Biliary atresia: East versus west.

Biliary atresia (BA) is a common cause of surgical jaundice during the neonatal period. It is currently considered as a spectrum of diseases with a common final pathology characterized by obliteration of the extrahepatic biliary tract and the absence of normally branching intrahepatic ducts. Though it is a global disease that can be found in all ethnicities there are some clear differences between BA arising in the East and the West. This is likely to be related to different genetic, environmental and cultural factors. BA is more frequently found in Far Eastern infants (both Chinese and Japanese) though the syndromic associations are much less common. Many Eastern countries have national screening programmes not seen in the West possibly due to debate over its cost effectiveness in countries where incidence is low. Kasai portoenterostomy (KPE) is considered as the primary treatment of BA but its outcome still remains unsatisfactory across the region. Given the complexity of BA, it is unlikely that strategic advances could be made by the sole effort of individual countries and we believe that collaboration between the East and West is the way forward.

Diagnostic Yield of Newborn Screening for Biliary Atresia Using Direct or Conjugated Bilirubin Measurements.

Importance: Treating biliary atresia in newborns earlier can delay or prevent the need for liver transplant; however, treatment typically occurs later because biliary atresia is difficult to detect during its early stages. Objective: To determine the diagnostic yield of newborn screening for biliary atresia with direct or conjugated bilirubin measurements and to evaluate the association of screening implementation with clinical outcomes. Design, Setting, and Participants: A cross-sectional screening study of 124 385 infants born at 14 Texas hospitals between January 2015 and June 2018; and a pre-post study of 43 infants who underwent the Kasai portoenterostomy as treatment for biliary atresia at the region's largest pediatric hepatology center before (January 2008-June 2011) or after (January 2015-June 2018) screening implementation. Final follow-up occurred on July 15, 2019. Exposures: Two-stage screening with direct or conjugated bilirubin measurements. In stage 1, all newborns were tested within the first 60 hours of life, with a positive screening result defined as bilirubin levels exceeding derived 95th percentile reference intervals. In stage 2, infants who had a positive screening result in stage 1 were retested at or before the 2-week well-child visit, with a positive screening result defined as bilirubin levels greater than the stage 1 result or greater than 1 mg/dL. Main Outcomes and Measures: The primary outcomes of the screening study were sensitivity, specificity, positive predictive value, and negative predictive value based on infants testing positive in both stages. The reference standard was biliary atresia diagnosed at the region's pediatric hepatology centers. The primary outcome of the pre-post study was the age infants underwent the Kasai portoenterostomy for treatment of biliary atresia. Results: Of 124 385 newborns in the screening study, 49.2% were female, 87.6% were of term gestational age, 70.0% were white, and 48.1% were Hispanic. Screening identified the 7 known infants with biliary atresia with a sensitivity of 100% (95% CI, 56.1%-100.0%), a specificity of 99.9% (95% CI, 99.9%-99.9%), a positive predictive value of 5.9% (95% CI, 2.6%-12.2%), and a negative predictive value of 100.0% (95% CI, 100.0%-100.0%). In the pre-post study, 24 infants were treated before screening implementation and 19 infants were treated after screening implementation (including 6 of 7 from the screening study, 7 from screening at nonstudy hospitals, and 6 from referrals because of clinical symptoms). The age infants underwent the Kasai portoenterostomy was significantly younger after screening was implemented (mean age, 56 days [SD, 19 days] before screening implementation vs 36 days [SD, 22 days] after screening implementation; between-group difference, 19 days [95% CI, 7-32 days]; P = .004). Conclusions and Relevance: Newborn screening with direct or conjugated bilirubin measurements detected all known infants with biliary atresia in the study population, although the 95% CI around the sensitivity estimate was wide and the study design did not ensure complete ascertainment of false-negative results. Research is needed in larger populations to obtain more precise estimates of diagnostic yield and to better understand the clinical outcomes and cost-effectiveness of this screening approach.

Management of Biliary Atresia in France 1986 to 2015: Long-term Results.

OBJECTIVES: This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available. METHODS: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. RESULTS: A total of 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 µmol/L) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6-199) was stable over time. Survival with native liver after KOp was 41%, 35%, 26%, and 22% at 5, 10, 20, and 30 years, stable in the 4 cohorts. 25-year survival with native liver was 38%, 27%, 22%, and 19% in patients operated in the first, second, third month of life or later, respectively (P = 0.0001). Center caseloads had a significant impact on results in the 1986 to 1996 cohort only. 16%, 7%, 7%, and 8% of patients died without LT in the 4 cohorts (P = 0.0001). A total of 753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. Five-year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (P < 0.0001). Actual BA patient survival (from diagnosis) was 81%. Five-year BA patient survival was 72%, 88%, 87%, and 87% in cohorts 1986 to 1996, 1997 to 2002, 2003 to 2009, and 2010 to 2015, respectively (P < 0.0001). CONCLUSIONS: In France, 87% of patients with BA survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.

Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood.

BACKGROUND & AIMS: In patients with biliary atresia (BA), the rate of native liver survival (NLS) to adulthood has been reported as 14-44% worldwide. Complications related to portal hypertension (PHT) and cholangitis are common in adulthood. For those requiring liver transplantation (LT), the timing can be challenging. The aim of this study was to identify variables that could predict whether young people with BA would require LT when they are >16 years of age. METHODS: This study was a single-centre retrospective analysis of 397 patients who underwent Kasai portoenterostomy (KP) between 1980-96 in the UK. After KP, 111/397 (28%) demonstrated NLS until 16 years of age. At final follow-up, 67 showed NLS when >16 years old (Group 1) and 22 required LT when >16 years old (Group 2). Laboratory, clinical and radiological parameters were collected for both groups at a median age of 16.06 years (13.6-17.4 years). RESULTS: The need for LT when >16 years old was associated with higher total bilirubin (hazard ratio 1.03, p = 0.019) and lower creatinine (hazard ratio 0.95, p = 0.040), at 16 years, on multivariate analysis. Receiver-operating characteristic curve analysis demonstrated that a total bilirubin level of ≥21 µmol/L at 16 years old (AUROC = 0.848) predicted the need for LT when >16 years old, with 85% sensitivity and 74% specificity. Cholangitis episode(s) during adolescence were associated with a 5-fold increased risk of needing LT when >16 years old. The presence of PHT or gastro-oesophageal varices in patients <16 years old was associated with a 7-fold and 8.6-fold increase in the risk of needing LT, respectively. CONCLUSIONS: BA in adulthood requires specialised management. Adult liver disease scoring models are not appropriate for this cohort. Bilirubin ≥21 µmol/L, PHT or gastro-oesophageal varices at 16 years, and cholangitis in adolescence, can predict the need for future LT in young people with BA. Low creatinine at 16 years also has potential prognostic value. LAY SUMMARY: Patients with biliary atresia commonly require liver transplantation before reaching adulthood. Those who reach adulthood with their own liver are still at risk of needing a transplant. This study aimed to identify tests that could help clinicians predict which patients with biliary atresia who reach the age of 16 without a transplant will require one in later life. The study found that the presence of bilirubin ≥21 µmol/L, lower creatinine levels, and a history of portal hypertension or gastro-oesophageal varices at 16 years, as well as cholangitis in adolescence, could predict the future likelihood of needing a liver transplant for young people with biliary atresia.

Predictors of Need for Liver Transplantation in Children Undergoing Hepatoportoenterostomy for Biliary Atresia.

PURPOSE: The purpose of this study was to determine perioperative risk factors for need of liver transplantation following hepatoportoenterostomy. METHODS: A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia at our institution from 1990 to 2016 was completed. RESULTS: A total of 81 patients were identified with a median age of 51 days (IQR: 33-68) at hepatoportoenterostomy and a median follow-up time of 5.7 years (IQR: 1-11.6). Ten-year overall survival was 93% (95% CI: 84-97). Thirty-six patients (44%) ultimately required transplantation at a median time from hepatoportoenterostomy of 8.9 months (IQR: 5.2-19). The 10-year transplant-free survival was 36% (95%CI: 24-49). Steroid use (N=42) was not associated with improved 10-yr transplant-free survival (33% vs. 38%, p=0.690). Age at hepatoportoenterostomy was not significantly associated with the need for transplantation. Multivariable logistic regression analysis demonstrated that total bilirubin >2mg/dL (OR: 97, p<0.001) and albumin < 3.5g/dL (OR: 24, p=0.027) at 3 months after surgery were independent predictors of the need for transplantation, while adjusting for age, sex, prematurity, and steroid use. CONCLUSION: Overall survival for children with biliary atresia is excellent, although most patients will ultimately require liver transplantation. Total bilirubin and albumin level at 3 months following hepatoportoenterostomy are predictive of the need for transplantation. Steroid use is not associated with improved outcomes.

Learning Curve Analysis of Open Kasai Portoenterostomy for Biliary Atresia.

BACKGROUND: Open Kasai portoenterostomy (KPE) remains the mainstay of treatment for biliary atresia (BA) even in the era of minimally invasive surgery and is generally considered to be technically challenging. This study describes the learning curve (LC) of open KPE and its effect on outcomes. MATERIALS AND METHODS: In our center, the caseload of BA was relatively low with average annual caseload below five in the past, and open KPE was started in the early 2000s. This study retrospectively enrolled the first 35 consecutive children undergoing open KPE for BA by the same surgeon in our center between June 2003 and April 2014. The LC was evaluated using the cumulative sum (CUSUM) method for operative time (OT). RESULTS: The CUSUM LC of OT, which peaked at the 23rd case, was best modeled as a second-order polynomial with equation CUSUM (min) = -0.8851 × case number2 + 33.54 × case number -45.978 (R2 = 0.7287). The LC consisted of two distinct phases: phase 1 (the initial 23 cases), representing the initial learning phase and phase 2 (the remaining 12 cases), representing acquisition of technical competency. Preoperative parameters were comparable between the two phases (each P > 0.05). Of note, OT and estimated blood loss significantly decreased in phase 2 compared with phase 1 (P < 0.001; P < 0.001, respectively). Furthermore, the rates of early jaundice clearance and 2-y native liver survival significantly increased in phase 2 (P = 0.032; P = 0.034, respectively). CONCLUSIONS: The two phases identified by CUSUM analysis of OT represents characteristic stages of LC for open KPE of the surgeon in our center. It is possible for surgeons to achieve competency of this demanding technique in centers with relatively low caseload of BA and late start of KPE.

Multicenter, retrospective, comparative study of laparoscopic and open Kasai portoenterostomy in children with biliary atresia from Japanese high-volume centers.

BACKGROUND: Multicenter study was undertaken to analyze the results of laparoscopic and open Kasai portoenterostomy. METHODS: Subjects were infants with type III biliary atresia who underwent open operation (n = 106) or laparoscopic operation (n = 21) between January 2012 and December 2015. Clinical data were compared between open and laparoscopic operations (2016-0534). Propensity score matching was performed to reduce the effect of treatment selection bias. Multivariate analyses were used to estimate the effect of the surgical approach on the jaundice clearance rate and the native liver survival rate. RESULTS: The postoperative jaundice clearance rate and the 1-year native liver survival rate were not significantly different between open and laparoscopic operations. Rates of cholangitis and major complications of laparoscopic operation were comparable to those of open operation. Blood loss, time to resume oral intake, time to drain removal, and duration of analgesic usage of laparoscopic operation were significantly superior to those of open operation. Similar results were observed when analysis was adjusted based on propensity score. Multivariate analyses demonstrated that only age at operation was a poor prognostic factor. CONCLUSION: Laparoscopic Kasai portoenterostomy was associated with several favorable perioperative outcomes compared with open Kasai portoenterostomy. The difference of surgical approach was not a significant independent predictor.

Biliary atresia: 20-40-year follow-up with native liver in an Italian centre.

INTRODUCTION: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients. MATERIALS AND METHODS: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire. RESULTS: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient. CONCLUSIONS: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors. TYPE OF THE STUDY: retrospective case series. LEVEL OF EVIDENCE: IV.

Learning curve of laparoscopic Kasai portoenterostomy for biliary atresia: report of 100 cases.

BACKGROUND: Laparoscopic Kasai portoenterostomy (LKPE) is performed for biliary atresia (BA). As LKPE is a technically demanding operation, a learning curve should be defined to guide training. The aim of this study was to identify the learning curve of LKPE for BA. METHODS: Metrics of perioperative safety and efficiency for 100 cases of LKPE were evaluated. Outcomes were followed to 67.2 ± 12.1 months. Cumulative sum (CUSUM) analysis was used to identify inflexion point corresponding to the learning curve. Outcome measures included operative time (ORT), rate of clearance of jaundice (CJ) and survival with native liver (SNL). RESULTS: Between May 2009 and May 2013, 100 consecutive patients with BA underwent LKPE. The rate of conversion from LKPE to open Kasai portoenterostomy (OKPE), intraoperative transfusion and any perioperative complications was 11, 26 and 16%, respectively. There was no perioperative mortality. The CUSUM analysis revealed a learning curve of 50 for LKPE. Precipitous ORT reductions from an initial mean operative time of 316.3 min that was observed in the first 50 to 232.2 min of the late 50 cases (P < 0.01). Subsequently, cases 1 to 50 were considered 'early experience', whereas cases 51 and higher were considered as 'late experience' for statistical analysis. The rate of CJ and SNL was significantly higher after the early 50 cases (P < 0.05). In contrast, the rate of intraoperative transfusion, the median time of oral feeding initiated after operation, and the length of hospital stay was not different between the both groups (P > 0.05). CONCLUSIONS: In this experience, improved perioperative and postoperative parameters for LKPE were observed in the last 50 patients when compared with the first 50 patients. The dedicated training is likely to contribute to significantly shorter learning curves in future adopters.

Using 99mTc-DTPA galactosyl human serum albumin liver scintigraphy as a prognostic indicator in jaundice-free patients with biliary atresia.

BACKGROUND: We evaluated the clinical significance of follow-up data, including 99mTc-DTPA galactosyl human serum albumin (99mTc-GSA) liver scintigraphy data, as prognostic indicators for jaundice-free patients with biliary atresia (BA). METHODS: Of 87 patients who underwent Kasai portoenterostomy (KP) between 1991 and 2012, 45 jaundice-free patients aged 1-2 years underwent 99mTc-GSA scintigraphy and were classified into 2 groups: those who survived with a native liver (Group A, n = 34) and those who required liver transplantation (LTx) (Group B, n = 11). We compared 99mTc-GSA scintigraphy data (HH15, LHL15, and HH15/LHL15 [H/L15]) and liver function test (LFT) results between the groups. The patients underwent a second 99mTc-GSA scintigraphy at approximately 5 years of age. RESULTS: All patients survived. HH15, H/L15, total bilirubin, direct bilirubin, gamma-glutamyl transpeptidase, and alanine transaminase levels were higher in Group B than in Group A (p<0.05). Total and direct bilirubin levels were associated with H/L15 (p<0.05). There were no significant changes in results between the first and second 99mTc-GSA scintigraphy in Group A. CONCLUSIONS: Mid- and long-term prognoses may be predicted using 99mTc-GSA scintigraphy data and LFTs in patients aged 1-2 years. We recommend regular monitoring of postoperative data following KP, even in jaundice-free patients. LEVEL OF EVIDENCE: III.

Liver transplant score for prediction of biliary atresia patients' survival following Kasai procedure.

OBJECTIVES: Recently, a scoring system has been developed to predict which patients with biliary atresia (BA) who underwent a Kasai procedure should be considered for liver transplant. Here, we applied the scoring system to predict the survival of BA patients following the Kasai procedure at Dr. Sardjito Hospital, Yogyakarta, Indonesia from January 2012 to January 2016. RESULTS: There were 26 patients, of whom 14 were males and 12 females. Outcomes of BA patients after the Kasai surgery were 15 survived and 11 died. There were significant associations between ascites and sepsis with the liver transplant score of ≥ 8 (p value = 0.006 and 0.014, respectively), whereas post-operative bilirubin level, ALT level, prothrombin time, cirrhosis, esophageal varices, portal hypertension, and cholangitis did not significantly correlate to the score. The patients with a score ≥ 8 have a relatively greater risk by 3.5-fold to die compared with patients with a score < 8, but it did not reach a significant level (p value = 0.13). In conclusions, the incidence of ascites and sepsis might predict the poor prognosis of BA patients following the Kasai procedure. Moreover, patients with a score ≥ 8 are prone to die after the Kasai surgery if they do not undergo a liver transplant.

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

BACKGROUND: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: 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). RESULTS: 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. CONCLUSION: Jaundice clearance at 3 months post surgery is a good early indicator of long term success.

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

BACKGROUND: The 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. METHODS: Gastroenterologists and surgeons in all 14 Canadian pediatric tertiary centers were invited to complete an online survey of their BA management practices. RESULTS: Of 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. CONCLUSION: In 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 EVIDENCE: Survey study. Level IV evidence.

Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia.

INTRODUCTION: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 µmol/l, and/or more than 20% of total bilirubin. This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations. METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period. RESULTS: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in µmol/l and the percentage value were significantly above the DHMA threshold limit: mean 129.7 µmol/l (42-334 µmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 µmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION: not relevant.

Design and validation of an early scoring system for predicting early outcomes of type III biliary atresia after Kasai's operation.

BACKGROUND/PURPOSE: The purpose of this study was to determine reliable predictors of outcome of biliary atresia (BA) after Kasai's operation. PATIENTS AND METHODS: This study included two consecutive stages of infants with biliary atresia. First stage, 200 BA cases who underwent Kasai's operation at our institution between May 2006 and May 2010 were retrospectively reviewed. In the first stage, we wanted to calculate a clinical scoring system by logistic regression. For cut-off determination, receiver operating characteristic (ROC) analysis was employed. Second stage (validation of scoring system), we used our scoring system to prospectively predict the outcomes of patients underwent a Kasai operation from inspecting the accuracy of our system. RESULTS: Early cholangitis, age at operation, JC time, post-operative TB, DB, AST, ALT and surgical method entered into our scoring system. The most reliable cut-offs determined by ROC analysis were 7.71 (sensitivity: 86.0%, specificity: 98.0%). We used our scoring system to predict the prognosis of the 15 BA patients and found that 13 of 15 patients were correctly predicted at the cut-off value of 7.71. CONCLUSION: Our scoring system is considered to be a reliable and useful predictor of the prognosis of biliary atresia.

Pulse oximetry is insufficient for timely diagnosis of hepatopulmonary syndrome in children with liver cirrhosis.

OBJECTIVE: To prospectively investigate the prevalence of hepatopulmonary syndrome (HPS), the importance of pulse oximetry in diagnosing HPS, and the longitudinal course after liver transplantation in children with cirrhosis referred for liver transplantation. STUDY DESIGN: Fifty-six patients aged 1-17 years (mean age, 4.6 ± 5.0 years) with liver cirrhosis were screened for HPS by hyperemic capillary blood gas (CBG) analysis and contrast-enhanced transthoracic echocardiography. Eleven patients were excluded owing to conditions that can produce cardiopulmonary dysfunction, including 5 with cystic fibrosis, 1 with pulmonary arterial hypertension, and 5 with an intracardial shunt. HPS was classified in accordance with the European Respiratory Society Task Force criteria on pulmonary-hepatic disorders. Patient groups were compared for biochemical and clinical characteristics. RESULTS: Eighteen children (40%) with cirrhosis were intrapulmonary vasodilatation (IPVD)-positive and had a pulse oximetry oxygen saturation level >98%. Two of these patients (11%) exhibited moderate HPS with an elevated alveolar arterial oxygen gradient >15 mm Hg and PaO2 <70 mm Hg; they died before undergoing liver transplantation. The sensitivity and specificity of CBG analysis for detecting elevated alveolar arterial oxygen gradient in children with IPVD was 94% and 53%, respectively. HPS was associated with late hepatoportoenterostomy (P < .04). Liver transplantation led to resolution of HPS in all patients. CONCLUSION: IPVD is frequent in children with liver cirrhosis (40%). Pulse oximetry is insufficient for timely HPS diagnosis. Pathological CBG analysis data indicate IPVD in the majority of cases, but are imprecise in children aged <2 years. Contrast-enhanced transthoracic echocardiography and CBG analysis are recommended for evaluation of HPS in children with cirrhosis, regardless of liver synthesis capacity and clinical chemistry data.

Long-term results of biliary atresia in the era of liver transplantation.

PURPOSE: The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival. METHODS: Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed. RESULTS: Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002). CONCLUSIONS: Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.

Trends in age for hepatoportoenterostomy in the United States.

BACKGROUND: Biliary atresia is a rare but devastating disease for which hepatoportoenterostomy remains the primary intervention. Increased age at the time of hepatoportoenterostomy is associated with unfavorable outcomes. In this study, we examined trends in age at the time of hepatoportoenterostomy and explored hospital and patient factors associated with more timely diagnosis and treatment. METHODS: Median ages of patients undergoing hepatoportoenterostomy for biliary atresia were compared using the Kids' Inpatients Database from 1997, 2000, 2003, and 2006. The patient and hospital factors associated with later treatment were compared. RESULTS: Of 192 patients, 13.5% had surgery in 1997, 13.5% in 2000, 36.5% in 2003, and 36.5% in 2006. The overall median age was 65.5 days; the median age was 64 days in 1997, 57.5 days in 2000, 69 days in 2003, and 64 days in 2006 (P = .80). Overall, 71% of patients were treated at nonchildren's hospitals, and although the proportion has increased over time, the trend did not reach significance (P = .12). Hispanic and African American patients were more likely to undergo hepatoportoenterostomy after 60 days of life compared with white patients (Hispanic patients: odds ratio, 3.6; 95% confidence interval, 1.1-12.5; P = .04; African American patients: odds ratio, 2.2; 95% confidence interval, 0.8-6.3; P = .14). Compared with specialized children's centers, treatment at nonchildren's hospitals was associated with delayed hepatoportoenterostomy (odds ratio, 3.5; 95% confidence interval, 1.2-9.8; P = .02). CONCLUSION: Although early hepatoportoenterostomy is associated with improved outcomes for children with biliary atresia, our study shows the median age at surgery has not significantly changed over 2 decades. Both hospital and socioeconomic factors play a role in the early treatment of biliary atresia.

Early outcomes of laparoscopic surgery for biliary atresia.

AIM: The aim of the study was to present early outcomes of the laparoscopic technique for biliary atresia with some technical modifications. MATERIALS AND METHODS: We reviewed charts of all patients with biliary atresia who underwent laparoscopic portoenterostomy from July to December 2008. There were 11 patients with biliary atresia, including 5 boys and 6 girls. The operation was carried out using 4 trocars. The liver was elevated by 2 transcutaneous stay sutures: one on the round ligament and the other on the gallbladder remnant. The left and right hepatic arteries and portal veins were dissected and retracted laterally by 2 transcutaneous sutures to expose the liver hilum. A stay suture was placed on fibrotic tissue at the liver hilum to facilitate its maximal removal. A jejunal end-to-side anastomosis was constructed extracorporeally. Portoenterostomy was carried out laparoscopically. RESULTS: Mean operative time was 245 +/- 31 minutes. No patient required conversion. There were no operative deaths. Blood loss during operation was minimal. One patient died on day 65 after operation because of intractable hepatic liver. Follow-up after discharge from 10 to 16 months revealed that 6 patients still survived and 4 patients died. One patient died because of milk aspiration at 12 months of age. Three patients died because of repeated cholangitis and liver failure at 10, 10, and 14 months, respectively. CONCLUSION: With a modified laparoscopic technique, good early outcomes of laparoscopic surgery for biliary atresia were achieved.

Biliary atresia: the Croatian experience 1992-2006.

The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan-Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400-1/27,200). The median age at Kasai operation was 66 days (range 22-192). Median follow-up was 2.65 years (range 0.2-14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.