Hepatectomy for cholangiocarcinoma complicated with right umbilical portion: anomalous configuration of the intrahepatic biliary tree

The right umbilical portion (right-sided round ligament) has been discussed as an intrahepatic portal venous anomaly associated with "left-sided gallbladder" in several reports. We treated two patients with right umbilical portion (RUP) associated with cholangiocarcinoma. Left hepatectomies were performed, preserving the residual hepatic blood flow and biliary continuity. From our experience in these patients we propose the presence of anomalous configuration of the intrahepatic biliary tree in RUP, because both patients showed medial segmental bile ducts ramified from the right and left hepatic ducts. In general, although the medial segmental bile duct ramified from the left, we surmised that this abnormal bilateral drainage pattern may not be a rare phenomenon in RUP. Special attention may be required to focus on the anatomy of the portal tributaries and biliary ramifications in RUP. Received for publication on July 7, 1999; accepted on Nov. 11, 1999     

Anomalous intrahepatic portal system in a patient with hilar bile duct cancer

We report a case of anomaly of the intrahepatic portal system in a 65-year-old man with hilar bile duct cancer. Preoperatively, percutaneous transhepatic portography demonstrated that there was a right posterior portal vein arising from the main portal vein. In addition, a large portal branch originated from the left portal vein and coursed toward the right hepatic lobe. Following portal embolization of the right posterior branch, the patient underwent an extended right hepatectomy with a caudate lobectomy. Intraoperatively, to the left at the porta hepatis and then it first gave off the right anterior portal vein originated from the left portal vein and coursed toward the right hepatic lobe horizontally behind the gallbladder and then separated into superior and inferior segmental branches to supply the right anterior segment of the liver. The ramification of some major branches without malposition of the gallbladder or round ligament was the important clinical feature of this anomaly.     

Resection of metastatic liver cancer in a patient with a left-sided gallbladder and intrahepatic portal vein and bile duct anomalies: A case report

INTRODUCTIONThe presence of left-sided gallbladder is closely associated with multiple combined anomalies of the portal vein, hepatic vein, hepatic artery, and bile duct. This requires special attention for preoperative evaluation for the purpose of preventing postoperative complications.PRESENTATION OF CASEA 70-year-old woman with metastatic liver cancer and intrahepatic portal vein, biliary system and hepatic artery anomalies with left-sided gallbladder is reported. On computed tomography (CT), a solitary low density mass occupied from the right anterior to the posterior segment of the liver. The gallbladder bed was on the left of the hepatic fissure. On drip-infusion-cholangiography (DIC) CT three-dimensional (3D) reconstruction, the left medial bile duct arose from the right umbilical portion after arising from the left lateral bile duct. Following a right hepatectomy and lymph node dissection of the hepatoduodenal ligament, hepaticojejunostomy was conducted separately to the left medial and left lateral bile duct.DISCUSSIONThe left-sided gallbladder accompanies with several anomalies of hepatic vascular and bile duct anomalies in a frequent manner. A safe hepatectomy needs accurate operative plans to ascertain the range of hepatectomy, because it often has the diversity of a combined anomaly.CONCLUSIONPreoperative DIC-CT 3D reconstruction was extremely useful because it provided an important information that could not be obtained with 2D-DIC-CT. 3D imaging has the ability to demonstrate complex anatomical relationships, this devise is a effective new tool for making appropriate preoperative strategy.     

Are left-sided gallbladders really located on the left side?

OBJECTIVE: The aim of this investigation was to establish the association between left-sided gallbladders and right-sided round ligaments. SUMMARY BACKGROUND DATA: The left-sided gallbladder is a rare anomaly and has been classified into two types: 1) gallbladder migration to the left side and 2) development of a second gallbladder with atrophy of the original one. Recently, left-sided gallbladders were reported to be associated with right-sided round ligaments. METHODS: The authors reviewed 3 patients treated in their departments and 15 patients reported in the literature diagnosed as having left-sided gallbladders accompanied by right-sided round ligaments. RESULTS: Although the gallbladders of all 18 patients were located at the normal site, they were diagnosed as being left sided because of the right-sided round ligaments. This anomaly was accompanied by abnormal intrahepatic portal venous branching, which could be classified into two types. In eight patients, the first branch of the portal vein ran to the posterior segment and then the portal vein formed a trunk of the left and right anterior portal veins. The latter portal vein formed the umbilical portion and finally joined the right-sided round ligament (trifurcation type). In five, the portal vein diverged normally to form the left and right portal veins, then the latter branched to form the anterior and posterior segments, and finally the anterior branch joined the round ligament (bifurcation type). In the other five, the branching type could not be determined. CONCLUSIONS: A right-sided round ligament causes a gallbladder at the normal site to be located on the left side. This anomaly should not be diagnosed as a left-sided gallbladder but as a right-sided round ligament. Recognition of this anomaly clinically is important when performing hepatectomy, because it is always associated with abnormal intrahepatic portal venous branching.     

Left-sided gallbladder: report of a case and study of 26 cases in Japan

A case of left-sided gallbladder with an anomaly of the intrahepatic portal vein and cholesterol polyps of the gallbladder is presented. A 23-year-old man with sudden onset of epigastric pain, fever and abdominal irritation was admitted to our hospital and an emergency celiotomy was carried out. The gallbladder lay on the left side of the round ligament, that is, it was a left-sided gallbladder. Since operative findings such as cholecystitis, gastrointestinal perforation and pancreatitis were not observed, no more procedures were done. Postoperative ultrasonography showed some interesting findings as follows: multiple echogenic nonshadowing small polypoid lesions which did not change in size and shape during a 2.5 year interval were detected and were thought to be cholesterol polyps. The tail of the gallbladder was situated on the left side of the round ligament and the motion of the gallbladder as determined by postural exchange was not floating. These findings are compatible with a left-sided gallbladder. The left branch of the portal vein did not form an umbilical portion as is usually expected and the right branch formed a cystic structure similar to a normal umbilical portion with the anterior, posterior and medial branches ramified. The round ligament arose from the cul-de-sac of the umbilical portion. Twenty-six cases of left-sided gallbladder from the Japanese literature were reviewed and discussed.     

True left-sided gallbladder with a portal anomaly: report of a case

A 65-year-old female who presented with back pain was diagnosed to have the presence of biliary sludge in the gallbladder. Computed tomography showed that the round ligament connected to the left portal umbilical portion was in the normal anatomical position. However, the gallbladder was located to the left of the middle hepatic vein and the round ligament, attached to the left lateral segment of the liver. The right posterior portal vein diverged alone from the main portal vein, and there was a long stem from the right anterior and left portal veins. Laparoscopic cholecystectomy confirmed the abnormal location of the gallbladder. Most reported cases of left-sided gallbladder are caused by a right-sided round ligament, which is called a "false" left-sided gallbladder. A case of left-sided gallbladder with a normal left-sided round ligament, which is designated as a case of "true" left-sided gallbladder, is extremely rare.     

Left-sided gallbladder associated with congenital hypoplasia of the left lobe of the liver: a case report and review of literature

Left-sided gallbladder is a rare anomaly that is often associated with other abnormal anatomy in the hepatobiliary system. We report our experience of a case of left-sided gallbladder associated with the congenital hypoplasia of the left lobe of the liver. A 71-year-old woman underwent cholecystectomy for acute cholecystitis. Intraoperative findings revealed the absence of the left lobe of the liver. The gallbladder was located in the left side of the round ligament, which was associated with abnormal intrahepatic portal branching. The incidence of left-sided gallbladder without situs inversus is very rare. The following anomalous anatomy associated with left-sided gallbladder should be a concern when a surgeon encounters a left-sided gallbladder: right-sided round ligament associated with abnormal intrahepatic portal branching and ectopic gallbladder attached to the left lobe of the liver that connects to the left hepatic duct via the cystic duct or the accessory bile duct.     

Extended liver resection for hilar cholangiocarcinoma

Liver resection for hilar cholangiocarcinoma should be designed for individual patients, based on both precise diagnosis of cancer extent and accurate evaluation of hepatic functional reserve. Therefore we have developed various types of hepatic segmentectomy. Combined caudate lobectomy is essential in every patient with separated hepatic confluence. So-called extensive hepatectomy, resection of 50% or more of the hepatic mass, includes right lobectomy and right or left trisegmentectomy. Right lobectomy with caudate lobectomy is indicated when the progression of cancer is predominant in the right anterior and posterior segmental bile ducts. The plane of liver transection is along the Cantlie line, and the left hepatic duct is divided just at the right side of the umbilical portion of the left portal vein. Right trisegmentectomy with caudate lobectomy is performed in carcinoma which involves the right hepatic ducts in continuity with the left medial segmental bile duct. The umbilical portion of the left portal vein is freed from the umbilical plate by dividing the small portal branches arising from the cranial side of the umbilical portion. Then the left lateral segmental bile ducts are exposed and divided at the left side of the umbilical portion of the left portal vein. Left trisegmentectomy with caudate lobectomy is suitable for carcinoma which involves the left intrahepatic bile duct in continuity with the right anterior segmental bile duct. Liver transection is advanced along the right portal fissure. The right posterior segmental bile duct is usually divided distal to the confluence of the inferior and superior branches.     

肝内胆管手术入路的解剖及临床应用

目的 探讨显露肝内叶、段胆管的手术入路。方法 研究30例成人肝脏标本的肝内叶、段胆管与血管的毗邻关系。结果 左右肝管均位于肝脏脏面门静脉门静脉左右干的前上缘,左内叶、右前叶胆管位于相应门静脉的前内侧。右后叶胆管位于门静脉右面支或右前叶下段支脏面深侧者占73％（22／30）;位于门静脉右后支脏面深侧或后上缘者占80％（24／30）。左外叶胆管位于门静脉矢状部脏面深侧者占93％（28／30）。选择经肝的脏面显露肝门、左右肝管,经肝的膈面显露肝内叶、段胆管相结合的手术入路,治疗复杂性肝内胆管结石并狭窄患者38例,均获成功。结论 经肝的脏面与膈面相结合的手术入路,比较容易显露和切开肝内胆管及其狭窄段、便于取出结石。     

Left-sided gallbladder with accessory liver accompanied by intrahepatic cholangiocarcinoma]

A 66-year-old female of left-sided gallbladder with accessory liver was reported. These anomalies were recognized during an operation of intrahepatic cholangiocarcinoma. The gallbladder was located with its liver bed at the lateral segment of the liver. The cystic duct branched from the right side of the common bile duct, and turned in hairpin form in the left direction. The cystic artery branched from the right hepatic artery. There was the accessory liver on the wall of the gallbladder. A careful identification of the cystic duct and the portal vein was advocated in surgery of the left-sided gallbladder.     

Rare Portal Venous Anomaly in a Living Liver Donor: A Case Report

Knowledge of the anatomy of the portal system is essential for safe liver resection. We report a very rare anatomic anomaly of the portal system in a living liver donor. A 24-year-old female living liver donor was found to have anomalies of the portal system on preoperative contrast-enhanced computed tomography. The ventral branch of the right anterior segment arose from the transverse portion of the left portal vein. The gallbladder and round ligament were positioned normally. Intraoperative cholangiography for evaluation of biliary anatomy revealed very low confluence of the right and left hepatic ducts. All the bile ducts from the right lobe merged into the right hepatic duct. A right lobe graft was performed, including the ventral area of the right anterior segment. The portal branch of the ventral area of the right anterior segment could be transected extrahepatically. In the recipient operation, each of the right main portal branches, including the right posterior segment branch and the dorsal branch of the right anterior segment, and the ventral branch of the right anterior segment, were anastomosed to the right and left branches of the portal vein, respectively, of the recipient. The transected right hepatic duct of the graft was anastomosed with the recipient's common hepatic duct. Sixteen years after the liver transplant, the recipient continues to do well and has good portal flow.     

Left-sided gallbladder with right-sided ligamentum teres hepatis: rare associated anomaly of exomphalos

Purpose

The aim of this investigation was to establish the association of left-sided gallbladder and right-sided ligamentum teres hepatis with exomphalos and to clarify the portal venous anomaly associated with right-sided ligamentum teres.

Methods

Three male infants with exomphalos major associated with left-sided gallbladder and right-sided ligamentum teres hepatis were identified during a retrospective study of exomphalos cases (n = 35) in which exomphalos major comprised 18 cases over 19 years.

Results

Three infants with exomphalos major (8.6%) had a left-sided gallbladder and right-sided ligamentum teres hepatis. One male infant survived. Computed tomography showed that there is anomalous intrahepatic portal venous branching associated with right-sided ligamentum teres hepatis. The first branch of the portal vein ran to the right posterior segment, and then the portal vein formed a trunk of the left portal vein and right anterior portal veins. The latter vein formed the umbilical segment of the portal vein and finally joined the ligamentum teres hepatis. The inferior vena cava was on the left side below the kidney and crossed to the right side at the level of the kidney.

Conclusions

Left-sided gallbladder with right-sided ligamentum teres hepatis may not be such a rare associated anomaly in infants with exomphalos. However, there are no reports describing this anomaly with exomphalos in the English medical literature. We believe this is the first report. As long-term survival after repair of exomphalos is increasing, perhaps some of the survivors may develop liver disease which requires liver resection later in life. Recognition of this anomalous relationship with exomphalos is clinically important because it is associated with abnormal intrahepatic portal venous branching and intraabdominal vascular anomalies.     

What's right when the gallbladder's left? A case report

During laparoscopic cholecystectomy for cholelithiasis in a 40-year-old woman, we came upon a case of incidentally discovered left-sided gallbladder (LSG). Two anatomic variants of LSG are known: (a) "true LSG," in which, according to Gross, an accessory gallbladder originates from the left hepatic duct (LHD), the right embryonic bud is readsorbed, and the cystic duct joins either the CBD from the left or the LHD directly. Otherwise, a normal right-sided gallbladder adheres to the inferior left hepatic lobe, and the cystic duct joins the CBD from the right side (as in our case); (b) gallbladder under the fourth hepatic segment, medial to a right-sided round ligament, probably resulting from a prenatal obliteration of the right umbilical vein. Left-sided gallbladder is a paraphysiologic condition that when identified before surgery, must be studied by CT or MRI, when incidentally discovered during surgery must be promptly recognized by the surgeon, who must be aware of the unpredictable confluence of the cystic duct into the CBD. The following operative precautions are useful for avoiding a lesion of the CBD: The surgeon should start dissection of Calot's triangle as close as possible to the gallbladder margin, prepare and clip the cystic duct as close as possible to the infundibulum, and a 30 degrees angled telescope. If in doubt, the surgeon should perform an intraoperative cholangiography.     

Fusion of the planes of the liver: an anatomic entity merging the midplane and the left intersectional plane

BACKGROUND: Alignment of the gallbladder fossa and the round ligament may be associated with an almost unknown portal vein branching anomaly. STUDY DESIGN: Ultrasonographic imaging allowed detection of this anomaly, which we characterized as fusion of the planes of the liver. When appropriate, additional specific radiologic examinations were performed (CT scanner supplemented with a three-dimensional reconstruction, a biliary cartography, or an angiography). Surgical consequences were studied from this series and from the literature. RESULTS: Seven patients (0.5%) had the following criteria: 1) round ligament, gallbladder fossa, and termination of the portal vein occurring in the same plane; 2) typical portal vein branching, including a right posterior branch, left branches, and a main medial branch terminated by the Rex's recessus; 3) two main hepatic veins without a significant middle hepatic vein; and 4) absence of the horizontal part of the left hepatic duct. Fusion of the planes may have been involved in two cases of iatrogenic bile duct injury and contraindicated a tumor resection and a right-liver donation. A review of the literature revealed that lack of recognition of the fusion of the planes led to a high proportion of surgical iatrogenic injury. Fusion of the planes could result from incomplete development of the central part of the liver, in agreement with embryologic knowledge. CONCLUSIONS: Knowledge of the fusion of the planes by hepato-biliary surgeons is important. This anomaly may lead to serious complications if it remains undetected during liver resection or bile duct surgery.     

Surgical anatomy of the left lateral segment as applied to living-donor and split-liver transplantation: a clinicopathologic study

OBJECTIVE: To evaluate intrahepatic vascular and biliary anatomy of the left lateral segment (LLS) as applied to living-donor and split-liver transplantation. SUMMARY BACKGROUND DATA: Living-donor and split-liver transplantation are innovative surgical techniques that have expanded the donor pool. Fundamental to the application of these techniques is an understanding of intrahepatic vascular and biliary anatomy. METHODS: Pathologic data obtained from cadaveric liver corrosion casts and liver dissections were clinically correlated with the anatomical findings obtained during split-liver, living-donor, and reduced-liver transplants. RESULTS: The anatomical relation of the left bile duct system with respect to the left portal venous system was constant, with the left bile duct superior to the extrahepatic transverse portion of the left portal vein. Four specific patterns of left biliary anatomy and three patterns of left hepatic venous drainage were identified and described. CONCLUSIONS: Although highly variable, the biliary and hepatic venous anatomy of the LLS can be broadly categorized into distinct patterns. The identification of the LLS duct origin lateral to the umbilical fissure in segment 4 in 50% of cast specimens is significant in the performance of split-liver and living-donor transplantation, because dissection of the graft pedicle at the level of the round ligament will result in separate ducts from segments 2 and 3 in most patients, with the further possibility of an anterior segment 4 duct. A connective tissue bile duct plate, which can be clinically identified, is described to guide dissection of the segment 2 and 3 biliary radicles.     

Split-liver orthotopic liver transplantation: how to divide the portal pedicle.

It is possible to obtain two good-quality hepatic transplants from a single cadaveric liver by separation of right and left lobes of the liver or section between the left medial segment and the left lateral segment medial to the umbilical cleft. We attempt to define the ideal basis of separation of the structures of the portal pedicle, based on anatomic study of 25 livers. Ideally, the following should be sectioned: the left portal vein, longer and more constant than the right; the right branch of the hepatic artery, larger and more constant than the left; and the left hepatic duct, being aware of the vascularization of the common hepatic duct. Use of the left portal vein necessitates the interruption of all left venous portal branches of segment I, which should be systematically resected. A section between the left medial segment and the left lateral segment interrupts all portal venous branches of the left medial segment, which should then be resected. Before hepatic division, a cholangiogram and arteriogram are indispensable to detect variations and avoid an extensive dissection that may endanger bile duct vascularity.     

Extended right hepatic lobectomy with resection of inferior vena cava and portal vein for intrahepatic cholangiocarcinoma

We report a case of intrahepatic cholangiocarcinoma treated by extended right lobectomy and resection of the inferior vena cava (IVC) and portal vein. A 53-year-old man was referred with elevated serum alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (γ-GTP) levels on April 23, 1999. He was not jaundiced and did not have any symptoms. Endoscopic retrograde cholangiopancreatography (ERCP) revealed irregular strictures in both the anterior and posterior segmental ducts. Contrast-enhanced computed tomography (CT) scan demonstrated a low-density tumor with an unclear margin in the right lobe of the liver. The patient underwent extended right hepatic lobectomy and total caudate lobectomy. Partial resection of the IVC (6 cm) was performed under total hepatic vascular exclusion. The main portal trunk and left portal vein were resected and reconstructed with an end-to-end anastomosis. Macroscopically, a 5.0 × 5.0 × 4.5-cm periductal infiltrating-type tumor occupied the right hepatic parenchyma along the posterior and anterior segmental ducts. Histological examination revealed moderately differentiated tubular adenocarcinoma with marked perineural invasion. Lymph node metastasis was observed in the hepatoduodenal ligament and posterior surface of the pancreatic head. The resected margins of the common bile duct and left hepatic duct were free of tumor. The patient's postoperative course was uneventful, and he was discharged from hospital on the 28th postoperative day. Nine months after the operation, he suddenly developed obstructive jaundice, and died with recurrent disease. This is the first reported case of intrahepatic cholangiocarcinoma treated with major hepatectomy and resection of the IVC and portal vein except ex situ procedure. This aggressive surgical approach may offer hope for patients with intrahepatic cholangiocarcinoma involving the IVC. Received: March 27, 2000 / Accepted: August 8, 2000     

Surgical anatomy of the hepatic hilar area

It is important to understand the main variations of the biliary and vascular elements inside the plate system for hilar bile duct carcinoma because all variations of these elements occur in this plate system. The plate system consists of the hilar plate, cystic plate, and umbilical plate which cover the extrahepatic vascular system and are fused with the hepatoduodenal ligament. The bile duct and vascular system that penetrate the plate system form Glisson's capsule in the liver, but the caudate branch and the medial segmental branch are exceptions. The bile duct and hepatic artery accompanying the plate system can be exfoliated from the portal vein with numerous lymph ducts and nerves. The bile ducts in the right hepatic lobe are classified into 4 types, and the standard type is present in 53-72% of cases. In the left bile duct, the medial segmental bile duct is connected in the vicinity of the hilar area in 35.5% of cases, and these cases should be treated the same as the caudate lobe in hilar bile duct carcinoma. Generally, there is little main variation of the portal vein (16-26%), but more variation in the hepatic artery (31-33%). During surgery for hilar bile duct carcinoma, it is important to observe the plate system and the many variations of the bile duct and vascular system.     

Truncoumbilical bypass of the portal vein in radical resection of biliary tract tumour involving the hepatic duct confluence

Before combined resection of the right hepatic lobe and the hepatoduodenal ligament, an autogenous external iliac vein was interposed between the root of the portal vein trunk and the umbilical portion of the vein in four patients with biliary tract tumour. The left hepatic arterial pathway was preserved, except in one case in whom resection of this artery was followed by reconstruction. This procedure allowed successful and safe extended right hepatic lobe resection and combined en bloc resection of the hepatoduodenal ligament while preserving hepatic blood perfusion.     

Ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system and a right-sided round ligament: Report of a case

We report the case of a ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system (AJPBDS) and a right-sided round ligament, misdiagnosed preoperatively as advanced gastric cancer with pancreatic head invasion. A 72-year-old woman presented with chest pain, but laboratory data showed only anemia. Gastroscopy revealed a bleeding polypoid gastric tumor in the anterior wall of the stomach, herniating into the duodenum (ball-valve syndrome), and a Bormann type-2 tumor in the posterior wall. Ultrasonography showed gallbladder stones, dilatation of the intrahepatic bile duct and pancreatic duct, and a left-sided gallbladder (attributed to a right-sided round ligament with anomalous branches of the portal veins). Laparotomy revealed that the gastric tumors were not advanced cancer invading the pancreatic head. Intraoperative cholangiography showed an AJPBDS, causing dilatation of the intrahepatic bile duct and pancreatic duct. We performed distal gastrectomy and cholecystectomy without biliary diversion. Microscopy revealed that the polypoid tumor was a hyperplastic polyp.