Current status in the surgical management of adult polycystic liver disease.

INTRODUCTION: In patients with adult polycystic liver disease (APLD), there is considerable debate surrounding the most effective way of managing symptomatic cysts. Conservative approaches like percutaneous aspiration or cyst fenestration are associated with low morbidity but high recurrence rates. Conversely, liver resection and hepatectomy with orthotopic liver transplantation is drastic and associated with high morbidity and mortality rates. Our aim is to review the current understanding of liver cystogenesis in these patients and the therapeutic options available in order to provide a rationale guide to management of this intriguing condition. METHODS: This article summarises the findings of published papers in major international journals indexed on MEDLINE on APLD using the key words--adult polycystic liver disease, liver cysts, fenestration, liver resection, liver transplantation and polycystic kidney disease. The period of search includes papers between 1965 and 2000. RESULTS: Published studies have suggested a 'two-hit' hypothesis to explain the development of liver cysts in patients with APLD. This will provide the rationale for future management. Meanwhile, the indications, pitfalls and results of the various therapeutic options are reviewed. Management of symptoms has to be tailored to the underlying severity of the liver cystic disease, co-morbidity and procedural risks and recurrence rates. CONCLUSION: Good long-term relief of symptoms can be achieved with the correct procedure at acceptable morbidity and mortality rates. We have provided guidelines on the various options available to enable a structured approach to the management of APLD.

Does laparoscopic fenestration provide long-term alleviation for symptomatic cystic disease of the liver?

BACKGROUND: The use of laparoscopic technique for management of symptomatic liver cysts is documented to be a feasible and safe procedure with good short-term symptomatic relief. However, it cannot be recommended as the standard of care as long-term results are scarce. The present study was initiated to review the long-term results of this approach in the management of symptomatic liver cysts. METHODS: A retrospective review of all patients with symptomatic liver cysts that were treated by laparoscopic fenestration in our department over an 8-year period from 1993 to 2001. The clinical and radiographical data were analysed at follow-up to assess the -outcome. RESULTS: Eleven patients were treated using a laparoscopic approach; 10 patients with solitary cysts and one with adult polycystic liver disease. All patients achieved short-term alleviation of symptoms and an uneventful postoperative course. The mean hospital stay was 3 days. Long-term follow up was available for 9 patients with a mean of 44 months. Histologically, one of the patients was diagnosed with a biliary cystadenoma and she had a symptomatic recurrence and a liver resection at 20 months. In the other seven patients, there was no clinical recurrence but a radiographical recurrence of 28.5%. The patient with adult polycystic liver disease had two symptomatic recurrences: at 26 months where he underwent a repeat laparoscopic fenestration and at 43 months where he underwent an open fenestration. CONCLUSION: The present study confirms that with adequate patients election, long-term alleviation of symptoms can be achieved with the laparoscopic approach for solitary simple liver cysts but not for polycystic liver disease or cystic tumours of the liver.