biliary atresia is the most common neonatal cholestatic disorder with a varying incidence of 1:8000 - 1:18000 . the disease is characterized by obliteration or discontinuity of the extrahepatic bile ducts with persistent jaundice in an infant who usually looks well . most patients with extrahepatic biliary atresia ( ehba ) ( 70 - 95% ) have no associated extrahepatic congenital abnormality and are classified as having  classical  biliary atresia . the remaining cases present in association with one or more extrahepatic congenital anomalies , including polysplenia , portal vein abnormalities , intestinal malrotation , abdominal situs inversus , absence of inferior vena cava , intrapulmonary shunting , asplenia , pancreatic anomalies , and congenital heart disease . these anomalies might be the result of a late intrauterine or early neonatal insult associated with a presumed inflammatory process whereas cases of ehba with major unrelated congenital anomalies could arise through malformations , disruptions or chromosomal abnormalities . the liver damage may progress despite re - establishment of biliary drainage after corrective surgery . the medical records of 137 patients with ehba treated by the senior author ( vb ) in the department of pediatric surgery , aiims , new delhi during the past 12 years were reviewed . retrospective data were collected from each record including the patient 's age at the time of surgery , sex , duration of onset of jaundice , liver function tests , imaging studies , presence of any associated anomalies ( polysplenia , hepatic vasculature and portal vein anomalies , intestinal malrotation , situs inversus or congenital heart disease ) , date of kasai procedure ( kp ) , liver histology , and postoperative outcome . imaging evaluation was done with ultrasonography , magnetic resonance cholangiopancreatography , post - primingtc iminodiacetic acid hepatic scan , and peroperative cholangiography . the mean age was 81  33 days ( range = 20 - 150 days ) . these anomalies were divided into four groups  hepatobiliary , gastrointestinal , cardiovascular , and others [ table 1 ] . of these 40 patients , 26 patients had hepatobiliary anomalies which included hepatic vascular inflow anomalies in 22 patients , choledochal cyst in 4 patients , and 2 patients had spleniculi . the right hepatic artery arose from the superior mesenteric artery in 7 patients , 10 patients showed early branching of right hepatic artery outside liver parenchyma , in 2 patients right hepatic artery gave a small branch to the left lobe , cystic artery was found to be arising from the left hepatic artery in 2 patients , in 2 patients two cystic arteries were supplying the gall bladder , accessory right hepatic artery was seen in 4 patients , early division of portal vein was seen in 5 patients . gastrointestinal anomalies were seen in 13 patients ( 22.4% ) and these included intestinal malrotation in 7 patients ( 12% ) , jejunoileal atresias ( jejunal-2 , ileal-1 ) in 3 patients ( 5% ) , 2 patients ( 3.4% ) had esophageal atresia and tracheoesophageal fistula , 1 patient ( 1.7% ) had meckel 's diverticulum . cardiac anomalies included an atrial septal defect in 2 patients and two had situs inversus . others included umbilical hernias ( n = 10 ) and inguinal hernia ( n = 3 ) . congenital anomalies associated with biliary atresia the esophageal atresia and jejunoileal atresia patients had undergone corrective surgeries in the 1 week of life and none of them had received parenteral nutrition . all the patients ( n = 7 ) with malrotation underwent ladd 's procedure simultaneously with kp . furthermore , patients who had hernias ( umbilical / inguinal ) underwent hernia repair simultaneously . all infants ( n = 4 ) of ehba with choledochal cyst had type iii biliary atresia and were treated surgically with bilioenteric bypass procedures . it occurs slightly more often in females than in males ( 1.4:1 ) , and affects children of all races . the coexistence of ehba with other congenital anomalies has been widely reported , with an incidence ranging from 10% to 15% . in our study , ehba occurred as an isolated defect in 70.8% patients and was associated with other anomalies in 29.2% of infants . in larger series of ehba , either an equal gender distribution or only a slight female predominance was observed . in our study , not only a higher prevalence of ehba but also a higher incidence of associated anomalies was seen in boys . various anomalies including malrotation , liver asymmetry , situs inversus , absent inferior vena cava , preduodenal portal vein , and polysplenia have been reported in association with biliary atresia ; teratogenesis during the critical period of fetal embryogenesis of the foregut might be responsible for these anomalies . in our series , vascular anomalies at porta hepatis the anatomy of the hepatic artery and its variants has been described adequately in literature , but new emphasis was given to this anatomy with the introduction of liver transplantation . four patients with atresia of the distal common bile duct had a proximal choledochal cyst and were treated surgically with bilioenteric bypass procedures . the occurrence of associated anomalies in infants with ehba might support the hypothesis that the disease represents more than one clinical entity with different causes . in literature , among congenital structural anomalies associated with ehba , splenic malformations such as polysplenia or asplenia have been known to be very common . polysplenia syndrome has been reported in 25% of all biliary atresia cases ; this might be due to the insult to the bile duct early in gestation at a time coinciding with the development of the spleen and gut rotation . however , it was seen in only two infants ( 3.4% ) in our series . malrotation of the intestinal tract is the result of a well - defined aberrant sequence of embryological events . in the present series , among gastrointestinal anomalies , malrotation of the intestine was the most frequent anomaly and it was incidentally detected during kp . none of these patients had manifested any symptoms related to intestinal malrotation . in all these patients , technical difficulties were , however , encountered while constructing the roux en y loop in the derotated bowel for kp . the most frequent form of intestinal atresia associated with ehba has been reported to be duodenal atresia . the association of biliary atresia and duodenal malformation might be presupposed from their common points of embryonic development . the incidence of ehba in cases of jejunoileal atresia ranges from 0% to 0.5% . in our study , there were 2 jejunal atresia and 1 ileal atresia patients . the postulation for the coexistence of biliary atresia and jejunoileal atresia might be bacterial or viral infection in the peri or postnatal period . furthermore , the coexistence of esophageal and biliary atresia was seen in two patients ; the literature has reported this association rarely . biliary atresia is reported to occur in 28% of infants born with situs inversus as compared to the 0.01% of the general population and genetic factors were suggested for this coexistence . in our series , reviews conducted by silveira et al . on 237 biliary atresia patients reported associated structural lesions in 47 patients ( 19.8% ) this finding was confirmed by carmi et al . in a multicenter study of 251 biliary atresia cases in which 51 children ( 20.3% ) had associated anomalies . reported that 25 infants ( 45% ) showed several forms of congenital heart disease and nine infants ( 16% ) required cardiac surgery ; of these , the initial operation predated the biliary exploration in 7 . in the present series , only two patients showed structural cardiac anomalies ; however , echocardiography was not a part of the routine work - up of the patients with biliary atresia and these anomalies might get unnoticed unless looked for specifically . the existence of anomalies in distantly developing anatomic regions in patients with ehba supports the possibility of a  generalized  insult during embryogenesis rather than a  localized  defect . the distant anomalies , unless life threatening on their own part however , anomalies of gut rotation and those in and around the porta hepatis can adversely affect the performance of kp and hence the outcome of treatment . in the present study , the incidence of vascular anomalies at the porta hepatis , abdominal wall hernias , malrotation of intestine , and digestive anomalies was much more common as compared to splenic and cardiac anomalies as reported in other studies . in addition , male infants were observed to have a higher incidence of associated anomalies as compared with the female infants in contrast to other reported studies . the presence of anomalies did not affect the timing of the surgery which is important for favorable results .