Q. What is biliary atresia?
A. It is a progressive inflammatory process of unknown etiology (probably viral) which leads to fibrosis and obliteration of the intra and extra hepatic biliary tree. The etiopathogenesis of neonatal hepatitis and biliary atresia is probably the same, both being different points of time in the same clinical spectrum. Innovations and refinements in surgical treatment have improved the prognosis for this disease. Even for patients who do not become jaundice free after Kasai, liver transplantation (which is now becoming available in major Indian cities) brings new hope for cure. The current recommended treatment for all cases of biliary atresia is sequential treatment i.e. Portoenterostomy as the primary treatment followed by a delayed liver transplantation (live-related or cadaveric) in those patients who do not improve with portoenterostomy.

Q. Does viral infection lead to biliary atresia?
A. Many molecular biological studies have supported an inflammatory or infectious etiology for biliary atresia - NH syndrome. Clinically too, the dynamic nature of the disease process, the demonstration of inflammatory and giant cells in the tissues (liver and bile duct), the fibrotic response, presence of prominent lymph nodes at the porta hepatis and the demonstration of viral inclusion bodies indicate the inflammatory nature of this entity, rather than a purely embryological malformation as suggested by some. Of the various microorganisms, a viral etiology has been strongly suspected.

Association of reovirus type 3, rotavirus type CRNA and rotavirus type A have been suggested by serological studies but not confirmed. In our initial experience in this area there seems to be a strong evidence of an association of an association of antenatal or perinatal CMV infection and BA. In our experience the results of surgery also seem to be influence by the CMV positivity status.

Q. What is Kasai operation -Portoenterostomy (PE)?
A.In 1959, Morio Kasai from Sendai, Japan, first proposed that complete excision of the fibrous extrahepatic biliary tree and anastomosis of an intestinal loop to the raw transected area of the porta hepatis lead to a dramatic reduction in bilirubin levels due to bile drainage (Fig.1)

The bile flow after portoenterostomy was because of the microscopically patent biliary channels at the porta, although macroscopically the entire extrahepatic biliary tree was atretic. The chances of obtaining good bile flow after surgery, depend on the size and number of patent biliary channels.

Pre-operative preparation :
Bowel Preparation should begin 48 hours pre-operative orally.

Neomycin 12.5 mg/kg           four times a day
Metronidazole 7.5 mg/kg       three times a day

Postoperative care after portoenterostomy :
In the immediate postoperative period attention is given to prevention of accumulation of ascites and maintenance of tissue oxygenation. For first few days, transfusions of fresh frozen plasma are given and if albumin levels are low, album transfusions are indicated. The serum bilirubin, serum electrolytes and hematocrit are monitored every day for 4 days.

It is usually possible to commence oral feeds from the 4^th or 5^th day and the child is discharged on the 8^th or 9^th day. Prior to discharge, massive doses of Vitamin A and D are given and supplemental Vitamin E is given and the child is sent home on long term antibiotics (to be continued for at least 4 months), phenobarbitone, other vitamin supplements, cholestyramine and Tinospora. Patient is then asked to follow up after one and a half-month. LFT is done every once a month for at least 6 months.