Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by chronic inflammation and stricture formation of the biliary tree. Symptoms include itch and lethargy and i...Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by chronic inflammation and stricture formation of the biliary tree. Symptoms include itch and lethargy and in advanced cases cholangitis and end-stage liver disease, however increasing numbers of asymptomatic individuals are being identified. The disease is rare in the general population but is strongly associated with inflammatory bowel disease (IBD) affecting up to 5% of patients with Ulcerative Colitis, with a slightly lower prevalence (up to 3.6%) in Crohns disease. The strength of this association means that the vast majority ( 〉 90%) of patients with PSC also have IBD, although many may have only mild gastro-intestinal symptoms. Usually IBD presents before PSC, although vice-versa can occur and the onset of both conditions can be separated in some cases by many years. Mean age of diagnosis of PSC is in the fitch decade of life with a strong male predominance. Risk is increased in those with a family history of PSC, suggesting a genetic predisposition and the disease is almost exclusive to non-smokers. The ulcerative colitis associated with PSC is characteristically mild, runs a quiescent course, is associated with rectal sparing, more severe right sided disease, backwash ilieitis and has a high risk of pouchitis post-colectomy. Most worrisome is the high risk of colorectal malignancy which necessitates routine colonoscopic surveillance. Cholangiocarcinoma is also a frequent complication of PSC with a 10%-15% lifetime risk of developing this condition. Treatment with high dose ursodeoxycholic acid offers some chemoprotective effects against colorectal malignancy and may decrease symptoms, biochemical and histological progression of liver disease. Small duct PSC patients characteristically have normal cholangiography, and liver biopsy is required for diagnosis, it appears to have a more favourable prognosis. Autoimmune Hepatitis (AIH) is also more prevalent in patients with IBD, with up to 16% of patients with Autoimmune Hepatitis also having ulcerative colitis. A small subgroup of patients have a AIH-PSC overlap syndrome and the management of these patients depends on liver histology, serum IgM levels, autoantibodies, degree of biochemical cholestasis and cholangiography as some of these patients may respond to immunosupression.展开更多
We report a case of a 74-year-old woman with a 16-year history of a double bilo-enteric anastomosis due to resected hilar cholangiocarcinoma [Type IIIb Klatskin tumor]. The patient presented with cholangitis secondary...We report a case of a 74-year-old woman with a 16-year history of a double bilo-enteric anastomosis due to resected hilar cholangiocarcinoma [Type IIIb Klatskin tumor]. The patient presented with cholangitis secondary to benign anastomotic stenosis which resulted in a large intrahepatic biloma. In order to restore the patency of the anastomosis and overcome cholangitis, several attempts took place, including endobiliary stenting, balloon-assisted biloplasty and transhepatic billiary drainage. Anastomotic patency was achieved, complicated, however, by persistent upper gastro-intestinal bleeding, presented as hemobilia. A biloma-induced pseudoaneurysm of the left hepatic artery was diagnosed. This had ruptured into the biliary tract, and presented the actual cause of the hemobilia. Selective embolism of the pseudoaneurysm resulted in control of the hemorrhage, and was successfully combined with transhepatic dilatation of the anastomosis and percutaneous drainage of the biloma. The patient was ultimately cured and seems to be in excellent condition, 5 mo after treatment.展开更多
Hemobilia is a rare manifestation of hemophilia and is usually iatrogenic following liver biopsy. There are only few reports of spontaneous hemobilia in hemophilia patients. Cholangiocarcinoma is a well-established ca...Hemobilia is a rare manifestation of hemophilia and is usually iatrogenic following liver biopsy. There are only few reports of spontaneous hemobilia in hemophilia patients. Cholangiocarcinoma is a well-established cause of hemobilia. We describe a case of a 70-year-old male, with known haemophilia B and a past history of papillotomy, who presented with classical symptoms of hemobilia. The initial diagnostic work-up failed to demonstrate a potential cause of bleeding other than the coagulopathy. Three months later, he was readmitted to our hospital with a second episode of hemobilia. During the second work-up, a cholangiocarcinoma was diagnosed both by imaging studies and by a significant elevation of cancer antigen 19-9. Although hemobilia could be attributed to hemophilia, especially in a patient with previous papillotomy, an underlying malignancy of the biliary tree should be suspected.展开更多
AIM: Intrabiliary rupture (IBR) is a common and serious compli-cation of hepatic hydatid cyst. The incidence varies from 1% to 25%. The treatment of IBR is still controversial. We aimed to design an algorithm for the ...AIM: Intrabiliary rupture (IBR) is a common and serious compli-cation of hepatic hydatid cyst. The incidence varies from 1% to 25%. The treatment of IBR is still controversial. We aimed to design an algorithm for the treatment of hepatic hydatidosis with IBR by reviewing our cases.METHODS: Eight cases of IBR were analyzed retrospectively.Patients were evaluated according to age, sex, clinical findings, cyst number and stage, abdominal ultrasonography and CT-scan, surgical methods, complica-tions, results and coincidental diseases.RESULTS: Female/male ratio was 1/7. Mean age was 52.12±18.26 years (range 24-69 years). Right upper quadrant pain, flatulence, palpable hepatic mass were symptoms common in all patients. Cholestatic jaundice was found in four cases. In all patients, cyst evacuation and omento-plasty were performed, followed by either choledochod-uodenostomy, T-tube drainage, intracavitary suturing of the orifice, two cases in each. Whereas in two patients diagnosed post-operatively percutaneous drainage of biliary collection or ERCP and sphincteroplasty were added. Morbidity and hospital stay were higher in these cases.CONCLUSION: When the diagnosis of IBR can be done pre-or intra-operatively, morbidity decreases. If a biliary fistula is seen post-operatively, endoscopic procedures such as ERCP, sphincteroplasty or nasobiliary drainage can be applied.展开更多
AIM: To analyze the clinical characteristics of patients diagnosed with Fitz-Hugh-Curtis syndrome.METHODS: The clinical courses of patients that visited St. Mary's Hospital with abdominal pain from January 2005 to...AIM: To analyze the clinical characteristics of patients diagnosed with Fitz-Hugh-Curtis syndrome.METHODS: The clinical courses of patients that visited St. Mary's Hospital with abdominal pain from January 2005 to December 2006 and were diagnosed with Fitz-Hugh-Curtis syndrome were examined.RESULTS: Fitz-Hugh-Curtis syndrome was identified in 22 female patients of childbearing age; their mean age was 31.0 ± 8.1 years. Fourteen of these cases presented with pain in the upper right abdomen alone or together with pain in the lower abdomen, and six patients presented with pain only in the lower abdomen. The first impression at the time of visit was acute cholecystitis or cholangitis in 10 patients and acute appendicitis or pelvic inflammatory disease in eight patients. Twenty-one patients were diagnosed by abdominal computer tomograghy (CT), and the results of abdominal sonography were normal for 10 of these patients. Chlamydia trichomatis was isolated from 18 patients. Two patients underwent laparoscopic adhesiotomy and 20 patients were completely cured by antibiotic treatment.CONCLUSION: For women of childbearing age with acute pain in the upper right abdomen alone or together with pain in the lower abdomen, Fitz-Hugh-Curtis syndrome should be considered during differential diagnosis. Moreover, in cases suspected to be Fitz-Hugh-Curtis syndrome, abdominal CT, rather than abdominal sonography, assists in the diagnosis.展开更多
Fasciola hepatica, a leaf shaped trematode that is common in cattle, sheep and goats, is acquired by eating raw water plants like watercress or drinking water infected with the encysted form of the parasite. The varie...Fasciola hepatica, a leaf shaped trematode that is common in cattle, sheep and goats, is acquired by eating raw water plants like watercress or drinking water infected with the encysted form of the parasite. The varied clinical presentations of fascioliasis still make a high index of suspicion mandatory. Besides having a wide spectrum of hepatobiliary symptoms like obstructive jaundice, cholangitis and liver cirrhosis, the parasitic infection also has extrabiliary manifestations. Until recently, extrahepatic fascioliasis has been reported in the subcutaneous tissue, brain, lungs, epididymis, inguinal lymph nodes, stomach and the cecum. In this report, a strange manifestation of the fasciola infection in a site other than the liver, a colonic fascioliasis, is presented.展开更多
Various benign and malignant conditions could cause biliary obstruction. Compression of extrahepatic bile duct (EBD) by right hepatic artery was reported as a right hepatic artery syndrome but all cases were compresse...Various benign and malignant conditions could cause biliary obstruction. Compression of extrahepatic bile duct (EBD) by right hepatic artery was reported as a right hepatic artery syndrome but all cases were compressed EBD from stomach side. Our case compressed from dorsum was not yet reported, so it was thought to be a very rare case. We present here the first case of bile duct obstruction due to the compression of EBD from dorsum by right hepatic artery.展开更多
We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal p...We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone,after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.展开更多
Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclamps...Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclampsia, syndrome of hemolysis, elevated liver tests and low platelets (HELLP), acute fatty liver of pregnancy, and intrahepatic cholestasis of pregnancy are pregnancy-specific disorders that may cause elevations in liver tests and hepatic dysfunction. Chronic liver diseases, including cholestatic liver disease, autoimmune hepatitis, Wilson disease, and viral hepatitis may also be seen in pregnancy. Management of liver disease in pregnancy requires collaboration between obstetricians and gastroenterologists/hepatologists. Treatment of pregnancy-specific liver disorders usually involves delivery of the fetus and supportive care, whereas management of chronic liver disease in pregnancy is directed toward optimizing control of the liver disorder. Cirrhosis in the setting of pregnancy is less commonly observed but offers unique challenges for patients and practitioners. This article reviews the epidemiology, pathophysiology, diagnosis, and management of liver diseases seen in pregnancy.展开更多
文摘Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by chronic inflammation and stricture formation of the biliary tree. Symptoms include itch and lethargy and in advanced cases cholangitis and end-stage liver disease, however increasing numbers of asymptomatic individuals are being identified. The disease is rare in the general population but is strongly associated with inflammatory bowel disease (IBD) affecting up to 5% of patients with Ulcerative Colitis, with a slightly lower prevalence (up to 3.6%) in Crohns disease. The strength of this association means that the vast majority ( 〉 90%) of patients with PSC also have IBD, although many may have only mild gastro-intestinal symptoms. Usually IBD presents before PSC, although vice-versa can occur and the onset of both conditions can be separated in some cases by many years. Mean age of diagnosis of PSC is in the fitch decade of life with a strong male predominance. Risk is increased in those with a family history of PSC, suggesting a genetic predisposition and the disease is almost exclusive to non-smokers. The ulcerative colitis associated with PSC is characteristically mild, runs a quiescent course, is associated with rectal sparing, more severe right sided disease, backwash ilieitis and has a high risk of pouchitis post-colectomy. Most worrisome is the high risk of colorectal malignancy which necessitates routine colonoscopic surveillance. Cholangiocarcinoma is also a frequent complication of PSC with a 10%-15% lifetime risk of developing this condition. Treatment with high dose ursodeoxycholic acid offers some chemoprotective effects against colorectal malignancy and may decrease symptoms, biochemical and histological progression of liver disease. Small duct PSC patients characteristically have normal cholangiography, and liver biopsy is required for diagnosis, it appears to have a more favourable prognosis. Autoimmune Hepatitis (AIH) is also more prevalent in patients with IBD, with up to 16% of patients with Autoimmune Hepatitis also having ulcerative colitis. A small subgroup of patients have a AIH-PSC overlap syndrome and the management of these patients depends on liver histology, serum IgM levels, autoantibodies, degree of biochemical cholestasis and cholangiography as some of these patients may respond to immunosupression.
文摘We report a case of a 74-year-old woman with a 16-year history of a double bilo-enteric anastomosis due to resected hilar cholangiocarcinoma [Type IIIb Klatskin tumor]. The patient presented with cholangitis secondary to benign anastomotic stenosis which resulted in a large intrahepatic biloma. In order to restore the patency of the anastomosis and overcome cholangitis, several attempts took place, including endobiliary stenting, balloon-assisted biloplasty and transhepatic billiary drainage. Anastomotic patency was achieved, complicated, however, by persistent upper gastro-intestinal bleeding, presented as hemobilia. A biloma-induced pseudoaneurysm of the left hepatic artery was diagnosed. This had ruptured into the biliary tract, and presented the actual cause of the hemobilia. Selective embolism of the pseudoaneurysm resulted in control of the hemorrhage, and was successfully combined with transhepatic dilatation of the anastomosis and percutaneous drainage of the biloma. The patient was ultimately cured and seems to be in excellent condition, 5 mo after treatment.
文摘Hemobilia is a rare manifestation of hemophilia and is usually iatrogenic following liver biopsy. There are only few reports of spontaneous hemobilia in hemophilia patients. Cholangiocarcinoma is a well-established cause of hemobilia. We describe a case of a 70-year-old male, with known haemophilia B and a past history of papillotomy, who presented with classical symptoms of hemobilia. The initial diagnostic work-up failed to demonstrate a potential cause of bleeding other than the coagulopathy. Three months later, he was readmitted to our hospital with a second episode of hemobilia. During the second work-up, a cholangiocarcinoma was diagnosed both by imaging studies and by a significant elevation of cancer antigen 19-9. Although hemobilia could be attributed to hemophilia, especially in a patient with previous papillotomy, an underlying malignancy of the biliary tree should be suspected.
文摘AIM: Intrabiliary rupture (IBR) is a common and serious compli-cation of hepatic hydatid cyst. The incidence varies from 1% to 25%. The treatment of IBR is still controversial. We aimed to design an algorithm for the treatment of hepatic hydatidosis with IBR by reviewing our cases.METHODS: Eight cases of IBR were analyzed retrospectively.Patients were evaluated according to age, sex, clinical findings, cyst number and stage, abdominal ultrasonography and CT-scan, surgical methods, complica-tions, results and coincidental diseases.RESULTS: Female/male ratio was 1/7. Mean age was 52.12±18.26 years (range 24-69 years). Right upper quadrant pain, flatulence, palpable hepatic mass were symptoms common in all patients. Cholestatic jaundice was found in four cases. In all patients, cyst evacuation and omento-plasty were performed, followed by either choledochod-uodenostomy, T-tube drainage, intracavitary suturing of the orifice, two cases in each. Whereas in two patients diagnosed post-operatively percutaneous drainage of biliary collection or ERCP and sphincteroplasty were added. Morbidity and hospital stay were higher in these cases.CONCLUSION: When the diagnosis of IBR can be done pre-or intra-operatively, morbidity decreases. If a biliary fistula is seen post-operatively, endoscopic procedures such as ERCP, sphincteroplasty or nasobiliary drainage can be applied.
文摘AIM: To analyze the clinical characteristics of patients diagnosed with Fitz-Hugh-Curtis syndrome.METHODS: The clinical courses of patients that visited St. Mary's Hospital with abdominal pain from January 2005 to December 2006 and were diagnosed with Fitz-Hugh-Curtis syndrome were examined.RESULTS: Fitz-Hugh-Curtis syndrome was identified in 22 female patients of childbearing age; their mean age was 31.0 ± 8.1 years. Fourteen of these cases presented with pain in the upper right abdomen alone or together with pain in the lower abdomen, and six patients presented with pain only in the lower abdomen. The first impression at the time of visit was acute cholecystitis or cholangitis in 10 patients and acute appendicitis or pelvic inflammatory disease in eight patients. Twenty-one patients were diagnosed by abdominal computer tomograghy (CT), and the results of abdominal sonography were normal for 10 of these patients. Chlamydia trichomatis was isolated from 18 patients. Two patients underwent laparoscopic adhesiotomy and 20 patients were completely cured by antibiotic treatment.CONCLUSION: For women of childbearing age with acute pain in the upper right abdomen alone or together with pain in the lower abdomen, Fitz-Hugh-Curtis syndrome should be considered during differential diagnosis. Moreover, in cases suspected to be Fitz-Hugh-Curtis syndrome, abdominal CT, rather than abdominal sonography, assists in the diagnosis.
文摘Fasciola hepatica, a leaf shaped trematode that is common in cattle, sheep and goats, is acquired by eating raw water plants like watercress or drinking water infected with the encysted form of the parasite. The varied clinical presentations of fascioliasis still make a high index of suspicion mandatory. Besides having a wide spectrum of hepatobiliary symptoms like obstructive jaundice, cholangitis and liver cirrhosis, the parasitic infection also has extrabiliary manifestations. Until recently, extrahepatic fascioliasis has been reported in the subcutaneous tissue, brain, lungs, epididymis, inguinal lymph nodes, stomach and the cecum. In this report, a strange manifestation of the fasciola infection in a site other than the liver, a colonic fascioliasis, is presented.
文摘Various benign and malignant conditions could cause biliary obstruction. Compression of extrahepatic bile duct (EBD) by right hepatic artery was reported as a right hepatic artery syndrome but all cases were compressed EBD from stomach side. Our case compressed from dorsum was not yet reported, so it was thought to be a very rare case. We present here the first case of bile duct obstruction due to the compression of EBD from dorsum by right hepatic artery.
文摘We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone,after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.
文摘Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclampsia, syndrome of hemolysis, elevated liver tests and low platelets (HELLP), acute fatty liver of pregnancy, and intrahepatic cholestasis of pregnancy are pregnancy-specific disorders that may cause elevations in liver tests and hepatic dysfunction. Chronic liver diseases, including cholestatic liver disease, autoimmune hepatitis, Wilson disease, and viral hepatitis may also be seen in pregnancy. Management of liver disease in pregnancy requires collaboration between obstetricians and gastroenterologists/hepatologists. Treatment of pregnancy-specific liver disorders usually involves delivery of the fetus and supportive care, whereas management of chronic liver disease in pregnancy is directed toward optimizing control of the liver disorder. Cirrhosis in the setting of pregnancy is less commonly observed but offers unique challenges for patients and practitioners. This article reviews the epidemiology, pathophysiology, diagnosis, and management of liver diseases seen in pregnancy.