Kasai Hepatic Portoenterostomy Research Articles

Radial nerve palsy caused by a rapidly growing intramuscular hematoma in an infant with biliary atresia: a case report

BackgroundBiliary atresia (BA) is a rare cause of persistent jaundice in infants that can result in vitamin K malabsorption and vitamin K deficiency bleeding (VKDB). We present an infant with BA who developed a rapidly growing intramuscular hematoma in her upper arm after a vaccination which caused a radial nerve palsy.Case presentationAn 82-day-old girl was referred to our hospital because of a rapidly growing left upper arm mass. She had received three doses of oral vitamin K before age 1 month. At age 66 days, she received a pneumococcal vaccination in her left upper arm. On presentation, she showed no left wrist or finger extension. Blood examination revealed direct hyperbilirubinemia, liver dysfunction, and coagulation abnormalities, indicating obstructive jaundice. Magnetic resonance imaging showed a hematoma in the left triceps brachii. Abdominal ultrasonography revealed an atrophic gallbladder and the triangular cord sign anterior to the portal vein bifurcation. BA was confirmed on cholangiography. VKDB resulting from BA in conjunction with vaccination in the left upper arm were considered the cause of the hematoma. The hematoma was considered the cause of her radial nerve palsy. Although she underwent Kasai hepatic portoenterostomy at age 82 days, the obstructive jaundice did not sufficiently improve. She then underwent living-related liver transplantation at age 8 months. The wrist drop was still present at age 1 year despite hematoma resolution.ConclusionsDelayed detection of BA and inadequate prevention of VKDB can result in permanent peripheral neuropathy.

Current and emerging adjuvant therapies in biliary atresia.

Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure. Preventing liver transplantation, or even delaying eventual liver transplantation, is the key to improving long-term outcomes. This review first examines the commonly used adjuvant therapies in post-HPE biliary atresia and the strength of the evidence supporting these therapies. Next, it examines the evolving frontiers of management through a comprehensive evaluation of both recently completed and ongoing clinical trials in biliary atresia. Promising therapies used in other cholestatic liver diseases with potential benefit in biliary atresia are discussed. Improving post-HPE management is critical to prevent complications, delay liver transplantation, and ultimately improve the long-term survival of patients with biliary atresia.

Association of Early Direct Bilirubin Levels and Biliary Atresia Among Neonates

Biliary atresia (BA) is a fibrosclerosing cholangiopathy resulting in obstruction of the biliary tree. Diagnosis and early performance of the Kasai hepatic portoenterostomy improves outcomes.1 Current guidelines recommend measurement of direct bilirubin (DB) or conjugated bilirubin levels in neonates who are still jaundiced at age 2 to 3 weeks.2,3 However, recent studies have documented the elevation of DB or conjugated bilirubin levels within the first days after birth in neonates with BA,4,5 suggesting that BA is a developmental cholangiopathy associated with a genetic, prenatal, or developmental event. To test this hypothesis, this case series evaluated DB levels in the first days after birth among neonates who were subsequently diagnosed with BA.

Effect of Repeat Kasai Hepatic Portoenterostomy on Pediatric Live-Donor Liver Graft for Biliary Atresia

Treatment for patients with biliary atresia is a Kasai hepatic portoenterostomy; however, the efficacy of repeat Kasai hepatic portoenterostomy is unclear. This study sought to examine the effect of a prior Kasai hepatic portoenterostomy, especially a repeat Kasai hepatic portoenterostomy, on the outcomes of living-donor liver transplant. One hundred twenty-six of 170 children that underwent a living-donor liver transplant between May 2001, and March 2010, received a living-donor liver transplant for biliary atresia. These patients were divided into 2 groups according to the number of previous portoenterostomies: 1 (group A, n=100) or 2 or more Kasai hepatic portoenterostomies (group B, n=26). Portoenterostomy was performed twice in 24 patients in group B, 3 times in 1, and 4 times in 1. Preoperative, operative factors, mortality, morbidity, and survival rates were examined and compared between groups. The surgical factors such as operative time, blood loss per weight, cold ischemia time, and weight of the native liver were significantly greater in group B than they were in group A. The patient survival rates were comparable in the 2 groups (94.5% in group A and 93.3% in group B), and the difference was not statistically significant. No statistically significant difference was observed between the groups with regard to vascular complications, biliary complications, and other factors including postoperative variables. Bowel perforation requiring surgical repair was more frequent in group B than it was in group A. Repeat Kasai hepatic portoenterostomy might have a negative effect on patients who undergo living-donor liver transplant for biliary atresia patients with potential lethal complications such as bowel perforation. More biliary atresia patients could have a liver transplant, with improved survival and better life expectancy, if they have inadequate biliary drainage after the initial Kasai hepatic portoenterostomy.

Early detection of biliary atresia: past, present & future

The age threshold at the time of Kasai hepatic portoenterostomy associated with a prognosis of biliary atresia (BA) is becoming clearer as 10-year native liver survival data become more frequent in the recent literature, whereas the age at diagnosis has not dramatically decreased during the last 3 decades. A stool color card screening implemented in 1994 in Japan is now expanding worldwide. However, the contribution of this modality will probably be limited because of the nature of this disease, for example, ‘progressive obliterative cholangiopathy’. A cholic stool was actually observed only in 50% before diagnosis according to the Japanese BA Registry data. Thus, color card screening does not appear to be instrumental in detecting patients with BA early enough before 1 month of age. A highly sensitive, adequately specific, noninvasive and quantitative method may be expensive, but the overall cost would be lower than that of liver transplant.

Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Biliary atresia (BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy. Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent. Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare. We herein report two infants: one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy, and the other at 4 wk after Kasai hepatic portoenterostomy, and also provide a review of the literature. Moreover, we conclude that clinicians should consider BA in infants with bone fracture during early infancy.

Newly Formed Hepatic Masses in Children with Biliary Atresia after Kasai Hepatic Portoenterostomy

Newly Formed Hepatic Masses in Children with Biliary Atresia after Kasai Hepatic Portoenterostomy

Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia

Biliary atresia (BA) is the leading indication for liver transplantation (LTx) during childhood. Predictive markers for progression are lacking. We investigated the correlation between quantified fibrosis of the liver at the time of Kasai hepatic portoenterostomy (KPE) and the clinical course. Liver tissue was obtained at the time of KPE in 53 children and stained with Picrosirius red. The mean volume of fibrosis per number of periportal fields (Vfib) and the Ishak score were calculated. Vfib was 3.9%. Transplant-free survival was significantly higher in those patients with Vfib < 2.5% (P < 0.05, Kaplan-Meier analysis). Vfib < 2.5% predicted transplant-free survival with a sensitivity of 100% and a specificity of 48% (positive predictive value: 100%, negative predictive value: 17%). Vfib was correlated with bilirubin 6 months after KPE (Bili6m). The negative predictive value could be enhanced to 23% if Vfib < 2.5% was combined with Bili6m < 20 micromol/L. The Ishak score showed no correlation with transplant-free survival or Vfib. Detection of Vfib at the time of KPE is a valid marker in predicting transplant-free survival in children with BA. This method should be considered an integral part of scoring systems predicting the indication for LTx. Liver Transpl 15:876-882, 2009. (c) 2009 AASLD.

Oxidative stress profile in the post-operative patients with biliary atresia

Many post-operative patients with biliary atresia (BA) suffer from liver dysfunction, such as chronic inflammation even without jaundice after a Kasai's hepatic portoenterostomy. The presence and degree of oxidative stress were evaluated in the post-operative patients with BA. Twelve outpatients who underwent a Kasai's hepatic portoenterostomy were evaluated. The active oxygen products, the rate of bioantioxidant, the markers of oxidative stress, and the degree of hepatic oxidative stress were examined by immunohistochemical staining of biopsied specimens. All of the oxidative stress markers in the post-operative patients with BA increased in comparison to those in the controls. Moreover, 8-OHdG immunohistochemical staining was positive in 84+/-4.8% in hepatic cells in the portal area in the post-operative patients with BA. The post-operative patients with BA were under increased oxidative stress, even if their liver dysfunction was mild without jaundice. Antioxidant therapy might be necessary to decrease of oxidative stress in the post-operative patients with BA.

Mother-to-daughter occurrence of biliary atresia: a case report

We herein report a case of mother-to-daughter occurrence of biliary atresia (BA). The mother underwent a Kasai hepatic portoenterostomy at the age of 64 days with a diagnosis of type III BA. Her postoperative course was uneventful, with a good bile discharge, and she became pregnant at the age of 20 years. At 21 weeks of gestational age, fetal ultrasonographic examination of the baby revealed a cystic mass at the hepatic hilum. The baby was born via vaginal delivery at 41 weeks of gestation with a birth weight of 3269 g. Because of an increasing direct bilirubin level and the absence of bile excretion, a laparotomy was performed at the age of 29 days. Intraoperative cholangiography revealed the diagnosis of BA I-cyst, and a hepaticojejunostomy was performed with a good bile discharge. To our knowledge, these patients are the first occurrence of BA in a transmission pattern of mother to daughter.

Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful "Kasai" hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was found when she was 5-years old. Needle biopsy could not establish a definitive diagnosis. Because the tumor size was not changing, she was conservatively followed by abdominal echo at an outpatient clinic. She showed progressive liver dysfunction and ongoing cirrhosis, and so at the age of 10 years, she received living donor liver transplantation at our institute. The tumor was pathologically diagnosed as a FNH. Though a FNH in biliary atresia patients is extremely rare, there are only two cases reported in the literature; it should be considered in the differential diagnosis for a hepatic nodule during a long follow-up course in patients with biliary atresia.

Closing in on Biliary Atresia

Biliary atresia (BA) is a rare congenital condition which results in significant morbidity and mortality. The Kasai hepatic portoenterostomy and liver transplantation have significantly improved survival in this disease; however, unraveling the etiology/etiologies will be required to improve transplantation free survival. At present, BA is still the single most common condition in children on pediatric liver transplant waiting lists and accounts for up to 50% of those waiting for liver transplantation [1]. It is the most rapidly fibrosing liver disease in man and accounts for ∼10% of all liver transplant procedures performed [2]. The rarity of this condition and the likelihood of a multi-factorial etiology have limited progress in research. Recently, collaborative research networks in North America (BARC) and Europe (EBAR) have been established [3,4]. The following discussion outlines the most recent information available from these and other groups concerning epidemiology, pathophysiology, etiology, diagnosis, management, long term outcome and conclusions/ recommendations for future research. Keywords: Biliary atresia, neonatal cholestasis, cholangiopathy, hepatic fibrosis, portoenterostomy, biliary epithelial cells

Mucosal intussusception to avoid ascending cholangitis

Many methods have been devised to prevent ascending cholangitis following Kasai's hepatic portoenterostomy for biliary atresia. To investigate the effectiveness of mucosal intussusception in preventing ascending cholangitis, 20 mongrel dogs were randomized to undergo Roux-en-Y cholecystjejunostomy and common bile duct ligation with or without mucosal intussusception. Aeromonas hydrophila was instilled into the gastrointestinal tract. Bacterial cultures were grown from samples taken from the gallbladders and lower jejunum, and blood chemistry and barium studies were performed. The barium studies showed satisfactory lack of reflux in all ten dogs with mucosal intussusception. Only two of them had positive cultures for Aeromonas from the gallbladder 3 days after bacterial instillation into the jejunum. All ten dogs in the control group had positive cultures for Aeromonas from the gallbladder. Our results showed that the mucosal intussusception group had a significantly lower rate of recovery of organisms from the gallbladder compared with the control group when the organisms were instilled into distal intestine (P less than 0.05). The method is worthy of clinical trial as a means of preventing or treating ascending cholangitis.

Biliary atresia—a fifteen-year review of clinical and pathologic factors associated with liver transplantation

Purpose The aim of this study was to identify clinical and pathologic factors associated with liver transplantation in infants with biliary atresia initially treated with Kasai hepatic portoenterostomy (KHPE). Methods Institutional Review Board approval was obtained. Records of patients with biliary atresia diagnosed between January 1986 and December 2000 were reviewed. Patients were divided into those who never required transplantation, those who underwent transplant in the first year after KHPE, and those who required transplantation later in childhood. Analysis of variance (ANOVA) compared multiple factors among the 3 groups. Proportional analysis compared those who required transplantation against those who did not. Statistical significance was considered achieved if P was less than .05. Results Forty-five patients were identified. Survival after KHPE was 96% (43 of 45). Sixteen (37%) never required transplantation, 13 (30%) underwent transplant within 1 year after KHPE, and 14 (33%) underwent transplant more than 1 year after KHPE. ANOVA comparison showed that the duration of jaundice before KHPE as a predictor for liver transplantation approached significance ( P = .082). Proportional analysis found that a longer initial duration of jaundice before KHPE ( P = .016) and failure to establish biliary flow ( P = .033) were also significant predictive factors. An initial requirement for phototherapy ( P = .057) and ductules less than 200 μm in diameter ( P = .060) showed a trend toward predictor of liver transplantation. Conclusions A longer duration of jaundice before KHPE, failure to establish bile flow, requirement for phototherapy in the neonatal period, and ductules smaller than 200 μm are associated with liver transplant after KHPE.

Past and future of biliary atresia

Background: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? Methods: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. Results: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P <.001). Conclusions: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome. J Pediatr Surg 35:717-720. Copyright © 2000 by W.B. Saunders Company.

Effects of invaginating anastomosis in Kasai hepatic portoenterostomy on resolution of jaundice, and long-term outcome for patients with biliary atresia

Background/Purpose: This report describes a technique of hepatic portoenterostomy (HPE) to keep sustained bile drainage in biliary atresia patients. In conventional HPE, the medial posterior row of the anastomosis is placed behind the main portal vein. But around both lateral corners where the portal branches enter the liver parenchyma, the anastomosis is placed across and outside of the portal veins. Resultantly, the proximal part of the portal veins become included in anastomosis attaching directly to the dissected area where bile ducts are copious. In a devised procedure designated as invaginating anastomosis (IA), an anastomotic line was placed to invaginate the medial branch of the right portal vein and also placed behind the veins even at their roots. This technique puts all portal branches outside of the anastomosis, preventing cicatricial adhesion at the dissected area with the posterior wall of the veins. Methods: The effect of IA was evaluated in terms of resolution of jaundice and long-term outcome compared with conventional anastomosis (CA). Group IA consisted of 20 patients and group CA of 18 patients. Results: Patients in whom jaundice resolved included 19 (95%) in group IA and 11 (61%) in group CA ( P < .05). Reoperation was needed in only one infant in group IA, and in six in CA ( P < .05). In patients with reoperation in group CA, the portahepatis was found to be contracted with dense cicatricial tissues. The survival rates for patients with a native liver, calculated excluding those in whom the cause of death was not liver deterioration, was 87% for IA compared with 41% for CA ( P < .05). Averaged biliary atresia prognostic index at the last follow-up evaluation was 20.2 ± 24.5 for group IA and 62.6 ± 29.8 for group CA ( P < .001). Conclusion: The invaginating anastomosis in portoenterostomy promised a sustained bile drainage and improved long-term outcome by preventing cicatrical contraction of the portahepatis.

Biliary atresia

Biliary atresia is a dynamic process ultimately resulting in complete extrahepatic biliary obstruction. The cause is unknown, but if left untreated, progressive hepatic failure leads to death. Treatment, including timely diagnosis and early biliary reconstruction by Kasai's hepatic portoenterostomy, results in sustained postoperative bile flow in 90% of patients. Nearly 50% of those treated survive to 10 years and beyond. Liver transplantation rescues many patients who develop liver failure. Advances in patient selection, surgical technique, postoperative management, and liver transplantation have improved the outlook for these patients. Quality of life is excellent in many long-term survivors, and they will benefit from advances in our understanding of the specific nutritional, hormonal, and immunologic aspects of this disease.

BILIARY ATRESIA AND THE POLYSPLENIA SYNDROME

Currently, there Is a widely held but unsubstantiated perception that infants with biliary atresia and co-existing polysplenia uniformly fall Kasai operations; referral for transplantation should be done inmediately and without attempted corrective surgery. 14 of 118 consecutive patients (12%) having Kasai hepatic portoenterostomy for biliary atresia at our institution had one or more of the additional components of the polysplenia syndrome: intestinal malrotation (6), preduodenal portal vein (3), aberrant hepatic artery (3), situs inversus (2), absent inferior vena cava (2). Although technically more demanding, 11 of the 14 polysplenia patients achieved biliary drainage after operation. Three patients had subsequent liver transplantation; 2 of whom died. Long-term survival (mean follow-up, 4 years) was achieved in 5 (45%), again not significantly different from that in the larger group (40.2%). The Kasai operation is the initial operation of choice in patients with coexisiting biliary atresia and the polysplenia syndrome.

Corticosteroid therapy in biliary atresia

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.

Conduit decompression in biliary atresia

Catheter decompression of the bilioenteric conduit was routinely carried out coincident with stomal closure in 15 consecutive patients with biliary atresia after Kasai hepatic portoenterostomy operation. Postoperatively the catheter was systematically, gradually occluded over a 3- to 8-week period and then withdrawn. Complications were relatively minor and early postoperative cholangitis was avoided.