Overview

Our recent approach to perihilar cholangiocarcinoma has gradually changed, from the very detailed preoperative percutaneous transhepatic biliary drainage (PTBD), cholangioscopy, and vascular imaging to multidetector-row computed tomography (MDCT) and three-dimensional CT angiography that facilitates sufficient assessment of tumor extension and staging to selective endoscopic nasobiliary drainage (ENBD), only for the future remnant liver. When cholangiography provides insufficient information of the tumor extent, or segmental cholangitis develops, additional PTBD should be applied to the undrained bile ducts. In this chapter we introduce a recently developed “Japanese” extended resection for difficult perihilar cholangiocarcinoma that might differ from the Western approach.

Preoperative Diagnosis and Management of Hilar Cholangiocarcinoma

Accurate preoperative staging using extensive investigation is indispensable prior to radical resection for hilar cholangiocarcinoma (Nagino et al, 1998; Nimura, 1994, 1997; Nimura et al, 1990 a, 1995 b, 2000). Most cases of hilar cholangiocarcinoma are associated with jaundice as a result of obstruction of the proximal bile ducts, either within or outside of the liver. Obstruction usually involves multiple ducts as a result of proximal spread of carcinoma along the intrahepatic segmental bile ducts. Multiple applications of PTBD (Nagino et al 1992; Nimura et al 1995a; Takada et al 1976) to relieve the cholestasis and restore the functional reserve of the future remnant liver, we believe, is essential prior to operation involving extensive hepatic resection.

In the third edition of this text, we explained that the first step in the preoperative management of hilar cholangiocarcinoma was PTBD; however, MDCT is taken before biliary drainage to define which side of the liver should be resected, and PTBD applied by the direct anterior approach under fluoroscopic guidance (Takada et al, 1976) or ENBD for the future liver remnant (FLR) are selected to relieve cholestasis of the FLR. This offers both diagnostic and therapeutic advantage (Nagino et al, 1995a), and it minimizes PTBD sessions and the number of PTBD catheters. On the other hand, magnetic resonance cholangiopancreatography (MRCP) is occasionally insufficient to diagnose the difficult local anatomy of the separated intrahepatic segmental and/or sectoral ducts and to design an appropriate operative procedure, especially in patients with Bismuth type III or IV tumors (Bismuth & Corlette, 1975). In such cases, high-quality cholangiograms through the PTBD catheters, taken in various positions—supine, right and left anterior oblique, right and left lateral, and cephalad-anterior-oblique positions (Kamiya et al, 1994)—often clearly demonstrate the tumor location and provide the most reliable information on the proximal extension of hilar cholangiocarcinoma along the intrahepatic segmental bile ducts (Ebata et al, 2002; Sakamoto et al, 1998; Fig. 90C.1).

FIGURE 90C.1 A cholangiogram through four percutaneous transhepatic biliary drainage catheters (arrows) shows stricture at the hepatic hilum and suggests cancer extension along the intrahepatic segmental bile ducts (arrowheads). Numerals refer to the Couinaud’s segments of the liver: 8b is a lateral branch of the right anterior superior segmental bile duct, and 8c is a dorsal branch of the right anterior superior segmental bile duct.

When cholangiography provides insufficient information on the tumor extent, additional PTBD is performed to the undrained bile ducts. After dilating the sinus tract of PTBD, percutaneous transhepatic cholangioscopy (PTCS) can also provide reliable information on anatomic variations of the biliary tree and mucosal extension of the cancer (Kamiya et al, 1994; Nimura, 1993; Nimura et al, 1989). Superficial mucosal extension of the carcinoma is often associated with polypoid and/or nodular tumors of a macroscopic type (Igami et al, 2009). Thus PTCS or per oral cholangioscopy with biopsy may be valuable in diagnosis of the extent of cancer (Itoi et al, 2000, 2007; Nimura et al, 1988; Sakamoto et al, 1997). On the other hand, coexistence of biliary tract infection negatively affects the functional reserve of the liver. When intrahepatic segmental cholangitis develops, even in patients with PTBD catheters during preoperative period, urgent additional selective PTBD to the affected segmental duct should be performed (Kanai et al, 1996; Fig. 90C.2).

FIGURE 90C.2 Percutaneous transhepatic biliary drainage (PTBD), to the right anterior sectoral ducts performed for intrahepatic segmental cholangitis. 5, A branch of the right anterior inferior segmental bile duct; 8a, a ventral branch of the right anterior superior segmental bile duct.

Concomitant vascular resection and reconstruction is indicated in some advanced cases of hilar cholangiocarcinoma (Nimura et al, 1991). We previously performed visceral angiography, percutaneous transhepatic portography, and selective retrograde hepatic venography to assess the vascular invasion of the tumor (Nishio et al, 1999, 2003). Recently, three-dimensional images of MDCT using techniques of volume rendering, multiplanar reformation, and maximum intensity projection have taken over because of the high-quality images and reduced invasiveness (see Fig. 90C.13). We usually estimate the relationship between a tumor and the vascular system in the liver and hepatoduodenal ligament and diagnose the vascular involvement of the tumor on the multiplanar reformatted images; we plan the methods of resection and reconstruction—wedge or segmental resection, direct end-to-end anastomosis, or segmental auto vein grafting—by volume-rendered images and maximum-intensity projection images (Senda et al, 2009; Sugiura et al, 2008).

FIGURE 90C.13 Three-dimensional reconstructed images of multidetector-row computed tomography. A, Coronal view of multiplanar reformatted images shows left hepatic duct tumor invading the portal bifurcation (arrow). B, Anteroposterior view of volume-rendered image demonstrating obstruction of the left portal vein and narrowing of the contralateral side of the portal bifurcation (arrow). Portal vein resection was planned along the lines shown. C, Craniocaudal view of maximum-intensity projection image reveals narrowing of the portal bifurcation (arrow). This view cannot be obtained by conventional arterial portography or percutaneous transhepatic portography.

Postoperative morbidity and mortality rates remain considerable, particularly if extensive resection is necessary (Nagino et al, 2001). Postoperative liver failure sometimes occurs and results in death. For this reason, liver resection is usually scheduled only after the liver has been decompressed and the serum total bilirubin concentration falls below 2 mg/dL. In addition, since 1989, we have routinely performed percutaneous transhepatic portal vein embolization (PVE) (Nagino et al, 1993, 1996, 2000, 2006a) as preoperative preparation for extensive liver resection. This is done to minimize the incidence of postoperative liver failure. After estimation of the resultant compensatory hypertrophy by volumetric CT scan (Nagino et al, 1995b, 1995c) and of the functional reserve of the liver as assessed by the indocyanine green clearance rate (Uesaka et al, 1996), hepatectomy is performed, usually 2 to 3 weeks after PVE. A Japanese series implemented a management strategy for patients with hilar cholangiocarcinoma that consisted of preoperative biliary drainage, PVE, and major hepatobiliary resection; it reported a 0% mortality rate in more than 100 consecutive cases (Sano et al, 2006).

Impaired intestinal barrier function does not recover with PTBD without bile replacement, which can restore the intestinal barrier function during external biliary drainage in patients with biliary obstruction, primarily as a result of repair of physical damage to the intestinal mucosa. Thus, in patients with hilar cholangiocarcinoma and external biliary drainage, we routinely replace bile prior to operation (Kamiya et al, 2004; Kanazawa et al, 2005). Also, perioperative administration of synbiotics can enhance immune responses, attenuate systemic postoperative inflammatory responses, and improve the intestinal microbial environment (Sugawara et al, 2006). These procedures may reduce postoperative infectious complications after major hepatobiliary resection in difficult patients with hilar cholangiocarcinoma.