Published online: 2019-08-27 214 Endovascular and Percutaneous Treatment of Pediatric Portosystemic Varices: A Case Review Alexander Dabrowiecki, MD1 Eric J. Monroe, MD2 Rene Romero, MD3 Anne E. Gill, MD1,4 C. Matthew Hawkins, MD1,4 1 Department of Radiology and Imaging Sciences, Emory University Address for correspondence Alexander Dabrowiecki, MD, 1364 School of Medicine, Atlanta, Georgia Clifton Rd. NE, Suite #D112, Atlanta, GA 30322 2 Department of Interventional Radiology, Seattle Children’s Hospital, (e-mail: [email protected]). Seattle, Washington 3 Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Children’s Pediatric Institute, Children’s Healthcare of Atlanta and Emory University, Atlanta, Georgia 4 Division of Interventional Radiology and Image Guided Medicine, Children’s Pediatric Institute, Children’s Healthcare of Atlanta and Emory University, Atlanta, Georgia Dig Dis Interv 2019;3:214–226. Abstract Portal hypertension is a significant cause of morbidity and mortality in pediatric patients. Complications of portal hypertension include development of portosystemic varices. The most common type of portosystemic varices are gastroesophageal varices; however, other ectopic varices can also be a cause of recurrent, life-threatening gastrointestinal bleeding. Problematic ectopic varices include isolated gastric, ano- rectal, small bowel, roux-limb, and stomal varices. There are no standardized treatment Keywords guidelines on how to manage ectopic varices in children; however, new innovations in ► portosystemic varices endovascular treatment options provide potential therapeutic alternatives when ► ectopic varices varices are refractory to conventional therapy. This review provides a case-based ► venous intervention literature review for endovascular treatment of isolated gastric, anorectal, small bowel, ► pediatric roux-limb, and stomal ectopic varices in children (age 0-9 years) and adolescents (age interventions 10-19 years). Portal hypertension in children is a significant cause of feared complication of all types of EcV. Traditionally, in morbidity and mortality. One common complication associ- children, most EcV have been treated both endoscopically ated with portal hypertension is the development of porto- and surgically. However, minimally invasive percutaneous systemic varices, such as gastroesophageal and other ectopic and endovascular therapies have evolved as potential thera- varices (EcV). Portal hypertension in adults is defined as a peutic options to treat EcV not amenable to endoscopic or hepatic venous pressure gradient greater than 5 mm Hg, and surgical treatment in children. Transjugular intrahepatic variceal formation occurs from a hepatic venous pressure portosystemic shunt (TIPS) creation has been extensively This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. gradient 10 mm Hg leading to venous collateralization, described in the peer-review literature to treat pediatric – classically in predictable locations.1 The most common type esophageal and gastroesophageal varices,2 5 and will, thus, of varices are esophageal and gastroesophageal varices. not be extensively reviewed herein. This review, rather, However, less-common, yet equally as problematic, EcV focuses on the literature to date in a case-based and loca- include isolated gastric, anorectal, small bowel (most com- tion-based approach to percutaneous and/or endovascular monly duodenal), roux-limb, and stomal varices (►Figs. 1 treatment of pediatric isolated gastric, anorectal, small bow- and 2). Acute life-threatening variceal bleeding is the most el, roux-limb, and stomal EcV. received Issue Theme Pediatric Gastrointestinal Copyright © 2019 by Thieme Medical DOI https://doi.org/ April 10, 2019 Interventions; Eric J. Monroe, MD Publishers, Inc., 333 Seventh Avenue, 10.1055/s-0039-1694978. accepted after revision New York, NY 10001, USA. ISSN 2472-8721. June 6, 2019 Tel: +1(212) 584-4662. published online August 27, 2019 Treatment of Pediatric Portosystemic Varices Dabrowiecki et al. 215 Fig. 1 Drawing illustrates the common portosystemic venous collateral Fig. 2 Drawing illustrates the various routes that can be used to pathways: the left gastric (coronary) and azygos veins at the lower access the portal venous system in complicated portal hypertension, a b esophagus ( ); the splenic and left renal-adrenal veins at the spleen ( ); the including transjugular (a), transhepatic (b), trans-splenic (c), tran- c superior rectal and middle-inferior rectal veins at the anal canal ( ); the veins sumbilical (d), transvariceal (stomal) (e), and transfemoral (systemic- of the ligamentum teres and the superior-inferior epigastric veins at the portal) (f) routes. (Referenced from Glen Oomen, MSc, McMaster d umbilicus ( ); and the colonic-mesenteric veins, body wall veins, and IVC in University, Dundas, Ontario, Canada.94) the retroperitoneum (e). (Referenced from Glen Oomen, MSc, McMaster University, Dundas, Ontario, Canada.) The pediatric literature is limited in its description of the However, more recent literature demonstrates pediatric risk factors for developing EcV, as invasive hemodynamic mortality from variceal bleeding is uncommon,10 although measures are rarely performed and thus inferences are made long-term morbidity in these patients is not well described.6 from the adult literature including using similar hepatic vein Often times, gastrointestinal bleeding from a ruptured varix pressure gradient thresholds as adults.6 In adults, there is a is the most common presenting symptom of portal hyper- This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. direct correlation between the degree of hepatic cirrhosis tension. Life-threatening bleeding and complications occur measured by Child Pugh or Model for End-Stage Liver Disease mainly in patients with portal hypertension secondary to (MELD) scores and degree of hyperdynamic circulation and hepatic cirrhosis due to the downstream complications of portal pressure gradient.7 Up to 60% of patients with decom- hepatic decompensation. pensated cirrhosis (as evidenced by coagulopathy, ascites, and hepatic encephalopathy) and up to 30% of patients with Pediatric Etiologies compensated cirrhosis have varices. Approximately 10% to 20% of these patients’ varices increase in size within 1 to 2 There are fundamental differences in the pathophysiology years. Importantly, size of esophageal varices is directly resulting in portal hypertension and subsequent EcV in correlated with risk of variceal bleeding8 and variceal size pediatric patients when compared to adults. There are is directly related to the degree of hepatic vein wedge many different causes of pediatric portal hypertension, pressure gradient elevation. Significant acute variceal bleed- which can further be subdivided into extrahepatic (prehe- ing is associated with up to 50% mortality9 in adults, and, patic and infrahepatic), intrahepatic, and post hepatic therefore, close attention to patients with EcV is necessary. (►Table 1). Digestive Disease Interventions Vol. 3 No. 3/2019 216 Treatment of Pediatric Portosystemic Varices Dabrowiecki et al. – Table 1 Causes of pediatric portal hypertension91 93 clinical prediction rules have been created based on spleen size, hypoalbuminemia, and platelet count to predict risk of Intrahepatic Biliary Biliary atresia forming esophageal varices.11,18 However, no such prediction disease Progressive familial intrahepatic rules have been delineated for the less common types of EcV cholestasis Primary sclerosing cholangitis discussed in this review likely due to the lack of published Cystic fibrosis treatment and outcomes data . Intestinal failure-associated liver disease Hepatic Autoimmune hepatitis Baveno Consensus Recommendations: disease Chronic viral hepatitis B/C Prophylaxis versus Acute Variceal Bleeding Alpha1-Antitrypsin deficiency Nonalcoholic fatty liver disease Beginning in 1990, in response to the scarce literature on Wilson’sdisease approaches to management of pediatric variceal bleeding, an Glycogen storage disease expert panel has convened every 5 years in Baveno, Italy, to Fibrotic Congenital hepatic fibrosis explore the data in pediatric variceal bleeding. The most disease Caroli disease recent consortium of the “Controversies in the Management Prehepatic Portal vein Portal vein thrombosis of Varices in Children—An International Forum” met in 2015, fi occlusion Tumor in ltration or and from this meeting, experts recommend no specific compression Congenital atresia/agenesis primary prophylaxis with either medication (nonselective beta-blockers), endoscopic variceal ligation, or sclerotherapy Splenic vein thrombosis based on the lack of evidence. Meso-Rex Bypass (a vascular graft between the superior mesenteric vein and left portal Increased Arteriovenous fistula fi portal flow vein) may be bene cial for primary and secondary prophy- laxis in certain cases of extrahepatic portal vein occlusion.19 Nodular Drug therapy regenerative (6-thioguanine and Endoscopic ligation and sclerotherapy can be performed to azathioprine) achieve hemostasis in gastroesophageal varices. In addition, Turner syndrome for acute variceal bleeding, octreotide has been shown to be Portal Schistosomiasis safe and effective in the majority of cases.20 stenosis Idiopathic cause Within the newest 2015 Baveno recommendations, endo- HIV infection vascular therapy, specifically TIPS, was not directly addressed.