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Biliary atresia is a disease of unknown origin characterized by partial or total absence of the biliary tract. While this condition is rare, the medical and surgical management makes the ramifications for dental treatment increasingly complex. This article reviews the disease and its complications and documents dental treatment of two patients.
Congenital biliary atresia or congenital biliary dilatation in siblings have been reported, although the genetic influences in these diseases are considered to be obscure. We report a case of congenital biliary atresia and congenital biliary dilatation in sisters.
Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai procedure. Surgical treatment of congenital biliary atresia is associated with substantial perioperative morbidity and mortality, as well as long-term sequelae. Portal hypertension and hypersplenism can complicate the course of up to 50% of patients who survive for more than 2 years. Not surprisingly, few of these patients reach adulthood. We report the case of a woman with congenital biliary atresia surgically corrected with the Kasai operation who subsequently reached adulthood and became pregnant. Despite a prenatal course complicated by portal hypertension, bleeding esophageal varices, and hypersplenism, the woman delivered a healthy infant at 36.5 weeks' gestation.
Although biliary atresia is characterized by luminal obstruction of the extrahepatic bile ducts, the etiology and the pathophysiology of the liver are still controversial. The prognosis of biliary atresia has been improved after the introduction of Kasai's hepatic portoenterostomy, but there are still many problems to be solved in the treatment of this disease. Successful results of hepatic portoenterostomy depend on early diagnosis and operation, adequate operative technique, prevention of postoperative cholangitis, and precise postoperative management. However, we are on the verge of a new era in the therapy of biliary atresia combining portoenterostomy with liver transplantation.
The objective of KASAI's operation is to obtain a postoperative bile excretion and to maintain an internal biliary fistula. The purpose of this report is to study the relationship between the liver histopathology and the postoperative bile flow and survival. From july 1976 to january 1990, 66 patients underwent a corrective operation for biliary atresia. In 24 patients we performed a KASAI's operation with internalization of the biliary conduit, 36 with externalization, four gall-bladder KASAI and two liver biopsy. The mean age at operation was 66 days and postoperative bile excretion was obtained in 43 of them (66 per 100). The survival rate was 70 per 100. The liver histopathology shown: minimum portal fibrosis (7.8 per 100), moderate (49 per 100) and severe (43 per 100). We found bile ducts in 30 patients (46 per 100), in three bile ducts were less than 50 mu, in 15 between 50 and 100 mu and more than 100 mu in 12. We did not found statistic correlation between grade of liver fibrosis size of bile duct and postoperative flow. We found a statistic correlation between the presence of bile ducts and postoperative flow (p less than 0.05).
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 is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.
Four infants with biliary atresia had gross obliteration of the common hepatic duct but residual patency of the gallbladder, cystic duct and common bile duct. The patients were treated by hepatic portocholecystostomy utilizing the extant bile ducts for biliary reconstruction. Bile drainage was achieved in all four infants. There was a conspicuous absence of postoperative cholangitis. Subsequent obstruction of the distal ducts in two patients necessitated reoperation and construction of a standard biliointestinal conduit. The other two children are surviving, jaundice-free, 5 1/2 and 5 years after operation with minimal sequelae of biliary atresia. Hepatic portocholecystostomy is a feasible surgical alternative to intestinal reconstruction in patients with biliary atresia in whom the disease is limited to the proximal extrahepatic bile ducts.
Fourteen patients with "noncorrectable" biliary atresia are living without jaundice for more than 2 yr after hepatic portoenterostomy or its modification. Retardation of physical growth was observed in one of them, and mental retardation in another, both of which seemed irrelevant to biliary atresia. Serial tests for liver function after operation revealed early recovery of serum bilirubin, transminase, and turbidity, and delayed improvement of alkaline phosphatase. Postoperative needle biopsy of the liver disclosed that changes in hepatic parenchyma and ductular proliferation were rapidly improved after successful operation. Improvement of fibrosis of the liver was delayed, and it was not satisfactory in patients whose preoperative changes in the liver were severe or in whom ascending cholangitis had been a frequent complication. Histologic features of hepatic cirrhosis were observed in the liver in three cases, in two of which there had been frequent episodes of cholangitis. Only one of these showed clinical signs of portal hypertension. Functional and morphologic cure can be achieved in "noncorrectable" biliary atresia by hepatic portoenterostomy or its modifications, although varying degree of hepatic fibrosis may remain according to severity of preoperative changes of the liver and postoperative complication of ascending cholangitis.
The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. Liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.
Aetiological factors were sought prospectively in 55 babies with extrahepatic biliary atresia, in 105 with neonatal hepatitis, and in 11 with intrahepatic biliary atresia, seen as a result of nearly complete ascertainment of these conditions in the State of Victoria between 1963 and 1974. In neonatal hepatitis infective causes were shown in 22 babies, galactosaemia in 6 and alpha1-antitrypsin deficiency in 8; familial occurrence was noted in 10 further babies and unrelated second diseases were present in 24 of the remaining 59 babies. The only clues to aetiology in extrahepatic biliary atresia were a suspicion of time-space clusters, a deficiency of affected babies born to young primiparous women, and an unexpected number of spontaneous abortions in the histories given by the mothers. Genetic factors appeared to be important in intrahepatic biliary atresia, but are not reported in detail. Hypotheses for the aetiology of neonatal hepatitis and of extrahepatic biliary atresia are presented. Both are considered syndromes with multiple causes. Recurrence risks in sibs are discussed, and are 1 in 7 for neonatal hepatitis of unknown cause, negligible in extrahepatic biliary atresia, and usually 1 in 2 or 1 in 4 in intrahepatic biliary atresia, depending upon the family history.
Two children with biliary atresia are described in whom focal nodular hyperplasia of the liver occurred following portoenterostomy. The lesions were low-or iso-dense on unenhanced CT and became hypodense post-contrast enhancement. There was normal 99mTc phytate on hepatic colloid scintigraphy. On T2-weighted spin echo MR images, there was increased signal intensity within the masses, and the surrounding liver parenchyma was divided by linear septa in one of the two cases.
Ninety patients with biliary atresia surviving more than 5 years were analysed with respect to late complications occurring after the age of 4 years. Thirty-five had complications including cholangitis, portal hypertension, hypersplenism, gastrointestinal bleeding, and esophageal varices. These complications occurred at various times. The background factors of late complications were past history of cholangitis soon after the operation, advanced age at operation, re-operation, high portal pressure at initial operation, and a long interval before disappearance of jaundice after surgery. These factors are mostly related to the first operation and its postoperative course. Therefore, we stress that late complications can be prevented by intensive treatment of the patient at the time of the first operation.
Noninfectious obliterative cholangitis results from biliary tract inflammation in clinical conditions such as biliary atresia and sclerosing cholangitis. The purpose of this study was to develop an animal model of noninfectious biliary tract inflammation and fibrosis. An implantable osmotic pump was connected to a catheter placed into the gallbladder of hamsters. Phorbol myristate acetate (PMA) was infused into the biliary tract for periods of 6 hours to 28 days. After 7 days the animals developed neutrophil infiltration, cellular necrosis, and edema of the biliary ducts. After 14 days, the animals demonstrated intrahepatic cholestasis with bile duct fibrosis and acute and chronic inflammatory cell infiltration. By 28 days pronounced portal fibrosis was present, some of which created an early bridging cirrhosis pattern. In addition there was evidence of neocholangiogenesis. We conclude that long-term PMA infusion into the biliary tract generates an inflammatory response characterized by obliterative cholangitis and fibrosis, sharing many of the histologic features of human biliary atresia. This model may provide a relatively simple technique for investigating the process of nonpyogenic biliary tract inflammation.
This study assessed the usefulness of ERCP in the diagnosis of biliary atresia. We evaluated 57 infants with prolonged cholestasis with abdominal ultrasound, liver biopsy, and ERCP. Using clinical observations alone, 22 infants were thought to have biliary atresia; whereas 35 children were thought to have neonatal hepatitis. The ERCP was performed with a prototype duodenoscope and was successful in all infants except two with biliary atresia. In 20 infants three types of radiological findings consistent with biliary atresia were seen: type 1, no visualization of biliary tree (35%); type 2, opacification of the distal common duct and gallbladder without visualization of the main hepatic duct (35%); and type 3, opacification of the distal common duct, the gallbladder, and a segment of the main hepatic duct with biliary lakes at the porta hepatis (30%). Twenty-five of 35 infants with suspected neonatal hepatitis were excluded because of a liver biopsy that was diagnostic. In the remaining 10 infants the liver biopsy had some features of extrahepatic biliary atresia and ERCP was performed prior to surgery. A normal extrahepatic biliary tree was obtained in all of them. In conclusion, ERCP permits the visualization of the biliary tree in young infants and is useful in selecting those infants who should be considered for exploratory laparotomy.
The occurrence of biliary atresia in one of a pair of male twins is reported. The current concepts regarding the aetiology of biliary atresia are examined in relation to this case; and neither the concept of a transplacental agent nor that of a genetic trigger mechanism seems probable. It is concluded that, as with many congenital defects, biliary atresia is probably the end-result of different aetiological factors, among which viruses and other agents may cross the placenta to cause it, whether or not there is a genetic predisposition in the individual fetus, or the biliary system may simply fail to develop.
Care of the child with biliary atresia is a challenge for pediatric nurses. The cause of the disease is not known, and new diagnostic tests and treatment options are continually being developed. This article addresses fundamental issues specific to the care of children with biliary atresia. In order to understand the clinical manifestations and the treatment rationale, pathophysiology and theories of etiology are discussed. Nursing care of the child and issues regarding the teaching and support of the family system are addressed, with suggestions for independent reading.
Twenty-eight infants suspected of having biliary atresia were studied with radionuclide imaging and quantitation of liver activity clearance using rose bengal sodium I 131. Scanning the abdomen for the presence or absence of gut radioactivity was a sensitive indicator of complete biliary obstruction. Absence of gut radioactivity correctly predicted biliary atresia in all 11 infants proved to have that diagnosis. Biliary atresia was excluded by the presence of gut radioactivity in 13 of 17 infants with other forms of neonatal juandice. When present, gut activity was identifiable within 24 hours after injection. Serial tests improved specificity. Plotting the rate of clearance of liver activity did not aid diagnosis.