胆道胰腺疾病介绍

胆胰恶性肿瘤与黄疸,山东大学齐鲁医院,胆胰恶性疾病key point,树立壶腹周围癌的概念症状: 典型的梗阻性黄疸 (皮肤黄染白陶土样便全身瘙痒)影像学特征：复杂性，往往没有明显的占位性改变,诊断依靠间接征象。对造成胰腺癌症状和低切除率的解剖学因素加以了解,关于胆胰恶性疾病,胆胰恶性疾病的诊断思路胆胰恶性肿瘤与梗阻性黄疸胆胰恶性疾病的特殊影像学表现胆胰恶性肿瘤的有关解剖胆胰恶性肿瘤的鉴别诊断胆胰恶性肿瘤的治疗,胆胰恶性疾病的诊断思路,胆胰恶性疾病的诊断思路,化验：直接胆红素升 高为主的胆红素升高 GGT与ALP的升高 CA199、CA242升高 影像学表现,Spleen,Bone Marrow,Senescent Red Cells8085%,Ineffective Erythropoiesis1520%,Hemoglobin,Heme,Globin,Reticulo-endothelialsystem,Unconjugated Bilirubin,ALB-boundbilirubin,Plasma,Unconjugated Bil,HepaticHemoproteins,Conjugated Bilirubin,Liver,UDPGT,Bilirubin:4mg/kg/d,UptakeConjugationExcretion,Transportation,Production,Bilirubin Metabolism,*,Unconjugated Bil,Unconjugated Bilirubin,Kidney,Urine,Small intestine,Bile duct,Stool80-85% UrobilirubinStercobilin,Portal vein,Urobilirubin15-20%,UrineUrobilirubin 2% Bilirubin product,Unconjugated bilirubinConjugated bilirybinUrobilirubin,stercobilin,Enterohepatic Circulation of Bilirubin,Colon,Bacteria,Unconjugated Bilirubin,10% Urobilirubin,*,Liver,Clinical Classification of Hyperbilirubinemia,Predominant Unconjugated Hyperbilirubinemia Predominant conjugated HyperbilirubinemiaMixed Hyperbilirubinemia,Predominant Unconjugated Hyperbilirubinemia,1. Overproduction Hemolysis due to a variety of etiologies,e.g., hemolytic jaundice Ineffective erythropoiesise.g., megaloblastic anemia2. Decreased hepatic uptake Gilbert syndrome, etc. Drugs: rifampin, etc. Neonatal jaundice,3. Decreased conjugation: due to defect or absence of UDP-glucuronyl transferase or other causes Neonatal jaundice Hepatocellular disease: e.g.,post-hepatitic hyperbilirubinemia Drugs: chloramphenicol, etc. Crigller-Najjar syndrome, Gilbert syndrome,Predominant Unconjugated Hyperbilirubinemia,1. Intrahepatic cholestasis Cholestatic hepatitis: viral, alcoholic, auto- immune hepatitis Drug-induced: androgens, estrogens, cyclo- sporins, thiouracils, phenothiazines,captopril Pregnancy Sepsis with Gram negative organisms Primary biliary cirrhosis (PBC) Inheritary: Dubin-Johnson,Rotor syndromes,Predominant Conjugated Hyperbilirubinemia,2. Extrahepatic biliary obstruction Gallstones Tumors: pancreatic, bile duct, ampula of Vater Bile duct compression: chronic pancreatitis, lymph nodes Post-operative biliary stricture Primary sclerosing cholangitis (PSC) Congenital biliary atresia,Predominant Conjugated Hyperbilirubinemia,Due to hepatocellular disease or damage Hepatitis: viral, alcoholic, autoimmune Cirrhosis, Wilsons disease Sepsis Congestive heart failure, Budd-Chiari syndrome Drug-induced hepatic injury: alcohol, rifampin, cimetidine, sulfonamide, colchicine, verapramil, etc.,Mixed Hyperbilirubinemia,Decreased bile acid excretion Pruritus Absorption of fat & fat-soluble vitamins Coagulopathy: due to Vit K deficiency Calcium absorption and osteoporosisHypercholesterolemia, xanthomaIncreased synthesis and secretion of canalicular enzymes: ALP, GGT,Consequences of Sustained Hyperbilirubinemia,Renal failurePigment gallstonesIncreased postoperative mortality and morbidityKernicterus in infants Decreased cardiovascular response to vasopressors,Consequences of Sustained Hyperbilirubinemia,Distinguish between hepatocellular and cholestatic jaundiceHepatocellular Dis. Cholestasis Bilirubin CB/TB 0.2-0.5 0.5ALT,AST ALP,GGT 3Bilirubinuria + or +Pale stool not present present,Laboratory Differentiation of Jaundice,Distinction between intracellular and extrahepatic cholestasis Intrahepatic ExtrahepaticUS,CT, Extra- & intra- Extra- & intrahepatic hepatic bile bile ducts dilated, ducts normal ERCP: stones, ERCP normal tumors, stricturesALP,GGT Auto-Abs AMA,M2 +-+ Inspecific auto-Abs for PBC for PSCSite of Hepatocytes and Extrahepatic bile lesions canaliculi/ductules ducts,Laboratory Differentiation of Jaundice,History, Physical Examination,Hb, Blood cells, Bil, ALT, Alb, ALP/GGT, PT+A, HBsAg, Urine, Stool,Predominant unconj. bil.,Mixed,Predominant conj. bil.,Markers for viral hepatitisImmunological tests Microbial examinationSystemic diseasesLiver biopsy if necessary,Ultrasonography,Extrahepaticbile ductsnot dilated,Extrahepaticbile ductsdilated,CT ERCPMRCPPTC,Tests for hemolysisBone marrowLiver biopsy if necessary,Summary:Approachesto Diagnosisof Jaundice,*,胆胰恶性肿瘤的影像学表现,胆胰疾病的特殊检查,超声检查：胆囊疾病的金标准CTMRI各种造影检查：PTCD与ERCP,PTCD与ERCP,涵义适应证：诊断价值基本被无创方法取代，已经倾向于疾病的治疗有创性：可导致致命并发症,重要疾病四：恶性疾病-胆囊癌,胆囊癌: 与胆囊结石的密切关系Nevin分级转移与侵袭能力较强,胆管癌-间接征象,胰腺癌-模糊的占位效应,胰腺癌-诊断的复杂性,胆胰恶性肿瘤的有关解剖,胆胰疾病解剖的密切联系：opie共同通道学说（1901）,肝胆相照“肝胆胰相照 ”,胆道解剖-肝外胆管,分部胆总管分部胆总管与胰管vater氏壶腹，oddi氏括约肌,胆道的生理,胆道系统：分泌、贮存、浓缩、输送胆汁胆囊：储存与浓缩胆汁的生理功能：脂肪与脂溶性维生素,胰腺解剖,人体第二大腺体头颈体尾四部分+勾突主胰管与副胰管由腹腔干供血为主，静脉回流入门静脉 return,胰腺生理,独特的、以血糖调节为主的内分泌功能：A与B细胞超强的外分泌功能，负责三大营养素的代谢急性胰腺炎时可以导致自身的消化并产生炎性介质危及全身,横结肠,空肠,下腔静脉,胆总管,右膈脚,降结肠,胰头,右肾,胰颈,肠系膜上静脉,肠系膜上动脉,腹主动脉,左肾,胰 头、胰 颈、肠 系 膜 上 静 脉 断 层,胰头,下腔静脉,十二指肠降部,钩突,肾窦,胆囊,肾门,腹主动脉,肠系膜上动脉,肠系膜上静脉,空肠,胰 头、肠 系 膜 上 静 脉 断 层,胆胰恶性肿瘤的鉴别诊断,胆胰系统重要疾病-共性疾病,炎症：急慢性胆囊炎，胆管炎，急慢性胰腺炎肿瘤：胆管癌，胆囊癌，胰腺癌，壶腹癌先天性疾病：胆总管囊肿，胰腺囊肿,胆胰系统重要疾病-特殊疾病,结石疾病：胆囊结石与胆、胰管结石胰腺内分泌肿瘤：功能性与无功能胰腺内分泌肿瘤,胆囊息肉、胆囊结石与胆囊癌,胆囊结石的成因：脂质代谢异常造成的胆固醇 过饱和 症状与其造成的多种并发症有关胆囊结石与胆囊癌,胆囊炎、胆管炎、梗阻性黄疸、胆源性胰腺炎、胆囊十二指肠瘘,胆囊疾病与胆囊息肉样病变,定义分类：手术适应症,独特的胆固醇性息肉炎性息肉腺瘤性息肉腺肌症恶性息肉,胆囊疾病与腹腔镜胆囊切除术：微创外科与胰腺胆道手术,1987年问世外科技术的重大革命胰腺肿瘤的局部切除是最佳的适应症（eneucleation）胰体尾手术成为常规重建仍然是弱项,重要疾病一：胆囊疾病与胆石症,胆囊的分部：Hartmann袋，螺旋瓣Calot三角：是由胆囊管、肝总管、肝脏下缘所构成的三角区。其内有胆囊动脉、肝右动脉、副右肝管通过。胆石症：胆固醇、胆色素与混合结石,认识一下慢性胰腺炎,酒精、胆道疾病为主要病因胰腺内外分泌功能的丧失,胰腺内分泌肿瘤,重要疾病五：胰腺内分泌肿瘤,胰腺独有的肿瘤如何与外分泌肿瘤鉴别胰岛素瘤和无功能胰岛细胞瘤发病率较高Whipple三联症：与血糖关系密切,胆胰恶性疾病的治疗,恶性疾病的胰十二指肠切除术,壶腹周围癌的经典手术方式复杂的切除与重建工作：涉及胆、胰、胃等脏器的切除与重建梗阻性黄疸造成的凝血机制障碍、全身营养不良加重了手术难度，术后易发生肾功能衰竭、低氧血症,治疗（掌握）,肿瘤侵及门静脉、肠系膜上静脉者可将其一段血管连同肿瘤切除，再行血管移植吻合行全胰切除术。对不能切除的胰腺癌，为了解除黄疸，有条件者首先争取作内瘘。,胆胰恶性疾病的辅助治疗,疼痛：疼痛的阶梯治疗 第一阶段 轻度疼痛，非阿片类镇痛药（阿司匹林、布洛芬、对乙酰氨基酚）第二阶梯 在轻、中度疼痛时，弱阿片药物（可待因、强痛定等） 第三阶梯 选用强阿片类药，代表药物是吗啡、杜冷丁等。其选用应根据疼痛的强度 ( 如中、重剧痛者 ) 而不是根据癌症的预后或生命的时限。常用缓释或控释剂型,Case Discussion(1),A 30 y.o. female patient was admitted because of anorexia, fatigue, and pain over right costal area for 2 weeks. Her