Rabu, 29 Desember 2010

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/mm2, 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.

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.

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