Hepatic vein stenting for recurrent ascites in polycystic liver and kidney disease
David W. Mudge1,
James Taylor2 and
Kym M. Bannister1
1 Department of Renal Medicine and 2 Department of Interventional Radiology, Royal Adelaide Hospital, Adelaide, South Australia
Correspondence and offprint requests to: David W. Mudge, Department of Nephrology, Level 2, ARTS Building, Princess Alexandra Hospital, Ipswich Road, Woolloongabba, Queensland, Australia 4102. Email: david_mudge{at}health.qld.gov.au
Keywords: hepatic vein; polycystic disease; stent
 |
Case
|
---|
A 74-year-old female with autosomal-dominant polycystic kidney disease (ADPKD) had been on maintenance haemodialysis for end-stage kidney disease for 18 months, via an upper-arm arteriovenous fistula. The polycystic kidney disease was diagnosed in her 50s, and she was also known to have mild cystic disease of the liver on prior ultrasound scanning.
She was otherwise well, and had no history of any other liver disease. Other past medical history was unremarkable. She was a non-smoker and did not consume alcohol. She was taking erythropoietin, calcium carbonate, folic acid, omeprazole and rosuvastatin. After 6 months of haemodialysis, she began to complain of abdominal distension, and an ultrasound examination was performed which revealed multiple, large, hypodense lesions with the appearance of simple cysts. There was marked ascites, and she was admitted to hospital for further diagnostic procedures. A paracentesis was performed, yielding ascitic fluid with biochemical characteristics of a transudate (serum to ascitic fluid albumin gradient of 7 g/L). Cytology and microbiologic culture were negative, and a total of 5.5 L of fluid was drained. Serum biochemistry at this time showed an albumin concentration of 35 g/L, and normal liver function tests.
Her condition slowly deteriorated over several weeks with weight loss, anorexia and recurrent abdominal discomfort related to re-accumulation of the ascites. A computed tomography (CT) scan revealed no evidence of malignancy, but extensive ascites was again present. A diagnostic laparoscopy was also normal. Serology for hepatitis B and C was negative, and auto-immune liver disease was excluded. She proceeded to percutaneous liver biopsy to exclude cirrhosis. The biopsy showed a degree of architectural distortion, with fibrosis encircling some portal tracts, but with no cellular infiltrate and no hepatocyte necrosis. The criteria for a histopathological diagnosis of cirrhosis were therefore not met, and it was thought that the pathological changes were solely due to polycystic disease.
Her body weight continued to decline, but the serum albumin concentration remained normal only with albumin infusions during dialysis, and she required repeat paracentesis every 4 weeks. Eventually, some 18 months after commencing haemodialysis, she underwent a repeat CT scan with CT angiography and cavography with intravenous contrast. The liver was mildly enlarged by the presence of several cysts (Figure 1, left panel). The portal vein was clearly seen to be patent, and normal in calibre. The middle hepatic vein, however, was dilated up to the point where it joined the inferior vena cava (IVC). At this point, the middle hepatic vein appeared to be compressed by the hepatic cysts (Figure 1, right panel). Although the right and left hepatic veins appeared to be of normal calibre (not shown), even segmental outflow obstruction has been previously reported to promote ascites [1]. In view of these findings and the absence of any alternative pathology, the liver cysts were thought to be the cause of the hepatic venous outflow obstruction by way of extrinsic compression of the middle hepatic vein.

View larger version (67K):
[in this window]
[in a new window]
|
Fig. 1. CT scan in sagittal section (left panel) showing multiple hepatic cysts, and in coronal section (right panel) showing extrinsic compression of the middle hepatic vein by cysts.
|
|
Selective venography was performed, and confirmed a high grade stenosis of the middle hepatic vein, which was treated with angioplasty and insertion of a 10 mm self-expanding metal stent (Figure 2). The pressure in the hepatic vein was 23/21 mmHg, compared to 16/4 mmHg in the IVC, which itself was mildly elevated (normal mean pressure up to 8 mmHg). After the procedure the pressure in the hepatic vein fell to 17/12 mmHg and the angiographic appearance of the hepatic vein was satisfactory.

View larger version (51K):
[in this window]
[in a new window]
|
Fig. 2. Digital subtraction angiography (DSA) of the middle hepatic vein before (left panel) and after (right panel) insertion of a 10 mm expanding metal stent.
|
|
Within month of the procedure, the patient was regaining weight, had improved appetite and no evidence of recurrence of the ascites, and is presently well 18 months later.
 |
Discussion
|
---|
This case, to the best of our knowledge, is the first reported case of radiological hepatic vein stenting for extrinsic compression due to polycystic liver disease. Ascites rarely complicates polycystic kidney disease, and when it does it is due to either cirrhosis (usually from chronic hepatitis B or C), congenital hepatic fibrosis (which is usually manifest before 25 years of age in ADPKD), or hepatic vein thrombosis (Budd-Chiari syndrome) due to architectural disturbance in massively enlarged cystic liver disease [2].
In these situations, patients usually require liver resection, hepatic fenestration procedures, or orthotopic liver transplantation, all of which carry a high morbidity and significant mortality [3,4]. One series of four cases has been published in which patients with polycystic liver disease (but not significant renal disease) were found to have hepatic venous outflow obstruction due to compression of one or more hepatic veins by cysts. Three of these patients required liver transplantation, and one required a mesocaval shunt [1].
Surgical cyst de-roofing was considered in our patient, but in view of her age and frailty, we anticipated that there would be a significant risk. Instead, a minimally invasive, angiographic technique was successfully employed. The patient suffered no complications from the procedure and had an excellent clinical result. We believe that in patients with polycystic liver disease presenting with ascites, the hepatic veins as well as the portal vein should be imaged adequately to exclude extrinsic compression by a cyst, as this is readily treated radiologically.
Conflict of Interest Statement. None declared.
 |
References
|
---|
- Uddin W, Ramage JK, Portmann B et al. Hepatic venous outflow obstruction in patients with polycystic liver disease: pathogenesis and treatment. Gut 1995; 36: 142145[Abstract]
- Chauveau D, Grunfeld JP, Durand F, Belghiti J. Ascites in a polycystic patient. Nephrol Dial Transplant 1997; 12: 228230[Free Full Text]
- Que F, Nagorney DM, Gross JB Jr, Torres VE. Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology 1995; 108: 487494[CrossRef][ISI][Medline]
- van Erpecum KJ, Janssens AR, Terpstra JL, Tjon ATR. Highly symptomatic adult polycystic disease of the liver. A report of fifteen cases. J Hepatol 1987; 5: 109117[CrossRef][ISI][Medline]
Received for publication: 1. 3.05
Accepted in revised form: 8. 7.05