EXTRAHEPATIC BILIARY ATRESIA

Aetiology and physiology

1-Atresia is present in approximately 1 per 12 000 live births,and affects males
and females equally.
2-The extrahepatic bile ducts are progressively destroyed by an inflammatory
process which starts around the time of birth.
3-The aetiology is unclear.Intrahepatic changes also occur and eventually
result in biliary cirrhosis and portal hypertension.
4-Untreated, death from the consequences of liver failure occurs before the
age of three years.

5-The inflammatory destruction of the bile ducts has been classified into three
main types;-

• type I: atresia restricted to the common bile duct;

• type II: atresia of the common hepatic duct;
• type III: atresia of the right and left hepatic ducts
.
Clinical features
About one-third of patients are jaundiced at birth. In all,however,
jaundice is present by the end of the first week and deepens
progressively. Liver function tests show an obstructive pattern with
elevated bilirubin and alkaline phosphatase.The meconium may be a
little bile stained, but later the stools are pale and the urine is dark.
Prolonged steatorrhoea gives rise to osteomalacia (biliary rickets).
Pruritus is severe. Clubbing and skin xanthomas, probably related to a
raised serum cholesterol,may be present.
Differential diagnosis
This includes any form of jaundice in a neonate giving a cholestatic
picture. Examples are;
1-alpha -antitrypsin deficiency,
1

2-cholestasis associated with intravenous feeding,

3-choledochal cyst
4-inspissated bile syndrome.
5-Neonatal hepatitis is the most difficult to differentiate.Both
extrahepatic biliary atresia and neonatal hepatitis are associated with
giant cell transformation of the hepatocytes. Liver biopsy and
radionuclide excretion scans are essential
Treatment
Patent segments of proximal bile duct are found in 10 per cent of type I
lesions. A direct Roux-en-Y hepaticojejunostomy will achieve bile flow in 75 per
cent, but progressive fibrosis results in disappointing long-term results.
A simple biliary-enteric anastomosis is not possible in the majority of cases in
which the proximal hepatic ducts are either very small (type II) or atretic (type
III). These are treated by the Kasai procedure, in which radical excision of all
bile duct tissue up to the liver capsule is performed. A Roux-en-Y loop of
jejunum is anastomosed to the exposed area of liver capsule above the
bifurcation of the portal vein creating a portoenterostomy.
Postoperative complications include bacterial cholangitis,which occurs in 40
per cent of patients. Repeated attacks lead to hepatic fibrosis and 50 per cent
of long-term survivors develop portal hypertension, with one-third having
variceal bleeding.
Liver transplantation should be considered in children in whom a
portoenterostomy is unsuccessful. Results are improving with 70–80 per cent
alive two to five years following transplant
Choledochal cyst
Cystic disease of the biliary system is rare. Choledochal
cysts are congenital dilations of the intra- and/or
extrahepatic biliary system.
The pathogenesis is unclear.Patients may present at any age with
jaundice, fever, abdominal pain and a right upper quadrant mass.
60 per cent of cases are diagnosed before the age of ten years.
Pancreatitis is not an infrequent presentation in adults.Patients with
choledochal cysts have an increased risk of developing
cholangiocarcinoma.
Diagnosis by US,CT scan,.MRI,and MRCP
Radical excision of the cyst is the treatment of choice 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.Resection and Roux-en-Y
reconstruction is also associated with a reduced incidence of stricture
formation and recurrent cholangitis
Classifications of the cholodochal cyst;
1 a&b;-diffuse cystic type(fusiform or saccular)
11;-diverticulum)
111;-diverticulum within the pancreas
1V;-extension within the liver
V;-only intrahepatic diletation
GALLSTONES (CHOLELITHIASIS)
Gallstones are the most common biliary pathology. It is estimated that gallstones affect 10–
15 per cent of the population in western societies.
Causal factors in gallstone formation;-
Gallstones can be divided into three main types
1-cholesterol 2-pigment (brown/black) 3- mixed stones.
In the United States and Europe, 80 per cent are cholesterol or mixed stones, whereas
in Asia, 80 per cent are pigment stones
Pathophysiology of gall stones formation;-.
1-metabolic theory;For Cholesterol to remain in the solution need the bile acid and
phospholipid and bile acid with the cholesterol will form the Micell which is
hydrophilic and can remain and absorbed in the solution and any cause that increase
the cholesterol or decrease the bile acid lead to supersaturated bile(lithogenic bile)
and lead to formation of the gall stones ;-
Causes of lithogenic bile;-
a-high caloric diet
B-obesity
C-contraceptive pills and cholystyramine
D-Resection of the terminal ileum, which diminishes the enterohepatic circulation, will
deplete the bile acid pool and result in cholesterol supersaturation
2-nidus of infection lead to aggregation of nucleated
cholesterol crystal and stone formation
3-stasis of bile lead to stone formation e.g vagatomy,total
parenteral therapy,
4-pigmented stones associated with haemolysis e.g
spherocytosis,sickle cell anemia
5-Stone formation is related to the deconjugation of
bilirubin deglucuronide by bacterial-glucuronidase.
Insoluble unconjugated bilirubinate precipitates.
6-Brown pigment stones are also associated with the
presence of foreign bodies within the bile ducts, such as
endoprosthesis (stents), or parasites, such as Clonorchis
sinensis and Ascaris lumbricoides.
Clinical features;
Usually female more than male ,fertile ,forty years of age,fatty and flatulence
dyspepsia(5 f)
1-patient may be asymptomatic and we call it silent gall stone
2-chronic cholecystitis;
A-pain in the epigastric or right hypochondrial region of the abdomen radiating to the back and
interscapular region and to the right shoulder,agrivated by fatty meal associated with nausea and
vomiting,belching and dyspepsia and particularly flatulence dyspepsia
B-on examination;tenderness in the epigastric and right hypochondrium
3-acute cholecystitis;when the pain continue for more than 12 hrs and abdomen tenderness more
sever with muscle guarding and positive murphy's and Boas sign with leukocytosis and moderately
elevated liver function tests
Differential diagnosis of acute cholecystitis
Common
Appendicitis
Perforated peptic ulcer
Acute pancreatitis
Rare
Acute pyelonephritis
Myocardial infarction
Pneumonia – right lower lobe
Complication of gall stones
1-Biliary colic
2-Acute cholecystitis
3-Chronic cholecystitis
4-Empyema of the gall bladder
5-Mucocoele
6-Perforation
7-Biliary obstruction
8-Acute cholangitis
9-Acute pancreatitis
10-Intestinal obstruction (gallstone ileus)
Diagnosis;-
1-history and physical examination
2-US and Scan
3-MRCP
Treatment
Treatment of symptomatic gall stones is surgery and it is save to
observe patients with asymptomatic gall stones but the indications
of cholecystectomy in asymptomatic gall stones are;-
1-diabetic patient or immunosuppressed patient
2-stones more than 2.5 cm
3-porcelian gall bladder carry 25% risk of malignancy
4-those with congenital haemolytic anaemia when doing
splenectomy
5-those patients who are undergoing bariatric surgery for morbid
obesity
Conservative treatment of acute cholecystitis;-
more than 90 per cent of cases,the symptoms of acute cholecystitis
subside with conservative measures. Non-operative treatment is
based on four principles:
• Nil per mouth (NPO) and intravenous fluid administration until the pain resolves.
• Administration of analgesics.
• Administration of antibiotics. As the cystic duct is blocked in most instances, the
concentration of antibiotic in the serum is more important than its concentration
in bile. A broad-spectrum antibiotic effective against Gram-negative aerobes is
most appropriate (e.g. cefazolin, cefuroxime or gentamicin).
• Subsequent management. When the temperature, pulse and other physical
signs show that the inflammation is subsiding, oral fluids are reinstated followed
by regular diet.
.
The timing of surgery in acute cholecystitis remains controversial
with many units favouring an early intervention within the first week,
whereas others suggest that a delayed approach is preferable.
1-Early cholecystectomy during acute cholecystitis appears to be safe
and shortens the total hospital stay.Provided that the operation is
undertaken within 5–7 days of the onset of the attack,
the conversion rate in laparoscopic cholecystectomy is higher in acute
than in elective surgery.
2-If an early operation is not indicated, one should wait approximately 6
weeks for the inflammation to subside before operating
Cholecystectomy;-
1-conventional(open) approach
2-laproscopic cholecystectomy(lap.chole.)

Preparation for operation

1-Full blood count

2-Renal profile and liver function tests
3-Prothrombin time
4-Chest x-ray and electrocardiogram (if over 45 years or
medically indicated)
5-Antibiotic prophylaxis
6-Deep vein thrombosis prophylaxis
7-Informed consent

The golden role in cholecystectomy open or lap. is the

identification of the Calots triangle
Lap.chole;-
1-supine position and general anesthesia with anti
trendlenberg position with tilt to the left
2-umbilical pneumoperitonium(Co2 gas) with
open(Hasson method)or close method using verres
needle
3-add epigastric port(surgeon port) and mid-clavicular
port with additional optional port
4-identy the calot triangle
5-release the anterior and posterior peritoneal reflection
6-identification and clipping of the cystic artery and
cystic duct
7-removal of the gall bladder with secure bed
Open cholecystectomy
Indication;
1-For patients in whom a laparoscopic approach is not indicated
2-in whom conversion from a laparoscopic approach is required
an upper midline or a short right upper transverse incision is made
centred over the lateral border of the rectus muscle.
The gall bladder is appropriately exposed and packs are placed on the
hepatic flexure of the colon, the duodenum and the lesser omentum
to ensure a clear view of the anatomy of the porta hepatis. These
packs may be retracted using the hand of the assistant (‘It is the left
hand of the assistant that does all the work’ – Moynihan)
or, alternatively, a stabilised ring retractor can be used to keep the
packs in position.