Therapeutic intervention is warranted in the management of symptomatic liver cysts or when complications occur. For symptomatic cases, procedures available include: percutaneous aspiration with ethanol sclerotherapy, laparoscopic or open cyst fenestration (deroofing), hepatic resection and liver transplantation. The somatostatin analogue, lanreotide, reduces the volume of polycystic livers but has a modest clinical effect[15
]. Complications of cystic liver disease are rare, and we have demonstrated in this series that both operative and non-operative strategies have defined roles in management.
Biliary obstruction can initially be relieved with ERCP and stent insertion but definitive treatment is required. In this series, definitive treatment was with liver cyst fenestration. Laparoscopic fenestration has largely superseded the open approach for symptomatic liver cysts. This treatment is particularly suited to solitary liver cysts, particularly if more anteriorly located. With careful selection, good operative technique and, in a high-volume centre, good results can be obtained. A recent series of 51 patients reported complete relief of symptoms at a median follow-up of 13 mo, nine minor perioperative complications and a median hospital stay of 2 d[16
]. Symptomatic relief and a median volume reduction of 12.5% has been achieved in patients with polycystic liver disease[17
]. Percutaneous aspiration with ethanol sclerotherapy would be an acceptable alternative strategy[18
], as although the cysts will not disappear, they will reduce in size and this may be sufficient to relieve the extrinsic compression by the cyst on the bile duct.
Liver cyst rupture was managed conservatively with percutaneous drainage in the second patient in this series, due to the patient’s multiple medical co-morbidities and age. Although simple percutaneous drainage of liver cysts is almost inevitably associated with recurrence[19
], it was used in this case because the cyst had already ruptured and surgery was not an appropriate option for this patient. Sclerotherapy was not performed because the cyst had already ruptured.
Diagnostic uncertainty often results in surgical resection, in order not to miss a neoplastic condition, such as cystadenoma or cystadenocarcinoma or where the cyst has features of hydatid disease. The latter consideration, coupled with symptoms, led to the decision to undertake open resection of the cyst, with an adjacent segment of small bowel in patient 3. The bowel was intimately adhered to the cyst wall, presumably as a result of a cyst rupture, which the bowel had contained. Hydatid serology was negative in this case but a negative result is not completely reliable and immunodiagnosis plays a minor role in the definitive diagnosis of hydatid disease[20
Portal vein occlusion, associated with splenic varices occurred as a complication in patient 4. Although segment II and III liver resection is now commonly performed laparoscopically, the presence of portal hypertension and varices indicated that the patient was at high risk of haemorrhage so open resection of the cyst was performed. Experience with liver resection for cystic liver disease is limited; the largest series published to date reported on 124 patients with polycystic liver disease undergoing partial hepatectomy with cyst fenestration, over a 21-year period[21
]. Although good symptom relief were obtained, this was at a cost of a 63% in-hospital complication rate and a 3% mortality rate. This operation is technically demanding, as the polycystic liver is large and rigid with decreased mobility and reduced access to vascular inflow and outflow. Hepatic transection is complicated by displacement of hepatic veins and bile ducts from their normal anatomical positions. Liver resection is not, therefore, a first-line treatment for liver cysts but has a particular role when doubt as to the nature of the cyst exists[22
The fifth patient presented with ascites and obstruction of the inferior vena cava. This was treated by a combination of cyst aspiration, diuretics and somatostatin analogues. The rationale for somatostatin analogue therapy is its inhibitory effect on cholangiocyte proliferation and cyst fluid secretion[23
]. The clinical effect of this has recently been evaluated in a randomised double-blind, placebo-controlled trial with the somatostatin analogue, lanreotide in patients with polycystic liver disease[15
]. Although an effect on the natural course of the disease was confirmed, this only amounted to a 2.9% reduction of liver volume. Further clinical trials are required before this treatment can be recommended for adoption into practice.
Finally, we demonstrate the utility of PET-CT imaging in demonstrating complete resolution of infected liver cysts with appropriate antibiotic therapy. Multiple proven recurrences have led to this patient with polycystic liver disease being referred for liver transplantation. Liver transplantation remains the only curative option for the minority of patients with severe polycystic liver disease who have disabling symptoms and poor quality of life. Recent results of liver transplantation are encouraging. In a large cohort of 58 patients with isolated polycystic liver disease the 5-year patient survival was 92% and in the cohort with 121 patients with polycystic kidney and liver disease this survival rate was 89%[24
]. The collective experience on 218 patients from the European Liver Transplant Registry indicated a 5-year survival of 80%[25
The current series has demonstrated several rare complications of simple liver cysts and polycystic liver disease. As these are rare, there are no consensus treatment strategies, but we highlight the role of tailored individual treatment, taking into account, cyst site and size, possibility of malignancy, medical co-morbidities and technical feasibility. We have shown that a role for liver resection remains, especially when there is diagnostic uncertainty. The mainstays of treatment are either aspiration/sclerotherapy or, alternatively laparoscopic fenestration. Medical management with somatostatin analogues is a potentially new and exciting treatment option but requires further study.