Overview
Definition:
Biliary atresia (BA) is a rare, progressive, fibrosing obliterative process affecting the extrahepatic bile ducts, leading to cholestasis and ultimately biliary cirrhosis if untreated
A redo portoenterostomy is a surgical procedure performed when a primary Kasai procedure has failed, aiming to re-establish biliary drainage
It involves revising the existing portoenterostomy site or creating a new one, often with modifications to improve bile flow and reduce complications.
Epidemiology:
Biliary atresia affects approximately 1 in 10,000 to 1 in 20,000 live births worldwide, with regional variations
It is a leading indication for pediatric liver transplantation
Redo portoenterostomy is considered in a subset of patients who develop recurrent cholangitis, progressive jaundice, or portal hypertension after an initial successful Kasai procedure.
Clinical Significance:
The decision to perform a redo portoenterostomy is complex, balancing the potential benefits of improved biliary drainage against the risks of further surgery and the eventual need for liver transplantation
Understanding the indications, techniques, potential complications, and prognostic factors is crucial for surgical residents preparing for DNB and NEET SS examinations, as well as for optimizing patient outcomes.
Indications For Redo
Failure Of Primary Kasai:
Persistent or recurrent jaundice (serum bilirubin > 5 mg/dL with conjugated fraction > 50%) 6 months post-primary portoenterostomy
Progressive failure to thrive
Recurrent episodes of cholangitis.
Post Kasai Complications:
Development or worsening of portal hypertension (splenomegaly, ascites, varices) despite adequate initial bile drainage
Development of biliary strictures at the anastomosis site
Persistent pruritus refractory to medical management.
Patient Selection Criteria:
Absence of advanced biliary cirrhosis (stage 3 or 4 fibrosis)
Adequate liver synthetic function
Absence of significant anatomical anomalies that preclude further reconstructive surgery
Patient and family understanding of the limited success rate and potential need for transplantation.
Diagnostic Approach Redo
History And Physical:
Detailed history of previous surgeries, complications (cholangitis, bleeding), and response to initial treatment
Assessment of nutritional status, growth, and presence of pruritus
Physical examination focusing on jaundice, hepatosplenomegaly, ascites, and signs of portal hypertension.
Laboratory Investigations:
Complete blood count (CBC) with differential to assess for anemia and infection
Liver function tests (LFTs) including total and direct bilirubin, ALT, AST, ALP, GGT, albumin, and prothrombin time/INR to assess synthetic function and degree of cholestasis
Serological markers for viral hepatitis
Alpha-fetoprotein for hepatocellular carcinoma screening.
Imaging Modality:
Abdominal ultrasonography to assess liver size and texture, spleen size, ascites, and portal vein diameter/flow
Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) to delineate the biliary tree, identify strictures, assess patency of the porta-enteric anastomosis, and rule out other causes of obstruction
Cholangiography during surgery is essential.
Surgical Technique Considerations
Anesthesia And Access:
General anesthesia with meticulous attention to fluid management and hemodynamic monitoring
Laparoscopic or open approach depending on surgeon preference, previous surgical history, and complexity of revision
Careful adhesiolysis to identify the porta-enteric anastomosis and surrounding structures.
Portoenterostomy Revision:
Dissection and mobilization of the porta-enteric anastomosis
Excision of fibrotic tissue at the hepatic hilum
Consideration of different approaches: revising the existing anastomosis, performing a distal Roux-en-Y diversion, or creating a completely new portoenterostomy
Techniques to minimize tension and promote healthy mucosa-to-mucosa apposition
Careful inspection of the jejunal limb for viability.
Adjunctive Measures:
Intraoperative cholangiography to confirm patency and identify any residual strictures or leaks
Placement of a T-tube or stent if necessary for drainage or later cholangiographic assessment
Management of portal hypertension, including ligation of varices if present and feasible
Prophylactic antibiotics to reduce the risk of cholangitis.
Postoperative Management
Monitoring And Support:
Close monitoring of vital signs, urine output, and abdominal distension
Pain management with analgesics
Intravenous fluids and electrolyte balance
Nutritional support with appropriate formula and vitamin supplementation (fat-soluble vitamins A, D, E, K)
Monitoring for signs of infection, bleeding, or anastomotic leak.
Bile Drainage Assessment:
Assessment of bile output from the drains and stoma (if applicable)
Monitoring of stool color for improvement in acholic stools
Serial LFTs to assess the trend of bilirubin and liver enzymes
Early recognition and management of cholangitis through appropriate antibiotic therapy.
Long Term Follow Up:
Regular outpatient follow-up with assessment of growth, nutritional status, and LFTs
Serial imaging to monitor for recurrence of biliary strictures or development of portal hypertension
Echocardiography for evaluation of pulmonary hypertension
Screening for hepatocellular carcinoma with ultrasound and AFP
Consideration for liver transplantation if redo surgery fails to improve cholestasis or if decompensated cirrhosis develops.
Complications And Outcomes
Early Complications:
Bleeding from the surgical site or varices
Anastomotic leak or stricture
Cholangitis
Wound infection
Ileus
Sepsis.
Late Complications:
Recurrent cholangitis
Progressive biliary cirrhosis
Portal hypertension with its sequelae (esophageal varices, ascites, encephalopathy)
Malabsorption
Nutritional deficiencies
Hepatocellular carcinoma.
Prognosis And Survival:
The success rate of redo portoenterostomy is significantly lower than the primary Kasai procedure, with reported rates of achieving adequate bile drainage ranging from 20% to 50%
Long-term survival without liver transplantation is highly variable and dependent on factors such as the degree of fibrosis at reoperation, the success of re-establishing bile flow, and the absence of severe cholangitis
Many patients eventually require liver transplantation, with the timing dictated by the severity of liver disease and complications.
Key Points
Exam Focus:
Indications for redo portoenterostomy are recurrent jaundice/cholangitis or worsening portal hypertension post-primary Kasai
Meticulous intraoperative cholangiography is crucial
Complications include recurrent cholangitis, strictures, and progression to cirrhosis
Outcomes are generally poorer than primary surgery, with transplantation often being the definitive treatment.
Clinical Pearls:
Always consider nutritional status and fat-soluble vitamin supplementation in patients with ongoing cholestasis
Aggressive management of cholangitis is paramount
Involve a multidisciplinary team including pediatric gastroenterologists and transplant surgeons for optimal care.
Common Mistakes:
Proceeding with redo surgery in patients with advanced cirrhosis
Inadequate dissection or failure to identify the porta-enteric area
Poorly performed intraoperative cholangiography leading to missed residual strictures
Underestimating the risk of recurrent cholangitis.