Types of biliary atresia. From Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet 2009 Nov 14;374(9702):1704–1713. Reprinted with permission from Elsevier Limited.
In our institution, we advocate HIDA scan after completed medical workup. If non-secretion is confirmed, the patient is taken to the operating room for laparoscopic-guided percutaneous transhepatic or open cholangiogram and percutaneous liver biopsy. In cases of a micro-gallbladder, the cholangiogram is performed through a minilaparotomy. If biliary atresia is confirmed, a generous wedge liver biopsy is taken and a Kasai procedure is performed.
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.
In many situations, the preoperative workup does not yield a definitive diagnosis. In other instances, the gallbladder or bile ducts may appear morphologically normal (as in type II BA) or found to contain pigmented bile on laparoscopic examination and aspiration. Contrast images of the biliary tree may provide valuable evidence when faced with equivocal evidence . Confirmatory cholangiography can be performed laparoscopically in combination with open or laparoscopic PE under the same anesthetic.
The patient is positioned with the operating table level and trocars are placed as for laparoscopic Kasai (see below). Following inspection of the peritoneum and biliary structures, a purse-string suture is placed around the planned cholecystostomy either at the gallbladder infundibulum or at the fundus, depending on the degree of fibrotic change. If an initial percutaneous aspiration is attempted and yields clear fluid, further interrogation of the biliary tree is not indicated. Otherwise, the gallbladder is then opened using laparoscopic shears or the monopolar hook. An existing port site or percutaneous 14-gauge angiocath placed the right subcostal space can be used to deploy a cholangiogram catheter or rigid cannula, which is then navigated into the gallbladder lumen and secured with the purse-string suture. A second outer purse-string may be necessary following catheter placement if extravasation is noted with a test bolus of saline. Contrast is diluted to half-strength with normal saline and injection proceeds slowly under fluoroscopy. If the common bile duct is observed overlying the spine, rotation of the C-arm or the operating table can produce off-axis images. Trendelenburg positioning may also augment contrast delivery to the intrahepatic bile ducts if initial images are equivocal, and administration of intravenous glucagon may improve drainage of contrast into the duodenum if such flow is not initially evident . Alternatively, an angiocath may be guided percutaneously transhepatically under direct vision with the laparoscope into the gallbladder to prevent leakage of contrast into the peritoneum, obscuring the cholangiogram. If one is versed with abdominal ultrasound, this technique could be employed entirely percutaneously without laparoscopic guidance. In typical cases of BA with a micro-gallbladder, all minimally invasive techniques may be technically difficult, and unroofing of the gallbladder to reveal a drop of white bile may be diagnostic.
Since first described by Esteves et al. in 2002 , the laparoscopic approach to PE has been subject to significant discussion and refinement. During the 16th Meeting of the International Pediatric Endosurgery Group (IPEG) in 2007 in Argentina, several international presenters reported poor short- and middle-term results after the minimally invasive Kasai procedure, which led to the recommendation by that group to abandon the laparoscopic approach until further evidence could be gathered. Several high-volume centers, predominantly in Asia and Argentina, have continued and refined the minimally invasive approach to date and report good mid- and long-term results.
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).
Trocar positions during laparoscopic portoenterostomy. Note the 5-mm trocar in the epigastrium, specifically for the LigaSure device. Numbers indicate trocar size (mm). From Yamataka A, Lane GJ, Koga H, Cazares J, Nakamura H. Role of laparoscopy during surgery at the porta hepatis. S Afr Med J 2014;104(11):820–824. Doi:10.7196/SAMJ.8921.
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 bipolar dissector isolates the biliary remnant and divides portal vein branches at the porta hepatis draining into the caudate lobe (PV portal vein, BR biliary remnant, RHA right hepatic artery, LHA left hepatic artery). From Yamataka A, Lane GJ, Koga H, Cazares J, Nakamura H. Role of laparoscopy during surgery at the porta hepatis. S Afr Med J 2014;104(11):820–824. Doi:10.7196/SAMJ.8921.
Shallow transection of the biliary remnant being performed with laparoscopic shears. Adapted from Yamataka A. Laparoscopic Kasai portoenterostomy for biliary atresia. J Hepatobiliary Pancreat Sci 2013 Jun;20(5):481–486.
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.
Customizing the Roux-en-Y limb. (a) The jejunal loop is placed at the umbilicus and the distal end (E) of the limb is brought up to the xiphoid process. (b) The customized Roux-en-Y limb is approximated to the native jejunum for 8 cm cranially (triangles) to streamline flow into the distal jejunum (arrows), eliminate reflux into the Roux-en-Y limb, and prevent stasis in the Roux-en-Y limb. From Yamataka A, Lane GJ, Koga H, Cazares J, Nakamura H. Role of laparoscopy during surgery at the porta hepatis. S Afr Med J 2014;104(11):820–824. Doi:10.7196/SAMJ.8921.
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 . 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 shallow suture to the connective tissue at 2 o’clock and a shallow suture to the liver parenchyma at 4 o’clock. From Yamataka A. Laparoscopic Kasai portoenterostomy for biliary atresia. J Hepatobiliary Pancreat Sci 2013 Jun;20(5):481–486.
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 . 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 . 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 . 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 , and repeated infections place the patient at risk for progressive cirrhosis. For this reason, perioperative corticosteroids and choleretics (ursodeoxycholic acid)  and routine prophylaxis with oral antibiotics  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  or be undertaken prophylactically for asymptomatic lesions . Other treatments for bleeding varices include endoscopic ligation  and placement of percutaneous transjugular intrahepatic  or surgical portosystemic shunts . 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].