Cystic biliary atresia: an etiologic and prognostic subgroup

Introduction

Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA. Because some cases can be detected antenatally, CBA in general may have its origins in utero life. We assessed our experience with CBA.

Methods

Single-center retrospective review of infants with CBA over a 13-year period (January 1994 to December 2006) was done. Data are given as medians (range).

Results

Of 270 infants with BA, 29 (9 male) were identified as CBA. Antenatal ultrasonography had detected an abnormality in 12 (41%) infants at a median of 22 weeks (17-34 weeks) of gestation. All infants underwent postnatal excision and Kasai portoenterostomy (KP). Those with antenatally detected CBA came to surgery younger (36 [14-67] vs 48 days [35-147 days], P = .004). Twenty cysts (69%) had a fibroinflammatory wall with no biliary epithelial lining and 6 (26%) contained bile. Age at KP was significantly and positively correlated (r = 0.46, P = .01) with liver fibrosis, as assessed in liver biopsy materials obtained at KP, but not with grade of “hepatocyte disarray” (P = .74). Twenty infants (69%) cleared their jaundice (bilirubin <20 μmol/L) within 6 months after KP. Age at KP markedly affected outcome.

Conclusion

Cystic BA is a clinically distinct variant of BA. Despite onset in prenatal life, earlier than presumed for isolated BA, it has a better prognosis, particularly with early surgery.     

Biliary atresia in England and Wales: results of centralization and new benchmark

Introduction

Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice. Typically, it is treated by an initial attempt to restore bile flow (the Kasai portoenterostomy [KP]) as soon as possible after diagnosis and, if this fails, liver transplantation. Since 1999, the treatment of BA has been centralized to 3 centers in England and Wales able to offer both treatment options. The aim of this study was to review the outcome of this policy change and provide a national benchmark.

Methods

The management of all infants born within England and Wales during the period January 1999 to December 2009 was assessed using 3 key performance indicators such as median time to KP, percentage clearance of jaundice (≤20 mol/L) post-KP, and 5- and 10-year native liver and true survival estimates. Data are quoted as median (range), and P < .05 was considered significant.

Results

A total of 443 infants had confirmed BA; and of these, most were isolated BA (n = 359), with 84 having other significant anomalies (but predominantly BA splenic malformation syndrome). Four infants died before any biliary intervention. Kasai portoenterostomy was performed in 424 infants (median age, 54 [range 7-209] days), and a primary liver transplant was performed in 15. Clearance of jaundice post-KP was achieved in 232 (55%). There were 41 deaths, including 4 (10%) without any intervention, 24 (58%) post-KP usually because of end-stage liver disease and mostly on a transplant waiting list, and 13 (32%) post-LT usually because of multiorgan failure. Overall, the 5- and 10-year native liver survival estimates were 46% (95% confidence interval [CI], 41-51) and 40% (95% CI, 34-46), respectively. The 5- and 10-year true patient survival estimates were 90% (95% CI, 88-93) and 89% (95% CI, 86-93), respectively. Outcome was worse for those with other anomalies (lower clearance of jaundice post-KP [43% vs 57%; odds ratio, 1.7; 95% CI, 1.04-2.8]; P = .02) and an increased mortality overall (eg, at 5 years, 72 [95% CI, 64-83] vs 94 [95% CI, 91-96]; χ² = 33; P < .0001).

Conclusions

National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.     

Five-year native liver survival analysis in biliary atresia from a single large Chinese center: The death/liver transplantation hazard change and the importance of rapid early clearance of jaundice

PurposeThis study aimed to analyze the change of death/liver transplantation hazard and biochemical indexes over time after Kasai procedure (KP) based on a retrospective biliary atresia (BA) cohort, and to evaluate the predictive value of early jaundice clearance rate to 5-year native liver survival (NLS).MethodsA retrospective cohort with follow-up results of 139 BA patients from January 2009 to December 2012 was established, and the pre- and postoperative data were collected. NLS rates were estimated with Kaplan–Meier curves, and any differences between groups were tested by log-rank test. Hazard curve of death/liver transplantation was fitted with Weibull distribution, and hazards at certain time points were calculated. Trend charts of biochemical indexes were drawn to show any changes over time. Rate of jaundice clearance was indicated as the proportion of decreased total bilirubin level at a certain postoperative time point to preoperative total bilirubin level. In multivariate analysis for prediction of 5-year NLS, COX proportional hazard regression model was used and results were expressed as hazard ratios with 95% confidence intervals (CIs). The predictive value of early jaundice clearance rates for 5-year NLS was analyzed by receiver operating characteristic (ROC) curve, and a cut-off value of 4-week jaundice clearance rate was determined.ResultsThe estimated 5-year NLS rate of the 139 patients was 58.0%. The patients had a high hazard of death/liver transplantation early after KP, which gradually decreased and stabilized at a lower level 1 year later. Most death/liver transplantation events occurred within 1 year after KP. The total bilirubin (TBIL), direct bilirubin (DBIL) and total bile acid (TBA) levels of successful Kasai group decreased continuously after KP, and the biggest decline was seen in the first month. The aspartate aminotransferase (AST), alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) levels increased during the first month after KP and decreased continuously thereafter. All the biochemical indexes of successful Kasai group tended to stabilize within/close to normal range 1 year after KP. On the contrary, all the biochemical indexes of failed Kasai group fluctuated at obvious abnormal levels after KP. The estimated 5-year NLS rates of successful Kasai group and failed Kasai group were 90.1% and 10.7% (p = 0.000). The most significant clinical protective factor of 5-year NLS was 4-week jaundice clearance rate, revealed by COX proportional hazard regression model, and the HR was 0.089 (95%CI 0.018–0.432, p = 0.003). In predicting 5-year native liver survival, the largest area under ROC (AUROC) curve belonged to 4-week jaundice clearance rate, which was 0.731 (p = 0.000). A cut-off value of 0.457 was determined, with sensitivity 0.827, specificity 0.552, positive predictive value 0.720, and negative predictive value 0.696. NLS rates of patients divided by cut-off value showed significant statistical difference demonstrated by Kaplan–Meier curve and log-rank test (p = 0.000).ConclusionsThe 5-year death/liver transplantation hazard of biliary atresia patients reduces greatly and stabilizes 1 year after KP. A successful KP enables patients to achieve long-term stable normal biochemical indexes. A rapid clearance of jaundice is of great positive significance to 5-year NLS, and the 4-week jaundice clearance rate is of some predictive value.Type of studyPrognosis study.Level of evidenceLevel II, retrospective study.     

Intrahepatic mast cell population correlates with clinical outcome in biliary atresia

PURPOSE: To determine if mast cells influence the clinical outcome in biliary atresia (BA), the authors examined the intrahepatic mast cell population in BA. METHODS: Mast cells were identified histochemically using Toludin Blue and immunohistochemically using antimast cell tryptase antibody in formalin-fixed paraffin-embedded sections from 21 cases of BA. Patients were divided into 3 groups; group I (n = 8) with good liver function, group II (n = 8) with moderate liver dysfunction, and group III (n = 5) with severe liver dysfunction. Liver biopsies from patients with choledochal cysts (CDC, n = 5), and normal liver (NL, n = 4) served as controls. The results were compared among the groups. RESULTS: Both histochemical and immunohistochemical methods showed similar data. Mast cells were seen mostly in the portal tracts. Mast cell numbers per medium power field (20 x magnification) were higher in BA than in the controls (15. 03 +/- 2.25 v 3.85 +/-.65, [mean +/- SEM], P <.05, BA v CDC; 15.03 +/- 2.25 v 1.73 +/-.06, [mean +/- SEM], P <.05, BA v NL, immunohistochemical data). Clinical correlation showed an association between higher mast cell number and liver dysfunction (32.62 +/-.80 v 8.52 +/-.87 [mean +/- SEM], group III v group I; P <. 05, immunohistochemical data). CONCLUSION: Increased mast cell population in BA adversely affects liver function and raises the possibility that type I allergic reaction may play role in the pathology of BA.     

Serum IFN-inducible protein-10: a new clinical prognostic predictor of hepatocyte death in biliary atresia

PURPOSE: The aim of this study was to determine whether chemokines such as serum IP-10 levels in patients with biliary atresia (BA) correlate with liver function and histology and assess its value as a medium to long-term prediction of prognosis in postoperative BA patients. METHODS: Thirty postoperative BA patients (mean age, 10.8+/-3.5 years) and eight normal controls (mean age, 10.3+/-3.3 years) were studied. The BA patients were divided into three groups according to liver function. Group I (n = 8) was jaundice free, had normal liver function and no evidence of severe cholangitis or portal hypertension. Group II (n = 12) had moderate liver dysfunction. Group III (n = 10), had severe liver dysfunction. Hepatic histology was assessed using conventional needle biopsy. Serum IP-10 levels were determined using a specific enzyme-linked immunosorbent assay (ELISA). RESULTS: Serum levels of IP-10 in group III (458.0+/-240.0 pg/mL) were significantly higher than those in group II (233.6+/-126.9 pg/mL; P < .0001). Levels in group II were also significantly higher than those in group I (144.8+/-23.4 pg/mL; P < .05), but there was no significant difference between group I and controls (107.9+/-34.0 pg/mL). Liver biopsy findings showed a progression of fibrosis and mononuclear cell infiltration from group I to group III. There was intimal hyperplasia and swelling of endothelial cells of branches of the hepatic artery in the portal area in group III. CONCLUSION: Because IP-10 levels correlate closely with histological findings in postoperative BA patients, it would appear to play a specific role in hepatocyte death and hepatic artery changes, thus providing important information about progressive fibrosis in BA patients that facilitates treatment decision making and prediction of prognosis.     

Initial response to portoenterostomy determines long-term outcome in patients with biliary atresia

PURPOSE: The aim of this study was to assess the efficacy of portoenterostomy in biliary atresia and determine factors that predict outcomes. METHODS: The outcomes after portoenterostomy for biliary atresia from 1976 to 1996 were graded into 3 defined groups: G (good, jaundice free); I (intermediate, progressive liver disease with liver transplant if needed after 3 years of age); P (poor, liver transplant or death at less than 3 years of age). RESULTS: Twenty-seven of 49 children (55%) who underwent portoenterostomy responded. Of these, 13 (26%) were jaundice free (G). Of 14 children (28%) in I, 6 underwent liver transplant after 3 years of age. Of 22 children (44.8%) in P, 14 (28%) died before 3 years and 8 underwent liver transplant before 3 years. Thus, 42% of the children in this series are surviving with native liver. Age at operation in P was significantly different compared with G. Preoperative values of bilirubin and alanine transaminase were comparable in the 3 groups. Postoperative values of bilirubin and alanine transaminase were lower in G and I compared with P Complications were significantly lower in group G. CONCLUSIONS: Portoenterostomy alone in biliary atresia is beneficial in 40% of cases. Factors associated with the outcome include age at operation, postoperative cholangitis, and change in values of bilirubin and alanine transaminase. A classification based on decline of bilirubin and enzyme levels 3 months after portoenterostomy is proposed to predict the long-term outcome of an individual case of biliary atresia. It is particularly relevant in the intermediate group in which timing of referral may preclude or compromise liver transplant.     

Adjuvant therapy of cytomegalovirus IgM + ve associated biliary atresia: Prima facie evidence of effect

Aim of studyCMV-IgM + ve associated biliary atresia (CMV-BA) is a distinct etiological subgroup characterized by older age at presentation and a greater degree of inflammation and hepatic fibrosis, leading to a worse outcome. We report our experience with adjuvant antiviral therapy after Kasai portoenterostomy (KPE).MethodsSingle-center prospective database identification of CMV-IgM + ve associated BA managed between 2003 and 2017. Since 2011, IV ganciclovir (5 mg/kg b.d.) and/or oral valganciclovir (520 mg/m² b.d.) were started in the early postoperative period in selected cases and continued until negativity of CMV DNA load [Anti-Viral Therapy (AVT) Group 1]. Clearance of jaundice was defined as achieving a total bilirubin ≤ 20 μmol/L in post-KPE period and tested with a Fisher test; native liver survival (NLS) and overall actuarial survival (OS) were compared with untreated BA CMV IgM + ve patients (Group 2) using a Log-Rank test. A P value of < 0.05 was regarded as significant. Data are quoted as median (IQ range).ResultsDuring the 14-year period, 376 infants with histologically confirmed BA were treated; of those 38(10%) were CMV IgM + ve at presentation. One child was considered too late at presentation for KPE and underwent primary liver transplantation while another only started AVT one month after KPE. Both were excluded from survival analysis. Therefore 36 underwent KPE [AVT Group 1 (n = 8) and Control Group 2 (n = 28)]. Overall age at surgery was 67(53–77) days. There was no difference in age at surgery (P = 0.26); bilirubin (P = 0.12); or AST (P = 0.15) between Group 1 and Group 2. Viral load data were available in 16 with a trend towards higher counts in the AVT group 1 [4935 (2668–18,817) vs. 1296 (253–10,471) c/ml; P = 0.06]. Clearance of jaundice was higher in AVT Group 1 (75% vs 21%, P = 0.009). There was no difference in OS (P = 0.24) but NLS was improved in the AVT Group 1 (75% vs. 25% at 2 years; P = 0.04).ConclusionsAlthough this finding may be regarded as preliminary, adjuvant antiviral therapy appeared to improve outcome in infants with CMV IgM + ve BA.Level of evidenceIII.     

Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution

Purpose

We reevaluated the impact of age at Kasai operation on the short- and long-term outcomes of type III biliary atresia (BA).

Patients and Methods

From 1953 to 2009, 242 patients with type III BA underwent Kasai operation at ages ranging between 12 and 421 days (average, 79.7 days). The relationship between age at Kasai operation and jaundice disappearance rates (JDRs), and 10-, 20-, and 30-year native liver survival rates (NLSRs) were assessed retrospectively (JDR [%] = the number of patients in whom jaundice disappeared/the number of patients in each group × 100).

Results

Age at Kasai operation had a significant impact on the JDRs (P < .001). However, there was no statistical relationship between long-term NLSR of the patients in whom jaundice disappeared after Kasai operation and operative age. From the results of the cumulative NLSRs estimated by Kaplan-Meier method, each survival rate was quite dependent on the age at operation until 30 years after Kasai operation, but the difference became much smaller in the later period provided age at operation was 4 months or younger.

Conclusion

The operative age as a prognostic factor might be less significant in the long-term outcome than in the short-term outcome.     

Hyaluronic acid: a specific prognostic indicator of hepatic damage in biliary atresia

BACKGROUND/PURPOSE: Hepatic fibrosis can progress in biliary atresia (BA) and is associated with capillarization of hepatic sinusoids. The significance of serum hyaluronic acid (HA) as a noninvasive indicator of histological sinusoidal endothelial cell (SEC) damage and hepatic fibrosis in BA, is investigated. METHODS: A total of 28 postoperative BA patients (mean age, 11.0+/-3.7 years) and 20 normal controls (mean age, 10.5+/-2.8 years) were studied. BA patients were divided into group I, good liver function (n = 8); group II, moderate liver dysfunction (n = 10); and group III, severe liver dysfunction (n = 10). Serum HA was determined using a one-step sandwich enzyme immunoassay, and liver histological damage was confirmed immunohistochemically using an antibody against factor VIII-related antigen (FVIIIRAg), which is specific for detecting damaged SEC. RESULTS: Serum HA was significantly higher (P < .0001) in group III (84.6+/-36.5 ng/mL) than in group I (15.9+/-6.9 ng/mL) or group 11 (28.7+/-10.7 ng/mL). Although immunoreactive products of FVIIIRAg were abundant in group III, they were not detected in SEC from group II. CONCLUSION: Serum HA may be of value for monitoring postoperative BA patients as a noninvasive indicator of SEC damage and progressive hepatic fibrosis.     

Cadaveric liver transplantation in biliary atresia splenic malformation syndrome with the absence of retrohepatic inferior vena cava, preduodenal portal vein, and intestinal malrotation: a case report

A 9-month-old female infant with biliary atresia underwent cadaveric liver transplantation due to progressive cholestatic hepatitis following a Kasai operation. She had biliary atresia splenic malformation syndrome (BASM) composed of an absent retrohepatic inferior vena cava with an azygous connection, preduodenal portal vein, polysplenia, and intestinal malrotation. A portal vein thrombosis developed on the 4th postoperative day requiring immediate treatment by thrombectomy. The patient is well with normal liver function at 3 months follow-up. Although BASM may render the transplantation more difficult, the presence of BASM is no longer a contraindication to liver transplantation.     

The spectrum of surgical jaundice in infancy

Background

Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life.

Methods

A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors’ institution from January 1992 to December 1999.

Results

There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P < .01) and aspartate aminotransferase levels (P < .001) and came to surgery earlier (P < .01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention.

Conclusions

Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is currently expected.     

超声检查胆囊及其收缩功能鉴别胆汁淤积性肝炎和胆道闭锁

目的通过动态观察胆囊及其收缩功能改变,探讨超声鉴别诊断胆汁淤积性肝炎与胆道闭锁的临床价值。方法将44例住院黄疸患儿分成胆汁淤积性肝炎组（n=25）和胆道闭锁组（n=19）,均行腹部三维彩超检查。由同一观察者利用5 MHz凸阵探头和9 MHz线阵探头观察空腹、餐后1 h及餐后2 h胆囊大小和收缩率,比较和分析胆囊的不同表现。结果胆汁淤积性肝炎组与胆道闭锁组的空腹胆囊大小差异有统计学意义（P〈0.05）,但胆汁淤积性肝炎组中18例（18/25,72.00%）空腹胆囊空虚状态者与胆道闭锁组17例（17/19,89.47%）患者的胆囊大小差异无统计学意义（P=0.091）。胆道闭锁组餐后1 h及2 h胆囊均无明显收缩,与胆汁淤积性肝炎组比较差异有统计学意义（P〈0.05）。结论超声检查空腹胆囊及其收缩功能变化,能够为临床医生提供诊断和鉴别诊断胆汁淤积性肝炎与胆道闭锁的客观依据,具有重要临床意义。     

Short-term results of Kasai operation for biliary atresia: experience from one institution

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.     

Serum hyaluronic acid as an early prognostic marker in biliary atresia

PURPOSE: The aim of this study was to assess whether serum concentrations of hyaluronic acid (HA), measured at diagnosis is an early biochemical marker of prognosis in biliary atresia. METHODS: Serum HA was measured at diagnosis using a radiometric assay in 84 infants with biliary atresia (BA), and related to outcome by 5 years of age. RESULTS: Serum HA was higher in the 29 patients who died or required liver transplant by 5 years of age compared with the 56 who survived to 5 years without transplant (490 +/- 216 microg/L v 262 +/- 163 microg/L; P <.001, 95% confidence intervals of the difference 145 to 311 microg/L). CONCLUSION: High serum concentrations of HA at diagnosis may help to identify at an early stage those patients with BA who have a poor prognosis and will require liver transplant by 5 years of age.     

Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

Background

Biliary atresia (BA) with hilar cyst is an uncommon variant, which constitutes less than 10% of all types of BA, and the operative procedure for this type of BA remains controversial.

Methods

We have had 200 cases with BA from 1963 to 2008 in our institute and our branch hospitals, in which 12 cases (6%) were BA with a hilar cyst. The clinical records of all cases were evaluated retrospectively.

Results

Twelve BA patients with a hilar cyst included 2 boys and 10 girls. The diagnosis of BA was confirmed by intraoperative cholangiography (cloudy or treelike pattern). In all 12 cases, a hepaticojejunostomy was performed at a median age of 71.6 days (range, 24-136 days). The follow-up periods were 1.2 to 23.2 years. The current mean total bilirubin level was 0.8 mg/dL (range, 0.2-3.5 mg/dL), and the mean direct bilirubin level was 0.2 mg/dL (range, 0.0 to 0.8 mg/dL). Methylprednisolone or oral prednisolone was administered in 8 cases after operation, and 10 of 12 cases achieved a jaundice free state. The postoperative complications were cholangitis (n = 10), gastroesophageal varices (n = 7), splenomegaly (n = 3), ileus (n = 1), and pulmonary hypertension (n = 1). The overall survival rate with a native liver was 10 (83.3%) of 12 cases, of which 9 cases were jaundice-free and only 1 case showed recurrent jaundice. The other 2 cases underwent living-donor liver transplantation at age of 2 and 20 years, respectively, and they are currently doing well.

Conclusions

Most cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy.     

Portal venous pressure in biliary atresia

Biliary atresia (BA) is characterized by a variable degree of fibrosis within the liver, causing portal hypertension sometimes evident at the time of presentation.AimThe aims of this study are to measure portal venous pressure (PVP) at time of Kasai portoenterostomy (KP) and to investigate the value of surrogate indices.MethodsAt the time of KP and before any liver manipulation, an attempt was made to recanalize the umbilical vein, allowing a catheter to be sited. Preoperative noninvasive variables included maximum splenic diameter (on ultrasound); platelet count, aspartate aminotransferase, and bilirubin; and the aspartate aminotransferase/platelet index ratio (APRi). Clearance of jaundice was defined as achieving a bilirubin of less than 20 μmol/L. Data are quoted as median (range). Nonparametric statistical tests were used, and P < .05 was regarded as significant.ResultsPortal venous pressure measurements were available in 61 infants, who underwent a KP during the period February 2007 to October 2010. Median age at KP was 52 (19-151) days. Median PVP was 9 (3-26) mm Hg and was significantly lower in those with isolated (n = 47) BA vs cytomegalovirus-associated BA (n = 6) (8 vs 17 mm Hg; P = .02). Overall, PVP correlated with spleen size (r_s = 0.38; P < .0001), APRi (r_s = 0.5; P < .0001), bilirubin (r_s = 0.38; P = .001), and age at KP (r_s = 0.33; P = .0004).Thirty-three infants (56%) cleared their jaundice (to <20 μmol/L). There was no significant difference in median PVP (7 vs 9.5 mm Hg; P = .1) between these groups. To date, 6 children have undergone endoscopy, with 2 requiring intervention for significant varices. Neither infant at time of KP had elevated PVP (3 and 9 mm Hg).ConclusionsPortal venous pressure is a key variable in the assessment of the BA liver, and differences can be shown depending on underlying cause (eg, cytomegalovirus status). Furthermore, it has modest correlation with noninvasive indices (eg, spleen size and APRi) but appears of limited use when used as prognostic index of outcome and indeed in the later development of varices.     

Biliary atresia splenic malformation syndrome--is it a result of embryonically midline rotational defects? A case report

The authors present a case of biliary atresia splenic malformation (BASM) syndrome in a 34-week gestational age boy who died shortly after birth. Autopsy results showed polysplenia, short pancreas, extrahepatic biliary atresia, heterotaxy of liver, tracheoesophageal and duodenal atresia, right lung hypoplasia, cardiac defects with tetralogy of Fallot, axial skeletal defects, and minor genitourinary disorders. Additional histopathologic findings were congenital hepatic fibrosis and bronchopulmonary dysplasia. The authors consider that the abnormalities of this case are possibly a unique combination. Midline defects, which are the most notable features of the presented case, may be closely related to pathogenesis of BASM syndrome.     

Long-term postsurgical outcome of biliary atresia

BACKGROUND/PURPOSE: A successful Kasai procedure is effective in creating biliary drainage and radically altering the natural history of infants with biliary atresia (BA). Since its introduction in the 1950s, long-term follow-up would appear to show that only 30% to 50% of patients have a good long-term prognosis despite initially good surgical outcome. The authors reviewed their experience in treating BA from 1968 to 1997 to assess long-term outcome. MATERIALS AND METHODS: The records of 163 patients treated surgically for BA from 1968 to 1997 were reviewed. Forty-eight (29%) were alive at the end of 1997, of whom, 14 had received liver transplants (LT). Surviving patients who had not undergone transplantation were divided into two groups according to clinical condition: group A, normal liver function without cholangitis (CG) and portal hypertension (PH) and group B, liver dysfunction with CG or PH. The study period was divided arbitrarily into three periods, 1968 to 1975 (period I, n = 34); 1976 to 1985 (period II, n = 81); 1986 to 1997 (period III, n = 48). RESULTS: Thirty-four patients were alive without LT at the end of 1997. There were eight patients (mean age, 16.3+/-4.8 years) in group A, and 26 patients (mean age, 14.3+/-7.6 years) in group B. Recently, four group A patients (mean age, 19.3+/-1.9 years) shifted to group B because of sudden deterioration in condition involving severe CG with multiple bile lakes (n = 2), uncontrollable intestinal bleeding (n = 1), and liver atrophy (n = 1). Survival deteriorated with length of follow-up. There were three survivors from 34 patients treated in period I, 16 survivors from 81 patients treated in period II (three had LT), and 29 survivors from 48 patients treated in period III (11 had LT). CONCLUSIONS: Although satisfactory bile drainage can be obtained with portoenterostomy, our data suggest that liver function can deteriorate progressively, with a possible turning point in late adolescence, indicating that as the length of follow-up increases, clinical assessment should be regular and comprehensive. The timing of LT in postoperative BA patients with deteriorating liver function is a vital management issue.     

The outcome of the older (> or =100 days) infant with biliary atresia

Background

There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis.

Methods

All infants who had undergone surgery for BA during the period 1980 through 2000 aged ≥100 days were reviewed. Actuarial survival was calculated using 2 end-points (death and transplantation). A retrospective review of their ultrasonography (n = 12) and preoperative liver histology (n = 22) was also undertaken to ascertain possible predictive criteria.

Results

A total of 422 infants had BA diagnosed during this period, of which 35 (8.2%) were ≥100 days at surgery (median [interquartile range], 133 [range, 108 to 180] days). Surgery included portoenterostomy (n = 26), hepaticojejunostomy (n = 7), and a resection and end-to-end anastomosis (n = 1). A laparotomy only was performed in 1. Five- and 10-year actuarial survival rate with native liver was 45% and 40%, respectively. Currently, 12 (35%) patients are alive with their native liver (8 are anicteric), 9 (28%) have undergone transplantation, and 13 have died. Although there were some survival advantages for types 1 or 2 BA and “noncirrhosis” at time of surgery, neither reached statistical significance. Individual histologic features (eg, degrees of fibrosis, giant cell transformation, bile duct destruction) in the retrospective review of available material were not discriminatory. The finding of a “heterogeneous” parenchyma on ultrasonography was predictive of poor outcome but lacked sensitivity.

Conclusions

The potential for reasonable medium-term survival is present in about one third of infants 100 days or older coming to primary corrective surgery. In the absence of accurate discrimination, the authors continue to favor this option rather than subject all to transplant simply on the basis of age.