Portal hypertension - wikipedia Portal hypertension Portal hypertension The portal vein and its tributaries Classification and external resources Specialty Gastroenterology ICD - 10 K76. 6 ICD - 9 - CM 572.3 DiseasesDB 10388 eMedicine radio / 570 med / 1889 MeSH D006975 ( edit on Wikidata ) Portal hypertension is hypertension ( high blood pressure ) in the hepatic portal system, which are the portal vein and its branches, which drain from most of the intestines to the liver. Portal hypertension is defined as a hepatic venous pressure gradient. Cirrhosis ( a form of chronic liver failure ) is the most common cause of portal hypertension ; other, less frequent causes are therefore grouped as non-cirrhotic portal hypertension. When it becomes severe enough to cause symptoms or complications, treatment may be given to decrease portal hypertension itself or to manage its complications. Contents 1 Signs and symptoms 2 Causes 3 Pathophysiology 4 Diagnosis 5 Treatment 5.1 Portosystemic shunts 5.2 Prevention of bleeding 5.3 Ascites 5.4 Hepatic encephalopathy 6 References 7 Further reading 8 External links Signs and symptoms Signs and symptoms of portal hypertension include : Ascites ( free fluid in the peritoneal cavity ), Abdominal pain or tenderness ( when bacteria infect the ascites, as in Spontaneous bacterial peritonitis ). Increased spleen size ( Splenomegaly ), which may lead to lower platelet counts ( thrombocytopenia ) Swollen veins of the oesophagus ( Oesophageal varices ), which may bleed and cause vomiting of blood ( haematemesis ) Swollen veins on the anterior abdominal wall ( sometimes referred to as Caput medusae ) Hemorrhoids ( swollen or dilated hemorrhoidal veins ) Anorectal Varices In addition, a widened portal vein as seen on a CT scan or MRI may raise the suspicion about portal hypertension. A cutoff of 13 mm is widely used in this regard, but the diameter is often larger than this is in normal individuals as well. Causes The causes for portal hypertension are classified as originating in the portal venous system before it reaches the liver ( prehepatic causes ), within the liver ( intrahepatic ) or between the liver and the heart ( post-hepatic ). The most common cause is cirrhosis ( chronic liver failure ). Other causes include : Conditions associated with non-cirrhotic portal hypertension Prehepatic causes Portal vein thrombosis or splenic vein thrombosis Arteriovenous fistula Splenomegaly ( increased portal blood flow ) Hepatic causes Any cause of cirrhosis, e.g. alcohol abuse, chronic viral hepatitis, biliary atresia, etc. Primary biliary cirrhosis Primary sclerosing cholangitis Chronic pancreatitis Hereditary haemorrhagic telangiectasia Schistosomiasis Congenital hepatic fibrosis Nodular regenerative hyperplasia Fibrosis of space of Disse Granulomatous or infiltrative liver diseases ( Gaucher, mucopolysaccharidosis, sarcoidosis, lymphoproliferative malignancies, amyloid deposition,... ) Toxicity ( from arsenic, copper, vinyl chloride monomers, mineral oil, vitamin A, azathioprine, dacarbazine, methotrexate, amiodarone,... ) Viral hepatitis Fatty liver disease Veno - occlusive disease Posthepatic causes Inferior vena cava obstruction ( Right - sided ) heart failure, e.g. from constrictive pericarditis hepatic vein thrombosis Budd - Chiari syndrome Pathophysiology Stellate cell The pathophysiology of portal hypertension is indicated by increased vascular resistance via different causes ; additionally, stellate cells and myofibroblasts are activated. Increased endogenous vasodilators in turn promote more blood flow in the portal veins. Nitric oxide is an endogenous vasodilator and it regulates intrahepatic vascular tone ( it is produced from L - arginine ). According to Maruyama, et al., in laboratory studies nitric oxide inhibition increases portal hypertension and hepatic response to norepinephrine is increased. Diagnosis Portal hypertension due to cirrhosis resulting in revascularization of the umbilical vein Ultrasonography ( US ) is the first - line imaging technique for the diagnosis and follow - up of portal hypertension because it is non-invasive, low - cost and can be performed on - site. Signs of portal hypertension on ultrasound include dilatation of the portal vein of over 13 mm in diameter, a portal flow mean velocity of less than 12 cm / s on Doppler ultrasound, porto -- systemic collateral veins ( patent paraumbilical vein, spleno -- renal collaterals and dilated left and short gastric veins ), splenomegaly and signs of cirrhosis ( including nodularity of the liver surface ). The hepatic venous pressure gradient ( HVPG ) measurement has been accepted as the gold standard for assessing the severity of portal hypertension. Portal hypertension is defined as HVPG greater than or equal to 5 mm Hg and is considered to be clinically significant when HVPG exceeds 10 to 12 mm Hg. Treatment The treatment of portal hypertension is divided into : Portosystemic shunts Fluoroscopic image of transjugular intrahepatic portosystemic shunt ( TIPS ) Selective shunts select non-intestinal flow to be shunted to the systemic venous drainage while leaving the intestinal venous drainage to continue to pass through the liver. The most well known of this type is the splenorenal. This connects the splenic vein to the left renal vein thus reducing portal system pressure while minimizing any encephalopathy. In an H - shunt, which could be mesocaval ( from the superior mesenteric vein to the inferior vena cava ) or could be, portocaval ( from the portal vein to the inferior vena cava ) a graft, either synthetic or the preferred vein harvested from somewhere else on the patient 's body, is connected between the superior mesenteric vein and the inferior vena cava. The size of this shunt will determine how selective it is. It should be noted that with the advent of transjugular intrahepatic portosystemic shunting ( TIPS ), portosystemic shunts are less performed. TIPS has the advantage of being easier to perform and does n't disrupt the liver 's vascularity. Prevention of bleeding Both pharmacological ( non-specific β - blockers, nitrate isosorbide mononitrate, vasopressin such as terlipressin ) and endoscopic ( banding ligation ) treatment have similar results. TIPS ( transjugular intrahepatic portosystemic shunting ) is effective at reducing the rate of rebleeding. The management of active variceal bleeding includes administering vasoactive drugs ( somatostatin, octreotide ), endoscopic banding ligation, balloon tamponade and TIPS. Ascites This should be gradual to avoid sudden changes in systemic volume status which can precipitate hepatic encephalopathy, renal failure and death. The management includes salt restriction, diuretics ( spironolactone ), paracentesis, and transjugular intrahepatic portosystemic shunt. Hepatic encephalopathy A treatment plan may involve lactulose, enemas, and use of antibiotics such as rifaximin, neomycin, vancomycin, and the quinolones. Restriction of dietary protein was recommended but this is now refuted by a clinical trial which shows no benefit. Instead, the maintenance of adequate nutrition is now advocated. References Jump up ^ `` Portal hypertension Disease Overview Genetic and Rare Diseases Information Center ( GARD ) -- an NCATS Program ''. rarediseases.info.nih.gov. Retrieved 2016 - 01 - 08. ^ Jump up to : `` Portal Hypertension. Learn about Portal Hypertension Patient ''. Patient. Retrieved 2016 - 01 - 08. Jump up ^ `` Spontaneous Bacterial Peritonitis ''. www.karger.com. Retrieved 2016 - 01 - 08. Jump up ^ Sun, Z ; Migaly, J ( March 2016 ). `` Review of Hemorrhoid Disease : Presentation and Management ''. Clinics in colon and rectal surgery. 29 ( 1 ) : 22 -- 9. doi : 10.1055 / s - 0035 - 1568144. PMID 26929748. Jump up ^ `` Anorectal varices ''. Wikipedia. 2017 - 02 - 21. Jump up ^ Stamm, Elizabeth R. ; Meier, Jeffrey M. ; Pokharel, Sajal S. ; Clark, Toshimasa ; Glueck, Deborah H. ; Lind, Kimberly E. ; Roberts, Katherine M. ( 2016 ). `` Normal main portal vein diameter measured on CT is larger than the widely referenced upper limit of 13 mm ''. Abdominal Radiology. 41 ( 10 ) : 1931 -- 1936. doi : 10.1007 / s00261 - 016 - 0785 - 9. ISSN 2366 - 004X. Jump up ^ Bloom, S. ; Kemp, W. ; Lubel, J. ( 2015 - 01 - 01 ). `` Portal hypertension : pathophysiology, diagnosis and management ''. Internal Medicine Journal. 45 ( 1 ) : 16 -- 26. doi : 10.1111 / imj. 12590. ISSN 1445 - 5994. Jump up ^ Perkins, ( edited by ) Vinay Kumar, Abul K. Abbas, Jon C. Aster ; artist, James A. Robbins basic pathology ( 9th ed. ). Philadelphia, PA : Elsevier / Saunders. p. 608. ISBN 978 - 1 - 4377 - 1781 - 5. CS1 maint : Extra text : authors list ( link ) 2013 Jump up ^ `` Portal Hypertension : Practice Essentials, Background, Anatomy ''. Jump up ^ Maruyama, Hitoshi ; Yokosuka, Osamu ( 2012 ). `` Pathophysiology of Portal Hypertension and Esophageal Varices ''. International Journal of Hepatology. 2012 : 1 -- 7. doi : 10.1155 / 2012 / 895787. access - date = requires url = ( help ) ^ Jump up to : Jaime Bosch, MD, PhD ; Annalisa Berzigotti, MD, PhD ; Susana Seijo, MD ; Enric Reverter, MD ( 2013 - 01 - 28 ). `` Assessing Portal Hypertension in Liver Diseases : Noninvasive Techniques to Assess Portal Hypertension ''. CS1 maint : Multiple names : authors list ( link ) Jump up ^ Al - Busafi SA, McNabb - Baltar J, Farag A, Hilzenrat N ( 2012 ). `` Clinical manifestations of portal hypertension ''. Int J Hepatol. 2012 : 203794. doi : 10.1155 / 2012 / 203794. PMC 3457672. PMID 23024865. Jump up ^ Shah, Omar Javed ; Robbani, Irfan ( 2005 - 01 - 01 ). `` A Simplified Technique of Performing Splenorenal Shunt ( Omar 's Technique ) ''. Texas Heart Institute Journal / from the Texas Heart Institute of St. Luke 's Episcopal Hospital, Texas Children 's Hospital. 32 ( 4 ) : 549 -- 554. ISSN 0730 - 2347. PMC 1351828. PMID 16429901. Jump up ^ Moore, Wesley S. ( 2012 - 11 - 23 ). Vascular and Endovascular Surgery : A Comprehensive Review. Elsevier Health Sciences. p. 851. ISBN 1455753866. Jump up ^ Yin, Lanning ; Liu, Haipeng ; Zhang, Youcheng ; Rong, Wen ( 2013 ). `` The Surgical Treatment for Portal Hypertension : A Systematic Review and Meta - Analysis ''. ISRN Gastroenterology. 2013 : 1 -- 10. doi : 10.1155 / 2013 / 464053. access - date = requires url = ( help ) Jump up ^ Pomier - Layrargues, Gilles ; Bouchard, Louis ; Lafortune, Michel ; Bissonnette, Julien ; Guérette, Dave ; Perreault, Pierre ( 2012 ). `` The Transjugular Intrahepatic Portosystemic Shunt in the Treatment of Portal Hypertension : Current Status ''. International Journal of Hepatology. 2012 : 1 -- 12. doi : 10.1155 / 2012 / 167868. access - date = requires url = ( help ) ^ Jump up to : Bari, Khurram ; Garcia - Tsao, Guadalupe ( 2012 - 03 - 21 ). `` Treatment of portal hypertension ''. World Journal of Gastroenterology. 18 ( 11 ) : 1166 -- 1175. doi : 10.3748 / wjg. v18. i11. 1166. ISSN 1007 - 9327. PMC 3309905. PMID 22468079. Jump up ^ `` Portal Hypertension Medication : Somatostatin Analogs, Beta - Blockers, Nonselective, Vasopressin - Related, Vasodilators ''. emedicine.medscape.com. Retrieved 2016 - 01 - 08. Jump up ^ Dib, Nina ; Oberti, Frédéric ; Calès, Paul ( 2006 - 05 - 09 ). `` Current management of the complications of portal hypertension : variceal bleeding and ascites ''. Canadian Medical Association Journal. 174 ( 10 ) : 1433 -- 1443. doi : 10.1503 / cmaj. 051700. ISSN 0820 - 3946. PMC 1455434. PMID 16682712. Jump up ^ http://www.patient.info/doctor/Hepatic-Encephalopathy.htm Further reading Garbuzenko D.V., Arefyev N.O., Belov D.V. Restructuring of the vascular bed in response to hemodynamic disturbances in portal hypertension. World J. Hepatol. 2016 ; 8 ( 36 ) : 1602 - 1609. Garbuzenko D.V., Arefyev N.O., Belov D.V. Mechanisms of adaptation of the hepatic vasculature to the deteriorating conditions of blood circulation in liver cirrhosis. World J. Hepatol. 2016 ; 8 ( 16 ) : 665 - 672. Garbuzenko D.V. Current approaches to the management of patients with liver cirrhosis who have acute esophageal variceal bleeding. Curr. Med. Res. Opin. 2016 ; 32 ( 3 ) : 467 - 475. Garbuzenko D.V. Contemporary concepts of the medical therapy of portal hypertension under liver cirrhosis. World J. Gastroenterol. 2015 ; 21 ( 20 ) : 6117 - 6126. `` Functional aspects on the pathophysiology of portal hypertension in cirrhosis -- Journal of Hepatology ''. www.journal-of-hepatology.eu. Retrieved 2016 - 01 - 08. Rossi, Plinio ( 2012 - 12 - 06 ). Portal Hypertension : Diagnostic Imaging and Imaging - Guided Therapy. Springer Science & Business Media. ISBN 9783642571169. Imanieh, Mohammad Hadi ; Dehghani, Seyed Mohsen ; Khoshkhui, Maryam ; Malekpour, Abdorrasoul ( 2012 - 10 - 01 ). `` Etiology of Portal Hypertension in Children : A Single Center 's Experiences ''. Middle East Journal of Digestive Diseases. 4 ( 4 ) : 206 -- 210. ISSN 2008 - 5230. PMC 3990125. PMID 24829658. External links Ascites at Merck Manual of Diagnosis and Therapy Home Edition Portal Hypertension Resource Guide and Support Diseases of the digestive system ( primarily K20 -- K93, 530 -- 579 ) Upper GI tract Esophagus Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory - Weiss syndrome UES Zenker 's diverticulum LES Barrett 's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease ( GERD ) Laryngopharyngeal reflux ( LPR ) Esophageal stricture Megaesophagus Stomach Gastritis Atrophic Ménétrier 's disease Gastroenteritis Peptic ( gastric ) ulcer Cushing ulcer Dieulafoy 's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus Lower GI tract : Intestinal / Enteropathy Small intestine ( Duodenum / Jejunum / Ileum ) Enteritis Duodenitis Jejunitis Ileitis Peptic ( duodenal ) ulcer Curling 's ulcer Malabsorption : Coeliac Tropical sprue Blind loop syndrome Small bowel bacterial overgrowth syndrome Whipple 's Short bowel syndrome Steatorrhea Milroy disease Bile acid malabsorption Large intestine ( Appendix / Colon ) Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis / Diverticulosis Large and / or small Enterocolitis Necrotizing Gastroenterocolitis IBD Crohn 's disease Vascular : Abdominal angina Mesenteric ischemia Angiodysplasia Bowel obstruction : Ileus Intussusception Volvulus Fecal impaction Constipation Diarrhea Infectious Intestinal adhesions Rectum Proctitis Radiation proctitis Proctalgia fugax Rectal prolapse Anismus Anal canal Anal fissure / Anal fistula Anal abscess Hemorrhoid Anal dysplasia Pruritus ani GI bleeding / BIS Upper Hematemesis Melena Lower Hematochezia Accessory Liver Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd - Chiari syndrome Hepatic veno - occlusive disease Portal hypertension Nutmeg liver Alcoholic liver disease Liver failure Hepatic encephalopathy Acute liver failure Liver abscess Pyogenic Amoebic Hepatorenal syndrome Peliosis hepatis Metabolic disorders Wilson 's disease Hemochromatosis Gallbladder Cholecystitis Gallstones / Cholecystolithiasis Cholesterolosis Rokitansky - Aschoff sinuses Postcholecystectomy syndrome Porcelain gallbladder Bile duct / Other biliary tree Cholangitis Primary sclerosing cholangitis Secondary sclerosing cholangitis Ascending Cholestasis / Mirizzi 's syndrome Biliary fistula Haemobilia Gallstones / Cholelithiasis Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction Pancreatic Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula Abdominopelvic Hernia Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit 's Grynfeltt - Lesshaft Undefined location Incisional Internal hernia Richter 's Peritoneal Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum Cardiovascular disease ( vessels ) ( I70 -- I99, 440 -- 456 ) Arteries, arterioles and capillaries Inflammation Arteritis Aortitis Buerger 's disease Peripheral artery disease Arteriosclerosis Atherosclerosis Foam cell Fatty streak Atheroma Intermittent claudication Critical limb ischemia Monckeberg 's arteriosclerosis Arteriolosclerosis Hyaline Hyperplastic Cholesterol LDL Oxycholesterol Trans fat Stenosis Carotid artery stenosis Renal artery stenosis Other Aortoiliac occlusive disease Degos disease Erythromelalgia Fibromuscular dysplasia Raynaud 's phenomenon Aneurysm / dissection / pseudoaneurysm torso : Aortic aneurysm Abdominal aortic aneurysm Thoracic aortic aneurysm Aneurysm of sinus of Valsalva Aortic dissection Coronary artery aneurysm head / neck Intracranial aneurysm Intracranial berry aneurysm Carotid artery dissection Vertebral artery dissection Familial aortic dissection Vascular malformation Arteriovenous fistula Arteriovenous malformation Telangiectasia Hereditary hemorrhagic telangiectasia Vascular nevus Cherry hemangioma Halo nevus Spider angioma Veins Inflammation Phlebitis Venous thrombosis / Thrombophlebitis primarily lower limb Deep vein thrombosis abdomen Hepatic veno - occlusive disease Budd -- Chiari syndrome May -- Thurner syndrome Portal vein thrombosis Renal vein thrombosis upper limb / torso Mondor 's disease Paget -- Schroetter disease head Cerebral venous sinus thrombosis Post-thrombotic syndrome Varicose veins Gastric varices Portacaval anastomosis Caput medusae Esophageal varices Hemorrhoid Varicocele Other Chronic venous insufficiency Chronic cerebrospinal venous insufficiency Superior vena cava syndrome Inferior vena cava syndrome Venous ulcer Arteries or veins Angiopathy Macroangiopathy Microangiopathy Embolism Pulmonary embolism Cholesterol embolism Paradoxical embolism Thrombosis Vasculitis Blood pressure Hypertension Hypertensive heart disease Hypertensive emergency Hypertensive nephropathy Essential hypertension Secondary hypertension Renovascular hypertension Benign hypertension Pulmonary hypertension Systolic hypertension White coat hypertension Hypotension Orthostatic hypotension GND : 4174101 - 8 NDL : 00567833 Retrieved from `` https://en.wikipedia.org/w/index.php?title=Portal_hypertension&oldid=810380431 '' Categories : Accessory digestive gland disorders Hepatology Hypertension Hidden categories : CS1 maint : Extra text : authors list Pages using citations with accessdate and no URL CS1 maint : Multiple names : authors list All articles with unsourced statements Articles with unsourced statements from November 2016 Wikipedia articles with GND identifiers Talk Contents About Wikipedia বাংলা Català Čeština Deutsch Español فارسی Français Galego Italiano Nederlands 日本 語 Polski Português Русский Српски / srpski Svenska தமிழ் 中文 Edit links This page was last edited on 14 November 2017, at 22 : 15. 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Question: what is the most common manifestation of portal hypertension–induced splenomegaly
Answer: Cirrhosis