The open PE requires a generous laparotomy to access two or more quadrants to permit mobilization of the bilateral triangular hepatic ligaments to prolapse the liver anterior to the abdominal wall while using the corners of the incision as retractors. Excision of the remnant gallbladder and bile duct as well as fibrous plate at the porta hepatis is performed, followed by Roux-en-Y reconstruction for bilioenteric drainage via the portoenterostomy. The key of the procedure is dissection of the cone-shaped fibrous remnant at the level of the liver surface, just anterior to the portal vein bifurcation. For best long-term results, the bilious scar tissue must be removed directly on the liver surface without injuring the actual liver. The dissection must be carried as much lateral as possible with typical limitations of right and left portal venous and hepatic artery branches as well as below the portal vein bifurcation. The completely exposed liver surface at the porta hepatis is then covered by the Roux limb as a quasi-“sewer” to catch dripping bile from the area.

The infant is positioned at the foot of the operating table on a forced-air warming blanket, followed by placement of a nasogastric tube and urinary catheter. The surgeon is positioned at the patient’s feet, with an assistant to the surgeon’s right. A 12-mm trocar is placed either supraumbilically or transumbilically using a Veress needle and Step™ dilating sheath (Medtronic Minimally Invasive Therapies, Minneapolis, MN) or via open placement with Hasson technique. A 30° 5-mm laparoscope is used to inspect the peritoneal contents and to facilitate placement of three additional 5-mm radially dilating trocars under laparoscopic vision: two ports are placed, each on either side of the umbilical port, for the surgeon’s right and left hands, slightly above the umbilical level, just lateral to the rectus abdominis. The third 5-mm port is placed between the umbilical port and the left upper quadrant port, slightly below the umbilicus for use by the assistant (Fig. 42.2).

Liver retraction provides critical exposure of the porta hepatis, and various means have been described to attain adequate visualization. The transabdominal placement of a subxiphoid retention suture allows the surgeon to suspend the liver by the falciform ligament, and additional sutures can then be placed to retract the right and left lobes. Alternately, a 5-mm Nathanson retractor (Mediflex Surgical Products, Islandia, NY) may be placed through an epigastric incision through a dilating trocar sheath. The retractor is then affixed in place with a flexible table-mounted retractor clamp.

Monopolar electrocautery with a laparoscopic hook is then used in combination with blunt dissection with atraumatic graspers to expose the cystic duct and trace it to the biliary remnant. If cholangiography is indicated, the gallbladder may then be accessed as described in the section above. Liver core biopsies may also be obtained at this time, if needed. The biliary remnant is then divided at the level of the duodenum and elevated as dissection is carefully continued to skeletonize the distal remnant until the fibrous cone is visualized (Fig. 42.3). Dissection may be accomplished with monopolar cautery using hook or scissor, 5-mm ultrasonic shears (Ethicon, Somerville, NJ), depending on the surgeon preference. The JustRight Vessel Sealing System (JustRight Surgical, LLC, Louisville, CO), which utilizes a 3-mm instrument shaped like a Maryland dissector , can also be used. Excision of the fibrous cone (Fig. 42.4) exposes bile ductules, the microscopic structures that are the basis of biliary drainage following PE. It is therefore extremely important that thermal spread and related injury are minimized with the use of sharp dissection and judicious application from energy sources to control portal venous tributaries as they are encountered. Following completion of the distal remnant excision, the specimen is sent to pathology for histologic examination of the resected ductules and the PE is fashioned.

A pancreatico-enteric jejunal limb is measured out to 10–15 cm distal to the ligament of Treitz, and the bowel at this point is delivered per the umbilical incision following removal of the trocar. The bowel is then divided using a stapling device or electrocautery and oversewing of the distal enterotomy. The distal limb is then measured either intracorporeally or using the xiphoid as an external landmark to ensure that no tension will be imposed by the jejuno-jejunostomy. The standard length of the Roux limb is between 35 and 45 cm which is thought to prevent ascending cholangitis. The anastomosis is completed in hand-sewn end-to-side extracorporeal fashion (Fig. 42.5), and seromuscular tacking sutures are applied to affix the proximal limbs longitudinally to promote antegrade flow through the alimentary limb. A 1-cm longitudinal enterotomy is created using electrocautery on the antimesenteric aspect of the proximal Roux limb, and the bowel is inspected for perfusion and orientation prior to returning it into the abdomen. The 12-mm trocar is then replaced and laparoscopy resumes.

The transverse colon is elevated and a retrocolic mesenteric defect is carefully fashioned with electrocautery, through which the Roux limb is passed to lie at the porta hepatis. The PE is constructed using interrupted absorbable 5-0 monofilament suture, incorporating full-thickness bites of the bowel wall and small bites of hepatic parenchyma posterior to the portal plate along the back wall (Fig. 42.6). Anteriorly, suture placement should avoid any identifiable remnant of the left and right bile ducts to preserve these structures, which are typically found at two o’clock and ten o’clock. It is most important to extend the PE as far laterally as possible to include the highest possible number of bile ductules. The investing perivascular connective tissue of the hepatic arteries may be used to bolster closure at these positions [27]. Upon completion of the procedure, closed-suction drains may be placed at the porta hepatis and adjacent to the jejuno-jejunostomy using the existing 5-mm trocar sites prior to termination of laparoscopy. However, most operators avoid the postoperative use of drains and the risk of related complications may outweigh that of an occult anastomotic leak. The umbilical port site is closed in layers, and fascial defects at 5-mm trocar sites are separately closed at the surgeon’s discretion, taking into account the patient’s size and the presence of ascites.

Very limited surgeon experience has been reported with respect to a robotic approach to the Kasai operation. Two separate case series were reported in 2007. Dutta and colleagues describe three such cases, of which two were performed using the da Vinci robotic surgical system (Intuitive Surgical, Inc., Sunnyvale, CA) for portal dissection and PE, while a third was performed as a “hybrid procedure,” utilizing the robotic approach only for PE [28]. Meehan et al. present two robotic Kasai procedures in which the dissection and PE were performed with the da Vinci system without prior experience in laparoscopic Kasai [29]. Notably, both groups of investigators opted to perform construction of the Roux limb extracorporeally. Patient positioning, robot docking, movement arcs of wristed 5-mm instruments, instrument size proportional to an infant, and absent haptics feedback when manipulating the liver were cited as factors increasing the difficulty of robotic Kasai. Advantages described included improved endoscope image quality, three-dimensional visualization, surgeon comfort, movement downscaling, and degrees of freedom in movement for greater precision in instrument placement and manipulation. Little can be said regarding long-term outcomes in patient cohorts of this size, but no immediate major complications were reported, though one patient proceeded to transplant within the first postoperative year. Although it presents technical challenges and may not greatly improve the performance of the early stages of the operation, robotic-assisted laparoscopic PE may provide distinct advantages and merits further study.

Complications following PE include malabsorption of fat-soluble vitamins that may progress to overt malnutrition despite supplementation and growth retardation [30]. Cholangitis is a relatively common complication, occurring most frequently within months to a few years postoperatively. It is generally believed to result from reflux of enteric contents and bacteria up the Roux limb, although other mechanisms, such as bacterial translocation into the portal system and periportal lymphedema, have been suggested. The microorganisms in question are typically enteric flora [31], and repeated infections place the patient at risk for progressive cirrhosis. For this reason, perioperative corticosteroids and choleretics (ursodeoxycholic acid) [32] and routine prophylaxis with oral antibiotics [33] are used to decrease the frequency of such attacks. As mentioned above, some patients will not achieve adequate biliary drainage following PE, with progressive decline in liver function. Children that suffer recurrent jaundice and cholangitis that cannot be successfully medically managed are generally not helped by surgical revision [34–36], and most authors would refer such children for liver transplantation. Portal hypertension is also a common postoperative finding, and can evolve to ascites and variceal bleeding. Esophageal varices can be monitored and treated with flexible upper endoscopy. Endoscopic sclerotherapy can effectively address episodes of bleeding [37] or be undertaken prophylactically for asymptomatic lesions [38]. Other treatments for bleeding varices include endoscopic ligation [39] and placement of percutaneous transjugular intrahepatic [40] or surgical portosystemic shunts [41]. Transplantation is generally indicated for primary failure of PE, developmental failure secondary to progressive hepatic dysfunction, and recurrent episodes of cholangitis or complications of portal hypertension that are refractory to medical or endoscopic management. Five-year survival following liver transplantation for biliary atresia exceeds 90 % [42, 43].