Biliary atresia is a rare disease characterized by obstruction of the intrahepatic and extrahepatic bile ducts. What are the symptoms ? The causes ? The treatment ? Insight with Pr Olivier Goulet, gastroenterologist and hepatologist at the Necker-Enfants Malades hospital.
Definition: what is biliary atresia?
Biliary atresia is a rare disease linked to the degradation and involution of the intra- and extra-hepatic bile ducts. These become more and more narrow until their total obstruction, causing the stagnation of bile in the liver which defines cholestasis which, in the absence of treatment, leads to fibrosis followed by hepatic cirrhosis and, in the absence of liver transplant, at death.
What causes biliary atresia?
“The origin of biliary atresia is, to date, not really identified. There are different hypotheses but none dominates. Of the toxic as well as certain genetic abnormalities might be in question. It is a very debilitating disease which has not evolved much in the knowledge of its origin for more than forty years that I have known it. She is not genetically identifiable and she is not accessible to prenatal diagnosis“says Professor Olivier Goulet, gastroenterologist and hepatologist.
What are the symptoms of biliary atresia?
Biliary atresia is manifested in children by a jaundicethat is, the “jaundice“. Jaundice”usual” of the newborn disappears quickly, whereas this one does not attenuate at the end of fifteen days and is even accentuated, accompanying itself usually but not always (trap), discolored stools (putty color).
What are the complications of biliary atresia?
“The main complication of biliary atresia is complete obstruction of the bile ducts which quickly leads to cirrhosis even deathin the medium term, of the child in the absence of liver transplantation“, says Professor Olivier Goulet.
The diagnosis is evoked before newborn jaundice, associated with discolored stools. Biologically, it is conjugated bilirubin jaundice. All of these symptoms should immediately suggest biliary atresia. Liver ultrasound, possibly supplemented by a liver biopsy, confirms the diagnosis. The most common cause of cholestasis neonatal (jaundice with conjugated bilirubin, associated with discolored stools), is biliary atresia. She constitutes almost a therapeutic emergency…These are easily identifiable symptoms even by a mother. “Neonatal jaundice is misleading because it is mostly regressive and is related to incompatibilities in the ABO group“, says the specialist.
The treatment must be carried out as soon as possible, before the age of 6 weeks
What is the treatment for biliary atresia?
The initial treatment, and who should be done as early as possible, preferably before six weeks of agerests on a surgical procedure which consists of mounting an intestinal loop at the level of the hepatic hilum (hepato-porto-enterostomy) so as to drain the intra-hepatic bile ducts before they are completely obstructed. At the end of this intervention which bears the name of the Japanese who developed it (Kasaï), the result is very variable according to the age of the child on the date of its realization and the possible post-operative complications. and in the longer term (intrahepatic bile duct infections).
What is the life expectancy in case of biliary atresia?
20 to 25% of Kasaï interventions allow a lasting positive evolution. However, performing Kasai too late and/or repeating biliary tract infections leads inexorably to end-stage cirrhosis requiring liver transplantation foie. “After transplantation, the best teams get “more than 95% of “good results” (survival of grafts and children). It is essential to insist on the role of all the actors, from the midwife to the mother, passing through the hepatologists and the surgeons, in short, all those who will look into the newborn in the days following his birth. From the moment when the diagnosis is suspected, there are simple ways to confirm this diagnosis and to move, as quickly as possible, towards the intervention of Kasaï. In France, the two major liver transplant centers are located the Bicêtre children’s hospital and the Necker-Enfants Malades hospital. Living-donor liver transplantation is possible in children, significantly increasing survival“, insists the specialist
Thanks to Pr Olivier Goulet, gastroenterologist and hepatologist at the Necker-Enfants Malades hospital.
