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Biliary Atresia

Contents

  1. Introduction

  2. Differential Diagnosis

Introduction

It is progressive sclerosing cholangiopathy of unknown etiology

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BIliary Atresia occurs in 1 in 10,000 to 1 in 167000 live births with Female:Male ratio to 1.4:1.7. 

Types of Biliary Atresia (Japanese Classification)

  • Type I: Level of CBD

  • Type II: Level of CHD (2a: CHD, 2b: CBD, CHD, Cystic)

  • Type III: At the porta hepatis - Most frequent. 

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Possible theories:

  1. Genetic: more in certain populations, associated with other congenital anomalies. 

  2. Inflammatory

  3. Infectious (Virus) - Reo and Rota virus 

  4. Local ischaemia during fetal hepatobiliary development resulting in abnormal vascular-ductal interactions.

  5. Abnormal bile metabolism 

  6. Pancreaticobiliary maljunction 

  7. Environmental toxins

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60% have pigmented stools sometimes in the postnatal period. Seasonal clustering of biliary atresia in winter months. 

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Davenport Classification

  1. Syndromic Biliary Atresia: Laterality defects and Biliary Atresia Splenic Malformation (BASM) with cardiac defects, inferior vena cava malformations, azygous continuation, preduodenal portal vein. 

  2. Cystic Biliary Atresia: Cystic change in obliterated biliary tract 

  3. CMV associated 

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Composition of bile

97% water

Bile salts 

glycorolate

Torocolate 

cholesterol 

phospholipid

electrolytes

Fatty acids

Proteins

IgA

in the remaining three percents

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Bile metabolism

RBC --> Heme --> Bilivirdin (unconjugated bilirubin) --> albumin transport protein to the liver where conjugation occurs by UDP glucoronyl transferase  (UGT) converted to conjugated bilirubin --> bile in intestine --> urobilinogen --> converted to urobilin (excreted by urine) and stercobilin (excreted via faeces)

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Associated syndromes with BA

MPS CIA 

Malrotation

Preduodenal portal vein

Polyspenia 

Cardiac malformation

Interrupted IVC

Azygous continuation

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Growth failure

is an important consequence of liver disease indicating liver transplantation.

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Physiological Jaundice

Jaundice that occurs after 24 hours and before 15 days of life due to breakdown of fetal Hb.

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Pathological Jaundice

Jaundice that persists beyond 2 weeks of life and is in the first day of life

Differential Diagnosis

  1. Biliary Atresia (Progressively worsening jaundice, clay coloured stool and dark urine)

  2. Bile Puct Paucity Syndrome (Static Jaundice)

  3. Inspissated Bile Syndrome (History of delayed passage of meconium and MI)

  4. Viral Hepatitis (Fever)

  5. Infantile Choledocal Cyst (palpable mass)

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The differential diagnosis for jaundice in early infancy is vast and requires a full workup. However, the two main causes of cholestatic jaundice are idiopathic neonatal hepatitis and biliary atresia. Causes of neonatal cholestatic jaundice can be divided into intrahepatic and extrahepatic causes. Extrahepatic causes include biliary atresia, choledochal cyst, bile duct stenosis, strictures and cholelithiasis, spontaneous perforation of the common bile duct, and tumours or masses. Intrahepatic causes are numerous and are generally divided into six possible categories: infectious, metabolic, genetic, toxic, cholangiopathies and others.

 

Gilbert’s disease is an inherited cause of unconjugated hyperbilirubinaemia. Although it is rare, Gilbert’s disease is the most common genetic cause of unconjugated hyperbilirubinaemia. Haemolytic anaemia is caused by the destruction of premature erythrocytes and is also characterised by unconjugated hyperbilirubinaemia.

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The pathology of biliary atresia is characterised by an obstructive biliary pattern that includes fibrosis, inflammation and proliferation. The differential diagnosis for these findings on liver biopsy includes choledochal cyst, bile duct strictures and stones, total parenteral nutrition–associated cholestasis, alpha-1 antitrypsin deficiency, cystic fibrosis, multi drug resistance 3 deficiency, North American Indian childhood cirrhosis (cirrhin deficiency) and Alagille’s syndrome.

Investigations

Blood investigations

  1. Liver function tests (Increased conjugated bilirubin, increased ALP, increased GGT [more specific])

  2. Clotting cascade abnormalities and abnormal albumin (in case of liver decompensation)

  3. Viral markers (Hep B, Hep C, TORCH screening - CMV indicates worse prognosis)

  4. Alpha 1 antitrypsin levels

  5. Serum Lipoprotein X

​Ultrasound Abdomen (4 hours fasting and postprandial to see for the change in the size of the gallbladder lumen, Ultrasound will show contracted gall bladder, triagular cord sign, ghost sign, vascular patency, echotexture of the liver, ascites, status of the spleen)

  1. If dilated proximal bile ducts on USG --> Inspissated bile ducts (manage by observation or operative cholangiogram with or without biliary irrigation) 

  2. Hilar cystic structure --> Cystic biliary atresia or infantile choledocal cyst 

  3. Triangular cord sign (Hyperechogenic liver hilum between the two portal veins representing fibrotic biliary structures), ghost sign (Atretic Gall bladder, irregular contour, lack of echogenic mucosal lining) --> BA

  4. Use of doppler to improve accuracy (echotexture, presence of ascite, patency of hepatic vasculature)

Bedside test to check for biliary atresia 

Pass NG tube and aspirate, if yellowish green coloured bile, then biliary atresia is ruled out)

Percutaneous Liver biopsy

Non operative, most accurate way of diagnosing BA. It will show inflammation with ductal proliferation, bile stasis with plugging and giant cell formation. 

Intraoperative Cholangiogram

  1. Apply a purse string suture on the gallbladder

  2. Insert an angiocatheter/laparoscopic cholangiogram catheter/butterfly in the lumen of the gallbladder and tie the purse string suture. 

  3. Diatrizoic Acid (Hypaque or Gastrograffin) is diluted 1:1 with N/S and injected under real time flouroscopy.

  4. If contrast flows freely in the duodenum and into the intrahepatic bileducts then patency has been established --> perform liver bipsy, remove the catheter and close the cholecystostomy. 

  5. If the liver is fibrotic, nodular with a green colour and the gallbladder is contracted and fibrotic then the diagnosis is confirmed, proceed to Kasai

  6. If the gallbladder is normal then put a purse string suture and aspirate, if white bile comes --> diagnosis is confirmed and one can proceed to portal plate dissection without a cholangiogram. 

  7. If the aspirate is dark and there is ongoing doubt then perform a cholangiogram. 

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Hepatobiliary Scintography 

HIDA: Hydroxyiminodiacitic acid

DISIDA: Diisopropyliminodiacetic acid: More effective in symptomatic cholestasis.

Presence of the isotope in intestine excludes the diagnosis. In liver dysfunction, get a delayed assessment of isotopic secretion after 24 hours. 

In jaundiced patients, pretreatment with phenobarbitone 5mg/kg/day for 5 days. It induces hepatic microsomal 

This tracer is taken up by the hepatocytes and secreted into the bile. Pictures are taken by a gamma camera. 

Kramer Rule

Tells about the probable levels of bilirubin according to the extent of jaundice. 

Face (5mg/dl)

Face + Upper trunk (10mg/dl)

Face + Upper trunk + lower trunk + thighs (12mg/dl)

Extending to the arms and legs (15mg/dl)

Going to the soles (>15mg/dl)

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Osteopontin, basic fibroblast growth factor and hepatocyte growth factor are associated with biliary atresia progression. A raised level of transforming growth factor-β is associated with improved biliary atresia outcomes.

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Screening for cystic fibrosis, hypothyroidism, galactosemia and other metabolic and genetic conditions

Pathology

The characteristic features include expansion of portal tracts, infiltration by inflammatory cells and portal tracts with bile plugs. The expansion of the portal tracts may carry varying degrees of fibrosis that may be predictive of the long-term success of the Kasai procedure. There may also be parenchymal changes such as syncytial giant cell formation, lobular disarray and extramedullary haematopoiesis.

In the pile ducts

Bile duct proliferation, expansion of portal tracts

Bile duct stasis

Varying degrees of fibrosis, infiltration of inflammatory cells

In the liver parenchyma

Syncitial giant cell formation 

Lobular disarray 

Extramedullary hematopoesis

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Limitation: Cannot differentiate between BA and IFLD, alpha 1 antitrypsin deficiency)

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Aim of Kasai: After portoenterostomy the ductules responsible for bile drainage, overtime these shoulf form stable bile conduits.

Procedure

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

Complications after the procedure

Cholangitis 

Occurs due to gram -ve organisms and anaerobes

  1. Reflux of intestinal contents

  2. Portal venous infections

  3. Impaired lymphatic drainage at porta

  4. Bacterial translocation

  5. Intrahepatic biliary stasis

It causes fever, abdominal pain and jaundice 

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Treat with antibiotics (TPM + SMX that delays the onset of cholangitis and use of steroid pulses)

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Prognosis

Untreated Biliary atresia is fatal within 2 years 

Survival <10% at 3 years

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Outcome

pigmented stools immediately or within 10-14 days in 2/3rd cases: half of which will continue to drain bill well, efficient clerance of jaundice and normal development, in other half ongoing fibrosis and scarring causing progressive liver failure = will require liver transplat at 5.4 years

Remaining 15-30% will continue to have acholic stools and liver failure within months

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Long term

Abnormalities in menstruation, pubertal disorders Challenges in pregnancy, PTN

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Good Prognostic Factors in Kasai

Age < 60 days at the time of Kasai

Gross appearance of gall bladder

Ductules > 110-150micrometer

Experienced surgeon and center 

Colic stools within first 2 weeks 

Decrease in bilirubin

Type I and II BA

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Worse prognostic factors

BASM

Nodular Liver 

CMV associated BA

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Indication of liver transplant: 

  1. No bile drainage after operation with progressive liver disease

  2. Episodic or inefficient bile drainage with slow deterioration of liver function and growth failure 

  3. Development of one or complications of CLD ie cholangitis, portal HTN that cannot be managed despite functional portoenterostomy 

  4. >3 months of age, 

  5. Signs of decompensated liver disease at any age. 

Follow up

Follow up at 2 weeks then at 6 weeks till 6 months for 

weight 

nutritional management 

vitamin D levels

LFTs

USG for liver echotexture

Portal Hypertension

34-76% of children even after seemingly excellent results of bile drainage can develop drainage. 

Ascites is the most common finding in 60% of children. 

Half of the patients will present with esophageal varices so surveillance endoscopy (half yearly to yearly) is recommended and then prophylactic endoscopic sclerotherapy if varices are detected 

If varices are bleeding then:

Multiple sessions of sclerotherapy

Octreotide

Beta adrenergic blocking agents

variceal ligation

Variceal bleeding doesnt signify end stage liver disease. 

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