extra hepatic biliary atresia

1. Extra Hepatic Biliary Atresia
Prepared by: V. Pravallika
M.Sc (Nursing)

2. Biliary atresia is obliteration or hypoplasia of one or more
components of the bile ducts due to arrested development in fetal
life resulting in persistent jaundice, liver damage, ranging from
biliary cirrhosis and progressive portal hypertension with
splenomegaly

3. Usually there is absence or atresia of extra hepatic ducts
and the common bile duct. In some instances. There is also
absence of intrahepatic ducts. Gallbladder may be absence
or distended with bile if the obstruction is distal

4. Types

5. - Type I: Atresia of common bile duct
- Type II: Atresia of common hepatic duct
- Type III: Atresia of porta hepatis
- Type IV: No patent bile ducts at porta hepatis

6. causes

7. - Developmental aberration of bile duct system in early
period of gestation or
- May be related to autoimmune reaction, or
intra uterine viral infection or
ischemia

8. Clinical manifestations

9. - Obstructive jaundice with pale putty like clay colored stools due
to absence of bile pigment in stools
- jaundice is usually not present at birth but appear within 2 to 3
weeks of life.
- The skin color deepens and becomes bronze olive green.
- urine becomes dark in color due to heavily excretion of bile
pigments
- the liver is enlarged due to biliary obstruction

10. - Cirrhosis and portal hypertension
- Enlargement of spleen
IN ADVANCED CASES
- hepatic failure
- bleeding disorders
- oesophageal varices
- generalized cachexia due to nutritional failure are found

11. Diagnosis

12. - Operated cholangiogram (before 8wks of age)
- USG
- Scanning and liver biopsy
- delay in corrective surgery beyond 3 months of age complicates the
problem and liver biopsy at that time may show intracellular and
intracanalicular cholestasis, inflammatory cell infiltration, intestinal fibrosis,
degeneration of proliferation of ducts and even giant cell formation

13. Management

14. - Surgical exploration and correction of the defect, within one or two
months of age
- Symptomatic treatment with vitamin ‘K’,
vitamin ‘D’, antihistaminics, antibiotics, diuretics may be needed in
inoperable cases
- Kesai heaptic portoenterostomy is performed as surgical interventions in
selected cases for extrahepatic biliary atresia
- in intrahepatic biliary atresia, cholysteramine may be useful to reduce
bilirubin level and itching

15. Prognosis

16. Prognosis is poor in untreated and inoperable cases, average
period of survival being 1.5 to 2 years. Survival period is longer in
intrahepatic type rather than extrahepatic type. With early
surgery, 40 to 50 percent long-term survival is reported in case of
EHBA