Obstructive jaundice in neonates can be caused by several conditions including biliary atresia, choledochal cysts, and inspissated bile. Biliary atresia, the most common cause, involves obstruction of the bile ducts inside or outside the liver. It is typically treated with the Kasai procedure to reconnect bile flow to the intestine or liver transplantation. Choledochal cysts are rare congenital cysts of the bile ducts that may cause pain, jaundice, and abdominal mass. Surgical excision and reconstruction is the primary treatment. Inspissated bile syndrome can also cause obstructive jaundice in newborns due to thickened bile but sometimes resolves spontaneously

1. Obstructive Jaundice in Neonate
Presenter:Dr. Shashi K. Singh
Moderater:Dr. R.P. Chuadhary,Dr. Puskar
pokharel, Dr. Ramana Raj kansakar

2. Jaundice
• Yellowish discoloration of the skin, mucous
membranes, and sclerae of the eyes
• Hyperbilirubinemia
• Deposition of bile salts in these tissues

3. • Jaundice in the first few weeks of life
categorised into
Hematologic,
Enzymatic/metabolic,
Infectious and
Obstructive

4. Biliary atresia
Choledocal cyst
Inspisseted bile
Obstructive jaundice

5. Post hepatic(Obstructive jaundice )
an interruption in the drainage of bile in
the biliary system.

6. • Neonatal cholestasis is defined as prolonged
elevation of serum levels of conjugated
bilirubin beyond the first 14 days of life.

7. 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

8. • Obstructive jaundice in infancy
surgical challenge
• Short time between the appearance of the
jaundice and the optimal time for surgical
intervention
between 4 and 6 weeks

9. Bile
Fluid made by the liver
Two main functions:
1. Carrying toxins and waste products out of the
body
2. Helping the body digest fats and absorb the
fat-soluble vitamins A, D, E, and K

10. • Bile becomes trapped, builds up, and damages
the liver. The damage leads to scarring, loss of
liver tissue, and cirrhosis
• Cirrhosis, portal hypertension, liver failure,
and death
• Deficiency of vitamin A,D,E,K – clotting factor
deficiency – bleeding disorder

11. • Biliary atresia: most common cause of
obstructive jaundice
requiring operation in children
• Choledochal cyst: 2nd most common cause

12. Biliary atresia
• Life-threatening condition
• Bile ducts inside or outside the liver do not
have normal openings

13. Incidence
• Rare
• 1:18,000 infants
• More common in
Females
Premature babies,
Children of Asian or African American
heritage

14. 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 correctable
type was reported for the first time in 1928
• In the late 1950s, Morio Kasai reported the
presence of patent microscopic biliary channels
at the porta hepatis in young infants with biliary
atresia- proposed kasai portoenterostomy

15. Causes
• Multiple causes: none proven yet
• Not an inherited disease
• Some are:
1. Viral or bacterial infection after birth
cytomegalovirus, reovirus, or rotavirus
2. Immune system problem
when the immune system attacks the liver
or bile ducts for unknown reasons
3. Genetic mutation
4. Problem during liver and bile duct
development in the womb

16. Biliary atresia – two types
1. Fetal(syndromic) : appears while the baby is in the
womb
• known as the embryonic type
• associated congenital anomalies such as an interrupted
inferior vena cava, preduodenal portal vein, intestinal
malrotation, situs inversus, cardiac defects, and
polysplenia.
• In this variety, which accounts for 10% to 20% of all
2.Perinatal(Nonsyndromic):
more common
become evident at 2 to 4 weeks after birth

17. Morphologic classification of biliary
atresia
• Type I:
occlusion of common bile duct;
• type IIa
obliteration of common hepatic duct;
• type Iib:
obliteration of common bile duct, hepatic and
cystic ducts, with cystic dilatation of ducts at the
porta hepatis and no gallbladder involvement;
• type III:
obliteration of common, hepatic, and cystic ducts
without anastomosable ducts at porta hepatis.

20. Symptoms/Signs
• Jaundice
• Dark urine
• Gray stools
From a lack of bilirubin reaching the intestines
• Slow weight gain and growth
• Hepatomegaly

21. • Routine Examinations
• Color of stool
• Consistency of the liver
• Conventional liver function tests, including
test for γ-glutamyl transpeptidase
• Coagulation times (PT, aPTT)

22. Special Examinations
Special biochemical studies
• Hepatitis A, B, C serologic studies
• TORCH titers
• α1-Antitrypsin level
• Serum lipoprotein-X
• Serum bile acid

23. Confirmation of patency of extrahepatic bile
ducts
• Duodenal fluid aspiration
• Ultrasonography
• Hepatobiliary scintigraphy
• Endoscopic retrograde
cholangiopancreatography
• Near-infrared reflectance spectroscopy

24. Other:
• Needle biopsy of the liver for histopathologic
studies
• Laparoscopy
• Surgical cholangiography

25. Treatment
• Biliary atresia is treated by
surgery- Kasai procedure or
a liver transplant
• Kasai opretaion- Named after the surgeon
Morio Kasai
• This procedure is most effective in infants
younger than 3 months old
• As they usually haven’t yet developed
permanent liver damage

26. • Surgeon removes the infant’s damaged bile
ducts and brings up a loop of intestine to
replace them.
• As a result, bile flows straight to the small
intestine

27. Kasai
operation
(Portoenterostomy)

28. Complications After
Portoenterostomy(Kasai operation)
• Cholangitis
• Portal hypertension
• Esophageal varices
• Hypersplenism
• Fat-soluble vitamin deficiency
Vitamin E-penpheral neuropathy
Vitamin D-rickets
Vitamin A- visual defects
Vitamin K-coagulation defects
• Zinc deficiency

29. Prognosis
• No bile drainage (10%)
• Bile drainage (90%)
1/3 Fail- severe liver disease
1/3 indeterminate- moderate liver disease
1/3 “Cured”- minimal liver disease

30. Liver Transplant
• Liver transplantation is the definitive
treatment for biliary atresia
• Survival rate after surgery has increased
dramatically in recent years

31. • Infants with fetal type of biliary atresia:
more likely to need a liver transplant
• For those children whose bile fails to drain or
children who have major/progressive
parenchymal damage, liver transplantation is
now a well accepted therapeutic option.

32. • Regimen of medications is used to prevent the
immune system from rejecting the new liver.
• Health care providers may also prescribe
blood pressure medications and antibiotics,
along with special diets and vitamin
supplements.

33. Complications of
Liver Transplantation
• Technical failure
• Hepatic artery thrombosis
• Biliary obstruction
• Rejection
• Infection
Bacterial
Viral
Fungal

34. CHOLEDOCHAL CYST

35. • A choledochal cyst is a rare congenital swelling
of the hepatic or bile duct .
• These cysts can be intrahepatic, meaning that
they occur in the part of the duct located
inside of the liver.
• They can also be extrahepatic, meaning part
of the bile duct that is located outside the
liver.

36. • First reported by Douglas in 1852
• Relatively rare
• Incidence in Western populations- 1 in 13,000
to 15,000 live births
• East- 1 per 1000 live births
• Etiology remains unknown
• Likely to be congenital

37. Pathologic features
• Frequently include an anomalous junction of
the pancreatic duct and CBD
(pancreaticobiliary malunion [PBMU])
• Intrahepatic bile duct dilatation with or
without downstream stenosis
• Varying degrees of hepatic fibrosis

39. Pathogenesis
• Congenital weakness of the bile duct wall, a
primary abnormality of proliferation during
embryologic ductal development, and
congenital obstruction have been postulated
• In 1969, the “long common channel theory”
was Proposed:
PBMU allows reflux of pancreatic enzymes
into the CBD, which leads to disruption of the
duct walls

40. • Pancreaticobiliary ductal junction has been
demonstrated to be outside the duodenal wall
before the eighth week of gestation and then
migrates normally toward the duodenal
lumen.
• Thus, PBMU may persist as a result of arrest in
this migration.

41. • PBMU(pancreaticobiliary malunion)and
congenital stenosis are the basic causative
factors of choledochal cyst

42. Todani Classification of Choledochal Cysts
• Type I:
Classic cyst type characterized by cystic dilatation of
the common bile duct; most common, comprising 50–
85% of all biliary cysts; subdivided into
IA -cystic
IB -fusiform
IC -saccular
• Type II:
Simple diverticulum of the extrahepatic biliary tree,
comprising less than 5% of all cysts; located proximal
to the duodenum
• Type III:
Cystic dilatation of the intraduodenal portion of the
extra hepatic common bile duct; also known as a
choledochocele; comprise approximately 5%

43. • Type IV:
Involve multiple cysts of the intrahepatic and
extrahepatic biliary tree
subdivided into
type IVA: Both intrahepatic and extrahepatic cysts
Second most common type 30–40%
Type IVB: multiple extrahepatic cysts without
intrahepatic involvement
• Type V :
Isolated intrahepatic biliary cystic disease
Known as Caroli's disease
Associated with periportal fibrosis or cirrhosis
Multilobar or confined to a single lobe

45. Presentation
• The classic triad
Pain, jaundice, and abdominal mass.
Conjugated bilirubin (80%),
Failure to thrive
• Intermittent jaundice and recurrent
cholangitis
• pancreatitis

46. Investigations
• Raised white blood cell count, (increased immature
neutrophils in patients with cholangitis).
• Abnormal LFTs - cholestasis.
• Serum amylase and lipase concentrations may be increased
in the presence of pancreatitis.
• Serum amylase concentrations also may be elevated in
biliary obstruction and cholangitis.
• Abdominal ultrasonography
• Technetium 99m Tc hepatobiliary iminodiacetic acid (HIDA)
scan is often used and is particularly useful for showing
continuity with bile ducts and diagnosis of cyst ruptur
• Abdominal CT scan and MRI help to delineate the anatomy
of the lesion and of the surrounding structures
• Percutaneous transhepatic cholangiography (PTC) or
endoscopic retrograde cholangiopancreatography (ERCP)

47. Treatment
• If a patient presents with pancreatitis or
cholangitis,
treated supportively prior to definitive operative
management
• Radical excision of the cyst with reconstruction of
the biliary tract using a Roux-en-Y loop of
jejunum.
• Complete resection of the cyst is important
because of the association with the development
of cholangiocarcinoma.

48. • Type I:
The goal is then to excise the intrapancreatic
portion of the cyst without injuring the
pancreatic duct or the long common channel.
The distal-most portion of the choledochal
cyst is encircled and transected as it enters the
pancreas

49. • Type II:
treated with simple cyst excision. After the
cyst has been exposed, the common bile duct
wall defect is closed transversely
Type III:
endoscopic sphincterotomy
resection is typically approached via a transverse
duodenotomy in the second or third portion
of the duodenum

50. • Type IVA and type IVB cysts are managed
similarly to type I cysts with regard to
extrahepatic biliary resection,
cholecystectomy, and biliary reconstruction
• Type V:
If unilateral: Lobar resection
if B/L : Roux-en-Y hepaticojejunostomy with
bilateral transhepatic Silastic stents may be
indicated to improve biliary drainage

52. Complication
• Ascending cholangitis
• Intrahepatic bile duct stones
• Intrapancreatic terminal choledochus calculi
• Pancreatic duct calculus
• Stones in the blind pouch of the end-to-side
Roux-en-Y hepaticojejunostomy
• Bowel obstruction
• Cholangiocarcinoma
• Liver dysfunction
• Pancreatitis

53. Inspissated Bile Syndrome
• Inspissated bile within the distal common bile
duct may cause obstructive jaundice in newborns
• Due to haemolysis, diuretic therapy, parenteral
nutrition, prematurity, or cystic fibrosis.
• Inspissated bile plug syndrome difficult to
distinguish from biliary atresia.
• In both conditions-
jaundice and acholic stools, conjugated
hyperbilirubinaemia, and no biliary excretion on a
radionuclide scan.
• USG reveals dilated proximal bile ducts and
inspissated bile.

54. Treatment
• Spontaneous resolution
• Treatment with ursodeoxycholic acid may help.
• More persistent obstruction can be cleared by
percutaneous, transhepatic irrigation of the bile
ducts, ERCP and retrograde irrigation, or
cholecystectomy and bile duct irrigation.
• Occasionally,
transduodenal sphincteroplasty required
to remove an impacted mass of material or
stones.

55. Biliary Hypoplasia
• Exceptionally small but grossly visible and
radiographically patent extrahepatic biliary
duct system
• Neonatal hepatitis,α1-antitrypsin deficiency,
intrahepatic biliary atresia, Alagille syndrome,
and
• Non cannot be improved by surgical
maneuvers. The prognosis is highly variable
and depends on the primary disease.

56. Alagille syndrome
• Genetic disorder
• Inherited in an autosomal dominant pattern, and
its estimated prevalence is 1 in every 100,000 live
births
• Typical features: peculiar facies with a high,
prominent forehead and deep-set eyes, chronic
cholestasis, butterfly-like vertebral arch defects,
and heart disease (usually peripheral pulmonary
stenosis)
• respond to supportive measures such as
treatment with ursodeoxycholic acid and
phenobarbital.
• May need liver transplantation as well

58. Summary
• Jaundice beyod the age of 14 days need
meticulous investigation and obstructive
causes to be ruled out.
• Obstructive jaundice, timely intervention can
save a great hazard of liver failure and need of
liver transplantation.

59. THANKYOU