Biliary Atresia

Biliary atresia is a progressive fibro obliterative disease of the infant biliary tree causing obstruction of bile flow, cholestasis and rapidly progressive hepatic fibrosis.

Without restoration of biliary drainage, cirrhosis and liver failure develop early in life.

The Kasai portoenterostomy restores bile drainage in a proportion of infants and can preserve the native liver for years or decades, but it does not remove the underlying intrahepatic disease.

Early diagnosis is critical because successful native liver outcomes decline substantially with delayed surgery, particularly beyond approximately 90 days.

The infant typically appears well initially but develops persistent jaundice.

High value features are:

Jaundice persisting beyond approximately 2 weeks
Pale or acholic stool
Dark urine
Hepatomegaly
Progressive splenomegaly
Poor weight gain with advancing disease

Any infant with pale stool and conjugated hyperbilirubinaemia requires urgent investigation rather than reassurance for physiological neonatal jaundice.

Measure fractionated bilirubin. A direct bilirubin above approximately 1 mg/dL after 2 weeks of age is abnormal and requires evaluation.

Ultrasound: first imaging study; assess the gallbladder and porta hepatis for features supporting biliary atresia.

Liver biopsy can demonstrate obstructive cholangiopathy with ductular proliferation, bile plugs and fibrosis.

Intraoperative cholangiography: provides definitive assessment when the diagnosis remains uncertain and can be followed immediately by Kasai portoenterostomy when biliary atresia is confirmed.

Do not allow repeated nondiagnostic testing to delay referral to paediatric hepatobiliary surgery.

Kasai portoenterostomy

Perform Kasai portoenterostomy as early as possible, ideally during the first several weeks of life. Outcomes are generally better when surgery occurs before approximately 60 days, although age is a continuum rather than an absolute cutoff and potentially suitable infants presenting later should still receive specialist assessment.

The fibrotic extrahepatic biliary remnant is excised to the porta hepatis until microscopic bile ductules are exposed.

A Roux jejunal limb is then anastomosed to the porta to drain bile directly from these residual ductules.

Successful drainage is suggested by progressive reduction in conjugated bilirubin and return of pigmented stool.

Postoperative care

Nutrition is aggressive because cholestasis causes fat malabsorption and growth failure.

Use energy dense feeding with medium chain triglyceride supplementation where necessary and replace vitamins A, D, E and K according to measured deficiency and cholestasis severity.

Post Kasai ascending cholangitis is common. Fever, worsening jaundice, pale stool or inflammatory deterioration should trigger immediate IV antibiotic treatment.

Antibiotic prophylaxis for several months after Kasai is commonly used, but the optimal agent and duration remain uncertain and current evidence has not demonstrated a consistent reduction in cholangitis across all populations.

Liver transplantation

Refer early for transplant assessment when there is:

Failed Kasai with persistent severe jaundice
Progressive synthetic liver failure
Recurrent severe cholangitis
Portal hypertension with significant complications
Refractory ascites
Severe growth failure
Hepatopulmonary complications
End stage cirrhosis

An infant presenting very late with established advanced cirrhosis may be better served by primary liver transplantation rather than a low probability Kasai procedure.

Even children with successful bile drainage require lifelong hepatology surveillance because portal hypertension, cholangitis and progressive fibrosis can develop despite a functioning Kasai.

Clinical toolsCalculators

BMI GCS EDD Parkland Formula

Corrected Sodium Corrected Calcium Anion Gap Creatinine Clearance Insulin Calculations qSOFA Wells PE Wells DVT CHA₂DS₂-VASc HAS-BLED CURB-65 Centor/McIsaac

Acute Calculous Cholecystitis

Also known as: Cholecystitis

Acute calculous cholecystitis is acute inflammation of the gallbladder caused by persistent cystic duct obstruction by a gallstone.

Obstruction produces gallbladder distension, venous and lymphatic compromise, inflammatory injury and eventually secondary bacterial infection in some patients.

Progression can produce empyema, gangrenous cholecystitis, perforation, pericholecystic abscess or biliary peritonitis.

Definitive treatment in a surgically fit patient is early laparoscopic cholecystectomy during the same acute admission.

The typical presentation is persistent right upper quadrant or epigastric pain, usually lasting more than several hours, with nausea or vomiting.

Fever and local right upper quadrant tenderness are common.

Murphy sign: arrest of inspiration due to pain when the examiner palpates the right subcostal region during deep inspiration.

Older, diabetic and immunosuppressed patients may have limited fever or peritoneal signs despite advanced disease.

Severe persistent pain, high fever, sepsis or localised peritonism should raise concern for gangrene, empyema or perforation.

Jaundice requires assessment for common bile duct obstruction, Mirizzi syndrome or cholangitis.

Check full blood count, C reactive protein, renal function and liver biochemistry.

Leukocytosis and inflammatory marker elevation support the diagnosis but do not determine severity alone.

Right upper quadrant ultrasound: first line imaging; identify an obstructing gallstone with the key inflammatory features of gallbladder wall thickening, distension or pericholecystic inflammation.

If ultrasound is equivocal but clinical suspicion remains strong, hepatobiliary scintigraphy or cross sectional imaging can clarify the diagnosis.

Evaluate for common bile duct stones when bilirubin, alkaline phosphatase or duct calibre is abnormal.

Keep fasting initially, establish IV access and correct dehydration.

Provide adequate analgesia:

Paracetamol 1 g orally or IV every 6 to 8 hours, maximum 4 g daily in an adult without significant hepatic risk.

Add an NSAID when appropriate.

Start antimicrobial treatment for acute cholecystitis.

A practical community acquired regimen is:

Ceftriaxone 2 g IV every 24 hours.

Add:

Metronidazole 500 mg IV every 12 hours

when anaerobic coverage is clinically warranted, particularly with severe infection, perforation or altered biliary gastrointestinal anatomy.

For septic shock, gangrene, perforation or significant healthcare associated infection:

Piperacillin tazobactam 4.5 g IV every 8 hours by extended infusion, adjusted for renal function.

Definitive surgery

Perform laparoscopic cholecystectomy during the index admission and as early as clinically feasible after initial resuscitation. Early surgery reduces total hospitalisation and avoids recurrent gallstone complications without requiring an interval cooling off period.

During surgery, prioritise prevention of bile duct injury.

Achieve the critical view of safety before clipping or dividing structures.

If severe inflammation prevents safe identification:

Stop unsafe dissection
Consider a fundus first approach only when anatomy remains controllable
Perform subtotal cholecystectomy when necessary
Convert to open surgery when this improves safety, although conversion alone does not guarantee clearer anatomy

Subtotal cholecystectomy can be fenestrating or reconstituting according to anatomy and surgeon judgement.

High operative risk

A patient with severe acute illness who cannot safely undergo cholecystectomy may require percutaneous cholecystostomy for source control. Do not delay drainage simply to complete a prolonged failed conservative trial when the gallbladder remains the uncontrolled septic source.

Cholecystostomy is generally a bridge rather than definitive treatment in a patient who later becomes fit for cholecystectomy.

Antibiotic duration

After uncomplicated laparoscopic cholecystectomy for mild or moderate acute cholecystitis with satisfactory source control, routine postoperative antibiotics are unnecessary.

For severe cholecystitis with organ dysfunction or infection extending beyond the gallbladder, continue antibiotics according to clinical response, generally for no more than approximately 4 days after adequate source control.

Biliary Atresia

Overview

Clinical Features

Diagnosis

Management

Overview

Clinical Features

Diagnosis

Management