Billiary atresia cases among attendants of Menoufiya National Liver Institute: [a retrospective study]

This study included 126 cases of neonatal cholestasis syndrome [NCS] from the attendants of Pediatric Department of the National Liver Institute, Menoufiya University from 1994 up to 2004 . They were divided into two groups: First group[1stGr.] included 58 biliary atresia [BA] cases [46%] and the second group [2ndGr.] included 68 [54%] were due to other causes of NCS . The etiology of NCS due to causes other than BA were as follows: 28 cases [22.22%] neonatal hepatitis, 11 cases [8.73%] septicemia, 8 cases [6.35%] paucity of intrahepatic bile ducts, 5 cases [3.97%] inspessated bile syndrome, 4 cases [3.17%] choledochal cyst, 2 cases [1.59%] Byler's disease, 2 cases [1.59%] galactosemia, 2 cases ? 1 antitrypsin deficiency [1.59%], 1 case [0.79%] Alagille syndrome, and 5 cases [3.97%] due to unknown causes. Onset of jaundice whether early or late does not differentiate cholestatic cases due to BA from other causes of NCS.Clay coloured [acholic] stools were more frequently detected in BA cases [77.6%] than 2ndGr. [27.9%] [p<0.05]. Triangular cord sign [Tc sign] and absence of and/or gall bladder abnormality were detected in significantly higher proportion of BA group more than the 2ndGr.[p<0.05]. By histopathological examination portal tract fibrosis, bile duct proliferation, bile plugs in portal ductules and preservation of hepatic lobular architecture were detected more frequently among BA cases than the other group, while interface hepatitis and giant cell detection were observed more frequently among 2ndGr. than BA cases [p < 0.05]. The mean of alkaline phosphatase and gamma glutamyl transpeptidase was found to be statistically higher among BA group than that of the 2ndGr. [p<0.05] . Kasai operation was done for only 20 cases of BA cases [34.5%] and the mean of age of patients at time of operation was 75 days +/- 17.8. Complications encountered in BA cases were recurrent cholangitis, ascites, itching, coagulopathy, hematemesis and end-stage liver failure in 48%, 50%, 41.7%, 39.6, 18.8% and 54.2% of cases respectively. In conclution, the results of the present study indicate that clinical evaluation by an experienced pediatric hepatologist and liver biopsy together with careful ultrasonographic evaluation are considered as the most reliable methods for early differentiation of BA from other causes of neonatal cholestasis. Management of BA cases will be improved by public and professional education to encourage early referral of infants with neonatal cholestasis [>14 days] to specilised liver centers for early diagnosis to facilitate initial surgery before 8 weeks of age

Interposition jejunal loop with valve for management of biliary atresia

Kasai's operation has changed significantly the prognosis of patients with biliary atersia. However, postoperative ascending cholangitis is still a relatively frequent complication with high morbidity and mortality rates. Several surgical procedures to overcome the high incidence of postoperative ascending cholangitis after Kasai operation have been introduced. The aim of this study is to investigate the effect of the jejunal interposition hepatic portoduodenostomy with an intussusception type intestinal valve on the incidence of the post-operative cholangitis in cases of biliary atresia at our institute. Ten cases of biliary atresia [from June 1995 to January 1997], were included in the study. Seven were females and 3 males, with mean age at the time of operation of 67.8 days and mean body weight of 4.37 Kgm. There was no intraoperative mortality, while hospital mortality was 30%; the cause of death was not related to the surgical procedure. The remaining 7 patients were followed up for a period ranged from 6 to 17 months with a mean of 10.7. Three [42.8%] of the 7 patients did not develop any attacks of cholangitis, the other 3 developed one attack of grade I; cholangitis and the remaining one had 4 attacks of grade II; cholangitis and reoperation for him was considered. Upper GIT study using Gastrografin demonstrated competence of the artificially constructed valve in all survivors. The results obtained in this study demonstrate that interposition jejunal loop with an anti-reflux intestinal valve has prevented the incidence of cholangitis and reduced the number as well as the severity of the attacks of cholangitis in 85.7% of the studied patients with biliary aterisa