Analysis of the clinical features and diagnostic criteria for suspected cases of biliary atresia

Abstract

Objective To analyse the clinical features and causes of misdiagnosis of biliary atresia in a retrospective study.Methods Six hundred and two infants who were suspected to have biliary atresia were recruited into our study from 2004 to 2010.All cases were divided into non-biliary atresia group and biliary atresia group according to intraoperative cholangiography findings.The annual proportion of patients was calculated,while the time of the occurrence of juandice,liver function and ultrasound results in two groups were compared.Moreover,the positive predictive value and false positive rate of hepatobiliary scintigraphy in biliary atresia were calculated.In addition,the disease spectrum of non-biliary atresia group patients was analysed.Results In the 602 cases,83 patients were diagnosed to be in non-biliary atresia group.The remaining 519 cases were comfirmed to have biliary atresia.There was no significant decline in the proportion of non-biliary atresia in all cases by years.Time of the occurrence of jaundice,TBIL(169.9 mmol/L vs 172.3 mmol/L,P0.05),DBIL(128.7mmol/L vs 132.5 mmol/L,P＞0.05),DBIL/TBIL(0.76 vs 0.77,P＞0.05)and ALT(141.3 mmol/L vs 114.9 mmol/L,P＞0.05)values before cholangiography showed no statistically significant difference between these two groups.However,the mean level of γ-GT was 263.2 mmol/l in non-biliary atresia group while 902.7 mmol/l in biliary atresia group(P＜0.01).The liver size below the costal margin detected with ultrasound was smaller in non-biliary atresia group than that in biliary atresia group(2.99 cm and 3.61 cm respectively,P＜0.05).Among the 498 infants who received hepatobiliary scintigraphy examination,the false positive rate was 13.3％(66/498)and the positive predictive value was 86.7％(432/498).In the non-biliary atresia group,58 infants suffered from hepatitis syndrome,16 cases were biliary dysplasia,5 cases were TPN related cholestasis,two cases were bile duct perforation and two were Bile-Plug Syndrome.Conclusions The similarity of liver function tests and excessive dependence on hepatobiliary scintigraphy examination might cause the misdiagnosis of patients with jaundice.The time of jaundice occurrence,the level of γ-GT and liver size below the costal in the ultrasound are helpful in diagnosis for these jaundice infants. Key words: Biliary atresia; Differential diagnosis; Suspected cases