.Extremitiesshown
noedema.Skinalloverthebody
looked yellowish including the
palmandthesole.Pruriticscar
Figure 1. Abdominal ultrasonography showed a cystic lesion in
extrahepatalbileduct.
was noted in some area but no
pustulenorbullaedocumented.
Thelaboratoryinvestigation
resultwasnormal.Completeblood
countwithhemoglobin(Hb)12.1
g/dL, hematocrite (Ht) 38.7%,
leukocytes 10.200 mm 3 and
thrombocytes321.000mm3.Liver
functiontestshowedincreaseof
totalbilirubinat4.49mg/dLwith
dominanceofdirectbilirubin4.39
mg/dL, serum aspartate
transaminase (AST) 158.4 U/L,
alanineaminotransferase(ALT)
198.3 U/L, alkaline phosphates
(ALP) 483 mg/dL and gamma
glutamyltranferase(GGT)715U/
L. Coagulation profile, shown
international normalized ratio
(INR) 1.11 with partial
thromboplastin time (PPT) was
13.0withcontrolat12.4,activated
partial thromboplastin time
(APTT)was36.6withcontrolat
31.9. Renal function test shown
BUN 9.0 mg/dL and creatinin
serum 0.44 mg/dL. Urinalysis
shownbrowncolor,urobilinogen8
mg/dL (+++), bilirubin 6 mg/dL
(+++).AntibodytestingofHBsAg
(Elisa)andantiHAVIgMresult
showednoevidenceofhepatitisA
orBinfection.Thebloodandurine
cultureshownnobacterialgrowth.
Stoolsampleshownpalecolor.
Abdominalultrasonography
revealed a cystic lesion in
extrahepatalbileduct.Thefinding
was most compatible with a
choledochalcyst(Figure 1).
JURNAL ILMIAH KEDOKTERAN 57
MEDICINA VOLUME 46 NOMOR 1 JANUARI 2015
Figure 2. Abdominal computed tomography scan showed type IC
choledochalcyst.
Abdominal computed tomo-
graphyscan(CTscan)revealeda
celledinhepaticcommunisductal
confirmed with the diagnosis
of type IC choledochal cyst
(Figure 2).
Based on clinical manifes-
tation,andimagingfindings,we
assessedthepatientwithtypeIC
choledochalcysts.Treatmentplan
were cyst removal surgery and
Roux-en-y hepaticojejunostomy.
Before underwent the surgery,
patient had none per os, hence
Figure 3. Cystexcisitionintraoperative.
58 JURNAL ILMIAH KEDOKTERAN
intravenous5%glucoseto0.25%
normalsalinefluidwasinstituted.
A300mgintravenousceftriaxone
twice daily with 120 mg metro-
nidazole three times daily were
given for preoperative injection
prophylaxis. A 2 mg K vitamin
injectionintra-muscularevery3
weeks,120mgoralursodioxycholic
acid(UDCA)threetimesdaily,4
goralcholestyramineoncedaily,
10.000 IU oral vitamin A once
daily,300IUoralvitaminEonce
daily,2.5goralvitaminDonce
Figure 4. Choledochalcyst.
daily were given to over come
cholestasis.
Patientunderwentdigestive
surgery, including laparotomy,
common bile duct (CBD) explo-
ration,biliaryreconstruction,and
cystexcisionwithbiopsy(Figure
3). After the surgery was
performed,thecholedochalcystas
abiopsysample(Figure 4)sent
to Anatomy Pathologic depart-
ment for examination. Clinical
improvementwasrecordedwith
jaundice appearance gradually
disappeared, while intensity of
abdominal pain decreased and
there was no more itchy skin.
Laboratoryresultaftersixdays
post-surgeryrevealedanormalized
level of serum transaminase,
normalized bilirubin, and no
bacteriagrowthforthebloodand
theurineculture.Nocomplication
wasnotedpostsurgicalprocedure
andpatientwasdischargedina
goodconditionafterseventhday.
Twoweekslater,thepatientcame
forfollowupingoodconditionof
physical and laboratory exami-
nation. Pathologic examination
from biopsy sample confirmed
diagnosisoftypeICcholedochal
cyst.
 Type IC Choledochal Cyst Presenting An Extrahepatal Cholestasis In A 3 Year Old Boy | Muhammad Reza., dkk.
DISCUSSION
Choledochalcystsarecystic
dilation of extrahepatic duct,
intrahepatic duct, or both that
mayresultinsignificantmorbidity
and mortality, unless it is
identified early and managed
appropriately.3Dilatedcystsand
distal stricture due to chronic
inflammationleadstobilestasis,
whichresultsinstoneformation
andinfectedbile,whichinturn
results in ascending cholangitis
and further obstruction causing
abdominal pain, fever, and
obstructive jaundice. Chronic
inflammation and formation of
albuminrichexudatesorhyper-
secretionofmucinfromdysplastic
epitheliumleadstoproteinplugs
in pancreatic duct, which along
with distal CBD stone causes
pancreatitis. About half of the
patientswithantenataldiagnosed
choledochalcystareasymptomatic
at birth. 4,5 Since most of the
patientshavestenosisdistaltothe
cyst, clinical symptoms such as
recurrentjaundice,liverdysfunc-
tion, ascending cholangitis,
pancreatitis and rupture of the
cystsareusuallyseen.6
Inourcase,patientcamewith
symptomandsignsofcholestasis.
Cholestasisincludesretentionof
conjugatedbilirubin,bilesaltsand
othercomponentsofthebile.Itis
a symptom of many diseases,
therefore,itisasignalthatdisease
exists.Themechanismsbywhich
diseasesproducecholestasiscanbe
classifiedaseitherintrahepatalor
extrahepatalcholestasis.1Thefirst
diagnosticconcernofcholestasis
disordersshouldbetodifferentiate
eithertheentityisofintrahepatal
orextrahepatal.Thisisimportant
as the management will be
significantly different between
medicalandsurgical.
Early identification of
extrahepatalcholestasisthatneed
promptinterventionwillimprove
outcome. However, lack of
distinctiveclinicalfeaturesoften
leading to difficulty in differen-
tiationbetweenintrahepataland
extrahepatal cholestasis.2 When
diagnosticprocedureincorporate
clinical,biochemical,radiological
and histological features or
sometimeserologictestorimaging
study then probable cause of
cholestasismaybeidentified.3In
ourcase,extrahepatalcholestasis
was identified based on clinical
manifestation and biochemical
test,whileabdominalultrasono-
graphyandCTscan,identifiedthe
causeoftheanatomicalproblem
causingextrahepatalcholestasis.
Thetypeofcholedochalcysts
symptomsdependslargelyonthe
ageatpresentation.Jaundiceis
reportedasthemainpresenting
symptom in infants, while
abdominalpainhasbeenreported
tobethemostfrequentsymptom
atpresentationinolderchild.7It
hasbeensuggestedthatagerelated
difference in presentation is
determinedbywhetherthereisa
reflux of activated pancreatic
juice.7Itwasfoundthatpatients
withcholedochalcystspresenting
withabdominalpainwereolder
thanoneyearandthatinthese
patientsthereisarelationwith
elevatedserumamylaseandsigns
of chronic inflammation in
histologysectionsoftheresected
cyst.8
Previous studies found
jaundiceasthemainpresenting
symptomofextrahepaticcystsand
cholangitis and gall stones of
intrahepaticcysts.1,8Thismaybe
explainedbythelocalizationofthe
lesion. Extrahepatic cysts may
givecompleteobstructionofthe
biliary tree leading to jaundice,
whereas intrahepatic cysts will
leadtopartialobstructiongiving
lateandlocalizedcomplications.
The classic triad of abdominal
pain, jaundice, and abdominal
Figure 5.Classificationofcholedochalcystsaccordingtomodified
Todani.1
masshasprovedtoberare.8,9These
wereconfirmedinourcase,with
initialpresentationofjaundiceand
abdominal pain but with no
abdominal mass suggesting a
choledocalcyst.
Choledochal cysts belong to
thefibropolycysticdisorders.Type
I cysts are the most frequent
choledochal cysts encountered
(Figure 5).Theintrahepaticpart
oftypeIVAandtypeVcholedochal
cystlocateddiffuselyorinapart
in the liver, type IVB, features
multipleextrahepaticdilatations.4,5
Type V (Carolis disease) and
probablytheintrahepaticpartof
typeIVAcystsarethoughttobe
ductal plate malformations
(DPM).4
The precise etiology of
extrahepaticcystisstillunclear.
TypeIcystareassociatedwithan
abnormal arrangement of the
pancreatobilliaryducts(APBD),
alsoknownascommonchannel.
Alongcommonchannelcanbethe
cause of a variety of pathologic
conditions,suchaspancreatitis,
stenosisofthepapillaofVater,and
choledochal cyst. 2,6 A common
channel may enhance reflux of
pancreatic juice into bile duct,
henceexposethecommonbileduct
walltopancreaticenzymes,aswell
asincreasepressureincholedochal
ductresultingincystformation.2,6
Thecompletetypeofcholedochal
cysts are as follows:1,2 type IA:
cystic dilatation of the
extrahepaticduct;typeIB:focal
segmental dilatation of the
extrahepatic duct; type IC:
JURNAL ILMIAH KEDOKTERAN 59
MEDICINA VOLUME 46 NOMOR 1 JANUARI 2015
fusiformdilatationoftheentire
extrahepatic bile duct and
commonbileduct;typeII:simple
diverticulumofthecommonbile
duct;typeIII:cyst/choledochocele
distalintramuraldilationofthe
common bile duct; type IVA:
combined intrahepatic and
extrahepaticbileductdilatation;
typeIVB:multipleextrahepatic
bileductdilations;typeV:multiple
intrathepaticbileductdilatation.
Inmostpatients,abdominal
ultrasonography is the primary
imagingtechniquefordetectionof
choledochal cysts and usually
sufficestoestablishthediagnosis.9
Sensitivityofultrasonographyis
about 7197%. 13 It is also the
preferred investigation in post
operation surveillance. After a
preliminary ultrasonography,
other supportive imaging
techniquesshouldbeorderedto
evaluate biliary system and
pancreatic duct. Computed
tomography scan is the most
accurate for choledochal cyst
diagnosisalsohelpsinplanning
surgical approaches. It is well
delineatestheintrahepaticbiliary
dilationintypeIVAandCarolis
disease, also the extent of
intrahepaticdilation.CTscanalso
identify cyst wall thickening
related to malignancy. 13 Plain
abdominalfilms,laparoscopy,and
gastroduodenoscopyarenotused
as standard diagnostic tools for
choledochal cyst, preoperative
biopsies were performed when
cancer was suspected, and the
lesionwasacholedochalcyst.1,9,10In
our case the diagnosis of
choledochal cyst type IC was
established with an abdominal
ultrasonographyandCTscan.
Treatmentofcholedochalcyst
priortotheonsetofsymptomsis
recommended, as the benefit of
reducing risk
cholangiocarcinoma out weighs
risk of surgery prior to the
development of symptoms. 11
Historically, management of
choledochal cyst consisted of
variousdrainageoperations,but
high complication rates and
retainedthreatofmalignancyhave
led to the use of excision or
transplantation as first line
therapy. The preferred surgical
operation is cyst removal and
60 JURNAL ILMIAH KEDOKTERAN
Roux-en-yhepaticojejunostomy.10,11
Ourcaseunderwentopensurgery
andhadarightextendedsubcostal
incision without an operative
cholangiography. The complete
excisionofthecystwithacreation
ofRoux-en-Yhepaticojejunostomy
wasdoneasitisthetreatmentof
choice. Long term outcome of
patientsafterunderwentcomplete
excision with Roux-en-Y
hepaticojejunostomy are alive,
withsymptomsfreesurvivalrate
andoverallsurvivalratewere89%
and 96%, respectively. If
choledochalcystsarenotresected,
ahighincidence(20%to30%)of
cholangiocarcinoma has been
reported,mainlyafterthesecond
decadeoflife,whichformedthe
basisofresectionasstateofthe
artsurgicaltreatment.Thispolicy
is further supported by a study
that found increasing rate of
premalignantchangesinresected
cystswithadvancingage.6
SUMMARY
Wereportedacholedochalcystin
a3yearoldboywhopresentedwith
thetwooutofthreeclassictriadof
abdominal pain, palpable
abdominal mass, and jaundice.
Ultrasonography and CT scan
confirmeddiagnosisandthetype
of choledocal cyst. Primary cyst
excision with Roux-en-Y bilio-
enteric drainage showed a good
result post surgically with
normalized clinical and
biochemical profile. Long term
followupstillneededtoensurefree
survivalstate.
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