Kasai Portoenterostomy (Biliary Atresia)
Biliary atresia, neonatal cholestasis with [direct hyperbilirubinemia / acholic stools / HIDA scan non-excretion]
Same
Hepatic portoenterostomy (Kasai) with Roux-en-Y reconstruction
[***, MD/DO]
[Resident name]
General endotracheal. Orogastric tube placed.
Patient is a [X]-day-old [male / female] (age [X] days, weight [X] kg) presenting with neonatal cholestasis and direct hyperbilirubinemia (direct bilirubin [X] mg/dL), acholic stools, and [HIDA scan without biliary excretion / liver biopsy consistent with biliary atresia]. Evaluation complete with no correctable anatomic cause identified. Risks including cholangitis (most common postoperative complication), failure of bile flow, cirrhosis progression, and eventual liver transplant discussed with parents. Best outcomes when performed before 60 days of life. Consent obtained.
Triangular cord sign confirmed intraoperatively. Extrahepatic biliary tree fibrotic and atretic ([no identifiable lumen at common bile duct / gallbladder small and fibrotic / common hepatic duct atretic at hilum]). [Intraoperative cholangiogram confirmed no biliary drainage.] Liver [enlarged / normal size] with [early cirrhotic / mild fibrotic] changes. [Ohi Type [I / II / III] biliary atresia.] Hilar plate dissected to [X] mm above bifurcation. Patent ductules [present / not visualized] at cut surface.
The patient was positioned supine. A limited right upper quadrant incision was made. [Intraoperative cholangiogram confirmed biliary atresia. The incision was extended.] The liver was mobilized by dividing the falciform and triangular ligaments. The porta hepatis was exposed. The gallbladder was dissected free and used as a handle for liver retraction.
The extrahepatic biliary tree was found to be completely atretic. The fibrous remnant was dissected at the porta hepatis to the level of the hilar plate using sharp dissection without electrocautery (electrocautery is avoided at the hilar plate to prevent thermal injury to microductules). The hilar plate was dissected across the full width of the porta hepatis, extending to the Rex recess on the left and to the right anterior and posterior sectoral duct origins, with identification and protection of the portal vein bifurcation and hepatic artery branches. The fibrous cone was excised with fine scissors at the level of the bifurcation, exposing the cut surface for any patent ductules. The liver capsule was not entered.
A 40-cm Roux-en-Y jejunal limb was constructed. The Roux limb was brought up to the liver hilum in a [retrocolic / antecolic] position. A portoenterostomy was created by suturing the Roux limb to the hilar plate circumferentially with [interrupted / running 5-0 PDS], creating a wide anastomosis to any patent bile ductules.
[Liver biopsy taken with needle from left lobe (Tru-Cut technique) and sent to pathology.]
[Gallbladder removed en bloc with the fibrous remnant and sent to pathology.]
The hilar anastomosis was inspected. Fascia closed with [running 0-PDS]. Skin closed. Patient tolerated the procedure well.
None
[Fibrous biliary remnant and gallbladder to pathology / Liver biopsy to pathology]
[X] mL
[Jackson-Pratt drain near hilum / None]
Patient transferred to NICU/PICU intubated in stable condition.
Epic SmartPhrase Version
PREOPERATIVE DIAGNOSIS: Biliary atresia with neonatal cholestasis, direct hyperbilirubinemia, acholic stools
POSTOPERATIVE DIAGNOSIS: Same
PROCEDURE PERFORMED: Hepatic portoenterostomy (Kasai) with Roux-en-Y reconstruction
ATTENDING SURGEON: ***, MD/DO
FIRST ASSISTANT: ***
ANESTHESIA: General endotracheal, OGT placed
INDICATIONS: The patient is a ***-day-old .PTSEX (weight *** kg) with neonatal cholestasis, direct bilirubin *** mg/dL, acholic stools, and HIDA scan without biliary excretion consistent with biliary atresia. Risks including cholangitis, failure of bile flow, cirrhosis, and eventual transplant discussed with parents. Informed consent obtained.
FINDINGS: Triangular cord sign confirmed. Extrahepatic biliary tree completely atretic. [Ohi Type ***.] [Intraoperative cholangiogram: no bile flow.] Liver [enlarged / mildly fibrotic]. Hilar plate dissected; patent ductules [present / not visualized] at cut surface.
DESCRIPTION OF PROCEDURE:
Patient supine. Limited RUQ incision; [cholangiogram confirmed atresia; incision extended.] Liver mobilized (falciform, triangular ligaments divided). Porta hepatis exposed; gallbladder used as handle. Fibrous extrahepatic biliary remnant dissected to hilar plate with sharp dissection (no electrocautery at hilar plate). Dissection extended to Rex recess left, right anterior/posterior sectoral origins; portal vein and hepatic artery protected. Fibrous cone excised with fine scissors at bifurcation. Liver capsule not entered. 40-cm Roux-en-Y jejunal limb constructed and brought [retrocolic / antecolic] to hilum. Portoenterostomy created with circumferential [interrupted / running] 5-0 PDS anastomosis to hilar plate. [Needle liver biopsy taken from left lobe.] Gallbladder removed en bloc with remnant and sent to pathology. Fascia closed with 0-PDS. Skin closed. Patient tolerated procedure well.
ESTIMATED BLOOD LOSS: *** mL
SPECIMENS: Biliary remnant and gallbladder to pathology; [liver biopsy to pathology]
COMPLICATIONS: None
DRAINS: [JP drain near hilum / None]
DISPOSITION: Patient to NICU/PICU intubated, stable.
Signed: .ME, .MYDEGREE
.TODAYVariants
Failed Kasai / transplant evaluation
Patients with inadequate bile flow post-Kasai (total bilirubin >2 mg/dL at 3 months or >6 mg/dL at 6 months) should be referred for liver transplant evaluation. Document bile flow response in follow-up notes.
BASM syndrome (biliary atresia-splenic malformation)
Approximately 10% of biliary atresia cases. Associated with polysplenia or asplenia, preduodenal portal vein, absent IVC, situs inversus, and malrotation. Significantly worse native liver survival than isolated BA. Document all associated anomalies. Preduodenal portal vein requires modified dissection — identify before dividing any hilar structures. Malrotation should be corrected at the same setting if present.
Cholangitis after Kasai
Most common postoperative complication. Treated with IV antibiotics and steroids. Early cholangitis (<6 months post-op) is the strongest predictor of native liver failure. Recurrent cholangitis is an indication for transplant evaluation.
Charting Tips
- Document age at surgery in days (not weeks) — the 60-day threshold is the primary prognostic cutoff; outcomes decline progressively, with best native liver survival at surgery before 30 days
- Document weight at surgery — required for low-weight-infant reporting eligibility (<4 kg) and prognostic assessment
- Record Ohi classification (Type I: common duct atresia; Type II: common hepatic duct atresia; Type III: porta hepatis atresia — most common) in findings
- Confirm hilar plate dissection level, extent (full width to Rex recess left and right sectoral origins), and that no electrocautery was used at the portal plate
- Note bile ductule visualization at cut surface (patent ductules present or not visualized) — a key prognostic variable
- Document Roux limb length (40 cm standard) and route (retrocolic vs. antecolic)
- Send fibrous remnant AND gallbladder to pathology to confirm biliary atresia histologically
- Document intraoperative cholangiogram technique and findings if performed (gallbladder cannulation via purse-string with diluted contrast)
Documentation & Reimbursement Considerations
- Report Kasai Portoenterostomy (Biliary Atresia) as the primary service when it is the definitive operation performed.
- No modifier or add-on is needed for the Roux-en-Y reconstruction, which is bundled in the primary service. The gallbladder is also bundled — do not separately bill cholecystectomy codes.
- Document findings: the cholangiogram drives the decision to proceed with Kasai vs. close.
- Low-weight-infant reporting (procedure performed on infant less than 4 kg): applies when patient weight is under 4 kg. Many Kasai patients are in this range. Document weight at time of surgery explicitly — this modifier increases reimbursement by approximately 20-25% on eligible codes and requires contemporaneous weight documentation.
- Use related unplanned return-to-the-operating-room reporting if within 90-day global of original Kasai (unplanned return for complication) or staged-or-related-procedure reporting if planned staged revision. Document indication for revision.
- 90-day global: cholangitis admissions, steroid protocols, and NICU/floor management are not surgical fee items within the global, but they generate separate E/M and hospital billing. Patients who fail the Kasai and proceed to liver transplant: the transplant is billed as a new procedure with its own global period.