Comparison of drainage techniques for biliary atresia

Purpose

Traditional Kasai portoenterostomy and porto-appendiceal duodenostomy have been utilized for biliary atresia. Differences in outcome between patients who underwent either Kasai portoenterostomy or porto-appendiceal duodenostomy were compared.

Methods

A review of all children who underwent a drainage procedure for biliary atresia from 1986 to 2000 (n = 30) was performed. Age at drainage procedure, subsequent liver transplantation, and outcomes were evaluated. Outcome variables included success rates (total bilirubin < 2.0 mg/dL) and survival rate. Statistical analysis was done with χ² and Student’s t test.

Results

Long-term follow-up was available on 28 of 30 patients. Age at biliary drainage was insignificant. Success rates between porto-appendiceal duodenostomy (31%) and Kasai portoenterostomy (82%) were statistically significant. Survival rate for patients who underwent a Kasai portoenterostomy was 10 of 11 patients. Survival rate for patients who underwent porto-appendiceal duodenostomy was 14 of 16 patients. Overall survival rate was comparable between porto-appendiceal duodenostomy (88%) and Kasai portoenterostomy (91%).

Conclusions

Although overall survival rate was comparable, patients who underwent porto-appendiceal duodenostomy were less successful in alleviating hyperbilirubinemia compared with Kasai portoenterostomy. This is shown further by the greater incidence of subsequent liver transplantation in infants with prior porto-appendiceal duodenostomy. Although the appendix may serve as an alternative biliary conduit, traditional Kasai portoenterostomy appears to achieve better biliary drainage.     

Kasai portoenterostomy--new insights from hepatic morphology

Background/Purpose

The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers.

Methods

Explanted livers from 13 consecutive children undergoing transplantation for biliary atresia were examined in detail using a standardized protocol. Group 1 (n = 6) had no Kasai procedure before transplantation at a median age of 8 m. Group 2 (n = 4) were transplanted at a median age of 10 m after a failed Kasai portoenterostomy. Group 3 (n = 3) had a successful Kasai but required transplantation for complications of chronic liver disease at 12-14 years. Pathology findings were correlated with hepatic morphology determined by pretransplant magnetic resonance imaging.

Results

Large perihilar regenerative nodules (8-14 cm diameter) were observed in 2 patients after successful Kasai portoenterostomy, less well-defined perihilar nodules in group 2 patients, and no regenerative nodules in group 1. Microscopically, group 1 had diffuse biliary cirrhosis with evidence of progressive ductopenia during infancy. In group 2, perihilar regenerative nodules showed variable portal fibrosis but no cirrhosis and bile ducts were present with 68%-100% of hepatic arteries; in peripheral cirrhotic areas, bile ducts were absent in patients older than 9 m. The perihilar regenerative nodules in group 3 patients had a noncirrhotic architecture with preserved bile ducts, but the peripheral parenchyma was cirrhotic; one patient had diffuse macronodular cirrhosis. These morphologic findings correlated well with magnetic resonance images, highlighting the preservation of relatively normal perihilar liver architecture after successful Kasai portoenterostomy.

Conclusions

Unoperated biliary atresia is associated with progressive intrahepatic ductopenia leading to diffuse biliary cirrhosis. Kasai portoenterostomy can result in the growth of large perihilar regenerative nodules, probably as a consequence of surviving intrahepatic ducts in this region. In some patients, long-term success after Kasai portoenterostomy may depend on hyperplasia of the perihilar liver.     

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.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

Predicting factor of quality of life in long-term jaundice-free survivors after the Kasai operation

Background/Purpose

The aim of this study was to determine simple predictors for quality of life (QOL) in long-term jaundice-free survivors after the Kasai operation.

Methods

Kasai’s original portoenterostomy was performed on 55 patients with biliary atresia. Among them, records were reviewed retrospectively of 35 long-term (at least 5 years) and jaundice-free (clearance in bilirubin level less than 1.0 mg/dL after Kasai operation) survivors. The patients were divided into 2 groups based on QOL, and the differences in clinical and laboratory data were analyzed statistically.

Results

The ages at Kasai operation, histologic, fibrosis grade of liver biopsy specimen at operation, the first onset and frequency of postoperative cholangitis, and postoperative clearance speed of jaundice after Kasai operation were not significantly different between the 2 groups. The aspartate aminotransferase (AST) level at 1 year was significantly correlated with the serum concentration of hyaluronic acid and an independent predictor for QOL in long-term jaundice-free survivors of the Kasai operation.

Conclusions

The serum AST level at 1 year was a simple, strong predicting factor of QOL and liver dysfunction in long-term jaundice-free survivors after Kasai operation and may prove useful in planning liver transplantation.     

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.     

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.     

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 significance of serum hepatocyte growth factor levels in planning follow-up of postoperative jaundice-free patients with biliary atresia

Purpose

The aim of this study was to clarify the significance of serum hepatocyte growth factor (HGF), interleukin (IL)-6, and IL-1 receptor antagonist (ra) levels in the evaluation of disease status in jaundice-free survivors with biliary atresia after Kasai operation.

Patients and Methods

Serum concentrations of HGF, IL-6, and IL-ra were measured in 31 long-term jaundice-free patients with biliary atresia after Kasai operation and 29 controls. Patients were divided into 4 groups: group A (n = 8), normal liver function; group B (n = 9), mild liver dysfunction without portal hypertension; group C (n = 9), moderate liver dysfunction with controllable portal hypertension; and group D (n = 5), receiving liver transplantation.

Results

Serum IL-6 levels were significantly higher in patients than in controls. There was no difference in serum IL-6 levels among groups B, C, and D. Serum IL-1ra levels were elevated according to liver dysfunction. Serum HGF levels in group D were significantly higher than in controls and the other groups. Serum hyarulonic acid levels were positively correlated with serum levels of IL-1ra and HGF.

Conclusions

Elevation of serum IL-1ra and HGF levels correlated with the progression of liver fibrosis and dysfunction. In particular, serum HGF levels could be used as a predictor for requiring liver transplantation.     

A novel approach to the management of late-onset liver failure in biliary atresia

Purpose

Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing severe liver failure. The authors recently have treated 2 patients who had acute hyperbilirubinemia years and months after a successful PE and had dilated intrahepatic cysts. A combined operative and percutaneous approach reestablished drainage and a reduction in their bilirubin levels.

Methods

Data from 2 cases of BA and late-onset hyperbilirubinemia from obstruction were reviewed and analyzed.

Results

Two patients (15-year-old boy and a 2.5-year-old girl) presented with increasing serum bilirubin after a PE for BA in infancy. Both had extensive preoperative workup, which showed intrahepatic biliary dilatation in one and a large bile lake in the other. They underwent attempted percutaneous transhepatic cholangiography and stenting, followed by an intraoperative transhepatic approach in which the dilated ducts were connected to the PE. A rapid and sustained reduction in the serum bilirubin level was noted in these patients.

Conclusions

When patients with BA after a successful PE present with sudden onset of hyperbilirubinemia, imaging for biliary obstruction should be carried out. If biliary dilatation is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant.     

Cholangitis as a late complication in long-term survivors after surgery for biliary atresia

Background/purpose

A retrospective review was performed to assess the utility of diagnostic imaging (DI), efficacy of treatment, and outcome of late cholangitis in long-term survivors after surgery for biliary atresia.

Methods

Sixty-one patients surviving without liver transplantation (LTx) for more than 20 years were divided into 2 groups depending on whether cholangitis developed after age 20. Clinical factors including the type of obstruction, the age at the initial operation, and the early complication with cholangitis were compared between the 2 groups. DI such as computed tomography scan and magnetic resonance imaging, clinical courses after treatment of cholangitis, and current status of the patients were also evaluated.

Results

Thirteen patients had cholangitis after age 20. There was no statistical difference in the clinical factors studied between the 2 groups. Abnormal DI findings including dilatation of intrahepatic bile ducts and hepatic fibrosis were seen in 10 patients with late cholangitis. One patient died, and 2 ultimately underwent LTx. The remaining 10 patients including 4 with normal or near-normal liver function have survived without LTx.

Conclusions

Although the majority of the patients had potential predisposing factors for cholangitis such as dilatation of intrahepatic bile ducts, a few patients unexpectedly had cholangitis without such abnormal findings after an excellent, long-term postoperative course.     

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.     

Medial thickening of hepatic artery branches in biliary atresia. A morphometric study

Background/Purpose

Medial layer hypertrophy of hepatic arterial branches may be associated with biliary atresia (BA) pathogenesis. This study aimed at evaluating medial layer thickness in hepatic arterial branches at portoenterostomy and liver transplantation.

Methods

The authors evaluated 1274 arterial branches both in BA cases and in control subjects involving a total of 1108 arterioles and 166 arteries. Arterial branch characteristics were morphometrically evaluated in 47 BA patients at the time of portoenterostomy. Controls were patients with intrahepatic cholestasis (n = 3), immature neonates (n = 7), and infants (n = 7) without liver disease. Progression of medial layer thickening between the time of portoenterostomy and transplantation was evaluated in 7 BA patients. Biliary atresia patients at the time of transplantation were compared with non-BA-transplanted patients (n = 4).

Results

The arterial medial layer of BA cases at portoenterostomy was thicker than that of infants without liver disease (P = .03). The arterial medial thickness increased during the interval between portoenterostomy and transplantation (P = .05). Arterioles and arteries with thickened medial layers were found in transplanted BA patients but not in patients transplanted for other liver diseases (P = .05 and P = .01). Thickening of the medial layer of the hepatic arteries was associated with focal distribution of interlobular bile ducts in portal spaces in BA (P = .02).

Conclusions

In BA, there is a progressive thickening of the arterial medial layer, suggestive of vascular remodeling, which is associated to the disappearance of interlobular bile ducts.     

Review of historical cohort: ursodeoxycholic acid in extrahepatic biliary atresia

Background

Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hepatocytes, and dissolve gallstones.

Purpose

The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia.

Methods

We used a statistical analysis of data of records of infants and children having extrahepatic biliary atresia who underwent Kasai portoenterostomy and attended Hepatology Clinic, New Children's Hospital, Cairo University, Egypt, from May 1985 until June 2005.

Results

Of 141 infants with extrahepatic biliary atresia, 108 received ursodeoxycholic acid for mean duration ± SD of 252.6 ± 544.9 days in a dosage of 20 mg/kg per day. The outcome of infants who did not receive ursodeoxycholic acid and those who did was the following: 8 (24.2%) and 11 (10.18%) had a successful outcome (P = .043), 0 (0%) and 7 (6.4%) improved (P = .148), 25 (75.7%) and 84 (77.7%) had a failed outcome (P = .489), and none vs 5 died (4.6%) (P = .135), respectively. The predictors of successful outcomes were age less than 65 days at portoenterostomy (P = .008) and absence of ursodeoxycholic acid intake (P = .04) with a likelihood of a successful outcome that was 2.8, that associated with ursodeoxycholic acid intake.

Conclusion

In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications.     

Continuous monitoring tools for pediatric surgical outcomes: an example using biliary atresia

Purpose

Continuous monitoring tools can be used to monitor surgical outcomes over time. We illustrate the use of CUmulative SUM (CUSUM) charts in monitoring outcomes of Kasai portoenterostomy for treatment of biliary atresia at a supraregional unit.

Methods

Data on 57 consecutive infants who underwent a Kasai portoenterostomy performed by a single surgeon between June 1994 and June 2006 were collected. A procedure was defined as successful if clearance of jaundice (plasma bilirubin level <20 μmol/l) was achieved within 6 months of surgery. We applied cumulative observed-minus-expected, sequential probability ratio test (SPRT), and zero-resetting SPRT CUSUM charts and compared the results with those of standard aggregate data analyses. An expected failure rate of 43.0%, based on the national average failure rate, was used.

Results

The failure rate observed after 57 operations was 29.8%. The zero-resetting SPRT chart indicated a lower-than-expected failure rate earlier than did the aggregate data analyses and any of the other continuous monitoring techniques.

Conclusions

The CUSUM chart method provides ongoing feedback that can be used for continuous monitoring of the outcome of a procedure to ensure that standards of care are maintained. Its use as a routine monitoring tool in pediatric surgery deserves wider recognition.     

The robotic approach to complex hepatobiliary anomalies in children: preliminary report

Purpose

Robotic technology allows surgeons to perform complex procedures which may be difficult with standard laparoscopic instruments. We believe that complex hepatobiliary procedures are ideally suited for robotic surgery in children and present our experience with Kasai portoenterostomy and excision of choledochal cysts.

Methods

We performed 4 complex hepatobiliary procedures in children using the Da Vinci surgical robot (Intuitive Surgical, Sunnyvale, CA): 2 Kasai portoenterostomies and 2 choledochal cyst resections. Both Kasais had the Roux-en-Y jejunojejunostomy performed extracorporeally through the 12mm umbilical trocar site. Both choledochal cysts had the Roux-En-Y jejunojejunostomy performed intracorporeally. All patients had their hepatobiliary to enteric anastomosis performed intracorporeally.

Results

Total average time was 6 hours and 12 minutes for the Kasai and 7 hours and 38 minutes for the choledochal cysts. The average robotic console time for all cases was 6 hours. No intraoperative or perioperative complications occurred. Average length of hospital stay was 4 days. Both choledochal cyst patients were doing well after 9 and 12 months with no complications. One Kasai patient is doing well 14 months after Kasai with a normal bilirubin. The other Kasai patient did well for a year with a normal bilirubin. However, the patient slowly developed intrahepatic bile lakes despite a normal bilirubin and a well draining Kasai as demonstrated by hepatobiliary iminodiacetic acid (HDA) scan. He began having recurrent episodes of cholangitis and we referred him for liver transplantation.

Conclusion

Minimally invasive robotic complex hepatobiliary surgery is safe and effective in children. The 3-dimensional imaging and improved articulations make these procedures particularly suited for robotics over standard laparoscopy.     

The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis

Background/Purpose

In biliary atresia (BA), a derangement in the biliary system remains, despite portoenterostomy performance. Many factors can influence the disease progression rate. This study aimed to analyze the association between biliary proliferation extent in biopsies from BA patients and postoperative prognosis.

Methods

Biliary proliferation was evaluated by a morphometric analysis of the cytokeratin 7 positivity percentage (PCK7) in wedge liver biopsies from 47 BA patients. The extent of fibrosis was evaluated by a fibrosis score (FS). The outcome 1-year native liver survival was correlated, using a multivariable regression analysis, with PCK7, FS, and age at portoenterostomy.

Results

The PCK7 ranged between 0.80% and 14.79% (M ± SD = 7.36% ± 4.15%). Patients who died or underwent transplantation had higher PCK7 than survivors with their native livers (P < .001). The area under the receiver operating characteristic curve for PCK7 in relation to the outcome was 0.845 (P < .001). The cutoff point of PCK7 for the maximal effect on postoperative prognosis was 10.18% (sensitivity = 0.71, specificity = 0.88). The PCK7 was the only studied variable associated with 1-year native liver survival, independently of age and FS (P = .002).

Conclusion

The extent of biliary proliferation at portoenterostomy, evaluated by PCK7, was associated with 1-year native liver survival of BA patients.     

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.     

Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen

Aim

The role of adjuvant therapy with corticosteroids and choleretics after Kasai portoenterostomy for biliary atresia (BA) remains uncertain. Experience with a novel postoperative adjuvant therapy regimen is reported.

Methods

Between 1994 and 2006, 71 infants with BA were referred. Four died from uncorrectable congenital heart disease/cardiorespiratory failure without undergoing portoenterostomy, 7 underwent primary liver transplantation (3 referred ≥19 weeks of age), and 60 underwent portoenterostomy at a median of 51 (10-104) days. Of these, 55 (92%) had type 3 BA and 6 had the BA splenic malformation syndrome. Fifty (83%) received the following adjuvant therapy beginning on postoperative day 5: oral dexamethasone 0.3 mg/kg bd for 5 days, 0.2 mg/kg bd for 5 days, and 0.1 mg/kg bd for 5 days together with oral ursodeoxycholic acid 5 mg/kg bd and phenobarbitone 5 mg/kg nocte, both of which were continued for 1 year. All infants received routine perioperative prophylactic antibiotics.

Results

Overall, 42 of 60 (70%) infants cleared their jaundice (bilirubin <20 μmol/L): 38 of 50 (76%) with the dexamethasone/ursodeoxycholic acid regimen compared with 4 of 10 (40%) not receiving this adjuvant treatment. There were 4 late deaths after portoenterostomy: 2 from associated congenital disorders and 2 after liver transplantation. Of the remaining 56 children, 39 (70%) are currently alive with their native liver at a median follow-up of 3.3 years and 17 are alive after liver transplantation. Surgical complications occurred in 3 after portoenterostomy: adhesive bowel obstruction (2) and an anastomotic leak. One infant had gastrointestinal bleeding that may have been related to dexamethasone, but this resolved with ranitidine. There were no perioperative septic complications.

Conclusion

In this series, adjuvant postoperative treatment with a short course of oral dexamethasone and longer-term ursodeoxycholic acid significantly improved the outcome after Kasai portoenterostomy.     

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.