Kasai Procedure in The Management of Biliary Atresia

Kasai Procedure in the Management of Biliary Atresia
Kasai Procedure in the Management of Biliary Atresia

Mo Tualeka, Muqodriyanto, Ahmad Afandi, Erwin Haryono
Translator: Adrian Salim, Andrio Wishnu Prabowo, Arnetta Naomi L. Lalisang, Julistian, Muliyadi, Sony Sanjaya,
Stefanny, Zamzania Anggia Shalih.
General Surgery, Department of Surgery, Faculty of Medicine University of Indonesia/ Ciptomangunkusumo
Hospital, Jakarta, Indonesia
Case Illustration
A male baby, aged 2months presented with the chief complaints of jaundice in the whole body
since the age of one month, bloated abdomen, and sometimes accompanied by vomiting milk,
pale feces, and tea-colored urine.
The physical examination showed that the baby was awake and alert, although with decreased
activities. His vital signs were still within normal limits. The eye sclera were jaundiced, the skin
was jaundiced extending to the legs. The abdomen was distended, with enlarged veins; the liver
was palpable 4 cm below the costal arch with rubbery consistency.
The laboratory examination revealed: total bilirubin level of 14.34 mg/dL, direct bilirubin of 9.66
mg/dL, Indirect bilirubin of 4.68 mg/dL, Leukocyte count of 14,400/mm 2, G6PD of 162 mg/dL,
SGOT of 96 mgl, SGPT 14 mg/dL, Hb 10 mg/dL, and 12 mg/dL after correction.
Abdominal US: The images of biliary atresia, hepatomegaly, and ascites. Signs of portal
hypertension were not observed
Hepatic biopsy revealed hard fibrotic cholestasis with extrahepatic biliary atresia.
The patient was diagnosed with Biliary Atresia and was admitted for surgery preparation.
Operation Report:
Laparotomy with supraumbilical transversal incision was performed, and the peritoneum was
exposed. The liver was observed to be hard with the image of cholestasis.
The gallblader was very atretic. Holangiography was performed, there were no biliary ducts.
There was no passage of contrast in the liver and duodenum.
It was decided to perform Kasai Procedure: The liver was liberated by cutting the ligaments and
hepatic portal was dissected, bypass portojejunostomy was performed, in which the jejunum was
cut 20 cm from the Treitz ligament, the distal jejunum was then anastomosed with the hepatic
portal, then roux-enzyme Y jejuno-jejunostomy anastomosis was made 40 cm away from the
hepatic portal. The abdominal cavity was washed until it was clean, bleeding was controlled, and
abdominal cavity was closed layer-by-later. The surgery lasted for 5 hours and 30 minutes.
Post-operation
The patient was admitted to the ICU for stabilization; on day 5 of hospitalization, the patient was
moved to the patient ward. He was hospitalized for 17 days, during which he underwent
hipoalbuminemia, anemia, melena and diarrhea. Corrections of these conditions have been
performed. The patient was then discharged in good condition, without fever and vomiting,
adequately breastfed, normal defecation and urination. However the patient was still jaundiced,
with the total bilirubin level of 12.51 mg/dL, direct bilirubin level 10.07 mg/dL, indirect bilirubin
2.44 mg/dL. His treatment was continued in Ciptomangunkusumo Hospital Outpatient Clinic.
Discussion
Due to the impossibility to differentiate various etiologies of neonatal cholestasis, the evaluation
should determine the anatomical causes or obstruction in jaundice, including biliary
atresia, biliary duct cyst, or spontaneous perforation of the biliary tract. There are numerous
non-surgical causes of neonatal jaundice, including cholangitis, metabolic, genetic, and toxic
causes.
There are three types of Biliary Atresia:
1. Type I : atresia of the common bile duct

2. Type II : atresia of the common hepatic duct
3. Type III : atresia of the left and right hepatic duct.
The patient was diagnosed with Type III Biliary Atresia based on the clinical findings and
supporting examination, primarily intraoperative cholangiography.
Jaundice in infants persisting for >2 weeks is not considered physiologic, especially if the main
fraction found is conjugated/direct bilirubin. Infants with biliary atresia usually looks normal at
birth, and become jaundiced at the age of 306 weeks. The color of their feces may be normal, or
might be yellow initially but changed to pale yellow or to clay colored. Urine may become dark
or tea-colored.
Laboratory In biliary atresia, hyperbilirubinemia is observed, usually with the level of 6-12
mg/dL, 50% of which was conjugated. Transaminase and phosphatase alkaline is increased 2-3
times its normal level. The ϒ-glutamil transpeptidase is usually high.
Ultrasonography usually showed the small or invisible gallbladder. The biliary duct was
invisible and the liver may have increased echogenicity. Hepatobiliary imaging could be
performed using technetium-99m. Iminodiacetic acid (IDA) is beneficial to differentiate
obstructive from parenchymal jaundice. In biliary atresia, especially in its early stage, the
nucleotide uptake is rapid, but there is no excretion to the intestines.
Liver biopsy showed proliferation of the biliary tract, portal and periportal inflammation.
Fibrosis spreading between the branches of portal suggests the development of early cirrhosis.
Cholangiography is the final diagnostic measures usually performed as the initial step prior to
the portoenterostomy. From the small side of the right upper quadrant, the wrinkled gallbladder
will be visible. Usually the gallbladder lacks of lumen, or only has a small lumen containing
several drops of clear fluid. Contrast demonstration to the duodenum and continuity with
intrahepatic biliary duct will exclude biliary atresia. If cholangiography is not possible (the
lumen of gallbladder was nonexistent or obstructed), the incision is enlarged to bilateral
subcostal laparotomy to prepare for Kasai portoenterostomy
Kasai Procedure
The only therapy that gives hope of a cure for biliary atresia is surgery. the only procedure that
provides long-term success is portoenterostomi (kasai procedure) and liver transplantation.
Portoenterostomi procedure begins with the mobilization of the liver and gallblader, the
dissection of the cystticus duct into the common bile duct residual fiber (Figure 1). the
Peritoneum is opened to show the hepatic artery and biliary structures. The fibrous of communal
duct is carefully cut dissection continued proximally. Cysticus artery was ligated. be careful to
avoid misidentification of the right hepatic artery.

Fibrous bile duct extends into cone-shaped mass and enters the liver between the bifurcation and
the portal vein (figure 2). the branches of small vein must be divided carefully. the cone-shaped
fibrous is cut exactly at same way with the substance of the liver (figure 3). there is no cutting
cautery is used at the hilus.

Fibrous bile duct extends into cone-shaped mass and enters the liver between the bifurcation and
the portal vein (figure 2). the branches of small vein must be divided carefully. the cone-shaped
fibrous is cut exactly at same way with the substance of the liver (figure 3). there is no cutting
cautery is used at the hilus.
Referensi :
1. * Oldham, Keith T. et all (eds); Biliary Atresia at Principles and Practice of Pediatric Surgery, 4th
Edition
2. * Karrer, F. M. & Pence, J. C.; Atresia Bilier
at http://www.ningrumwahyuni.files.wordpress.com/2010/03
* Glossary of Medical Terms in Biliary Atresia Research
Consortium; http://www.barcnetwork.org/families/terms.html
* Biliary Atresia (Pediatric Gallbladder and BT) in
http://imaging.consult.com/image/topic/dx/Gastrointestinal?title=Biliary%20Atresia
%20%28Pediatric%20Gallbladder%20and%20BT%29&image=fig4&locator=gr4&pii=S1933-
0332%2806%2970865-5

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Kasai Procedure in the Management of Biliary Atresia

1. Type I : atresia of the common bile duct

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