OBSTRUCTIVE JAUNDICE

Dr.B.Selvaraj MS;Mch;FICS;Professor Of Surgery

Melaka Manipal Medical collegeMelaka 75150 Malaysia

BILIARY ATRESIA

BILIARY ATRESIA- Objectives

To be aware of possible causes of surgical jaundice in infancy

To be aware of clinical features of biliary atresiaTo be familiar with the methods of investigationTo understand the management optionsTo be aware of the surgical outcome and results

BILIARY ATRESIABiliary atresia is also known as progressive obliterative

cholangiopathyBiliary atresia causes a progressive damage of the extrahepatic

and intrahepatic bile ducts with cholestasis because of obstructive cholangiopathy secondary to inflammation, leading to fibrosis, and if not recognized and treated, it leads to biliary cirrhosis and liver failure.

The incidence of biliary atresia is 1:10,000 to 1:15,000 live births

Biliary atresia affects girls more often than boys.

BILIARY ATRESIAAsians and African-Americans are affected more frequently than

Caucasians - more common in Chinese and Japanese.Biliary atresia should be recognized and distinguished from

neonatal jaundice. Infants with prolonged jaundice should be thoroughly investigated for biliary atresia.

Biliary atresia should be considered in all neonates with direct hyperbilirubinemia

A high index of suspicion is important to make the diagnosis of biliary atresia because surgical treatment by age 2 months has clearly been shown to improve the outcome and establishment of bile flow and to prevent the development of biliary cirrhosis.

BILIARY ATRESIA-Etiology

The exact cause of biliary atresia is not known and many factors have been incriminated

1. Reovirus 3 infection.2. Congenital malformation. Early studies postulated a congenital

malformation of the biliary ducts leading to their obstruction.3. Congenital cytomegalovirus (CMV) infection.4. Autoimmunity5. A possible association with the gene GPC1, which is located on

the longarm of chromosome 2 (2q37).6. Identification of active and progressive inflammation and

destruction of the biliary system

BILIARY ATRESIA-Types

Clinically, biliary atresia occurs in two distinct forms: The fetal-embryonic form: − Appears in the first 2 weeks of life. − About 10–20 % of affected neonates have associated congenital defects. The postnatal form: − Appears in neonates and infants aged 2–8 weeks. − Progressive inflammation and obliteration of the extrahepatic bile ducts occur after birth. − Not associated with congenital anomalies. − Infants may have a short jaundice-free interval.

BILIARY ATRESIA-Types

Type I: atresia of the common bile duct

Type IIa: atresia of the common hepatic duct

Type IIb: atresia of common bile duct, cystic duct, and common hepatic duct

Type III: atresia of the common bile duct, cystic duct, and hepatic ducts up to the porta hepatis. This is the subtype present in over 90% of patients with biliary atresia

ASSOCIATED ANOMALIES

Associated anomalies are seen in 10 to 20 % of biliary atresia cases.

Polysplenia syndrome: These include:− Cardiac malformations − Polysplenia− Situs inversus− Absent vena cava− A preduodenal portal vein-- Bilobed symmetric liver-- Bilobed lungs

BASM Syndrome

Clinical FeaturesInfants with biliary atresia are typically full termThe symptoms are seen usually between 1 and 6 weeks of life Conjugated Jaundice which is prolongedDark urine because of bilirubin in urineClay-colored stools because of absence of stercobilin.- acholic

stoolsVitamin K deficiency and coagulopathyAntenatally detected subhepatic cystCirrhosis (ascites and hepatosplenomegaly), rarely younger than

3 months

Biliary Atresia- Investigations

LFT: Total bilirubin and direct bilirubin are elevated ALP & GGT are elevated; AST & ALT mildly elevatedMedical causes, such as a1-antitrypsin deficiency, Alagille’s

syndrome, cystic fibrosis and neonatal hepatitis, should be excluded.

Biliary USG: whether the gallbladder is atrophic, dilated or abnormal.

to exclude other etiologies like choledochal cyst Triangular cord sign: Presence of fibrous cone of bile

duct remnant at porta hepatis

Triangular Cord Sign

Triangular Cord Sign Micky Mouse Sign

Biliary Atresia- Investigations

Hepatobiliary isotope scan- HIDA scan: In biliary atresia, there is normal uptake by the liver and failure of secretion of the isotope, while in neonatal hepatitis there is poor liver uptake of isotope. Intestinal excretion of the isotope confirms patency of the extrahepatic bile ducts

Percutaneous liver biopsy: bile ductular proliferation; bile duct plugs; inflammatory cell infiltrate, hepatocyte giant cell transformation

Intraoperative cholangiography: this procedure definitively demonstrates anatomy and patency of the extrahepatic bile ducts

Investigations- HIDA Scan

Investigations- IOCIntra Operative Cholangiogram

Biliary Atresia- Treatment

Kasai’s portoenterostomy: Once biliary atresia is suspected, surgical intervention in the form of intraoperative cholangiogram and Kasai portoenterostomy is indicated.

This procedure is not usually curative, but ideally does buy time until the child can achieve growth and undergo liver transplantation

A considerable number of these patients, even if Kasai portoenterostomy has been successful, eventually undergo liver transplantation

Kasai’s Portoenterostomy

Kasai’s Portoenterostomy

Biliary Atresia- Postop care

In the immediate postoperative period, Methylprednisolone should be given for it’s anti-inflammatory and choleretic effects

Ursodeoxycholic acid has also been shown to enhance bile flow.In order to prevent cholangitis postoperatively, immediate

postop give IV antibiotics, then prophylaxis with trimethoprim–sulfamethoxazole has been used on a long-term basis.

Biliary Atresia- Outcome

Prognosis is good if operated before 2 months of age Risk factors for failure liver fibrosis &Post op cholangitis

episodes1/3rd of pts remain asymptomatic No transplant1/3 never have bile flow and require early transplant1/3 initially have good bile flow but subsequently develop

cirrhosisWithout surgery or liver transplant life span – 19 monthsDeath is due to liver failure, bleeding esophageal varices and

sepsis

Biliary Atresia - Mindmap

Differential Diagnosis

Infantile Surgical Jaundice- Algorithm

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