Presenter: Dr. Pashi V. MRegistrar peadiatrics surgery

Yellowish discoloration of the skin, mucous membranes, and sclerae of the eyes

Hyperbilirubinemia Deposition of bile salts in these tissues

Jaundice in the first few weeks of life categorised into

Hematologic, Enzymatic/metabolic, Infectious and Obstructive

Biliary atresia Choledocal cystInspisseted bile

Obstructive jaundice

Post hepatic(Obstructive jaundice ) an interruption in the drainage of bile

in the biliary system.

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

• 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

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

BileFluid made by the liverTwo main functions:1.Carrying toxins and waste products out of

the body2. Helping the body digest fats and absorb

the fat-soluble vitamins A, D, E, and K

• 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

Biliary atresia: most common cause of obstructive jaundice

requiring operation in children

Choledochal cyst: 2nd most common cause

Life-threatening condition

Bile ducts inside or outside the liver do not have normal openings

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

heritage

• 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

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

• 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.

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.

• Jaundice

• Dark urine • Gray stools From a lack of bilirubin reaching the

intestines

• Slow weight gain and growth • Hepatomegaly

Routine Examinations Color of stool Consistency of the liver Conventional liver function tests, including

test for γ-glutamyl transpeptidase Coagulation times (PT, aPTT)

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

Confirmation of patency of extrahepatic bile ducts

Duodenal fluid aspiration Ultrasonography Hepatobiliary scintigraphy Endoscopic retrograde

cholangiopancreatography Near-infrared reflectance spectroscopy

Other: Needle biopsy of the liver for

histopathologic studies Laparoscopy Surgical cholangiography

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

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

Kasai operation(Portoenterostomy)

• 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

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

Liver Transplant Liver transplantation is the definitive

treatment for biliary atresia Survival rate after surgery has increased

dramatically in recent years

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

CHOLEDOCHAL CYST

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.

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

• 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

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.

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

• 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%

• 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

The classic triad Pain, jaundice, and abdominal mass.

Conjugated bilirubin (80%), Failure to thrive Intermittent jaundice and recurrent

cholangitis pancreatitis

• 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)

• 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.

• 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

• 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.

• 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.

• 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

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.

The endThank you!!!!!