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 .
 background / purpose : this study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia ( ehba ) , compare their frequency with those quoted in the existing literature and assess their role in the overall management.materials and methods : a retrospective study was performed on 137 infants who underwent the kasai procedure for ehba during the past 12 years . 
 the medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the ehba.results:of the137 infants , 40 ( 29.2% ) were diagnosed as having 58 anomalies . 
 the majority of patients had presented in the 3rd month of life ; mean age was 81  33 days ( range = 20 - 150 days ) . 
 there were 32 males and 8 females ; boys with ehba had a higher incidence of associated anomalies . 
 of these 40 patients , 22 ( 37.9% ) had vascular anomalies , 13 patients ( 22.4% ) had hernias ( umbilical-10 , inguinal-3 ) , 7 patients ( 12.1% ) had intestinal malrotation , 4 patients ( 6.8% ) had choledochal cyst , 1 patient ( 1.7% ) had meckel 's diverticulum , 3 patients ( 5% ) had undergone prior treatment for jejunoileal atresias ( jejunal-2 , ileal-1 ) , 2 patients ( 3.4% ) had undergone prior treatment for esophageal atresia and tracheoesophageal fistula , 2 patients ( 3.4% ) had spleniculi , and 2 patients ( 3.4% ) were diagnosed as having situs inversus.conclusions:the most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system . 
 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 . 
 in addition , male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports . 
INTRODUCTION
MATERIALS AND METHODS
RESULTS
DISCUSSION
CONCLUSION