Note: This guideline is currently under review. 

Introduction
Aim
Definition of Terms Assessment
Management
Resources                 
Evidence Table
References  

Introduction

Oesophageal variceal bleeds (and indeed any variceal bleeds) are a rare but serious complication of portal hypertension. Portal hypertension is defined by an increase in pressure within the portal circulatory system. This increase is caused by vascular resistance in blood flow through the liver. In RCH’s patient population, portal hypertension is frequently a result of biliary atresia, but it can also be a manifestation of post-hepatic, pre-hepatic (eg portal vein obstruction), or other intra-hepatic problems such as cystic fibrosis, congenital hepatic fibrosis or other cirrhosis. With increased resistance in the portal vascular system, blood begins to shunt through collateral systemic vessels to return to the vena cava. Prolonged elevation in portal vein pressure causes dilatation of the collateral vessels, which can form internal haemorrhoids at the rectum, caput medusae at the umbilicus and varices in the fragile gastro-oesophageal veins. Ruptures occur when the variceal wall tension exceeds the wall strength. Children with liver disease also have liver dysfunction, which results in clotting cascade problems and a deficit in Vitamin K-dependent factors. This adds to the risk of significant haemorrhage in this patient group.

In several large studies of children with portal hypertension, approximately 2/3 presented with haematemesis or melaena, usually from rupture of an oesophageal varix. Twenty to thirty percent of children with biliary atresia have variceal bleeds and they tend to develop varices early, with an estimated risk of bleeding of 15% before the age of two. Mortality rates associated with large bleeds range from 0-15%.

Aim

The aim of this guideline is to assist nurses and other health professionals in the acute management of infants and children experiencing an oesophageal variceal bleed. The guideline will also outline ongoing assessment and adjunctive therapies for the ongoing care of this patient group.

Definition of Terms

• ANTT – Aseptic Non-touch technique
• NGT – Nasogastric tube
• PPIs – Proton pump inhibitors (eg., omeprazole, pantoprazole)
• RBC –Red blood cells
• TIPSS – Transjugular Intrahepatic Porto-Systemic Shunt

Assessment – ABCDE

Initial acute 

• Airway – Does the patient have a patent airway? Are they at significant risk of losing their airway?
• Breathing – Respiratory rate, oximetry, respiratory effort, air entry?
• Circulation – Heart rate, blood pressure, central and peripheral capillary refill time? IV access? How much blood is the patient losing?
• Disability: Level of consciousness? Are they distressed? Irritable (consider encephalopathy)? Are they in pain? Are they febrile? Receiving antibiotics? Antihypertensives? Clotting agents? PPIs? Have they had recent endoscopy?
• Gastrointestinal: Are they vomiting blood (fresh or coffee ground)? Is there fresh (bright) or old (dark) blood in their stool? How much? Is the patient known to have varices? Do they have a history of bleeding? Do they have an NGT insitu? Is their abdomen more distended than usual? Do they have ascites? Do they have protruding umbilical veins?

Investigations

• FBE, UEC, VBG, Coagulation Screen, Group & hold, blood cultures, BGL, ammonia. 

Social history/issues

• Is there any family present? Have they been contacted?
• Has caregiver consent for theatre or blood transfusion been obtained (see Consent- Informed Procedure)?
• What are the education needs (patient and caregiver)?
  

Management

(Refer to algorithm overleaf)

Acute management

• Seek urgent medical/ ICU review/ MET (ext 22 22).
• Protect airway, support breathing as required (see Resuscitation guidelines). Give oxygen to patients with significant circulatory impairment or shock.
• Secure large bore IV access.
• Consider need to activate Massive Transfusion Procedure.
• Correct hypovolaemia, remembering the potential for harm with excessive fluid administration (no more than 20ml/kg NaCl bolus then albumin, RBCs if Hb <70g/L). Maintain strict fluid balance.
• Consider vasoactive therapy (Octreotide, 1-2mcg/kg bolus followed by 1-5mcg/kg/hr, max 1.5mg/kg/day). Always discuss with a fellow or consultant before commencing vasoactive therapy.
• Continuous cardio respiratory monitoring (see Clinical Guidelines (Nursing): Observation and Continuous Monitoring).
• Biochemistry investigations (as listed above).
• If NGT insitu, place on free drainage. Do NOT aspirate. NGT should only be inserted under endoscopic guidance or with gastroenterologist consent.
• Consider prophylatic intravenous antibiotics.
• Consider treating coagulopathies (vitamin K, platelets, cryoprecipitate and FFP).
• If bleeding is ongoing and uncontrollable, patient will require Balloon Tamponade (Foleys Catheter if child <15kg or Sengstaken Blakemore tube if child >15kg). (See Links and Clinician websites). This will ideally be performed in the ICU or theatre environment.
• Transfer to ICU or theatre for management as clinically appropriate.

Post Resuscitation Care

• Continuous cardio respiratory monitoring (see Clinical Guidelines (Nursing): Observation and Continuous Monitoring).
• Monitor bloods as clinically indicated (FBE, UEC, albumin, coags, ammonia, BGLs Patients receiving octreotide should have 4-6/24 BGLs monitoring).
• Gut rest may be ordered. 

Adjunctive (Non-acute) Therapies

• Diagnostic and surveillance endoscopy by gastroenterology team ±Sclerotherapy or Variceal Ligation.
• TIPPS or other surgical shunts if clinically indicated.
• Prophylactic PPIs and beta-blockers.
• Existing NGT should be left in-situ and only removed in the ICU or surgical environment.
• The Liver and Transplant Clinical Nurse Consultant will lead education and support for patients and caregivers. Reinforcement will be provided by ward nurses. General advice to parents: new medication actions, side effects, what concerns caregivers should report to the health care team.

Safety initiatives

• ANTT for CVAD management (see Central Venous Access Device)
• Contact precautions for body fluid exposure (see Infection Prevention and Control Policy).
• Blood transfusion safety adherence (see Blood Transfusion Procedure).
  

Algorithm

Resources

Links

• PICU Guidelines: Liver Protocols
  
• RCH Liver Transplant Protocol (Green Book)

Clinician websites

• American College of Gastroenterology Practice Guideline
• British Society of Gastroenterology Guidelines
• The National Institute for Health and Care Excellence Clinical Guideline
• Starship Liver Disease and Transplant Resource 
• World Gastroenterology Guideline

Information for parents

• RCH Support group 
• Children’s Liver Disease Foundation
• Starship Parent Guide to Liver Transplant
• Liver Kids Australia 

Evidence Table

Click here to view the evidence table.

References

• Avgerinos, A., Armonis, A., Stefanidis, G., Mathou, N., Vlachogiannakos, J., Kougioumtzian, A., Triantos, C., Papaxoinis, C., Manolakopoulos, S., Panani, A., & Raptis, S.A. (2004). Sustained rise of portal pressure after sclerotherapy, but not band ligation, in acute variceal bleeding in cirrhosis. Hepatology, 39(6): 1623-1630.
  
• Cales, P., Masliah, C., Bernard, B. Garnier, P.P., Silvain, C., Szostake-Talbodec, N., Bronowicki, J.P., Ribard, D., Botta-Fridlund, D., Hillon, P., Besseghir, K., & Lebrec, D. (2001). Early administration of vapreotide for variceal bleeding in patients with cirrhosis. New England Journal of Medicine; 334: 23-28.
  
• Costaguta, A., & Alvarez, F. (2012). Etiology and Management of Hemorrhagic Complications of Portal Hypertension in Children. International Journal of Hepatology, 1-8. 
  
• De Franchis, R. (2005). Evolving consensus in portal hypertension: Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. Journal of Hepatology, 43: 167-176.
  
• Felicilda-Reynaldo, R.F.D. (2012). Block the Bleed: Pharmacologic Therapies for Gastroesophageal Variceal Bleeding. MedSurg Nursing, 21(2): 107-111.
  
• Gana, J., Turner, D., Roberts, E., & Ling, S. (2010). Derivation of a Clinical Prediction Rule for the Noninvasive Diagnosis of Varices in Children. Journal of Pediatric Gastroenterology and Nutrition, 50(2): 188-193.
  
• Garcia-Pagán, J., & Bosch, J. (2005). Endoscopic band ligation in the treatment of portal hypertension. Nature Clinical Practice Gastroenterology & Hepatology, 2(11): 526-535.
  
• Garcia-Tsao, G., Sanyal, A.J., Grace, N.D., & Carey, W. (2007). American Association for the Study of Liver Diseases Practice Guidelines for the Prevention and Management of Gastroesophageal Varices and Variceal Hemorrhage in Cirrhosis. Hepatology, 46(3): 922-938.
  
• Ling, S.C. (2005). Should children with esophageal varices receive beta-blockers for the primary prevention of variceal hemorrhage? Canadian Journal of Gastroenterology, 19(11): 661–666.
  
• MIMS Australia (2018). Octreotide Acetate (DBL). Retrieved from: https://www.mimsonline.com.au/Search/FullPI.aspx?ModuleName=Product Info&searchKeyword=octreotide&PreviousPage=~/Search/QuickSearch.aspx&SearchType=&ID=75570001_2#an-Precautions7234
  
• Sarin, S., Kumar, A., Angus, P., Baijal, S., Baik, S., Bayraktar, Y., & ... Zhang, C. (2011). Diagnosis and management of acute variceal bleeding: Asian Pacific Association for Study of the Liver recommendations. Hepatology International, 5(2): 607-624.
  
• Shneider, B., Emre, S., Grozmann, R., Karani, J., McKiernan, P., Sarin, S., Shashidar, H., Squires, R., Superina, R., de Ville de Goyet, J., & de Franchis, R. (2006). Expert pediatric opinion on the Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. Pediatric Transplantation, 10(8): 893-907.
  
• Starship Children’s Health (2012). Paediatric Liver Disease and Liver Transplant: A Resource for Nurses in New Zealand. Retrieved from: https://www.starship.org.nz/media/170136/liver_transplant_nursing_resource_edition_oct_2012_web.pdf.
  
• Tsoi, K., Ma, T., & Sung, J. (2009). Endoscopy for upper gastrointestinal bleeding: how urgent is it? Nature Reviews Gastroenterology & Hepatology, 6(8): 463-469.
  
• Tursi, T. (2010). Use of beta-blocker therapy to prevent primary bleeding of esophageal varices. Journal of American Academy of Nurse Practitioners, 22(12): 640-647.
  
• Watson, E.F., & Church, N.I. (2013). Haematemesis and melaena. Africa Health, 35(5): 32-38.
  
  

Please remember to read the disclaimer.

The development of this nursing guideline was coordinated by Katherine Butler, RN, Emergency Department, and approved by the Nursing Clinical Effectiveness Committee. Updated October 2018.