CHAPTER 23 Biliary Atresia
Step 1: Surgical Anatomy
♦ Biliary atresia is thought to be the result of an inflammatory process that destroys the bile ducts, causing fibrous obliteration of the intrahepatic and extrahepatic bile ducts and consequent biliary cirrhosis.
♦ Biliary atresia has been classified by subtype based on macroscopic and cholangiographic findings (Fig. 23-1):
Step 2: Preoperative Considerations
♦ The cardinal signs and symptoms are jaundice within weeks after birth and the development of acholic stools, dark urine, and hepatomegaly. Any newborn with persistent jaundice after 4 weeks of age should be aggressively investigated. The clinical examination is typically normal in early presentations, and an enlarged liver is often appreciated by 6 to 8 weeks of age.
♦ Laboratory examination demonstrating direct (conjugated) hyperbilirubinemia is the hallmark of biliary atresia. This should never be confused with physiologic jaundice of the newborn, which shows indirect (unconjugated) hyperbilirubinemia. Because the cause of pathologic jaundice in an infant is diverse, biochemical evaluation must include hepatitis serologies, TORCH (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex) titers, α1-antitrypsin level, serum ferritin, and a sweat chloride test or direct genetic analysis to rule out cystic fibrosis.
♦ Abdominal ultrasound is useful in the evaluation of cholestatic jaundice. Absence of the gallbladder or identification of a small, shrunken, noncontractile gallbladder is suggestive of biliary atresia. Occasionally the fibrous cord remnant of the biliary ducts can be identified as the “triangular cord” sign (Fig. 23-2, portal vein and hepatic artery with echogenic cord in expected region of bile duct).
♦ Liver biopsy is considered the most reliable preoperative test in the diagnosis of biliary atresia. Histologic findings consistent with biliary atresia include preservation of basic hepatic architecture (Fig. 23-3, A); bile ductular proliferation (Fig. 23-3, B); bile duct plugs (Fig. 23-3, C); inflammatory cell infiltrate, hepatocyte giant cell transformation, and edema and fibrosis of the portal tract (Fig. 23-3, D). Uncorrected biliary atresia results in histologic evidence of cirrhosis as early as 3 to 4 months of age (Fig. 23-4).
Step 3: Operative Steps
Anesthetic Induction
Incision
♦ A short subcostal incision (Fig. 23-5, solid line) overlying the gallbladder region of the liver will allow initial investigation and cholangiography. If the diagnostic phase of the operation confirms biliary atresia, we prefer to extend this incision to a full subcostal incision to allow for mobilization of the liver.
♦ The suspensory ligaments of the liver, the coronary and falciform ligaments, are divided to mobilize the liver in the abdomen. The liver is then externalized and rotated to allow clear and unobstructed observation of the hilum and the vascular structures. We find that this exposure facilitates safe dissection of the lateral fibrous mass regions, and it does not significantly complicate liver transplantation if needed in the future.
Operative Procedure Step 1: Diagnostic Confirmation
♦ The liver in biliary atresia is characteristically firm, dark, and cholestatic in color, and it typically has many subcapsular telangiectatic vessels on its surface, representing early cirrhosis and portal hypertension changes (Fig. 23-6). Metabolic liver diseases rarely demonstrate these features.
♦ Intraoperative cholangiography is performed by placing a 20-gauge intravenous catheter into the gallbladder remnant under direct vision and securing it with a purse-string suture.
Full-strength contrast is infused under fluoroscopic guidance, taking care not to extravasate the contrast through forceful or unobserved injection.
No passage of contrast into the liver confirms the diagnosis of biliary atresia (Fig. 23-7). Intrahepatic flow into anatomically correct ducts, even when they are small, is inconsistent with biliary atresia, and Kasai portoenterostomy is not indicated in such scenarios.
In cases of diminutive size of the distal bile duct resulting in increased resistance to bile flow (see Fig. 23-7), we would elect standard Roux-en-Y portoenterostomy. In selected cases in which distal duct patency exists and low-resistance bile outflow is demonstrated by a normal-sized distal bile duct, a “gallbladder Kasai” can be used as an alternative to the conventional Roux-en-Y reconstruction.
Full-strength contrast is infused under fluoroscopic guidance, taking care not to extravasate the contrast through forceful or unobserved injection.
No passage of contrast into the liver confirms the diagnosis of biliary atresia (Fig. 23-7). Intrahepatic flow into anatomically correct ducts, even when they are small, is inconsistent with biliary atresia, and Kasai portoenterostomy is not indicated in such scenarios. In cases of diminutive size of the distal bile duct resulting in increased resistance to bile flow (see Fig. 23-7), we would elect standard Roux-en-Y portoenterostomy. In selected cases in which distal duct patency exists and low-resistance bile outflow is demonstrated by a normal-sized distal bile duct, a “gallbladder Kasai” can be used as an alternative to the conventional Roux-en-Y reconstruction.
Operative Procedure Step 2: Kasai Portoenterostomy
♦ The initial dissection of the fibrous remnant is begun just below the level of the cystic duct junction with the fibrous mass. We prefer to mobilize the gallbladder from its bed before beginning the dissection of the fibrous mass. Superior dissection of the fibrous tissue proceeds just anterior to the portal vein and hepatic artery.
♦ As the dissection of the fibrous remnant ascends into the area within the portal vein bifurcation, it is important to recognize that the direction of the dissection proceeds in a posterior direction within the bifurcation of the portal vein (Fig. 23-8). Ligation of small communicating portal branches within this bifurcation is often necessary.
♦ Dissection should proceed until the fibrous remnant has come into approximation with the capsular surface of the liver within the bifurcation of the portal vein. The lateral extent of dissection on the left side is the umbilical fissure and the obliterated umbilical vein insertion into the left portal vein. On the right side, the lateral extent of dissection is to the bifurcation of the right portal vein into its anterior and posterior branches (Fig. 23-9, arrows indicate fibrous mass before division, with yellow vessel loops marking the lateral dissection margins; Fig. 23-10, dashed line represents dissection margin of fibrous remnant mass at the liver surface).
♦ The lateral dissection margins and the central hilar mass are the areas containing the remnant biliary ductules required for drainage (Fig. 23-11, remnant biliary ductules are present in region within dashed line).
♦ Division of the fibrous mass is then performed. The plane of division should be at the level of the surface of the liver capsule (see Fig. 23-8, plane of division marked with “x”). There is no advantage to deep dissection (“taking a core”) at the location of the fibrous mass.
♦ Hemostasis at the site of the divided fibrous mass is best attained with pressure and warm compresses to the surface. Small bile ductules travel with the microvascular structures, and coagulation risks the possibility of ductal injury.
♦ While awaiting hemostasis at the site of the divided fibrous mass, we proceed with construction of the Roux-en-Y jejunal limb. Using a convenient but proximal portion of jejunum, we fashion an isoperistaltic retrocolic Roux-en-Y limb measuring 35 to 40 cm in length. The jejunojejunostomy is fashioned end-to-side with several stitches at the junction of the inflow jejunum and the Roux limb to prevent reflux of contents into the Roux limb itself (Fig. 23-12).
♦ The anastomosis of the Roux limb to the divided fibrous remnant site is performed using the antimesenteric side of the intestine. Care is taken to avoid leaving a significant blind segment of Roux limb between its closed end and the portoenterostomy anastomosis, thereby avoiding a poorly emptying pouch, which is prone to stasis and bacterial overgrowth. Some surgeons prefer to use the opened end of the jejunum for this anastomosis, and this is also acceptable when the size match is appropriate.
♦ The portoenterostomy anastomosis is undertaken by securing the posterior wall first. We prefer to use interrupted 5-0 or 6-0 absorbable monofilament sutures for this wall of the anastomosis unless optimal visualization allows a carefully constructed running suture line. The suture line should “invaginate” itself around the vascular elements, taking care to avoid incorporation of the portal vein into the anastomosis. Exposure of the vein wall to biliary secretions could contribute to portal vein stenosis or occlusion frequently seen in infants with late biliary atresia at the time of transplantation.
♦ It is critical to place the sutures into the hepatic parenchyma just outside of the fibrous remnant margin. Sutures should incorporate the capsule of the liver but not deeply transgress the parenchyma to avoid occluding superficial ductal remnants. The anterior wall of the anastomosis can then be closed using a running suture technique (Fig. 23-13).
Closing
Step 4: Postoperative Care
♦ To reduce the risk of postoperative cholangitis, intravenous antibiotics are continued until bowel function has returned and a diet has been initiated. Thereafter oral antibiotics (trimethoprim-sulfamethoxazole) are used for long-term (6 months) prophylaxis. Use of prolonged intravenous antibiotic courses has been advocated by some centers to prevent early cholangitis.
♦ Many centers administer methylprednisolone during the immediate postoperative period for its anti-inflammatory and choleretic effects. Intravenous methylprednisolone is transitioned to oral prednisone, tapering to discontinuation. The duration of treatment in noncontrolled studies spans from several days to longer than 2 to 3 weeks. Doses also vary widely. The recent King’s College randomized, controlled trial showed no benefit when used for short duration at low doses. A randomized, controlled trial of a higher-dose, longer-duration steroid regimen is in progress in the United States (BARC protocol).
Step 5: Pearls and Pitfalls
♦ Cholangitis is the most frequent complication that occurs following Kasai portoenterostomy, affecting nearly 50% of patients. It most commonly occurs within 2 years after operation and manifests as fever, jaundice, and decreasing color of the stools. Blood cultures may be positive, and early institution of broad-spectrum intravenous antibiotics is imperative. The earliest use of intravenous steroids following biliary atresia surgery was for the treatment of cholangitis, with the hope of decreasing the injury caused by the inflammatory process. No studies have confirmed the efficacy of this practice, although it continues in many centers.
♦ In patients who have had active bile drainage following Kasai portoenterostomy with resultant anicteric state, abrupt cessation of bile excretion is manifest by loss of stool bile pigment. Cholangitis must be excluded. In patients who had good bile flow following initial Kasai procedure (bilirubin < 2 mg/dL), reoperation may be considered if treatment of presumed cholangitis fails to re-establish bile drainage. Reoperation is not helpful in circumstances where the initial operation was felt to be correctly undertaken but the bilirubin has failed to return to a level below 2 mg/dL.
♦ Liver transplantation is the only option for long-term survival in patients who fail to establish active bile drainage following Kasai portoenterostomy. Even in patients who have a successful Kasai procedure, progression of hepatic fibrosis and cirrhosis often occurs. Such patients manifest sequelae of portal hypertension, including ascites, hypersplenism, and variceal hemorrhage. Patients with worsening liver function (e.g., hyperbilirubinemia, coagulopathy, hypoalbuminemia) are considered for liver transplantation. Numerous long-term studies have shown that liver transplantation can be anticipated in up to 85% of biliary atresia patients by age 20. Fully half of these patients will require transplantation before their second birthday because of rapid progression of liver failure.
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