View source for Gastrointestinal varices overview

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{{Gastrointestinal varices}}
{{CMG}}; {{AE}} {{HK}}

==Overview==
Gastrointestinal varices were initially described as “dilated [[veins]] that bulge into the [[Lumen (anatomy)|lumen]], producing uneven worm like surface of the inside of [[esophagus]]. Varices in the gastrointestinal tract are thought to arise due to increased pressure in the portal venous system, leading to establishment of porto-systemic shunts. In the 20th century, [[sclerotherapy]] became an important treatment option in the management of variceal haemorrhage, especially with the advent of fibre-optic endoscopy. In 1950, The Sengstaken-Blakemore tube’s use was first described by Sengstaken and Blakemore and later used as a treatment option. Gastrointestinal varices may be further classified into [[esophageal]] and [[gastric]] varices. [[Gastrointestinal varices|Esophageal varices]] may be further divided according to various classification systems such as the Dagradi classification, Conn's classification, Pachquet classification, Westaby classification, Soehendra classification and Cales classification. [[Gastrointestinal varices|Gastric varices]] may be classified according to Hoskins and Johnson’s classification, Mathur’s classification, Hashizume classification and Sarin's classification system. Varices arise from [[Hemodynamics|hemodynamic]] disturbance between the systemic and [[portal venous system]]. The majority of [[venous]] drainage of the [[gastrointestinal system]] occurs via the [[portal venous system]]. Whenever there is an interruption of drainage through the [[Portal venous system|portal system]] (for example due to [[cirrhosis]]), the [[Blood vessel|vessels]] contributing to the [[Portocaval anastomoses|porto-caval shunts]]<nowiki/>become more prominent due to increased pressure gradient. The interruption in [[blood flow]] leads to the creation collateral [[Blood vessel|vessels]] that involve [[veins]] of the [[esophagus]], [[stomach]], [[pelvis]] ([[hemorrhoids]]), [[retroperitoneum]], [[liver]], [[abdominal wall]], and other areas. Causes of gastointestinal varices include all the causes of increased [[Portal venous system|portal venous pressure]], which may be divided into [[Cirrhosis|cirrhotic]] and non-[[Cirrhosis|cirrhotic]] causes. Non-bleeding varices are [[asymptomatic]]. Ruptured esophageal and gastric varices may lead to [[upper gastrointestinal bleeding]] and present with [[hematemesis]]. They must be differentiated from other causes of [[upper gastrointestinal bleeding]] such as [[Peptic ulcer|peptic ulcer disease]], [[esophagitis]], [[gastritis]], [[angiodysplasia]], [[Dieulafoy's lesion]], vascular ectasia, [[Mallory-Weiss tear|Mallory-Weiss tears]], aorto-enteric fistulas and upper gastrointestinal [[tumors]]. Gastroesophageal varices are present in approximately 50000 per 100,000 patients with [[cirrhosis]], depending upon the clinical stage of the disease. The annual [[incidence]] of gastrointestinal varices ranges from a low of 7000 per 100,000 individuals to a high of 8000 per 100,000 individuals. Variceal [[hemorrhage]] occurs at a rate of around 10%-15% per year. If left untreated, recurrent variceal hemorrhage occurs in 60% of patients, usually within 1-2 years of the initial [[hemorrhage]]. Complications include, transient [[dysphagia]], [[chest pain]], [[esophageal]] [[ulceration]], ulcerogenic bleeding, post-therapeutic [[hemorrhage]], [[esophageal]] [[strictures]], [[Pleural effusion|pleural effusions]], [[pericarditis]] and [[portal vein thrombosis]]. Factors associated with a poor [[prognosis]] of presence of [[bacterial]] infections, HVPG >20 mm Hg, [[alcohol]] intake and [[obesity]]. The AIMS65 score is the best predictor of [[mortality]] in patients with variceal [[bleeding]]. [[Esophagogastroduodenoscopy]] ([[Esophagogastroduodenoscopy|EGD]]) is the [[Gold standard (test)|gold standard]] test for the [[diagnosis]] of gastrointestinal varices. [[Esophagogastroduodenoscopy|EGD]] should be performed once the [[diagnosis]] of [[cirrhosis]] is established. [[Physical examination]] of patients with gastrointestinal varices is usually remarkable for [[ascites]], [[pallor]], [[jaundice]], [[tachycardia]], low [[blood pressure]] in case of [[shock]], [[hepatomegaly]] or shrunken [[liver]] (in case of [[cirrhosis]]), [[spleenomegaly|splenomegaly]], [[altered mental status]], [[palmar erythema]], [[cyanosis]] and [[clubbing]]. X-ray taken after a barium swallow in cases of gastrointestinal varices may show multiple radiolucent filling defects. On [[Computed tomography|CT scan]] gastrointestinal varices may appear as well-defined, tubular structures that have a smooth appearance with homogeneous attenuation. [[Upper endoscopy]] may be helpful in the [[diagnosis]] of [[Esophageal varices|gasteroesophageal varices]]. Findings on an [[upper endoscopy]] diagnostic of [[esophageal varices]] include visible [[submucosal]] tortuous veins, congested [[veins]] without compression during air [[insufflation]], and grape-like [[Varicose veins|varicose]] veins that may occlude the [[lumen]]. Medical therapy in cases of gastrointestinal varices includes goal-directed management of the cause of [[portal hypertension]] along with specific management of varices after their development. The treatment is aimed at optimizing [[Portal venous system|portal venous]] inflow, portal pressure and portal resistance. The pharmacological therapy includes [[vasoconstrictors]] ([[beta blockers]]) and [[Venodilator|venodilators]] ([[nitrates]]). These therapies may be employed alone or in combination with endoscopic variceal ligation/[[sclerotherapy]] and [[Transjugular intrahepatic portosystemic shunt|transjugular intrahepatic shunt]] ([[Transjugular intrahepatic portosystemic shunt|TIPS]]) therapy depending upon the condition of the patient.

==Historical Perspective==
In 1850s, Sappey for the first time, described esophageal varices. In 1877, well before the role of [[portal hypertension]] in the development of variceal disease was understood, Nikolai Eck had already established the role of [[Portocaval anastomoses|port-caval shunts]] to relieve animals from [[ascites]]. Popularly known as the ‘Eck fistula’, these shunts were employed in the treatment of eight dogs, seven of which died post-operatively and one escaped the laboratory. In 1906 Gilbert and Villaret coined the term ‘portal hypertension’. In 1928, Wolf first showed esophageal varices on thin barium roentgenograms as small dilated structures with a [[Lumen (anatomy)|lumen]]. In 1931, Schatzki published the first findings of gastric varices on roentgenograms of five patients, followed by 45 further patients with esophageal and gastric varices in 1933. Varices were initially described as “dilated [[veins]] that bulge into the [[Lumen (anatomy)|lumen]], producing uneven worm like surface of the inside of [[esophagus]]. In 1939, Crafoord and Freckner discovered sclerotherapy with the help of [[quinine]] for the management of esophageal varices. In the 20th century, [[sclerotherapy]] became an important treatment option in the management of variceal haemorrhage, especially with the advent of fibre-optic endoscopy. In 1950, The Sengstaken-Blakemore tube’s use was first described by Sengstaken and Blakemore and later used as a treatment option. [[Ethanolamine oleate]], [[Sodium tetradecyl sulfate|sodium tetradecyl sulphate]], [[polidocanol]], sodium morrhuate and [[ethanol]] have been used as treatment options in [[sclerotherapy]]. In 1988, endoscopic variceal band ligation (EVBL) was first used for the treatment of esophageal varices, based on the concept of banding [[haemorrhoids]] with elastic O-rings. EVBL became a treatment option for the treatment of esophageal varices in 1990s. [[Antibiotics]] were recently used for the first time during the management of varices . [[Antibiotics]] were found to decrease the rate of [[bacterial]] infections, recurrent [[bleeding]] and improve [[mortality]] in patients [[bleeding]] from esophageal varices.

==Classification==

Gastrointestinal varices may be further classified into [[esophageal]] and [[gastric]] varices. [[Gastrointestinal varices|Esophageal varices]] may be further divided according to various classification systems such as the Dagradi classification, Conn's classification, Pachquet classification, Westaby classification, Soehendra classification and Cales classification. [[Gastrointestinal varices|Gastric varices]] may be classified according to Hoskins and Johnson’s classification, Mathur’s classification, Hashizume classification and Sarin's classification system. These classificatication systems are based on morphological features, [[anatomical]] location, size and color of the varices.

== Pathophysiology ==
Varices arise from [[Hemodynamics|hemodynamic]] disturbance between the systemic and [[portal venous system]]. The majority of [[venous]] drainage of the [[gastrointestinal system]] occurs via the [[portal venous system]]. Whenever there is an interruption of drainage through the [[Portal venous system|portal system]] (for example due to [[cirrhosis]]), the [[Blood vessel|vessels]] contributing to the [[Portocaval anastomoses|porto-caval shunts]]<nowiki/>become more prominent due to increased pressure gradient. The interruption in [[blood flow]] leads to the creation collateral [[Blood vessel|vessels]] that involve [[veins]] of the [[esophagus]], [[stomach]], [[pelvis]] ([[hemorrhoids]]), [[retroperitoneum]], [[liver]], [[abdominal wall]], and other areas.

==Causes==
Causes of gastointestinal varices include all the causes of increased [[Portal venous system|portal venous pressure]], which may be divided into [[Cirrhosis|cirrhotic]] and non-[[Cirrhosis|cirrhotic]] causes.

==Differentiating Gastrointestinal Varices From Other Diseases==
Non-bleeding varices are [[asymptomatic]]. Ruptured esophageal and gastric varices may lead to [[upper gastrointestinal bleeding]] and present with [[hematemesis]]. They must be differentiated from other causes of [[upper gastrointestinal bleeding]] such as [[Peptic ulcer|peptic ulcer disease]], [[esophagitis]], [[gastritis]], [[angiodysplasia]], [[Dieulafoy's lesion]], vascular ectasia, [[Mallory-Weiss tear|Mallory-Weiss tears]], aorto-enteric fistulas and upper gastrointestinal [[tumors]].

==Epidemiology and Demographics==
Gastroesophageal varices are present in approximately 50000 per 100,000 patients with [[cirrhosis]], depending upon the clinical stage of the disease. The annual [[incidence]] of gastrointestinal varices ranges from a low of 7000 per 100,000 individuals to a high of 8000 per 100,000 individuals. Variceal [[hemorrhage]] occurs at a rate of around 10%-15% per year and depends on the severity of liver disease, size of varices, and presence of red wale marks (areas of thinning of the variceal wall).

==Risk Fators==
The most potent [[risk factor]] for the development of gastrointestinal varices is increased [[Portal venous system|portal venous]] pressure. Conditions that predispose an individual to development of increased [[Portal venous system|portal venous]] pressure and consequently leading to varices can be divided into those leading to development of varices, those involved in progression of varices from small to large size and those leading to variceal [[hemorrhage]].

==Screening==
[[Screening (medicine)|Screening]] [[esophagogastroduodenoscopy]] ([[Esophagogastroduodenoscopy|EGD]]) for the diagnosis of esophageal and gastric varices is recommended after the diagnosis of [[cirrhosis]] is made.

==Natural History, Complications and Prognosis==
If left untreated, recurrent variceal hemorrhage occurs in 60% of patients, usually within 1-2 years of the initial [[hemorrhage]]. Gastrointestinal varices are an indication of increased [[Portal venous system|portal venous]] pressure, especially in [[Cirrhosis|cirrhotic]] patients. The progressive increase in [[portal]] pressure leads to a progressive increase in size of the varices and an increased [[vascular]] wall tension. Variceal [[hemorrhage]] resulting from rupture occurs when the expanding force exceeds the maximal wall tension. Complications include, transient [[dysphagia]], [[chest pain]], [[esophageal]] [[ulceration]], ulcerogenic bleeding, post-therapeutic [[hemorrhage]], [[esophageal]] [[strictures]], [[Pleural effusion|pleural effusions]], [[pericarditis]] and [[portal vein thrombosis]]. Factors associated with a poor [[prognosis]] of presence of [[bacterial]] infections, HVPG >20 mm Hg, [[alcohol]] intake and [[obesity]]. The AIMS65 score is the best predictor of [[mortality]] in patients with variceal [[bleeding]].

==Diagnosis==
===Diagnostic study of choice===
[[Esophagogastroduodenoscopy]] ([[Esophagogastroduodenoscopy|EGD]]) is the [[Gold standard (test)|gold standard]] test for the [[diagnosis]] of gastrointestinal varices. [[Esophagogastroduodenoscopy|EGD]] should be performed once the [[diagnosis]] of [[cirrhosis]] is established.

===History and symptoms===
Patients suffering from gastrointestinal varices may present with other [[Comorbidity|co-morbid]] conditions which lead to [[portal hypertension]]. [[Chronic liver disease]] and [[portal vein thrombosis]]<nowiki/>commonly lead to the development of gastrointestinal varices. Patients with a [[family history]] of venous abnormalities, [[Hypercoagulable state|hypercoaguable states]], [[autosomal recessive polycystic kidney disease]] (may lead to [[hepatic fibrosis]]), [[Nephronophthisis|nephronophthisis 1]], [[Joubert syndrome]] and related disorders 5, cranioectodermal dysplasia (Sensenbrenner syndrome), [[Ellis-van Creveld syndrome]], [[Jeune's disease|Jeune asphyxiating thoracic dystrophy]], [[renal]]-[[hepatic]]-[[pancreatic]] [[dysplasia]], [[autosomal dominant polycystic kidney disease]] have an increased risk of developing gastrointestinal varices. Non-[[bleeding]] gastrointestinal varices do not produce any [[symptoms]], however [[bleeding]] gastrointestinal varices may lead to [[hematemesis]], [[abdominal pain]], [[lightheadedness]], [[loss of consciousness]], [[melena]], [[bloody stools]] (in severe cases), [[shock]] (in case of loss of a large volume of [[blood]]).

===Physical examination===
[[Physical examination]] of patients with gastrointestinal varices is usually remarkable for [[ascites]], [[pallor]], [[jaundice]], [[tachycardia]], low [[blood pressure]] in case of [[shock]], [[hepatomegaly]]<nowiki/>or shrunken [[liver]] (in case of [[cirrhosis]]), [[spleenomegaly]], [[altered mental status]], [[palmar erythema]], [[cyanosis]] and [[clubbing]].

===Laboratory findings===
[[Cirrhosis]] of the [[liver]] is the most common cause of portal hypertension worldwide. A range of laboratory values may be obtained in the evaluation of [[cirrhosis]], in order to determine [[disease]] severity and [[Causality|causation]]. [[Liver function tests]], [[complete blood count]], [[basic metabolic panel]] and [[coagulation factors]] are standard in the evaluation of [[cirrhosis]]. More specific testing for markers and serum [[enzymes]] may be performed when certain [[Etiology|etiologies]] are suspected.

===Electrocardiogram===
[[Electrocardiogram]] ([[The electrocardiogram|EKG]]) in case of variceal [[bleeding]] after rupture may show [[sinus tachycardia]].

===Echocardiography/Ultrasound===
[[Doppler echocardiography|Echo-Doppler]] may be helpful in the diagnosis of [[portal hypertension]]. Findings on an [[Doppler echocardiography|echo-doppler]] suggestive of [[portal hypertension]] include lack of increase in [[portal vein]]<nowiki/>diameter in response to meals, increased portal [[blood flow]] velocity, and decreased [[portal vein]] cross-sectional area. [[Doppler ultrasonography|Color-Doppler ultrasound]] may be helpful in the diagnosis of [[portal hypertension]]. Findings on an [[Doppler ultrasonography|color-doppler ultrasound]] suggestive of [[portal hypertension]] include increased diameter of [[left gastric vein]], increased diameter of [[portal vein]], and increased flow velocity in [[left gastric vein]]. [[Biphasic]] and reverse flow in [[portal vein]] along with re-canalization of [[umbilical vein]] are [[pathognomonic]] for [[portal hypertension]].

===X-Ray===
X-ray taken after a barium swallow in cases of gastrointestinal varices may show multiple radiolucent filling defects.

===CT scan===
On [[Computed tomography|CT scan]] gastrointestinal varices may appear as well-defined, tubular structures that have a smooth appearance with homogeneous attenuation.

===MRI===
There are no abnormal [[Magnetic resonance imaging|MRI]] findings associated with gastrointestinal varices.

===Other imaging findings===
[[Upper endoscopy]] may be helpful in the [[diagnosis]] of [[Esophageal varices|gasteroesophageal varices]]. Findings on an [[upper endoscopy]] diagnostic of [[esophageal varices]] include visible [[submucosal]]<nowiki/>tortuous veins, congested [[veins]] without compression during air [[insufflation]], and grape-like [[Varicose veins|varicose]] veins that may occlude the [[lumen]]. Three dimensional [[portal]] [[venography]] may be helpful in the diagnosis of [[Esophageal varices|gasteroesophageal varices]]. Findings on a [[portal]] [[venography]] diagnostic of [[esophageal varices]] include reverse flow in [[Portocaval anastomoses|porto-systemic shunts]], [[collateral]] [[Vein|veins]] draining into [[Inferior vena cava|inferior vena cava (IVC)]], and other [[collateral]] [[veins]].

===Other diagnostic studies===
Hepatic venous pressure gradient (HVPG) is the difference between [[Hepatic vein|hepatic venous]] [[wedge pressure]] (HVWP) and free [[Hepatic vein|hepatic venous]] pressure (FHVP). HVPG reflects the intra-[[sinusoidal]] [[pressure]]. HVPG is measured through insertion of a [[catheter]] in right [[internal jugular vein]].

==Treatment==
===Medical therapy===
Medical therapy in cases of gastrointestinal varices includes goal-directed management of the cause of [[portal hypertension]] along with specific management of varices after their development. The treatment is aimed at optimizing [[Portal venous system|portal venous]] inflow, portal pressure and portal resistance. The pharmacological therapy includes [[vasoconstrictors]] ([[beta blockers]]) and [[Venodilator|venodilators]] ([[nitrates]]). These therapies may be employed alone or in combination with endoscopic variceal ligation/[[sclerotherapy]] and [[Transjugular intrahepatic portosystemic shunt|transjugular intrahepatic shunt]] ([[Transjugular intrahepatic portosystemic shunt|TIPS]]) therapy depending upon the condition of the patient.

===Surgery===

== Prevention ==

===Primary prevention===

===Secondary prevention===
[[Secondary prevention]] of gastrointestinal varices involves [[Prevention (medical)|prevention]] of rebleeding. The choice of method chosen for [[secondary prevention]] of gastrointestinal varices depends upon the condition of the patient, medication history and response to treatment. Methods used for [[secondary prevention]] include the use of [[beta blockers]], band ligation, [[Transjugular intrahepatic portosystemic shunt|TIPS]] and [[liver transplantation]].