Laparoscopic vs open portoenterostomy in biliary atresia ...
Biliary atresia- Obstructive jaundice/ Pediatric surgery
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Transcript of Biliary atresia- Obstructive jaundice/ Pediatric surgery
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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