DESCRIPTION

• Increased portal venous pressure results in portal–systemic shunts.
• Shunts at gastroesophageal junction result in fragile submucosal esophageal varices.

ETIOLOGY

• 10–30% of all cases of upper GI bleeding
• 90% of upper GI bleeding in patients with cirrhosis
• Variceal hemorrhage occurs in 30% of patients with cirrhosis:
  • 50% will stop bleeding spontaneously
  • 30% mortality per episode
  • 70% have recurrent bleeding
• In adults:
  • Cirrhosis due to alcoholism or chronic hepatitis
  • Storage disease: Wilson or hemochromatosis
  • Middle East: Schistosomiasis
• In children:
  • Intrahepatic obstruction from biliary cirrhosis
  • Biliary atresia
  • Cystic fibrosis
  • β-antitrypsin deficiency
  • Hepatitis

[Outline]

• DESCRIPTION
• ETIOLOGY

SIGNS AND SYMPTOMS

• General:
  • Weakness and fatigue
  • Tachycardia
  • Tachypnea
  • Hypotension
  • Cool, clammy skin; prolonged capillary refill
• Abdominal:
  • Significant active upper GI bleeding:
    • Hematemesis
    • Hematochezia
    • Melena
    • 20–40% of total blood volume loss possible
  • Abdominal pain
• Stigmata of severe hepatic dysfunction:
  • Jaundice
  • Spider angiomata
  • Palmar erythema
  • Pedal edema
  • Hepatosplenomegaly
  • Ascites
• History of portal hypertension:
  • Most commonly alcoholic cirrhosis
  • Others, including:
    • Primary biliary cirrhosis
    • Schistosomiasis
    • Budd–Chiari syndrome
    • Severe CHF
    • Sarcoidosis
• Cardiovascular:
  • Chest pain/shortness of breath
• CNS:
  • Syncope
  • Confusion and agitation initially
  • Lethargy and obtundation later

Pediatric Considerations

• Massive hematemesis: Typical initial presentation:
  • Hypotension may be a late finding.

History

• Gastroesophageal varices are present in 50% of patients with cirrhosis and correlate with severity of disease.
• The most important predictor of hemorrhage is size of the varices. Other factors include number of varices, severity of hepatic disease and endoscopic findings.
• Patients with PBC develop varices and variceal hemorrhage early in their course of disease, even prior to development of cirrhosis.

Physical Exam

• Vitals signs may be normal or may show tachycardia (early) and hypotension (late).
• Altered mental status with encephalopathy or poor perfusion
• Active hematemesis
• Stigmata of alcoholic liver disease:
  • Ascites
  • General edema
  • Jaundice

ESSENTIAL WORKUP

• Gastric tube placement:
  • Determines whether patient is actively bleeding
  • Decompresses stomach that may aid in hemostasis. Possible role in reducing aspiration risk
  • Facilitates endoscopic exam
  • Will not increase or cause esophageal variceal bleeding
• Emergent endoscopy

DIAGNOSIS TESTS & INTERPRETATION

Lab

• Type and cross-match 6–8 U:
  • Significant transfusion requirements
• ABG for:
  • Acidosis
  • Hypoxemia
• CBC:
  • Hematocrit is an unreliable indicator of early rapid blood loss.
  • Perform serial CBCs to follow blood loss.
• Electrolytes, BUN, creatinine, glucose:
  • Evaluate renal function.
  • BUN:creatinine ratio > 30 suggest significant blood in GI tract.
• PT/PTT/INR and platelets:
  • Coagulopathy
  • Prolonged bleeding times
  • Thrombocytopenia

Imaging

• Chest radiograph (portable) for aspiration/perforation
• ECG for myocardial ischemia

DIFFERENTIAL DIAGNOSIS

• Bleeding/perforated peptic ulcer
• Erosive gastritis
• Mallory–Weiss syndrome
• Boerhaave syndrome
• Aortoenteric fistula
• Gastric varices
• Gastric vascular ectasia

[Outline]

• SIGNS AND SYMPTOMS
• ESSENTIAL WORKUP
• DIAGNOSIS TESTS & INTERPRETATION
• DIFFERENTIAL DIAGNOSIS

PRE-HOSPITAL

• Airway stabilization
• Treat hypotension 0.9% normal saline infusion bolus through 2 large-bore 16G or large IV lines.
• Cardiac and pulse oximetry monitoring

INITIAL STABILIZATION/THERAPY

• ABCs with early aggressive airway control/intubation:
  • Early intubation = easier intubation
  • For AMS or massive hemoptysis
  • Facilitates emergency endoscopy
• Establish central IV access with invasive intravascular monitoring for hypotension not responsive to initial fluid bolus.
• Replace lost blood as soon as possible:
  • Initiate with O-negative blood until type-specific blood available.
  • 10 mL/kg bolus in children
  • Fresh-frozen plasma and platelets may be required.
• Place gastric tube nasally (awake) or orally (intubated)
• Controversy:
  • Overly aggressive volume expansion may lead to rebound portal HTN, rebleeding, and pulmonary edema.
  • Transfusion goal is Hb = 8.
  • rFVIIa may decrease hemostasis failure rates in Child–Pugh class B/C patients

Pediatric Considerations

• Initiate intraosseous access if peripheral access unsuccessful in unstable patient.
• Most bleeding in children stops spontaneously.
• Vital sign changes may be a late finding in children:
  • Subtle changes in mental status, capillary refill, mild tachycardia, or orthostatic changes may indicate significant blood loss.
  • Overaggressive correction in infants can quickly lead to significant electrolyte abnormalities.

ED TREATMENT/PROCEDURES

• Emergent endoscopy required for active bleeding:
  • Use pharmacologic and tamponade devices as temporizing measures.
• Endoscopy
  • Emergent with active bleeding in nasogastric tube
  • Procedure of choice in acute esophageal bleeding
  • Esophageal band ligation equivalent to sclerotherapy with fewer complications:
    • May be difficult to visualize in cases of massive bleeding
  • Sclerotherapy with massive bleeding
  • Gastric varices are not amenable to endoscopic repair due to high rebleeding rate:
    • Treat pharmacologically.
  • Administer antibiotics at time of procedure to decrease risk for spontaneous bacterial peritonitis:
    • Fluoroquinolone or ceftriaxone
• Pharmacological Therapy
  • Somatostatin is 1st-line therapy where available (not widely available in US) due to greater efficacy and fewer side effects when compared to octreotide
  • Octreotide is 1st-line therapy where somatostatin not available:
    • Complications include hyperglycemia and abdominal cramping.
  • Vasopressin replaced by octreotide/somatostatin secondary to high incidence of vascular ischemia
• Balloon Tamponade
  • Initiate in massive uncontrollable bleed.
  • Sengstaken–Blakemore and Minnesota tubes
  • Applies direct pressure but risks esophageal perforation and ulceration
  • Temporary benefit only with massive uncontrolled bleeding in the hands of experienced clinician
• Refractory Bleeding Therapy
  • Interventional radiology:
    • Transjugular intrahepatic portosystemic shunt procedure. Recommended for refractory gastric varices or for patients who are poor surgical candidates
  • Surgical options:
    • Portacaval shunt
    • Variceal transection
    • Stomach devascularization
    • Liver transplantation

MEDICATION

• Ceftriaxone: 2 g (peds: 50–75 mg/kg/24 h) IV q24h in Child–Pugh class B/C or in quinolone-resistant areas
• Cefotaxime: 2 g (peds: 50–180 mg/kg/24 h) IV q8h
• Erythromycin 250 mg IV:
  • Shown to aid in gastric clearing for better visualization during endoscopy
• Norfloxacin 400 mg PO q12 or Ciprofloxacin 500 mg IV q12 if cannot tolerate PO (contraindicated in peds)
• Octreotide: 50 µg bolus, then 50 µg/h infusion for 5 days
• Somatostatin: 250 µg IV bolus followed by 250 µg/h IV infusion for 5 days

First Line

• Somatostatin or octreotide (if somatostatin not available)
• Norfloxacin PO or ciprofloxacin IV

Second Line

• Erythromycin
• Ceftriaxone

[Outline]

• PRE-HOSPITAL
• INITIAL STABILIZATION/THERAPY
• ED TREATMENT/PROCEDURES
• MEDICATION

DISPOSITION

Admission Criteria

• ICU admission for actively bleeding varices
• Recent history of variceal bleeding
• High risk for early rebleeding:
  • Age > 60 yr, renal failure, initial hemoglobin count < 8

Discharge Criteria

Nonbleeding varices

Issues for Referral

• Continued hemorrhage requiring surgery or higher level of care
• Liver transplant

FOLLOW-UP RECOMMENDATIONS

• Timely outpatient GI follow-up:
  • Will need annual surveillance endoscopies
• Medication and lifestyle modifications

[Outline]

• DISPOSITION
• FOLLOW-UP RECOMMENDATIONS

• Intubate early, especially in patients with hepatic encephalopathy or hemodynamic instability.
• Begin prophylactic antibiotics prior to endoscopy. Improves survival
• In US, octreotide has replaced vasopressin owing to better side-effect profile. If vasopressin is required, use IV nitroglycerin infusion concomitantly to reduce end-organ ischemia.
• Control the airway prior to placement of balloon tamponade device, which provides only a temporizing measure prior to surgery or TIPS
• Hematochezia in a hemodynamically unstable patient is an upper GI bleed until proven otherwise.
• Consult your GI specialists early, since endoscopy is the 1st-line diagnostic and therapeutic procedure.

ICD9

• 456.0 Esophageal varices with bleeding
• 456.1 Esophageal varices without mention of bleeding
• 456.21 Esophageal varices in diseases classified elsewhere, without mention of bleeding

ICD10

• I85.00 Esophageal varices without bleeding
• I85.01 Esophageal varices with bleeding
• I85.10 Secondary esophageal varices without bleeding

[Outline]

• ICD9
• ICD10

• Garcia-Tsao G, Sanyal AJ, Grace ND, et al. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Am J Gastroenterol. 2007;102:2086–2102.
• Nevens F. Review article: A critical comparison of drug therapies in currently used therapeutic strategies for variceal haemorrhage. Aliment Pharmacol Ther. 2004;20(suppl 3):18–22.
• Sass DA, Chopra KB. Portal hypertension and variceal hemorrhage. Med Clin N Am. 2009;93:837–853.

See Also (Topic, Algorithm, Electronic Media Element)

• Cirrhosis
• Gastrointestinal Bleeding

Galeta C. Clayton