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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].
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].
85% of children have at least 2 Therewerenohistoryofprevious was noted in some area but no
featuresofclassictriad,whereas hepatobiliary disorders, routine pustulenorbullaedocumented.
only25%ofadultspresentwithat medicationorhospitalizationsince Thelaboratoryinvestigation
least2featuresoftheclassictriad. birth. There was no familial resultwasnormal.Completeblood
Neonateswhohasbeendetected history of recurrent cholestasis countwithhemoglobin(Hb)12.1
antenatally are usually norjaundice. g/dL, hematocrite (Ht) 38.7%,
asymptomaticatbirthbutneedto The patient was the second leukocytes 10.200 mm 3 and
beintervenedearlybeforetheonset childinthefamily.Hewasborn thrombocytes321.000mm3.Liver
ofcomplications.1,2Complications full term at hospital, functiontestshowedincreaseof
of choledochal cyst include spontaneously, birth weight of totalbilirubinat4.49mg/dLwith
pancreatitis, cholangitis, 3.100gramsandhealthycondition dominanceofdirectbilirubin4.39
secondary biliary cirrhosis, and breast fed for a year. The mg/dL, serum aspartate
spontaneousruptureofcyst,and immunizationrecordwascomplete transaminase (AST) 158.4 U/L,
cholangiocarcinoma. Improved according to the government alanineaminotransferase(ALT)
imagingmodalitieshavefacilitated recommendation.Hisfoodrecall 198.3 U/L, alkaline phosphates
the diagnosis at any time from wasinaccordancetorecommended (ALP) 483 mg/dL and gamma
antenataltoadultlife.4,5 dailyallowance(RDA).Thepatient glutamyltranferase(GGT)715U/
Surgical management has never had a blood transfusion L. Coagulation profile, shown
evolved from cystenterostomy, history. international normalized ratio
which was associated with Duringphysicalexamination, (INR) 1.11 with partial
recurrence of symptoms and thepatientwasalertandthevital thromboplastin time (PPT) was
malignancy to primary cyst sign was normal. According to 13.0withcontrolat12.4,activated
excision with Roux-en-Y WHO 2006 growth chart, the partial thromboplastin time
bilioentericdrainageeitheropenor patient was well nourished. (APTT)was36.6withcontrolat
laparoscopic.Inafewtypesuchas Developmental stages was also 31.9. Renal function test shown
inIVAandVtypecholedochalcyst normal.Therewasnodysmorphic BUN 9.0 mg/dL and creatinin
patients may need hepatic face,nopaleconjunctivas.Sclera serum 0.44 mg/dL. Urinalysis
resection or liver transplan- were yellowish. Ear, nose, and shownbrowncolor,urobilinogen8
tation.4,5 throataswellasneckexamination mg/dL (+++), bilirubin 6 mg/dL
Thepurposeofthisreportwas withinnormallimits.Theheart (+++).AntibodytestingofHBsAg
to present our diagnosis and and lung sounds were normal. (Elisa)andantiHAVIgMresult
managementexperienceinarare Bowel sound was normal. The showednoevidenceofhepatitisA
caseoftypeICcholedochalcystin abdomenwastenderatrightupper orBinfection.Thebloodandurine
children,attemptingtoelucidate quadrant,nodistention,nosignof cultureshownnobacterialgrowth.
asurgicaltreatmentisthemain generalizedperitonitis,nopalpable Stoolsampleshownpalecolor.
theraphy. liver,spleen,normass.Therewas Abdominalultrasonography
noshiftingdullness,norsignof revealed a cystic lesion in
CASE ILLUSTRATION livercirrhosis.Extremitiesshown extrahepatalbileduct.Thefinding
A3yearoldboywasbrought noedema.Skinalloverthebody was most compatible with a
by parents to Sanglah Hospital looked yellowish including the choledochalcyst(Figure 1).
OutpatientclinicsonApril4th2014 palmandthesole.Pruriticscar
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 Figure 1. Abdominal ultrasonography showed a cystic lesion in
hospitalization. No fever noted. extrahepatalbileduct.