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At least 19 recordsLinked to original sources
PubMed1994

Hepatic calcification in an anicteric patient with biliary atresia after hepatic portoenterostomy.

Hepatic calcification was found in an anicteric infant with biliary atresia after hepatic portoenterostomy. When she was 2 years of age, ultrasonography detected an echogenic structure with acoustic shadowing. Computerized tomography located it at the proximal portion of Segment 6 in the right lobe. Although both techniques failed to exhibit dilatation of the peripheral bile ducts, we believe intrahepatic cholelithiasis provides the most likely diagnosis. Although seven cases with biliary atresia were reported to have this complication, all of them were icteric when the diagnosis was confirmed. The patient should be carefully followed up since the succeeding stones may cause jaundice by obstructing the main duct.

Biliary Atresia↗
PubMed2004

Evidence for segmental bile drainage by hepatic portoenterostomy for biliary atresia: Cholangiographic, hepatic venographic, and histologic evaluation of the liver taken at liver transplantation.

BACKGROUND: The result of hepatic portoenterostomy for biliary atresia (BA) has improved, but there are some patients who experience worsened liver function in the long term after one decrease in jaundice owing to portoenterostomy. However, the cause of the liver dysfunction in the long term has not been clearly ascertained. METHODS: Five patients (5 to 28 years of age) with BA underwent liver transplantation (LT) because of liver dysfunction after successful portoenterostomy. To clarify the cause of liver dysfunction occurring in the long term, the authors performed a cholangiogram, hepatic venogram, and macroscopic/microscopic examination of the liver just after LT. RESULTS: (1) Macroscopically, the liver could be divided into 3 areas, the hypertrophic, atrophic, and intermediate, with findings between those of the hypertrophic and atrophic areas. (2) The divided areas clearly corresponded to the liver segments. Segment IV was the hypertrophic area in all patients, but segments VI and VII were the atrophic areas in 4 of the 5 patients. (3) Based on the cholangiographic and microscopic findings, the hypertrophic area had near-normal structure with bile ducts. The atrophic area had severe fibrosis and contained only a few bile ducts in the intralobular spaces of liver. CONCLUSIONS: It seems that segmental bile drainage must have been established by hepatic portoenterostomy in some patients and that some postoperative patients might have worsened liver function in the long-term follow-up period accompanied with progression of fibrosis and impaired bile drainage. These pathologic changes occur in each liver segment.

Adolescent↗
PubMed2006

Hepatolithiasis after hepatic portoenterostomy for biliary atresia.

BACKGROUND: Hepatolithiasis after hepatic portoenterostomy for biliary atresia has been paid little attention, with only 22 reported cases. PATIENTS AND METHODS: Fifteen patients underwent living-related liver transplantation for biliary atresia after hepatic portoenterostomy in our hospital between 1998 and 2004. The resected livers were examined for the existence and location of hepatolithiasis, composition of the calculi, and bacterial infection of bile. The relation between a history of cholangitis and the presence of hepatolithiasis was analyzed. RESULTS: Intrahepatic calculi were found in 8 (53%) of 15 patients. The calculi consisted of almost 100% calcium bilirubinate. Calculi were found in bile lakes in 8 patients. Bacteria were present in the bile in 8 (53%) of the 15 patients. Of the 8 patients, 7 (88%) had a history of ascending cholangitis. CONCLUSIONS: Hepatolithiasis occurs after hepatic portoenterostomy for biliary atresia more frequently than previously thought. Bile stasis and possibly bile infection are the main causes of calculi formation.

Adolescent↗
PubMed2006

Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia.

We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5-0 double-needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin, <2 mg/dL) was achieved in 13 (92.9%) of 14 patients.

Biliary Atresia↗
PubMed1976

Treatment of biliary atresia: microsurgery for hepatic portoenterostomy.

Microsurgery was used for hepatic portoenterostomy as the treatment of biliary atresia in 46 patients whom we treated since 1972. The microscope had a higher power (eight or ten times) magnification. As for the results of the operative procedure with the microscope, of 46 patients, 32 (70 percent) showed satisfactory bile flow postoperatively.

Common Bile Duct↗
PubMed1989

Intrahepatic biliary cysts after hepatic portoenterostomy in four children with biliary atresia.

We report our experience with 4 cases of cystic dilatation of intrahepatic bile ducts following hepatic portoenterostomy for biliary atresia. Two of the cases did not achieve satisfactory bile excretion and all four cases developed recurrent cholangitis after hepatic portoenterostomy. The attacks of cholangitis seemed to be associated with the presence of intrahepatic cysts. Although one case resulted in death from hepatic failure, three other cases are now outpatients. Patients who develop recurrent cholangitis following hepatic portoenterostomy, should be examined to exclude the presence of intrahepatic biliary cysts. Ultrasonography, computed tomography and percutaneous transhepatic cholangiography were all effective in detecting cysts and provided valuable information for planning treatment. Percutaneous transhepatic or surgical drainage of the bile ducts was effective in reducing jaundice, and recurrent cholangitis.

Bile Duct Diseases↗
PubMed2001

Outcome of early hepatic portoenterostomy for biliary atresia.

BACKGROUND: The outcome of the hepatic portoenterostomy (Kasai) procedure for biliary atresia is improved when it is performed before 90 days of age. However, it is not known whether intervention before 30 days is better than intervention between 30 and 90 days. METHODS: The authors reviewed the records of all patients seen by the Pediatric Gastroenterology Service at St. Louis Children's Hospital from 1984-1999 to ascertain the outcome of patients who underwent Kasai procedure before or after 30 days of age. RESULTS: Of 92 patients with biliary atresia treated at St. Louis Children's Hospital over 15 years, 9 underwent the Kasai procedure before 30 days of age. Liver transplantation was necessary in 77.8% of these patients at a mean age of 11.0 +/- 4.26 months, as compared with 53.4% at 32.14 +/- 7.14 months for the remainder of the patients who underwent the procedure after 30 days of age. CONCLUSIONS: Although these data suggest that outcomes are worse for patients who undergo the procedure before 30 days of age, they may reflect a difference in the pathogenesis of biliary atresia that brings it to clinical attention earlier and may provide further evidence that biliary atresia is a phenotype for a number of distinct underlying disease processes.

Age Factors↗
PubMed1989

Granulation at the porta hepatis following hepatic portoenterostomy for biliary atresia: the healing of experimental hepatoenterostomy.

After hepatic portoenterostomy for biliary atresia, granulation that formed at the porta hepatis caused biliary obstruction in seven out of 27 patients (26%). Six of the seven developed the complication during the first 6 weeks after surgery. The mortality rate was 29% (2/7). Among the same group, the incidence and mortality rate of ascending cholangitis was 19% (5/27) and 20% (1/5), respectively. The features characteristic of biliary obstruction caused by granulation, as compared with those of ascending cholangitis, were the absence of signs of infection and the lack of response to medical treatment. Quantitative analysis of the healing process of an experimental hepatoenterostomy in the rat showed that the mean thickness of the granulation formed at the anastomotic site (area, 5 x 20 mm) decreased almost constantly--from 1.49 mm on day 7 to 0.64 mm on day 42. Mucosa covered 20% of the granulation on day 7, 55% on day 14, 63% on day 21, 76% on day 28, and 92% on day 42. The increase in coverage was greatest during the second postoperative week. The results suggested that the healing of the hepatoenteostomy should be almost complete within 6 weeks. The operative method and postoperative management to prevent excessive granulation should be chosen so as to decrease the incidence of postoperative biliary obstruction.

Animals↗
PubMed2007

Intrahepatic cystic lesions after hepatic portoenterostomy for biliary atresia with bile lake and dilated bile ducts.

BACKGROUND: Intrahepatic cystic lesions develop after hepatic portoenterostomy in some patients with biliary atresia and have been regarded as an indication of poor prognosis; however, there is confusion about the terminology and concepts of the lesions. We investigated whether the cystic lesions constituted a single entity. PATIENTS AND METHODS: From 1980 to 2005, we encountered 80 patients with biliary atresia. Abdominal ultrasonography disclosed intrahepatic cystic lesions in 20 (25%) patients after hepatic portoenterostomy. The cystic lesions were analyzed morphologically with radiologic imaging studies and histologically in 13 patients who underwent liver transplantation. RESULTS: Fifteen patients had solitary simple cystic lesions, and 5 patients had multiple continuous oval or beaded lesions. Solitary lesions had a fibrotic cyst wall and lacked epithelia. Continuous lesions had a cyst wall covered with biliary epithelia. Thirteen patients with solitary cysts died or required liver transplantation. In 2 patients with continuous lesions, surgical reboring of the porta hepatis could eliminate cystic lesions and jaundice. CONCLUSIONS: Intrahepatic cystic lesions include 2 different conditions. Solitary cysts are retention pseudocysts, which should be referred to as a bile lake, and are associated with poor prognosis. Continuous beaded cysts are dilated bile ducts, which may be reversed. This distinction is important when considering the treatment strategy.

Bile Duct Diseases↗
PubMed1994

Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy.

Five cases of intrahepatic biliary cyst or cystic dilatation and one suspicious case were found (6.4%) among 93 survivors of biliary atresia. The age at manifestation of such cysts ranged from 6 months to 12 years. These cysts or cystic dilatations were treated surgically in two cases, by percutaneous transhepatic cholangiodrainage (PTCD) in two, and not treated in one. Four patients are alive; one died of biliary atresia. Twenty-nine well-documented cases of intrahepatic biliary cyst were found in the literature, including five treated by the authors. The modes of dilatation were as follows: type A, noncommunicating cyst; type B, cyst with tiny communication with the intestinal loop; and type C, cystic dilatation. Clinical symptoms were fever, jaundice, acholic stool, and "cholangitis." Nineteen patients (66%) had symptoms within 4 years after the Kasai operation (first group); the modes of dilatation were mostly types A and B. However, in five of six patients with type C dilatation, symptoms developed after 10 years of age (second group). The prognosis for the second group appears poor because the condition of the liver worsens once cystic dilatation develops.

Bile Duct Diseases↗
PubMed1992

The future role of hepatic portoenterostomy as treatment of biliary atresia.

According to our recent study, 38 of 93 patients (40.9%) who underwent portoenterostomy at Juntendo University Hospital between 1977 and 1986 survived for more than 5 years. In order to learn the future improvement of the prognosis of biliary atresia patients who undergo portoenterostomy, we investigated the relationship between the prognosis of biliary atresia patients and the age and the histological changes of liver at the time of surgery. Twenty-seven of 30 patients (90%) who survived for more than 5 years with no jaundice and no findings of liver cirrhosis had a mild degree of liver fibrosis (F1 or F2) and a mild degree of degeneration of intrahepatic bile ducts (B1 or B2) at the time of surgery, in spite of the difference of size of intrahepatic bile ducts at the porta hepatis. On the other hand, all 8 patients who showed severe liver fibrosis (F3) and severe degeneration of intrahepatic bile ducts (B3) did not survive for more than 5 years. Accordingly, we can expect promising prognoses in patients who undergo refined portoenterostomy procedures and receive proper postoperative treatment before they have severe histological changes of liver. The portoenterostomy will still play an important role as treatment of biliary atresia in the future.

Bile Ducts, Intrahepatic↗
PubMed2001

Magnetic resonance angiography of portal collateral pathways after hepatic portoenterostomy in biliary atresia: comparisons with endoscopic findings.

BACKGROUND/PURPOSE: This study was undertaken to assess the usefulness of magnetic resonance angiography (MRA) in detecting varices and to investigate the possibility of avoiding routine endoscopy after surgery for biliary atresia (BA). METHODS: The subjects are 21 patients who have undergone periodical MRA since 1996. The esophageal and gastric vessels were investigated as the sites of potential development of collateral vessels. The collateral vessels on MRA were compared with endoscopic findings. RESULTS: (1) Detection of collateral vessels: of 21 patients examined, 9 patients had 12 varices. MRA depicted collateral vessels in all of the 12 varices (sensitivity, 100%). However, all MRA findings, but one was compatible with endoscopic findings (specificity, 92.9%). Outside of a series of these 21 patients, there were 3 additional patients who had undergone endoscopic treatments previously with success, and in whom esophageal vessels were not depicted on MRA, but endoscopy showed remnant varices. (2) Time-dependent relationship between the appearance of collateral vessels and varices: of 4 varices that appeared after the start of MRA, 3 esophageal varices were found endoscopically simultaneously with or after delineation of collateral vessels on MRA. In the remaining patient, varices were found enodscopically 6 months before the MRA delineation, because a simultaneous MRA was not performed in this case. All these varices were in the early stage. CONCLUSIONS: MRA was highly sensitive and specific in detecting esophagogastric collateral vessels of the portal venous system. An endoscopic examination is unnecessary until collateral vessels are seen on MRA after surgery for BA.

Biliary Atresia↗
PubMed1990

Fatal air embolism following endoscopy of a hepatic portoenterostomy.

The case reported is that of a 10-year-old girl born with congenital biliary atresia who had undergone a Kasai procedure in the first year of life. She presented with ascending cholangitis, and investigations revealed a stricture of her portoenterostomy with an intrahepatic stone above the stricture. At laparotomy there were numerous adhesions and dilated varices, so the descending loop of the Kasai was exteriorized to enable a subsequent percutaneous endoscopic manipulation of the stone. During manipulation of the stricture via a pediatric endoscope in the jejunal loop the patient collapsed and died. Post mortem revealed a massive air embolism and varices of the jejunal loop.

Biliary Atresia↗
PubMed2003

Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors.

Cholangitis occurred in 59% of 77 patients who underwent the portoenterostomy procedure for biliary atresia between 1980 and 2000. Good postoperative bile drainage was associated with a lower risk of cholangitis than partial (odds ratio, 5.72; 95% CI, 2.89-11.3) or poor (odds ratio, 3.29; 95% CI, 1.89-5.7) bile drainage. Cholangitis was not an independent risk factor for death or liver transplantation.

Biliary Atresia↗
PubMed1999

Incidence of intrahepatic biliary cysts in biliary atresia after hepatic portoenterostomy and associated histopathologic findings in the liver and porta hepatis at diagnosis.

BACKGROUND/PURPOSE: The association of intrahepatic biliary cysts in biliary atresia (BA) after hepatic portoenterostomy has drawn the attention of many pediatric surgeons and has become an important subject in clinical management, but the incidence and causes of this association are not well known. METHODS: During the last 14 years, we operated on 33 BA patients by hepatic portoenterostomy. Five patients could not be followed up over a 1-year period, so the remaining 28 patients had their intrahepatic structure investigated by periodic magnetic resonance imaging (MRI), and were confirmed at autopsy. The authors also reviewed the preoperative conditions of these patients and the histopathologic changes in the liver biopsy and the porta hepatis specimens taken at portoenterostomy. RESULTS: (1) Intrahepatic biliary cysts were found in 6 of the 28 patients studied (21%). Of 16 patients who were operated on after 1989 and are undergoing follow-up by MRI, 4 (25%) had cysts. (2) Five of the 6 patients with cysts had episodes of cholangitis before or at the time of discovery of the cysts, and 4 of them have had their jaundice reappear since the discovery of the cysts. (3) The intralobular spaces (ILS) with fibrosis and the number of bile ducts had increased in the group with cysts compared with those in the group without cysts. (4) The bile duct maximum size in the porta hepatis (PH) was similar in the groups with and without cysts, but a denudation of the mucosal lining cells in the duct, and a fibrosis and inflammation around the duct had increased in the group with cysts compared with those in the group without cysts. (5) Statistically, the fibrosis found in the ILS and the inflammation around the bile duct found in the PH were induced as a correlative factor affecting cyst formation. CONCLUSIONS: The association of intrahepatic biliary cysts in BA after portoenterostomy is frequent. The fibrous change in the ILS and the inflammatory process around the bile ducts might be potential causes of cyst formation.

Bile Ducts↗