Obstructive Jaundice in a Patient with Polycystic Liver Disease Complicated with Polycystic Kidney and Polycystic Lung: A Case Report

Overview

Journal Medicine (Baltimore)

Specialty General Medicine

Date 2020 Apr 4

PMID 32243367

Citations 1

Authors

Liling Zhang

Linwang Gan

Qi Liu

Ying Li

Jiaru Lin

Santao Ou

Affiliations

Soon will be listed here.

Abstract

Rationale: Polycystic liver disease (PLD) is an autosomal-dominant disorder that is commonly associated with autosomal-dominant polycystic kidney disease (PKD) but rarely complicated with polycystic lung. Here, we report the first case of severe obstructive jaundice caused by multiple liver cysts in a patient with PLD complicated by PKD and polycystic lung.

Patient Concerns: A 72-year-old man with a history of PLD complicated with polycystic kidney presented with progressive jaundice, hematuria, poor appetite, nausea, and weight loss since 3 months.

Diagnosis: PLD complicated with PKD and polycystic lung was identified using computed tomography, and obstructive jaundice was identified using magnetic resonance imaging and magnetic resonance cholangiopancreatography.

Interventions: The patient could not undergo surgery, and was therefore treated with combined bilirubin adsorption and continuous veno-venous hemofiltration.

Outcomes: The patient's symptoms and laboratory findings improved after bilirubin adsorption and continuous veno-venous hemofiltration. Unfortunately, the patient was unable to continue the treatment due to financial reasons, and died of shock most likely due to cyst rupture.

Lessons: Imaging examination of the lungs is necessary for patients with PLD. Although infrequent, jaundice can occur in these patients and cause severe hyperbilirubinemia. When surgery is contraindicated, blood purification may serve as an alternative treatment for patients with PLD-related obstructive jaundice.

Citing Articles

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Misak C, Naylor C, Tonelli M, Greenhalgh T, Foster G Wellcome Open Res. 2023; 7:158.

PMID: 37502738 PMC: 10369008. DOI: 10.12688/wellcomeopenres.17759.1.

References

Wittig J, Burns R, LONGMIRE Jr W . Jaundice associated with polycystic liver disease. Am J Surg. 1978; 136(3):383-6. DOI: 10.1016/0002-9610(78)90300-8. View

Jones W, Mountain J, WARREN K . Symptomatic non-parasitic cysts of the liver. Br J Surg. 1974; 61(2):118-23. DOI: 10.1002/bjs.1800610211. View

Gevers T, Drenth J . Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol. 2013; 10(2):101-8. DOI: 10.1038/nrgastro.2012.254. View

Everson G, Taylor M, Brian Doctor R . Polycystic disease of the liver. Hepatology. 2004; 40(4):774-82. DOI: 10.1002/hep.20431. View

van Keimpema L, de Koning D, van Hoek B, van den Berg A, van Oijen M, de Man R . Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases. Liver Int. 2010; 31(1):92-8. DOI: 10.1111/j.1478-3231.2010.02247.x. View

Raynaud P, Carpentier R, Antoniou A, Lemaigre F . Biliary differentiation and bile duct morphogenesis in development and disease. Int J Biochem Cell Biol. 2009; 43(2):245-56. DOI: 10.1016/j.biocel.2009.07.020. View

Tong F, Liang Y, Zhang L, Li W, Chen P, Duan Y . Fatal liver cyst rupture in polycystic liver disease complicated with autosomal dominant polycystic kidney disease: A case report. Forensic Sci Int. 2016; 262:e5-8. DOI: 10.1016/j.forsciint.2016.03.045. View

Dumont P, Rode A, Mabrut J, Ducerf C, Golse N . Polycystic liver disease complicated by obstructive jaundice. J Visc Surg. 2016; 153(2):149-51. DOI: 10.1016/j.jviscsurg.2015.11.016. View

Khoonsari M, Zamani F, Asoubar M, Vafaeimanesh J . Obstructive Jaundice in a Patient with Polycystic Liver. Middle East J Dig Dis. 2018; 10(2):117-120. PMC: 6040926. DOI: 10.15171/mejdd.2018.101. View

10.

Bae K, Zhu F, Chapman A, Torres V, Grantham J, Guay-Woodford L . Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2007; 1(1):64-9. DOI: 10.2215/CJN.00080605. View

11.

Delis S, Bakoyiannis A, Triantopoulou C, Paraskeva K, Athanassiou K, Dervenis C . Obstructive jaundice in polycystic liver disease related to coexisting cholangiocarcinoma. Case Rep Gastroenterol. 2011; 2(2):162-9. PMC: 3075137. DOI: 10.1159/000129600. View

12.

Bellomo R, Tipping P, Boyce N . Continuous veno-venous hemofiltration with dialysis removes cytokines from the circulation of septic patients. Crit Care Med. 1993; 21(4):522-6. DOI: 10.1097/00003246-199304000-00011. View

13.

Lee P, Matson J, Pryor R, Hinshaw L . Continuous arteriovenous hemofiltration therapy for Staphylococcus aureus-induced septicemia in immature swine. Crit Care Med. 1993; 21(6):914-24. DOI: 10.1097/00003246-199306000-00022. View

14.

Ergun H, Wolf B, Hissong S . Obstructive jaundice caused by polycystic liver disease. Radiology. 1980; 136(2):435-6. DOI: 10.1148/radiology.136.2.7403520. View