Log in

Schistosomiasis: Hepatosplenic Disease and Portal Hypertensive Complications

  • Published:
Current Hepatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Schistosomiasis is an important and often neglected disease in tropical climates and is associated with significant comorbidity due to the complication of periportal fibrosis, termed hepatosplenic schistosomiasis. Clinically significant portal hypertension can develop as a complication of periportal fibrosis and is associated with life-threatening gastrointestinal bleeding due to the formation of esophagogastric varices. Unfortunately, patients with established infections can be asymptomatic and may not present for evaluation before significant clinical symptoms are identified.

Recent Findings

Effective screening and prevention programs are paramount to prevent infection and the associated long-term Sequelae. The diagnosis of a chronic infection typically relies on the detection of parasite eggs in the patient’s feces or urine. A combination of clinical examination and imaging is required for the diagnosis of hepatosplenic schistosomiasis. Periportal fibrosis, hepatosplenomegaly or clinically significant portal hypertension are most commonly identified through ultrasound-based imaging. The management of hepatosplenic disease relies on the application of parasite-directed pharmaceuticals, with endoscopic, surgical, or interventional radiology techniques directed toward portal hypertensive complications. Endoscopic band ligation is currently one of the most popular methods used to manage esophageal varices in patients with hepatosplenic complications.

Summary

Given the lack of head-to-head treatment trials, a combination of esophagogastric devascularization with subtotal splenectomy, postoperative band ligation or sclerotherapy, and beta-blockers may be the best approach for patients with refractory symptoms. Further studies are needed to identify the potential role of the transjugular intrahepatic portosystemic shunt procedure in hepatosplenic schistosomiasis. Liver transplantation is rarely performed in patients with hepatosplenic schistosomiasis. Given the significant comorbidities of long-term infections, the most effective strategy is a combination of early screening and eradication programs, along with preventative programs targeting the socioeconomic determinants of schistosomiasis infections.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price includes VAT (Germany)

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Olveda DU, Li Y, Olveda RM, Lam AK, Chau TN, Harn DA, Williams GM, Gray DJ, Ross AG. Bilharzia: Pathology, Diagnosis, Management and Control. Trop Med Surg. 2013;1(4):135. https://doi.org/10.4172/2329-9088.1000135.

    Article  PubMed  PubMed Central  Google Scholar 

  2. • Schistosomiasis — Level 3 cause . Institute for Health Metrics and Evaluation. 2020 [cited 2023 May 18]. Available from: https://www.healthdata.org/results/gbd_summaries/2019/schistosomiasis-level-3-cause. (Important updated health metrics which provide an overview of the global burden of schistosomiasis.)

  3. McManus DP, Dunne DW, Sacko M, Utzinger J, Vennervald BJ, Zhou XN. Schistosomiasis. Nat Rev Dis Primers. 2018;4(1):13.

    Article  PubMed  Google Scholar 

  4. Hotez PJ, Bethony JM, Diemert DJ, Pearson M, Loukas A. Develo** vaccines to combat hookworm infection and intestinal schistosomiasis. Nat Rev Microbiol. 2010;8(11):814–26.

    Article  CAS  PubMed  Google Scholar 

  5. Masi B, Perles-Barbacaru TA, Bernard M, Viola A. Clinical and preclinical imaging of hepatosplenic schistosomiasis. Trends Parasitol. 2020;36(2):206–26.

    Article  PubMed  Google Scholar 

  6. Elbaz T, Esmat G. Hepatic and intestinal schistosomiasis: review. J Advert Res. 2013;4(5):445–52.

    Article  Google Scholar 

  7. Tamarozzi F, Fittipaldo VA, Orth HM, Richter J, Buonfrate D, Riccardi N, et al. Diagnosis and clinical management of hepatosplenic schistosomiasis: a sco** review of the literature. PLoS Negl Trop Dis. 2021;15(3):e0009191.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Gryseels B, Polman K, Clerinx J, Kestens L. Human schistosomiasis. Lancet. 2006;368(9541):1106–18.

    Article  PubMed  Google Scholar 

  9. Colley DG, Bustinduy AL, Secor WE, King CH. Human schistosomiasis. Lancet. 2014;383(9936):2253–64.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Gordon CA, Kurscheid J, Williams GM, Clements ACA, Li Y, Zhou XN, et al. Asian schistosomiasis: current status and prospects for control leading to elimination. Trop Med Infect Dis. 2019;4(1):40. https://doi.org/10.3390/tropicalmed4010040.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Zhu HR, Liu L, Zhou XN, Yang GJ. Ecological model to predict potential habitats of Oncomelania hupensis, the intermediate host of Schistosoma japonicum in the Mountainous Regions, China. PLoS Negl Trop Dis. 2015;9(8):e0004028.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Cisse M, Sangare I, Djibougou AD, Tahita MC, Gnissi S, Bassinga JKW, et al. Prevalence and risk factors of Schistosoma mansoni infection among preschool-aged children from Panamasso village, Burkina Faso. Parasit Vectors. 2021;14(1):185.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Morgan JA, Dejong RJ, Snyder SD, Mkoji GM, Loker ES. Schistosoma mansoni and Biomphalaria: past history and future trends. Parasitology. 2001;123(Suppl):S211–28.

    Article  PubMed  Google Scholar 

  14. Matthys B, Tschannen AB, Tian-Bi NT, Comoé H, Diabaté S, Traoré M, et al. Risk factors for Schistosoma mansoni and hookworm in urban farming communities in western Côte d’Ivoire. Trop Med Int Health. 2007;12(6):709–23.

    Article  PubMed  Google Scholar 

  15. Lai YS, Biedermann P, Ekpo UF, Garba A, Mathieu E, Midzi N, et al. Spatial distribution of schistosomiasis and treatment needs in sub-Saharan Africa: a systematic review and geostatistical analysis. Lancet Infect Dis. 2015;15(8):927–40.

    Article  PubMed  Google Scholar 

  16. Nelwan ML. Schistosomiasis: life cycle, diagnosis, and control. Curr Ther Res Clin Exp. 2019;91:5–9.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Ross AG, Vickers D, Olds GR, Shah SM, McManus DP. Katayama syndrome. Lancet Infect Dis. 2007;7(3):218–24.

    Article  PubMed  Google Scholar 

  18. Wang T, Hua H, Zhang J, Li W, Yang K. Noninvasive diagnosis of liver fibrosis in Schistosomiasis japonica: opportunities and challenges. Portal Hypertension & Cirrhosis. 2023;2(1):43–5.

    Article  Google Scholar 

  19. Veiga ZST, Fernandes FF, Guimarães L, Piedade J, Pereira GHS. Natural history of hepatosplenic schistosomiasis (HSS) non-cirrhotic portal hypertension (NCPH): influence of gastrointestinal bleeding and decompensation in prognosis. Trop Med Infect Dis. 2023;8(3):145. https://doi.org/10.3390/tropicalmed8030145.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Clerinx J, Van Gompel A. Schistosomiasis in travellers and migrants. Travel Med Infect Dis. 2011;9(1):6–24.

    Article  PubMed  Google Scholar 

  21. Whitty CJ, Mabey DC, Armstrong M, Wright SG, Chiodini PL. Presentation and outcome of 1107 cases of schistosomiasis from Africa diagnosed in a non-endemic country. Trans R Soc Trop Med Hyg. 2000;94(5):531–4.

    Article  CAS  PubMed  Google Scholar 

  22. Bierman WFW, Wetsteyn JCFM, van Gool T. Presentation and diagnosis of imported schistosomiasis: relevance of eosinophilia, microscopy for ova, and serology. J Travel Med. 2005;12(1):9–13.

    Article  PubMed  Google Scholar 

  23. WHO guideline on control and elimination of human schistosomiasis (pp 144). World Health Organization, Geneva; 2022.

  24. WHO Expert Committee on the Control of Schistosomiasis (‎2001 : Geneva, Switzerland)‎ & World Health Organization. (‎2002)‎. Prevention and control of schistosomiasis and soil-transmitted helminthiasis : report of a WHO expert committee. World Health Organization, vol 912. WHO technical report series, p 57

  25. Jones ME, Mitchell RG, Leen CL. Long seronegative window in schistosoma infection. Lancet. 1992;340(8834–8835):1549–50.

    Article  CAS  PubMed  Google Scholar 

  26. Tsang VC, Wilkins PP. Immunodiagnosis of schistosomiasis Screen with FAST-ELISA and confirm with immunoblot. Clin Lab Med. 1991;11(4):1029–39.

    Article  CAS  PubMed  Google Scholar 

  27. Sulahian A, Garin YJF, Izri A, Verret C, Delaunay P, van Gool T, et al. Development and evaluation of a Western blot kit for diagnosis of schistosomiasis. Clin Diagn Lab Immunol. 2005;12(4):548–51.

    CAS  PubMed  PubMed Central  Google Scholar 

  28. Coulibaly JT, N’Gbesso YK, Knopp S, N’Guessan NA, Silué KD, van Dam GJ, et al. Accuracy of urine circulating cathodic antigen test for the diagnosis of Schistosoma mansoni in preschool-aged children before and after treatment. PLoS Negl Trop Dis. 2013;7(3):e2109.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Khanna R, Sarin SK. Idiopathic portal hypertension and extrahepatic portal venous obstruction. Hepatol Int. 2018;12(Suppl 1):148–67.

    Article  PubMed  Google Scholar 

  30. Tukahebwa EM, Magnussen P, Madsen H, Kabatereine NB, Nuwaha F, Wilson S, et al. A very high infection intensity of Schistosoma mansoni in a Ugandan Lake Victoria Fishing Community is required for association with highly prevalent organ related morbidity. PLoS Negl Trop Dis. 2013;7(7):e2268.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Homeida M, Abdel-Gadir AF, Cheever AW, Bennett JL, Arbab BM, Ibrahium SZ, et al. Diagnosis of pathologically confirmed Symmers’ periportal fibrosis by ultrasonography: a prospective blinded study. Am J Trop Med Hyg. 1988;38(1):86–91.

    Article  CAS  PubMed  Google Scholar 

  32. Nardelli MJ, Veiga ZDST, Faria LC, Pereira GHS, da Silva CF, Barbosa FA, et al. Noninvasive predictors of esophageal varices in patients with hepatosplenic Schistosomiasis mansoni. Acta Trop. 2022;226:106.

    Article  Google Scholar 

  33. Nogueira RA, Lira MGS, Licá ICL, Frazão GCCG, Dos Santos VAF, Filho ACCM, et al. Praziquantel: an update on the mechanism of its action against schistosomiasis and new therapeutic perspectives. Mol Biochem Parasitol. 2022;252:111531.

    Article  CAS  PubMed  Google Scholar 

  34. Kong D, Zhou C, Guo H, Wang W, Qiu J, Liu X, et al. Praziquantel targets M1 macrophages and ameliorates splenomegaly in chronic schistosomiasis. Antimicrob Agents Chemother. 2018;62(1):10–1128. https://doi.org/10.1128/AAC.00005-17.

    Article  Google Scholar 

  35. Ruiz-Guevara R, de Noya BA, Valero SK, Lecuna P, Garassini M, Noya O. Clinical and ultrasound findings before and after praziquantel treatment among Venezuelan schistosomiasis patients. Rev Soc Bras Med Trop. 2007;40:505–11. https://doi.org/10.1590/s0037-86822007000500003.

    Article  PubMed  Google Scholar 

  36. Richter J. Evolution of schistosomiasis-induced pathology after therapy and interruption of exposure to schistosomes: a review of ultrasonographic studies. Acta Trop. 2000;77(1):111–31.

    Article  CAS  PubMed  Google Scholar 

  37. Homeida MA, el Tom I, Nash T, Bennett JL. Association of the therapeutic activity of praziquantel with the reversal of Symmers’ fibrosis induced by Schistosoma mansoni. Am J Trop Med Hyg. 1991;45(3):360–5.

    Article  CAS  PubMed  Google Scholar 

  38. Boisier P, Ramarokoto CE, Ravaoalimalala VE, Rabarijaona L, Serieye J, Roux J, et al. Reversibility of Schistosoma mansoni-associated morbidity after yearly mass praziquantel therapy: ultrasonographic assessment. Trans R Soc Trop Med Hyg. 1998;92(4):451–3.

    Article  CAS  PubMed  Google Scholar 

  39. Salvador-Recatalà V, Greenberg RM. Calcium channels of schistosomes: unresolved questions and unexpected answers. Wiley Interdiscip Rev: Membr Trans Signal. 2012;1:85–93. https://doi.org/10.1002/wmts.19.

    Article  CAS  Google Scholar 

  40. Kohn AB, Roberts-Misterly JM, Anderson PAV, Khan N, Greenberg RM. Specific sites in the beta interaction domain of a schistosome Ca2+ channel beta subunit are key to its role in sensitivity to the anti-schistosomal drug praziquantel. Parasitology. 2003;127(Pt 4):349–56.

    Article  CAS  PubMed  Google Scholar 

  41. Pica-Mattoccia L, Ruppel A, **a CM, Cioli D. Praziquantel and the benzodiazepine Ro 11–3128 do not compete for the same binding sites in schistosomes. Parasitology. 2008;135(1):47–54.

    Article  CAS  PubMed  Google Scholar 

  42. Pica-Mattoccia L, Orsini T, Basso A, Festucci A, Liberti P, Guidi A, et al. Schistosoma mansoni: lack of correlation between praziquantel-induced intra-worm calcium influx and parasite death. Exp Parasitol. 2008;119(3):332–5.

    Article  CAS  PubMed  Google Scholar 

  43. Gnanasekar M, Salunkhe AM, Krishna Mallia A, He YX, Kalyanasundaram R. Praziquantel affects the regulatory myosin light chain of Schistosoma mansoni. Antimicrob Agents Chemother. 2009;53:1054–60. https://doi.org/10.1128/aac.01222-08.

    Article  CAS  PubMed  Google Scholar 

  44. Chienwichai P, Ampawong S, Adisakwattana P, Thiangtrongjit T, Limpanont Y, Chusongsang P, et al. Effect of praziquantel on Schistosoma mekongi proteome and phosphoproteome. Pathogens. 2020;9(6):417. https://doi.org/10.3390/pathogens9060417.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Pax R, Bennett JL, Fetterer R. A benzodiazepine derivative and praziquantel: effects on musculature of Schistosoma mansoni and Schistosoma japonicum. Naunyn Schmiedebergs Arch Pharmacol. 1978;304(3):309–15.

    Article  CAS  PubMed  Google Scholar 

  46. Doenhoff MJ, Cioli D, Utzinger J. Praziquantel: mechanisms of action, resistance and new derivatives for schistosomiasis. Curr Opin Infect Dis. 2008;21(6):659–67.

    Article  CAS  PubMed  Google Scholar 

  47. Eyoh E, McCallum P, Killick J, Amanfo S, Mutapi F, Astier AL. The anthelmintic drug praziquantel promotes human Tr1 differentiation. Immunol Cell Biol. 2019;97(5):512–8.

    Article  CAS  PubMed  Google Scholar 

  48. Vale N, Gouveia MJ, Rinaldi G, Brindley PJ, Gärtner F, Correia da Costa JM. Praziquantel for schistosomiasis: single-drug metabolism revisited, mode of action, and resistance. Antimicrob Agents Chemother. 2017;61(5):10–1128. https://doi.org/10.1128/AAC.02582-16.

    Article  Google Scholar 

  49. Gönnert R, Andrews P. Praziquantel, a new board-spectrum antischistosomal agent. Z Parasitenkd. 1977;52(2):129–50.

    Article  PubMed  Google Scholar 

  50. **ao SH, Yue WJ, Yang YQ, You JQ. Susceptibility of Schistosoma japonicum to different developmental stages to praziquantel. Chin Med J. 1987;100(9):759–68.

    CAS  PubMed  Google Scholar 

  51. Buchter V, Schneeberger PHH, Keiser J. Validation of a human-serum-based in vitro growth method for drug screening on juvenile development stages of Schistosoma mansoni. PLoS Negl Trop Dis. 2021;15(3):e0009313.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  52. Castro N, Medina R, Sotelo J, Jung H. Bioavailability of praziquantel increases with concomitant administration of food. Antimicrob Agents Chemother. 2000;44(10):2903–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Salas-Coronas J, Pérez AP, Roure S, Peinador CS, Larrégola LS, Izquierdo JA, et al. Documento de consenso para el manejo de la esquistosomiasis en atención primaria. Atención Primaria. 2022;54:102408. https://doi.org/10.1016/j.aprim.2022.102408.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Erko B, Degarege A, Tadesse K, Mathiwos A, Legesse M. Efficacy and side effects of praziquantel in the treatment of Schistosomiasis mansoni in schoolchildren in Shesha Kekele Elementary School, Wondo Genet, Southern Ethiopia. Asian Pac J Trop Biomed. 2012;2:235–9. https://doi.org/10.1016/s2221-1691(12)60049-5.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Watt G, Baldovino PC, Castro JT, Fernando MT, Ranoa CP. Bloody diarrhoea after praziquantel therapy. Trans Royal Soc Trop Med Hyg. 1986;80:345–6. https://doi.org/10.1016/0035-9203(86)90053-2.

    Article  CAS  Google Scholar 

  56. Ferrari MLA, Coelho PMZ, Antunes CMF, Tavares CAP, da Cunha AS. Efficacy of oxamniquine and praziquantel in the treatment of Schistosoma mansoni infection: a controlled trial. Bull World Health Organ. 2003;81(3):190–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  57. Cucchetto G, Buonfrate D, Marchese V, Rodari P, Ferrari A, Zanotti P, et al. High-dose or multi-day praziquantel for imported schistosomiasis? A systematic review. J Travel Med. 2019;26(7):taz050. https://doi.org/10.1093/jtm/taz050.

    Article  PubMed  Google Scholar 

  58. da Cunha AS, da Cunha AS, Romeu Cançado J, de Rezende GL. Therapeutical evaluation of different dose regimens of praziquantel in Schistosomiasis mansoni, based on the quantitative oogram technique. Rev Inst Med Trop São Paulo. 1987;29:295–304. https://doi.org/10.1590/s0036-46651987000500006.

    Article  PubMed  Google Scholar 

  59. Guimarães Cavalcanti M, de Araujo-Neto JM, Peralta JM. Schistosomiasis: clinical management of liver disease. Clin Liver Dis. 2015;6(3):59–62.

    Article  Google Scholar 

  60. de Abreu ES, Nardelli MJ, Lima AMC, Cardoso JB, Osório FMF, Ferrari TCDA, et al. Carvedilol as secondary prophylaxis for variceal bleeding in hepatosplenic schistosomiasis. Trans R Soc Trop Med Hyg. 2022;116(7):663–7.

    Article  PubMed  Google Scholar 

  61. Cançado GGL, Nardelli MJ, Barbosa FA, Silva CF, Osório FMF, Ferrari TCA, et al. Portal vein thrombosis in patients with hepatosplenic schistosomiasis who underwent oesophagogastric devascularization combined with splenectomy. Trans R Soc Trop Med Hyg. 2021;115(9):1004–9.

    Article  PubMed  Google Scholar 

  62. Gunda DW, Kilonzo SB, Manyiri PM, Peck RN, Mazigo HD. Morbidity and mortality due to Schistosoma mansoni related periportal fibrosis: could early diagnosis of varices improve the outcome following available treatment modalities in Sub Saharan Africa? A sco** review. Trop Med Infect Dis. 2020;5(1):20. https://doi.org/10.3390/tropicalmed5010020.

    Article  PubMed  PubMed Central  Google Scholar 

  63. • Costa Lacet CM, Neto JB, Ribeiro LT, Oliveira FS, Wyszomirska RF, Strauss E. Schistosomal portal hypertension: randomized trial comparing endoscopic therapy alone or preceded by esophagogastric devascularization and splenectomy. Ann Hepatol. 2016;15(5):738–44. (Important randomized controlled trial demonstrating the superior efficacy of combined surgical and endoscopic treatment for the prevention of recurrent esophageal variceal bleeding.)

    PubMed  Google Scholar 

  64. Strauss E. Hepatosplenic schistosomiasis: a model for the study of portal hypertension. Ann Hepatol. 2002;1:6–11. https://doi.org/10.1016/s1665-2681(19)32186-6.

    Article  PubMed  Google Scholar 

  65. Camacho-Lobato L, Borges DR. Early liver dysfunction in schistosomiasis. J Hepatol. 1998;29:233–40. https://doi.org/10.1016/s0168-8278(98)80008-1.

    Article  CAS  PubMed  Google Scholar 

  66. Barbosa FA, Nardelli MJ, Cançado GGL, Silva CF, Osório FMF, Melo RFQ, et al. Sarcopenia, body composition and factors associated with variceal gastrointestinal bleeding and splenectomy in hepatosplenic Schistosomiasis mansoni. Trans R Soc Trop Med Hyg. 2022;116(12):1145–53.

    Article  CAS  PubMed  Google Scholar 

  67. Raia S, Da Silva LC, Gayotto LCC, Forster SC, Fukushima J, Strauss E. Portal hypertension in schistosomiasis: a long-term follow-up of a randomized trial comparing three types of surgery. Hepatology. 1994;20:398–403. https://doi.org/10.1002/hep.1840200220.

    Article  CAS  PubMed  Google Scholar 

  68. • Ede CJ, Nikolova D, Brand M. Surgical portosystemic shunts versus devascularisation procedures for prevention of variceal rebleeding in people with hepatosplenic schistosomiasis. Cochrane Database Syst Rev. 2018;8(8):CD011717. ()

    PubMed  Google Scholar 

  69. Li Y, Ju S, Li X, Zhou YL, Qiang JW. Prediction of minimal hepatic encephalopathy by using an radiomics nomogram in chronic hepatic schistosomiasis patients. PLoS Negl Trop Dis. 2021;15(10):e0009834.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  70. Bittencourt PL. Portal fibrosis and schistosomal portal hypertension: what is the best strategy for primary and secondary prevention of hemorrhage from esophageal varices. Arq Gastroenterol. 2003;40(1):1–3.

    Article  PubMed  Google Scholar 

  71. da Silva LC, Strauss E, Gayotto LC, Mies S, Macedo AL, da Silva AT, et al. A randomized trial for the study of the elective surgical treatment of portal hypertension in mansonic schistosomiasis. Ann Surg. 1986;204(2):148–53.

    Article  PubMed  PubMed Central  Google Scholar 

  72. Kiire CF. Controlled trial of propranolol to prevent recurrent variceal bleeding in patients with non-cirrhotic portal fibrosis. BMJ. 1989;298(6684):1363–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Ferraz AA, de Albuquerque PC, Lopes EP, de Araújo JG Jr, Carvalho AH, Ferraz EM. The influence of periportal (pipestem) fibrosis on long term results of surgical treatment for schistosomotic portal hypertension. Arq Gastroenterol. 2003;40(1):4–10. https://doi.org/10.1590/s0004-28032003000100002.

    Article  PubMed  Google Scholar 

  74. Sarin SK, Groszmann RJ, Mosca PG, Rojkind M, Stadecker MJ, Bhatnagar R, et al. Propranolol ameliorates the development of portal-systemic shunting in a chronic murine schistosomiasis model of portal hypertension. J Clin Invest. 1991;87(3):1032–6.

  75. Da Silva LC. Portal hypertension in schistosomiasis: pathophysiology and treatment. Mem Inst Oswaldo Cruz. 1992;87(Suppl 4):183–116.

    Article  PubMed  Google Scholar 

  76. Mies S, Neto OB, Beer A Jr, Baía CE, Alfieri F Jr, Pereira LM, et al. Systemic and hepatic hemodynamics in hepatosplenic Manson’s schistosomiasis with and without propranolol. Dig Dis Sci. 1997;42(4):751–61.

    Article  CAS  PubMed  Google Scholar 

  77. Kong DR, Ma C, Wang M, Wang JG, Chen C, Zhang L, et al. Effects of propranolol or propranolol plus isosorbide-5-mononitrate on variceal pressure in schistosomiasis. World J Gastroenterol. 2013;19(26):4228–33.

    Article  PubMed  PubMed Central  Google Scholar 

  78. Farias AQ, Kassab F, da Rocha ECV, Dos Santos BV, Vezozzo DCP, Bittencourt PL, et al. Propranolol reduces variceal pressure and wall tension in schistosomiasis presinusoidal portal hypertension. J Gastroenterol Hepatol. 2009;24(12):1852–6.

    Article  CAS  PubMed  Google Scholar 

  79. Sinkala E, Vinikoor M, Zyambo K, Besa E, Nsokolo B, Kelly P. Propranolol reduces portal vein diameter in schistosomal liver disease with portal hypertension: a prospective cohort study. Am J Trop Med Hyg. 2020;102(4):832–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. el Tourabi H, el Amin AA, Shaheen M, Woda SA, Homeida M, Harron DW. Propranolol reduces mortality in patients with portal hypertension secondary to schistosomiasis. Ann Trop Med Parasitol. 1994;88(5):493–500.

    Article  PubMed  Google Scholar 

  81. •• de Franchis R, Bosch J, Garcia-Tsao G, Reiberger T, Ripoll C. Baveno VII Faculty. Baveno VII — renewing consensus in portal hypertension. J Hepatol. 2022;76(4):959–74. ()

    Article  PubMed  Google Scholar 

  82. Razafindrazoto CI, Razafindrabekoto LDE, Hasina Laingonirina DH, Raveloson R, Rasolonjatovo AS, Rakotozafindrabe ALR, et al. Carvedilol versus propranolol in the prevention of variceal rebleeding in hepatosplenic schistosomiasis: efficacy and safety. JGH Open. 2022;6(3):213–8.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Garcia-Tsao G, Abraldes JG, Berzigotti A, Bosch J. Portal hypertensive bleeding in cirrhosis: risk stratification, diagnosis, and management: 2016 practice guidance by the American Association for the study of liver diseases. Hepatology. 2017;65(1):310–35.

    Article  PubMed  Google Scholar 

  84. Siqueira ES, Rohr MR, Libera ED, Castro RR, Ferrari AP. Band ligation or sclerotherapy as endoscopic treatment for oesophageal varices in schistosomotic patients: results of a randomized study. HPB Surg. 1998;11(1):27–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Cordeiro F. Variceal sclerosis in schistosomotic patients: a 5-year follow-up study. Gastrointest Endosc. 1990;36(5):475–8.

    Article  CAS  PubMed  Google Scholar 

  86. Mudawi HMY, Ibrahim KB. Endoscopic variceal sclerotherapy in patients with Symmers periportal fibroses. Trop Doct. 2007;37(3):179–81.

    Article  CAS  PubMed  Google Scholar 

  87. Neto WDBDS, da Silva Neto WDB, Tredicci TM, Coelho FF, Makdissi FF, Herman P. Portal pressure decrease after esophagogastric devascularization and splenectomy in schistosomiasis: long-term varices behavior, rebleeding rate, and role of endoscopic treatment. Arq Gastroenterol. 2018;55:170–4. https://doi.org/10.1590/s0004-2803.201800000-30.

    Article  Google Scholar 

  88. de Cleva R, Herman P, D’albuquerque LAC, Pugliese V, Santarem OL, Saad WA. Pre- and postoperative systemic hemodynamic evaluation in patients subjected to esophagogastric devascularization plus splenectomy and distal splenorenal shunt: a comparative study in schistomomal portal hypertension. World J Gastroenterol. 2007;13(41):5471–5.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Makdissi FF, Herman P, Machado MAC, Pugliese V, D’Albuquerque LAC, Saad WA. Trombose de veia porta após desconexão ázigo-portal e esplenectomia em pacientes esquistossomóticos: Qual a real importância? Arq Gastroenterol. 2009;46:50–6. https://doi.org/10.1590/s0004-28032009000100014.

    Article  PubMed  Google Scholar 

  90. de Cleva R, Herman P, Saad WA, Pugliese V, Zilberstein B, Rodrigues JJG, et al. Postoperative portal vein thrombosis in patients with hepatosplenic mansonic schistosomiasis: relationship with intraoperative portal pressure and flow. A prospective study. Hepatogastroenterology. 2005;52(65):1529–33.

    PubMed  Google Scholar 

  91. Newland A, Provan D, Myint S. Preventing severe infection after splenectomy. BMJ. 2005;331(7514):417–8.

    Article  PubMed  PubMed Central  Google Scholar 

  92. • Liu J, Zhou B, Chen D, Zhou C, Shi Q, Zheng C, et al. Transjugular intrahepatic portosystemic shunt placement in patients with schistosomiasis-induced liver fibrosis. Cardiovasc Intervent Radiol. 2019;42(12):1760–70. (Important retrospective study which demonstrated the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) placements in patients with hepatosplenic schistosomiasis and portal hypertensive complications.)

    Article  PubMed  PubMed Central  Google Scholar 

  93. Santo MCCDE, Gryschek RCB, Farias AQ, Andraus W, Carvalho NB, Leite OHM, et al. Management and treatment of decompensated hepatic fibrosis and severe refractory Schistosoma mansoni ascites with transjugular intrahepatic portosystemic shunt. Rev Inst Med Trop Sao Paulo. 2022;64:26.

    Article  Google Scholar 

  94. Kraef C, Arand J, Galaski J, Jordan S, Kluwe J, Lohse AW, et al. Transjugular intrahepatic portosystemic shunt (TIPS) for primary and secondary prophylaxis of variceal bleeding in hepatic schistosomiasis. Travel Med Infect Dis. 2019;30:130–2.

    Article  PubMed  Google Scholar 

  95. Nordmann T, Schlabe S, Feldt T, Gobbi F, Krieg A, Bode JG, et al. TIPS and splenorenal shunt for complications of portal hypertension in chronic hepatosplenic schistosomiasis—a case series and review of the literature. PLoS Negl Trop Dis. 2021;15(12):e0010065.

    Article  PubMed  PubMed Central  Google Scholar 

  96. El Moghazy W, Kashkoush S, O’hali W, Abdallah K. Long-term outcome after liver transplantation for hepatic schistosomiasis: a single-center experience over 15 years. Liver Transpl. 2015;21(1):96–100.

    Article  PubMed  Google Scholar 

  97. Domingues ALC, de Medeiros TB, Lopes EPDA. Ultrasound versus biological markers in the evaluation of periportal fibrosis in human Schistosoma mansoni. Mem Inst Oswaldo Cruz. 2011;106(7):802–7.

    Article  PubMed  Google Scholar 

  98. Lambertucci JR, Silva LCDS, Antunes CM. Aspartate aminotransferase to platelet ratio index and blood platelet count are good markers for fibrosis evaluation in Schistosomiasis mansoni. Rev Soc Bras Med Trop. 2007;40(5):599.

    Article  PubMed  Google Scholar 

  99. Barreto AVMS, Domingues ALC, Diniz GTN, Cavalcanti AMS, Lopes EP, Montenegro SML, et al. The Coutinho index as a simple tool for screening patients with advanced forms of Schistosomiasis mansoni: a validation study. Trans R Soc Trop Med Hyg. 2022;116(1):19–25.

    Article  PubMed  Google Scholar 

  100. Xu XD, Xu CF, Dai JJ, Qian JQ, Pin X. Ratio of platelet count/spleen diameter predicted the presence of esophageal varices in patients with schistosomiasis liver cirrhosis. Eur J Gastroenterol Hepatol. 2016;28(5):588–91.

    Article  PubMed  Google Scholar 

Download references

Funding

Juan Pablo Arab receive support from the Chilean government through the Fondo Nacional de Desarrollo Científico y Tecnológico (FONDECYT 1200227). Figures were partially made with Biorender.

Author information

Authors and Affiliations

Authors

Contributions

Authors confirm contribution to the article as follows: DH: investigation, writing — original draft, writing — review and editing. GGLC: investigation, writing — original draft, writing — review and editing. TA: investigation, writing — original draft, writing — review and editing. GM: investigation, writing — original draft, writing — review and editing. ST: investigation, writing — original draft, writing — review and editing. JPA: conceptualization, writing — review, editing and supervision.

Corresponding author

Correspondence to Juan Pablo Arab.

Ethics declarations

Competing Interests

The authors declare no competing interests.

Conflict of Interest

The authors declare that there is no conflict of interest.

Human and Animal Rights and Informed Consent

All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/international guidelines).

AST, asparate aminotransferase; ULN, upper limit of normal; ALP, alkaline phosphatase.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

David Hudson and Guilherme Grossi Lopes Cançado share first authorship.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hudson, D., Cançado, G.G.L., Afzaal, T. et al. Schistosomiasis: Hepatosplenic Disease and Portal Hypertensive Complications. Curr Hepatology Rep 22, 170–181 (2023). https://doi.org/10.1007/s11901-023-00612-9

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11901-023-00612-9

Keywords

Navigation