A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers.

OBJECTIVE: The authors investigated risk factors for failure after portoenterostomy for biliary atresia using univariate and multivariable methods. SUMMARY BACKGROUND DATA: Kasai's portoenterostomy has gained worldwide acceptance as the initial surgical therapy for infants with biliary atresia. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. METHODS: The authors analyzed risks for failure in 266 patients treated from 1972 to 1996 by the Kaplan-Meier product limit estimate and Cox proportional hazards model. Failure was defined as death or transplant. RESULTS: Age at surgery, surgical decade, and anatomy of atretic bile ducts were identified as independent risk factors. Five-year survival was 49% and median survival was 15 years when bile drainage was achieved. Sixty-five patients had liver transplants. Mean age at transplant was 5.4 years. CONCLUSIONS: The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anatomy of the atretic extrahepatic bile ducts. Liver transplant will salvage patients with failed Kasai with 10-year posttransplant survival of 71%.

Management of esophageal varices in children by endoscopic variceal ligation.

Endoscopic variceal sclerotherapy (EVS) has been considered the mainstay of therapy for bleeding esophageal varices in adults. However, recent data have shown that endoscopic variceal ligation (EVL) is just as efficacious and has fewer complications than EVS. Although there are many reports concerning EVL in adults, only a few studies have been done in children. This report describes experience with EVL in 22 children with esophageal variceal hemorrhage. Eighty-seven EVL procedures were performed during a 9-year period in 22 children. The causes of portal hypertension were biliary atresia (10), portal vein thrombosis (8), chronic active hepatitis (1), cirrhosis secondary to cystic fibrosis (2), and primary sclerosing cholangitis (1). The age range at the onset of variceal bleeding was 8 months to 19 years. Twelve patients had EVS before EVL treatment was begun. Distal esophageal varices (one to four per session) were mechanically ligated using an elastic band ligature device attached to a flexible endoscope. The aim of therapy was obliteration of distal esophageal varices by EVL, every 2 to 4 weeks, until eradication. Subsequent EVL was dictated by the status of the varices. Outcome was assessed with respect to survival, rebleeding, status of varices, and complications. The patients underwent a mean of four sessions of EVL (range, one to eight). Four patients subsequently underwent liver transplantation. Of the 18 patients remaining (average follow-up period, 5.3 years), 12 had their varices eradicated (average of four EVL sessions), four are still in treatment, one has not been evaluated in the past 4 years, and one died of liver failure. Complications included bleeding between sessions (6 patients), cervical esophageal perforation (1 patient), and transient fever (2 patients). No child has experienced symptoms of esophageal stenosis or gastroesophageal reflux. Two patients died of liver disease, unrelated to bleeding from portal hypertension. EVL is effective in controlling variceal hemorrhage in children with portal hypertension, regardless of etiology. The complication rate is low, and EVL is an acceptable and perhaps preferable alternative to EVS in children with esophageal varices.

Long-term results with the Kasai operation for biliary atresia.

OBJECTIVE: To evaluate long-term outcome in a series of children with biliary results atresia treated by portoenterostomy. DESIGN: Case series of consecutive infants with biliary atresia with 10-year follow-up. Data were obtained by retrospective chart review or phone interview. SETTING: A tertiary academic medical center and regional children's hospital. PATIENTS: A consecutive series of 104 infants diagnosed with biliary atresia more than 10 years ago were evaluated. Eighty-nine had totally obliterated extrahepatic ducts, 4 had proximal hilar cysts (correctable type), and 11 had patency of the gallbladder and distal common duct. INTERVENTIONS: Ninety-eight patients underwent biliary reconstruction and 6 had exploration only. Seventy-four infants underwent reconstruction using a Rouxen-Y with exteriorization. The 11 infants with distal patency underwent a portocholecystostomy ("gallbladder Kasai"). The remainder had various modifications of the Kasai operation. MAIN OUTCOME MEASURES: Survival, liver function, complications, growth, and development. RESULTS: The 6 patients who did not have a portoenterostomy died. Of the 98 who had a reconstruction, 63 died (mean age at death, 27 months; median, 13.4 months), 10 following liver transplantation. Twelve of the 35 survivors ultimately required liver transplants. Twenty-three children are alive more than 10 years after portoenterostomy without the need for transplantation. Two thirds have experienced some manifestation of portal hypertension (ie, variceal bleeding, hypersplenism, ascites). Nineteen patients (79%) are anicteric with normal liver synthetic function and are in an age-appropriate school grade or working and living independently. CONCLUSIONS: We found that surgical correction of biliary atresia offers long-term survival for about one quarter of patients, provides palliation until liver transplantation becomes necessary, and if surgical correction is not feasible, biliary atresia is uniformly fatal. The outlook is good for those children who survived more than 10 years and justifies continued attempts to establish bile flow in infants with biliary atresia.

Thoracoscopic repair of hiatal hernia following fundoplication: a new approach to an old problem.

Three children with symptomatic postfundoplication hiatal hernias were repaired using a novel thoracoscopic approach. All repairs were successful as shown by follow-up barium swallows. The average operative time was 86 mm (range 50-120 mm) and hospital stay averaged 2.3 days. This approach appears to be a feasible means of handling this relatively frequent complication.

The intussusception antireflux valve is ineffective in preventing cholangitis in biliary atresia.

Cholangitis is the most common complication after portoenterostomy for biliary atresia. The construction of an intussusception valve in the Roux-en-Y limb of the portoenterostomy has been advocated as a means to ameliorate this complication. The authors reviewed the records of children who underwent portoenterostomy to assess the incidence and severity of cholangitis, as well as outcome. The children were divided into two groups according to whether they did not have (group I) or did have (group II) an antireflux valve in the Roux limb of the bilioenteric conduit. There were 19 children in each group. There were no significant differences between the groups with respect to age at time of portoenterostomy or length of follow-up (Table 1). Nine group I patients are alive and well; five died and five have had or are awaiting transplantation. In group II, 10 patients are alive and well, eight have had transplantation, and one is awaiting transplantation. Outcome with respect to death or the need for liver transplantation because of progressive hepatic failure is not different between the groups (P = 1.0, Fisher's 2 x 2). The incidence of cholangitis was evaluated by comparing the average number of episodes of cholangitis during the follow-up period, the number of episodes per year, and the number of episodes in the first postoperative year (when this complication is most prevalent). Analysis of the data showed no difference in incidence between those with valved and nonvalved biliary conduits. The severity of cholangitis, judged by total length of antibiotic treatment, did not differ between the groups. It appears that the presence of an intussusception valve in the Roux-en-Y biliary conduit does not affect the incidence of cholangitis or the outcome after portoenterostomy, over short-term follow-up.

Hepatic concentrations of zinc, copper and manganese in infants with extrahepatic biliary atresia.

The objective of this study was to determine the concentration of Zinc (Zn), Copper (Cu) and Manganese (Mn) in hepatic tissue from extrahepatic biliary atresia (EHBA). Liver biopsy samples were obtained at time of portoenterostomy from 49 infants ages 1.1 to 20.7 months (median 2.1) with EHBA. Samples were dry ashed and analyzed by flame (Zn) or flameless (Cu and Mn) atomic absorption spectrophotometry. Hepatic Cu concentrations are physiologically elevated at birth and decline rapidly during the first 2 month of life, therefore only samples from 29 infants, ages greater than 8 weeks were considered for Cu. Concentrations (mg/kg dry weight, mean and range) were: Zn 142 (70-507), Cu 204 (19-570), Mn 9.1 (2.8-21.8) vs. literature controls in the same age range: Zn 262 (82-543), Cu 92, Mn 4.3 (3.3-11.5). No correlations were found between serum alkaline phosphatase, AST or total bilirubin and hepatic trace element concentrations, between trace element concentrations and age, or between Cu and Mn. Decreased bile flow with intrahepatic cholestasis may result in hepatic accumulation of Mn as well as Cu. The low hepatic Zn concentrations indicate the need for further study of Zn metabolism in this population.

Portal vein thrombosis: treatment of variceal hemorrhage by endoscopic variceal ligation.

Thirty-one endoscopic variceal ligation (EVL) procedures have been done in seven consecutively treated children who had esophageal varices resulting from portal vein thrombosis. Using an elastic band ligature device attached to a standard flexible endoscope, the varices in the distal 5 cm of the esophagus were mechanically strangulated. Typically, one to three varices were banded at each session. The children were between 8 months and 19 years of age at the onset of variceal bleeding. Treatment initially required frequent procedures (every 2 to 4 weeks), but the interval was gradually extended to biannual or annual. Treatment was assessed by survival, complications, incidence of rebleeding, and obliteration of varices. Each patient had from 3 to 9 EVL sessions. There were no deaths related to EVL. There were no complications. Only one patient had rebleeding in the interval between sessions. The follow-up period is 3 to 12 years. In three of the six surviving patients, the varices have been completely eradicated. The remaining three are completing treatment. Endoscopic variceal ligation is safe efficacious treatment for control of variceal bleeding caused by portal vein thrombosis. In our experience, the technique has eliminated the need for portosystemic shunting in this patient population.

The pediatric diaphragm in acute gastric volvulus.

Acute gastric volvulus occurred in nine infants and one older child during the past 19 years; all patients had an associated left diaphragmatic anomaly. There were seven examples of eventration of the diaphragm, two of giant hiatal hernia and one Bochdalek hernia. Nine of the ten patients presented with vomiting and one with acute respiratory distress. The gastric volvulus was mesenteroaxial in eight patients and organoaxial in two. Operative treatment consisted of repair of the diaphragmatic anomaly and gastric fixation in eight patients. Gastric fixation alone was performed in one patient. A single patient died preoperatively and had gastric necrosis at postmortem examination. Of the nine patients treated operatively, all remain alive and well without recurrence. Acute gastric volvulus should be considered in any infant presenting with unexplained vomiting in association with a left diaphragm anomaly, and once diagnosed, immediate operation is mandatory.

Surgical jaundice in infants: other than biliary atresia.

After biliary atresia, the lesions responsible for surgical jaundice in the infant are perforation of the common bile duct, choledochal cyst, bile plug syndrome, and miscellaneous congenital lesions in descending order of frequency. Perforation of the common bile duct commonly presents with an insidious onset of bilious ascites and is best treated by simple peritoneal drainage. Choledochal cyst usually presents later in childhood but presents in infancy if obstruction of the biliary tree is complete or near complete. Excision is the treatment of choice. Any condition leading to alteration in bile composition may cause bile plug syndrome. Spontaneous resolution is the rule: occasionally, intraoperative irrigation is necessary. Most miscellaneous lesions lend themselves to operative correction.

Biliary atresia and the polysplenia syndrome.

There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989, 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), aberrant hepatic artery (4), or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference). Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai's operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Acturial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation.

Congenital biliary tract disease.

Jaundice in the pediatric patient requires prompt and directed evaluation. This dictum is highlighted in infants with biliary atresia, in whom the progressive sclerosing process results in complete obliteration of patent but microscopic hilar biliary structures by 4 months of age. Kasai's operation, if done before that time, will re-establish bile drainage in 90% of infants. One fourth to one third of patients achieve long-term jaundice-free survival. Complications of cholangitis, portal hypertension, and fat malabsorption are experienced by many patients. In children with early or late operative failure, liver replacement now offers legitimate hope for extended survival. Choledochal cyst is a conglomerate of pancreaticobiliary anomalies consisting of a choledochal cyst, a common-channel-type pancreaticobiliary junction, intrahepatic cystic disease, and partial obstruction of the distal common bile duct. Many patients have one or more of these malformations. It is now widely accepted that the preferred treatment of choledochal cyst is total excision of the diseased biliary duct with reconstruction by Roux-en-Y choledochojejunostomy. "Internal" excision avoids injury to other structures in the hepatoduodenal ligament, particularly if pericystic inflammation is present. Congenital perforation of the common bile duct responds in most cases to simple peritoneal drainage of the perforation. Retention of the tube cholecystostomy is useful for subsequent cholangiographic follow-up. Tube cholecystostomy may also be useful for irrigation of the biliary tract in infants with inspissated bile syndrome.

Biliary atresia registry, 1976 to 1989.

The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.

The blind loop syndrome in children.

Anatomical abnormalities of the small bowel that cause intestinal stagnation result in bacterial overgrowth and a blind loop syndrome (BLS). Bacterial breakdown of bile salts and deamination of protein lead to malabsorption, steatorrhea, and fat-soluble vitamin deficiencies. Four children developed BLS as a complication of necrotizing enterocolitis, jejunal atresia, gastroschisis, and biliary atresia. BLS was suggested by abdominal pain, feculent vomiting, steatorrhea, and hypoalbuminemia. Dilated, stagnant bowel loops were demonstrated in each instance by upper gastrointestinal contrast study. Positive intestinal bacterial aspirates were confirmatory. Antibiotic treatment in two patients improved symptomatology but all children ultimately required surgery. Surgical procedures consisted of blind loop resection, intestinal plication, and catheterization of the bilioenteric conduit. All patients are now asymptomatic but one child suffers from parenteral nutrition-related cirrhosis and another requires chronic antibiotic therapy.

Agenesis of the extrahepatic bile ducts: report of five cases.

Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.