NON-INVASIVE AND EARLY DIAGNOSIS OF BILIARY ATRESIA WITH SPECIAL REFERENCE TO THE ULTRASONOGRAPHIC ”TRIANGULAR CORD" SIGN
Woo-Hyun Park, M.D.
Dept. of Pediatric Surgery, Keimyung University Dongsan Medical Center, Taegu, Korea

Ultrasonography (US) often provides valuable information and has been performed as a first step in the work-up of the infants with jaundice. In the past, US of the gall bladder in the fasting infant was thought to be useful in distinguishing biliary atresia from neonatal hepatitis or other causes of infantile cholestasis but study with gall bladder shape has limited value because of its low diagnostic accuracy. Some authors stressed on the importance of the contractility of gall bladder in excluding biliary atresia. Although in infants with BA, the gall bladder is usually nonvisualized or small, it is not surprising to demonstrate contractility of the gall bladder after oral feeding, because a patent bile duct from the gall bladder to the duodenum (Kasai type 3a) can be seen in 19-35% of cases. With the development of high resolution grey-scale recording and real-time imaging technique with a 7.0 MHz or 10 MHz transducer, the author recently reported the sonographic feature of the fibrous remnant at the porta hepatis as a triangular or tubular echogenic density just cranial to the portal vein bifurcation on transverse or longitudinal scan in the infants with biliary atresia. We named this echogenic density the triangular cord (TC). This echogenic density was confirmed to represent the fibrous remnant in the porta hepatis of BA cases at surgery. Moreover, Guibaud et al. and Hatakejama et al. have shown that this triangular cord was clearly demonstrated on magnetic resonance (MR) cholangiography in infants with BA.
In our series of 79 infants with cholestasis (25 cases of biliary atresia and 54 cases of intrahepatic cholestasis ), the accuracy, positive predictive value, and negative predictive value of this TC in the diagnosis of biliary atresia were 94%, 95%, and 93%, respectively. As for positive predictive value in the diagnosis of biliary atresia by the ultrasonographic TC coupled with gall bladder shape, it was 100% when positive TC was coupled with an abnormal gall bladder and 88% when positive TC was coupled with a normal gall bladder. It decreased down to 25% when negative TC was coupled with an abnormal gall bladder and only 2% when negative TC was coupled with a normal gall bladder. In conclusion, we believe that the TC appears to be a simple, time-saving, highly reliable, and definite ultrasonographic finding in the early diagnosis of biliary atresia, representing a 95% positive predictive value. Moreover, if the TC is coupled with gall bladder images, more specific diagnostic prediction of biliary atresia on each combination can be anticipated. Now the author would like to propose a new diagnostic approach in the evaluation of infantile cholestasis with emphasis on the ultrasonographic TC and to discuss pitfalls associated with this proposal in detail.

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HEPATIC ARTERY RESISTANCE INDEX CAN PREDICT EARLY DEATH IN CHILDREN WITH BILIARY ATRESIA.
5E. Broide, 2P. Farrant, 4F.. Reid, 1A. Baker, 2H. Meire, 3M. Rela, 1M. Davenport, 3N. Heaton,1G. Mieli-Vergani.
1. Department of Child Health, Variety Club Children's Hospital. 2. The Radiology Department. 3. The Liver Transplantation Unit, King's College Hospital, Denmark Hill, London SE5 9RS. 4. The Department of Public Health and Epidemiology, KCSMD, Bessemer Rd, London, SE5 9PJ 5. The Institute of Gastroenterology and Liver Disease, Tel-Aviv University Medical School, Assaf Harofeh Medical Center, Zerifin Israel, 70300.

Hepatic artery resistance index has been measured by ultrasound Doppler and found to predict rapid deterioration and death in children with biliary atresia Clinical, biochemical, ultrasonographic and outcome data were collected prospectively and retrieved on 32 patients with resistance: index equal to or more than 1.0 (group A). These were compared with the same data for 32 age and sex matched patients with biliary atresia and resistance indexlessthan1.0(group B).
Group A was found to have significantly worse liver function tests than group B. ln group A all patients died (n=11) or underwent transplantation (n=21 of whom 4 died), compared with only 2 of group B who died, and 4 who were transplanted without fatality. Survival at 2 years was 52% in group A versus 94% in group B.
We suggest that regular ultrasound Doppler examination in patients with biliary atresia can detect a group with a resistance index >1.0 who have a very high risk of early mortality. Such patients require early evaluation and listing for transplantation. Those listed for liver transplantation on other grounds require 2-3 monthly ultrasound examinations with immediate upgrading of the priority of those found to have a resistance index of 1.0 or above.

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TPN-INDUCED HEPATOBILIARY DYSFUNCTION - MAJOR DIFFERENTIAL DIAGNOSIS TO BILIARY ATRESIA IN NEWBORN-ICU
S Loff, M.D., A Dzakovic, M.D., M Langbein, K-L Waag, M.D.
Kinderchirurgische Universitäts-Klinik, Klinikum Mannheim, Theodor-Kutzer-Ufer 1-3 D-68167 Mannheim

When newborns or prematures in ICU under total parenteral nutrition (TPN) develop direct hyperoilirubinemia, most common differential diagnosis are biliary atresia and TPN-induced hepatobiliary dysfunction, also known as TPN-associated cholestasis. Although the latter might finally end in cirrhosis as well, it usually resolves after reinstallation of full enteral support, whereas especially extrahepatic biliary atresia must be diagnosed and corrected at the age of eight weeks to prevent from cirrhosis. We studied cases of TPN-induced hepatobiliary dysfunction to assess typical clinical, radiological and operative findings for this entity to exclude biliary atresia.
Methods: We examined 10 patients with TPN-induced hepatobiliary dysfunction concerning onset and dynamics of symptoms, radiographic and invasive diagnostics and time point of exclusion of biliary atresia.
Results: All 10 infants underwent laparotomy and liver biopsy. 8 of them intraoperative cholangiography.
Laboratory examination: In all patients, TPN was started within the first 4 days of life. Decline of physiological unconjugated hyperbilirubinemia was incomplete in all and led to a conjugated bilirubinemia >2mg% after 4 weeks. Maximum peaks of conjugated bilirubine varied from 3.9mg% to 11.7mg% with a mean of 7.4mg% after a mean of 12 weeks of parenteral nutrition. In 6 cases, an obvious decline of conjugated bilirubine under the limit of 2mg% was observed 4-6 weeks after complete cessation of lipid infusions, probably indicating a causal relationship. In all patients, the rise and fall of direct bilirubine was accompanied without delay by GOT and GPT. For this reason, considerable cell destruction during hepatobiliary dysfunction must be assumed. Peak GOT/GPT levels varied from 54/49 U/l to 379/203 U/l with means of 148 U/l and 95 U/l respectively. Corresponding g-GT peak values were between 73 and 293U/1 (mean 166 U/l). These peaks did not necessarily coincide with GOT/GPT/bilirubine peaks.
Tc-Liverscan: In four infants Technetium-liverscan was performed for suspected biliary atresia at the age of 5, 8, 11 and 16 weeks because of rising laboratory parameters. In 3 of them no bilirubine excretion into the gut could be demonstrated and biliary atresia could not be excluded.
Histology: The age of our patients at the time of liver biopsy varied from 6 to 30 weeks. At 6 weeks we found intracellular cholestasis, slight bile duct proliferation and infiltration. At 8 weeks additionally canalicular cholestasis, distorsion of histoarchitecture, hydropic degeneration and beginning fibrosis. After 9 to 11 weeks fibrosis, hydropic degeneration and distorsion of architecture became more pronounced. After 14 to 17 weeks porto-portal fibrosis was detectable. The specimens at the age of 20 and 30 weeks showed slight to severe intracellular and canalicular cholestasis, little inflammation, fibrosis and bile duct proliferation. Intrahepatic atresia could be excluded in all cases. Extrahepatic atresia was expressively considered by the pathologist as differential diagnosis of the presented histopathologic changes.
Intraoperative cholangiography: Paracenthesis of the gallbladder with simple aspiration of bile and irrigation with saline solution were performed in 2 patients. 3 patients underwent the same procedure with additional cholangiography. In 5 patients, a cholecystostomy tube was implanted with cholangiography for repeated irrigation. In all cases, extrahepatic biliary atresia could be ruled out.
Conclusions: TPN-induced hepatobiliary dysfunction in premature infants should be acknowledged as one of the major differential diagnoses of biliary atresia. Its clinical signs and course can mimic those of biliary atresia. Laboratory parameters and liver scan are not appropriate diagnostic means for discrimination. While liver histology is essential to rule out intrahepatic atresia, intraoperative cholangiography has the highest sensitivity to exclude extrahepatic biliary atresia and establish the proper diagnosis.

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