Post hepatic(Obstructive jaundice )an interruption in the drainage of bile inthe biliary system.
• Neonatal cholestasis is defined as prolongedelevation of serum levels of conjugatedbilirubin beyond the first 14 days of life.
Obstructive cholestasis• Biliary atresia• Choledochal cyst• Gallstones or biliary sludge• Alagille syndrome• Inspissated bile• Cystic fibrosis• Neonatal sclerosing cholangitis• Congenital hepatic fibrosis/Caroli’s disease• Intrahepatic hypoplasia• spontaneous perforation of the bile duct
• Obstructive jaundice in infancysurgical challenge• Short time between the appearance of thejaundice and the optimal time for surgicalinterventionbetween 4 and 6 weeks
BileFluid made by the liverTwo main functions:1. Carrying toxins and waste products out of thebody2. Helping the body digest fats and absorb thefat-soluble vitamins A, D, E, and K
• Bile becomes trapped, builds up, and damagesthe liver. The damage leads to scarring, loss ofliver tissue, and cirrhosis• Cirrhosis, portal hypertension, liver failure,and death• Deficiency of vitamin A,D,E,K – clotting factordeficiency – bleeding disorder
• Biliary atresia: most common cause ofobstructive jaundicerequiring operation in children• Choledochal cyst: 2nd most common cause
Biliary atresia• Life-threatening condition• Bile ducts inside or outside the liver do nothave normal openings
Incidence• Rare• 1:18,000 infants• More common inFemalesPremature babies,Children of Asian or African Americanheritage
History• 1st reported in Edinburgh Medical Journal in 1891• In 1916, the concepts of “correctable” and“noncorrectable” types of disease introduced• Successful surgical treatment for the correctabletype was reported for the first time in 1928• In the late 1950s, Morio Kasai reported thepresence of patent microscopic biliary channelsat the porta hepatis in young infants with biliaryatresia- proposed kasai portoenterostomy
Causes• Multiple causes: none proven yet• Not an inherited disease• Some are:1. Viral or bacterial infection after birthcytomegalovirus, reovirus, or rotavirus2. Immune system problemwhen the immune system attacks the liveror bile ducts for unknown reasons3. Genetic mutation4. Problem during liver and bile ductdevelopment in the womb
Biliary atresia – two types1. Fetal(syndromic) : appears while the baby is in thewomb• known as the embryonic type• associated congenital anomalies such as an interruptedinferior vena cava, preduodenal portal vein, intestinalmalrotation, situs inversus, cardiac defects, andpolysplenia.• In this variety, which accounts for 10% to 20% of all2.Perinatal(Nonsyndromic):more commonbecome evident at 2 to 4 weeks after birth
Morphologic classification of biliaryatresia• Type I:occlusion of common bile duct;• type IIaobliteration of common hepatic duct;• type Iib:obliteration of common bile duct, hepatic andcystic ducts, with cystic dilatation of ducts at theporta hepatis and no gallbladder involvement;• type III:obliteration of common, hepatic, and cystic ductswithout anastomosable ducts at porta hepatis.
Symptoms/Signs• Jaundice• Dark urine• Gray stoolsFrom a lack of bilirubin reaching the intestines• Slow weight gain and growth• Hepatomegaly
• Routine Examinations• Color of stool• Consistency of the liver• Conventional liver function tests, includingtest for γ-glutamyl transpeptidase• Coagulation times (PT, aPTT)
Special ExaminationsSpecial biochemical studies• Hepatitis A, B, C serologic studies• TORCH titers• α1-Antitrypsin level• Serum lipoprotein-X• Serum bile acid
Confirmation of patency of extrahepatic bileducts• Duodenal fluid aspiration• Ultrasonography• Hepatobiliary scintigraphy• Endoscopic retrogradecholangiopancreatography• Near-infrared reflectance spectroscopy
Other:• Needle biopsy of the liver for histopathologicstudies• Laparoscopy• Surgical cholangiography
Treatment• Biliary atresia is treated bysurgery- Kasai procedure ora liver transplant• Kasai opretaion- Named after the surgeonMorio Kasai• This procedure is most effective in infantsyounger than 3 months old• As they usually haven’t yet developedpermanent liver damage
• Surgeon removes the infant’s damaged bileducts and brings up a loop of intestine toreplace them.• As a result, bile flows straight to the smallintestine
Prognosis• No bile drainage (10%)• Bile drainage (90%)1/3 Fail- severe liver disease1/3 indeterminate- moderate liver disease1/3 “Cured”- minimal liver disease
Liver Transplant• Liver transplantation is the definitivetreatment for biliary atresia• Survival rate after surgery has increaseddramatically in recent years
• Infants with fetal type of biliary atresia:more likely to need a liver transplant• For those children whose bile fails to drain orchildren who have major/progressiveparenchymal damage, liver transplantation isnow a well accepted therapeutic option.
• Regimen of medications is used to prevent theimmune system from rejecting the new liver.• Health care providers may also prescribeblood pressure medications and antibiotics,along with special diets and vitaminsupplements.
• A choledochal cyst is a rare congenital swellingof the hepatic or bile duct .• These cysts can be intrahepatic, meaning thatthey occur in the part of the duct locatedinside of the liver.• They can also be extrahepatic, meaning partof the bile duct that is located outside theliver.
• First reported by Douglas in 1852• Relatively rare• Incidence in Western populations- 1 in 13,000to 15,000 live births• East- 1 per 1000 live births• Etiology remains unknown• Likely to be congenital
Pathologic features• Frequently include an anomalous junction ofthe pancreatic duct and CBD(pancreaticobiliary malunion [PBMU])• Intrahepatic bile duct dilatation with orwithout downstream stenosis• Varying degrees of hepatic fibrosis
Pathogenesis• Congenital weakness of the bile duct wall, aprimary abnormality of proliferation duringembryologic ductal development, andcongenital obstruction have been postulated• In 1969, the “long common channel theory”was Proposed:PBMU allows reflux of pancreatic enzymesinto the CBD, which leads to disruption of theduct walls
• Pancreaticobiliary ductal junction has beendemonstrated to be outside the duodenal wallbefore the eighth week of gestation and thenmigrates normally toward the duodenallumen.• Thus, PBMU may persist as a result of arrest inthis migration.
• PBMU(pancreaticobiliary malunion)andcongenital stenosis are the basic causativefactors of choledochal cyst
Todani Classification of Choledochal Cysts• Type I:Classic cyst type characterized by cystic dilatation ofthe common bile duct; most common, comprising 50–85% of all biliary cysts; subdivided intoIA -cysticIB -fusiformIC -saccular• Type II:Simple diverticulum of the extrahepatic biliary tree,comprising less than 5% of all cysts; located proximalto the duodenum• Type III:Cystic dilatation of the intraduodenal portion of theextra hepatic common bile duct; also known as acholedochocele; comprise approximately 5%
• Type IV:Involve multiple cysts of the intrahepatic andextrahepatic biliary treesubdivided intotype IVA: Both intrahepatic and extrahepatic cystsSecond most common type 30–40%Type IVB: multiple extrahepatic cysts withoutintrahepatic involvement• Type V :Isolated intrahepatic biliary cystic diseaseKnown as Carolis diseaseAssociated with periportal fibrosis or cirrhosisMultilobar or confined to a single lobe
Presentation• The classic triadPain, jaundice, and abdominal mass.Conjugated bilirubin (80%),Failure to thrive• Intermittent jaundice and recurrentcholangitis• pancreatitis
Investigations• Raised white blood cell count, (increased immatureneutrophils in patients with cholangitis).• Abnormal LFTs - cholestasis.• Serum amylase and lipase concentrations may be increasedin the presence of pancreatitis.• Serum amylase concentrations also may be elevated inbiliary obstruction and cholangitis.• Abdominal ultrasonography• Technetium 99m Tc hepatobiliary iminodiacetic acid (HIDA)scan is often used and is particularly useful for showingcontinuity with bile ducts and diagnosis of cyst ruptur• Abdominal CT scan and MRI help to delineate the anatomyof the lesion and of the surrounding structures• Percutaneous transhepatic cholangiography (PTC) orendoscopic retrograde cholangiopancreatography (ERCP)
Treatment• If a patient presents with pancreatitis orcholangitis,treated supportively prior to definitive operativemanagement• Radical excision of the cyst with reconstruction ofthe biliary tract using a Roux-en-Y loop ofjejunum.• Complete resection of the cyst is importantbecause of the association with the developmentof cholangiocarcinoma.
• Type I:The goal is then to excise the intrapancreaticportion of the cyst without injuring thepancreatic duct or the long common channel.The distal-most portion of the choledochalcyst is encircled and transected as it enters thepancreas
• Type II:treated with simple cyst excision. After thecyst has been exposed, the common bile ductwall defect is closed transverselyType III:endoscopic sphincterotomyresection is typically approached via a transverseduodenotomy in the second or third portionof the duodenum
• Type IVA and type IVB cysts are managedsimilarly to type I cysts with regard toextrahepatic biliary resection,cholecystectomy, and biliary reconstruction• Type V:If unilateral: Lobar resectionif B/L : Roux-en-Y hepaticojejunostomy withbilateral transhepatic Silastic stents may beindicated to improve biliary drainage
Complication• Ascending cholangitis• Intrahepatic bile duct stones• Intrapancreatic terminal choledochus calculi• Pancreatic duct calculus• Stones in the blind pouch of the end-to-sideRoux-en-Y hepaticojejunostomy• Bowel obstruction• Cholangiocarcinoma• Liver dysfunction• Pancreatitis
Inspissated Bile Syndrome• Inspissated bile within the distal common bileduct may cause obstructive jaundice in newborns• Due to haemolysis, diuretic therapy, parenteralnutrition, prematurity, or cystic fibrosis.• Inspissated bile plug syndrome difficult todistinguish from biliary atresia.• In both conditions-jaundice and acholic stools, conjugatedhyperbilirubinaemia, and no biliary excretion on aradionuclide scan.• USG reveals dilated proximal bile ducts andinspissated bile.
Treatment• Spontaneous resolution• Treatment with ursodeoxycholic acid may help.• More persistent obstruction can be cleared bypercutaneous, transhepatic irrigation of the bileducts, ERCP and retrograde irrigation, orcholecystectomy and bile duct irrigation.• Occasionally,transduodenal sphincteroplasty requiredto remove an impacted mass of material orstones.
Biliary Hypoplasia• Exceptionally small but grossly visible andradiographically patent extrahepatic biliaryduct system• Neonatal hepatitis,α1-antitrypsin deficiency,intrahepatic biliary atresia, Alagille syndrome,and• Non cannot be improved by surgicalmaneuvers. The prognosis is highly variableand depends on the primary disease.
Alagille syndrome• Genetic disorder• Inherited in an autosomal dominant pattern, andits estimated prevalence is 1 in every 100,000 livebirths• Typical features: peculiar facies with a high,prominent forehead and deep-set eyes, chroniccholestasis, butterfly-like vertebral arch defects,and heart disease (usually peripheral pulmonarystenosis)• respond to supportive measures such astreatment with ursodeoxycholic acid andphenobarbital.• May need liver transplantation as well
Summary• Jaundice beyod the age of 14 days needmeticulous investigation and obstructivecauses to be ruled out.• Obstructive jaundice, timely intervention cansave a great hazard of liver failure and need ofliver transplantation.