portal hypertension 门脉高压症 the first affiliated hospital, college of medicine, zhejiang...
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Portal Hypertension门脉高压症
the First Affiliated Hospital, College of Medicine, Zhejiang University
浙江大学医学院附属第一医院
Prof. Zhang Min, MD张 珉
What do we know about Portal Hypertension?
• As early as the 17th century, it was realized that structural changes in the portal circulation could cause gastrointestinal bleeding.
• In 1902, Gilbert and Carnot introduced the term "portal hypertension" to describe this condition.
Introduction of Portal Hypertension
Then, What's Portal Hypertension?
Definition
• Portal hypertension is defined as a pressure > 12 mmHg.
• This increased pressure results from a functional obstruction to blood flow from any point in the portal system's origin (in the splanchnic bed) through the hepatic veins (exit into the systemic circulation) or from an increase in blood flow in the system.
Pathophysiology
• The normal pressure of portal vein:
5-10mmHg (13~24 cmH2O)
Anatomy of Portal System
The portal vein supplies 70% of the blood flow to the liver, but only 40% of the liver oxygen supply. The remainder of the blood comes from the hepatic artery, and blood from both of these vessels mixes in the sinusoids.
Ducts and vessles of the liver
Tow concepts:
Glisson’s sheath
Lobule of the liver
Liver lobule
Portal triad
Central v.
Sinus
Four ramus communicans betweenportal and systemic circulations
esophageal and gastric veins
inferior rectal-anal veins
anterior abdominal wall veinsretroperitoneal venous plexus
• Portal hypertension should be divided into three types as its blood flow obstructed positions.
Etiology & Types
Types
pre-hepatic
intra-hepaticpre-sinusoidal
Sinusoidal
post-sinusoidal
post-hepatic
• the portal vein trunk itself obstruction.Congenital deformity: obliteration, stenosis 先天性畸形:闭塞、狭窄Thrombosis: portal vein thrombosis, splenic vein
thrombosis, ect 血栓形成:门静脉、脾静脉 Infection: Tropical splenomegaly, ect 感染:热带巨脾综合症Trauma: arterio-venous fistula, ect 创伤:动静脉瘘
Pre-hepatic type
• About 95% patients belong to this type of portal hypertension
• A classification by pathology:pre-sinusoidal obstructions
sinusoidal obstructions
post-sinusoidal obstructions
Intra-hepatic type
• Schistosomial cirrhosis. 血吸虫病
– Schistosomial eggs deposit in small branches of portal vein, resulting in obstruction of blood flow and increase of portal vein pressure.
• Primary biliary cirrhosis 原发性胆汁性肝硬化 • Chronic active hepatitis 慢性活动性肝炎
Pre-sinusoidal obstruction
Schistosomiasis cirrhosis
Schistosomiasis eggs
• Cirrhosis – Post hepatitic cirrhosis, 肝炎后肝硬化– Alcohol cirrhosis, 酒精性肝硬化 – Cryptogenic cirrhosis, 隐匿性肝硬化 – Metabolic liver disease (e.g. Wilson's Disease) 代谢性疾病( Wilson’s 病)
• Non-cirrhotic – Cytotoxic drugs, 细胞毒性药物– Vitamin A intoxication. 维生素 A 中毒
Sinusoidal obstruction
liver cirrhosis
Pseudolobules formation in liver cirrhosisThe regeneration nodules distort the vascular tree, impeding the flow of blood.
Post-sinusoidal obstruction• Budd-Chiari syndrome
– Hepatic veins or inferior vena cava in hepatic level obstruction included congenital deformity or thrombosis, called Budd-Chiari syndrome.
• Veno-occlusive disease
Post-hepatic type
• Posthepatic obstruction occurs at any level between liver and right heart, including – Hepatic vein thrombosis, 肝静脉血栓形成– IVC thrombosis, 下腔静脉血栓形成– IVC congenital malformation, 下腔静脉先天性畸形 – Constrictive pericarditis. 缩窄性心包炎
Pathophysiology
congestive splenomegaly
充血性脾肿大 ramus communicans dilatation
交通支扩张 ascites
腹水
Symptoms and Signs ( 1)
• congestive splenomegaly and hypersplenism– Splenomegaly is defined as the spleen size >12cm in
length.– Hypersplenism is a type of disorder which causes the
spleen to rapidly and prematurely destroy blood cells
Splenomegaly
Splenomegaly
Symptoms and Signs ( 2)
• ramus communicans dilatation:– esophageal and gastric veins: varices rupture and g
astrointestinal hemorrhage – inferior rectal-anal veins: hemorrhoid & bleeding– anterior abdominal wall veins: paraumbilical
varices (caput medusae)– retroperitoneal veins plexus: dilatation & congestio
n
Caput Medusae
Esophageal varices
Symptoms and Signs ( 3)
• Ascites– disordered albumin synthesis and decreased plas
ma colloid osmotic pressure caused by hepatocellular function damage
– increased capillary filter pressure due to increased portal hypertension
– lymph liquid leakage into abdominal cavity from surface of the liver because of lymph back-flow obstruction
– salt and water retention by aldosterone and antidiuretic hormones deactivation disturbance
Ascites
Symptoms and Signs ( 4)• Non-specific systemic symptoms:
– Fatigue
乏力– Lethargy
嗜睡– loss of appetite
纳差
Diagnosis and Differential Diagnosis
Diagnosis
• Medical history: hepatitis, schistosomiasis,
alcohol, or drugs?
• Clinical presentation: splenomegaly and hypersplenism, hematemesis and melena, ascites
• Lab exams and images
Laboratory Exams & Images
• Blood RT : WBC↓, Plt↓
• Liver Function: albumin↓, A/G ratio reversing, prothrombin time↑
• Markers of hepatitis B or C
• Ultrasound
• CT/MRI
• Esophageal endoscopy
• Esophageal barium swallow
• Angiography
Images
• Ultrasound and Doppler: cirrhosis, splenomegaly, ascites, thrombosis and occlusion of the portal, superior mesenteric and splenic vein, enlargement of portal vein>13mm and of splenic vein>10mm
Images
• CT scan
Images
• Esophageal endoscopy:
white, pink, red, cherry red varices
Images
• Esophageal endoscopy
Images
• esophageal barium swallow
multiple irregular filling defects as “string of beads” or “earthworm”
Images
Normal Portal hypertension
• Angiography
Differential Diagnosis
• peptic ulcer gastritis gastric cancer biliary tract
Treatment
• Non-surgical treatment during Massive Vari
ceal Bleeding
• Management of Ascites
• Surgical treatment
Evaluation of liver function reserve
What for ?
How ?
Child-Pugh classification of liver function
Child-Pugh classification is a scoring system developed for evaluating surgical risk in patients with cirrhosis.
Child’ grade 1 point 2 points 3 points
Serum bilirubin (umol/L) <34.2 34.2-51.3 >51.3
Albumin (g/l) >35 30-35 <30
Prothrombin (s' prolonged)
1-4 4-6 >6
Ascites absent slight moderate
Encephalopathy nonenone or minimal
coma
Child-Pugh classification of liver function
Class A ( low operative risk ): 5 or 6 points
Class B ( moderate risk ): 7 to 9 points
Class C (high risk ): 10 to 15 points
Non-surgical treatment for Massive Variceal Bleeding
• Anti-shock • Pharmacotherapy and control of bleeding
• vasopressin • sandostatin
• Beta blockers (propranolol) • general hemostatic drugs
• Local treatment • endoscopic variceal sclerosis or banding • hemostatic drugs injection per oral or stomach tube 8mg% noradrenaline ice saline? • balloon tamponade
• TIPS(Transjugular interhepatic portasystemic shunts)
endoscopic variceal sclerosis or ligation
balloon tamponade
(life-saving procedure)
balloon tamponade
Sengstaken-Blakemore tube(1950)
esophageal balloon (100-150ml) gastric balloon (150-200ml) one lumen to gastric balloon one lumen for gastric aspiration one lumen to esophageal balloon
Attention!
check the balloons for air leakage before use of the
tube
24-72 h of placement and 10-20min /12h removal
of air
filling the air firstly to the gastric balloon and
removing the air firstly from esophageal balloon
observation of the patient’s breath and enhancing
the respiratory tract nursing
TIPSS
Transjugular intrahepatic portasystemic stent-shunt
TIPSS is a small, tubular metal device commonly called a stent that is placed in veins in the middle of the liver to permit blood flow to bypass the liver. In a TIPSS procedure, interventional radiologists use image guidance to make a tunnel through the liver to connect the portal vein to one of the hepatic veins. A stent is then placed in this tunnel to keep the pathway open.
TIPSS
Transjugular intrahepatic portasystemic stent-shunt
TIPSS
stent
TIPSS
Transjugular intrahepatic portasystemic stent-shunt
A TIPSS is used to treat the complications of portal hypertension, including:
variceal bleeding, bleeding from any of the veins that normally drain the stomach, esophagus, or intestines into the liver. portal gastropathy, an engorgement of the veins in the wall of the stomach, which can cause severe bleeding. severe ascites (the accumulation of fluid in the abdomen) and/or hydrothorax (in the chest). Budd-Chiari syndrome, a blockage in one or more veins that carry blood from the liver back to the heart
Management of Ascites
salt restriction diuretic therapy paracentesis peritoneal venous shunt
Surgical treatment
◈ splenectomy portosystemic shunt or bypass◈ extensive devascularization around ◈
the cardia ◈ liver transplantation
Splenectomy
◈ reduction of portal blood flowBecause of “Higher volume, Higher pressure”
Portosystemic shunt or bypass
Anastomose the portal vein or its main branches (splenic vein and superior mesenteric vein) to vena cava or its main branches (renal vein) by use of operative procedures, and put the hypertensive portal blood flow into the low-pressured inferior vena cava. To reduce the portal vein pressure and thus decrease the blood flow through collateral venous beds
portacaval end-to-side shunt portacaval side-to-side shunt
mesocaval shunt splenorenal shunt
Limited Side-to-Side Portacaval Shunt
Selective distal splenorenal shunt (Warren’s operation)
During the surgery, the splenic vein is detached from the portal vein and reattached to the left renal vein.
Devascularization operations
◈ Disconnection of the venous circulation of the distal esophagus and cardiac from the hypertensive portal circulation by division of all the feeding vessels.
ligation of lower esophageal and gastric variceal veinsdisconnection of cardiac portal systemic venous shuntresection of lower esophagus and gastric fundus esophagogastrostomy
Devascularization operations
Comparing the shunt with devascularization
Shunt devascularization
decrease pressure clear, obvious none or increaseencephalopathy maybe nonehepatic perfusion decrease increaseoperative procedure difficult simpleanastomotic obstruction maybe none
Liver transplantation
Liver transplantation is potentially applicable to any acute or chronic condition with irreversible liver dysfunction.
Most liver transplants are performed for chronic liver diseases that lead to irreversible scarring of the liver, or cirrhosis of the liver.
Liver transplantation