This document discusses obstructive jaundice and neonatal hyperbilirubinemia. Neonates are more prone to hyperbilirubinemia due to physiological factors such as greater red blood cell mass and shorter red blood cell lifespan. It describes physiological jaundice and criteria for identifying pathological jaundice. It also discusses conditions that can cause neonatal cholestasis such as biliary atresia and choledochal cyst, including their etiology, clinical presentation, investigations and management. Biliary atresia involves atresia of the extrahepatic bile ducts and has a poor prognosis if not treated early via surgery such as Kasai portoenterostomy. Choledochal cyst is a

1. Obstructive JaundiceObstructive Jaundice

2. Neonatal HyperbilirubenemiaNeonatal Hyperbilirubenemia
o Devastating consequences like acute bilirubinDevastating consequences like acute bilirubin
encephalopathyencephalopathy
o increased propensity in neonates due to theincreased propensity in neonates due to the
following physiological handicaps:following 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 toDefective uptake & conjugation of bilirubin due to
liver immaturityliver immaturity
 Decreased intestinal flora, therefore decreasedDecreased intestinal flora, therefore decreased
conversion to stercobilin.conversion to stercobilin.

3. 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

4. Maisels criteriaMaisels criteria
 jaundice in first 24 hrsjaundice in first 24 hrs
 Serum bilirubin increases by > 5mg% perSerum 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 1Clinical jaundice persisting more than 1
week in full term & 2 weeks in pretermsweek in full term & 2 weeks in preterms

5. Neonatal CholestasisNeonatal Cholestasis
 Increased conjugated bilirubin in theIncreased conjugated bilirubin in the
circulation.circulation.
Conjugated Hyperbilirubinemia (Direct Bili > 2mg% or
> 15% of total bilirubin
Obstructive disorders
Non-obstructive
disorders
1.Biliary atresia 1. Infective - TORCH
2.Choledochal Cyst 2. Idiopathic
3.Inspissated bile syndrome 3. Toxic
4.Tumor or band 4. Metabolic
5.Rotor & Dubin Johnson 5. Hypothyroidism
syndromes
6.Cystic Fibrosis
7.Alagille’s syndrome

6. Biliary AtresiaBiliary Atresia
o Atresia of extrahepatic bile ducts that occursAtresia of extrahepatic bile ducts that occurs
in newborns as a result of destructivein 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 fromExtrahepatic Biliary tree is derived from
Hepatic bud.Hepatic bud.
 The development of intrahepatic ducts isThe development of intrahepatic ducts is
intimately related to the branching pattern ofintimately related to the branching pattern of
portal vein radicles.portal vein radicles.

7.  Mesenchyme condenses around the portalMesenchyme condenses around the portal
vein radicles. Primitive hepatocytes form avein radicles. Primitive hepatocytes form a
sleeve around the PVR & associatedsleeve around the PVR & associated
mesenchyme. This sleeve is called ductalmesenchyme. This sleeve is called ductal
plate.plate.
 Portions of the sleeve are duplicated to formPortions of the sleeve are duplicated to form
small linear tubules which grow into thesmall linear tubules which grow into the
mesenchyme & differentiate into bile ducts.mesenchyme & differentiate into bile ducts.
 Subsequently remodeling into adult systemSubsequently remodeling into adult system
of adult system of tubular anastamosingof adult system of tubular anastamosing
bile ducts occurs.bile ducts occurs.

8. Defective remodeling results in weak or non-
communicating ducts with resultant bile leak
Ductal Plate malformation theory

9. PathologyPathology
 Widening of portal tracts with edema,Widening of portal tracts with edema,
proliferation of bile ductules, bile stasisproliferation of bile ductules, bile stasis
within canaliculi & hepatocytes,within canaliculi & hepatocytes,
Multinucleate giant cells.Multinucleate giant cells.
 Progressing disease leads to increasingProgressing disease leads to increasing
hepatocellular damage.hepatocellular damage.

10. Type 1: Atresia confined to CBD Type 2: Atresia of CBD & Common hepatic
duct with residual patency of Rt & Lt hepatic ducts
Type 3: Atresia of whole Extra Hepatic duct system

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

12. Clinical PresentationClinical Presentation
 Incidence: 1/10,000 live birthsIncidence: 1/10,000 live births
 First indication may be prolonged jaundiceFirst indication may be prolonged jaundice
following neonatal physiological jaundice.following neonatal physiological jaundice.
 Baby passes clay colored stools & darkBaby passes clay colored stools & dark
urine.urine.
 Infant usually thrives & feeds well duringInfant usually thrives & feeds well during
early stages. After 3 – 4th month theearly stages. After 3 – 4th month the
condition deteriorates.condition deteriorates.
 Hepatosplenomegaly may be seen. AscitesHepatosplenomegaly may be seen. Ascites
is a late developmentis a late development
 Vitamin K related Haemorrhaghic diathesisVitamin K related Haemorrhaghic diathesis
in the form of bruising or overt bleeding .in the form of bruising or overt bleeding .

13. InvestigationsInvestigations
 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 spectroscopyfor bile, stool spectroscopy
 USGUSG: Classical finding is absence of or: Classical finding is absence of or
shrunken gall bladdershrunken gall bladder

14.  Percutaneous liver biopsyPercutaneous liver biopsy is useful in earlyis useful in early
stages. In late stages there is significantstages. In late stages there is significant
hepatocyte damage & picture is difficult tohepatocyte 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 theIn normal system there is good uptake by the
liver, visualisation of gall bladder & EHDs andliver, visualisation of gall bladder & EHDs and
radioactivity in the small bowel s/o excretionradioactivity in the small bowel s/o excretion
into bowel.into bowel.

15. • In EHBA, uptake is relatively good in theIn EHBA, uptake is relatively good in the
early stages, there is no visualisation of GB &early stages, there is no visualisation of GB &
EHD & no radioactivity in the gut even afterEHD & no radioactivity in the gut even after
24 hrs.24 hrs.
 In other forms of neonatal cholestasis thereIn other forms of neonatal cholestasis there
is poor liver function even in neonatal period,is poor liver function even in neonatal period,
hence poor uptake & excretion.hence poor uptake & excretion.
 Per – op CholangiogramPer – op Cholangiogram is the definitiveis the definitive
imaging study.imaging study.

16. ManagementManagement
 Early surgery, before 8 weeks gives bestEarly surgery, before 8 weeks gives best
prognosis.prognosis.
 Type 1 has best prognosisType 1 has best prognosis
 In other types there is progressive liverIn other types there is progressive liver
damage even after early surgery and thereforedamage even after early surgery and therefore
it is a stop gap treatment till liver transplantit is a stop gap treatment till liver transplant
can be done.can be done.
 Preop preparation:Preop preparation:
- Adequate vitamin K- Adequate vitamin K

17. 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 anastamosisdile ducts into the intestine by anastamosis
roux-en-y loop of jejunum to segmentroux-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 segmentthe ducts proximal to the atretic segment
drain well.drain well.

18.  HepaticojejunostomyHepaticojejunostomy for type1for type1
atresia seen in <10%atresia seen in <10%

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

21. Post-op carePost-op care
 Supplement fat soluble vitaminsSupplement fat soluble vitamins
A,D,E,K.A,D,E,K.
 Prolonged antibioticsProlonged antibiotics
 Phenobarbitone, steroids & UDCAPhenobarbitone, steroids & UDCA
to potentiate hepatic function &to potentiate hepatic function &
secretionsecretion

22. ComplicationsComplications
 Bacterial cholangitisBacterial cholangitis
 Portal HypertensionPortal Hypertension
 Metabolic problemsMetabolic problems
 Hepatopulmonary syndrome.Hepatopulmonary syndrome.

23. Choledochal CystCholedochal Cyst
 Cystic dilatation of the biliary treeCystic dilatation of the biliary tree
 More common in females, orientalMore common in females, oriental
racesraces
 Incidence 1 in 1,00,000 to 1,50,000Incidence 1 in 1,00,000 to 1,50,000
live birthslive births

24. EtiologyEtiology
 Congenital weakness due to disorderlyCongenital weakness due to disorderly
recanalisationrecanalisation
 Abnormal innervation leading to functionalAbnormal innervation leading to functional
obstruction.obstruction.
 Actual anatomic obstruction at lower end ofActual anatomic obstruction at lower end of
CBD.CBD.
 Long common pancreaticobiliary channelLong common pancreaticobiliary channel
(>10mm) whereby the ducts unite well(>10mm) whereby the ducts unite well
outside the duodenal wall & are notoutside the duodenal wall & are not
surrounded by normal sphincteric mechanism.surrounded by normal sphincteric mechanism.
This results in reflux of pancreatic juice intoThis results in reflux of pancreatic juice into
CBD as excretion pressure of pancreatic ductCBD as excretion pressure of pancreatic duct
exceeds that of CBD.exceeds that of CBD.

26. 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 withinType III – Dilatation of terminal CBD within
duodenal wall - Choledochoceleduodenal wall - Choledochocele
 Type IV – Multiple cysts of the entireType IV – Multiple cysts of the entire
hepatobiliary tree or extrahepatic ductshepatobiliary tree or extrahepatic ducts
 Type V – Single or multiple intrahepatic ductType V – Single or multiple intrahepatic duct
cyst.cyst.

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

28. PathologyPathology
 Wall of choledochal cyst is thickened, 2 –Wall of choledochal cyst is thickened, 2 –
7 mm, composed of fibrous tissue with7 mm, composed of fibrous tissue with
occasional smooth muscle.occasional smooth muscle.
 Epithelium ulcerated, only patches ofEpithelium ulcerated, only patches of
viable epithelium may remain.viable epithelium may remain.
 Liver histology varies from mildLiver histology varies from mild
inflammatory infiltration of portal tractsinflammatory infiltration of portal tracts
to cirrhosis.to cirrhosis.

29. Clinical PresentationClinical 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 featureshepatomegaly & 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.

30. InvestigationsInvestigations
 Liver function testsLiver function tests – Conjugated– Conjugated
hyperbilirubenemia, elevated serum alkalinehyperbilirubenemia, 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 ofjunction & provides excellent visualisation of
biliary tree.biliary tree.
 Intraoperative cholangiogramIntraoperative cholangiogram - on table- on table
injection of dye into the cyst under C ARMinjection of dye into the cyst under C ARM
fluoroscopy.fluoroscopy.
 HIDA / BULLIDAHIDA / BULLIDA – shows filling defect in the– shows filling defect in the
liver followed by gradual accumulation ofliver followed by gradual accumulation of

31. ComplicationsComplications
 InfectionInfection
 RuptureRupture
 Pancreatic disease CholelithiasisPancreatic disease Cholelithiasis
 Progressive liver disease & portalProgressive liver disease & portal
hypertensionhypertension
 Malignancy – 14% after the age ofMalignancy – 14% after the age of
20yrs. Usually adenocarcinoma,20yrs. Usually adenocarcinoma,
cholangiocarcinomacholangiocarcinoma..

32. TreatmentTreatment
 Radical Cyst excision & reconstruction byRadical Cyst excision & reconstruction by
hepaticojejunostomy is the optimumhepaticojejunostomy is the optimum
treatmenttreatment