Suprahepatic gallbladder and right lobe anomaly of the liver in patients with biliary cancers

Summary The suprahepatic region is a rare ectopic location of the gallbladder. It usually combines with right lobe anomaly of the liver. Here we report two unusual cases of suprahepatic gallbladder with agenesis or hypogenesis of the right lobe of the liver and biliary cancer. A patient with a gallbladder tumor was admitted to our emergency room with acute cholecystitis and liver abscess. Imaging examinations and operation confirmed the suprahepatic position of gallbladder, agenesis of the right lobe, and dissemination of gallbladder cancer. In the patient with cholangiocarcinoma, CT scans and percutaneous transhepatic cholangiography documented the presence of a hilar tumor and hypogenesis of the right lobe. Both of these patients died from biliary tract cancer soon after operation.     

Hypogenesis of right hepatic lobe accompanied by portal hypertension: Case report and review of 31 Japanese cases

We report a rare case of hypogenesis of the right lobe of the liver with portal hypertension and a review of 31 cases of agenesis or hypogenesis of the right hepatic lobe reported in Japan. A 74-year-old man consulted our hospital for further examination after a mass screening for gastric cancer. On physical examination liver enlargement was palpable, but liver function tests were normal. Abdominal ultrasonography, computed tomography, technetium-99m liver scintigraphy, and endoscopic retrograde cholangiopancreatography revealed a small right hepatic lobe and moderate splenomegaly, in contrast to a hypertrophic lateral segment of the left hepatic lobe, as well as ectopic dislocation of the gallbladder. Endoscopic examination revealed esophageal varices, indicating portal hypertension. Abdominal angiography demonstrated mild shunt flow between the hepatic artery feeding from the gastroduodenal artery and the portal vein. A biopsy specimen taken from both lobes of the liver showed normal liver tissue histologically. Based on these findings, we made a definite diagnosis of hypogenesis of the right lobe of the liver with portal hypertension. The present case appears to be the first such case accompanied by portal hypertension reported in Japan.     

CT和MRI对肝右叶发育不良的诊断价值探讨

目的探讨电子计算机断层扫描（CT）和磁共振成像（MRI）对肝右叶发育不良的诊断价值。方法选取24例在本院行CT和MRI扫描的肝右叶发育不良患者,结合文献资料综合分析患者肝脏CT和MRI影像学检查特点。结果本组肝右叶发育不良患者24例,男性 8例,女性16例;年龄在40~85岁,平均年龄为（60.7±3.2）岁;采用MRI检查发现者6例,采用CT检查发现者18例,两种检查方法诊断者4例;影像学检查表现为肝右叶体积缩小,门静脉及其分支出现细小或者缺如。肝左叶增大18例,肝左叶正常6例。尾状叶体积缩小,伴有尾叶内发生胆汁淤积3例,尾状叶体积增大者15例。14例出现肝外胆管结石,12例胆囊右后旋,8例患者行胆囊切除。24例患者均出现了肝内胆管扩张,12例出现脾脏增大,9例伴发肝脏肿瘤,并发腹膜转移1例。结论CT和MRI检查对于诊断肝右叶发育不良具有重要的临床意义。     

Agenesis of the lung. Report of four patients with unusual anomalies

Four patients had pulmonary agenesis. All were symptomatic in infancy. In three, symptoms improved during two to six years of follow-up. One, who died at one month, had a unique combination of agenesis of the right upper and middle lobes and a hypoplastic right lower lobe supplied by systemic arteries from below the diaphragm, total anomalous pulmonary venous drainage to the left superior vena cava, tricuspid regurgitation with severe right atrial dilatation, patent ductus arteriosus, and reduplication of the right thumb. Abnormalities of the thumb were encountered in three patients. Parental consanguinity in all four patients suggests an autosomal recessive mode of inheritance. Cardiac catheterization and angiography are essential procedures for diagnosis and elucidation of the anatomic abnormalities and identification of associated cardiac defects.     

Pulmonary developmental anomaly associated with Klippel-Feil syndrome and anomalous atrioventricular conduction.

We report the findings in a patient with Klippel-Feil syndrome and associated agenesis of right upper and middle lobes, hypoplasia of the right lower lobe of the lung, and Lown-Ganong-Levine syndrome. To our knowledge, such an association has not been previously described.     

Bilobar Thyroid Agenesis

A case of a 27-year-old female patient presenting with bilateral agenesis of the thyroid gland and a benign euthyroid adenoma of the isthmus and the pyramidal lobe is reported. The patient had for several years been followed for a cystic nodule that was presumed to have developed from the left thyroid lobe, having recently increased in size. Scintigraphic and echographic studies evoked the absence of right lobe. Direct examination at cervicotomy found hypoplasia of the two lobes. This report highlights the similarity of this rare anomaly to widely known hemiagenesis.     

A comparative study of the anatomy of rat and human livers

To compare the fundamental structure of the human liver, in relation to that of the rat a comparative study was performed, in which 20 rat livers and 78 human cadaver livers were examined. The rat livers had four lobes (left, middle, right, and caudate). The left and middle lobes formed a single lobe but the middle lobe had a deep notch to which the round ligament attached. The right lobe was split into two sub-lobes and the caudate lobe was divided into the paracaval portion and the Spiegel lobe, which was split into two sub-lobes. The left, right, and caudate lobes had one primary portal branch, whereas the middle lobe had two portal branches. The left and the right sub- and caudate lobes had one large hepatic vein each, whereas three large hepatic veins were observed in the middle lobe. Based on the ramifying patterns of the portal and hepatic veins, the rat middle lobe possessed left and right hepatic components and a main portal fissure. The following rat hepatic lobes were equivalent to the following human liver segments: the left lobe to segment II; the middle lobe to segments III, IV, V, and VIII; and the right lobe to segments VI and VII. The fundamental structures of rat and human livers were similar, and the findings demonstrated a new interpretation of the anatomy of the human liver.     

Fulminant liver failure as a sequelae of acute dystrophy of the right hepatic lobe

A 38-year-old female patient developed signs of fulminant hepatic failure, caused by extensive, spontaneous, devastating ischaemia of the right liver lobe. The patient survived the acute attack. During the subsequent eight years hypertrophy of the intact left liver lobe occurred which eventually completely replaced the loss of the dominant right lobe.     

Pancreatic pseudocysts of the right hepatic lobe during acute biliary pancreatitis

The occurrence of pancreatic pseudocysts of the right hepatic lobe during acute biliary pancreatitis is a rare event. We report the unusual case of a 69-year-old woman who was hospitalised for biliary pancreatitis. The patient suffered from right hypochondrial pain. A CT-scan performed at day 12 showed pancreatic pseudocysts in the right hepatic lobe. A favorable outcome was obtained after percutaneous drainage. Most hepatic pseudocysts are described in the left hepatic lobe after alcoholic pancreatitis. Different hypotheses have been suggested to explain the extension of pancreatic pseudocysts in the liver, due to proteolytic effect of pancreatic enzymes that reach the lesser sac and then the liver either directly through the liver capsule, or indirectly through the hepatic hilum vessels, or the hepatic ligament. We suggest another reason for hepatic invasion: pancreatic enzymes could also cause liver damage, through the para - renal anterior space, often infiltrated during acute pancreatitis, reaching right hepatic lobe through area nuda.     

Peritoneoscopy for the diagnosis of left lobe lesions of the liver.

Peritoneoscopy and direct-vision liver biopsy are simple, safe, and accurate methods for the documentation of focal lesions predominantly involving the left lobe of the liver or the left portion of the right lobe. Focal lesions of the left lobe of the liver constitute a major indication for peritoneoscopy and direct-vision biopsy of the liver when its use is not contraindicated and when blind percutaneous biopsy or biopsies and cytologic examination have failed to establish the diagnosis.     

Selective hypertrophy of the lobus caudatus as a novel approach enabling extended right hepatectomy in the presence of a non-perfused left lateral liver lobe

Introduction

Portal vein embolization (PVE) is a well-established technique to enhance functional hepatic reserves of segments II and III before curative extended right hepatectomy for tumors of the right liver lobe. However, an adequate hepatopetal flow of the left lateral portal vein branches is required for a sufficient PVE-associated hypertrophy.

Case report

Here, we report a 65-year old patient suffering from a locally advanced intrahepatic cholangiocarcinoma in the right liver lobe and segment IV. A curative extended right hepatectomy after preoperative PVE of liver segments IV–VIII was initially impossible because of partial thrombosis of the left lateral portal vein branches resulting in an ischemic-type atrophy of segments II and III. However, due to a massive hypertrophy of the caudate lobe following PVE of liver segments IV–VIII, subsequent extended right hepatectomy with intraoperative thrombectomy of segments II and III was made possible.

Conclusions

To our knowledge this is the first case in which an extended right hepatectomy for a liver malignancy, in the presence of atrophic left lateral section, was made possible by a massive PVE-associated hypertrophy of the caudate lobe.     

Dynamic studies on the portal hemodynamics of scintiphotosplenoportography. Streamline flow in the human portal vein.

To demonstrate streamline flow in the human portal vein, the hepatic lobar distribution of splenic flow was studied in 6 patients without liver disease and 4 patients with chronic hepatitis using a new technique, scintiphotosplenoportography, followed by 198Au-colloid liver scintigraphy. In two patients, a 99mTcO4-bolus injected into the spleen drained predominantly into the left lobe of the liver. A repeat examination performed in one patient revealed that the bolus was chiefly directed to the other lobe, the right lobe. In 3 patients, the distribution of 99mTcO4- radioactivity was the same as that observed with 198Au-colloid. In the other 5 patients the predominant distribution of 99mTcO4- was observed in the right lobe. In 8 of 11 studies, splenic flow was distributed either to the right or left lobe instead of diffusely to both lobes. This result suggests that flow in the human portal vein is streamline rather than turbulent.     

裸露DNA经胆道分支导入活体肝脏的局部表达

目的 经胆道分支逆行导入裸露DNA,观察目的基因在正常及急性损害大鼠局部肝脏的定量和定位表达.方法 运用D-氨基半乳糖制作大鼠急性肝损害模型;经胆管分支将Cy3荧光标记CMV β质粒通过胆道分支逆行导入局部肝脏,观察裸露DNA导入肝脏的情况.同时运用包含萤火虫荧光素基因质粒pGL3及包含大肠埃希氏菌β半乳糖苷酶基因质粒pCMV β逆行导入大鼠局部肝脏,定量检测荧光素酶和定位检测β半乳糖苷酶在肝脏的表达.应用非参数Kruskal-Wallis法秩和检验进行统计分析.结果 肝组织冰冻切片观察到Cy3荧光标记DNA在汇管区附近聚集.按100 μg/ml浓度进行基因导入,荧光素酶基因在正常大鼠肝脏,急性损害大鼠肝脏均有显著表达.大肠埃希氏菌β半乳糖苷酶定位检测结果显示正常大鼠肝脏及急性肝损害大鼠肝脏内有β半乳糖苷酶显著表达,并可见较多肝细胞表达β半乳糖苷酶. 结论 裸露DNA经胆道逆行导入正常及急性损害肝脏局部能获得较好表达,且无明显加重肝脏损害,该项方法为肝脏基因治疗的实验研究提供了良好的技术平台.     

Hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis

A 22-year-old man was referred to our hospital because of thrombocytopenia. Abdominal computed tomography (CT) revealed hypoplasia of the right hepatic lobe, the development of porto-systemic collateral vessels, splenomegaly and a periaortic soft-tissue mass. Laboratory tests and needle liver biopsy indicated no evidence of liver cirrhosis. Consequently, a diagnosis of hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis was established. Portal hypertension and hypersplenism was thought to be the cause of the thrombocytopenia. CT arterioportography revealed that anomalies of the portal venous system could have resulted in the hypoplasia of the right hepatic lobe. This is the first report describing hypoplasia of the right hepatic lobe accompanied by supervening idiopathic retroperitoneal fibrosis.     

Clinical study on safety of adult-to-adult living donor liver transplantation in both donors and recipients

AIM: To investigate the safety of adult-to-adult living donor liver transplantation (A-A LDLT) in both donors and recipients. METHODS: From January 2002 to July 2006, 50 cases of A-A LDLT were performed at West China Hospital, Sichuan University, consisting of 47 cases using right lobe graft without middle hepatic vein (MHV), and 3 cases using dual grafts (one case using two left lobe, 2 using one right lobe and one left lobe). The most common diagnoses were hepatitis B liver cirrosis, 30 (60%) cases; and hepatocellular carcinoma, 15 (30%) cases in adult recipients. Among them, 10 cases had the model of end-stage liver disease (MELD) with a score of more than 25. Donor screening consisted of reconstruction of the hepatic blood vessels and biliary system with 3-dimension computed tomography and volumetry of whole liver and right liver volume. Various improved surgical techniques were adopted in the procedures for both donors and recipients . RESULTS: Forty-nine right lobes and 3 left lobes (2 left lobe grafts for 1 recipient, 1 left lobe graft for 1 recipient who had received right lobe graft donated by relative living donor) were obtained from 52 living donors. The 49 right lobe grafts, without MHV, weighed 400 g-850 g (media 550 g), and the ratio of graft volume to recipient standard liver volume (GV/SLV) ranged from 31.74% to 71.68% (mean 45.35%). All donors' remnant liver volume was over 35% of the whole liver volume. There was no donor mortality. With a follow-up of 2-52 mo (media 9 mo), among 50 adult recipients, complications occurred in 13 (26%) cases and 4 (8%) died postoperatively within 3 mo. Their 1-year actualsurvival rate was 92%. CONCLUSION: When preoperative CT volumetry shows volume of remnant liver is more than 35%, the ratio of right lobe graft to recipients standard liver volume exceeding 40%, A-A LDLT using right lobe graft without MHV should be a very safe procedure for both donors and recipients, otherwise dual grafts liver transplantation should be considered.     

Assessment of Hepatic Functional Reserve in Cirrhotic Patients by Computed Tomography of the Caudate Lobe

In order to investigate whether the size of thecaudate lobe of the cirrhotic liver is related to thehepatic functional reserve, the morphometric analysis ofthe caudate lobe was preformed retrospectively using computed tomography in 106 consecutivepatients of whom 67 had compensated (group 1), and 39uncompensated (group 2) liver cirrhosis. In 51 patients,hepatic measurements were repeated in follow-up. Age- and gender-matched controls were studied.The size of the caudate lobe and the ratio of thecaudate to right lobe were correlated with liverfunction. The caudate lobe was larger in the studypatients than in the controls, and larger in group 1than in group 2 (P < 0.01). The caudate to right loberatios were also greater in the study patientsespecially in group 1 (P < 0.01). In follow-up, the regression coefficient for the caudate to rightlobe ratio was positive in group 1 and negative in group2 (P < 0.05). Even though the caudate lobe ofpatients with uncompensated liver cirrhosis was larger than that of the controls, patients withcompensated liver cirrhosis had a larger caudate lobeand higher caudate to right lobe ratio compared to thepatients with uncompensated liver cirrhosis. The caudate lobe may have played an importantrole in maintaining proper liver function in thesepatients.     

Riedel's Lobe of the Liver: A Case Report

Riedel lobe of the liver is a simple anatomical variation, a downward tongue-like projection of the anterior edge of the right lobe of the liver to the right of the gallbladder with its typical case to be rare.We report the case of a 71-year-old woman with typical feature of a nonpalpable Riedel''s lobe of the liver, as an incidental finding who was referred for reported hypergammaglobulinemia (22.7% [9%–19%]). Both features were attributed to a chronic inflammation because of an abscess in the right iliopsoas caused by infection due to bilateral hip replacement which underwent revision surgery. This was confirmed by her medical history, the imaging findings combined with elevated C-reactive protein, and by cross-reaction weak positive autoantibodies.Generally, knowledge or suspicion of Riedel''s lobe of the liver is important, as it does not always remain clinically latent, as in our case, and it can be complicated by its torsion or hepatic tumors.     

Marked atrophy of the right lobe seen in idiopathic portal hypertension confirmed by laparoscopy

A 70‐year‐old‐male was hospitalized for the treatment of esophageal varices and close examination of the liver. Blood chemistry tests revealed mild liver dysfunction. Abdominal ultrasound and computed tomography scan revealed marked atrophy of the right and quadrate lobes of the liver without abnormalities of the biliary system. Abdominal angiography revealed marked atrophy of the right lobe of the liver, without obliteration in the portal venous system, but it could not be determined whether the atrophy was congenital or secondary. Subsequently performed laparoscopy revealed marked atrophy of the anterior segment of the right lobe and quadrate lobe with the whitish scarred edge demarcating the border between the edge and neighboring liver parenchyma. The liver surface appeared to be undulant, but non‐cirrhotic. These findings suggest secondary lobar atrophy of the liver, without cirrhosis. Liver biopsy of the left lobe showed the findings to be compatible with idiopathic portal hypertension (IPH), and we diagnosed IPH based on these findings and hepatic lobar atrophy was attributable to IPH. There have been few reports of cases with hepatic lobar atrophy associated with IPH, and the mechanism of atrophy is unclear. We report a case of IPH with marked liver atrophy in which laparoscopy is a decisive means whether liver atrophy is congenital or secondary.     

Efficacy of only left side approach in a case of unsuccessful reconstruction of the right hepatic artery after left hepatic lobectomy with caudal lobectomy

We report two cases that underwent extended left hepatic lobectomy combined with resection of the caudate lobe and extrahepatic bile duct only from the left side approach for hilar cholangiocarcinoma. The first case was a 54-year-old man and the second one was a 63-year-old man. Both patients had hilar cholangiocarcinoma with predominant left hepatic duct involvement and required resection and reconstruction of the right hepatic artery as well as left hepatic lobectomy. In both cases, the right hepatic lobe was never mobilized to protect the mechanical damage in the remnant liver and keep co-lateral blood supply route to the remnant liver from the diaphragm or retroperitoneum. Although arterial blood flow to the remnant right hepatic lobe was unfortunately insufficient after reconstruction of the right hepatic artery, the postoperative course was uneventful. The postoperative angiography showed co-lateral arterial blood supply to the right lobe via the subdiaphragmatic artery. In case of extended left hepatic lobectomy combined with resection of the caudate lobe and right hepatic artery, ipsilateral approach (approach only from the left side) is recommended.     

Isolated congenital spleen agenesis: a rare cause of chronic thromboembolic pulmonary hypertension in an adult

This report describes a case of isolated congenital spleen agenesis complicated by chronic thromboembolic pulmonary hypertension (CTPH) in a 44-year-old female patient. The patient had increasing exertional dyspnoea and thrombocytosis. An echocardiogram showed severe pulmonary hypertension and right ventricular hypertrophy, and contrast-enhanced chest CT revealed multiple thromboemboli within both pulmonary arteries. A perfusion lung scan demonstrated multiple segmental defects and no spleen was detected by abdominal CT, ultrasonography or scintigraphy. Comprehensive clinical examinations disclosed no evidence of a thrombus elsewhere or of an associated malformation such as a cardiac anomaly. Anticoagulation therapy was started, and a perfusion lung scan revealed partial improvement of the hypoperfusion in the right lower lobe. However, repeat echocardiography showed the pulmonary hypertension persisting for 1 year. The multiple segmental defects in the perfusion lung scans were also persistent. Collectively, a diagnosis of CTPH with isolated congenital spleen agenesis was established. This is the first documented case of CTPH in an adult with isolated congenital asplenia. Although congenital spleen agenesis is a rare condition, this case report suggests that this possibility should be considered when a diagnosis of CTPH and thrombocytosis is made.