• Type 1 involves obliteration of the common duct, while the proximal ducts are patent;
• Type II is characterized by Atresia of the hepatic duct, with cystic structures found in the porta-hepatis;
• And type III (> 90% of patients) involves Atresia of the right and left hepatic ducts at the level of the portal hepatis. (these variants can be easily confused with intrahepatic biliary hypoplasia, which is essentially a disorder that cannot be rectified surgically)

• The clinical presentation of neonatal cholestasis is remarkably similar in most infants.
• Typical symptoms include variable degrees of jaundice, dark urine, and light stools.
• In the case of biliary Atresia, most infants are full - term, though a higher incidence of low birth weight may exits.
• In most cases, acholic-white stools are not noted at birth but develop over the first few weeks of life. Appetite, growth, and weight gain may be normal.

• Physical findings do not identify all cases of biliary Atresia. None of the findings are typical for the disorder.
• These infants typically are full term and may manifest normal growth and weight gain during the first few weeks of life.
• Liver enlargement may be present early, and the liver is often firm or hard to palpation. Enlarged Spleen is common, and suggests onset of progressive cirrhosis with portal hypertension
• Consider biliary Atresia in all neonates with direct hyperbilirubinemia. Direct hyperbilirubinemia is always an abnormal finding and may be present from birth in BA.
• In the more common postnatal form, physiologic jaundice frequently merges into conjugated byperbilirubinemia. The clinician must be aware that physiologic unconjugated hyperbilirubinemia rarely persists beyond 2 weeks. Infants with prolonged physiologic jaundice must be evaluated for other causes.
• In patients with the fetal/neonatal form (polysplenia/asplenia syndrome) a midline liver may be palpated in the hypogastrium.
• The presence of cardiac murmurs suggests the presence of associated cardiac anomalies.
• A high index of suspicion is key to making a diagnosis because surgical treatment before the age of 2 months has clearly been shown to improve the likelihood of establishing bile flow and to prevent the development of irreversible biliary cirrhosis.

• The existence of the fetal/perinatal form of biliary Atresia, frequently associated with other gastrointestinal and cardiac anomalies, suggests the possibility of a disorder n ontogenesis. Studies have identified specific genetic mutations in mice with visceral heterotaxy and cardiac anomalies, defects similar to those found in conjunction with the fetal/perinatal form of biliary Atresia.
• A variety of other genetic abnormalities, including deletion of the mouse c-jun gene (a proto-oncogene transcription factor) and mutations of home box transcription factor genes are associated with hepatic and splenic defects; however a direct link to biliary Atresia has not been described.

• Disorders of bile acid synthesis are part of the differential diagnosis of biliary Atresia. In fact, bile acids almost certainly contribute to ongoing hepatocellular and bile ductular damage in infants with the disorder. Although associated defects in bile acid metabolism may hasten progression of liver disease, no primary role for bile acids in the development of biliary Atresia has been identified.
• Several investigators have studied the potential effects of other etiological agents, including teratogens and immunological factors. Again, no clear correlations with biliary Atresia have been demonstrated.

• Alpha - 1 - anti - trypsin deficiency
• Byler disease
• Choledochal cyst
• Idiopathic neonatal hepatitis
• Inborn errors of bile acid synthesis
• Nonsyndromic intrahepatic bile duct hypoplasia
• Total parenteral Nutrition - associated (TPN) cholestasis
• Viral infections (eg. Toxoplasmosis. Other infections, rubella,
• Cytomegalovirus infection and herpes simplex (TORCH)

• Serum bilirubin (total & direct): Conjugated hyperbilirubinemia, defined as any level exceeding either 2.0 mg% or 20% if titak bilirubin, is always abnormal. Interestingly, infants with biliary Atresia typically show only moderate elevations in total bilirubin, which is commonly in the 6 to 12 mg% range with the direct (conjugated) fraction comprising 50 - 60% of total serum bilirubin
• Alkaline phosphatase (AP), 5' nucleotidase, gamma - glutamyl transpeptidase (GGTP), serum amonotransferases, serum bile acids.
• These candidate tests have been proposed as a means to increase both sensitivity and specificity of the routine laboratory evaluation. Unfortunately, no single biochemical determination accurately discriminates between biliary Atresia and the other causes of neonatal cholestasis.
• In addition to direct hyperbilirubinemia (a universal finding in neonatal cholestasis), enzyme abnormalities include elevations in AP. In some cases, skeletal sources of AP can be differentiated from hepatic sources by measuring the liver - specific AP fraction, 5" nucleotidase.
• GGTP is an integral membrane protein of the bile canaliculus and is elevated in cholestatic conditions. GGTP levels closely correlate with AP findings and are increased in all biliary obstructive conditions. However, GGTP may be normal in some forms of cholestasis of hepatocellular origin.
• Aminotransferase levels are not particularly helpful in establishing a diagnosis though a markedly elevated alanine aminotransferase (ALT >800 IU/L) indicates significant hepatocellular injury and is more consistent with the eneonatal hepatitis syndrome.
• Serum alpha - 1 antitrypsin with Pi typing: Alpha - 1 - antitrypsin deficiency represents the most common inherited liver disease that presents with neonatal cholestasis. The abnormal PiZZ phenotype, as determined by electrophoresis, is associated with neonatal cholestasis in approximately 10% of affected subjects.
• Sweat chlorine (CI): Biliary tract involvement is a well - recognized complication of Cystic fibrosis (CF, and an association between meconium ileus in the newborn and cholestasis has been described. A diagnosis of CF should be strongly considered in any infant with direct hyperbilirubinemia, particularly when other associated signs/symptoms (i.e. respiratory, GI) are present. Sweat CI iontophoresis remains the criterion standard for diagnosing CF

• In neonatal cholestasis Baby Humaira Tahmid HIDA/03 Bone 2003-11-07syndromes, ultrasound can exclude specific anomalies of the extrahepatic biliary system, particularly choledochal cyst. Today a diagnosis of choledochal cyst should be made in utero by fetal ultrasonography.
• In biliary Atresia, ultrasound may demonstrate absence of the gallbladder and no dilatation of the biliary tree. Unfortunately, the sensitivity and specificity of these findings in the most experienced centers probably do not exceed 80%. For this reason ultrasound has been found to be an unreliable diagnostic tool in the evaluation of biliary Atresia.

• Hepatobiliary imaging, utilizing technetium - labeled diisorpropyl iminodiacetic acid (DISIDA) nuclear scintiscan, is useful in evaluating infants with suspected biliary Atresia. Unequivocal evidence of intestinal excretion of radiolabel confirms patency of the extrahepatic biliary system
• Two cautionary notes are required, first, reliability of the scintiscan is diminished at very high conjugated bilirubin levels (>20 mg%). Second, the test has been associated with a 10% rate of false - positive/false - negative diagnostic errors.

• Percutaneous liver biopsy is widely regarded as the most valuable study for evaluating neonatal cholestasis. Morbidity is low in patients without coagulopathy . when examined by an experienced pathologist, an adequate biopsy specimen can differentiate between obstructive and hepatocellular causes of cholestasis, with 90% sensitivity and and specificity for biliary atresia.
• Of importance, several cholestatic conditions, including biliary Atresia, may demonstrate an evolving histopathological pattern. Accordingly, biopsies usually are not diagnostic in those younger that 2 weeks, and serial samples usually at 2 - week intervals, may be required to reach a definitive diagnosis.

• No primary medical treatment is relevant in the management of extrahepatic biliary Atresia. The pediatrician's objective is to confirm the diagnosis.
• Once biliary Atresia is suspected, surgical intervention is the only mechanism available for a definitive diagnosis (intraoperative choliangiogram) and therapy (Kasai portoenterostomy)

• Following a thorough evaluation for causes of neonatal cholestasis, intraoperative choloangiography establishes the diagnosis of extrahepatic biliary Atresia.
• At operation, the fibrotic biliary tract remnant is identified, and patency of the biliary system is assessed.
• Incases in which biliary patency is associated with ductual hypoplasia, further surgical intervention is not indicated, and bile may be collected to evaluate for disorders of bile acid metabolism.
• Under the unusual circumstances of distal patency of the common duct with acceptable proximal luminal caliber, a modified portoenterostomy may be considered in place of the traditional kasai procedure. However, the clinician must be aware that progression of diseases pathophysiology may occur. The author has observed patients undergo modified portoenterostomies (gallbladder Kasai), only to subsequently experience continued inflammation and obliteration of the estrahepatic biliary tree and to ultimately require classic portoenterostomies.
• In most cases of Atresia, dissection into the porta hepatis and creation of a Rouz-en-Y anastomosis with a 35 to 40 cm retrocolic jejunal segment is the procedure of choice.
• Recent studies have reported that extension of the portal dissection beyond the portal vein bifurcation and the umbilical point in the left hilum may improve the likelihood of achieving adequate biliary drainage.

• The evaluation of neonatal cholestasis may initially be carried out by the primary pediatrician, depending upon reliability of the laboratory in performing the necessary serum determinations indicated above.
• Obviously, further non-surgical testing (eg. Hepatobiliary imaging, liver biopsy) and surgical exploration should be carried out by pediatric hepatologists in liver transplant centers with considerable experience in managing this disorder.
• The physician must not delay in the diagnosis of extrahepatic biliary Atresia. Refer infants as soon as a diagnosis of obstructive jaundice is suspected, to HPB surgeon/ pediatric hepatologist with requisite experience.

• During the evaluation phase of biliary Atresia, the infant's diet typically is not changed.
• Postoperative breastfeeding is encouraged when possible, since breast milk contains lipases and bile salts to aid in lipid hydrolysis and micelle formation, theoretically, breast milk also may protect against cholangitis, a common complication following portoenterostomy, by suppressing the growth of gram-negative and anaerobic flora. However, to date is available to support this claim.
• Infants who are fed formula and who achieve adequate bile drainage should not require a special diet. Early in the postoperative course and when the status of biliary continuity may be in question, in of the medium-chain triglyceride-containing formulas (eg. Almentum, Pregestimil) may enhance lipid digestion.

Complications following portoenterostomy include both acute and chronic problems.

• In the early postoperative phase an unsuccessful anastomosis with failure to achieve adequate bile drainage is the most common complication. In this case, adequacy of bile flow may be predicted by the preoperative liver histology and the caliber of bile ductular remnants in the porta hepatis. In one third of ll patients, bile flow is inadequate following surgery and these children succumb to complications of biliary cirrhosis in the first few years of life unless othotopic liver transplantation is performed.
• Later in the course, complications related to progressive liver diseases and portal hypertension occur in more than 60% of infants who acheived initial surgical success.
• Hepatocellular carcinoma may be a risk for those patients with cirrhosis and no clinical evidence of portal hypertension. Progressive fibrosis and biliary cirrhosis develop in children who do not drain bile, and liver transplantation is the only option for ling - term survival.

• Younger than 2 months at operation
• Preoperative histology and ductual remnant size
• Presence of bile in hepatic lobular zone 1
• Absence of portal hypertension, cirrhosis and associated anomalies
• Experience of the surgical team
• Postoperative clearing of jaundice.

Re-operation: The following 3 categories of patients with extrahepatic biliary Atresia should be considered for re-exploration following a Kasai or modified Kasai portoenterostomy:

• Infants who become jaundiced after an initial anicteric phase postoperatively
• Infants with favorable hepatic and biliary duct remnant histology at initial operation who do not successfully drain bile
• Infants who my have had an inadequate initial surgery

• After surgery for removal of the Gall bladder (1 %)
• Primary cancer of the bile duct
• Stones in Gall bladder or the bile duct
• Cholangitis
• Choledochal cysts
• Pancreatitis or pseudo cysts
• Chrohn's disease