Source of evidence

• Dosing guide on Octreotide: The dose of octreotide is 1–5 µg/Kg by bolus over 20 minutes, followed by continuous infusion at 2 µg/Kg/h and should be continued for 2–5 days. 

• Retrospective review of treatment of gastrointestinal bleeding and subsequent rates of re-bleed/readmission 
• Discussed treatments including endoscopy, sclerotherapy and proton pump inhibitors.
  

• The authors outline possible clinical presentations and treatments for major complications arising from portal hypertension, including gastrointestinal and variceal bleeds.  
• Outlined management options relating to a bleed: Intravenous fluid resuscitation, crystalloid fluids, Red blood cell transfusion (target haemoglobin of 70–80 g/L), Nil by mouth, nasogastric tube on free drainage, correct coagulopathy (Vitamin K, fresh frozen plasma) and thrombocytopenia (if less than 20 × 10 9 /L), empiric broad-spectrum antibiotics,  
• Recommended monitoring vital signs, urine output, conscious level, blood sugar and haemoglobin levels,  
• Dosing of IV Octreotide: 1–5 mcg/kg/hour continuous infusion,  
• Dosing of IV Omeprazole: 1 mg/kg/dose od, or IV Ranitidine 1–3 mg/kg/dose tds 

• The paper recommends the following, regarding management of acute variceal bleeding: Monitor vital signs, obtain venous access, FBC, INR, LFTs, UECs, crossmatch. PRBCs to maintain Hb 7g/dL, carefully avoiding a rebound fluid overload which would lead to rebleeding.
  

• Compared efficacy of primary surgical or endoscopic prophylaxis in children with portal hypertensio
• Discussed common causes of gastrointestinal bleeding in children and pathophysiology of portal hypertension 
• The authors thus concluded that prevention of the first bleed in children with high-risk varices can be achieved by surgery or endoscopic treatment, and decreases mortality and morbidity
  

Giouleme, O. & Theocharidou, E. (2013). Management of Portal Hypertension in Children With Portal Vein Thrombosis. Journal of Pediatric Gastroenterological Nutrition, October 2013, 57(4): 419-425. DOI: 10.1097/MPG.0b013e3182a1c d7f 

• Describes portal vein thrombosis aetiology, clinical presentation, diagnosis, and treatment of portal hypertension, including pharmacologic, endoscopic and surgical options.  
• PVT is a common cause of PH, variceal bleeding and splenomegaly are the two most common initial manifestations (p419).  
• Extrahepatic portal vein obstruction (EHPVO) is a major cause of PH in children (419).  
• Oesophageal varices are present in 90-95% of patients with PVT, with PV occlusion leading to vasodilation of the hepatic artery and formation of collateral vessels (419) Anorectal varices are also common.  
• The use of non-selective beta-blockers to prevent variceal bleeding in adults is established and comparable to EVL. Some small case-series paediatric studies have been undertaken (421).  
• In the setting of an acute variceal bleed event, beta blockers may inhibit the protective effect of tachycardia - a crucial compensatory mechanism for shock in children (421).  
• Vasoactive agents such as vasopressin and somatostatin induce splanchnic vasoconstriction, thereby decreasing portal venous inflow (421)  
• Sclerotherapy and EVL are highly effective in paediatric variceal bleed management (422). 

• Describing the best practice management of portal hypertension in children, including primary and secondary prophylaxis, Emergency therapy of variceal bleeding, Pharmacology, Endoscopy, Sengstaken-Blackmore tubes Surgical and interventional radiology and Sclerotherapy and ligation therapy. While the evidence in the paper is now outdated, practice remains unchanged, as far as can be determined.  
• The initial management of variceal bleeding is stabilization of the patient. Patients on beta blocker therapy may not manifest the usual compensatory tachycardia and are at higher risk of developing significant hypotension.
• Conservative fluid resuscitation in the form of crystalloid initially, followed by red blood cell transfusion, is critical to avoid overfilling the intravascular space and increasing portal pressure. 
• Platelets should be administered for levels less than 50 × 109/L, and coagulopathy corrected with vitamin K and fresh frozen plasma. There may be a value to the use of recombinant factor VIIa in severe coagulopathy as the fluid requirements may be diminished.  
• Intravenous antibiotic therapy should be considered for all patients with variceal bleeding in light of the high risk of potentially fatal infectious complications. Once the patient is stabilized, endoscopy should be performed to document that haemorrhage is indeed from variceal rupture.
• The Sengstaken-Blackmore tube (SSBT) was designed to stop hemorrhage by mechanically compressing esophageal and gastric varices. The device consists of a rubber tube with at least two balloons. It is passed into the stomach, where the first balloon is inflated and pulled up snug against the gastroesophageal junction. Once the tube is secured in place, the second balloon is inflated in the esophagus at a pressure (60-70 mmHg) that compresses the varices without necrosing the esophagus. A channel in the rubber tube allows gastric contents to be sampled for evidence of bleeding. This therapy is very effective in controlling acute bleeding but is associated with significant number of complications and high incidence of re-bleeding when the tube is removed.  
• Its use in children requires significant sedation. Use of the SSBT increases the risk of aspiration pneumonia, which can be a life threatening complication in a patient with liver failure. 
• Re-bleeding has been reported in 33%-60% of patients. Given these problems it is reserved for severe uncontrollable haemorrhage and generally serves as a temporizing measure until a more definite procedure can be performed.

• This paper makes recommendations for the acute management of major upper intestinal bleed in children, including securing the airway, obtaining IV access, blood investigations, fluid resuscitation; pharmacological therapies (treatment of coagulopathies, acid suppression, somatostatin analogues and antibiotics) and subsequent management with endoscopy and surgery.  
• The paper contains a useful algorithm (p209) for management of a patient with a significant GI bleed in children, which is comparable to the algorithm in the RCH Clinical Guideline.  
• Determine the presence of shock and haemodynamic instability (p208)  
• Support for and reestablishment of an effective circulating volume is a resuscitation priority (p208) – recommend 20ml/kg NaCl bolus then blood products  
• At cannulation, obtain Group & Hold and crossmatch, FBE, coags, LFTs, albumin, UECs, blood gas and lactate, BCs (p210) Vitamin K could be administered empirically and INR corrected with FFP (p212)   
• The use of PPIs is not without controversy, given they require liver metabolism, however no paediatric data available to suggest they are unsafe (p212).  
• Whether bacterial translocation occurs as sequela or plays a role in pathogenesis of haemorrhage is unclear. Usual choice of antibiotics is ampicillin and cefotaxime (p212). 
• Somatostatin analogues (eg octreotide, 1-2mcg/kg bolus followed by 1-5mcg/kg/hr). 

• Evaluated the use of octreotide for the control of acute upper gastrointestinal bleeding in children with portal hypertension.  
• Seven of the 13 children reviewed received a loading dose (1.27 ± 0.76 µg/kg), and the remaining five children received a median starting dose of 1.44 ± 1.19 µg/kg/h. The mean maximum dose was 1.68 ± 1.38 µg/kg/h.  
• Re-bleeding occurred in one third; hemostasis was eventually achieved in all. 
• The study found that octreotide infusion appears to be safe and effective in controlling pediatric upper gastrointestinal bleeding due to portal hypertension.
  

• Pathophysiology of portal hypertension and makes recommendations for the prevention and management of variceal bleeding in children.
  
• The paper provides some useful discussion on balloon tamponade and complications: “The length of the esophageal balloon limits the use of the Minnesota and Sengstaken-Blakemore tubes to children weighing more than 40kg.” “Bleeding can be stopped in almost 90% of cases by balloon tamponade; however, the tubes are only temporizing measures and cannot be kept in place for more than 24h [9]. “
  
• Tube placement should be attempted only by individuals who have been trained and are comfortable with their use, because complication rates are high when the tubes are not properly placed.
  
• Complications include esophageal perforation, aspiration, mucosal ischemia, and airway obstruction [9,10].
  
• When balloon tamponade is required, there should be a low threshold for intubation, and intubation is highly recommended.
  

Pimenta, J.R., Ferreira, A.R., Fagundes, E., Queiroz, T., Baptista, R. de Araújo Moreira, E.G., de Resende, C.B., Bittencourt, P., Carvalho, S.D., Neto, J. & Penna, F.J. (2017). Factors Associated With Bleeding Secondary to Rupture of Esophageal Varices in Children and Adolescents With Cirrhosis. Journal of Pediatric Gastroenterology and Nutrition. February, 64(2): e44–e48 

• This cross-sectional single centre study included 103 children and adolescents diagnosed with PHT and liver cirrhosis.  
• The most prevalent diagnoses in the study group were biliary atresia (33%), and autoimmune hepatitis (32%).  
• Patients with BA who had an UGIB had the bleed at a median age of 2.9 years. The autoimmune hepatitis group with UGIB had the bleed at median age 8.4 years (e46). The study found that statistically significant risk factors for UGIB include the presence of red spots on varices and the presence of gastric varices, consistent with other studies identified by the study authors (e47).
  

• It describes the more frequent aetiologies of GI bleeding in Indian children and treatment and management pathways for these children.  
• The paper contains a useful algorithm (p328) for management of a patient with a significant GI bleed, which is comparable to the algorithm in the RCH Clinical Guideline. 

• This review paper outlines primary care requirements for health practitioners to consider, when caring for children with portal hypertension.  
• It describes aetiology of disease, pathophysiology, clinical presentation, diagnostics, primary, secondary and tertiary treatment and management, including clinical pathways for referral if complications arise.  
• Complications discussed in the paper include haemorrhage from varices, ascites, hepato-pulmonary syndrome, porto-pulmonary hypertension, and hepatic encephalopathy (p39).  
• Variceal bleeding is the most serious complication of portal hypertension, which can occur from venous collaterals in the stomach or oesophagus. Varices and ascites are seen when portal pressure is 12 mm Hg or greater. This is not a subtle finding, as there is a 30% mortality with variceal bleeding (p 39). Acute management strategies listed in the paper include: octreotide, PPIs, PRBCs and/or platelets, and endoscopy. 

Wanty, C.,Helleputte, T., Smets, F., Sokal, E.M. & Stephenne, X. (2013). Assessment of risk of bleeding from esophageal varices during management of biliary atresia in children. Journal of Pediatric Gastroenterology and Nutrition, 56(5): 537–543. DOI:10.1097/MPG.0b013e318 282a22c 

• This retrospective study reviewed clinical, ultrasonographic, endoscopic, and laboratory data of 83 patients with biliary atresia from the beginning of medical management up to the occurrence of an upper gastrointestinal bleeding event.
• The researchers found that 20% of the patients had a gastrointestinal bleed incident, with a median age of 9.5 months (6-50 months).