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     

Hepatolithiasis after hepatic portoenterostomy for biliary atresia

Background

Hepatolithiasis after hepatic portoenterostomy for biliary atresia has been paid little attention, with only 22 reported cases.

Patients and Methods

Fifteen patients underwent living-related liver transplantation for biliary atresia after hepatic portoenterostomy in our hospital between 1998 and 2004. The resected livers were examined for the existence and location of hepatolithiasis, composition of the calculi, and bacterial infection of bile. The relation between a history of cholangitis and the presence of hepatolithiasis was analyzed.

Results

Intrahepatic calculi were found in 8 (53%) of 15 patients. The calculi consisted of almost 100% calcium bilirubinate. Calculi were found in bile lakes in 8 patients. Bacteria were present in the bile in 8 (53%) of the 15 patients. Of the 8 patients, 7 (88%) had a history of ascending cholangitis.

Conclusions

Hepatolithiasis occurs after hepatic portoenterostomy for biliary atresia more frequently than previously thought. Bile stasis and possibly bile infection are the main causes of calculi formation.     

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.     

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 biliary cysts in children with biliary atresia who have had a Kasai operation

Purpose

In patients with biliary atresia who had undergone a Kasai operation, treatment of intrahepatic biliary cysts (IBCs), particularly when complicated by cholangitis, is often difficult because the clinical implications and the course of IBCs are unclear. Thus, to determine the best treatment guideline, the morphology of IBCs, the clinical course, and the outcomes of such patients were evaluated.

Patients and Methods

A total of 44 patients with type III biliary atresia who underwent a Kasai operation from 1977 to 2005 were postoperatively examined for IBC by using ultrasonography and computed tomography. We classified the IBCs based on their number and shape.

Results

Intrahepatic biliary cysts developed in 12 of 54 patients. Three patients with solitary simple cysts and 1 patient with multiple simple cysts had no history of cholangitis. Two patients with multiple simple cysts had cholangitis at the time of IBC diagnosis and were treated with percutaneous transhepatic cholangiodrainage (PTCD). Patients with simple IBCs did not develop persistent cholangitis and their prognosis depended largely on their liver function; 3 of 6 patients remained healthy without cholangitis, whereas 3 patients required liver transplantation (LT) because of progressive liver failure or worsening hepatopulmonary syndrome, and not severe cholangitis. On the other hand, all 6 patients with multiple complicated IBCs had persistent cholangitis, eventually requiring LT. Even after bile flow to the intestine was reestablished after PTCD, both IBCs and cholangitis recurred. These patients required LT because of severe cholangitis.

Conclusions

Intrahepatic biliary cysts without cholangitis are not a source of infection and require no treatment. Simple IBCs with cholangitis can be controlled by antibiotics and/or PTCD. Patients with multiple complicated IBCs have a poor prognosis, requiring LT to control cholangitis. Although PTCD can control cholangitis in these patients as they wait for LT, PTCD does not alleviate it—LT is the final solution.     

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.     

A study of patients with long-term bile flow after hepatic portoenterostomy for biliary atresia

Thirty-five cases of biliary atresia that demonstrated the creation of internal fistula between intrahepatic bile ducts at the portahepatis and intestine and showed long-term bile flow after our hepatic portoenterostomy were investigated from various aspects. These 35 cases were divided into Group A cases, in which jaundice disappeared within three months after surgery, and Group B cases, in which persistent jaundice was seen more than three months after surgery. (1) Severe liver fibrosis and degeneration of intrahepatic bile ducts were more often seen in Group B cases than in Group A cases. (2) The age at the time of surgery and the size of intrahepatic bile ducts at the portahepatis did not have much influence on the operative results in those 35 cases. (3) Measurement of the amount of bile flow and bile acid excretion obtained from Suruga II enterostomy is useful for evaluating the postoperative results of biliary atresia patients. (4) Reoperation that includes curettage and rehepatic portoenterostomy, and serious postoperative ascending cholangitis were each closely related to postoperative persistent jaundice.     

Successful revision of portoenterostomy in an infant with biliary atresia

We present a case report of a boy with biliary atresia who, after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools. Nuclear medicine imaging study showed no excretion. Broad-spectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.     

Stimulation of bile output by gastrointestinal hormones following portoenterostomy for biliary atresia

For biliary atresia portoenterostomy with externally draining conduit provides a model for quantitation of hepatic excretory function and for assessment of the physiologic response of the intrahepatic biliary system to gastrointestinal hormones in a human hepatopathologic condition. Four patients with biliary atresia were serially evaluated from 2 weeks to 43 months following total bile diverting portoenterostomy. A fifth patient with no bile flow provided a control for these studies. The pattern of Rose Bengal excretion for three patients with a satisfactory clinical course was different from that of a fourth patient with highly variable flow and persistent cholestasis. Marked volume and bicarbonate concentration increases in bile were noted 30 to 45 minutes after secretin infusion but only in the four patients with bile flow. The volume response to glucagon was more diffuse. Bilirubin and bile acid concentrations decreased in the stimulated bile flow periods and hourly outputs of these cholephils were not increased above basal. During two intervals of low bile output, secretin markedly increased bile flow in the patient with persistent cholestasis establishing the patency of the hepatoenteric anastomosis (functional obstruction) in contrast to the lack of secretin response in the control (structural obstruction).     

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.     

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.     

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

Background/purpose

The role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA).

腹腔镜与开腹行肝门肠吻合术治疗小儿先天性胆道闭锁效果的对比研究

目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月~2005年9月,对26例胆道闭锁(Ⅲ型)患儿在腹腔镜下行肝门空肠吻合术,充分显露肝门,游离并于肝门纤维块处切除胆囊,提起距Tre itz韧带20 cm处空肠,经脐将空肠提出腹壁外并逐渐拉出远端40 cm范围,距Tre itz韧带20 cm横断空肠并封闭远端,将近端与远侧30~35 cm处空肠行端侧吻合,把肠管送回腹腔。将肝支空肠襻经结肠后隧道上提至肝下,肝门空肠端侧吻合。与同期同年龄段34例胆道闭锁(Ⅲ型)行开腹肝门空肠吻合术的患儿进行对照,比较两组手术时间、术中出血量、手术前后肝功能指标、平均住院天数和住院费用及术后转归情况。结果腹腔镜组手术出血量少于开腹组[(15.4±5.0)m l vs(33.8±19.4)m l,t=-4.709,P=0.000],住院费用高于开腹组[(19 153.9±619.5)元vs(15 116.7±898.4)元,t=19.607,P=0.000];两组手术时间、住院天数无显著差异。术后腹腔镜组血清总胆红素、直接胆红素、ALT和AST比开腹组下降明显。腹腔镜组术后切口疝1例;开腹组急性肝衰竭、伤口裂开各1例,伤口感染2例,两组早期并发症发生率无显著性差异(2χ=0.395,P=0.530)。术后4个月时随访,腹腔镜组13例(占50%)患儿退黄;开腹组18例(53%)患儿退黄。结论腹腔镜肝门空肠吻合术治疗小儿先天性胆道闭锁微创效果明显,方法安全、有效。     

Vitamin A can ameliorate fibrosis of liver in an established rat model of biliary atresia and Kasai portoenterostomy

Background

Antifibrosis therapy may prevent progressive liver fibrosis after successful Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. The aim of this study is to evaluate the efficacy of antifibrosis therapy in a rat model of BA and KPE.

Prognostic implications of bile lakes after surgery for biliary atresia

Purpose

The aim of the study was to establish the natural history of bile lakes (BLs) in patients with postportoenterostomy biliary atresia (BA) and assess their prognostic implications, in particular, if they are a risk factor for cholangitis.

Methods

One hundred twenty-one postsurgical BA patients were divided into 2 groups as follows: period 1, or preliver transplantation era group (n = 56), and period 2, or liver transplantation era group (n = 65). Presence of BL and outcome were examined in relation to the incidence of cholangitis.

Results

In period 1, there were more multiple BLs (MBLs) in nonsurvivors than survivors. Non-BL survivors had significantly lower mean total number of episodes of cholangitis than MBL survivors (P < .01).In period 2, 58 survived (native liver in 35; transplanted liver in 23). Bile lakes were present in 9/58 (16%) survivors (single in five, multiple BLs in four), and 1/7 (14%) nonsurvivors had MBLs. Mean total number of episodes of cholangitis in the 4 MBL survivors and the 49 non-BL survivors were not significantly different.

Conclusion

Our results suggest that presence of BL is a risk factor for cholangitis in the long-term but not in the short-term.     

Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia

We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5-0 double-needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin, <2 mg/dL) was achieved in 13 (92.9%) of 14 patients.