Hepatic cyst infection as a source of sepsis in liver polycystosis

Czech version

Authors: G. Růžičková; L. Gabalec
Authors‘ workplace: Interní oddělení – Endoskopie, Orlickoústecká nemocnice, Nemocnice Pardubického kraje, a. s., Ústí nad Orlicí
Published in: Gastroent Hepatol 2018; 72(2): 122-128
Category: Hepatology: Case Report
doi: https://doi.org/10.14735/amgh2018122

Overview

Liver cysts are commonly identified by imaging methods. Polycystic liver disease is diagnosed when there are ten or more cysts and often develops as part of polycystic kidney disease or an isolated polycystic liver disorder. This disease is usually asymptomatic and does not require treatment. Impairment of renal function is one of the main symptoms in patients with cystic kidney disease. Complications of polycystosis, such as cyst bleeding, portal hypertension, icterus, and hepatic failure, are rare. A hepatic cyst infection that requires both antibiotic therapy and radiological or surgical intervention is a serious complication due to the high failure rate of separate antibiotic therapy. Quinolones are the recommended antibiotic therapy. Medications, such as somatostatin analogues, mTOR inhibitors, and ursodeoxycholic acid, have only been used in clinical trials. Arterial embolization of the hepatic artery or percutaneous aspiration and subsequent sclerotherapy of the cyst can aid interventional radiology. Several clinical classifications listed in this article can help clinicians decide whether to perform surgical therapy or liver transplantation. We present the case of a 60-year-old man with hereditary polycystosis of the kidneys and liver who was treated for sepsis due to hepatic cyst infection. Although parenteral antibiotic therapy reduced his fever and improved his laboratory findings, improvement of subjective problems occurred after the radiological intervention. Due to disease progression and the risk of the cyst infection recurring, the patient attended a consultation at a transplant center and liver transplantation was indicated.

Key words:
polycystic liver disease – sepsis – antibiotic therapy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

Submitted:
5. 1. 2018

Accepted:
8. 2. 2018

Sources

1. Cnossen WR, Drenth JP. Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management. Orphanet J Rare Dis 2014; 9: 69. doi: 10.1186/1750-1172-9-69.

2. Lin HP, Ho WC, Lee CC et al. Infected simple hepatic cysts – 3 cases report. J Intern Med Taiwan 2009; 20 (4): 373–377.

3. Abu-Wasel B, Walsh C, Keough V et al. Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol 2013; 19 (35): 5775–5786. doi: 10.3748/wjg.v19.i35.5775.

4. Krechler T, Kaláb M, Brodanová M et al. Diagnosis and treatment of hepatic cysts. Vnitr Lek 2000; 46 (7): 395–397.

5. D‘Agnolo HM, Kievit W, van Munster KN et al. Center is an important indicator for choice of invasive therapy in polycystic liver disease. Transpl Int 2017; 30 (1): 76–82. doi: 10.1111/tri. 12875.

6. Redston MS, Wanless IR. The hepatic von Meyenburg complex: prevalence and association with hepatic and renal cysts among 2843 autopsies [corrected]. Mod Pathol 1996; 9 (3): 233–237.

7. Russell RT, Pinson CW. Surgical management of polycystic liver disease. World J Gastroenterol 2007; 13 (38): 5052–5059.

8. Lantinga MA, Geudens A, Gevers TJ et al. Systematic review: the management of hepatic cyst infection. Aliment Pharmacol Ther 2015; 41 (3): 253–261. doi: 10.1111/apt.13047.

9. Husa P, Freibergerová M, Svačinka R et al. Liver abscesses – one of possible causes of fever of unknown origin. Klin Mikrobiol Infekc Lek 2009; 15 (2): 58–64.

10. Lantinga MA, Drenth JP, Gevers TJ. Diagnostic criteria in renal and hepatic cyst infection. Nephrol Dial Transplant 2015; 30 (5): 744–751. doi: 10.1093/ndt/gfu227.

11. Kanaan N, Goffin E, Pirson Y et al. Carbohydrate antigen 19-9 as a diagnostic marker for hepatic cyst infection in autosomal dominant polycystic kidney disease. Am J Kidney Dis 2010; 55 (5): 916–922. doi: 10.1053/j.ajkd.2009.12. 023.

12. Holánek M, Pokrivčák T, Ševela K et al. Infekce jaterní cysty u pacienta s autozomálně dominantní polycystickou chorobou ledvin (ADPKD). Interní Med 2014; 16 (3): 116–118.

13. Grams J, Teh SH, Torres VE et al. Inferior vena cava stenting: a safe and effective treatment for intractable ascites in patients with polycystic liver disease. J Gastrointest Surg 2007; 11 (8): 985–990. doi: 10.1007/s11605-007-0182-3.

14. Gevers TJ, de Koning DB, van Dijk AP et al. Low prevalence of cardiac valve abnormalities in patients with autosomal dominant polycystic liver disease. Liver Int 2012; 32 (4): 690–692. doi: 10.1111/j.1478-3231.2011.02683.x.

15. Schievink WI, Spetzler RF. Screening for intracranial aneurysms in patients with isolated polycystic liver disease. J Neurosurg 1998; 89 (5): 719–721. doi: 10.3171/jns.1998.89.5. 0719.

16. Gigot JF, Jadoul P, Que F et al. Adult polycystic liver disease: is fenestration the most adequate operation for long-term management? Ann Surg 1997; 225 (3): 286–294.

17. Qian Q, Li A, King BF et al. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 2003; 37 (1): 164–171. doi: 10.1053/jhep.2003.50006.

18. Schnelldorfer T, Torres VE, Zakaria S et al. Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg 2009; 250 (1): 112–118. doi: 10.1097/SLA.0b013e3181ad 83dc.

19. Lantinga MA, D‘Agnolo HM, Casteleijn NF et al. Hepatic cyst infection during use of the somatostatin analog lanreotide in autosomal dominant polycystic kidney disease: an interim analysis of the randomized open-label multicenter DIPAK-1 study. Drug Saf 2017; 40 (2): 153–167. doi: 10.1007/s40264-016-0486-x.

20. Walz G, Budde K, Mannaa M et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med 2010; 363 (9): 830–840. doi: 10.1056/NEJMoa1003 491.

21. Wong MY, McCaughan GW, Strasser SI. An update on the pathophysiology and management of polycystic liver disease. Expert Rev Gastroenterol Hepatol 2017; 11 (6): 569–581. doi: 10.1080/17474124.2017.1309280.

22. D‘Agnolo HM, Kievit W, Takkenberg RB et al. Ursodeoxycholic acid in advanced polycystic liver disease: a phase 2 multicenter randomized controlled trial. J Hepatol 2016; 65 (3): 601–607. doi: 10.1016/j.jhep.2016.05.009.

23. Takei R, Ubara Y, Hoshino J et al. Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease. Am J Kidney Dis 2007; 49 (6): 744–752. doi: 10.1053/j.ajkd.2007.03. 018.

24. Drenth JP, Chrispijn M, Nagorney DM et al. Medical and surgical treatment options for polycystic liver disease. Hepatology 2010; 52 (6): 2223–2230. doi: 10.1002/hep.24036.