Biliary atresia is a serious disease characterized by occlusion of different segments of the intra- and extrahepatic bile ducts in the neonatal period, and if left untreated, the child will die around 2 years of age. Southern China, Taiwan and Southeast Asia are the regions with higher incidence of biliary atresia. Our hospital, Zhongshan First Hospital, was the first hospital in South China to carry out surgical treatment of biliary atresia, and is now an important center in South China for the admission of biliary atresia. I myself often need to deal with it in my work. Now I have organized the common problems encountered in the clinic and share them with you. If there are any mistakes or omissions, please make more comments.
1.Why do children get biliary atresia?
In fact, this question is not only troubling to many parents of children, but also to many clinical medical experts. To be honest, the exact cause of biliary atresia is not yet understood. Not only biliary atresia, but most of the causes of the disease are not understood by current medicine. But then again, we can still talk about the possible causes of biliary atresia.
Based on current research, biliary atresia can be broadly classified into two categories. One type may be due to a genetic defect, usually combined with a splenic malformation, also known as biliary atresia splenic malformation syndrome. This type of biliary atresia may occur early in pregnancy when the bile duct is atretic. The other type is due to perinatal pathogenic factors, such as viral infections, which cause an inflammatory response in the bile ducts leading to progressive atresia.
2. Is biliary atresia a congenital disease?
Combined with the answers above, it can be determined that some biliary atresia is congenital, which is manifested by jaundice at birth and does not subside. However, there are also some that may be caused after birth. I have seen such cases clinically, where there was a period of physiological jaundice that could subside after birth, followed by persistent elevated jaundice, where medical treatment was ineffective, and later surgical exploration revealed a biliary atresia. Therefore, not all cases of biliary atresia can be called “congenital biliary atresia”.
3. How is biliary atresia diagnosed?
If your newborn has persistent jaundice with white stools, you should suspect biliary atresia and go to a regular hospital. If ultrasonography reveals abnormalities in the gallbladder and fibrous masses in the hilar region, biliary atresia should be highly suspected and should be seen at a specialized hospital or a general hospital with pediatric surgery to perform a surgical investigation to exclude biliary atresia. It should be noted that surgical investigation and cholangiography (including percutaneous cholangiography and intraoperative cholangiography) are the means to confirm the diagnosis of biliary atresia, while other noninvasive methods are not. Therefore, even if the ultrasound does not indicate biliary atresia, if the jaundice does not subside with medical treatment and other factors causing the jaundice are ruled out, surgical consultation should be sought. Currently, pediatric surgeons recommend that if neonatal jaundice does not improve after one week of continuous medical treatment, surgical investigation should be performed.
4. What is the value of ultrasound in diagnosing biliary atresia?
Biliary atresia is an abnormality of the biliary system, which can be manifested by abnormal gallbladder development and inflammatory response of the bile ducts in the porta hepatis. The use of ultrasound to visualize the abnormalities of the gallbladder and the thickening caused by inflammation of the bile ducts in the hilar region can help diagnose biliary atresia. However, ultrasound is also a diagnostic tool that depends on the experience of the operator. For experienced physicians, the accuracy of diagnosing biliary atresia with ultrasound can reach over 90%. For inexperienced physicians, there may be more misdiagnoses and missed diagnoses. At present, most of the biliary atresia in Zhongshan First Hospital only do ultrasound examination before surgery.
5.What is the value of other imaging methods to diagnose biliary atresia?
CT is not helpful at all in diagnosing biliary atresia.
(1) Magnetic resonance water imaging (MRCP) can observe the presence of common bile duct (absence of common bile duct in some biliary atresia) and has some value for the diagnosis of biliary atresia. However, because it is expensive and requires absolute sedation (any movement of the child during the examination can affect the quality of the imaging), it is currently less used.
(2) Nuclear imaging (SPECT) is of greater value in the diagnosis of biliary atresia, but it has a high specificity and a low sensitivity. That is, when the nuclide is considered to be biliary atresia, it is not necessarily true; when the nuclide is not considered to be biliary atresia, biliary atresia can basically be ruled out. Another disadvantage of nuclide is that it is radioactive.
Currently, ultrasound is recognized as the tool of choice for the diagnosis of biliary atresia. Ultrasound is radiation-free, does not require sedation, and is inexpensive, and a more definitive conclusion can basically be obtained in the hands of an experienced ultrasonographer.
6.How do I need to prepare for the ultrasound examination of suspected biliary atresia?
Generally speaking, you need to fast from milk for 4 hours before the examination, not 8 hours like adults. During the examination, you can prepare a bottle of milk for your baby, so that your baby can cooperate with the examination quietly without crying. If you are breastfeeding, you need to trouble the mother to feed the baby during the examination.
7. Can all other diseases causing jaundice be cured by medication except biliary atresia?
Many parents ask, “If biliary atresia is ruled out, can we assume that the child can be cured by medication?
No, it does not. In addition to biliary atresia, there are other jaundice disorders for which there is no effective medical treatment. For example, familial cholestatic syndrome/intrahepatic biliary dysplasia and some specific metabolic diseases, medication does not work and liver transplantation is required to cure them. In addition, some jaundice is caused by abnormal intrahepatic portal vein shunts, which cannot be cured by pharmacological treatment. Ultrasonography can visually detect such portal shunts.
8.What are the treatments for biliary atresia?
The pathogenesis of biliary atresia is the occlusion of the extrahepatic bile duct, which leads to the inability of intrahepatic bile to be secreted through the bile duct and causes a series of metabolic disorders in the intestine. To use an inappropriate analogy, the extrahepatic biliary tract is like a river, and bile is the river water. When the river is blocked, it must be opened to prevent the river water from spilling out of the riverbed. In biliary atresia surgery, the way to open the river is a hilar-intestinal anastomosis, in which the atretic bile duct (i.e., fibrous mass) is removed from the hilar region and the intestine is then anastomosed to the hilar parenchyma. Some people may ask why the bile duct is not anastomosed directly to the intestine, as is done in adults with choledo-enteric anastomosis. This is because the intrahepatic bile ducts in children with biliary atresia are also often poorly developed and too slender to make an anastomosis. This procedure was first invented by the Japanese surgeon Kasai, so it is also known as Kasai’s technique, and in Chinese it is called Kasai’s technique.
It is important to note that Kasai is a palliative surgery. The radical cure for biliary atresia is liver transplantation. In Western countries, the most common cause of liver transplantation in children is biliary atresia. In recent years, more and more liver transplants for biliary atresia have been performed in China. I have seen many parents bring their children to Tianjin, Beijing and even Taiwan for liver transplantation.
9. What is the efficacy of the Geschi procedure (hepatic hilar anastomosis)?
There is a 1/3 theory about the efficacy of Geschi operation. In other words, 1/3 of all the children who underwent Geschi’s operation are ineffective, that is, they cannot establish effective bile drainage and cannot slow down the progression of cirrhosis; 1/3 can establish effective bile drainage but cannot slow down the progression of cirrhosis; and 1/3 are effective, that is, they can establish effective bile drainage and can survive with their own liver for a longer period of time.
Why is this the case? This may be related to the complexity of the etiology of biliary atresia. First, the current premise of the effective treatment with Geschi operation assumes that the intrahepatic bile duct is patent, but in fact biliary atresia can be a simultaneous occlusion of different segments of intrahepatic and extrahepatic bile ducts. Secondly, some scholars believe that the persistent inflammatory response of the biliary tract is an important cause of the progression of cirrhosis after Geschi operation.
10.How to choose the treatment?
If you have good financial condition and can find suitable liver source, liver transplantation is the first choice. Nowadays, the technology of liver transplantation for children is very mature and the success rate is also very high. I have seen several children who went to Beijing and Tianjin for surgery, and a large part of them had pro-liver transplantation (the mother or father donated the left outer lobe to the child), all of which were very successful.
For those who are not financially well-off enough to afford liver transplantation, it is recommended to opt for Gexi surgery. Later, when conditions are ripe, liver transplantation will be performed. Studies have shown that the more the average number of surgeries per year, the better the surgical outcome of the center with the same Gussy procedure. Therefore, the Gussy procedure should be performed in hospitals in China where this procedure is performed earlier and more often.