Journal VOL.
of Pediatric
Surgery APRIL
N, NO. 2
1975
EDITORIAL
Progressive
Extrahepatic Biliary Obstruction of the Newborn
N THIS ISSUE we publish Morio Kasai’s long-term results of his operation for what he calls “inoperable biliary atresia.” It is a significant report with follow-up as long as 17 years. I traveled to Japan a year ago and saw his patients; everything he says about them can be verified.
I
KASAI
GETS THE CREDIT
Morio Kasai was my resident I7 years ago, by which time he had already operated successfully on the oldest patient reported here. While working at the Children’s Hospital of Philadelphia, Kasai made several other contributions to our knowledge of biliary atresia. Using serial biopsies which we had previously obtained and extending these observations, he put to rest the then believed difference between.intraand extrahepatic biliary atresia. He showed that socalled extrahepatic atresia became intrahepatic atresia if the patient lived long enough. In other words, he described an intrahepatic dynamic process. Now as we see and understand some of what Kasai has been telling us, it seems evident that there is also an extrahepatic dynamic process at work which renders an operative situation (portoenterostomy) inoperable if the opportunity is not used advantageously early enough in the life of the jaundiced infant. A LESION IN SEARCH OF A NAME It is clear that Kasai is not operating on biliary atresia. He knows it. we know it, and yet we persist in calling the pathologic entity hiliury atresia when we are dealing with hiliary hypoplasia. Atresia means “no hole.” And if there are no lumina, Kasai’s operation cannot work. If Kasai’s operation works, there had to be lumina: the diagnosis could not be biliary atresia. Yet /diary hypoplasia alone does not fit the bill. We see patients whose operative cholangiograms show visible ducts of small caliber, and we call this biliary hypoplasia with early hepatic fibrosis or with hepatic cirrhosis and ductal proliferation, or neonatal hepatitis, or some other variant which describes the situation. Some of these patients go on to die; yet others put out bile and have variable courses postoperatively in reference to their jaundice and its clearing as well as to the progression of the hepatic fibrosis. Kasai is talking about hypoplasia so far advanced that the ducts are not visible to the naked eye as cords with lumina. How about calling the lesion progressive estrahepatic hiliar! obstruction ~$~he newborn?
Journal
of Pediatnc
Surgery,
Vol
10.
No
2 (Aprd).
1975
169
EDITORIAL:
170
BILIARY
OBSTRUCTION
In the January 1975 issue of Pediatrics I wrote a commentary to accompany John Lilly’s report of experience with the Kasai 0peration.l In it I said, “If there are no such ducts, no operation of the Kasai type will work. The question I do not know how to answer is how many of those who get well with the Kasai operation would get well without it.” I wrote that in the spring of 1974. Further reflection on what I saw with Kasai in Sendai last year leads me to believe that the hypoplasia we see on cholangiography and the hypoplasia Kasai operates upon successfully are not the same lesion (at least in severity). I am not sure yet whether they represent stages in the progressive obstruction alluded to above. I suggest that they do and that they and true biliary atresia and neonatal hepatitis-so-called-are all different facets of the same in utero insult to the fetal liver. CONCLUSION
There are, I believe, the following biliary obstructive lesions that we might be called upon to manage in the early months of life: (1) Extrahepatic discontinuity of the biliary tree (atresia) due to a developmental accident to the extrahepatic biliary tree. The liver shows the same histologic picture as an acquired obstruction in an older child or adult. These lesions are correctable. (2) Biliary hypoplasia as diagnosed by operative cholangiogram. The ducts are smaller than normal to miniscule in size. The hepatic histology shows variation in severity of cirrhosis and ductal proliferation. Perhaps here, surgical judgment must decide whether the Kasai operation or a period of waiting, perhaps accompanied by steroid therapy, is the plan to be followed. If there is no loss of jaundice in 1 month, re-exploration and the Kasai operation seem indicated at this stage of our knowledge. (3) No extrahepatic bile ducts visible to the naked eye. Some of these will have microscopic lumina in biliary cords and will respond to the Kasai operation in accordance to the stage of the obstruction, the status of the liver, the skill with which the operation is performed, and the lack of postoperative cholangitis. Other patients will not have ducts even when looked for microscopically. If this is indeed a dynamic situation, these patients not only will not be improved by the Kasai operation but they will be p:ltients in whom the progressive extrahepatic biliary obstruction of lhe newborn has progressed too far to be amenable to surgical correction. C. EVERETT KOOP, Editor-in-Chief
M.D.,
SC.D.
of Pediatric
Surgery APRIL
N, NO. 2
1975
EDITORIAL
Progressive
Extrahepatic Biliary Obstruction of the Newborn
N THIS ISSUE we publish Morio Kasai’s long-term results of his operation for what he calls “inoperable biliary atresia.” It is a significant report with follow-up as long as 17 years. I traveled to Japan a year ago and saw his patients; everything he says about them can be verified.
I
KASAI
GETS THE CREDIT
Morio Kasai was my resident I7 years ago, by which time he had already operated successfully on the oldest patient reported here. While working at the Children’s Hospital of Philadelphia, Kasai made several other contributions to our knowledge of biliary atresia. Using serial biopsies which we had previously obtained and extending these observations, he put to rest the then believed difference between.intraand extrahepatic biliary atresia. He showed that socalled extrahepatic atresia became intrahepatic atresia if the patient lived long enough. In other words, he described an intrahepatic dynamic process. Now as we see and understand some of what Kasai has been telling us, it seems evident that there is also an extrahepatic dynamic process at work which renders an operative situation (portoenterostomy) inoperable if the opportunity is not used advantageously early enough in the life of the jaundiced infant. A LESION IN SEARCH OF A NAME It is clear that Kasai is not operating on biliary atresia. He knows it. we know it, and yet we persist in calling the pathologic entity hiliury atresia when we are dealing with hiliary hypoplasia. Atresia means “no hole.” And if there are no lumina, Kasai’s operation cannot work. If Kasai’s operation works, there had to be lumina: the diagnosis could not be biliary atresia. Yet /diary hypoplasia alone does not fit the bill. We see patients whose operative cholangiograms show visible ducts of small caliber, and we call this biliary hypoplasia with early hepatic fibrosis or with hepatic cirrhosis and ductal proliferation, or neonatal hepatitis, or some other variant which describes the situation. Some of these patients go on to die; yet others put out bile and have variable courses postoperatively in reference to their jaundice and its clearing as well as to the progression of the hepatic fibrosis. Kasai is talking about hypoplasia so far advanced that the ducts are not visible to the naked eye as cords with lumina. How about calling the lesion progressive estrahepatic hiliar! obstruction ~$~he newborn?
Journal
of Pediatnc
Surgery,
Vol
10.
No
2 (Aprd).
1975
169
EDITORIAL:
170
BILIARY
OBSTRUCTION
In the January 1975 issue of Pediatrics I wrote a commentary to accompany John Lilly’s report of experience with the Kasai 0peration.l In it I said, “If there are no such ducts, no operation of the Kasai type will work. The question I do not know how to answer is how many of those who get well with the Kasai operation would get well without it.” I wrote that in the spring of 1974. Further reflection on what I saw with Kasai in Sendai last year leads me to believe that the hypoplasia we see on cholangiography and the hypoplasia Kasai operates upon successfully are not the same lesion (at least in severity). I am not sure yet whether they represent stages in the progressive obstruction alluded to above. I suggest that they do and that they and true biliary atresia and neonatal hepatitis-so-called-are all different facets of the same in utero insult to the fetal liver. CONCLUSION
There are, I believe, the following biliary obstructive lesions that we might be called upon to manage in the early months of life: (1) Extrahepatic discontinuity of the biliary tree (atresia) due to a developmental accident to the extrahepatic biliary tree. The liver shows the same histologic picture as an acquired obstruction in an older child or adult. These lesions are correctable. (2) Biliary hypoplasia as diagnosed by operative cholangiogram. The ducts are smaller than normal to miniscule in size. The hepatic histology shows variation in severity of cirrhosis and ductal proliferation. Perhaps here, surgical judgment must decide whether the Kasai operation or a period of waiting, perhaps accompanied by steroid therapy, is the plan to be followed. If there is no loss of jaundice in 1 month, re-exploration and the Kasai operation seem indicated at this stage of our knowledge. (3) No extrahepatic bile ducts visible to the naked eye. Some of these will have microscopic lumina in biliary cords and will respond to the Kasai operation in accordance to the stage of the obstruction, the status of the liver, the skill with which the operation is performed, and the lack of postoperative cholangitis. Other patients will not have ducts even when looked for microscopically. If this is indeed a dynamic situation, these patients not only will not be improved by the Kasai operation but they will be p:ltients in whom the progressive extrahepatic biliary obstruction of lhe newborn has progressed too far to be amenable to surgical correction. C. EVERETT KOOP, Editor-in-Chief
M.D.,
SC.D.
