Tsuneo Chiba

Long-term follow-up after surgery for patients with biliary atresia☆

Long-term results after surgery for biliary atresia (BA) in 48 patients, ranging in age from 10 to 33 years, were examined. There were 19 males and 29 females. Twelve had correctable type BA and 36 had the noncorrectable type. Forty-one cases had no jaundice; seven did. Thirty-seven of the 48 cases were leading normal lives. Among them, six cases were enjoying their lives after overcoming sequelae, such as portal hypertension. The main morbidities of the remaining 11 long-term survivors were jaundice and portal hypertension. The growth of most cases were comparable to those of the normal Japanese population. The data of liver function tests were variable and disclosed a moderate degree of abnormality in patients mainly complicated by cholangitis. Eleven cases, including six jaundice cases, required treatment for esophageal varices and/or hypersplenism. In conclusion, the cured states of most cases without jaundice are satisfactory and these former patients have achieved a favorable quality of life. Early operations are essential to obtain good short-term results as well as good long-term results.

Surgical limitation for biliary atresia: indication for liver transplantation

Of 245 patients undergoing corrective operations for biliary atresia, jaundice was cleared in 113. In January 1988, 84 of them were living and free of jaundice and the other 19 were alive with jaundice. A vast majority of long-term survivors showed normal growth and development, and were leading normal lives for their respective ages. Portal hypertension, a common late complication, improved spontaneously or after sclerotherapy in jaundice-free patients. Therefore, liver transplantation is not recommended for jaundice-free patients even with esophageal varices. Patients with persistent severe jaundice (serum bilirubin over 10 mg/dL) and those with moderate jaundice (serum bilirubin 5 to 10 mg/dL) and severe esophageal varices require liver transplantation. Patients with moderate jaundice having no or slight varices should be carefully followed. When varices become worse or serum bilirubin rises, liver transplantation is indicated. Patients with mild jaundice (serum bilirubin lower than 5 mg/dL) have a possibility of improvement in their condition before the age of 15 years, and are not recommended for liver transplantation. The high value of the lowest postoperative bilirubin level suggests the necessity of liver transplantation in early childhood. Liver transplantation as the primary treatment for biliary atresia may be indicated only for patients over 120 days of age with an enlarged and hard liver.

Surgical treatment of congenital dilatation of the bile duct with special reference to late complications after total excisional operation

The surgical treatment of 100 cases with congenital dilatation of bile duct with special reference to late complications was analyzed. There were no deaths nor occurrences of malignancy. Among 91 patients who had undergone the standard operation, namely total excision of the dilated extrahepatic bile duct and reconstruction after Roux-en-Y hepaticojejunostomy, there were one early complication (pancreatic juice leakage) and five late complications (four intrahepatic gallstones and one liver abscess). The cause of intrahepatic gallstone formation after a total excisional operation was attributed to the remaining intrahepatic bile duct dilatation and the stenosis located between the intrahepatic bile duct dilatation and the common hepatic duct. Accordingly, these results support the total excisional procedure for this condition; however, with regard to the cases associated with cystic dilatation of intrahepatic bile ducts, completely free bile drainage from the dilated intrahepatic biliary system should be performed at the radical operation.

Long-term follow-up study of patients with cholangitis after successful Kasai operation in biliary atresia: selection of recipients for liver transplantation.

Since the introduction of cyclosporine A, liver transplantation has become accepted as the therapy for end-stage liver disease. However, there are no definite criteria for liver replacement in biliary atresia. We investigated (a) the survival rate after hepatic portoenterostomy (n = 131), (b) liver function tests in fatal cases after an initially successful hepatic portoenterostomy (n = 9), and (c) liver function tests in the patients with episodes of cholangitis after a successful surgical treatment (n = 8). Patients with persisting jaundice after the surgery cannot be expected to survive long, and therefore they definitely should undergo liver transplantation. When total bilirubin concentration was above 10 mg/dl in patients with cholangitis after a successful operation, conservative therapy had almost no effect. Therefore, patients with total bilirubin levels above 10 mg/dl should be considered for liver transplantation. Of the liver function tests, only total bilirubin was reliable as a marker for hepatic failure in the end stage of biliary atresia. Prolongation of thrombo test and episodes of gastrointestinal bleeding also were used in selection of patients for liver replacement.

Bannayan-Zonana syndrome associated with lipomas, hemangiomas, and lymphangiomas.

Bannayan-Zonana syndrome is a rare disorder characterized by macrocephaly and multiple soft tissue and visceral hamartomas. This report presents a sporadic patient with macrocephaly, lipomas, hemangiomas, and lymphangiomas who died of cardiac and respiratory failure due to progressive cervicomediastinal arteriovenous fistulous hemangiomas at the age of 9 years.

A patient with biliary atresia who died 28 years after hepatic portojejunostomy.

A patient who died of hepatic failure nearly 29 years after hepatic portojejunostomy is reported. His death may be attributed to several faults in treatment. From our experiences it can be said that biliary atresia is a curable disease, although liver-transplantation is required in some patients in whom hepato portoenterostomy is unsuccessful.

Mesenteric teratoma in an 8-month-old girl

The authors report the case of a solid, mature teratoma of the mesentery occurring in an 8-month-old girl. The tumor was enucleated from the ileocecal mesentery. Histologically the tumor included all three embryonal layers. This is the youngest patient reported to have mesenteric teratoma.

Differentiation of biliary atresia from neonatal hepatitis by routine clinical examinations.

After analysis of 33 cases of neonatal hepatitis and 90 cases of biliary atresia, a score test from history, physical findings and daily laboratory examinations was studied for the differential diagnosis of these two diseases. The biliary atresia takes plus score, whereas the neonatal hepatitis takes minus score. The score test is of great value for the differential diagnosis between these two diseases.

Cholangiographic study of the pancreaticobiliary ductal junction in biliary atresia

The size and arrangement of each component of pancreaticobiliary ductal junction was investigated in 28 cases with biliary atresia in which cholangiography allowed visualization of the common bile duct, pancreatic duct, and duodenum and in which relevant data were available. As controls, seven cases of neonatal hepatitis and eight cases of intrahepatic bile duct hypoplasia were also studied. The following results were obtained. (1) The diameter of the common bile duct was shortest in biliary atresia, the value corresponding to about one-third of that for neonatal hepatitis. (2) The common bile duct was visualized in 100% of cases of intrahepatic bile duct hypoplasia and 78% of cases of biliary atresia, whereas the corresponding percentage was only 43% for neonatal hepatitis. The mean length of the common channel in cases of biliary atresia and that of intrahepatic bile duct hypoplasia was about 5 mm, which was longer than that of neonatal hepatitis. (3) The pancreatic duct type, in which the pancreatic duct merged into the bile duct, was frequent in cases of biliary atresia.

Gastric teratoma extending into the mediastinum

This is a case report of gastric teratoma of which a larger part extended into the mediastinum.