Laparoscopic Kasai Procedure of Treating Biliary Atresia with an Aberrant Right Hepatic Artery

Introduction

Biliary atresia (BA) is a severe anomaly of the newborn that causes persistent conjugated jaundice; and, if left untreated could cause cirrhosis and finally end-stage liver disease within the first year of life in most of the cases. ¹ In most of the patients (>90%) the obstruction is at the level of the porta hepatis (type III). ²

Although nowadays liver transplantation had greatly improved the prognosis of some cases with BA, the Kasai procedure is still regarded as the standard primary therapy especially for the young infants, and laparoscopic Kasai procedure had been gradually accepted by more and more pediatric surgeons. ³

Ji et al. ⁴ reported the learning curve of laparoscopic Kasai portoenterostomy for BA. Hussain et al. ⁵ reported the postoperative recovery of the laparoscopic Kasai group was more favorable than the laparotomy group. Sun et al. ⁶ compared their laparoscopic and conventional Kasai portoenterostomy in children with BA and concluded that the laparoscopic Kasai procedure did not improve the prognosis. Ure et al. ⁷ reported that among the laparoscopic group of 12 infants, the clearance of jaundice rate at 6 months after surgery was only 17% and then abandoned the procedure.

Our team had reported modified laparoscopic Kasai with favorable results, ⁸ but currently it still remains a challenge in pediatric surgery because of the complexity and even sometimes the uncontrollable bleeding.

Laparoscopic repositioning of an aberrant right hepatic artery (ARHA) and hepaticojejunostomy for congenital choledochal cyst had been reported occasionally, and some scholars advocated the ARHA crossing the anterior aspect of the common bile duct is a relatively frequent abnormality. ⁹ It could interfere with the dissection of the choledochal cyst and make the operation more difficult.

BA with an ARHA is very rare and can lead to a significantly increased difficulty in the Kasai procedure, particularly in the laparoscopic Kasai. Ten cases of BA with an ARHA were diagnosed and treated using the laparoscopic approach in our hospital. This report is to clarify the unique technique, as well as review the author's personal experience, and demonstrate the feasibility of this laparoscopic procedure for the treatment of infants.

Materials and Methods

Totally 135 patients with biliary were treated by laparoscopic procedure at our department during January 2012 and August 2021, among all the patients, 10 cases were diagnosed with BA associated an ARHA and laparoscopic Kasai was performed successfully. The initial stools were considered pigmented by the parents after birth. Over time, stools took on a lighter color gradually and become acholic when admitted. The medical records of the 10 patients were retrospectively reviewed.

The operations were carried out by the same surgical team. The technical options were determined by the senior surgeon B.L. Ethical approval of the laparoscopic Kasai from the Ethics Committee of Huai'an Children's Hospital (Jiangsu, China) was obtained. The written informed consents were obtained from the parents of all the BA infants before the entry into this study.

The age and the sex of the cases, the bilirubin level before the operation, the operating time, and blood loss were retrospectively reviewed. The early clearance of jaundice was considered as the total bilirubin <20 μmol/L within 6 months postoperatively. The native liver survival was defined at 6 months to 2 years after the laparoscopic Kasai.

Surgical Technique of Laparoscopic Kasai

Laparoscopic Kasai portoenterostomy is one of the most challenging operations and was first presented by Esteves et al. in 2002. ¹⁰ The laparoscopic Kasai for BA had been previously well described based on the primary Kasai procedure, and sometimes with mild variations between different pediatric centers. A nasogastric tube and a Foley urinary catheter were navigated to decompress the stomach and bladder respectively. After general endotracheal anesthesia was administered, the patient was placed on the operating table in a supine position, the surgeon was at the right side of the infant, an assistant and the camera man stood on the left side, and the display was placed at the patient's head. The operation was carried out using four trocars: the first 5-mm camera port was fixed at the umbilicus by an open technique. The peritoneal cavity was insufflated with CO₂ at a pressure of 8–10 mm Hg and insufflation flow of CO₂ was 3–4 L/min.

The second 5 mm port was at the anterior axillary line of the right upper quadrant of the abdomen. Under the laparoscopic guidance, the gallbladder was exteriorized through the second port site, the fundus of the gallbladder was incised, and then an operative cholangiogram was performed to confirm type III BA and the patency of the extrahepatic bile duct. Another two 3-mm trocars were at the left upper and right lower abdomen, respectively, just lateral to the edge of the rectus abdominis muscle.

A percutaneous suture was introduced just below the xiphoid process to snare the round ligament and elevate the liver. The atretic gallbladder is dissected from the gallbladder fossa and then a percutaneous stay suture was introduced to the remnant of the fundus to help retract the liver. The peritoneum before the hepatoduodenal ligament was incised to expose the proper hepatic artery, then the left hepatic artery, the right hepatic artery, and the middle hepatic artery. The middle hepatic artery was dissected, ligated and cut off if interrupted the fibrous cord mobilization. With the blunt dissection, the edge of the left branch of the portal vein could be exposed.

With the meticulous dissection, an ARHA anterior to the common bile duct was detected (Fig. 1). The common bile duct was isolated; the distal part was ligated and then cut off. The gallbladder was resected near the common bile duct (Figs. 1 and 2). Kept on mobilizing the common bile duct proximally, separating the connection with the right hepatic artery and the right branch of portal vein completely. The mobilization must be careful to avoid the chance of injury to the portal vein and the right hepatic artery and ensure a safe operation. After the complete mobilization, the common bile duct was pulled up from the gap between the right hepatic artery and the right branch of the portal vein to the hepatic hilum (Fig. 3).

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FIG. 1.

An ARHA anterior to the common bile duct was detected. ARHA, aberrant right hepatic artery.

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FIG. 2.

The common bile duct was behind the right hepatic artery.

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FIG. 3.

The common bile duct was pulled up from the gap between the right hepatic artery and the right branch of the portal vein to the hepatic hilum.

As the common bile duct was lifted up to the hepatic portal, the right branch of the portal vein would be exposed. Kept on retracting the remnant of the common bile duct, the fibrous tissue was separated from the portal vein by blunt and sharp dissection. Several venous tributaries at the porta plate from the left and right branches of the portal vein were isolated and dissected with the hook cautery to avoid the heat injury of the small bile ducts. Once the base of the fibrous cone beyond the bifurcation of the portal vein was reached, the fibrous cone could be transected meticulously with laparoscopic scissors.

The ligament of Treitz was identified, pneumoperitoneum was released, the jejunum about 10 cm distal of ligament was exteriorized through the slightly enlarged umbilical port incision, and the jejunojejunostomy was carried out to create a Roux-en-Y jejunal loop extracorporeally. The jejunum was reintroduced into the peritoneal cavity thereafter, and brought to the porta hilar by a retrocolic manner. Finally, the laparoscopic portoenterostomy was performed by using 2 PDS 5/0 running sutures, the posterior wall of the anastomosis was at the back of the portal vein. The mesenteric openings were closed when they were too big. But when the mesenteric openings were just fit the intestine, the openings did not need to be closed.

Abdominal drain was not used after operation. Cephalosporins were administered intravenously after surgery for 1 month before being given oral cotrimoxazole and cefminox until 6 months. Ursodeoxycholic acid and prednisolone were also used 1 week postoperatively until the laboratory results showed that total bilirubin was at normal levels (<20 μmol/L).

Results

Ten cases were diagnosed with BA associated an ARHA treated among all the 135 patients with biliary (7.41%). Among the 10 cases, 6 were male and 4 female. The mean age was 76.2 days (range 60–135 days) when admitted. The mean body weight was 5.4 kg (range 4.0–7.5 kg). All the patients survived the laparoscopic Kasai without any intraoperative complications. The mean operative time was 235 minutes (range 210–260 minutes) for each laparoscopic Kasai. The mean blood loss was 12.8 mL (range 8–20 mL), and no uncontrollable bleeding during the surgery. The resumption of oral intake was 4.1 days (range 3–6 days) after surgery. The mean postoperative hospital stay was 15.3 days (range 14–18 days). The mean follow-up time was 32.6 months (range 6–60 months).

Nine of the patients had pigmented stools within the first 7–14 days after surgery, but 1 patient had acholic stool again because repeated cholangitis. The total and direct bilirubin dropped to normal (the total bilirubin <20 μmol/L) within 6 months of surgery in 7 patients. One patient died of repeated cholangitis and liver failure 1 year after surgery. In the additional 2 patients the bilirubin levels dropped significantly after the surgery but elevated again because of repeated cholangitis. The 2 patients of 6 and 7 months after surgery, respectively, are still alive and requiring ongoing observation and intermittent treatment with antibiotics, ursodeoxycholic acid, and prednisolone. Age at operation, operative time, follow-up, and outcomes of the patients are presented in Table 1.

Discussion

Kasai portoenterostomy had revolutionized the surgical management of BA after it was introduced by Professor Kasai in 1959, and it has become the mainstay of treatment for BA since then. ¹¹ As instrumentation and techniques advances, laparoscopic Kasai has been also used in BA cases. ¹² However, the technical complexity limited the skill popularization, so nowadays many BA cases still cannot benefit from laparoscopic Kasai, and we are the only center currently to perform laparoscopic Kasai in Jiangsu province, China.

Our team specializes in minimally invasive surgery for children; we began to perform laparoscopic Kasai since 2012.^2,8 Actually, thanks to magnified vision of laparoscopic approach, a good visualization of portal structures could be obtained. To make laparoscopic Kasai technically feasible and safe, the surgeons must be familiar with the original concepts of the Kasai procedure and the anatomy of hepatic hilum, especially the portal vein and the hepatic arteries. Mastering the anatomy of the hepatic vessels is the cornerstone of laparoscopic Kasai. But sometimes the variation of the hepatic vessels can become a trap of the surgery. So laparoscopic Kasai is still technically challenging even for an experienced pediatric surgeon.

There are many anatomical variations in the hepatic vascular in clinic; an ARHA is a relatively frequent anomaly.^12–15 This anomaly had been reported in patients with jaundice occasionally, particularly in congenital choledochal cyst when laparoscopic choledochal cyst resection and hepaticojejunostomy were performed. This ARHA not only interfered with the mobilization of the common bile duct for choledochal cyst but could also induce postoperative anastomotic stenosis. Because the ARHA could lead to compression of the bile ducts with subsequent jaundice, this anomaly was also called right hepatic artery syndrome. ¹⁶

Todani et al. ¹⁷ first reported the technique of placing the ARHA behind the choledochal cyst to restore a normal anatomy. Diao et al.^18,19 reported the cases in which the ARHA was replaced dorsally during laparoscopic redo hepaticojejunostomy, and they also found this malformation was a primary cause of anastomotic stenosis after hepaticojejunostomy.

Masuya et al. ²⁰ reported laparoscopic repositioning of an ARHA for pediatric choledochal cyst, in their case, abdominal enhanced CT showed the ARHA crossed at the ventral side of the dilated common hepatic duct. The judgment concerning the need to reposition the ARHA is important for laparoscopic choledochal cyst resection and hepaticojejunostomy, and the decision to make must be based on intraoperative findings.

In the cases of BA, the common bile duct was not dilated; even the abdominal enhanced CT scan could not reveal the decompression of the common bile duct. So it is almost impossible to make an accurate diagnosis of ARHA in the BA patients before surgery. Actually all the diagnosis of ARHA in our BA cases was based on intraoperative findings. In my knowledge BA associated with an ARHA and the technique of laparoscopic Kasai for the group had not been reported in English literature before.

Once the ARHA was detected, the surgeon must determine the strategy to accomplish the laparoscopic Kasai. How to dissect the common bile duct from the hepatic vessels and then avoid uncontrollable intraoperative bleeding was the key to the success of laparoscopic Kasai.

If the surgeons wanted to mobilize the fibrous tissue without dissecting the common bile duct between the right hepatic artery and the portal vein, the right branch of the portal vein was covered by the proximal part of the common bile duct; several branches from the portal vein to the caudate lobe could not be thoroughly isolated and cauterized. The mobilization of the fibro tissue would be very difficult and uncontrollable intraoperative bleeding might be met possibly when the fibrous mass transection was performed. If the surgeons chose to cut the proximal part of the common bile duct near the fibrous tissue directly above the portal vein, the maneuver might result in accidental injury to the portal vein and the right hepatic artery, or laparoscopic surgery conversion, even operation failure.

The oldest patient among the group is female, aged 135 days, and the operating time tops them all (260 minutes). The edema in the hepatic hilum and hepatoduodenal ligament was more serious than other cases, resulted in significantly increased difficulty, so the laparoscopic surgery for this case needed to be more meticulous to avoid excessive bleeding. After that we suggested the laparoscopic Kasai is not recommended in BA patients >4 months.

Our experience demonstrated laparoscopic Kasai could be successfully performed in BA patients, even the case associated with an ARHA. In view of the high complexity of the surgery, the laparoscopic procedure should only be performed by an experienced senior surgeon in BA to increase the chances for successful results.