Most patients with BA have abnormal LFT results and vitamin K deficiency by the time of diagnosis. All infants should have parenteral vitamin K2 supplementation for several days before exploration. Bowel preparation should commence with oral kanamycin and glycerin enemas to decrease abundant colon microbiota to minimize intestinal gas. Patients should be nil by mouth for 24 hours prior to surgery. Parenteral broad-spectrum antibiotics should be administered preoperatively just before the skin incision. Preoperative blood tests should include complete blood count, coagulation profile, and LFTs.

All our Roux limbs are customized and short as we recommended, ^, because predetermined Roux limbs (30, 40, or 50 cm in length) will not be appropriate for the size of the patient. Moreover, they can become tortuous as the patient grows, causing stasis in the Roux limb that contributes to cholangitis. After creation of the jejunojejunostomy, the customized Roux limb should be approximated to the native jejunum for 8 cm cranially to prevent the contents of the native jejunum from refluxing into the Roux limb ( Fig. 41.5 ). A 10-mm long antimesenteric enterotomy is made next to the closed end of the Roux limb and the jejunojejunostomy should fit naturally into the splenic flexure when it is returned to the abdominal cavity. A scalpel should be used to make the enterotomy in the jejunum that will be used for the portoenterostomy anastomosis to prevent thermal injury, which could occur if diathermy is used. Thermal injury will cause scarring along the anastomotic line of the portoenterostomy and should never be used. The jejunal limb is passed through a retrocolic window to lie without tension at the porta hepatis.

(A) Short and custom Roux-en-Y (RY) is more appropriate for the size of the patient and can prevent stasis as the patient grows. (B) The length of the RY limb is determined by exteriorizing the jejunal loop through the umbilicus and measuring the distal end (E) of the limb to be 3 cm above the xiphoid process. The jejunojejunostomy ( arrowheads ) will then fit naturally into the splenic flexure after anastomosis. Arrows show where the RY limb has been approximated to the native jejunum for 8 cm cranially to streamline flow into the distal jejunum and eliminate reflux into and stasis in the RY limb.

From Yamataka A, Lane GJ, Cazares J. Laparoscopic surgery for biliary atresia and choledochal cyst. Sem Pediatr Surg . 2012;21:201.

Over the past 20 years, hepatic portoenterostomy for BA has been modified to involve more extensive lateral dissection around the porta hepatis and a much wider anastomosis. ^, In reality the current operation is essentially an extended portoenterostomy ( Fig. 41.6C ) that barely resembles Kasai’s original procedure ( Fig. 41.6B ). This extended portoenterostomy is still widely used for the open surgical treatment of BA. The patient is placed supine on an operating table that can allow intraoperative cholangiography to be performed. An extended right subcostal incision, dividing the muscle layers, is used to expose the inferior margin of the liver. After division of the falciform and triangular ligaments, the liver is delivered from the abdominal cavity. This maneuver provides an excellent operative field for dissection of the porta hepatis, although some surgeons do not recommend this maneuver because of risk for hepatic congestion due to kinking of the hepatic vein. Cholangiography is recommended to confirm the hepatobiliary anatomy and type of biliary atresia ( Fig. 41.7 ). The fundus of the atretic gallbladder is mobilized from the liver bed, and a fine feeding tube is passed into the gallbladder through a small cholecystotomy incision. If bile is detected on aspiration of the gallbladder, a small amount of contrast material is injected. However, in most patients with BA, the lumen of the atrophic gallbladder is already obstructed, and it is impossible to insert even a 4 French catheter, so cholangiography is not possible. In such cases, macroscopic inspection of the porta hepatis should be sufficient for diagnosing BA and the hepatic portoenterostomy is performed. ^,

Salient features of three portoenterostomy procedures are depicted. (A) Modified Kasai portoenterostomy. Interrupted shallow sutures ( thin broken lines ) are placed in the liver parenchyma around the transected biliary remnant, except at the 2 and 10 o’clock positions, where the right and left bile ducts should be. If sutures are necessary at the 2 and 10 o’clock positions to prevent an anastomotic leak, shallow interrupted sutures ( thin dotted lines ) are be placed only in the connective tissue near the right and left hepatic arteries or the hepatoduodenal ligament at the porta hepatis. (B) Kasai’s original portoenterostomy ^, : a continuous suture ( looped line ) is placed in the side of the transected biliary remnant, except at the 2 and 10 o’clock positions, where sutures ( dotted lines ) are placed in the connective tissue. (C) Extended portoenterostomy. Deep interrupted sutures (bold broken lines) are placed in the liver parenchyma, even at the 2 and 10 o’clock positions.

From Nakamura H, Koga H, Wada M et al. Reappraising the portoenterostomy procedure according to sound physiological/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg . 2012;28:205; and From Yamataka A, Lane GJ, Cazares J. Laparoscopic surgery for biliary atresia and choledochal cyst. Sem Pediatr Surg . 2012;21:201.

(A) Intraoperative cholangiogram; noncystic type I biliary atresia. (B) Intraoperative cholangiogram suggesting type III biliary atresia.

The mobilized gallbladder is used as a guide for locating the fibrous remnant of the common bile duct. After the caudal end of the common bile duct is ligated and divided at the upper border of the duodenum, the cephalad portion of the common bile duct with the gallbladder attached is dissected above the bifurcation of the portal vein. The portal vein and the hepatic arteries are exposed along their entire courses. Vessel loops should be applied to the right and left hepatic arteries and the right and left portal branches to facilitate portal dissection ( Fig. 41.8A ).

The current favored technique for a portoenterostomy involves an extended lateral dissection around the porta hepatis with a very wide anastomosis. (A) Photograph of the initial mobilization of the gallbladder and atretic bile ducts and dissection/exposure of the porta hepatis. After the common bile duct remnants are severed from the duodenal side, the dissection proceeds cephalad, and the portal bile duct remnants are freed from the underlying structures. The portal vein ( blue ) and hepatic artery ( red ) have been encircled with vessel loops. Several small vessel branches between the portal vein to the fibrous remnant can be identified and divided between ligatures. (B) The portal bile duct remnants must be dissected 5 or 6 mm proximal to the anterior branch of the right hepatic artery on the right side and as far left as the entrance of the obliterated umbilical vein into the left portal vein. The fibrous cone is sharply transected at the level of the posterior surface with scissors or a scalpel and is removed. The fibrous cone should have an extensive transected surface, which allows a wide anastomosis. (C) The end of the Roux-en-Y limb is anastomosed around the transected end of the fibrous remnant. Sutures should not be placed into the transected surface of the bile duct remnant, because minute bile ducts may be present. As much of the transected hilar surface as possible, including all potentially usable remnants of the intrahepatic ducts in the area between and beneath the branches of the right and left portal veins, is incorporated in the anastomosis. It is important to retract the right and left portal veins and hepatic arteries to allow extensive reception of the biliary remnant to allow a wide portoenterostomy anastomosis.

The hepatic ducts usually form a cone-shaped fibrous mass anterior to the bifurcation of the portal vein. Several small vessels connecting the portal vein to the fibrous cone are divided after being ligated. The posterior aspect of the fibrous cone is freed completely. The anterior aspect of the fibrous cone and the quadrate lobe of the liver are then exposed. The fibrous biliary plate should be dissected as far as the anterior branch of the right hepatic artery on the right side and as far as the point where the obliterated umbilical vein joins the left portal vein on the left. During dissection, 5-0 or 6-0 absorbable sutures are usually placed in the liver surface posterior and caudal to the fibrous plate (see Fig. 41.8B ).

The right and left portal veins and hepatic arteries must be retracted to allow extensive resection of the biliary remnant and a wide portoenterostomy. The fibrous cone is transected sharply at the level of the posterior surface of the portal vein with scissors or a scalpel and is removed. The fibrous cone should have an extensive transected surface sufficient to allow a wide anastomosis. In other words, in an extended portoenterostomy, dissection of the porta hepatis is not confined just to the area around the base of the fibrous ductal plate. ^, As much of the transected surface as possible, including all potentially usable remnants of the intrahepatic ducts in the area between and beneath the branches of the right and left portal veins, is incorporated in the anastomosis.

After transection of the biliary remnant, bleeding points are controlled by packing with gauze. Diathermy should not be used as it can cause thermal injury to any microscopic bile ducts that may be patent. A liver biopsy may be performed depending on the surgeon’s preference. If intraoperative histology of the transected portal fibrous plate cannot identify patent microscopic bile ducts at the transected surface, further cephalad transection of the portal fibrous plate is recommended.

The portoenterostomy anastomosis is performed either end-to-side or end-to-end beginning posteriorly using the 5-0 or 6-0 sutures that were placed previously to the fibrous plate (see Fig. 41.8C ). Next, the anterior edge of the jejunum is anastomosed to the liver parenchyma anterior to the transected fibrous remnant with interrupted 5-0 or 6-0 absorbable sutures, including the 2 and 10 o’clock positions. There should be adequate space between the anterior margin and the remnant fibrous plate. A drain is placed in the foramen of Winslow through a stab incision in the right abdominal wall and the wound is closed.

In contrast to the extended portoenterostomy technique just described, in both Kasai’s original portoenterostomy and our modified Kasai procedure (see Fig. 41.6B and A , respectively), dissection of the porta hepatis is confined to the area around the base of the fibrous biliary plate, which is transected shallowly. ^{, ,} As a result, the portoenterostomy anastomosis is not as wide as in the extended portoenterostomy procedure. Interrupted sutures are placed in the liver parenchyma around the outer edge of the transected biliary remnant shallowly. At the 2 and 10 o’clock positions, where the right and left hepatic ducts should be, sutures are placed very shallowly in the connective tissue near the right and left hepatic arteries or the hepatoduodenal ligament at the porta hepatis (see Fig. 41.6A ), to minimize microscopic bile duct injury. ^, (See Fig. 41.6 for a comparison of modified Kasai [ Fig. 41.6A ], Kasai [ Fig. 41.6B ], and extended [ Fig. 41.6C ] portoenterostomies.)

A study recently compared outcomes with respect to depth of dissection of the fibrous biliary remnant of the common bile duct performed at different periods of time as trends changed. They divided 256 cases into 4 groups; group 1 : limited dissection performed from 1972 to 1981 ( n = 91); group 2 : limited dissection performed from 1992 to 2000 ( n = 80); group 3 : extended dissection performed from 1992 to 2000 ( n = 46); and group 4 : moderately deep dissection performed from 2001 to 2014 ( n = 39). Jaundice clearance rates were 65.9%, 77.5%, 63.0%, and 87.2%, respectively; rates of early cholangitis were 60.4%, 53.8%, 37.0%, and 23.1%, respectively; redo portoenterostomy rates were 15.4%, 37.5%, 17.4%, and 5.1%, respectively; 10-year native liver survival rates were 53.8%, 60.1%, 44.1%, and 73.7%, respectively. Thus, they recommended shallow dissection of the fibrous biliary remnant of the common bile duct, but their recommended level is actually deeper than Kasai’s original and our modified Kasai portoenterostomies.

Kimura et al. reported that in correctable BA, although a wide and deep portal dissection is not required, excision of the patent common bile duct cephalad to the porta hepatis is important. Any cyst-like structure should be excised and should not be used for anastomosis to the intestine. Failure to remove all abnormal common bile duct or hepatic duct tissue may result in anastomotic stricture or cholangitis.

In another study, a series of 323 BA patients who underwent surgery between 1953 and 2004 were reviewed. Fifty were classified as type I. Of these 50, 28 had portoenterostomy and 22 had hepaticoenterostomy. Overall survival for type I patients who did not require liver transplantation was significantly better than for type II/III patients (52% vs. 33%). However, while there was a higher incidence of cholangitis in type I patients, the difference was not statistically significant.

A recent review of 200 BA patients treated surgically at one institution from 1963 to 2008 found that long-term outcome was excellent in 12 patients who had hilar cyst anatomy and underwent hepaticojejunostomy (type I cyst: 9 cases and a subtype of type II: 3 cases). The overall survival of patients with native livers in their series was 83.3%.

The first laparoscopic Kasai portoenterostomy procedure was described in 2002. Since then, few others have been reported, probably because of a combination of the technical difficulty and negative research findings, such as increased incidence of postoperative complications and worse early clinical outcomes, compromised liver perfusion during pneumoperitoneum causing liver cell damage, and elevated intraabdominal pressure decreasing proliferation and inducing apoptosis in hepatocytes in a rat model. Also a prospective study comparing laparoscopic with conventional portoenterostomy found that although the laparoscopic procedure was feasible, survival after 24 months was worse in the laparoscopic group. In another study, there was no measurable benefit of laparoscopy over open portoenterostomy when scarring and adhesions at the time of liver transplantation were compared. In fact, a recent meta-analysis of outcome of laparoscopic portoenterostomy for BA concluded that, based on native liver and patient survival rates, laparoscopic portoenterostomy should not be performed for the treatment of BA patients. However, a 2011 study found that outcomes after laparoscopic Kasai were relatively good after a mean of 72 months (range: 33–89 months), with 50% (eight cases) of patients being jaundice-free with normal bilirubin levels.

Nevertheless, at Juntendo University, we began performing the modified Kasai portoenterostomy procedure laparoscopically in 2009. During the laparoscopic Kasai, the LigaSure is used to divide portal vein branches draining into the caudate lobe at the porta hepatis. The LigaSure generates much less lateral heat than monopolar hook diathermy; thus, thermal injury to microscopic bile ductules in the fibrous plate during dissection can be minimized. ^, A customized Roux limb is created by exteriorizing the jejunum through the umbilical port. After returning the Roux limb to the abdominal cavity, the portoenterostomy is then performed as described in the modified Kasai portoenterostomy (see Fig. 41.6A ).

The minimally invasive Kasai portoenterostomy is gaining support. Recently, we reported on 22 BA patients from Juntendo (type III: n = 19, type II: n = 3) who underwent modified Kasai (laparoscopic) portoenterostomies between 2009 and 2016 and have good midterm outcomes. The mean age at modified Kasai (laparoscopic) portoenterostomy was 67.1 days (range: 29–119); the mean postoperative follow-up was 4.6 years (range: 1.3–8.3); the postoperative jaundice clearance (total bilirubin ≤1.5 mg/dL) was 77.3% (17/22) at 3 months and 90.9% (20/22) at 6 months; and the survival with the native liver was 90.9% (20/22) at 6 months of age, 77.3% (17/22) at 1 year of age, 73.7% (14/19) at 2 years of age, and 81.3% (13/16) at 3 years of age. Other authors also expressed support for the MIS approach, concluding that experienced pediatric laparoscopic surgeons should reconsider laparoscopic portoenterostomy in babies with BA. Another report investigated the efficacy of laparoscopic portoenterostomy compared to open portoenterostomy in the treatment of BA while employing biochemical markers for assessment of liver function. Historically, laparoscopic surgery for BA faced skepticism due to perceived inferior outcomes compared to the traditional open approach, leading to its limited adoption in certain regions. However, recent advancements in MIS have sparked a reevaluation of laparoscopic portoenterostomy efficacy. The study encompassed 70 consecutive BA patients treated with either laparoscopic or open surgery between 2009 and 2021. Notably, laparoscopic portoenterostomy demonstrated several advantages over its counterpart, including reduced intraoperative blood loss, faster recovery times, smaller incisions, and diminished postoperative pain. Additionally, laparoscopic portoenterostomy minimizes interference with liver hemodynamics and may result in fewer postoperative adhesions, enhancing its appeal as a surgical approach. Utilizing biochemical markers such as AST, ALT, cholinesterase (ChE), c-GTP, and PLT, the study rigorously evaluated liver function in both laparoscopic and open groups. Surprisingly, despite initial concerns, the study revealed no significant differences in biochemical marker outcomes between the two groups. This suggests that laparoscopic portoenterostomy yields comparable outcomes to open portoenterostomy in terms of liver function assessment. Furthermore, subjects were classified into biochemical status categories (BSCs) based on their postoperative outcomes, with similar distributions observed between both groups. Long-term follow-up underscores the rarity of achieving normal biochemical marker after portoenterostomy, underscoring the complexities of BA management. Additionally, the study investigated the efficacy of redo-portoenterostomy in cases of deteriorating clinical status, demonstrating promising outcomes akin to nonredo cases. These findings challenge earlier negative perceptions of laparoscopic portoenterostomy and underscore its potential as a valid treatment option for BA. Nevertheless, the study calls for further research with larger sample sizes to corroborate these findings and refine treatment protocols for BA patients. In a study conducted in Nagoya, the outcomes of laparoscopic portoenterostomy and open portoenterostomy as treatments for BA were compared from 2003 to 2020. The study analyzed 119 cases of noncorrectable BA, with 53 cases undergoing laparoscopic portoenterostomy and 66 cases undergoing open portoenterostomy. Despite the laparoscopic procedure requiring a longer operative duration, it resulted in significantly reduced blood loss and faster postoperative recovery, including a shorter time for resuming oral intake and removing drains. The rate of complete resolution of jaundice was similar between the laparoscopic and open portoenterostomy groups, at 79.3% and 68.2%, respectively. Native liver survival rates were >80% for both groups for the first half year postsurgery, suggesting similar efficacy in maintaining the native liver. No intraoperative complications were reported, and readmissions for cholangitis were similar between groups.

The application of the da Vinci Surgical System (Intuitive Surgical, Sunnyvale, CA) is progressively extending robotic surgery from adult patients to infants, proving beneficial in various pediatric cases including hepatobiliary diseases such as choledochal cysts and increasingly complex procedures like portoenterostomy. This robotic platform effectively overcomes many limitations encountered in laparoscopic surgery. Notably, tasks such as anastomotic suturing and knot tying are substantially simpler compared to their laparoscopic counterparts, primarily due to the increased comfort experienced by the operating surgeon without ergonomic constraints hindering performance. In 2007, Dutta et al. reported the successful implementation of robotic portoenterostomy in three cases, while Meehan et al. shared their experience with two cases, demonstrating the feasibility of this approach. ^, A recent study reported consistently successful outcomes, characterized by the absence of intraoperative complications, minimal blood loss, and favorable postoperative recovery. There was significant jaundice resolution in most patients and encouraging survival rates with the native liver at 1 year. Technical enhancements such as bipolar coagulation for portal vein tributaries and precise anastomosis substantially contribute to its efficacy. Despite its promising results, robotic portoenterostomy does present certain limitations, such as cosmetic concerns and the associated high equipment costs; additionally, the bulky size of the robotic system may result in collisions within the limited operating space. Further investigation, including prospective comparative studies and long-term follow-up, is imperative to validate its efficacy and ascertain its position in the treatment paradigm for BA.

The role of corticosteroids is controversial, but we strongly believe they are choleretic and decrease inflammation and scarring at the anastomotic site. Davenport et al. performed the first randomized, placebo-controlled, double-blinded study using a low-dose 6-week course of oral prednisolone starting at 2 mg/kg/day in BA infants at two centers in the UK. There was a significant reduction in bilirubin levels at 1 month, which was more obvious in those who had portoenterostomy before the age of 70 days, but there was no significant effect on clearance of jaundice at 6 months (47% vs. 49%) or subsequent native liver survival between steroids and placebo groups. They published a follow-up study in 2013, including 44 additional patients treated with high-dose prednisolone starting at 5 mg/kg/day, and found that there was a significant difference in jaundice clearance rates between both low- and high-dose corticosteroid groups and a placebo group (52% vs. 66%, P = .037). However, there was no significant improvement in native liver survival (56% vs. 48%) when comparing the high-dose group to placebo.

The North American Steroids in biliary Atresia Randomized Trial (START) reported that there was a nonsignificant improvement in jaundice clearance at 6 months after hepatoportoenterostomy between both steroid groups and a placebo group (59% vs. 49%), which was higher in the subgroup coming to portoenterostomy at less than 70 days (72% vs. 57%). The START trial reported serious adverse events and did not find an overall difference in the number of events in the steroid compared to the control group but did find that adverse events occurred earlier.

The Japanese Biliary Atresia Society conducted a multiple center randomized trial comparing high-dose and low-dose corticosteroid administration and found that bilirubin was significantly lower in the high-dose group at both 1 and 2 months postoperatively, although long-term outcomes were not reported.

At Juntendo, we have experienced no adverse effects of corticosteroid administration, and this was supported by Davenport’s study.

Intravenous administration of double agent broad-spectrum antibiotics (usually a cephalosporin and an aminoglycoside) is commenced at the start of surgery and continued postoperatively until C-reactive protein (CRP) is less than 0.3 mg/dL or leukocytosis has resolved. Oral antibiotics may be administered prophylactically according to the surgeon’s preference in the absence of cholangitis.

In general, the authors continue intravenous fluids and nasogastric aspiration until bowel activity can be confirmed, usually 3–4 days postoperatively. ^, Careful monitoring of blood glucose levels, electrolytes, and coagulation is important in the early postoperative period. Blood tests, including complete blood count, LFTs, and ChE (serum cholinesterase) are assessed routinely for the first 7 days postoperatively, and then as required. Liver biochemistry (including bilirubin) may well worsen in the first postoperative week, whatever the eventual outcome, and should not be seen as discouraging.

At Juntendo, our standard postoperative protocol includes administration of both cholagogues and corticosteroids. An intravenous cholagogue (usually dehydrocholic acid) is commenced on day 2 after portoenterostomy and continued until jaundice clearance is confirmed (total bilirubin ≤1.5 mg/dL). Oral cholagogues such as ursodeoxycholic acid or aminoethanesulfonic acid are administered once oral feeding is commenced, generally on day 5 after operation, and continued thereafter. Once CRP falls below 1.0 mg/dL, a decreasing dose regimen of prednisolone is begun intravenously. The starting dose is 4 mg/kg/day administered for 3 days, followed by 3, 2, 1, and 0.5 mg/kg/day, each for 3 days. This 15-day cycle can be repeated up to four or five times if there is evidence of clinical benefit such as decrease in total bilirubin or darkening of stools. By about the fourth postoperative week there should be a definite fall in bilirubin and consistently pigmented stools in those who will do well. During the 4 weeks after portoenterostomy, daily monitoring of stool color and CRP is important, because if pigmented stools become pale or CRP increases, the antibiotic regimen may need to be revised. Preventing inflammation at the portoenterostomy site during the first 4 weeks after portoenterostomy is crucial to enable bilioenteric fistulae to develop.

Strict attention to nutritional needs is important, and all postoperative cases require fat-soluble vitamin supplementation that may need to be aggressive in some cases, especially with regard to vitamin K. Medium-chain triglyceride formula milk is advocated to maximize calorie input and facilitate lipid absorption, as this milk is processed by the portal vein and is readily available as a source of cellular energy.

An important feature of the authors’ protocol is that if stools begin to turn pale, the corticosteroid cycle is either recommenced from the beginning or the previous dose is readministered, depending on clinical circumstances. The antibiotic regimen is also usually revised. Should jaundice persist (total bilirubin >1.5 mg/dL) without evidence of apparent clinical benefit, only three cycles of corticosteroids are administered, and the patient is actively considered for liver transplantation. In addition, a recent study at Juntendo explored the effectiveness of using stool color as a monitoring tool for administering prednisolone in patients with BA after undergoing laparoscopic portoenterostomy. It involved 47 patients who received a decreasing dose from our previously described prednisolone protocol. The progression of the prednisolone course was contingent on the observation of normal stools, repeated until jaundice clearance was achieved. An abnormal stool color observed over two consecutive courses prompted considerations for either redo surgery or liver transplantation. The study reported that jaundice clearance was accomplished in 80.9% of the initial cases, with an overall jaundice clearance rate of 89.4% when including redo procedures.

The outcomes varied based on the number of prednisolone courses administered, with jaundice clearance rates ranging from 80% after one course to 100% after five or more courses. The median total dose of prednisolone also increased with the number of courses, from 30.0 mg/kg for one course to 308 mg/kg for 10 courses. These findings indicate that monitoring stool color to guide the administration of prednisolone and the timing for potential redo surgery or liver transplant is an effective strategy. The high jaundice clearance ratio and successful outcomes of redo surgeries or liver transplant, as observed in this cohort, support the utility of stool color as a reliable indicator for managing BA postlaparoscopic portoenterostomy, potentially improving patient outcomes by optimizing the treatment protocol based on individual responses.

Postoperative cholangitis is a serious setback. It can occur in the early postoperative period, especially before bilioenteric fistulae have developed, and it should not be undertreated. Cholangitis is defined as elevated serum bilirubin (>2.5 mg/dL), leukocytosis, and a change from normal to acholic stools in a febrile patient (>38.5°C). It must be treated by intravenous antibiotics immediately, according to each center’s protocol. Once resolved, prophylactic antibiotics such as sulfamethoxazole/trimethoprim should be administered orally.

Cholangitis is the most common complication after hepatic portoenterostomy, usually seen during the first 2 years after operation and occurring in approximately 40% of infants. Patients with cholangitis present initially with fever, decreased quantity and quality of bile excretion, elevation in serum bilirubin, and signs of infection. Prompt treatment is necessary to prevent liver damage because recurrent attacks cause progressive liver damage. After initial blood cultures, broad-spectrum antibiotics with good Gram-negative coverage should be started and improvement should be rapid. If stools should remain acholic or revert to acholic, a pulse of corticosteroids should be trialed.

It is important to note that cholangitis may also be late-onset and precipitate liver failure, necessitating emergency liver transplantation. Late-onset cholangitis can be fatal even in long-term survivors, which is of particular concern in Japan where the availability of cadaveric donors is limited. Moreover, living donors (parents of siblings) may be inappropriate for long-term survivors because of advanced age or poor health. ^,

Cholestasis is the main risk factor for cholangitis, because bile ducts in patients with BA are very small and all conduits should be considered to have become colonized by potentially pathogenic biota within a month of surgery. To decrease the risk for cholangitis, Roux-en-Y biliary reconstruction has been modified by various maneuvers, including lengthening the Roux-en-Y limb from 50 to 70 cm, total diversion of the biliary conduit, creation of an intestinal valve, and the use of a physiologic intestinal valve. The antireflux intussusception valve is probably the modification most likely to result in a reduced rate of cholangitis. In one study, adding a Roux-en-Y anastomosis with a spur valve achieved a high jaundice clearance rate and significantly lowered the number of cholangitis episodes 1 year after surgery. A gallbladder conduit is not recommended when the lumen of the patent duct is narrow or when pancreaticobiliary anomalies are demonstrated on cholangiography. Stomas should be avoided to prevent complicating liver transplantation, which may be required later.

Prophylactic long-term oral antibiotics have been advocated to reduce the recurrence of cholangitis but evidence for their efficacy remains scant, with only one randomized controlled trial in a much younger age group.

Recurrent cholangitis significantly diminishes native liver survival rates in BA patients postportoenterostomy, emphasizing the necessity of prompt and effective cholangitis treatment. Conversely, successful early bile drainage postportoenterostomy is associated with improved native liver survival rates in patients without cholangitis, suggesting its predictive value for long-term outcomes. These findings underscore the importance of comprehensive management strategies to optimize native liver survival in BA patients post-Kasai. Interestingly, there is little information regarding diagnosing cholangitis after Kasai, with no clear guidelines for defining the disease. The Tokyo Guidelines, developed for adults, are not suitable for diagnosing cholangitis in children during the first year after Kasai. Hence, a Delphi process with an international expert panel was held to establish definitions, treatment, and prophylaxis guidelines for cholangitis in the first year after Kasai. That group identified a series of four clinical elements ( List A ), which included fever and/or shivering, stool color change, new/increasing jaundice, and abdominal discomfort. Four laboratory and imaging elements were defined ( List B) : inflammatory response, increased/increasing transaminases, increased/increasing GGT or bilirubin, and the presence of bile lakes. Suspected cholangitis consists of one item from List A and one item from List B. Treatment for suspected cholangitis was recommended for 10–14 days. Confirmed cholangitis includes two items from List A and two items from List B and had a recommended treatment duration of 14–21 days. The choice of antibiotics for treating cholangitis can vary depending on the specific patient’s condition, local antimicrobial resistance patterns, hospital’s protocol, and any known or suspected pathogens. However, some commonly used antibiotics for the treatment of cholangitis include ceftriaxone, piperacillin-tazobactam, ampicillin-sulbactam, ciprofloxacin and metronidazole, gentamicin, and meropenem.