Kasai Operation For Biliary Atresia Research Articles

Usefulness of Living Donor Liver Transplantation for Patients After Undergoing the Kasai Operation for Biliary Atresia

Living donor liver transplantation (LDLT) has been actively performed for patients with poor clearance of jaundice after the Kasai operation for biliary atresia (BA). The present study clarified the usefulness of LDLT for BA. Between 2000 and 2020, 24 patients (late group) underwent radical surgery for BA in our institute. The overall survival rate, native liver survival rate, and proportion of LDLT in the late group were retrospectively compared with those of 47 patients treated before 1999 (early group). P values <.05 was considered statistically significant. The overall survival rates at 5, 10, and 15 years were 57%, 54%, and 49%, respectively, in the early group and 100%, 100%, and 100% in the late group (P < .001). The native liver survival rates at 5, 10, and 15 years were 57%, 52%, and 39%, respectively, in the early group and 57%, 49%, and 42% (P=.993) in the late group. In the early group, LDLT was performed in 7 of 47 patients (15%), and the overall survival rate after LDLT was 71%. In the late group, LDLT was performed in 11 of 24 patients (46%), and the overall survival rate after LDLT was 100%. The long-term outcomes after the Kasai operation for BA have improved in recent years. There were no marked differences in long-term native liver survival before and after 2000. LDLT was actively introduced for patients with poor clearance of jaundice after the Kasai operation, and the survival rate significantly improved.

Peri-Operative Liver Fibrosis and Native Liver Survival in Pediatric Patients with Biliary Atresia: A Systematic Review and Meta-Analysis.

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08–0.33; I2=46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00–63.63; I2=96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01–0.73; I2=80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00–0.62; I2=69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00–3.22; I2=86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00–175.21; I2=94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

Side-to-side portal vein reconstruction for infant-to-infant deceased donor whole liver transplantation: Report of 2 cases with video.

Infant-to-infant whole liver transplantation (I2I-WLT) has been rarely performed in Korea. Unlike living donor liver transplantation or split liver transplantation, the donor graft portal vein (PV) in I2I-WLT is very small in diameter, so risk of PV complications increases significantly. We present two cases of I2I-WLT with application of side-to-side unification venoplasty for secure PV reconstruction. The first case recipient was a 10-month-old female baby who weighed 6.0 kg. She was diagnosed with progressive familial intrahepatic cholestasis. The deceased donor was a 12-month-old boy. The graft weight was 245 g, so the graft-recipient weight ratio (GRWR) was 4.1%. PV was reconstructed using side-to-side unification venoplasty. The function of graft liver recovered uneventfully and there was no evidence of PV complications. She is currently doing well for 4 years. The second case recipient was a 10-month-old female baby who weighed 8.8 kg. She had also undergone Kasai operation for biliary atresia. GRWR was 6.0%. Because the graft liver was much larger than the native liver, we designed the length of the extrahepatic PV more redundant than the precedent cases to avoid extrinsic compression by the large-sized caudate lobe. The PV was reconstructed using side-to-side unification venoplasty. The function of the graft liver recovered uneventfully. There was no evidence of PV complications. She is currently doing well for 2 years. As the PV size in infant donors and recipients is very small, PV reconstruction in I2I-WLT requires specialized surgical techniques of side-to-side unification venoplasty. Three supplementary video clips are provided.

Living donor liver transplantation for more than 30-year survived patients with native liver after Kasai operation for biliary atresia

ObjectivesA number of patients have developed liver failure after the Kasai operation for biliary atresia (BA), even after long-term postoperative course. We report our experience regarding four cases of biliary atresia required and treated with living donor liver transplantation (LDLT) during adulthood. MethodsAll four patients underwent Kasai operation for BA from 1974 to 1983 and had been followed-up for more than 30 years in our institute. Then, they developed liver failure and treated with LDLT. These four patients’ records were reviewed retrospectively. All data are described using the median value. ResultsThree female and one male were included. Kasai operation was performed at the age of 61 days. The type of biliary obstruction was type III-b1-ν in all. The survival period of the native liver was 440 months. The age at LDLT was 37 years. The reasons of deterioration of native liver were recurrent cholangitis in two and pregnancy and delivery in other two. The age of living donor was 40 years. Right liver grafts were used in all. Percentage of the real graft volume per standard liver volume was 47%. All patients were alive and overall graft survival rate was 100% at a follow-up period of 65 months after LDLT. ConclusionEven after more than 30 year-survival with native liver after Kasai operation, liver failure may be caused by recurrent cholangitis or pregnancy and delivery. LDLT may be effective treatment for those adult patients if an adequate living donor is available.

Hepatic subcapsular or capsular flow in biliary atresia: is it useful imaging feature after the Kasai operation?

To evaluate the implications of hepatic subcapsular and capsular flows using ultrasonography (US) in children after Kasai operation. Children who underwent liver US including color Doppler US and microvascular imaging (MVI) from May 2017 to October 2017 were retrospectively included. Children who underwent the Kasai operation for biliary atresia were included in the Kasai group and children with normal liver were included in the control group. Using US results, the number of intrahepatic biliary cysts and the maximum diameter of the spleen were evaluated in the Kasai group. Liver stiffness values were included when patients in the Kasai group had transient elastography (TE) or shear wave elastography (SWE) results. Hepatic subcapsular and capsular flows on color Doppler US and MVI were compared between the two groups using the following scores: 0, no flow reaching the hepatic capsule; 1, any flow reaching the hepatic capsule; and 2, contiguous hepatic capsular flow. The logistic regression test was used to identify associations between age, intrahepatic biliary cysts, spleen size, SWV, TE results, and flow scores measured on Doppler US and MVI in the Kasai group using the odds ratio (OR) and 95% confidence interval (CI). A total of 65 children (mean 7.6 ± 5.3years), 44 in the Kasai group and 21 in the control group, were included. In the control group, one child had score 1 on MVI and others had score 0 on both Doppler US and MVI. Among the Kasai group, 28 children (63.6%) had score 1, while others had score 0 using Doppler US. Using MVI, 24 children (54.5%) had score 2, 18 children had score 1, and one child had score 0. In the Kasai group, increased liver stiffness on TE was the only factor significantly associated with the presence of subcapsular flow on color Doppler US (OR 1.225, 95% CI 1.020-1.470) and increased spleen size was the only factor significantly associated with increased flow scores on MVI (OR 1.397, 95% CI 1.002-2.724). Detection of hepatic subcapsular, capsular flows on US would be meaningful for children after receiving the Kasai operation. • Hepatic subcapsular or capsular flows can be useful not only for the diagnosis but also for the postoperative follow-up in patients with biliary atresia. • Increased liver stiffness and splenomegaly after the Kasai operation were associated with presence of subcapsular or capsular flow on ultrasonography. • Evaluation of hepatic subcapsular and capsular flows could be needed to assess disease progression after receiving the Kasai operation.

Incidence and Risk Factors of Acute Kidney Injury after Kasai Operation for Biliary Atresia: A Retrospective Study.

Background: Biliary atresia is a progressive, inflammatory, and destructive pathology of the bile ducts. Patients who undergo surgery for correction of biliary atresia (Kasai operation) are at risk of acute kidney injury (AKI) because of their young age at the time of surgery, long operation time, and liver fibrosis or failure as complication of biliary atresia. Conversely, AKI is associated with poor outcomes after surgery. This study therefore aimed to evaluate the incidence, risk factors, and outcomes of AKI after Kasai operation.Methods: All consecutive patients who underwent Kasai operation between March 2006 and December 2015 in a single tertiary-care university hospital were enrolled. AKI was defined based on the Acute Kidney Injury Network criteria. Multivariate logistic regression models were used to assess risk factors for AKI.Results: One hundred sixty-six patients received Kasai operation during study period. Of these, AKI occurred in 37 of 166 patients (22.3%). In multivariate logistic regression analysis, age older than 30 days, higher preoperative estimated glomerular filtration rate, and preoperative contrast use within 7 days were associated with the development of AKI. Perioperative packed red blood cells transfusion was related to reduced occurrence of AKI. AKI was associated with longer ICU stay (OR = 1.015, p = 0.016). More patients with AKI were also found to receive additional surgery except liver transplantation within 1 year compared to those without AKI (10.8 % vs. 2.3 %, p = 0.045).Conclusions: Increased age is strongly associated with the development of AKI after Kasai operation. These findings indicate a rational basis for early corrective surgery for biliary atresia, early screening for AKI, and intervention to improve the results of Kasai operation.

Reconsideration of Laparoscopic Kasai Operation for Biliary Atresia.

Minimal invasive surgery for all kinds of surgical diseases had been practiced for years. The laparoscopic Kasai operation is one of the most challenging procedures and remains controversial for treating biliary atresia (BA). This work presented the initial experience of our Institute and compared the outcomes of open and laparoscopic Kasai operations for BA. Patients 18 years old and younger, and were operated in our Institute for BA from January 2011 to August 2017, were included in this study. General and operative data and outcomes from open and laparoscopic groups were retrospectively collected and analyzed. A total of 23 patients (13 for conventional open operation and 10 for the laparoscopic procedure) received Kasai portoenterostomy. The mean operative age and body weight in the open versus laparoscopic groups were 57.15 ± 20.14 days old and 4.03 ± 0.69 kg versus 57.70 ± 43.06 days old and 4.49 ± 1.48 kg, respectively, and no statistical difference was observed. The mean operative times were 209.62 ± 60.40 and 293.50 ± 39.09 minutes in the open and laparoscopic groups, respectively. The mean follow-up durations were 54.62 ± 22.00 and 23.30 ± 11.87 months for the open and laparoscopic patients, correspondingly. No statistically significant differences were observed for hospital stay and outcomes (including early jaundice clearance rate, episodes of cholangitis, and 2-year native liver survival rate) between the open and the laparoscopic Kasai operations. Experienced pediatric laparoscopic surgeons may reconsider the laparoscopic Kasai operation for application to BA treatment.

IS2-03 Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation

IS2-03 Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation

Multiple orifices and cholangiography with a “fire-like” appearance after Kasai hepatoportoenterostomy for biliary atresia

A 21-year-old female underwent a Kasai hepatoportoenterostomy with Roux-en-Y reconstruction for typeIII biliary atresia at age 63 days. At the age of 19 years, she developed cholangitis and CT scan revealed hepatolithiasis. She presented for treatment of the intrahepatic stone and the hepatportoenterostomy was directly visualized with double-balloon endoscopy (DBE). Endoscopic findings showed multiple intrahepatic bile ducts open to the jejunum through multiple orifices. Cholangiography showed narrowing of intrahepatic bile duct branches with a "fire-like" appearance. These findings have not been previously reported, since endoscopic approaches to patients with a hepaticojejunostomy were limited. DBE was useful to directly visualize the anastomosis in a patient status-post the Kasai operation for biliary atresia with a Rouxen-Y reconstruction.

Results of Kasai Operation for Biliary Atresia in a Single Institution

Results of Kasai Operation for Biliary Atresia in a Single Institution

Long-Term Outcome of Living Donor Liver Transplantation After Kasai Operation for Biliary Atresia: A More Than 15 Years Follow-Up in a Single Center.

Background. Since our department firstly introduced Kasai operation from 1960's, Kasai operation has been preferred as initial treatment for biliary atresia (BA), although long-term survival was not sufficient. After the introduction of liver transplantation, the long-term outcome of BA dramatically improved. We started living donor liver transplantation (LDLT) from 1991, allowing us to examine issues related to long-term survival. The aim of this study was to review the long-term outcome of LDLT after Kasai operation. Patients and Methods. We performed 160 LDLT from 1991 to 2013, including 22 biliary atresia patients who survived more than 15 years after LDLT. They were investigated for complication, immunosuppressive therapy and social status. Results. The average age at LDLT was 3.0 years old (from 5 months to 16 y.o.). The present average age is 20.5 y.o. (from 15 - 30 y.o.). The average age at Kasai operation was 67 days, and the average interval from Kasai operation to LDLT was 41 months. Five of the 22 recipients were frequently readmitted due to biliary stenosis even more than 5 years after LDLT. The present immunosuppressive therapy is tacrolimus or cyclosporine alone, and one recipient become immunosuppression free. Chronic rejection and secondary liver failure were not seen. All these patients, except for 1, attend a normal school or have an occupation. Conclusion. General status of the recipients was stable more than 15 years after LDLT. Biliary stenosis was the most important factor in terms of the long-term outcome.

Rescue Therapy with Infliximab for Steroid Resistant Refractory Acute Rejection in Liver Transplant Patients: A Report of Two Cases

Background: Long-term survival after liver transplantation has improved gradually; however, late-onset acute rejection and chronic rejection still remain obstacles for long-term survival. Among late-phase episodes, steroid-resistant refractory rejection may be a crucial stage regarding the possibility of graft failure, and therefore an appropriate therapeutic strategy is urgently needed. We introduce the use of infliximab, which is recognized as a promising anti-rejection agent in small bowel transplantation, as a rescue therapy agent in liver transplant patients. Methods and patients: Prior to clinical use, this trial was approved by the committee overseeing the quality of medicine and ethical issues at Mie University Hospital. Patient criteria are as follows: 1. liver transplant recipients exhibiting steroid-resistant acute rejection; 2. plasma TNF-α elevated beyond the normal range (2.8 pg/ml). Between 7/2011 and 1/2012, we included 2 patients in this trial. Case 1: A 31-year-old male received ABO-incompatible living-related liver transplantation (LDLT) from his brother in May 2005 due to liver failure after a Kasai operation for biliary atresia. He had been treated with calcineurin inhibitor (CNI), mycophenolate mofetil (MMF) and steroids until the beginning of 2010, but liver transaminase levels started to increase after MMF and steroids were tapering off. A liver biopsy performed in December 2010 suggested late-onset acute rejection. MMF and steroid treatment were re-started and liver transaminase levels returned to normal. However, a second rejection episode occurred in August and subsequent steroid bolus therapy (500 mg/body, 3 days) was followed by EBV-related lymphoproliferative disease with a high EBV-DNA titer (20000 copies/ml) and symptoms of high fever and lymphadenopathy. All immunosuppressive drugs were discontinued, but a second acute rejection occurred immediately and steroid bolus therapy was now ineffective. After confirming high plasma TNF-α (12.9 pg/ml), the patient was administered 300 mg of infliximab (5 mg/kg body weight) in addition to drug conversion from CNI to MMF monotherapy in October 2011. After infliximab administration, liver transaminase levels gradually returned to normal. Following a second infliximab therapy in December 2011, the patient has been treated with MMF+steroid. Case 2: A 56-year-old male underwent LDLT from his son in June 2008 due to acute liver failure caused by primary biliary cirrhosis. He was treated with CNI+steroid, but acute rejection occurred in February 2011 probably due to reduction of CNI for renal impairment. Steroid bolus therapy was introduced and followed with CNI+MMF+steroid treatment. However, 2 episodes of acute rejection occurred in November and December of 2011 in spite of steroid bolus therapy. The highest plasma TNF-α level was 8.4 pg/ml and the patient was administered 300 mg of infliximab (5 mg/kg body weight) following diagnosis of refractory acute rejection. After infliximab therapy, liver transaminase levels decreased to around the normal range. Conclusions: The effectiveness of infliximab has been known for intestine transplantation and graft-versus-host disease after bone marrow transplantation. Although further research is essential, infliximab is a potential agent for refractory acute rejection in liver transplantation recipients.

Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation

This study was conducted to reappraise the efficacy of redo-Kasai (or revision) in the era of liver transplantation as a treatment option in those patients with recurrent jaundice after initially successful Kasai procedure. We studied ten patients that received redo-Kasai, among a total of 102 patients diagnosed with biliary atresia after receiving Kasai operation from 1986 to 2011. Kasai operation was done at a median age of 55 days and redo-Kasai at 150 days. The bilirubin levels returned to normal in six patients after the procedure. Four of six enjoyed jaundice-free survival with native liver till the time of last follow-up. Three patients died and three received liver transplantation (LT). Only one out of seven patients with three or more episodes of cholangitis survived with native liver, while all the three patients with 1 or 0 episode survived with native liver. The difference was significant (P = 0.033). Re-do Kasai did not result in more blood loss or operative time during LT. Redo-Kasai is still valuable in the era of LT and the episodes of cholangitis are the decisive factors affecting the outcome of the procedure.

The outcome of laparoscopic portoenterostomy for biliary atresia in children

There were only a few reports on laparoscopic portoenterostomy for biliary atresia in children. We report our experience on laparoscopic Kasai operation for biliary atresia. A retrospective review of all children who had biliary atresia underwent laparoscopic portoenterostomy from January 2002 to September 2006 were included and analyzed. Sixteen children (five boys and eleven girls) with a mean age of 66 days (range 47-106 days) at operation were included in this study. All patients had type III biliary atresia. There was no conversion to open procedure. The operative time ranged from 193 to 435 min (mean 292 min). At a median follow-up of 72 months (range 33-89 months), eight patients were free of jaundice with bilirubin level <20 μmol/L and did not require liver transplantation. Seven patients underwent liver transplantation. One patient who had persistent elevated bilirubin defaulted follow-up. One patient complicated with volvulus of intestine post-operatively. All patients survived except one patient who had combined liver and intestine transplantation. Laparoscopic portoenterostomy in children with biliary atresia is technically feasible. 50% of children who had type III biliary atresia were free of jaundice and did not require liver transplantation. A longer follow-up is required to assess the long-term outcome.

Tentative Proposal of Optimal Timing of Kasai Operation for Biliary Atresia Based on Fibroscan Results

Purpose: Based on previous research findings, it is well-known that the timing of surgery is generally considered the most important prognostic factor for a Kasai por toenterostomy, the primary treatment for biliary atresia. This research aimed to identify the optimal timing of a Kasai portoenterostomy and to verify if the proposed optimal timing corresponds to previous studies. All patients were classified by the timing of surgery, and pre- and post-operative fibrotic changes of the liver were measured with the elasticity value from fibroscans. Methods: The stiffness scores of the pre- and post-operative fibroscans in 34 patients who were treated by Kasai portoenterostomy from October 2007 to September 2010 in Severance children's hospital were reviewed. Results: The earlier the patients were treated by Kasai portoenterostomy, the lower the fibroscan scores. When the patients were treated prior to the 8th week, the post-operative scores of the fibroscans were significantly better than those patients who were treated after the 8th week, and some even recovered partially. Moreover, when operated before the 8th week, the differences between each pre- and post- operative fibroscan score also showed statistical relevance (p=0.0002). Conclusion: The earlier the patient was treated by Kasai portoenterostomy, the less liver fibrosis that developed, the lower the level of post-operative fibrosis, and the less the degree of fibrotic progress before and after the operation. Thus, this research proposal reconfirms once more that the 8th week is the optimal timing for a Kasai portoenterostomy. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 74∼80)

Liver Transplantation for Primary Hepatic Tumors in Children

Background: Primary liver tumors account for less than 2% of pediatric malignancies, and the best treatment is complete surgical excision. The aim of this study was to review the results of liver transplantation (LT) for primary hepatomas in children. Methods: The medical records of patients who underwent LT for unresectable primary hepatoma between May 1996 and December 2009 were reviewed retrospectively. Results: Seven of 130 patients (5.3%, M:F=4:3) underwent LT for unresectable hepatoma. The median age at transplantation was 9 years (range, 6 months-14 years). Two patients were transplanted for hepatitis B virus-associated hepatocellular carcinoma (HCC), 2 for hepatoblastoma, 1 for hemangioendothelioma, 1 for angiosarcoma, and 1 for intrahepatic cholangiocarcinoma after a Kasai operation for biliary atresia. There was no post-LT treatment except in patients with HCC who were taking immunoglobulin prophylaxis against hepatitis B. Four patients (2 HCC, 1 hepatoblastoma, 1 hemangioendothelioma) are now alive and well after 7.8, 7.2, 7.7, 6.3 years of follow-up, respectively. Three patients died after transplantation; 1 for the recurrent cholangiocarcinoma in the transplanted liver 1 year after the transplantation and 1 who underwent LT for the recurrent hepatoblastoma for the primary non-function 10 days after the transplantation. One patient died of metastatic angiosarcoma (bone) 2.5 years after LT. Conclusions: LT can be tried for unresectable primary hepatoma in children and, although limited, the outcome was successful in patients with HCC, hepatoblastoma, or hemangioendothelioma. Careful patient selection, based on the pre-transplant histological diagnosis, seems to be related to better outcome.

Impact of Age at Kasai Operation on Its Results in Late Childhood and Adolescence: A Rational Basis for Biliary Atresia Screening

Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence. All patients with biliary atresia who were living in France and born between 1986 and 2002 were included. Median follow-up in survivors was 7 years. Included in the study were 743 patients with biliary atresia, 695 of whom underwent a Kasai operation; 2-, 5-, 10-, and 15-year survival rates with native liver were 57.1%, 37.9%, 32.4%, and 28.5%, respectively. Median age at Kasai operation was 60 days and was stable over the study period. Whatever the follow-up (2, 5, 10, or 15 years), survival rates with native liver decreased when age at surgery increased (< or =30, 31-45, 46-60, 61-75, and 76-90 days). Accordingly, we estimated that if every patient with biliary atresia underwent the Kasai operation before 46 days of age, 5.7% of all liver transplantations performed annually in France in patients younger than 16 years could be spared. Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. These findings indicate a rational basis for biliary atresia screening to reduce the need for liver transplantations in infancy and childhood.

Successful pediatric living donor liver transplantation from carrier to carrier of hereditary butyrylcholinesterase variant

Hypocholinesterasemia is often observed clinically, especially in various liver diseases. Not well known, however, is the fact that some patients have a hereditary BChE variant. Little has been reported on liver transplants associated with this hereditary BChE variant. Furthermore, no cases have been reported of a LDLT involving hereditary BChE variant that had been diagnosed preoperatively. A 23-month-old girl who had had a failed Kasai operation for biliary atresia underwent a liver transplant using as a graft her father's lateral segment. Preoperatively, she had been diagnosed with hypocholinesterasemia. As the donor, her father had undergone a preoperative examination, during which he was found to also have hypocholinesterasemia. DNA sequencing revealed that both had the hereditary BChE variant. The unique mutation caused a frame-shift mutation. Variant K was also detected. The patient was discharged 143 days after the operation and has had no problems with immunosuppression since. In conclusion, we report that the hereditary BChE variant is not a contraindication for either transplantation or living liver donation.

Short-term Results of Kasai Operation for Biliary Atresia: Experience from One Institution

The purpose of this study is to review the short-term outcome of patients with biliary atresia (BA) treated by the Kasai operation at our institution. Ninety-two BA patients treated by the Kasai operation between January 1996 and December 2002 were reviewed. The diagnosis of BA was confirmed by intraoperative cholangiography. The outcome of treatment was categorized into two groups: jaundice-free (total bilirubin < 2 mg%) and persistent jaundice (>or= 2 mg%). The outcome of Kasai operation was evaluated 1 year after surgery. Data are expressed as mean +/- SD. Average age at the time of surgery was 90.26 +/- 36.44 days. Only 22.8% (21/92) of patients had Kasai operation before 60 days of age. Histologically, 49 patients (54.4%) had liver fibrosis at the time of surgery. Of 92 patients, 17 were not included in outcome evaluation as they were less than 1-year postsurgery. Therefore, 75 patients could be evaluated for the outcome. Thirty-eight patients (50.67%) were jaundice-free 1 year after surgery. Liver histology and age at the time of the Kasai operation did not influence early outcome. The most common complication was ascending cholangitis. Half of our BA patients who underwent Kasai operation were jaundice-free 1 year after surgery. The lack of impact of age and liver pathology on outcome is presumably due to the briefness of the follow-up. In general, our patients underwent Kasai procedure too late. It is therefore important for us to conduct a campaign to highlight the plight of these patients and the urgency of referral for neonates with jaundice.

Late-presenting cholangitis and Roux loop obstruction after Kasai portoenterostomy for biliary atresia

Background/Purpose Late-onset cholangitis is an uncommon complication after the Kasai operation for biliary atresia. Experience with 3 recent patients illustrates appropriate management. Methods Retrospective review of patients with late unexplained deterioration in liver function attributed to cholangitis. All underwent quantitative radioisotope hepatobiliary scans to identify the Roux loop obstruction and confirm postoperative resolution. The role of percutaneous transhepatic cholangiography and computed tomography scanning was reviewed. Results Three patients aged 8, 13, and 17 years were identified. All 3 had had a successful Kasai portoenterostomy for type 3 biliary atresia and presented after several virtually trouble-free years with late-onset cholangitis. The radioisotope scans in all 3 patients showed poor tracer uptake by the liver and pooling of the radioisotope at the porta hepatis. Laparotomy confirmed a proximal Roux loop obstruction at the level of the mesocolic window in all. This required mobilization and adhesiolysis and, in 1, correction of an actual stenosis within the wall of the intestine at this level. All patients had normalization of their liver function postoperatively. All are now well and anicteric at a follow-up of 6 to 12 months. Conclusions Long-term survivors of the Kasai portoenterostomy for biliary atresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction. Resolution of the obstruction allows preservation of their native liver.