CME-Certified TrainingThe surgeonFebruary 2014, Volume 85, Issue 2, pp 155-168First line: 26 January 2014Central cholangiocarcinoma (Klatskin tumor)

GA Stavrou

SummaryThe perihilar cholangiocarcinoma (Klatskin tumor) is a rare tumor, starting from the extrahepatic bile duct fork. Given the close relationship of the anatomical biliary fork to liver parenchyma, and hepatic arteries Pfortadergabel the treatment of these patients represents a major challenge. With an incidence 2-4 illnesses / 100,000 inhabitants / year, treatment is only useful to centers that have the necessary experience.Histologically usually results from a moderately differentiated adenocarcinoma that can grow diffusely infiltrating along the bile ducts and Perineuralscheide proximally, but distally. As the only curative option, the radical surgical resection of bile ducts, bile duct fork en bloc has prevailed with liver resection and also Gefäßresektion, yet achieving a proximal and lateral safety distance from the tumor is technically problematic.Keywords

Klatskin cholangiocarcinoma hepatectomy imaging operation strategyAbbreviations

A. / Aa.Artery / arteries

ALPPSAssociated liver and portal vein ligation partition for staged hepatectomy

CA 19-9Carboanyhdrat antigen 19-9

CCCcholangiocellular carcinomas

CEAcarcinoembryonic antigen

CTComputed Tomography

ERCPendoscopic retrograde cholangiopancreatography

IORTIntraoperative radiotherapy

MRCPMagnetresonanzcholangiopankreatikographie

MRIMagnetic Resonance Imaging

PETPositron Emission Tomography

PSCprimary sclerosing cholangitis

PTCDpercutaneous transhepatic biliary drainage

PVE, portal venous embolization

V. / Vv.Vena / Venae

Perihilar cholangiocarcinoma (Klatskin tumor)

Abstract

Perihilar cholangiocarcinoma or Klatskin tumors are a rare entity Arising from the extrahepatic bile duct bifurcation. Considering the close anatomical relationship of the bile duct bifurcation with the portal vein bifurcation and hepatic arteries, surgical treatment is demanding. With an incidence of only 2-4 cases / 100,000 population / year patients Should be Referred to a specialized center. The tumors are poorly differentiated adenocarcinomas Usually diffusely growing Along the duct so the perineural sheath and. Only radical surgery offers a curative option and currently surgical strategy Usually Consists of en bloc resection of the bile duct, liver resection and portal vein extended resection. Proximal and lateral safety margin R0 resections are technically very demanding procedures Because of the local anatomy.Keywords

Klatskin tumor cholangiocarcinoma Liver resection Imaging Surgical Strategy

Learning Objectives

After reading this article ... You know the etiology and staging of Klatskin tumors, Do you know how diagnostic measures for this type of tumor can be

used wisely, are you the criteria of resectability known Know the potential surgical resection strategies.

Etiology and Staging

The central cholangiocarcinoma, also perihiläres cholangiocarcinoma or after his Erstbeschreiber Gerald Klatskin [1 called] Klatskin tumor, belongs to the family of cholangiocellular carcinomas (CCC; [2]). The CCCs arising from epithelial cells of the intra- or extrahepatic bile ducts. These are basically

divided into intra- and extrahepatic CCCs. The intrahepatic CCC arise from the small intrahepatic bile ducts or the large bile ducts of the right and left biliary system proximal to the bifurcation, beginning with the region of the biliary extrahepatic fork the CCCs. In principle are bile duct tumors that infiltrate the central bile duct fork, today irrespective of their origin (intra- or extrahepatic) as perihiläres cholangiocarcinoma referred. 50% of all CCCs are perihilar, 40% distal extrahepatic and intrahepatic tumors only 10% [3]. The CCCs are beyond the perihilar area according to their anatomical infestation pattern after the Bismuth-Corlette classification (Fig. 1divided) [4]. After this classification, vascular or Leberparenchyminfiltrationen will not be considered, so that they can be used is limited for the assessment of prognosis. The new TNM classification appears under these aspects more suitable (Tab. 1).The CCCs arising from epithelial cells of the intra- or extrahepatic bile ducts

Fig. 1

Classification of central bile duct tumors after Bismuth-Corlette. (Drawing: Prof. Giuliana Brogi, Siena, Italy, by kind permission.)

Tab. 1TNM classification of malignant tumors of the International Union Against Cancer 2010. (Mod. For [42])

T - primary tumor

TX Primary tumor can not be assessed

T0 No evidence of primary tumor

Tis Carcinoma in situ

T1Tumor on the bile duct with limited extension into the muscularis propria or the fibromuscular layer

T2a Tumor invades beyond the bile duct into the adjacent soft tissue

T2b Tumor invades adjacent liver parenchyma, the

T3 Tumor invades unilateral branches of the portal vein or hepatic artery

T4

Tumor invades the main branch of the portal vein or bilateral branches; or the common hepatic artery or branches 2nd order bilaterally; or unilateral branches of 2nd order of the bile duct with infiltration of contralateral branches of the portal vein or hepatic artery

N - Regional Lymph Nodes

NX Regional lymph nodes can not be assessed

N0 No regional lymph node metastasis

N1

, Portae Regional lymph nodes metastases in lymph nodes of the cystic duct, the common bile duct along the hepatic artery and the vena

M - distant metastases

MX Presence of distant metastases can not be assessed

M0 No distant metastasis

M1 Distant metastases

The majority of the CCCs (90%) are adenocarcinomas, squamous tumors are usually the remaining. The adenocarcinomas are further divided into nodular, sclerosing and papillary carcinoma, the papillary highest resection and cure rates have [5]. CCCs are rare, its incidence is 1 to 2 cases per 100,000 population.Interestingly, the incidence of extrahepatic CCC appears to be declining in recent years, however, the intrahepatic CCCs likely to rise [6]. The spread of cancers can be made longitudinally along the bile ducts, but also vertically. The growth pattern is diffuse mostly, but there was also a discontinuous growth described (so-called. "Skip lesions"). With the newer histopathological techniques can be next to a lymph vessel invasion also often a perineural growth along the bile ducts in the direction hepatofugaler observe what the R0 resection significantly more difficult [7]. It also comes with already small tumors in about 50% of cases of infiltration of the liver parenchyma or to a vascular infiltration.The majority of the CCCs (90%) are adenocarcinomas

The spread of cancer can be done longitudinally along the bile ducts, but also vertically

As the cause of the emergence of a CCC multiple risk factors are considered. These include recurrent cholangitis (cholelithiasis, primary

sclerosing cholangitis [PSC]), bile duct cysts, chronic hepatitis, but also the action of chemicals (dioxins, nitrosamines etc.) or drugs (isoniazid, methyldopa etc.), wherein the primary PSC and biliary cysts which common risk factors present [8]. In Asia, also parasitic infections in carcinogenesis play a role. It is estimated that it will take 15 years to develop a CCCs.The primary PSC and biliary cysts are the most common risk factors

Diagnostic

Patients with central CCCs are usually symptomatic, when the tumor occludes the bile ducts. The most common clinical signs are jaundice, pruritus (66%), pain (30-50%), weight loss (30-50%) and fever (20%).Patients observed due to biliary obstruction pale stools and dark urine. The sequence of the performed diagnosis should be discussed in an interdisciplinary team to achieve the best possible success for the patient.The most common clinical signs are jaundice, pruritus, pain, weight loss and fever

A histological or cytological securing the malignant process is only possible in a few cases, due to the difficult visualization of the lesion. The tumor marker CEA (carcinoembryonic antigen) and CA 19-9 (Carboanyhdrat antigen 19-9) are difficult to interpret at low values, because they are also increased in cholestasis and cholangitis benigen and therefore can not provide a decisive contribution to the diagnosis.When clinically reasonable suspicion of a tumor beyond the perihilar area, the operation is also indicated without histological confirmation.When clinically reasonable suspicion that surgery is indicated even without prior histological confirmation

Imaging

In jaundiced patients the ultrasound finds frequent application. Typical findings are dilated intrahepatic bile ducts and a lack of connection between the left and right bile duct. The ultrasound is very well suited for screening may present vascular infiltration. The endoscopic ultrasound does not matter much in the diagnosis of proximal bile duct tumors generally. In the first place the diagnosis before any manipulation of the bile duct MRI with MRCP (Magnetresonanzcholangiopankreatikographie) to assess the biliary conditions without the influence periinterventioneller inflammatory responses (imaging of choice) is recommended. Here already first conclusions of an operational strategy can be drawn. The method requires no direct contrast agent in the biliary system (Fig. 2). The MRCP also provides 3-D representations of the bile duct system.The MRCP is more appropriate to describe the extent of the tumor, as ERCP (Endoscopic retrograde cholangiopancreatography), but tends to "Under staging" [9]. The MRCP should be in front of a relief of cholestasis (ERCP / PTCD [percutaneous

transhepatic biliary drainage]) carried out due to a consecutive collapse of the duct system.In the first place the diagnosis before any manipulation of the bile duct MRI with MRCP is recommended

Fig. 2

Magnetresonanzcholangiopankreatikographie: 78-year-old patient with a Klatskin tumor type II, the stenosis begins just above the mouth of the cystic duct and ends in the hepatic

Computed tomography (CT) of the chest and abdomen allows a sufficient staging with respect to tumor extension and distant metastases, a positron emission tomography (PET) -CT was evaluated by several research groups, but brings no significant information gain for most patients [10]. The triphasic high-resolution CT allows a good statement regarding the individual anatomy, possible hilar vascular infiltration in portal venous and arterial system and can also be a virtual surgical planning serve the vascularization of the liver with regard to the Resektionsstrategie and a relatively accurate volumetrics of residual liver segments is possible [11]. The venous drainage can be properly assessed, particularly with regard to the Resektionsstrategie (right vs. left). If a PTCD catheter in situ can be moved into the CT also a fourth phase, with a 3-D segmentation of the biliary tract is possible.The thoracic and abdominal CT is adequate for a staging in terms of tumor extent and distant metastasis

PTCD / ERCP

For the relief of biliary ERCP associated with stent placement and the PTCD available. Most patients will be assigned only after a successful ERCP away at a center, so that the diagnosis is further complicated by the manipulation of the bile ducts. Advantage of ERCP is the possibility of sampling for histological confirmation - this, however, is rarely successful. Disadvantages are the bacterial contamination to the intrahepatic bile ducts with increased infectious complications and the potential tumor cell displacement during the necessary intraoperative stent removal. A tumor cell displacement within the meaning of implantations or increased incidence of liver metastases was, however, also postulated for PTCD [12]. The PTCD seems ERCP in assessing proximal tumor extension superior, but also provides only about half the cases, a precise analysis.Advantage of ERCP is the possibility of sampling for histological confirmation

Important for the relief of biliary drainage is a collusion between endoscopist and surgeon, as it should be decided jointly on the necessity and form of drainage - is further drainage of future residual liver after surgery usually

sufficient [13]. From the surgical point of view PTCD ERCP (is preferable because a more accurate and reproducible cholangiography enables easy and contamination of the biliary tract is minimized 3 fig.).Also possible to dispense with a drainage after percutaneous cholangiography, when only a slight cholestasis is present [14]. However, in the area of the left lobe of the percutaneous access is linked much more difficult and higher morbidity [1, 15]. With the increasing technological development and dissemination of ERCP however PTCD is increasingly becoming the exception process. Even Japanese groups who propagated this always have, and in particular the new possibilities of Fluoreszenzcholangiographieresponded and their preoperative approach towards ERCP changes [2, 16, 17].After percutaneous cholangiography can be dispensed with a drainage if only a mild cholestasis present

Fig. 3

Percutaneous transhepatic biliary drainage (PTCD) in a patient with a tumor type Klatskin IIIa. In of failed endoscopic retrograde cholangiography a PTCD was introduced on both sides to relieve the biliary tract. The right drainage is advanced into the duodenum. In the Cholangiography the stenosis can be seen, which extends into the common hepatic duct Dexter. A cholangiography right biliary system and b left biliary system

Preoperative drainage

The question of whether a drainage of the bile duct system is necessary, is controversial [3, 14, 18, 19, 20].Stents should be avoided in principle. Cholestasis of the liver, however, increases the dysfunction, which is responsible for an increased post-operative morbidity and mortality [4, 12]. However, stents are therefore often unavoidable. The necessary imaging should be completed to clarify the resectability before stent insertion. A biliary stent lying complicates the intraoperative assessment of resectability. A meta-analysis of 11 studies and a prospective study show no significant benefit of preoperative biliary drainage [5, 21]. Not infrequently, the patient is presented with already lying stent the surgeon. Furthermore, can be carried out not immediately sometimes surgery for diagnostic or logistical reasons.A biliary stent lying complicates the intraoperative assessment of resectability

Staginglaparoskopie

The Staginglaparoskopie has already become in many tumor types standard in the preoperative evaluation, since minimally invasive peritoneal tumor seeding can be diagnosed. Various working groups carry this in their routine

by [6, 10, 13]. The private general we perform laparoscopy only imaged by advanced tumors with a high probability extrahepatic tumor growth.By Staginglaparoskopie, a peritoneal tumor seeding are minimally invasive diagnoses

Liver function tests

For the surgical Resektionsstrategie is the assessment of liver function and the function of the future liver remnant tissue is of great importance. Various Liver function tests are available, which are used particularly in the Asian region. Their significance is disputed, in particular in the cholestatic liver. A developed by Stockmann and employees test also seems in this case to be able to provide additional information regarding the function of the residual liver, has not yet been accepted as a standard [22].

Resektabilitätseinschätzung

The biggest challenge for the surgeon is the assessment of resectability and the development of appropriate Resektionsstrategie. While tumors that have reached the same segment bile ducts of both sides, as well as tumors are considered unresectable infiltrate both hepatic arteries. The problem is that the intraductal linear expansion of the tumor by any method is reliably assessed preoperatively and even the combination of different methods, the proximal expansion often overestimated, so that in consequence of the exclusion of contraindications only exploration permits real clarification of resectability. This also means that not everyone as Bismuth IV classified tumor is actually unresectable [8, 23]. The diagnostic laparoscopy has it their priority to the exclusion of liver metastasis and peritoneal carcinomatosis, but is not suitable for the assessment of resectability in our opinion. A locoregional lymph node involvement does not constitute a contraindication for surgery. Therefore, high rates have for the exploration beyond the perihilar area bile duct carcinomas and despite optimal preoperative diagnosis are accepted [9, 12, 16].Only exploration permits real clarification of resectability

Surgical strategy and technique

From oncologic perspective, the R0 resection - ie free resection margins in the proximal, distal and lateral -. The aim of the operation. The close anatomical positional relation in particular to Pfortadergabel and the hepatic arteries makes compliance with the usual oncologic surgery criteria ("no-touch") is very difficult, and the intraoperative frozen section analysis is due to the possible discontinuous tumor growth of limited significance and Perineuralscheidenwachstums (that eludes this). Theoretically proximally longitudinally a safety distance of 1-2 cm, are laterally respected by 5 mm, this is, however, particularly in the right main bile duct considerably shorter often impossible [10, 24]. Even with macroscopically sufficient safety margin

and thus a potentially resectable situation intraparenchymal bile ducts can not be resected with histologically detectable unlimited infiltration, so that there is, despite all efforts to R1 situation. After distal insufficient safety distance can be achieved through an extension of the operation to a pancreas.Should longitudinally proximally a safety distance of 1-2 cm, are laterally respected by 5 mm

Criteria of Irresektablität

The reasons for leaving a surgical strategy may be oncological or technical nature. Resection at present distant metastasis or peritoneal does not make sense. Lymph node metastasis in hilar or within the range of the celiac trunk do not constitute a contraindication to resection [10, 11, 15]. An insufficient residual liver volume after resection is a technical problem, the z by conditioning. B. a, portal venous embolization (PVE) can be met [12, 25]. Resection of Pfortadergabel is technically feasible and does not constitute a contraindication for tumor invasion. An arterial infiltration in the area of the common hepatic artery or the artery of the remaining lobe of the liver means a technical irresectability, although there are also here the beginnings of a arterial reconstruction in selected patients [ 13, 26].Hilar lymph node metastasis in or near the Tr. celiac do not constitute a contraindication for surgery

Hypertrophiekonzept

If a Klatskin tumor type IIIa according to Bismuth, so a direct resection with an (extended) hemihepatectomy right is usually not possible because the Parenchymreserve the left-lateral liver segments is often too low.An exploration is recommended in this case only at sufficiently large left lobe in the imaging. Alternatively, of makuuchi [in this case 14, 27 for the first time used] and Neuhaus [25 refined approach] especially for right-sided tumors are applied. Advantage of hypertrophy in the range of segments II and III is a prevention of postoperative liver failure [28]. For the implementation of the PVE precise agreement between radiologists and surgeons is necessary, especially for dealing with portovenösen vascular variants from left.Advantage of hypertrophy in the range of segments II and III is a prevention of postoperative liver failure

The PVE is performed via a direct access to the portal system via puncture of the right, for the embolization itself are mostly metal coils and so-called. Plugs used [29, 30]. The decisive factor is the closure of all portals branches to segment IV, which often depart from the left portal system. Within 3 to 5 weeks after PVE can be expected with the greatest Hypertrophieschub - with heavily modified cholestatic liver hypertrophy may, however, analogous to cirrhotic livers certainly take considerably longer [31, 32]. A repeat CT diagnosis even after 8 weeks may be useful.

The decisive factor is the closure of all portals branches to segment IV, which often depart from the left portal system

Disadvantage of the PVE is the commitment to a Resektionsstrategie. If z. B. intraoperatively be an infiltration of the left hepatic artery, so the strategy can not be changed on an extended left resection. Also an increased tumor growth has been described after PVE in the remaining liver, this is, however, relevant [more metastases of other tumors than Klatskin tumors 33].The analysis of hypertrophy can be performed on CT data reliably together by radiologists and surgeons. In general, we prefer to own the volumetrics by the surgical planning software HepaVision the Fraunhofer MeVis Group. Advantage of this type of volumetry is that as a result of a well-vascularized liver volume is calculated, which is rather equivalent to a functional volume [34, 35]. This is particularly important with regard to the change in operating strategy intraoperatively helpful because a segment oriented volumetrics exists that takes into account the variation venous drainage. For a resection in Klatskin tumors a residual liver volume of 40% should be sought, as will be operated here in a previously damaged by cholestasis liver.For a resection in Klatskin tumors a residual liver volume of 40% should be sought

A new alternative to the PVE, the two-stage operation strategy through a in-situ split liver is, the so-called ALPPS ("Associated liver partition and portal vein ligation for staged hepatectomy") -. Approach [36], the principle also at Klatskin can be used tumors [36]. However, combining in this case the two surgical techniques highest level of difficulty, which should be applied in view of the already increased mortality and morbidity of this patients group only in exceptional circumstances [37].

Resection strategies

The spectrum of Resektionsmöglichkeiten ranges from the local resection of the intrahepatic bile ducts with Gallenwegsresektion on Mesohepatektomie as local hilar resection of the hepatic parenchyma to Hemihepatektomien left or right. Many writers always do a segment I-resection [15], since the segment I-bile ducts open directly in the bile duct and fork as a tumor growth direction Segment I is likely. If the segment-I resection is feasible in view of the residual liver volume, this should also be done.If the segment-I resection is feasible in view of the residual liver volume, this should also be done

Primarily be to increase the lateral safety distances in modern times Advanced Resektionsformen(Trisegmentektomie right or left) carried out under entrainment of the portal bifurcation in addition to resection of the bile ducts. Due to the Perineuralscheidenwachstums of tumors and the spread occurring with "skip lesions" the significance of intraoperative frozen

section diagnosis is very limited. This calls for the extended resections, since only so maximum safety distances can be achieved (Fig. 4). Also, a distal extension by a pancreas must already be included in the preoperative strategy, as this is sometimes the only way to a R0 resection.

Fig. 4

Macroscopic representation of Klatskin tumor on resected. You can see a significant change in the bile duct epithelium (s. Mark) in the area of the tumor, the distal bile duct epithelium shows regular (pictured above), by the liver resection and direction parenchyma of the safety distance is maintained

Hilar complicated bile / Mesohepatektomie

A hilar resection is hardly feasible under curative aspect, since the long-term results from large series forinsufficient radicalism speak. In a Klatskin tumor type Bismuth I it may be sufficient under certain circumstances. However, the local resection has a role in treating patients who z. B. benefit due to pre-existing conditions or contraindications to major hepatic resection of a palliative resection of the bile ducts within the meaning of quality of life. When a tumor Bismuth type 2 would be at least one segment I-resection should be considered, but better to increase a suprahiläre resection in terms of Mesohepatektomie to the safety distances. The Mesohepatektomie corresponds to a more radical form of Hilusresektion. Due to the preparation above the portal bifurcation the radical nature of the difficulty of resection is limited, however, very high. The reconstruction of the bile ducts on both sides is a challenge, so this resection is only useful in exceptional cases when tumors in early stages.A hilar resection is under curative aspect hardly feasible

(Advanced) hemihepatectomy left

With a hemihepatectomy left and an extended hemihepatectomy left a curative resection in patients succeed especially if the tumor vorwächst into the left bile duct system. The by Nimura [38 propagated] strategy has the particular advantage that the right-lateral liver segments usually guarantee a sufficient residual liver volume. A segment-I resection is always part of the strategy. Grows the tumor, however, up to the right Gallenwegssytem ago, the maximum attainable lateral safety margin due to the necessary curve of resection and the right existing earlier bifurcation of the biliary tract is limited. The reconstruction of the bile ducts in this case is also usually very complex, there must be at least 2, but often 4 segmental bile ducts connected in an anastomosis (Fig. 5). With extended left hepatectomy is the most technically demanding resection ever. Obtaining the venous drainage of the right-lateral segments may in this case be a challenge, since the middle hepatic vein has to be sacrificed in the resection.

The right-lateral liver segments usually guarantee a sufficient residual liver volume

With extended left hepatectomy is the most technically demanding resection ever

Fig. 5

Trisegmentektomie left - Situs after resection. Route the connection are marked with probes biliary

(Advanced) hemihepatectomy right

Due to the anatomy of the biliary tree fork with a much longer history of the left bile duct is the largest longitudinal and lateral safety distance in the extended hemihepatectomy right can theoretically (Trisegmentektomie right segment IV-VIII plus I) achieve, since in this case the bile duct is issued left the hilar level , This requires a sufficient size of the left-lateral segments, which may need to be augmented before. If the resection combined with a Pfortadergabelresektion, this is the only strategy that a "no-touch" technique allows the tumor resection, as the main tumor mass is not tampered with during resection (Fig. 6).The oncological results of the Berlin Working Group are equipped with a 5-year survival of 65% at application of this radical strategy exceptionally well [24]. Nevertheless, a small tumor can certainly also an anatomical hemihepatectomy law may be sufficient to achieve an R0 resection - this has to be decided intraoperatively.The extended right hepatectomy of the largest longitudinal and lateral safety distance can be achieved

Fig. 6

Trisegmentektomie right at Klatskin tumor type IIIa. The bile duct is distally severed and pulled on the thread side, the infiltration of Pfortadergabel is clearly visible. The intraparenchymal surgical margins left is tumor-free. The tumor can be removed with a Pfortadergabelresektion in a no-touch technique en bloc with the liver now

Palliative hepatojejunostomy

Is in the exploration impossible resection, there is a surgical option for palliation by investing a hepatojejunostomy on the liver segments III and V. Here, by "exposing" of bile ducts in these segments onethere sufficient drainage can be achieved directly on the liver parenchyma a spacious anastomosis what the patient an omission regular stent exchange and

potentially less infectious complications draws (Fig. 7).Palliative radiotherapy the tumor region is this reconstruction not in the way and is applied by us in individual cases.

Fig. 7

Palliative scale hepatojejunostomy a Roux-loop on the right (segment V) and left (segment III) liver lobe

Intraoperative radiotherapy

Intraoperative radiation the tumor region with a targeted volume is a possibility of additive palliative therapy for unresectable tumors, but is rarely available [due to the necessary technical requirements 39]. Whether intraoperative radiotherapy (IORT) may mean even with resectable tumors, an improvement of survival, is currently not available. With the technical advances in stereotactic radiotherapy, the effect of IORT in the future can certainly also achieved postoperatively, this, there are still no suitable randomized data.Irradiation of the biliary anastomosis also appears postoperatively safe [40].The IORT is a possibility of additive palliative therapy for unresectable tumors

Reconstruction of the bile ducts

The reconstruction of the bile ducts is carried out by a disabled jejunum by Roux. An accurate representation of all bile ducts to be connected is necessary with probes, this can be simplified. For the anastomosis itself offers a single-button technology with PDS 4/0 or 5/0 threads of strength. We prefer whenever possible the anastomosis of all biliary tract together as "Blanket" -Anastomose. The threads can thereby be fundamental part directly in the parenchyma. The endoluminäre splinting the anastomosis is controversial. A valid data location does not exist to some workgroups use principle [41]. Our own experiences suggest to drain the anastomosis sufficient. This is done by splinting with transhepatic drainage, which can be either "lost" or used as a classic endless drainage. With this technique, complications in the area of the bile duct can be reduced to a minimum, small gall leaks can heal over the drainage.The reconstruction of the bile ducts is carried out by a disabled jejunal Roux

Conclusions for clinical practice

The treatment of patients with central or beyond the perihilar area bile duct tumors constitutes a major challenge for the interdisciplinary treatment team. These patients should be supplied only at a specialized center. Preoperatively, the challenge is to develop a surgical strategy, this

has a direct influence on the nature of the preoperative conditioning of the patient. As a diagnostic algorithm makes sense:

1. 1.Contrast medium sonography, 

2. . 2MRI / MRCP, 

3. . 3triphasic CT, 

4. . 4ERCP / PTCD. 

Despite expansion of the diagnosis the true extent of the tumor can be seen only during a laparotomy, only intraoperatively may be decided on the resectability. Thus, a high rate of exploration must be accepted. In the case of the extended resection resections are preferable because only so the best safety distances can be achieved. The Trisegmentektomie right with Pfortadergabelresektion promises theoretically the best oncological results, but is mainly used for right-sided tumors of importance and has the disadvantage that almost the detour must be gone over the Hypertrophiekonzept always. The Trisegmentektomie left is the most technically demanding resection, but can achieve good oncological results speak especially when left-sided tumors. Because of cholestatic starting position before surgery and usually always present bacterial contamination of the bile ducts after their discharge treating Klatskin tumors with a significantly higher morbidity and mortality than usual in liver surgery is fraught usual.

CME questionnaire

After which the classification beyond the perihilar area bile duct carcinomas are classified?

A classification for perihilar tumors does not exist.

Several classifications are used for anatomic classification of bile duct carcinomas beyond the perihilar area, but there is no uniform classification.

The classification of beyond the perihilar area bile duct tumors is based on the Bismuth-Corlette classification can be distinguished in which 6 types.

The classification of beyond the perihilar area bile duct tumors is based on the Bismuth-Corlette classification can be distinguished in which 5 types.

The classification is according to the Child-Pugh classification.

In the preoperative diagnosis of a tumor beyond the perihilar area ...

is usually sufficient, a CT imaging to assess the resectability.

MRI is the imaging of choice.

the ultrasound examination of the abdomen is considered the "gold standard".

a contrast-enhanced MRI should be performed.

ERCP is the ultimate diagnostic tool for assessment of tumor extent.

In a present preoperatively jaundice ...

accepts this - even during long existence - not affect the liver function.

must be a relief of cholestasis by ERCP stent.

, the decision from the stenting of the level of bilirubin.

stenting is usually not necessary, since there is no cholestasis in beyond the perihilar area bile duct tumors.

there is no possibility for relief of cholestasis by ERCP and stent implant in beyond the perihilar area bile duct tumors.

With regard to the assessment of resectability in Klatskin tumors ...

this preoperatively usually no problem.

is often incorrectly classified in the preoperative diagnosis, the tumor extent.

is a diagnostic laparoscopy always indicated and necessary.

is necessary preoperative biopsy to confirm the diagnosis.

is well defined in preoperative imaging vascular infiltration.

For beyond the perihilar area bile duct tumors is no contraindication to surgery:

Locoregionally enlarged lymph nodes.

Peritoneal tumor seeding.

Preoperative pulmonary or hepatic metastases presentable.

An extensive infiltration of both lobes of the liver.

One infiltration of the hepatic artery.

Which of the following factors is no criterion of irresectability?

A too low forecasted postoperative residual liver volume in the first imaging.

Infiltration of both hepatic arteries.

Pfortadergabelinfiltration.

Tumor extension of type IV according to Bismuth.

Future residual liver volume 30%, bilirubin 15mg / dl.

The so-called. ALPPS Approach ...

is not considered a surgical procedure available in the therapy perihilärer bile duct tumors.

is not feasible in Klatskin tumors due to the anatomical circumstances.

is beyond the perihilar area in tumors currently no established method represents, but is feasible in selected cases to achieve a R0 resection.

is the method of choice in the surgical treatment of Klatskin tumors.

is indicated only for tumors of type I and II according to Bismuth.

In the intraoperative assessment of the resectability of a tumor Klatskin ...

applies a venous infiltration as a Irresektabilitätskriterium.

this is still not affected by a venous infiltration.

means the infiltration of the Pfortadergabel irresectability.

is only in very rare cases, before a vessel infiltration.

apply multiple metastases of both liver halves not as a criterion of irresectability.

Regarding the Resektionstrategie ...

can be achieved with the so-called. Mesohepatektomie insufficient radicalism.

provides a good but technically demanding alternative to resection of Klatskin tumors of type I or II according to Bismuth is the Mesohepatektomie.

is the extended right hepatectomy at Klatskin tumors no meaningful surgical treatment option

is the extended hemihepatectomy left at Klatskin tumors of the type IIIa according Bismuth the method of choice.

is the extended right hepatectomy at Klatskin tumors of type IIIb according Bismuth the method of choice.

Klatskin tumors ...

are afflicted with a good prognosis.

can be treated despite a very demanding surgical treatment in any general surgical department.

are tumors that are well-resectable and usually accompanied by a low perioperative morbidity and mortality.

are usually early clinically manifest by pain.

should be treated only in specialized and interdisciplinary liver surgical centers.Thanksgiving

The authors are of the artist Prof. Giuliana Brogi (Siena, Italy) for the preparation of Fig. 1 extremely grateful.

Compliance with ethical guidelines