Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
권호 표지
DOI QR코드

DOI QR Code

Portal Hypertension in Children: A Tertiary Center Experience in Turkey

  • Emine Nur Sunar Yayla (Department of Pediatrics, Faculty of Medicine, Gazi University) ;
  • Sinan Sari (Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University) ;
  • Neslihan Gurcan Kaya (Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University) ;
  • Odul Egritas Gurkan (Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University) ;
  • Hakan Sozen (Division of Transplantation Surgery, Faculty of Medicine, Gazi University) ;
  • Ibrahim Onur Ozen (Department of Pediatric Surgery, Faculty of Medicine, Gazi University) ;
  • Aydin Dalgic (Division of General Surgery, Faculty of Medicine, Gazi University) ;
  • Buket Dalgic (Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University)
  • Received : 2023.05.19
  • Accepted : 2023.09.16
  • Published : 2023.11.15
Download PDF
⟨ Previous Next ⟩

Abstract

Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. Results: The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Conclusion: Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.

Keywords

References

  1. Groszmann RJ, Garcia-Tsao G, Bosch J, Grace ND, Burroughs AK, Planas R, et al.Portal Hypertension Collaborative Group. Beta-blockers to prevent gastroesophageal varices in patients with cirrhosis. N Engl J Med 2005;353:2254-61. https://doi.org/10.1056/NEJMoa044456
  2. Chapin CA, Bass LM. Cirrhosis and portal hypertension in the pediatric population. Clin Liver Dis 2018;22:735-52. https://doi.org/10.1016/j.cld.2018.06.007
  3. Poddar U, Thapa BR, Rao KL, Singh K. Etiological spectrum of esophageal varices due to portal hypertension in Indian children: is it different from the West? J Gastroenterol Hepatol 2008;23:1354-7. https://doi.org/10.1111/j.1440-1746.2007.05102.x
  4. D'Amico G, Luca A. Natural history. Clinical-haemodynamic correlations. Prediction of the risk of bleeding. Baillieres Clin Gastroenterol 1997;11:243-56. https://doi.org/10.1016/S0950-3528(97)90038-5
  5. D'Amico G, Pagliaro L, Bosch J. The treatment of portal hypertension: a meta-analytic review. Hepatology 1995;22:332-54. https://doi.org/10.1002/hep.1840220145
  6. Imanieh MH, Dehghani SM, Khoshkhui M, Malekpour A. Etiology of portal hypertension in children: a single center's experiences. Middle East J Dig Dis 2012;4:206-10.
  7. Shashidhar H, Langhans N, Grand RJ. Propranolol in prevention of portal hypertensive hemorrhage in children: a pilot study. J Pediatr Gastroenterol Nutr 1999;29:12-7. https://doi.org/10.1097/00005176-199907000-00007
  8. Howard ER, Stringer MD, Mowat AP. Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. Br J Surg 1988;75:404-8. https://doi.org/10.1002/bjs.1800750504
  9. Abd El-Hamid N, Taylor RM, Marinello D, Mufti G, Patel R, Mieli-Vergani G, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience. J Pediatr Gastroenterol Nutr 2008;47:630-4. https://doi.org/10.1097/MPG.0b013e31817b6eea
  10. Bari K, Garcia-Tsao G. Treatment of portal hypertension. World J Gastroenterol 2012;18:1166-75. https://doi.org/10.3748/wjg.v18.i11.1166
  11. Istatistikler ile Aile [Internet]. TUIK; 2023 [cited 2023 Oct 10]. Available from: https://data.tuik.gov.tr/Bulten/Index?p=Istatistiklerle-Aile-2022-49683
  12. Sari S. Cocuklarda Non-sirotik Portal Hipertansiyona Yaklasim. In: Dalkilic B, ed. Cocuklarda Karaciger Hastaliklarinin Tani ve Tedavisi. 1st ed. Turkiye Klinikleri, 2021:72-86.
  13. Grimaldi C, De Goyet JdV, Nobili V. Portal hypertension in children. Clin Res Hepatol Gastroenterol 2012;36:260-1. https://doi.org/10.1016/j.clinre.2012.03.016
  14. Peter L, Dadhich SK, Yachha SK. Clinical and laboratory differentiation of cirrhosis and extrahepatic portal venous obstruction in children. J Gastroenterol Hepatol 2003;18:185-9. https://doi.org/10.1046/j.1440-1746.2003.02943.x
  15. Lv Y, Yee Lau W, Wu H, Han X, Gong X, Liu N, et al. Causes of peripheral cytopenia in hepatitic cirrhosis and portal hypertensive splenomegaly. Exp Biol Med (Maywood) 2017;242:744-9. https://doi.org/10.1177/1535370217693113
  16. Etzion O, Koh C, Heller T. Noncirrhotic portal hypertension: An overview. Clin Liver Dis (Hoboken) 2015;6:72-4. https://doi.org/10.1002/cld.497
  17. Sarin SK, Khanna R. Non-cirrhotic portal hypertension. Clin Liver Dis 2014;18:451-76. https://doi.org/10.1016/j.cld.2014.01.009
  18. Amarapurkar PD, Amarapurkar DN. Management of coagulopathy in patients with decompensated liver cirrhosis. Int J Hepatol 2011;2011:695470.
  19. Blake JC, Sprengers D, Grech P, McCormick PA, McIntyre N, Burroughs AK. Bleeding time in patients with hepatic cirrhosis. BMJ 1990;301:12-5. https://doi.org/10.1136/bmj.301.6742.12
  20. Lisman T, Porte RJ. Rebalanced hemostasis in patients with liver disease: evidence and clinical consequences. Blood 2010;116:878-85. https://doi.org/10.1182/blood-2010-02-261891
  21. Jaffe DL, Chung RT, Friedman LS. Management of portal hypertension and its complications. Med Clin North Am 1996;80:1021-34. https://doi.org/10.1016/S0025-7125(05)70478-8
  22. Bozic MA, Puri K, Molleston JP. Screening and prophylaxis for varices in children with liver disease. Curr Gastroenterol Rep 2015;17:27.
  23. Attard TM, Miller M, Pant C, Kumar A, Thomson M. Mortality associated with gastrointestinal bleeding in children: A retrospective cohort study. World J Gastroenterol 2017;23:1608-17. https://doi.org/10.3748/wjg.v23.i9.1608
  24. Gugig R, Rosenthal P. Management of portal hypertension in children. World J Gastroenterol 2012;18:1176-84. https://doi.org/10.3748/wjg.v18.i11.1176
  25. Weiss B, Shteyer E, Vivante A, Berkowitz D, Reif S, Weizman Z, et al. Etiology and long-term outcome of extrahepatic portal vein obstruction in children. World J Gastroenterol 2010;16:4968-72. https://doi.org/10.3748/wjg.v16.i39.4968
  26. Adami MR, Kieling CO, Ferreira CT, Santos J, Vieira SMG. Hipertensao portal em criancas: metodos nao invasivos preditores de varizes esofagicas. Boletim Cientifico de Pediatria 2014;3.
  27. Ferri PM, Ferreira AR, Fagundes EDT, Liu SM, Roquete MLV, Penna FJ. Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center. Arq Gastroenterol 2012;49:69-76. https://doi.org/10.1590/S0004-28032012000100012
  28. Stewart CA, Sanyal AJ. Grading portal gastropathy: validation of a gastropathy scoring system. Am J Gastroenterol 2003;98:1758-65. https://doi.org/10.1111/j.1572-0241.2003.07595.x
  29. Burak KW, Lee SS, Beck PL. Portal hypertensive gastropathy and gastric antral vascular ectasia (GAVE) syndrome. Gut 2001;49:866-72. https://doi.org/10.1136/gut.49.6.866
  30. Amarapurkar DN, Dhawan PS, Chopra K, Shankaran K, Kalro RH. Stomach in portal hypertension. J Assoc Physicians India 1993;41:638-40.
  31. El-Rifai N, Mention K, Guimber D, Michaud L, Boman F, Turck D, et al. Gastropathy and gastritis in children with portal hypertension. J Pediatr Gastroenterol Nutr 2007;45:137-40. https://doi.org/10.1097/MPG.0b013e318049cbe2
  32. Goel A, Ramakrishna B, Muliyil J, Madhu K, Sajith KG, Zachariah U, et al. Use of serum vitamin B12 level as a marker to differentiate idiopathic noncirrhotic intrahepatic portal hypertension from cryptogenic cirrhosis. Dig Dis Sci 2013;58:179-87.
  33. Shneider BL, Bosch J, de Franchis R, Emre SH, Groszmann RJ, Ling SC, et al.expert panel of the Children's Hospital of Pittsburgh of UPMC. Portal hypertension in children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension. Pediatr Transplant 2012;16:426-37. https://doi.org/10.1111/j.1399-3046.2012.01652.x
  34. Shneider BL, de Goyet JdV, Leung DH, Srivastava A, Ling SC, Duche M, et al. Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: summary of the Baveno VI pediatric satellite symposium. Hepatology 2016;63:1368-80. https://doi.org/10.1002/hep.28153
  35. Cifuentes LI, Gattini D, Torres-Robles R, Gana JC. Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev 2021;1:CD011561.
  36. D'Antiga L, Betalli P, De Angelis P, Davenport M, Di Giorgio A, McKiernan PJ, et al. Interobserver agreement on endoscopic classification of oesophageal varices in children. J Pediatr Gastroenterol Nutr 2015;61:176-81. https://doi.org/10.1097/MPG.0000000000000822
  37. Goncalves MEP, Cardoso SR, Maksoud JG. Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial. J Pediatr Surg 2000;35:401-5. https://doi.org/10.1016/S0022-3468(00)90203-3
  38. Ozsoylu S, Kocak N, Demir H, Yuce A, Gurakan F, Ozen H. Propranolol for primary and secondary prophylaxis of variceal bleeding in children with cirrhosis. Turk J Pediatr 2000;42:31-3.
  39. Turnes J, Garcia-Pagan JC, Abraldes JG, Hernandez-Guerra M, Dell'Era A, Bosch J. Pharmacological reduction of portal pressure and long-term risk of first variceal bleeding in patients with cirrhosis. Am J Gastroenterol 2006;101:506-12. https://doi.org/10.1111/j.1572-0241.2006.00453.x
  40. Botha JF, Campos BD, Grant WJ, Horslen SP, Sudan DL, Shaw BW Jr, et al. Portosystemic shunts in children: a 15-year experience. J Am Coll Surg 2004;199:179-85. https://doi.org/10.1016/j.jamcollsurg.2004.03.024