Obstructive Jaundice in Neonate

Presenter:Dr. Shashi K. SinghModerater:Dr. R.P. Chuadhary,Dr. Puskar

pokharel, Dr. Ramana Raj kansakar

Jaundice

• 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.

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

• 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

Biliary atresia

• Life-threatening condition

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

Incidence

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

heritage

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

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

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

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.

Symptoms/Signs

• 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 Examinations

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

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

• 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)

Complications AfterPortoenterostomy(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

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

Liver Transplant • Liver transplantation is the definitive

treatment for biliary atresia• Survival rate after surgery has increased

dramatically in recent years

• 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.

• 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.

Complications ofLiver Transplantation

• 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.

• 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

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

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

• 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

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%

• 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

Presentation

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

cholangitis • pancreatitis

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)

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.

• 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

• 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 sphincterotomyresection is typically approached via a transverse

duodenotomy in the second or third portion of the duodenum

• 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

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

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.

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.

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.

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

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.

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