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Portal Hypertension


Presentation

There is no specific symptom associated with portal hypertension. The chances of developing this disease increase with diseases of the liver such as cirrhosis. The complications or symptoms associated with portal hypertension are:

  • Gastrointestinal bleeding: If the stool has blood or appears tarry, it is a symptom of gastrointestinal bleeding. Sometimes blood in the vomit is caused due to spontaneous rupture of the varices and hence must not be neglected. 
  • Ascites: In this condition, there is an accumulation of the fluid in abdomen. 
  • Encephalopathy: Poor liver function can also cause confusion and forgetfulness.
  • Altered blood counts: Decline in the number of platelets and white blood cells (WBCs) are common in patients with portal hypertension [6].
Splenomegaly
  • The SBLS is more feasible and effective than ALS in patients with massive splenomegaly ( 30 cm) secondary to portal hypertension and liver cirrhosis.[ncbi.nlm.nih.gov]
  • In many reports, however, numerous treatment modalities focus on hypersplenism secondary to PH, irrespective of splenomegaly and PH. The proven benefits of laparoscopy seem especially applicable to patients with this complex disease.[ncbi.nlm.nih.gov]
  • Upon surgery, splenomegaly with polycystic content and varicose vessels over the omentum were noted. Autologous spleen transplantation was not performed because of limited orthotopic and vascularized spleen.[ncbi.nlm.nih.gov]
  • RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions.[ncbi.nlm.nih.gov]
  • Abstract We report 3 male IBD patients (2 Crohn's Disease, 1 Ulcerative Colitis) developing thrombocytopenia and splenomegaly on azathioprine treatment.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Abstract BACKGROUND Splenic arteriovenous fistula (AVF) is a rare cause of portal hypertension which may manifest with abdominal pain, diarrhea, ascites, and/or hematemesis. Fistula formation may be traumatic or spontaneous.[ncbi.nlm.nih.gov]
  • A 26-year-old male patient presented with abdominal pain. He had a history of operation for hydatid cyst of the lung 15 years ago. A left thoracotomy incision scar was observed during his physical examination.[ncbi.nlm.nih.gov]
  • CASE REPORT: A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours.[ncbi.nlm.nih.gov]
  • pain or fullness as a result Because portal hypertension is commonly caused by cirrhosis, liver failure symptoms can also indicate portal hypertension: Jaundice, or yellowing of the skin Easy bruising or bleeding Weakness Weight loss Abdominal pain Diagnosis[radiology.ucla.edu]
  • Portal hypertension symptoms may include: Vomiting blood - See a doctor immediately Confusion, forgetfulness or drowsiness - See a doctor immediately Difficulty breathing - See a doctor immediately Blood in stools Abdominal pain Abdominal swelling Dehydration[uvmhealth.org]
Melena
  • Dizziness, nausea, vomiting with red blood, melena was presented on admission. Esophagogastroduodenoscopy revealed III degree varicose of veins from middle part of the esophagus to cardiofundal part of the stomach.[ncbi.nlm.nih.gov]
  • Esophageal and gastric varices pose an ongoing risk of life-threatening hemorrhage, with hematemesis or melena. Back to top Vascular Surgery and Laser Vein Center Please contact us at 877.WMC.VEIN or 877.962.8346.[westchestermedicalcenter.org]
  • Patients present with sudden hematemesis and melena , as well as hypovolemic shock in some cases.[amboss.com]
  • Bleeding in the gut which may be sudden and severe : – Vomiting of blood (hematemesis) – Black tarry stools (melena) Fluid accumulation within the abdomen (ascites). Enlarged spleen (splenomegaly).[healthhype.com]
Loss of Appetite
  • Loss of appetite. Unplanned weight loss. Weakness and fatigue. Nausea. Jaundice (yellowing of your skin and eyes). Dark brown Red palms. Vomiting blood. Menstrual problems (in women). Mental confusion, such as a hard time focusing or remembering.[familydoctor.org]
  • […] of appetite Weight loss Portal Hypertension Causes The cause of portal hypertension is extensive.[healthhype.com]
Hematemesis
  • Abstract A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension.[ncbi.nlm.nih.gov]
  • Abstract BACKGROUND Splenic arteriovenous fistula (AVF) is a rare cause of portal hypertension which may manifest with abdominal pain, diarrhea, ascites, and/or hematemesis. Fistula formation may be traumatic or spontaneous.[ncbi.nlm.nih.gov]
  • CASE REPORT: A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours.[ncbi.nlm.nih.gov]
  • Esophageal and gastric varices pose an ongoing risk of life-threatening hemorrhage, with hematemesis or melena. Back to top Vascular Surgery and Laser Vein Center Please contact us at 877.WMC.VEIN or 877.962.8346.[westchestermedicalcenter.org]
  • Bleeding in the gut which may be sudden and severe : – Vomiting of blood (hematemesis) – Black tarry stools (melena) Fluid accumulation within the abdomen (ascites). Enlarged spleen (splenomegaly).[healthhype.com]
Abdominal Bruit
  • Physical examination revealed a soft abdomen, with no abdominal bruit, no pulsatile abdominal mass, and no stigmata of chronic liver disease. The hemoglobin declined acutely from 12.3 to 9.3 g/dL.[ncbi.nlm.nih.gov]
Jaundice
  • […] black stools Splenomegaly, or an enlarged spleen May have decreased platelets and left abdominal pain or fullness as a result Because portal hypertension is commonly caused by cirrhosis, liver failure symptoms can also indicate portal hypertension: Jaundice[radiology.ucla.edu]
  • Presentation History For causes of liver disease: History of jaundice. Alcohol consumption. Blood transfusion, especially abroad; lifestyles that predispose to hepatitis B or hepatitis C.[patient.info]
  • Symptoms Ascites (fluid build-up in the abdomen) Weight loss (malnutrition) Enlarged liver (at times) Varicose veins of the esophagus (varices) – with possible bleeding Jaundice (yellowing of the skin) Causes Cirrhosis of the liver (may be due to alcohol[vascularcures.org]
  • Jaundice (yellowing of your skin and eyes). Dark brown Red palms. Vomiting blood. Menstrual problems (in women). Mental confusion, such as a hard time focusing or remembering. Itchy skin.[familydoctor.org]
Hepatomegaly
  • Subsequent Problems That Can Develop Due to Portal Hypertension: Hepatomegaly (enlarged liver), splenomegaly (enlarged spleen due to increased pressure in the liver), esophageal varices (distended, weakened, and blood-filled veins under pressure, or sacs[arpkdchf.org]
  • .- ) cystic disease of liver (congenital) ( Q44.6 ) hepatic vein thrombosis ( I82.0 ) hepatomegaly NOS ( R16.0 ) pigmentary cirrhosis (of liver) ( E83.110 ) portal vein thrombosis ( I81 ) toxic liver disease ( K71.- ) Other diseases of liver Clinical[icd10data.com]
  • Ascites Caput medusae Hepatomegaly and splenomegaly Haemorrhoids Oesophageal varices (and thus haematemesis) Major complications of portal hypertension include the following: Variceal haemorrhage Portal hypertensive gastropathy Spontaneus bacterial peritonitis[derangedphysiology.com]
  • Hepatomegaly may also be seen, but the liver parenchyma is of normal echo texture.[saudijgastro.com]
Hepatosplenomegaly
  • CT scan revealed massive hepatosplenomegaly with focal splenic lesions, soft tissue around renal pelvis, mesenteric masses compressing bowel loops and perilymphatic nodules in lungs.[ncbi.nlm.nih.gov]
Asterixis
  • They may also have a flapping tremor (asterixis), fetor hepaticus (a sweet musty aroma of the breath), hyperventilation and hypothermia.[patient.info]
Confusion
  • Portal hypertension itself does not cause symptoms, but complications from the condition can lead to an enlarged abdomen, abdominal discomfort, confusion, drowsiness and internal bleeding.[rarediseases.info.nih.gov]
  • Portal hypertension can lead to a swollen abdomen ( ascites ), abdominal discomfort, confusion, and bleeding in the digestive tract.[msdmanuals.com]
  • You may also receive an antibiotic such as Rifaximin and/or lactulose, a type of laxative, to reduce confusion and other mental changes resulting from encephalopathy.[nyp.org]
  • Encephalopathy: Confusion and forgetfulness caused by poor liver function and the diversion of blood flow away from your liver. Reduced levels of platelets or decreased white blood cell count. Cleveland Clinic News & More Cleveland Clinic News & More[my.clevelandclinic.org]

Workup

  • Complete blood count: Though this is not a specific test to ascertain diagnosis of the disease, it is still advised. In patients with portal hypertension, levels of platelets and WBCs fall.
  • Liver function tests: Determining the levels of aspartate aminotransferase [AST], alanine aminotransferase [ALT], and bilirubin determines the functioning of the liver. 
  • Coagulation studies: Tests related to coagulation of blood can also prove beneficial in diagnosing the portal hypertension. Some of such tests are: prothrombin time [PT], international normalized ratio [INR] and partial thromboplastin time [PTT]. 
  • Some of the other important tests are: Blood urea nitrogen level, levels of creatinine and electrolytes, pH, arterial blood gas, iron indices, hepatic and hepatitis serologies, albumin and antinuclear antibody levels.
  • Imagining studies: Such studies include Duplex Doppler ultrasonography, magnetic resonance imaging, bleeding scan and CT scan. 
Gastric Varices
  • The management of oesophageal and gastric varices is particularly important, and both the emergency management together with prophylactic management of this condition are described.[ncbi.nlm.nih.gov]
  • KEYWORDS: Banding ligation; Endoscopic obturation; Esophageal varices; Gastric varices; Sclerotherapy[ncbi.nlm.nih.gov]
  • Cumulative occurrence rate of complete response (CR), progression-free survival (PFS), and overall survival (OS) were compared between patients with CEPH and those without CEPH (esophageal/gastric varices or low platelet count [ 3 ] associated with splenomegaly[ncbi.nlm.nih.gov]
  • UGIB occured in terms of refractory hemorrhage from gastric varices (3/9; 33%), hemorrhage from esophageal varices (3/9; 33%), and finally, hemorrhage from portal-hypertensive gastropathy (3/9; 33%).[ncbi.nlm.nih.gov]
Enlargement of the Spleen
  • Other symptoms associated with portal hypertension include enlarged veins, enlargement of the spleen , as well as anemia and vomiting blood.[belmarrahealth.com]
  • Splenomegaly (enlargement of the spleen) with consequent sequestration therein of red blood cells, white blood cells, and platelets, together leading to mild pancytopenia.[westchestermedicalcenter.org]
  • These include: Enlarged liver and spleen Enlarged veins (varices) of the esophagus and stomach. These can cause abnormal bleeding, such as vomiting blood.[urmc.rochester.edu]
  • These include: Enlarged liver and spleen Varicose veins of the esophagus and stomach, which can cause abnormal bleeding, such as vomiting of blood Internal hemorrhoids Weight loss from malnutrition Ascites, or fluid buildup in the abdomen Kidney malfunction[urmc.rochester.edu]
  • Many of the imaging studies such as CT, MRI and ultrasound can also show signs of portal hypertension such as varices and enlargement of the spleen.[vascularcures.org]
Paracoccidioides Brasiliensis
  • KEYWORDS: Acute juvenile paracoccidioidomycosis; Complications; Hepatic disease; Paracoccidioides brasiliensis S1; Portal hypertension[ncbi.nlm.nih.gov]
Pancreatic Necrosis
  • KEYWORDS: Acute pancreatitis; Collaterals; Gallstones; Portal hypertension; Self-expanding metallic stents; Transmural drainage; Walled-off pancreatic necrosis[ncbi.nlm.nih.gov]

Treatment

Treatment of portal hypertension is focused on the prevention and management of the complications associated with the disease, especially bleeding. The severity of the disease is dependent on the extent of liver function. Modification in diet, medications, surgery and endoscopic procedure can help combat the complications of portal hypertension.

  1. Endoscopic therapy: This procedure is the first line of treatment for variceal bleeding. Gastroenterologist can also use rubber bands to stop the bleeding by a procedure called banding. In cases where banding cannot be done, sclerotherapy is the option. In this procedure, a blood-clotting solution is injected to stop bleeding.
  2. Medications: Beta-blockers such as nadolol and propranolol may be prescribed which reduce the pressure in the varices, reducing the risk of bleeding. Mental changes that accompany encephalopathy can be managed with lactulose.
  3. Transjugular intrahepatic portosystemic shunt (TIPS): In this method, a stent is placed in the middle of the liver rerouting the blood flow to the liver.
  4. Devascularization: This is a surgical procedure to remove the bleeding varices.
  5. Liver transplant: In case of end stage liver disease, transplantation of the liver is the only option [10].

Prognosis

Higher mortality and morbidity rates are reported in patients with severe and persistent gastrointestinal hemorrhage. 90% of complicated cases (due to cirrhosis) of portal hypertension cause variceal hemorrhage.

The mortality rate of the patients who show the first episode of variceal hemorrhage is around 50%, while that with known diagnosis of esophageal varices is 30%.

Patients who report at least one episode of bleeding from the esophageal varices have 80% chances of re-bleeding within 1 year. In patients with esophageal varices, complications arising from immune, cardiovascular, pulmonary, cardiovascular systems contribute to around 20-65% of the deaths [5].

Complications

Common complications associated with portal hypertension are hepatic encephalopathy, aspiration pneumonia, sepsis, renal failure, cardiomyopathy, arrhythmias and hypotension.

Etiology

Cirrhosis of the liver is the most important cause of portal hypertension. Cirrhosis is the aftermath (healing) of the liver injury which is caused by hepatitis, or excessive alcohol consumption. The scar in the liver, which is caused due to cirrhosis, blocks the flow of blood through this organ affecting its function.

There are some other causes of portal hypertension namely:

  1. Blood clots in the portal vein 
  2. Blockage of the veins 
  3. Schistosomiasis: Parasitic infection
  4. Focal nodular hyperplasia [2]

Epidemiology

Portal hypertension is the most common manifestation of the liver. In the United States, the deaths associated with the liver cirrhosis (of which portal hypertension is a manifestation) is around 30,000. The international data for portal hypertension is not known, though 30% and 70% of patients with compensated and decompensated cirrhosis respectively suffer from portal hypertension at the time of diagnosis.

In Sub-Saharan Africa, Sudan and Egypt, the most common cause of portal hypertension is Schistosomiasis, while in South America, the Far-East and Southeast Asia, hepatitis B contribute to this condition.

More than 60% of the patients with cirrhosis (which eventually causes portal hypertension) are men; hence gender plays a significant important role in the etiology of the disease. Among children, portal vein thrombosis and secondary biliary cirrhosis contribute to esophageal varices [3] [4].

Sex distribution
Age distribution

Pathophysiology

Portal vein carries the blood from the large and small intestine, stomach and spleen to the liver. Any obstruction of this vein can cause complications leading to portal hypertension.

This change causes an increase in the venous pressure, leading to development of collateral circulation which diverts the blood to the systemic veins. High portal pressure is responsible for the development of the portosystemic collaterals, including esophageal varices [7] [8] [9].

Prevention

In patients with increased risk of portal hypertension, beta-blockers must be used to prevent the first variceal hemorrhage. To prevent the variceal re-bleeding, the combination of non-selective beta-blockers and a nitrate has been shown to be effective [11].

Summary

When there is an increase in blood pressure in the system of veins referred to as a portal system, it is called portal hypertension.

Normal portal pressure ranges between 5 mmHg and 10 mmHg. Generally a portal blood pressure of greater than 12 mmHg causes complications such as ascites and varices. Two factors that contribute to the development of portal hypertension are vascular resistance and blood flow [1].

Patient Information

Definition

Portal hypertension is the elevation of the blood pressure in the portal vein. The portal vein is vital for the normal functioning of the body.

Causes

Obstruction in the portal vein (the blood vessel that carries blood from the intestines, stomach and spleen to the liver) leads to the development of collateral circulation, causing portal hypertension.

Symptoms

Though there are no specific symptoms of the disease, blood in the stool or in vomit can be considered as reason enough to visit the doctor. Some of the other symptoms of portal hypertension are

Diagnosis

Diagnosis of the disease can be done by blood-tests, X-rays and endoscopic examination for the presence of ascites and/or varices.

Treatment

Though the treatment of the disease remains a challenge, management strategy of disease is focused on the prevention of the complications. Beta-blockers and lactulose are some medications which are prescribed to patients with portal hypertension. Treatment with endoscopic therapy has been proved to be most effective. 

References

Article

  1. Harmanci O, Bayraktar Y. Portal hypertension due to portal venous thrombosis: etiology, clinical outcomes. World J Gastroenterol. 2007 May 14;13(18):2535-40.
  2. Lubel JS, Angus PW. Modern management of portal hypertension. Intern Med J. Jan 2005;35(1):45-9.
  3. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey WD, and the Practice Guidelines Committee of the American Association for the Study of Liver Diseases, the Practice Parameters Committee of the American College of Gastroenterology. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Am J Gastroenterol. Sep 2007;102(9):2086-102.
  4. Kim WR, Brown RS Jr, Terrault NA, et al. Burden of liver disease in the United States: Summary of a workshop conducted by the AASLD. May 2001.
  5. Benedeto-Stojanov D, Nagorni A, Bjelakovic G, et al. The model for the end-stage liver disease and Child-Pugh score in predicting prognosis in patients with liver cirrhosis and esophageal variceal bleeding. Vojnosanit Pregl. 2009 Sep;66(9):724-8.
  6. Barua UK, Hossain AS, Roy GC, et al. Portopulmonary hypertension. Mymensingh Med J. 2013 Jul;22(3):618-24.
  7. Obara K. Hemodynamic mechanism of esophageal varices. Dig Endosc. Jan 2006;18(1):6-9.
  8. Ravindra KV, Eng M, Marvin M. Current management of sinusoidal portal hypertension. Am Surg. Jan 2008;74(1):4-10. 
  9. Cichoz-Lach H, Celinski K, Slomka M, et al. Pathophysiology of portal hypertension. J Physiol Pharmacol. 2008 Aug;59 Suppl 2:231-8.
  10. Bari K, Garcia-Tsao G. Treatment of portal hypertension. World J Gastroenterol. 2012 Mar 21;18(11):1166-75.
  11. Garcia-Tsao G, Sanyal AJ, Grace ND, et al. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology, vol. 46, no. 3, pp. 922–938, 2007.

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Last updated: 2018-06-22 04:29