Obstructive Jaundice Obstructive Jaundice

Neonatal HyperbilirubenemiaNeonatal Hyperbilirubenemiao Devastating consequences like acute bilirubin Devastating consequences like acute bilirubin

encephalopathyencephalopathy

o increased propensity in neonates due to the following increased propensity in neonates due to the following physiological handicaps:physiological handicaps:

Greater red cell mass per kg as compared to adultsGreater red cell mass per kg as compared to adults

Shorter life span of RBCs ( 80-90 days)Shorter life span of RBCs ( 80-90 days)

Increased destruction of fetal HbIncreased destruction of fetal Hb

Defective uptake & conjugation of bilirubin due to Defective uptake & conjugation of bilirubin due to liver immaturityliver immaturity

Decreased intestinal flora, therefore decreased Decreased intestinal flora, therefore decreased conversion to stercobilin. conversion to stercobilin.

Physiological JaundicePhysiological Jaundice

It is unconjugated hyperbilirubinemia.It is unconjugated hyperbilirubinemia.

Appears between 30-72 hrs after birthAppears between 30-72 hrs after birth

Maximum intensity by 4/5th dayMaximum intensity by 4/5th day

Total bilirubin not more than 15 mg%Total bilirubin not more than 15 mg%

Disappears by 7-10 daysDisappears by 7-10 days

Maisels criteria Maisels criteria jaundice in first 24 hrsjaundice in first 24 hrs

Serum bilirubin increases by > 5mg% per Serum bilirubin increases by > 5mg% per dayday

Total bilirubin exceeds 15 mg%Total bilirubin exceeds 15 mg%

Direct bilirubin > 2 mg%Direct bilirubin > 2 mg%

Clinical jaundice persisting more than 1 Clinical jaundice persisting more than 1 week in full term & 2 weeks in pretermsweek in full term & 2 weeks in preterms

Neonatal CholestasisNeonatal Cholestasis Increased conjugated bilirubin in the Increased conjugated bilirubin in the

circulation.circulation.Conjugated Hyperbilirubinemia (Direct Bili > 2mg% or > 15% of total bilirubin

Obstructive disordersNon-obstructive

disorders

1.Biliary atresia 1. Infective - TORCH2.Choledochal Cyst 2. Idiopathic 3.Inspissated bile syndrome 3. Toxic4.Tumor or band 4. Metabolic5.Rotor & Dubin Johnson 5. Hypothyroidism syndromes6.Cystic Fibrosis7.Alagille’s syndrome

Biliary Atresia Biliary Atresia o Atresia of extrahepatic bile ducts that occurs Atresia of extrahepatic bile ducts that occurs

in newborns as a result of destructive in newborns as a result of destructive inflammatory process of unknown etiology.inflammatory process of unknown etiology.

o Embryological factors:Embryological factors: Extrahepatic Biliary tree is derived from Extrahepatic Biliary tree is derived from

Hepatic bud.Hepatic bud.

The development of intrahepatic ducts is The development of intrahepatic ducts is intimately related to the branching pattern of intimately related to the branching pattern of portal vein radicles. portal vein radicles.

Mesenchyme condenses around the portal Mesenchyme condenses around the portal vein radicles. Primitive hepatocytes form a vein radicles. Primitive hepatocytes form a sleeve around the PVR & associated sleeve around the PVR & associated mesenchyme. This sleeve is called ductal mesenchyme. This sleeve is called ductal plate. plate.

Portions of the sleeve are duplicated to form Portions of the sleeve are duplicated to form small linear tubules which grow into the small linear tubules which grow into the mesenchyme & differentiate into bile ducts.mesenchyme & differentiate into bile ducts.

Subsequently remodeling into adult system Subsequently remodeling into adult system

of adult system of tubular anastamosing of adult system of tubular anastamosing bile ducts occurs.bile ducts occurs.

Defective remodeling results in weak or non-communicating ducts with resultant bile leak

Ductal Plate malformation theory

Pathology Pathology

Widening of portal tracts with edema, Widening of portal tracts with edema, proliferation of bile ductules, bile stasis proliferation of bile ductules, bile stasis within canaliculi & hepatocytes, within canaliculi & hepatocytes, Multinucleate giant cells.Multinucleate giant cells.

Progressing disease leads to increasing Progressing disease leads to increasing hepatocellular damage.hepatocellular damage.

Type 1: Atresia confined to CBD Type 2: Atresia of CBD & Common hepaticduct with residual patency of Rt & Lt hepatic ducts

Type 3: Atresia of whole Extra Hepatic duct system

Prognosis is poor except in type 1 Prognosis is poor except in type 1 which accounts for less than 10% which accounts for less than 10% cases. This is because the larger cases. This is because the larger intrahepatic ducts are abnormal, there intrahepatic ducts are abnormal, there is extensive ductal plate malformation, is extensive ductal plate malformation, a progressive cholangiopathic process a progressive cholangiopathic process and residual bile duct obstruction which and residual bile duct obstruction which all lead to accelerated cirrhosis.all lead to accelerated cirrhosis.

Clinical Presentation Clinical Presentation Incidence: 1/10,000 live birthsIncidence: 1/10,000 live births First indication may be prolonged jaundice First indication may be prolonged jaundice

following neonatal physiological jaundice. following neonatal physiological jaundice. Baby passes clay colored stools & dark Baby passes clay colored stools & dark

urine. urine. Infant usually thrives & feeds well during Infant usually thrives & feeds well during

early stages. After 3 – 4th month the early stages. After 3 – 4th month the condition deteriorates. condition deteriorates.

Hepatosplenomegaly may be seen. Ascites Hepatosplenomegaly may be seen. Ascites is a late developmentis a late development

Vitamin K related Haemorrhaghic diathesis Vitamin K related Haemorrhaghic diathesis in the form of bruising or overt bleeding .in the form of bruising or overt bleeding .

Investigations Investigations Liver function tests.Liver function tests.

Urine analysisUrine analysis for bile salts/pigments. for bile salts/pigments.

Hematological testsHematological tests to exclude infectious & to exclude infectious & metabolic causes of neonatal cholestasis.metabolic causes of neonatal cholestasis.

Coagulation profileCoagulation profile..

Duodenal aspirateDuodenal aspirate for bile, stool spectroscopy for bile, stool spectroscopy

USGUSG: Classical finding is absence of or : Classical finding is absence of or shrunken gall bladdershrunken gall bladder

Percutaneous liver biopsyPercutaneous liver biopsy is useful in early is useful in early stages. In late stages there is significant stages. In late stages there is significant hepatocyte damage & picture is difficult to hepatocyte damage & picture is difficult to distinguish from other forms of liver damage.distinguish from other forms of liver damage.

Radio-isotope hepatobiliary scans(HIDARadio-isotope hepatobiliary scans(HIDA / / BULLIDA) using technetium labelled agents:BULLIDA) using technetium labelled agents:

In normal system there is good uptake by the In normal system there is good uptake by the liver, visualisation of gall bladder & EHDs and liver, visualisation of gall bladder & EHDs and radioactivity in the small bowel s/o excretion radioactivity in the small bowel s/o excretion into bowel.into bowel.

• In EHBA, uptake is relatively good in the early In EHBA, uptake is relatively good in the early stages, there is no visualisation of GB & EHD stages, there is no visualisation of GB & EHD & no radioactivity in the gut even after 24 & no radioactivity in the gut even after 24 hrs. hrs.

In other forms of neonatal cholestasis there is In other forms of neonatal cholestasis there is poor liver function even in neonatal period, poor liver function even in neonatal period, hence poor uptake & excretion.hence poor uptake & excretion.

Per – op CholangiogramPer – op Cholangiogram is the definitive is the definitive imaging study.imaging study.

Management Management Early surgery, before 8 weeks gives best Early surgery, before 8 weeks gives best

prognosis. prognosis.

Type 1 has best prognosisType 1 has best prognosis

In other types there is progressive liver damage In other types there is progressive liver damage even after early surgery and therefore it is a stop even after early surgery and therefore it is a stop gap treatment till liver transplant can be done.gap treatment till liver transplant can be done.

Preop preparation:Preop preparation:

- Adequate vitamin K- Adequate vitamin K

- Bowel preparation - Bowel preparation

SurgerySurgery Principle of surgery: Principle of surgery: - To confirm with per-op cholangiogram- To confirm with per-op cholangiogram

- To excise the atretic segment - To excise the atretic segment

- To obtain drainage of bile from intrahepatic - To obtain drainage of bile from intrahepatic dile ducts into the intestine by anastamosis dile ducts into the intestine by anastamosis roux-en-y loop of jejunum to segment roux-en-y loop of jejunum to segment immediately proximal to the atretic segment.immediately proximal to the atretic segment.

- For success of surgery it is essential the - For success of surgery it is essential the

the ducts proximal to the atretic segment the ducts proximal to the atretic segment drain well.drain well.

HepaticojejunostomyHepaticojejunostomy for type1 for type1 atresia seen in <10%atresia seen in <10%

Kasai PortoenterostomyKasai Portoenterostomy in > 90% in > 90% cases.cases.

Post-op care Post-op care

Supplement fat soluble vitamins Supplement fat soluble vitamins A,D,E,K.A,D,E,K.

Prolonged antibioticsProlonged antibiotics

Phenobarbitone, steroids & UDCA Phenobarbitone, steroids & UDCA to potentiate hepatic function & to potentiate hepatic function & secretionsecretion

ComplicationsComplications

Bacterial cholangitisBacterial cholangitis

Portal HypertensionPortal Hypertension

Metabolic problemsMetabolic problems

Hepatopulmonary syndrome.Hepatopulmonary syndrome.

Choledochal CystCholedochal Cyst

Cystic dilatation of the biliary treeCystic dilatation of the biliary tree

More common in females, oriental More common in females, oriental racesraces

Incidence 1 in 1,00,000 to 1,50,000 Incidence 1 in 1,00,000 to 1,50,000 live birthslive births

Etiology Etiology Congenital weakness due to disorderly Congenital weakness due to disorderly

recanalisationrecanalisation Abnormal innervation leading to functional Abnormal innervation leading to functional

obstruction.obstruction. Actual anatomic obstruction at lower end of Actual anatomic obstruction at lower end of

CBD.CBD. Long common pancreaticobiliary channel Long common pancreaticobiliary channel

(>10mm) whereby the ducts unite well outside (>10mm) whereby the ducts unite well outside the duodenal wall & are not surrounded by the duodenal wall & are not surrounded by normal sphincteric mechanism. This results in normal sphincteric mechanism. This results in reflux of pancreatic juice into CBD as excretion reflux of pancreatic juice into CBD as excretion pressure of pancreatic duct exceeds that of pressure of pancreatic duct exceeds that of CBD.CBD.

ClassificationClassification

Alonso-LejAlonso-Lej Type I – Fusiform dilatation of CBDType I – Fusiform dilatation of CBD

Type II – DiverticulumType II – Diverticulum

Type III – Dilatation of terminal CBD within Type III – Dilatation of terminal CBD within duodenal wall - Choledochoceleduodenal wall - Choledochocele

Type IV – Multiple cysts of the entire Type IV – Multiple cysts of the entire hepatobiliary tree or extrahepatic ductshepatobiliary tree or extrahepatic ducts

Type V – Single or multiple intrahepatic duct Type V – Single or multiple intrahepatic duct cyst.cyst.

Most commonly the dilatation of CBD starts just above the Most commonly the dilatation of CBD starts just above the duodenum & ends just below the bifurcation of the duodenum & ends just below the bifurcation of the common hepatic duct. Cystic duct usually enters common hepatic duct. Cystic duct usually enters Choledochal cyst. GB usually normal or slightly dilatedCholedochal cyst. GB usually normal or slightly dilated

Pathology Pathology

Wall of choledochal cyst is thickened, 2 – Wall of choledochal cyst is thickened, 2 – 7 mm, composed of fibrous tissue with 7 mm, composed of fibrous tissue with occasional smooth muscle.occasional smooth muscle.

Epithelium ulcerated, only patches of Epithelium ulcerated, only patches of viable epithelium may remain.viable epithelium may remain.

Liver histology varies from mild Liver histology varies from mild inflammatory infiltration of portal tracts inflammatory infiltration of portal tracts to cirrhosis.to cirrhosis.

Clinical Presentation Clinical Presentation Infantile form: Infantile form: - 1–3 mnths of age - 1–3 mnths of age - obstructive jaundice, acholic stools, - obstructive jaundice, acholic stools,

hepatomegaly & clinical features hepatomegaly & clinical features indistinguishable from biliary atresia.indistinguishable from biliary atresia.

Adult form: Adult form: - after 2 yrs of age. - after 2 yrs of age. - Classical triad of abdominal pain, - Classical triad of abdominal pain,

abdominal mass & jaundice is seen. abdominal mass & jaundice is seen. Jaundice is intermittent.Jaundice is intermittent.

InvestigationsInvestigations Liver function testsLiver function tests – Conjugated – Conjugated

hyperbilirubenemia, elevated serum alkaline hyperbilirubenemia, elevated serum alkaline phosphatase.phosphatase.

USGUSG – size, contour, position of cyst, hepatic – size, contour, position of cyst, hepatic echotexture, proximal ducts, pancreatitis, echotexture, proximal ducts, pancreatitis, ascites, portal hypertensionascites, portal hypertension

ERCP/MRCPERCP/MRCP – best visualises pancreatobiliary – best visualises pancreatobiliary junction & provides excellent visualisation of junction & provides excellent visualisation of biliary tree.biliary tree.

Intraoperative cholangiogramIntraoperative cholangiogram - on table injection - on table injection of dye into the cyst under C ARM fluoroscopy.of dye into the cyst under C ARM fluoroscopy.

HIDA / BULLIDAHIDA / BULLIDA – shows filling defect in the liver – shows filling defect in the liver followed by gradual accumulation of tracer in the followed by gradual accumulation of tracer in the cyst.cyst.

ComplicationsComplications

InfectionInfection RuptureRupture Pancreatic disease CholelithiasisPancreatic disease Cholelithiasis Progressive liver disease & portal Progressive liver disease & portal

hypertensionhypertension Malignancy – 14% after the age of Malignancy – 14% after the age of

20yrs. Usually adenocarcinoma, 20yrs. Usually adenocarcinoma, cholangiocarcinomacholangiocarcinoma..

TreatmentTreatment Radical Cyst excision & reconstruction by Radical Cyst excision & reconstruction by

hepaticojejunostomy is the optimum hepaticojejunostomy is the optimum treatmenttreatment