PaedSurg.Net

Preop Preperation:​

Optimise Hb, clotting profile,

Vit K

10% Dextrose

PT, aPTT

Blood 

Consent and anesthesia fitness

Call for C-arm and flouroscopy

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Post Op

IV fluid

Remove NG and start oral after 48-72 hours

Remove abdominal drain at 5th POD or when drainage minimise. 

Notice colour of stool, should change immediately or after 10-14 days

NPO for 2-3 days, IV fluids and analgesia. 

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Start post kasai regime at 3rd to 5th POD

1. IV prednisolone 2mg/kg/day and taber off over 6 weeks (Steroids accelerate clearance of jaundice but donot result in improved survival of the native liver but has chloretic and antiinflammatory properties) - Side effects include: fluid retention and appetite suppression, infections and wound breakdown.

2. Ursodeoxycholic acid 10-15mg/kg/dose x BD for 1 year (it is a chloretic agent that replaces toxic bile acids, increases bile flow and stabilises hepatocyte membrane - it has an antiinflammatory and an antiapoptotic action)

3. Vit ADEK drops 1ml/day for 1 year

4. TP SMX 2.5mg/kg/day

5. Give PPI

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Principles:

1. Complete excision of fibrotic extrahepatic biliary remnants upto the porta hepatitis (portal plate)

2. Expose microscopic bile ductules at the porta hepatis to allow bile drainage 

3. Create a Roux en Y jejunal limb (classically 40-60cm)

4. Anastomose the jejunal limb to porta hepatis. a wide portoenterostomy reconstruction onto a portal plate, denuded of all tissue.

5. Ensure tension free and water tight anastomosis to prevent leakage and infection.

Incision: RIght subcostal 

Perform a cholangiogram. The laparotomy should look carefully for other anomalies (e.g. polysplenia, preduodenal portal vein, absence of the inferior vena cava, and malrotation

1. The liver should be fully mobilized by dividing the falciform ligament, coronary ligaments, and right and left triangular ligaments such that the organ can then be everted outside the abdominal cavity (t is necessary to warn the anesthetist at this stage as the maneuver impairs venous return to the heart by kinking the cava and will need an increase in intravenous volume support.)

2. A stay suture on the gallbladder allows its elevation and this is then separated with bipolar diathermy off its bed. The peritoneum overlying the portal triad (the hilar plate) is then divided and the various vascular (hepatic artery and portal vein) and biliary structures positively identified. 

3. The distal common bile duct is ligated and divided and the proximal part elevated from the underlying connective and lymphatic tissue. 

4. Following the divided end of the cystic artery should lead to identification of the right hepatic artery, which may be superficial or deep to the common duct and may need slinging

5. Continue dissection proximally along right and left portal veins, extending to: The umbilical fissure on the left. The bifurcation of the right portal vein on the right. Remove all overlying fibrous tissue carefully until a broad raw liver surface is created where microscopic ductules lie. Ensure small oozing points from the raw surface (often indicates good exposure).

6. Identify jejunum about 15–20 cm distal to the ligament of Treitz. Divide jejunum and create: Roux limb: ~30–40 cm (some use 40–60 cm) that will reach the porta without tension. Biliopancreatic limb: remains connected proximally. Jejunojejunostomy: Side-to-side or end-to-side anastomosis between the Roux limb and the distal jejunum, ~30–40 cm distal from division. Ensure good lumen and no twist. Pass the Roux limb: Usually retrocolic (through a window in transverse mesocolon) to the porta hepatis. Check there is no tension and no twist

7. Position the Roux loop snugly against the exposed portal plate. Make a small enterotomy on the anti-mesenteric border of the Roux limb: Length equal to or slightly greater than the width of the exposed portal plate. Posterior row: Interrupted fine absorbable sutures (e.g., 5/0 or 6/0 PDS/Vicryl). Take bites from jejunal mucosa and full thickness of the portal plate capsule (do not go too deep into the liver). Start from one lateral end and go across to the other. Anterior row: Similar interrupted sutures, ensuring: The jejunum is well spread over the whole raw liver surface. No gaps or dead space. Result: a broad mucosa-to-portal-plate anastomosis. Key concept: Extended Kasai = very wide portoenterostomy covering the entire hilar plate, not just a narrow central area.

Original Kasai - Shallow continuous sutures except at 10 and 2 o clock (if needed just place in the connective tissue)

Modified Kasai - Shallow interrupted sutures except at 10 and 2 o clock (if needed just place in the connective tissue)

Extended Kasai - take control of hepatic artery and portal vein on both sides, make a wide anastomosis with deep interrupted sutures through the liver parenchyma even at 10 and 2 o clock