This document provides information on obstructive jaundice in infants. It discusses the various causes of obstructive jaundice including biliary atresia, choledochal cysts, and inspissated bile. Biliary atresia is the most common cause requiring surgery and involves blockage of the bile ducts. It is typically treated with the Kasai procedure to reconnect bile flow or liver transplantation. Choledochal cysts are rare congenital cysts of the bile duct that can cause obstruction. Inspissated bile occurs when thickened bile blocks the ducts in newborns. The document outlines presentations, investigations, and treatments for different types of obstructive jaundice in infants.

1. Presenter: Dr. Pashi V. M
Registrar peadiatrics surgery

2.  Yellowish discoloration of the skin, mucous
membranes, and sclerae of the eyes
 Hyperbilirubinemia
 Deposition of bile salts in these tissues
 Causes of jaundice can be classified into pre-
hepatic, hepatic or post hepatic.

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

5. 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

6. • 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

7. Biliary atresia
Choledocal cyst
Inspisseted bile
Obstructive jaundice

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

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

11. • 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

12.  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

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

14.  Life-threatening condition
 Bile ducts inside or outside the liver do not
have normal openings

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

16. • Multiple causes: none proven yet
• Not an inherited disease
• Some are:
1. Viral or bacterial infection after birth
cytomegalovirus, retrovirus, 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

17. 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

18. • 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.  The biliary system originates from the hepatic
diverticulum of the foregut at 4 weeks’
gestation.
 This differentiates into cranial and caudal
components, which give rise to the
intrahepatic and extrahepatic bile ducts,
respectively.

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

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

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

25. The goals of imaging are:
 To confirm the presence of an extra-hepatic
obstruction
 To determine the level of the obstruction
 To identify the specific cause of the
obstruction
 To provide complementary information
relating to the underlying diagnosis

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

27. Other:
 Needle biopsy of the liver for histopathologic
studies
 Laparoscopy
 Surgical cholangiography

28.  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

29.  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

30. Kasai
operation
(Portoenterostomy)

31. • 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

32.  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

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

34. • Technical failure
• Hepatic artery thrombosis
• Biliary obstruction
• Rejection
• Infection
Bacterial
Viral
Fungal

35. CHOLEDOCHAL CYST

36.  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.

37.  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. • 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. • 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 -saccular
IC -fusiform
• 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.  The classic triad
Pain, jaundice, and abdominal mass.
Conjugated bilirubin (80%),
Failure to thrive
 Intermittent jaundice and recurrent
cholangitis
 pancreatitis

46. • 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 rupture
• 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. • 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.

49. • 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

50. • 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.

51. • 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.

52. • 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

53.  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.

54. The end
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