Successful liver resection for biliary atresia with intrahepatic biliary cysts after Kasai procedure

A 29-year-old woman with congenital biliary atresia underwent liver resection for intrahepatic biliary cyst and recurrent cholangitis long after Kasai procedure. The patient is alive with normal liver function and no episode of cholangitis after 12 months of follow-up.     

Intrahepatic cystic lesions in children with biliary atresia after Kasai procedure

BackgroundIntrahepatic cystic lesion (ICL) is a common complication for biliary atresia post-Kasai portoenterostomy. The purpose of this study was to review the cases in our hospital and assess the correlation between characteristics of ICL and clinical outcomes.MethodsWe retrospectively analyzed 787 cases of biliary atresia from 2012 to 2016. Demographics, clinical details, and postoperative outcomes were reviewed.ResultsA total of 76 patients were diagnosed with ICLs using ultrasound post-Kasai procedure, and the incidence was 9.7%. Preoperative characteristics showed no significant differences between ICL (+) and ICL (?) groups. Nearly 70% (53/76) of the patients with ICLs had a history of cholangitis. The 2-year native liver survival rate was 60.4% for those with a history of cholangitis and 87% for those without (P = 0.017). Further analysis showed that the 2-year native liver survival rate was 42.9% for those diagnosed within 3 months post-Kasai procedure, 54.2% for those diagnosed between 3 and 6 months, and 80.0% for those diagnosed beyond 6 months (P = 0.002), while no significance was observed for type (P = 0.094) or site (P = 0.406) of ICL.ConclusionPatients with ICLs had a high incidence of cholangitis. The prognosis was closely related with the history of cholangitis and the onset time of ICLs.Level of evidenceLevel II.     

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

Background/Purpose

Late-onset cholangitis is an uncommon complication after the Kasai operation for biliary atresia. Experience with 3 recent patients illustrates appropriate management.

Methods

Retrospective review of patients with late unexplained deterioration in liver function attributed to cholangitis. All underwent quantitative radioisotope hepatobiliary scans to identify the Roux loop obstruction and confirm postoperative resolution. The role of percutaneous transhepatic cholangiography and computed tomography scanning was reviewed.

Results

Three patients aged 8, 13, and 17 years were identified. All 3 had had a successful Kasai portoenterostomy for type 3 biliary atresia and presented after several virtually trouble-free years with late-onset cholangitis. The radioisotope scans in all 3 patients showed poor tracer uptake by the liver and pooling of the radioisotope at the porta hepatis.Laparotomy confirmed a proximal Roux loop obstruction at the level of the mesocolic window in all. This required mobilization and adhesiolysis and, in 1, correction of an actual stenosis within the wall of the intestine at this level. All patients had normalization of their liver function postoperatively. All are now well and anicteric at a follow-up of 6 to 12 months.

Conclusions

Long-term survivors of the Kasai portoenterostomy for biliary atresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction.Resolution of the obstruction allows preservation of their native liver.     

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.     

Risk factors and prognostic effects of cholangitis after Kasai procedure in biliary atresia patients: A retrospective clinical study

PurposeCholangitis after Kasai procedure has been previously shown to be related to poor prognosis in Biliary Atresia (BA). To investigate the risk factors and clinical outcomes of cholangitis, we did a retrospective study in post-Kasai BA patients.MethodsTwo-year follow-up data of 180 type-III BA patients after Kasai procedure in 2016 in our hospital were analyzed, including 119 cholangitis patients (66.11%). Among the cholangitis group, patients were further divided into early vs late cholangitis and single vs recurrent cholangitis groups. Liver pathology, liver function, cholangitis occurrence and frequency, jaundice clearance, and survival rates were examined.ResultsHigher gamma-glutamyl transferase level before Kasai is a risk factor for cholangitis (p = 0.0393). Older age and higher liver fibrosis score at Kasai are risk factors for recurrent cholangitis (p < 0.05). Shorter prophylactic intravenous antibiotics usage may contribute to early cholangitis, which may lead to higher cholangitis frequency (p < 0.0001). Recurrent cholangitis is associated with earlier cholangitis onsets (p < 0.0001). Cholangitis patients have a relatively delayed jaundice clearance, while early and recurrent cholangitis may contribute to lower overall survival.ConclusionsPersonalized treatment considering risk factors in individual BA patients is needed to prevent cholangitis, especially early onsets, and to improve postoperative outcomes.Level of evidenceIII     

术中门静脉测压对胆道闭锁患儿Kasai术后的预后评估

目的采用术中门静脉压力测定对胆道闭锁患儿Kasai术后的预后评估。方法1990年1月至1996年12月,法国巴黎Bic毢tre医院儿外科共对127例胆道闭锁患儿行Kasai手术或其变异术式,其中113例术中脐静脉插管行门静脉压力测定,术后平均随访9年。分别计算患儿带自体肝生存率和带自体肝且无门静脉高压生存率,进行比较;依据患儿手术时的年龄,胆道闭锁的类型,手术方式,有无polysplenia综合征以及术时门静脉压力对生存率进行评估。结果术中门脉压力正常患儿带自体肝5年和10年生存率较门静脉压力升高组显著延长。门静脉压力升高组5年带自体肝且无门静脉高压表现的生存率显著低于门静脉压力正常组。胆道闭锁的类型和门静脉压力改变较其他指标更显著反映对Kasai术后预后影响。结论术前门静脉压力改变对Kasai术后的预后有显著性指导意义,并且优于其他指标。     

Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst

Background

Laparoscopic approaches are possible for many pediatric surgical diseases, including complex biliary disorders. The authors describe their early experience with laparoscopic procedures for biliary atresia and choledochal cysts.

Methods

A retrospective review was performed with Institutional Review Board (IRB) approval of children undergoing laparoscopic Kasai portoenterostomy (n = 2) or laparoscopic excision of a choledochal cyst with Roux-en-y biliary reconstruction (n = 3). The authors report the operative technique for these procedures and present their initial results.

Results

All procedures were performed with 4 to 5 trocars using 3- and 5-mm ports. Excision of the fibrous biliary remnant (biliary atresia) or of the cyst (choledochal cysts) was performed laparoscopically in all cases. The Roux-en-y limb was created through a 1-cm extension of the umbilical port site. Laparoscopic biliary reconstruction was performed successfully in 4 of 5 patients. In one child, the proximal extent of the choledochal cyst was at the bifurcation of the hepatic duct, and the biliary reconstruction was performed via an open incision with separate anastomoses of the right and left ducts. The gallbladder was used as a handle for retraction of the liver for portal visualization, as in a cholecystectomy, and was resected at the end of the procedure. All children with choledochal cysts have normal bilirubin values. One of 2 children who underwent laparoscopic Kasai has a normal postoperative bilirubin level, whereas the other child did not drain bile and underwent a successful liver transplantation. Mean length of surgery was 338 minutes; mean length of stay was 5.5 days. There were no significant postoperative complications. Cosmesis was excellent in all cases.

Conclusions

Laparoscopic approaches for children with biliary atresia and choledochal cysts are possible. Excellent visualization of the portal structures can be achieved laparoscopically with adequate retraction of the liver. Further follow-up is needed to determine broader application.     

Idiopathic thrombocytopenic purpura complicated with biliary atresia: a rare occurrence and literature review

The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.     

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.     

Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy

Purpose

This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA.

Methods

Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained.

Results

Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (74%) was comparable to previously reported historical controls (82%).

Conclusions

The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.     

The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients

Purpose

The aim of this study was to utilize clinical outcome methodology through multivariable analysis of perioperative factors to predict a successful Kasai-portoenterostomy (PE).

Methods

Records of 81 patients treated for biliary atresia (BA) were reviewed. Outcome was defined as successful if the patient was alive and had no liver transplant (LT). To predict future successful or failed PE, patients were categorized at 6 months post-PE into 2 groups: Success: direct bilirubin (DB) less than 2.0 mg/dL; Failure: DB greater than 2 mg/dL, or the patient was listed/had undergone LT, or had died. Groups were analyzed for positive or negative predictive values (PPV, NPV) at 2 and 5 years after PE. Cox regression was used to determine risk factors for PE.

Results

PE was successful in 38% and failed in 62%. PPV of future success was 96% at 2 years post-PE and 95% at 5 years post-PE, NPV of failure was 76% and 74%, respectively. Bridging liver fibrosis at the time of PE and postoperative cholangitic episodes were interdependent risk factors for a failed PE (P < .05). Other covariates showed no significant relationship for PE outcome.

Conclusion

Classifying of patients 6 months postoperatively allowed us to determine a successful PE outcome. Bridging liver fibrosis at the time of the Kasai, and the increased number of postoperative cholangitic episodes were predictive of a poor PE outcome.     

Intrahepatic biliary strictures after liver transplantation

Biliary complication has been one of the most common complications after liver transplantation. Nonanastomotic strictures and dilatations involving the intrahepatic biliary tree have been recognized as biliary complications. These lesions were reported to be associated with hepatic artery thrombosis; prolonged preservation time; ABO-incompatible organs; and immunological injury, including injuries to vascular endothelial cells (chronic rejection) and the bile duct (primary sclerosing cholangitis). However, the etiology of these lesions appeared to be mostly related to ischemic injury. Anatomical research on the arterial supply of the bile duct has provided further insights into bile duct blood supply and its surgical implications. The biliary tract is supplied with arterial blood by a vasculature called the peribiliary vascular plexus. Any injury to the peribiliary vascular plexus may contribute to ischemic death of the biliary system mucosa. At many points, the process of liver transplantation exposes the endothelial cells and peribiliary vascular plexus to ischemic injury. The majority of intrahepatic biliary strictures (IHBS) are diffuse or bilateral. A percutaneous or an endoscopic approach has been used as the initial treatment. However, a low threshold for surgical intervention (retransplantation) should be adopted, because these patients demonstrate high mortality. The aim of this article is to review the anatomy, etiology, clinical picture, diagnosis, management, and prognosis of IHBS after liver transplantation.     

Reoperation in patients with biliary atresia

Twenty-seven reoperations were done on 23 patients among 100 infants with biliary atresia who have been treated at Tohoku University Hospital between 1971 and 1981. Nineteen patients had a single reoperation and 4 patients had 2 reoperations. We present the results and the role of reoperation in biliary atresia patients in our institution. Excellent bile drainage after reoperation was obtained in 13 of 15 patients with good bile flow after the initial operation. On the contrary, good bile drainage was not obtained by reoperation in 8 of 12 cases without active bile flow after the initial operation. Cessation of bile flow after successful initial operation is an absolute indication for reoperation. Aggressive reoperations under proper indications improve the surgical results in biliary atresia patients.     

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.     

Hepatobiliary scintigraphy after Kasai procedure for biliary atresia: clinical correlation and prognostic value

Background/Purpose

Kasai portoenterostomy (KP) is regarded as first-line treatment for biliary atresia, although its postoperative course is often unpredictable. Hepatobiliary scintigraphy using technetium-labeled iminodiacetic acid derivatives offers a dynamic, objective assessment both of parenchymal liver function and restored biliary excretion. The value of postoperative radionuclide scans was assessed prospectively in a large population of post-KP infants.

Methods

Radionuclide scans consisted of an intravenous dose of 20 MBq of ^99mTc mebrofenin iminodiacetic acid and subsequent gamma camera imaging. Four scan variables were evaluated: the hepatic extraction fraction (HEF; ie, initial liver uptake divided by the peak vascular uptake), the half-life of tracer excretion (TEX), the shape of the excretion curve, and the presence of activity in the Roux loop at 4 hours postinjection. All infants had type 3 biliary atresia with a median age at KP of 59 days (24-120 days). To assess predictive value, outcome (clearance of jaundice and need for transplant) was assessed at 6 months (for 1-week scan) and 2 years (for 6-month scan).

Results

Eighty-seven infants underwent a radionuclide scan at 1 week post-KP. The median HEF was 34% (10%-90%). No relationship could be identified between HEF (P = .2) or excretion curve shape (P = .9) and outcome (at 6 months), and there were too few examples of a measurable TEX to allow meaningful comparison. The only predictive element at this time point was Roux loop activity (positive predictive value, 79%; negative predictive value, 53%; for “good” isotope bowel activity).Forty-four infants completed a second scan at 6 months. Median HEF increased from a baseline of 37% (11%-90%) to 64% (8%-100%) (P < .0001), although there was no significant intercorrelation (P = .12). The most predictive variables (of outcome at 2 years) were curve shape (positive predictive value, = 95%, negative predictive value, 82%) and TEX, and the least predictive was now Roux loop activity.

Conclusions

Early (at 7 days) hepatic scintigraphy is not predictive of poor outcome in general, although Roux loop activity does indicate later success. Later hepatic scintigraphy (at 6 months) allows a detailed assessment of dynamic liver function with biliary excretion variables predictive of outcome in the medium term.     

Corticosteroid therapy in biliary atresia

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

Risk factors affecting late-presenting liver failure in adult patients with biliary atresia

Purpose

Following the Kasai operation, a number of patients have developed liver failure, even after long-term postoperative courses. We assessed the clinical parameters to clarify the early risk factors affecting late-presenting liver failure in biliary atresia.

Materials and Methods

From 1955 to 1991, 277 patients underwent a Kasai operation. Among those patients, 92 survived with their native liver for more than 20 years, and 72 continue to survive with their native liver in good condition (Group 1). In 20 patients, persistent jaundice recurred after the age of 20 years (Group 2). The postoperative courses of these patients were assessed retrospectively, and the clinical parameters, including age at the time of the Kasai operation (AGE, days), the period required for jaundice to disappear (PJD, days), and the association with early cholangitis (CG), were compared between the 2 groups.

Results

Of the 20 patients in Group 2, 8 survived after a liver transplantation (LTx). Eight patients had recurrent jaundice, including 4 on the waiting list for anLTx. Additionally, 2 patients died after anLTx at the ages of 22 and 39. Another patient died of liver failure at the age of 28. One patient died of massive esophageal variceal bleeding at the age of 29. Significant differences were confirmed with respect to AGE (Group 1 < Group 2, p < .001), PJD (Group 1 < Group 2, p < .001), and CG (Group 1: Group 2 = 47 %: 75 %, p = .028).

Conclusions

A considerable number of adult patients developed liver failure, even after the age of 20 years. AGE, PJD, and CG were found to be risk factors affecting late-presenting liver failure. Therefore, close patient follow-up is essential, especially for long-term survivors with a late operative age and early postoperative complications.