Native Liver Survival Rate Research Articles

Does the identity of the bacteria matter in post-Kasai cholangitis? A comparison between simple and intractable cholangitis

PurposeThis study compared the bacteriology and clinical outcomes between simple (SC) and intractable cholangitis (IC) after Kasai operation. MethodsPost-Kasai patients (n = 192) from 1980 to 2015 were retrospectively reviewed. The results of blood culture and clinical outcomes between the patients with SC and IC were compared. Main resultsA total of 102 cholangitic episodes in 68 patients were analyzed (SC vs IC = 76 vs 26). There were more IC episodes within the first year of Kasai operation (SC vs IC = 36.8% vs 61.5%, p = 0.022). The most common bacteria identified in SC and IC groups were Escherichia Coli and Staphylococcus aureus. Until the latest follow up, the native liver survival rates in patients with SC and IC were 75.0% and 50.0% (p = 0.89). Among the patients with IC, the native liver survival rate was significantly better in those with a positive culture (100% vs 20%, p = 0.001). ConclusionIntractable cholangitis is a common complication within the first year of Kasai operation and may be caused by a different spectrum of organisms. The identification of the bacteria by blood culturing may result in a better treatment outcome. Level of evidenceLevel III.

The Application of a Shorter Loop in Kasai Portoenterostomy Reconstruction for Ohi Type III Biliary Atresia: A Prospective Randomized Controlled Trial

BackgroundA 30-40 cm Roux loop is widely accepted in Kasai portoenterostomy in infants with Ohi Type III biliary atresia (BA), regardless of patient's size, to avoid reflux cholangitis. However, the redundant length of the jejunal limb may lead to complications; hence, we compared the outcome of a shorter Roux loop with the standard loop length in Kasai portoenterostomy in infants with Ohi Type III BA. MethodsThere were 166 patients with Ohi Type III BA who underwent the Kasai procedure and were prospectively randomized into two groups: (1) the long Roux loop group (LRLG) contained 91 patients, where a standard 30-40 cm Roux loop was used regardless of the child's size and (2) the short Roux loop group (SRLG) included 75 patients in which the Roux loop length was based on the distance between hepatic hilum and umbilicus. The follow-up periods were 3 to 50 mo. Contrast-enhanced ultrasonography of the upper gastrointestinal tract was studied, and laboratory tests were included during the follow-up period. ResultThe mean Roux loop length of SRLG was 17.42 ± 3.91 cm (13-20 cm); it was significantly shorter than that of the LRLG (32.87 ± 3.91 cm [30-40 cm], P = 0.00). There were no significant differences between the LRLG and SRLG in terms of age, body weight at time of the operation, length of operation, blood loss during the operation, and length of postoperative hospital stay (64.50 ± 20.41 d versus 68.34 ± 17.59 d, 4.3 ± 0.8 kg versus 4.1 ± 1.2 kg, 187.6 ± 35.7 min versus 172.3 ± 20.4 min, 21.71 ± 9.41 mL versus 25.32 ± 10.82 mL, 9.01 ± 3.97 d versus 8.16 ± 2.40 d; P = 0.63, 0.47, 0.09, 0.89, and 0.61, respectively). Six months after the operation, the clearance of jaundice rate in the LRLG was 45.1% (41/91), while the rate was 50.7% (38/75) in the SRLG; there was no statistically significant difference (P = 0.47). The incidence of cholangitis in the LRLG was 42.9% (39/91), which was similar to that of the SRLG (46.7%, 35/75) (P = 0.62). The incidence of overall complications (such as intestinal obstruction, gastrointestinal bleeding, bile leak, and anastomotic stenosis) in the SRLG was lower than that of the LRLG, but the difference did not reach statistical significance (4/91, 4.4% versus 5/75, 6.7%; P = 0.76). According to the Kaplan–Meier survival analysis and log rank test, the native liver survival rate in the LRLG and the SRLG also showed no significant difference (P = 0.964, log rank test). Grade I reflux was detected in three patients in the LRLG and two patients in the SRLG (P = 0.54). However, the reflux subsided 6 months later in all five patients. ConclusionsA shorter Roux-loop length in Kasai portoenterostomy reconstruction had a similar efficacy as the conventional Roux-loop length.

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.

Japanese Biliary Atresia Registry.

The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS). JBAR employed an initial questionnaire, a questionnaire for liver transplantation, and a follow-up questionnaire. The questionnaire has been sent to the pediatric surgeons of JBAS member institutions and the hospitals in which council members of the Japanese Society of Pediatric Surgeons are working. One hundred and twenty-three hospitals have been included in JBAR between 1989 and 2015. Each patient is to be followed up for 30years. Between 1989 and 2015, 3160 patients have been registered in JBAR. Among them, 1236 patients underwent liver transplantation. Follow-up questionnaire is also going on. 20-year overall and native-liver survival rates were 89 and 49%, respectively. The surgical outcome of BA has markedly improved owing to the co-operation of Kasai portoenterostomy and liver transplantation. Japanese big database of BA is now available.

Correlation between gamma-glutamyl transpeptidase activity and outcomes after Kasai portoenterostomy for biliary atresia

Background/purposeThe role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA). MethodsData were retrospectively reviewed for 169 BA patients categorized into jaundice-free (JF) (total bilirubin <2.0 mg/dL ≤6 months post-KPE) and persistent jaundice (PJ) groups (total bilirubin ≥2.0 mg/dL ≤6 months post-KPE). Serum biochemical markers, including GGT levels, were measured monthly after KPE, and mean GGT levels and APRi were compared between groups. Factors predicting native liver survival (NLS) were determined using a Cox regression analysis. ResultsGGT concentrations >550 IU/L at month 5 (hazard ratio: 1.74, P < 0.05), an APRi >0.605 at month 4 (hazard ratio: 3.78, P = 0.001), and being jaundice-free at 6 months (hazard ratio: 5.49, P < 0.001) were independent risk factors for decreased NLS. ConclusionsSerum GGT concentrations >550 IU/L at month 5 and an APRi >0.605 at month 4 post-KPE were associated with significantly lower NLS rates. Among JF patients, those with GGT concentrations >550 IU/L at month 5 and APRi >0.605 at month 4 showed poorer outcomes. Type of studyRetrospective comparative study Level of evidenceLevel III.

Screening for biliary atresia.

Early diagnosis followed by proper KP is essential for the improvement of long-term prognosis for patients with BA. It is increasingly accepted that KP at ≤ 30days of age significantly improves native liver survival rate. Published analyses in English and Japanese indicate that screening by SCC and DB/CB is potentially feasible. Screening with SCC has been implemented in Tochigi Prefecture, Japan, since 1994. The concept of SCC was introduced from Japan to Taiwan and resulted in nationwide screening with SCC for the first time in Taiwan in 2004, followed by Japan in 2012. Home-based screening using SCC is easy and cost-effective; however, it may cause some difficulties for families in case of stools with intermediate colors. Laboratory-based screening using DB/CB may detect the suspected cases earlier, resulting in an increase in the number of patients with BA who undergo KP at ≤ 30days of age; however, the recall rate is 1% and may be beyond an acceptable range. Further studies are needed to assess the feasibility and cost-effectiveness of both home-based (SCC) and laboratory-based (DB/CB) screening for BA.

Learning Curve Analysis of Laparoscopic Kasai Portoenterostomy.

To compare the surgical outcomes of Kasai Portoenterostomy (PE) and investigate the learning curve of laparoscopic Kasai Portoenterostomy (Lap-PE). Retrospective chart review of 80 cases of biliary atresia (type III) undergoing Lap-PE at Shenzhen Children's Hospital from January 2011 to June 2015, all of which were performed by the same surgical team. According to the operative sequences, the cases were equally divided into four phases (Phase I, II, III, and IV), which contain 20 cases in each. The age, weight, and gender, the volume of intraoperative blood loss, the postoperative clearance rate of bilirubin, the incidence of postoperative cholangitis, and the first and second year native liver survival rate were all reviewed. There was no significant difference among the four phases regarding age, weight at operation, and gender (P > .05). Statistical difference was observed with respect to operative time, the volume of intraoperative blood loss, the postoperative jaundice clearance, and the first and second year native liver survival rate among Phase III and IV compared to Phase I and II (P < .05), but showed no significant difference in neither the first two phases nor the latter two phases (P > .05). The incidence of postoperative cholangitis showed no significant variation among the four phases. A surgeon is able to become more experienced after performing approximately 40 laparoscopic Kasai Portoenterostomys.

Outcome of Biliary Atresia After Kasai's Portoenterostomy: A 15-year Experience.

To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. Medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year). 121 patients (61.9% males) were included; 32 (26.5%) were lost to follow-up at 1 year. At last follow-up, out of the 89, 42 (47.2%) were alive, 29 (32.6%) were jaundice-free, and 47 (52.8%) had died. The native liver survival rate at last follow up was 43.8%. 42 (47.2%) patients had complete clearance of jaundice at 3 months post-procedure. Jaundice-clearance rate was significantly high in patients alive (83.3% vs 16.7%, P<0.001)) as compared to those who died later. Jaundice clearance at 3 months post surgery is a good early indicator of long term success.

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.

Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival. Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 μmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 μmol/L, albumin >35 g/L; 2: TB ≤74 μmol/L, albumin ≤35 g/L; 3: TB >74 μmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy. Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.

Variability of diagnostic approach, surgical technique, and medical management for children with biliary atresia in Canada — Is it time for standardization?

BackgroundThe Canadian 4-year native liver survival rate for biliary atresia (BA) after Kasai Portoenterostomy (KP) is 39%. The Canadian Biliary Atresia Registry (CBAR) was used to examine variability of surgical and medical management of BA. MethodsGastroenterologists and surgeons in all 14 Canadian pediatric tertiary centers were invited to complete an online survey of their BA management practices. ResultsOf gastroenterologists, diagnostic procedures included liver biopsy (92%), HIDA scan (58%), and percutaneous cholangiogram (46%). Surgeons reported Roux-en-Y lengths of 20–50cm with 78% avoiding diathermy at the portal plate; 16% performed laparoscopic exploration, but none laparoscopic KP. Postoperative corticosteroids and antibiotics were used by 24% and 85% of gastroenterologists, respectively, with similar rates for surgeons. At discharge, gastroenterologists prescribed oral antibiotics (80%), and ursodeoxycholic acid (95%), while surgeons reported lower rates (62% and 55%). Considerable variation existed in follow-up monitoring. No center had a standard protocol for evaluating suspected cholangitis. There was a lack of consensus for defining failed KP and referral criteria for transplant evaluation. ConclusionIn Canada, treatment of BA is not centralized, and there is variability in diagnostic approaches and management. Collaboration through CBAR will allow for implementation and evaluation of standardized surgical and medical management with a goal to improve outcomes. Level of evidenceSurvey study. Level IV evidence.

Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: A systematic review

AimsBiliary atresia (BA) is a rare disease for which mainstay of treatment consists of open Kasai portoenterostomy. The aim of this review was to assess the outcomes of laparoscopic Kasai portoenterostomy, which offers potential benefits of minimally invasive surgery. Outcomes identified were postoperative cholangitis rates, incidence of adhesions at subsequent liver transplantation, native liver survival rates and actuarial survival rates. MethodsA comprehensive systematic literature search was conducted in the PubMed and Cochrane databases using the keywords hepatic portoenterostomy, biliary atresia and laparoscopy. Robotic cases were excluded. ResultsTen studies (n=149 patients) were included in this review. The mean age at the time of operation was 66 (range 14–119) days. The mean operative time was 261 (range 120–662) minutes. The rate of postoperative cholangitis was 34% (range 11%–50%). The mean native liver survival rate was 57% (range 33%–78%) at 6months and 47% (range 8%–76%) at 2years. Mean actuarial survival rate was 87% (range 54%–100%) at 2years. Subsequent adhesions were reported in 4 patients. Two patients had dense adhesions and 2 had no adhesions. ConclusionsAlthough laparoscopic Kasai portoenterostomy is a feasible operation, outcomes in terms of native liver survival rates and actuarial survival rates are unfavourable compared to conventional surgery. There is no evidence that laparoscopic Kasai is associated with fewer adhesions at subsequent liver transplantation. Level of evidenceIII. Type of study: Treatment study.

Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre.

Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evaluates prognostic factors for outcome. All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 µmol/L within 6 months after KP. Two-year and 5-year native liver survival (NLS) were determined. Prognostic factors examined included gender, ethnicity, associated anomalies, age at KP, post-KP cholangitis and clearance of jaundice within 6 months. Of 58 patients studied, 31(53.4%) were male. Median age at time of KP was 53 days (range: 28-127). Ethnic distribution showed 32 (55.2%) Chinese, 16 (27.6%) Malays and 10 (17.2%) others. Twenty-one (36.2%) patients achieved jaundice clearance by 6 months. Two-year NLS rate was 36 out of 50 (72%), while 5-year NLS rate was 16 out of 35 (45.7%). Only clearance of jaundice within 6 months had a significant association with NLS (P = 0.006). All other factors showed no significant impact on outcome. Our short- and medium-term outcomes after KP for BA are comparable with those reported by most international centres. However, prognostic factors such as age at KP, cholangitis episodes and associated anomalies did not show significant correlation; only clearance of jaundice within 6 months was significantly predictive of NLS.

Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis.

It is controversial whether adjuvant steroid treatment should be given to biliary atresia (BA) patients following a Kasai portoenterostomy (KPE). This study aimed to quantitatively and systematically evaluate the effect of adjuvant steroid therapy post-KPE in relation to major clinical outcomes of BA patients. We systematically reviewed the literature in PubMed, Embase, the Cochrane Library, China Knowledge Resource Integrated Database, Wanfang Database, Scholarly and Academic Information Navigator and manually searched for relevant papers published before August, 2015. We extracted data on the effects of steroid treatment following KPE on clinical outcome, including jaundice free rate and native liver survival rate at 6 months, 1 or 2 years after KPE. The weighted overall relative risk (RR) and 95% confidence intervals (CIs) were calculated by using a random-effects model. Eight cohort studies and two randomized controlled trials (RCTs) were identified (n=998). Of them, 6 cohort studies and 2 trials investigated the effect of steroid treatment as compared to non-users or placebo (n=566), and 2 cohort studies compared the effects of high-dose to low-dose steroid treatment (n=432). Steroid usage increased the clearance rates of jaundice at 6 months (pooled RR: 1.32; 95% CI: 0.995-1.76; I 2=72.6%) and 1 year (pooled RR: 1.35; 95% CI: 1.12-1.61; I 2=0.0%), but not 2 years (pooled RR: 0.82; 95% CI: 0.55-1.22; I 2=0.0%) after KPE. There was no solid evidence supporting that steroid treatment would improve native liver survival rate at 6 months (pooled RR: 1.02; 95% CI: 0.90-1.15; I 2=0.0%), 1 year (pooled RR: 1.10; 95% CI: 0.91-1.34; I 2=35.2%) or 2 years (pooled RR: 1.00; 95% CI: 0.73-1.35; I 2=57.4%) after KPE. Adjuvant steroid treatment following KPE may improve short-term (≤1 year) clearance rate of jaundice, but no significant effects on long-term (≥2 years) clearance rate of jaundice and native liver survival rate. Studies on doses and duration of steroids, and long-term follow-up studies are warranted.

Technical standardization of Kasai portoenterostomy for biliary atresia

BackgroundThe purpose of this study was to assess the clinical outcome of patients treated with the current technique of Kasai procedure compared with that of those treated with previous procedures. MethodsWe retrospectively assessed the outcomes of 256 patients with biliary atresia who underwent Kasai portoenterostomy at our hospital between 1972 and 2014. Patients were divided into four groups: group 1 (1972–1981, double Roux-en Y, n=91), group 2 (1982–1991, Suruga II, n=80), group 3 (1992–2000, double-valve Roux-en Y, n=46), and group 4 (2001–2014, total removal of the extrahepatic biliary remnants at hepatic capsule and Roux-en Y reconstruction with a spur valve, n=39). Clinical outcomes were compared between the four groups. ResultsIn groups 1, 2, 3, and 4, the rate of jaundice clearance was 65.9%, 77.5%, 63.0%, and 87.2%, respectively; incidence of early cholangitis was 60.4%, 53.8%, 37.0%, and 23.1%, respectively; requirement for redo Kasai surgery was 15.4%, 37.5%, 17.4%, and 5.1%, respectively; 10-year native liver survival rate was 53.8%, 60.1%, 44.1%, and 73.7%, respectively; and 10-year overall survival rate was 55.0%, 72.3%, 86.7%, and 97.3%, respectively. ConclusionThe standardized Kasai procedure was associated with favorable outcomes. Long-term outcomes remain to be evaluated. Level of evidenceCase–control/treatment study, level III.

The extended Kasai portoenterostomy for biliary atresia: A preliminary report.

Aims:In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing “extended” Kasai portoenterostomy (KPE), a modification of the original procedure.Materials and Methods:Since 2013, we have used the technique of “extended KPE” and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015).Results:Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months.Conclusion:In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA.

A prospective study comparing laparoscopic and conventional Kasai portoenterostomy in children with biliary atresia

ObjectiveThe objective of the study is to evaluate the outcome of laparoscopic Kasai portoenterostomy for type III biliary atresia in children. MethodsA total of 95 type III biliary atresia patients were treated at the Capital Institute of Pediatrics between September 2009 and August 2011. The patients were randomized into 2 groups preoperatively: the laparoscopic group (LP group, n=48) and the open group (OP group, n=47). In the LP group, 4 patients were converted to open operations, whereas the remaining 44 patients in the LP group and 47 patients in the OP group were included in the study. ResultsThe gender distribution and age of the LP group did not differ from those of the OP group (LP group F/M 21/23, median age 61.5days, OP group F/M 24/23, median age 67days, P=0.75 and 0.11). The operation time in the LP group was significantly longer than that in the OP group (median 169.5min vs 146min, P<0.01). Intraoperative blood loss in the LP group was significantly lower than in the OP group (median 10ml vs 15ml, P<0.01). The resumption oral intake was significantly faster in the LP group than in the OP group (median 3days vs 3days, P<0.01). There were no significant differences between the postoperative hospital stay in the two groups (median 12.5days vs 13days, P=0.21). The median follow-up period was 16months in the LP group and 17months in the OP group. There was no statistically significant difference in the jaundice clearance rate at the end of the third postoperative month or in the incidence of cholangitis, native liver survival rate, and liver function recovery between the 2 groups. ConclusionsThe short-term and mid-term results are comparable between the laparoscopic and open Kasai portoenterostomy groups. The laparoscopic Kasai procedure does not improve the prognosis of biliary atresia.

Long-term outcomes of biliary atresia with splenic malformation

BackgroundWe assessed the long-term outcomes of patients with biliary atresia with splenic malformation (BASM). MethodsWe retrospectively assessed outcomes of 255 patients who underwent the Kasai procedure (KP) at our hospital between 1972 and 2014. Clinical outcomes of 11 patients with BASM (group A: nine with polysplenia, two with asplenia) and 244 patients with isolated BA (group B) were compared. ResultsThe incidence of early cholangitis and hepatopulmonary syndrome (HPS) was significantly higher in group A than in group B. Of the 11 group A patients, three died of severe cardiac defects during early infancy. Seven became jaundice free following KP, with three patients subsequently requiring liver transplantation (LTx). Four survived with their native livers for 2, 5, 22, and 23years, respectively. Overall 20-year survival rates were 63.6% and 66.5% and 20-year native liver survival rates were 29.0% and 47.3% in groups A and B, respectively. No significant difference in cumulative survival rates was observed between both groups. ConclusionsLong-term outcomes in BASM patients without lethal cardiac defects were comparable to patients with isolated BA. Careful follow-up may be required in patients with BASM because of a potentially higher risk of secondary complications such as HPS.

From laparoscopic to open Kasai portoenterostomy: the outcome after reintroduction of open Kasai portoenterostomy in infant with biliary atresia

Apparently superior result was observed after open Kasai portoenterostomy in infant with biliary atresia. Our institute stopped performing laparoscopic portoenterostomy since 2007. We aimed to investigate the outcome after reintroduction of open portoenterostomy. 27 non-syndromic infants underwent open Kasai portoenterostomy from 2007 to 2012. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 μmol/L within 6 month of portoenterostomy), the native liver survival at 2 years after the operation were reviewed. The results were retrospectively compared with all 16 infants who underwent laparoscopic Kasai portoenterostomy before 2007. All infants had type III biliary atresia. No statistical difference was observed regarding the age at operation and the pre-operative bilirubin level. The early clearance of jaundice rate was 81% (22/27) after open operation and was 50% (8/16) after laparoscopic operation (p = 0.03). At 2 years after the operation, the native liver survival was 81% (22/27) after open operation and was 50% (8/16) after laparoscopic operation (p = 0.03). Reintroduction of open Kasai portoenterostomy was associated with superior early clearance of jaundice rate and 2-year native liver survival rate.

Analysis of the short-and mid-term curative effect and influence factors of hepaticojejunostomy for biliary atresia

Objective To explore the short-and mid-term curative effect and influence factors of hepaticojejunostomy(Kasai operation) for biliary atresia.Methods The clinical data of 66 children with biliary atresia treated with Kasai operation were analyzed retrospectively.The jaundice fading rate,2-year survival rate and influence factors of prognostic were analyzed.Results The jaundice fading rate of 62 cases was 58.1％ after Kasai operatiou.The patients also had a 2-year native liver survival rate of 53.2％.The jaundice fading rate,2-year survival rate of type Ⅰ,Ⅱ and Ⅲ had no significant differences (χ2 =1.91,0.76,all P ＞ 0.05).The 2-year survival rate of children underwent Kasai operation as younger than 60 days was higher rate than that of the children as 61 to 90 days and older than 90 days(χ2 =4.72,P ＜ 0.05).The 2-year survival rate of children with cytoinegalovirus infection and postoperative cholangitis were 43.6％,39.5％,respectively,which were lower than those of children without cytomegalovirus infection and postoperative cholangitis (69.6％.75.0％) (χ2 =3.92,7.46,all P ＜ 0.05).Conclusion Kasai operation is still the preferred method for the treatinent of biliary atresia.Type of the disease was not related with the short-and mid-term effects of Kasai operation.Age at operation was the main factor affecting the prognosis.Therefore,it is very important to make early surgery,enhance the anti-viral treatment and prevent postoperative cholangitis. Key words: Biliary atresia; Hepaticojejunostomy ; Treatment outcome

Early surgical outcomes and pathological scoring values of older infants (≥ 90 d old) with biliary atresia

PurposeThis study aimed to analyze the impact of age at Kasai operation on the short-term outcome of type III biliary atresia (BA) and to discuss if pathological scoring can be the prediction of effect in the Kasai procedure for the older (≥90days) infant. MethodsDuring the period 2004 through 2010, 452 infants with type III BA were reviewed. The relationship between ages at Kasai operation and jaundice clearance rates and two year native liver survival rates were assessed, retrospectively. Pathological slides were analyzed with a histological scoring system. ResultsAll of the patients were divided into 3 groups according to their ages at operation (group A: aged 60days or less (n=146), group B: age between 60 and 90days (n=222) , and group C: age on or over 90days (n=84)). The worst outcome of clearance of jaundice was found in group A but not in group C 2weeks after the operation (P<0.05). Jaundice clearance rates showed no difference among the three groups either at 3-months or 6-months after operation. Moreover, in group C patients, the pathological scores showed no difference between the jaundice clearance group and jaundice persistence group 6months after surgery. In group C, two year survival rate of patients with native livers was 36.1%. ConclusionSome patients over 90days of age at surgery can still benefit from a Kasai procedure. The pathological scoring system does not play a role in predicting jaundice clearance in patients over 90days of age at surgery.