The causes of jaundice in the first weeks of life can becategorised into hematologic, enzymatic/metabolic, infectiousand obstructive.[1,2] Diagnostic tests are definitive and surgicaltreatment options do not have a role in the first threecategories. Operative cholangiography is the next logical stepin the diagnosis of obstructive causes.[2,3] Obstructive jaundiceis caused by an interruption to the drainage of bile in the biliarysystem. Surgical causes of jaundice in neonates are biliaryatresia, inspissated bile syndrome, intrahepatic hypoplasia,choledochal cyst, Caroli’s disease and spontaneousperforation of the bile duct.[1,2,3]

Biliary atresia (BA)It is a pathologic process leading to fibrosis and obliterationof the ductal system resulting in obstruction to bile flow, andcholestasis. The aetiology of this entity is unknown. Theincidence of BA is high in China and Japan (1:8,000) ascompared to  Europe (1:17,000 live births), and is morecommon in girls than in boys.[4,5,6] A higher incidence is found ininfants less than 32 weeks of gestational age, where thematernal age at conception is more than 35 years, and inmothers with parity 4 or more.[7]

Aetiopathogenesis

Evidence suggests that a few cases related to abnormalmorphogenesis of the bile ducts occur early in gestation, whilstothers appear to arise from damage to the normally developingbile ducts.[4,5,6,7,8] Desmet’s theory suggests the interruption of thenormal remodelling process of the primitive ductal plate. Local ischemia during foetal hepatobiliary development turns intoflow obstruction. As per Howard’s hypothesis, bile leakagefrom abnormal ducts causes inflammatory reaction withsubsequent obliteration of the biliary tree.[9]

During pregnancy, exposure to environmental toxins leadsto infantile obstructive cholangiopathy. Some commonlyinvolved viruses, reovirus-3, cytomegalovirus, rotavirus C,human papilloma virus and retroviruses have been reportedin the literature.[1,2,3,9] Obstruction to the flow of bile causesprogressive cholestasis due to toxic, hydrophobic bile acids.BA may be an acquired rather than a hereditary disease asdiscordance has been seen in monozygotic twins.[10,11,12,13,14,15] Approximately 10% of all cases have been associated withanomalies such as polysplenia, asplenia, situs inversus,absence of the inferior vena cava and pre-duodenal portalvein, for which the term biliary atresia splenic malformation(BASM) syndrome has been coined in the literature.[3,16,17] Otherassociated anomalies are cleft lip, atresia of esophagus,duodenum, and/or jejunum; annular pancreas, malrotation ofgut, and polycystic kidney.[5,6]

Pathology

In the intralobular spaces of the liver, bile stasis, distortion,focal necrosis, hemosiderin deposition in liver cells andintralobular fibrosis occur.[18] In the interlobular spaces,widening of portal area, hepatic fibrosis, periportal oedema,ductular proliferation and bile stasis are noted.[19] Even aftersurgery, when bile flow is regular, intrahepatic bile ducts arenot restored to normal morphology; although some reductionin hepatic fibrosis and inflammatory cells is seen. So surgery is most effective within 60 days of birth, since the hepatic ductsundergo progressive destruction.[4,20,21]

Classification

Biliary atresia is classified as correctable (20%) with distalbiliary tree fibrosis and patent intrahepatic bile ducts andproximal biliary tree; and non–correctable (80%) with fibrosisupto the level of the porta hepatis. Anatomical classificationbased on the pattern of extrahepatic biliary tract remnant iswidely accepted. (Table 1)[4,5,6]

Clinical features

The neonate may present with a history of normal birth weight,varying degrees of jaundice, clay-coloured stools and darkyellow urine. Failure to thrive, coagulopathy and anaemia arealso common. On examination, hepatomegaly, signs ofadvanced disease and cirrhosis, such as ascites, umbilicalhernia, prominent abdominal veins and respiratory discomfortmay be present.

Biochemical  tests

A rise in total bilirubin and fall in proteins (albumin) with reversalof the albumin/globulin ratio is noted in advanced cases. Levelsof alkaline phosphatase and transaminases (ALT, AST) arealso raised. Deranged LFTs correspond to the degree ofparenchymal damage and not to the duration of disease.Hepatitis A, B, and C serology; TORCH titres, alpha-1-antitrypsin, gamma glutamyl transpeptidase (GGPT), andserum lipoprotein-X estimates are carried out to negate thepossibility of other causes of cholestasis.[4]

Ultrasonography

Shrunken, non-distended gallbladder without visible commonduct structure is the usual picture. Triangular cord sign, morethan 4 mm thickness of echogenic anterior wall of the rightportal vein on longitudinal scan, is most indicative of BA.[22,23]This modality may also be used for the differential diagnosisof cholestasis, choledochal cyst, and the polyspleniasyndrome.

Duodenal fluid aspiration test

Duodenal intubation may be carried out for 7-24 hours with aRyle’s tube and the aspirated fluid examined; the presence ofyellow bilirubin pigment, rules out BA.[24] Severe neonatalcholestasis can generate false negative results.

Nuclear imaging

It differentiates obstructive from parenchymal causes ofjaundice. Non-appearance of the isotope in the intestine is specific for biliary atresia in upto 50-75% cases only, becausesevere intrahepatic cholestasis and paucity syndrome mayyield similar results.[25]

Operative cholangiography

The gold standard for the diagnosis of BA and widely employedas a more practical tool is operative cholangiography.[26] Thegallbladder appears small and fibrotic and the dye fails toenter proximally into the hepatobiliary tree.

Percutaneous liver biopsy

This is usually carried out prior to surgery but may be doneperoperatively as well. It has a diagnostic accuracy of 90%.[27] Itreveals portal tract oedema, fibrosis, inflammation, intracellularand canalicular cholestasis, and proliferation of bile ductules.

Management

Currently BA is managed in two phases;[3,4,21] the first phasecomprises the Kasai procedure, which aims to restore bileflow. In the second phase liver transplantation is considered,if bile flow is not restored by the Kasai procedure or life-threatening complications of cirrhosis ensue. In patientspresenting late with advanced cirrhosis, liver transplantationis done as the primary procedure.

Kasai procedure (hepato-porto-enterostomy) has been thestandard for non-correctable BA since 1958.[28] Extended rightsubcostal incision cholangiography and liver biopsy are done.The fibrous remnant of the CBD is dissected beyond theconfluence and the transected liver hilum anastomosed withRoux-en-Y loop of jejunum.  Many technical variations arepossible, according to the anatomical pattern of the biliaryremnant.[20,21]

Type 1 :Cholecystoenterostomy, or hepaticoenterostomy
Type 2:Cystoenterostomy where the hilar cystcommunicates with the dystrophic intrahepaticbile ducts, demonstrated on cholangiography
Type 3:Hepatoportocholecystostomy

In portocholecystostomy, the gall bladder is used fordrainage, and requires a patent cystic duct and CBD.Complications such as bile leak, gallbladder obstruction,cholangitis, and kinking of the CBD are more frequent.29-31Liver transplantation is indicated after portoenterostomy. Thefive-year survival after transplantation is 80-90%.[30,31,32]

Postoperatively during the evaluation phase of biliaryatresia, the infant’s diet is typically not altered; breastfeedingis encouraged where possible, but an energeticsupplementation may be required to obtain a 150-180 Kcal/kg/day intake. As long as cholestasis persists,supplementation of fat-soluble vitamins is necessary.Administration of choleretics like ursodeoxycholic acid 20 mg/kg/day has been studied with benefits;[33] prednisolone useremains controversial.[34]

Complications

Cholangitis is the most common complication with anincidence of 40-60% following surgery. It can be reduced bylengthening of  50-70 cm of the roux-en-Y jejunum loop and total diversion of biliary conduit;[35] and construction of theintestinal valve by removing a segment of seromuscular layerof the bowel wall and intussuscepting the denuded mucosaas a nipple.[36] Other complications are portal hypertension,nutritional deficiencies like rickets, pulmonary arteriovenousfistula, ectopic variceal bleed, hepatopulmonary syndrome,pulmonary hypertension and malignancy.

Outcome after successful Kasai operation

If the Kasai operation succeeds in restoring bile flow, theevolution of biliary cirrhosis is prevented or at least delayed,and survival with the native liver has been reported up toadulthood. Factors influencing prognosis are the patient’s ageat the time of surgery, extension of liver fibrosis at surgery,degree of intrahepatic bile duct injury, number of episodes ofascending cholangitis, the surgeon’s expertise and the site ofbile duct obstruction.[30,31,32,37,38]

Choledochal cysts

These are congenital conditions associated with benign cysticdilatation of the bile ducts.  Its incidence, although as high as1:1000 in the Asian (Japan) population, is only 1:150 000 inthe West.[3] They are more frequently seen in female casesthan in male cases. About 60% are diagnosed during the firstdecade of life, whilst 20% remain undiagnosed until adulthood.

The preponderance in Asia suggests a role for eithergenetic or environmental factors. Anomaly of thepancreaticobiliary junction results in a long common channeland reflux of pancreatic enzymes into the CBD, leading tomucosal breakdown and dilatation.[39] This can present early(in children) with high grade reflux or later (adulthood) with lowgrade reflux.[40,41]

There can be abnormal autonomic innervation of theextrahepatic biliary tree and weakening of the duct wall due toincreased proximal pressure, because of distal obstruction,which is also responsible for the formation of choledochalcysts.[42,43]

The traditional classification system devised by Alonso-Lej[44] exclusively involved the extrahepatic duct. The clinicalclassification was revised in 1977 by Todani and colleagues.(Table 2)[45]

The patient usually presents with a classical triad ofabdominal pain, cholestatic jaundice (80%), and abdominalmass (30%).² In patients over 2 years of age, abdominal painis the most common presenting symptom. Bile and pancreaticjuice reflux and bile stasis lead to chronic inflammation, stone and stricture formation. This in turn leads to recurrentcholangitis, hepatic abscesses and pancreatitis, resulting insignificant pain and jaundice.[46,47,48,49,50,51,52] The main diagnostic tool fordetection of a choledochal cyst, especially in childhood, isultrasonography. In adults, computed tomography can confirmthe diagnosis; however, endoscopic retrogradecholangiography or magnetic resonance cholangiography, areconsidered the gold standard.[53]

Surgery is the treatment of choice;[2] excision of the cyst andreconstruction of the biliary tree by choledochal/hepatojejunostomy with a roux-en-Y loop is the standardprocedure for types I and II.[54,55,56] Type III cysts 3 cm or less insize are managed with endoscopic sphincterotomy and cystsmore than 3 cm in size require surgical excision withtransduodenal sphincteroplasty. In Type IVA cysts, for theextraphepatic component, excision and hepaticojejunostomyis done; however, for the intrahepatic component hepaticresection with hepaticojejunostomy is the treatment of choice.Transhepatic intubation may be used for intrahepatic cysts. InType IVB cysts, excision of the cyst with hepaticojejunostomyand transduodenal sphincteroplasty is carried out. For Type Vcysts restricted to one lobe, hepatic resection and livertransplantation are preferred. Bilobar disease represents anespecially challenging problem. Roux-en-Y intrahepaticcholangiojejunostomy or transhepatic silastic intubation for6-12 months may be indicated to improve biliary drainage.

12 months may be indicated to improve biliary drainage.Complications are commonly observed with types I, IV,and V, the overall morbidity rate is less than 10%. Post-surgicalcomplications include cholangitis, biliary stones, anastomoticstricture, residual debris, pancreatitis, and intrahepatic bileduct dilatation.[54] It is important to operate on cholodochal cystsas there is a 20- to 30-fold higher risk in these patients thanthe general population for cholangiocarcinoma. Completeexcision of these lesions is recommended as soon aspossible, preferably before puberty, in order to decrease thechance of developing cancer; the risk remains high even aftersurgery.[57]

Caroli’s Disease

In 1958 Caroli[58] described a congenital anomaly of the biliarytree characterised by multiple cystic dilatations of theintrahepatic bile ducts. In infants, almost all cases of congenitalintrahepatic bile duct dilatation appear to be associated withthe choledochal cyst. The intra-hepatic component isfrequently asymmetrical, involving predominantly one majorintrahepatic branch. Neonates present with jaundice in theearly period which is indistinguishable from presentation ofthe simple choledochal cyst. Ultrasound, CT scan,percutaneous transhepatic cholangiography, endoscopiccholangiopancreatography and magnetic resonancecholangiopancreatography are performed to establish thediagnosis.[59,60,61]

Definitive surgery for most patients is impossible sinceadequate drainage of intrahepatic cysts is difficult to achieve.At the moment, hepatic resection, liver transplantation androux-en-Y intrahepatic cholangiojejunostomy at the liver hilumprovide the best chance for unimpeded bile flow.[61]Transhepatic intubation is recommended as internal drainageis associated with frequent postoperative complicationslike recurrent cholangitis, biliary lithiasis, and hepaticabscess. Malignant degeneration has also beenreported.[62]

Inspissated bile plug

There is mechanical obstruction of the extrahepatic bile ductsby inspissated bile.[63] Inspissated bile syndrome, a misnomerfor a common sequel of erythroblastosis fetalis, results frommassive hemolysis due to Rh or ABO blood groupincompatibility.[64] Obstruction is secondary, as a consequenceof the earlier excessive bile pigment excretory load; it mayproceed to the production of bile pigment stones.[65]

There is persistent jaundice in newborns with hemolyticanaemia, with elevation of both direct and indirect bilirubincomponents. Operative cholangiography is required fordiagnosis. Simple irrigation of the extrahepatic bile ducts iscurative. If choledocholithiasis is present, manual extractionof biliary stones or duodenotomy and sphincterotomy may bedone.[66] The incidence has fallen due to early diagnosis ofblood group incompatibility and prompt exchangetransfusion.[63]

Intrahepatic hypoplasia

Biliary hypoplasia is not a discrete clinical entity; it is anoperative or radiographic finding found in a variety ofhepatobiliary disorders. It is a rare cause of neonatal jaundice.[2,3,4] Affected children have absent or reduced bile ductules withnormal branche distributions of the portal vein and hepaticartery within the liver parenchyma. Biliary hypoplasia is alsoidentified as paucity of interlobular bile ducts (PILBD) and  twotypes have been described.

Syndromic biliary hypoplasia (Alagille’s syndrome) ischaracterised by hyperbilirubinemia, characteristic facialappearance, pulmonic artery stenosis, vertebral anomalies,embryotoxon and delayed weight-height development.[67] Non-syndromic biliary hypoplasia is usually clinicallyindistinguishable from biliary atresia. Cholangiography andliver biopsy show diminutive intra- and extra-hepatic biliarytree. However, management is conservative and includespredigested formulae, ursodeoxycholic acids, phenobarbitone,and vitamin A, D, K, E replacement. PILBD has better long-term prognosis.[68]

Spontaneous perforation of the bile duct (SPBD)

Though uncommon, it is potentially fatal and occurs withsufficient frequency to be considered in the differentialdiagnosis of neonatal jaundice.[69] Predisposing factors forperforation are portal bacteremia, stone disease, protein plugs,distal atresia and viral gastroenteritis. Sites of perforation arethe cystic duct, common hepatic duct, and the CBD; thecommonest site is at the junction of the cystic and hepaticducts.[70,71] The perforation is most often pinhole in size,consequently, bile extravasation is gradual, permitting theformation of a biliary pseudocyst. Jaundice is the usual clinicalmanifestation of the disease which becomes apparent duringthe first three months of life. Systemic signs may be minimaland thus the infant easily blends into the diagnostic mix ofsurgical jaundice in infancy.[72]

Sonography shows either generalised ascitis or localisedcollection of fluid, the biliary tree is usually not dilated.Scintigraphy and abdominal paracentesis demonstrate thatthe intraperitoneal fluid originates from the biliary tract.Intraoperative cholangiography is carried out for confirmationof diagnosis at surgery. It can be misinterpreted as a choledochal cyst, which is disastrous if an intestinalanastomosis is performed on the pseudocyst.

Simple peritoneal drainage (percutaneous tube drainage)of the area of ductal perforation is sufficient in the majority ofcases.[73,74] Peritoneal drainage is followed by spontaneousclosure of the perforation, resolution of the biliary pseudocystand cure of the “mechanical obstruction” (probably bile sludge)of the distal common duct. Definitive treatment involves surgery,and repair of perforation.[69,74] Since the perforation of thecommon duct appears to be the only fault in biliaryembryogenesis, the long term prognosis is excellent.

Iatrogenic

Rarely some cases have been reported in the literature ofneonatal obstructive jaundice caused by a malpositionedgastrostomy tube (Foley’s catheter).[75,76,77]

Conclusion

Pediatricians should be aware of the perniciousconsequences of unresolved biliary obstruction and shouldthus refer neonates or infants with inexplicable jaundice forsurgical exploration at an earlier age than was previouslyconsidered appropriate. The specific operations advocatedfor congenital malformation of the biliary tract are 1) Correctablebiliary atresia: roux-en-Y choledochojejunostomy 2) Non-correctable biliary atresia: Kasai’s hepatic portoenterostomyoperation. In the few patients in whom the gallbladder and thedistal biliary tree are still patent, hepatic portocholecystostomyis advocated 3) Billary hypoplasia: Hepatic portoenterostomy4) Choledochal cyst: Total excision andcholedochojejunostomy (roux-en-Y) 5) Caroli’s disease:Resection of the dilated portion of the extra-hepatic bile ductand roux-en-Y choledochojejunostomy. Hepatic resection isdone for intrahepatic cystic disease limited to a single segmentor lobe 6) Inspissated bile plug: Irrigation of the extrahepaticbile ducts and for choledocholithiasis, manual extraction ofbiliary calculi. 7) Spontaneous perforation of the common bileduct: Simple peritoneal drainage at the site of ductal perforationand cholecystostomy.

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