When we think of severe hemorrhage in children, we typically think about trauma. Maybe injuries to the spleen or kidney come to mind. Perhaps thoughts of pelvic injuries or severe head injury dominate our considerations. Of course, we all have a healthy respect for post-tonsillectomy hemorrhage and we like to talk about Damage Control Resuscitation. Let us, however, not forget that children are, in fact, people… and people who have liver damage may develop portal hypertension and that creates another reason to bleed horrifically. Let us take a minute to digest a morsel on Acute Esophageal Variceal Bleeding in Children.
Acute Esophageal Variceal Bleeding: Basics
Pediatric liver disease may be due to numerous causes. [Banc-Husu, 2023]
- Inflammatory conditions
- Genetic and Metabolic diseases
Pediatric cirrhotic liver disease has many complications. [Banc-Husu, 2023; Luoto, 2020]
- Compensated cirrhosis may have:
- Portal Hypertension
- Decompensated cirrhosis may have:
- Ascites and Spotaneous Bacterial Peritonitis
- Hepatic encephalopathy
- Hepatopulmonary syndrome
- Portopulmonary hypertension
- Hepatic synthetic dysfunction
- Esophageal Variceal Hemorrhage
- Progressive cirrhotic disease is demonstrated by:
- Refractory ascites
- Hepatorenal Syndrome
Portal Hypertension and Esophageal Varices in Children
- Portal Hypertension occurs when the pressure difference between the portal and venous systems increases over the threshold (10 mmHg). [Luoto, 2020]
- Increase resistance can be related to pre-hepatic, intra-hepatic, or post-hepatic etiologies.
- Can be due to combination of causes also.
- Portal Hypertension in children is a heterogenous disease]. [Luoto, 2020]
- Pre-hepatic ex: Extra-hepatic portal vein obstruction is the most common
- Intra-hepatic ex: Biliary atresia, congenital hepatic fibrosis, and autoimmune liver diseases
- Post-hepatic ex: Budd-Chiari syndrome (rare in children)
- Increased resistance portal system pressures eventually leads to development of esophageal varices.
- Management and prevention of varices typically focuses on treatment of the underlying cause of the liver disease and also includes: [Banc-Husu, 2023; Luoto, 2020]
- Surveillance endoscopies
- Primary prophylaxis
- Surgical shunt procedures
- There is significant morbidity associated with acute variceal bleeding in children. [Carneiro de Moura, 2018]
- Seen in 57% of cohort.
- Infections occurred in 30% of those with acute variceal bleeding.
- Mortality, fortunately, is relatively low with appropriate care.
- Multimodal management can help avoid variceal bleeding. [Luoto, 2020]
Acute Esophageal Variceal Bleeding: Management
- Guidelines on the management of acute variceal bleeding in children are limited and debated due to limited pediatric-specific evidence. [Banc-Husu, 2023; Luoto, 2020; Carneiro de Moura, 2018]
- Basics are best (always)!
- Keep patient comfortable.
- Obtain large-bore access.
- Prepare airway management tools (hoping to avoid needing them).
- Type & Cross and check Coagulation Studies and CBC. (clearly, other labs may be useful later, but prioritize these.)
- Specific Therapies for Variceal Hemorrhage: [Luoto, 2020; Castillo, 2019; Garcia-Tsao, 2007]
- Antibiotic prophylaxis (usually for 7 days)
- Ceftriaxone is reasonable 1st choice.
- Fluoroquinolones are also reasonable.
- Pediatric patients with cirrhosis and acute Upper GI Bleeds are at risk of developing bacterial infections.
- This risk may be related to the disease process itself and/or the related risk that comes with repeated endoscopies/procedures.
- While most data is related to adults, there is evidence that antibiotics given to children with acute upper GI bleeds have positive impact on avoiding bacteremia and readmission rates. [Castillo, 2019]
- Somatostatin (or its analogues, like Octreotide)
- Guidelines do recommend this…
- There is great variability in its administration, though. [Gana, 2011]
- Endoscopy and other procedures
- Currently still debated, but reasonable to discuss additional options with your friendly Pediatric GI team.
- Call them early!
- Antibiotic prophylaxis (usually for 7 days)
Moral of the Morsel
- Liver Disease – it’s not just an adult problem. There are numerous conditions that may cause severe liver disease in children.
- It isn’t just a bleeding problem. There is a high risk for morbidity associated with acute variceal bleeding in children and infection needs to be high on our concern list.
- Kids are not aliens. They can get complications from portal hypertension just like adults… and those complications may need similar therapies that we use with our adult patients.
Banc-Husu AM, Shiau H, Dike P, Shneider BL. Beyond Varices: Complications of Cirrhotic Portal Hypertension in Pediatrics. Semin Liver Dis. 2023 Feb;43(1):100-116. doi: 10.1055/s-0042-1759613. Epub 2022 Dec 26. PMID: 36572031.
Luoto TT, Koivusalo AI, Pakarinen MP. Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices. J Pediatr Gastroenterol Nutr. 2020 May;70(5):628-634. doi: 10.1097/MPG.0000000000002643. PMID: 31990868.
Castillo L, Prachuapthunyachart S, Hall M, Shelby S, Quiros-Tejeira RE, Vo HD. Antibiotic use in cirrhotic children with acute upper gastrointestinal bleeding: A retrospective study using the pediatric health information system (PHIS) database. Medicine (Baltimore). 2019 Jul;98(29):e16505. doi: 10.1097/MD.0000000000016505. PMID: 31335721; PMCID: PMC6709249.
Carneiro de Moura M, Chen S, Kamath BM, Ng VL, Ling SC. Acute Variceal Bleeding Causes Significant Morbidity. J Pediatr Gastroenterol Nutr. 2018 Sep;67(3):371-376. doi: 10.1097/MPG.0000000000002039. PMID: 29851765.
Gana JC, Valentino PL, Morinville V, O’Connor C, Ling SC. Variation in care for children with esophageal varices: a study of physicians’, patients’, and families’ approaches and attitudes. J Pediatr Gastroenterol Nutr. 2011 Jun;52(6):751-5. doi: 10.1097/MPG.0b013e318213be81. PMID: 21593647.
Garcia-Tsao G, Sanyal AJ, Grace ND, Carey W; Practice Guidelines Committee of the American Association for the Study of Liver Diseases; Practice Parameters Committee of the American College of Gastroenterology. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology. 2007 Sep;46(3):922-38. doi: 10.1002/hep.21907. Erratum in: Hepatology. 2007 Dec;46(6):2052. PMID: 17879356.