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BILIARY TRACT DISEASE Dr Julia Epstein Gastroenterology department Hadassah Medical center Biliary disease • Gallstones • • • • Congenital anomalies Extrinsic compression of the bile ducts Hepatobiliary parasitism Noncancerous strictures Biliary tract anatomy Ampullary anatomy Biliary duct sphincter Papilla of Vater Pancreatic duct sphincter Sphincter of Oddi כיס מרה מבנה אגסי 7.5-10ס"מ • תכולה 30-50ס"מ• • מרה מיוצרת←כבד• • מרה נאגרת←כיס מרה• • אוכל שומני←תרסריון←הפרשת •CCK מרה מתכווץ + ספינקטר ע"ש אודי נפרה ← מעבר מיצי • מרה לתריסריון • ספיגה מחדש לוריד הפורטלי←חזרה • Enterohepatic Circulation :לכבד כבד←מרה←מעי • מיצי מרה מכיל :מים ,אלקטרוליטיים - Na, K, Ca, Cl, HCO3 לציטין,חומצות שומן ,כולסטרול, בילירובין ומלחי מרה מאיפה מגיע בילירובין? תפקיד: – ספיגה של השומנים לאחר תהליך אמולסיפיקציה – שפעול מיצי הלבלב – המרה (בסיסית) סותרת את מיץ הקיבה החומצי המגיע לתריסריון. מבנה מרה מלחי המרה ← מסיסים במים • פוספוליפידים +הכולסטרול ← לא מסיסים במים • הופכים למסיסים שנוצרות מיצלות • היחס בין שלושת המרכיבים עדין • כל חריגה מהיחס המסוים ← • מרה רוויה +שקיעה של • כולסטרול (יצירת אבנים) • Principal pathways of cholesterol metabolism in liver Gallstones – Pathophysiology • Cholesterol, ordinarily insoluble in water, comes into solution by forming vesicles with phospholipids • If ratio of cholesterol, phospholipids, and bile salts altered, cholesterol crystals may form • Gallstone formation involves a variety of factors: – Cholesterol supersaturation – Mucin hypersecretion by the gallbladder mucosa creates a viscoelastic gel that fosters nucleation. – Bile stasis Gallstone pathogenesis Cholesterol supersaturation of bile Nucleation Motility stasis of cholesterol molecules to crystals Gallstones • • • • • • • • Risk factors: Obesity Female gender Parity Maternal family history ( ABCB4 , CYP7A1) Ethnic predilection (Hispanic, Native americans) Increasing age Rapid weight loss Gallstones • Risk factors: Ileal disease Lipid abnormalities (high TG, low LDL) Medications (contraceptives, postmenopausal estrogens, lipid lowering agents fibric acid derivated) • TPN 5 F’s • Fair • Fat • Fertile • Female • Forty מצבים שכיחים • • • • • • • Cholelithiasis Biliary colic Hydrops Empyema Choledocholithiasis Acute Cholecystitis Ascending Cholangitis Gallstones – Types • Two main types: – Cholesterol stones (85%) – Pigment stones (15%) occur in 2 subtypes— brown and black. • Black stones result when excess bilirubin enters the bile and polymerizes into calcium bilirubinate (chronic hemolysis) • Brown stones are made up of calcium bilirubinate and calciumsoaps. Bacteria involved in formation via secretion of beta glucuronidase and phospholipase Gallstones – Natural History • 80% of patients, gallstones are clinically silent • 20% of patients develop symptoms over 15-20 years (1-2% per year) • 50-70% continued to have symptoms and complications • More than 90% of complications are preceded by biliary colic Biliary Colic • Intermittent obstruction of the cystic duct, no inflammation of GB • Severe epigastric/ RUQ pain growing over 15 min and remaining constant up to 3h • Frequency of attacks varies • Normal examination • Lab tests usually normal • DS: US, EUS • Natural history: 30% have no further symptoms 50% continue symptoms in 2y Acute Calculous Cholecystitis • Impacted stone in the cystic duct • 75% are preceded by attacks of biliary colic • Visceral epigastric pain – mod to severe, irradiated to RUQ, back, shoulder, chest and lasting > 6 h • Fever, Right subcostal tenderness with inspiratory arrest ( Murphy’s sign) , palpable GB • Leucocytosis, mild elevation of BIL, Amylase • 50% resolve spontaneously in 7-10 days without surgery • DS: US, EUS, CT • 10% are complicated by perforation. Empyema / Mucocoele • Mucocele refers to an overdistended gallbladder filled with mucoid or clear and watery content. • Empyema refers to a gallbladder filled with pus due to acute cholecystitis Choledocholithiasis • Intermittent obstruction of CBD • Often symptomatic – indistinguishable from biliary colic • Predisposed to acute cholangitis and pancreatitis • Signs : jaundice with pain. • Investigations – Elevated BIL, transient spike in Tranaaminases / Amylase, – US, EUS, CT • Treatment – ERCP - Endoscopic Retrograde CholangioPancreatography Ascending Cholangitis •Impacted stone in CBD causing bile stasis •Bacterial superinfection •Charcot’s triad : pain, jaundice, fever – 70% •Mental confusion, hypotension, RUQ tenderness •Jaundice (>80%) •Peritoneal signs (15%) •Elevated WBC, BIL, APH (blood cult usually pos) •Emergent decompression of the CBD (ERCP, PTC) Acute biliary pancreatitis • • Pancreatic duct obstruction or chemical inflammation • Signs - Variable – None to Sepsis (Severe pain, fever, tachycardia, low BP), Jaundice, acute abdomen Investigations • Bloods – U&E, FBC, LFT, Amylase, CRP • Ultrasound of abdomen • MRCP • CT Pancreas • Treatment Supportive / ERCP Gallstone ileus • Obstruction of the small bowel by a large gallstone –A stone ulcerates through the gallbladder into the duodenum and causes obstruction at the terminal ileum/rt colon • Symptoms : SBO - vomiting, abdominal pain, distension, obstructive bowel sounds • Investigations: X-ray, US/CT - air in CBD • Treatment : Laparotomy and removal of stone from small bowel and cholecystectomy. Mirizzi’s Syndrome Inflammatory phenomenon secondary to a pressure ulcer caused by an impacted gallstone at the gallbladder infundibulum The impacted gallstone causes first external obstruction of the CBD Eventually erodes into the bile duct evolving into a cholecystocholedochal fistula with different degrees of communication between the GB and CBD Mirizzi’s Syndrome Lemmmel’s Syndrome Duodenal diverticula syndrome Secondary to extrinsic compression by periampullary diverticula in the absence of additional pathology (cholelithiasis, tumor) • The hypothesized mechanisms: • Alterations of the papillary motility • Bacterial contamination • Extrinsic compression of the Main biliary tract Lemmmel’s Syndrome Acute Acalculous Cholecystitis • Presence of an inflamed gallbladder in the absence of an obstructed cystic or common bile duct • Typically occurs in the setting of a critically ill patient (eg, severe burns, multiple traumas, lengthy postoperative care, prolonged intensive care) • Accounts for 5% of cholecystectomies • Etiology is thought to have ischemic basis, and gangrenous gallbladder may result • Increased rate of complications and mortality • An uncommon subtype known as acute emphysematous cholecystitis generally is caused by infection with clostridial organisms and occlusion of the cystic artery associated with atherosclerotic vascular disease and, often, diabetes. Cholecystectomy • Laparoscopic cholecystectomy standard of care • Timing – Early vs interval operation • Patient consent – Conversion to open procedure 10% – Bleeding – Bile duct injury – Damage to other organs Biliary disease • Gallstones • Congenital anomalies • Extrinsic compression of the bile ducts • Hepatobiliary parasitism • Noncancerous strictures Biliary Tract Cysts • Choledochal cysts • Consist of cystic dilatations of the extrahepatic biliary tree • Uncommon abnormality and 90% diagnosed before age 30 • Infantile form presentation identical to biliary atresia • 50% present with combination of jaundice, abdominal pain, and an abdominal mass Choledochal cysts • Classified into 5 types • Can occur in the presence of pancreaticobiliary maljunction (PBM) • Treatment for choledochal cysts is surgical excision of the cyst with construction • Most ominous complication is malignancy Choledochal cysts PBM Caroli disease Biliary ductopenic disorders • Paucity of interlobular bile ducts 2 types: syndromic and non syndromic Syndromic ( Alagille’s syn) ADD d/t JAG1 gene Intrahepatic cholestasis and biliary hypoplasia Pruritus and hepatomegaly Extrahepatic manifeatations - congenital heart defects, eye defects, triangular face Biliary ductopenic disorders • interlobular ductopenia as result of inflammatory condition: • PSC • PBC • GVHD • Liver allograft resection • Drug induced liver disease • idiopathic Biliary disease • Gallstones • Congenital anomalies • Extrinsic compression of the bile ducts • Hepatobiliary parasitism • Noncancerous strictures Extrinsic compression of the bile ducts • • • • • Biliary tract tumor Carcinoma of the head of pancreas Acute and chronic pancreatitis Lymph nodes – lymphoma or metastasis Benign stricture of biliary ducts Biliary Tract Tumor Cholangiocarcinoma Cancer of the Gall Bladder Cholangiocarcinoma • Slow growing malignancy of biliary tract which tend to infiltrate locally and metastasize late • 90% adenocarcinoma • 60-70% at the bifurcation ofhepatic ducts • 20-30% - in the distal CBD • 5-10% - arise within the liver (peripheral) Biliary Tree Neoplasms • Clinical symptoms: – – – – – – • Weight loss (77%) • Nausea (60%) • Anorexia (56%) Abdominal pain (56%) • • Fatigue (63%) Pruritus (51%) Fever (21%) Malaise (19%) Diarrheoa (19%) Constipation (16%) Abdominal fullness (16%). • Symptomatic patients usually have advanced disease, with spread to hilar lymph nodes before obstructive jaundice occurs • Associated with a poor prognosis Risk factors • Liver flukes (Opistorchis viverrini, Chlonorchis sinensis) • Chemial exposition (Asbestosis) • Congenital predisposition (PBM, Choledochal cysts) • Intrahepatic biliary stones • PSC Cholangiocarcinoma Diagnosis and Initial Workup • Jaundice • Weight loss, anorexia, abdominal pain, fever • • • • • US – bile duct dilatation 3-phase CT MRCP/MRI ERCP with Brush biopsy Percutaneous Cholangiography with Internal Stent and Brush Biopsy MRCP: Cholangiocarcinoma at the Bifurcation Klatskin tumour - Cholangiocarcinoma of junction of right & left hepatic ducts ERCP: Distal CBD Cancer Surgical Removal – only 25% resectable at the time of diagnosis • Node Dissection in Bile Duct Excision • Roux-en-Y Hepaticojejunostomy Cholangiocarcinoma Palliative therapy : • Stent • Chemotherapy +/- Radiation Therapy • Survival with surgery and chemo/radiation is 24 to 36 months • With chemotherapy / radiation alone survival is 12 to 18 months • Liver transplantation – 80% 5-y survival rate • In selected patients who complete chemoradiation protocol Gallbladder Cancer • 6th decade • 1:3, Male:Female • Highest prevalence in Israel, Mexico, Chile, Japan, and Native American women. • Risk Factors: gallstones, porcelain gallbladder, polyps, Salmonella typhi carrier state, some drugs Gall Bladder Cancer • Uncommonly diagnosed preoperatively • >80% with gallstones • Clinical manifestation from abdominal pain to unexplained weight loss and jaundice • Palpable RUQ mass • Jaundice suggests local extention with ductal obstruction • DS: US, EUS, CT Gall Bladder Cancer • Discovered on pathology after a routine cholecystectomy (1-2%) • If negative for metastasis: – Radical cholecystectomy with nodal dissection, central hepatectomy, w or w/o bile duct excision – Excise port sites – Followed by Chemo/Radiation • 5 year survival = 60% Gallbladder Cancer • Cholecystectomy should not be offered for all patients with stones for fear of cancer • Cholecystectomy should be considered for calcified GB and for growths (adenomyoma, polyps > 15 mm) Pancreatic cancer • Pancreatic cancer is the most common malignant cause, followed by cancers of the gallbladder, bile duct, liver, and large intestine. Metastasis • 1st category involves local extension into the hilum by a tumor arising in an adjacent structure, such as the gallbladder, cholangio CA • The 2nd - includes metastases from a distant primary site, most often from solid tumors, such as carcinoma of the breast, colon, ovaries or lymphoma, melanoma • Mechanical cholestasis caused by the stricture, mostly of the common hepatic or common bile duct Patients have severe jaundice and associated symptoms such as pruritis, recurrent cholangitis and malaise. Metastasis Biliary disease • Gallstones • Congenital anomalies • Extrinsic compression of the bile ducts • Hepatobiliary parasitism • Noncancerous strictures Hepatobiliary parasitism • Pyogenic cholangitis &hepatic abscess, ductal stones, biliary obstruction • Clonorchis sinensis, Opisthorchis viverrini, O. felineus, Fasciola hepatica • Ascaris lumbricoides, Echinococcus spp. Clinical manifestation of hydatid cyst • Most patients with hepatic uncomplicated hydatid cyst are asymptomatic Possible symptoms and signs are: RUQ pain , epigastric pain , fever , fatigue , nausea and dyspepsia ,hepatomegaly and abdominal mass Complications: Superinfection of hydatid cysts Rupture to adjacent structures (peritoneal spillage, cholangitis, pancreatitis, anaphylaxis) Rare – portal HTN, hepatic vein thrombosis, secondary biliary cirrhosis Diagnosis History of exposure Chest and abdominal X-ray Ultrasound (diagnostic method of choice) CT (better information about location, depth , mandatory before planning operation) MRI (for NS, venous system and biliary complications ) Serology (positive – confirms infection, negative test does not exclude) Gharbi’s US classification Type I Type II Type III Type IV Type V - Pure fluid collection Fluid collection with a detached membrane Fluid collection with multiple septa and or daughter cysts Hyperechoic with high internal echoes Cysts with reflecting, calcified walls CT ERCP Histology Treatment of hepatic hydatid cyst The treatment of hepatic hydatid cysts is strongly indicated in order to prevent cyst complications The therapeutic options are: 1. Surgical intervention – remains the cornerstone of radical treatment 2. Percutaneous drainage 3. Drug therapy Surgical options Open surgical techniques Radical removal of pericystic membrane and parasitic content Marsupialization (partial cysto-pericystectomy) Contraindications: severe comorbidity Complications: biliary fistula, cyst infection, pleural effusion, peritonitis, abscess, anaphylactic shock Laparoscopic surgery Contraindications: Deep intraparenchimal cysts > 3 cysts, with thick calcified wall Complications: intra-abdominal seeding due to pneumoperitoneum Paliative procedures PAIR - puncture-aspiration-injection-reaspiration Indicated for type I , II and III cysts Inoperable patients, pregnant women Multiple disseminated cysts Contraindications: IV, V types of cysts, ruptured cysts into biliary tree or peritoneum ERCP – Naso-biliary drainage – biliary endoprosthesis Combined therapy with albendazole is an effective and safe alternative to surgery for uncomplicated hydatid cysts (Khuroo et al, 1998) Indications for drug therapy: WHO Guidelines 1996 Inoperable primary liver or lung echinococcosis Multiple echinococcal multiorgan and peritoneal cysts Preoperative or pre-drainage (at least 4 days before surgery and 1 m (ABZ) or 3 m (MBZ) after) Poor patient status Contraindications: Large cysts that are at risk of rupture Pregnancy ,chronic liver disease or depressed BM Biliary disease • • • • Gallstones Congenital anomalies Extrinsic compression of the bile ducts Hepatobiliary parasitism • Noncancerous strictures Biliary Stricture – Non Cancerous Causes Noncancerous causes of bile duct stricture include: • Injury to the bile ducts during surgery for gallbladder removal • Pancreatitis (inflammation of the pancreas) • Primary sclerosing cholangitis • Gallstones (benign CBD stricture, papillary stenosis • Blunt trauma to the abdomen Primary Sclerosing Cholangitis • Chronic cholestatic biliary disease characterized by non-suppurative inflammation and fibrosis of the biliary ductal system • Cause is unknown but is associated with autoimmune inflammatory diseases, such as chronic ulcerative colitis and Crohn’s colitis, and rare conditions, such as Riedel thyroiditis and retroperitoneal fibrosis • Most patients present with fatigue and pruritus and, occasionally, jaundice PSC •Natural history is variable but involves progressive destruction of the bile ducts, leading to cirrhosis and liver failure •Clinical features of cholangitis (ie, fever, right upper quadrant pain, jaundice) are uncommon unless the biliary system has been instrumented. PSC PSC Medical Care • Chronic progressive disease with no curative medical therapy • Goals of medical management are to treat the symptoms and to prevent or treat the known complications • Liver transplantation is the only effective therapy and is indicated in end-stage liver disease. Surgical Care • Indications for liver transplantation include variceal bleed or portal gastropathy, intractable ascites, recurrent cholangitis, progressive muscle wasting, and hepatic encephalopathy. • Recurs in 15-20% of patients after transplantation. Primary Biliary Cirrhosis •Progressive cholestatic biliary disease that presents with fatigue and itching or asymptomatic elevation of the alkaline phosphatase. •Jaundice develops with progressive destruction of bile ductules that eventually leads to liver cirrhosis and hepatic failure. •Autoimmune illness has a familial predisposition PBC Antimitochondrial antibodies (AMA) are present in 95% of patients Goals of treatment are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome) Liver transplantation appears to be the only lifesaving procedure. ERCP Endoscopic retrograde cholangiopancreatography (ERCP) • Endoscopic tube is placed into the patient’s mouth, through the stomach, and into the duodenal portion of the small intestine. • Contrast is introduced into the biliary tract through the endoscope, in a retrograde manner. • X-rays taken ERCP ספינקטרוטומיה • הוצאת אבנים (בלון ,בסקט) ,ריסוק אבנים • הכנסת סטנטם • PTC הזרקת חומר ניגוד דרך עור לדרכי מרה התוך כבדיים תחת סונר והדמיה ברנטגן PCC • PerCutaneous Cholecystostomy במקרים קשים בהם ניתוח עלול לסכן את החולה – ניקוז • מרה בשיטה מלעורית Indications For Biliary Stenting Indications for stent insertion include: • Ampullary Stenosis • Bile duct injury • Benign or malignant biliary obstruction • Prevention of obstruction where stone extraction is not possible at that time • Pancreatic duct strictures, stones and sphincter of Oddi dysfunction Stent Placement -Endoscopic Approach • A catheter is inserted through the endoscope into the ostium of the common bile duct. • While maintaining the endoscope position in the duodenum, a wire is inserted through the catheter into the bile duct. • The stent delivery system is then inserted over the wire to the site of obstruction, where the stent is deployed. Stent Placement – Endoscopic Approach Success rate of ERCP 90-95% Complication rate of approximately 3-5%. Complications: • • • • • Pancreatitis Bleeding Perforation Infection Cardiopulmonary depression from conscious sedation. Biliary Stent - Percutaneous transhepatic approach PTC For biliary stent placement using a percutaneous approach: •A fine needle is inserted between the 4th and 5th rib on the patient’s right side •The puncture is through the liver •The needle is inserted into an intrahepatic duct under image guidance. Photo on file at Medtronic Biliary Stent - Percutaneous Approach Success rate 95% when ducts are dilated • 5-10% rate of major complications which include: •Sepsis •Bile leak •Intraperitoneal haemorrhage, Haemobilia •Hepatic and perihepatic abscess, Pneumothorax •Skin infection and granuloma at the catheter entry site • Contraindicated in patients with bleeding diatheses and significant ascites.