Polycystic liver disease – something to consider in case of an acute abdomen

The aim of this study was to evaluate the immediate and long-term results in a retrospective series of patients with highly symptomatic adult polycystic liver disease (APLD) treated by extensive fenestration techniques. A classification of APLD was developed as a stratification scheme to help surgeons conceptualize which operation to offer to patients with APLD. Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration. Clinical symptoms, performance status, liver volume measurement by computed tomography (CT), and morbidity were recorded before surgery and after surgery. Adult polycystic liver disease was classified according to the number, size, and location of liver cysts and the amount of remaining liver parenchyma. Follow-up was obtained by clinical and CT examinations in all patients. Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 patients and by laparoscopy in 2 patients, 1 of whom conversion to laparotomy was required). The mean preoperative liver volume was 7761 cm3. There was no mortality. Postoperative morbidity occurred in 50%, mainly from biliary complications, requiring reintervention in two cases. Massive intraoperative hemorrhage occurred in one patient. During a mean follow-up time of 71 months (range, 17 to 239 months), all patients were improved clinically according to their estimated performance status. The mean postoperative liver volume was 4596 cm3, which represents a mean liver volume reduction rate of 43%. However, in type III APLD, despite absence of clinical symptoms, a significant increase in liver volume was observed in 40% of the patients. Extensive fenestration is effective in relieving symptoms in patients with APLD. Hemorrhage and biliary complications are possible consequences of such an aggressive attempt to reduce liver volume. The procedure can be performed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestration procedure. In type III APLD, significant disease progression was observed in 40% of the patients during long-term follow-up. Fenestration may not be the most appropriate operation for long-term management of all types of APLD.

This clinical study evaluated the results of and defined the indications for laparoscopic fenestration of symptomatic nonparasitic hepatic cysts, either solitary or diffuse. Different surgical treatments have been proposed for highly symptomatic hepatic cysts: enucleation, fenestration, hepatic resection, and liver transplantation. The advent of laparoscopic surgery has given new opportunities but, at the same time, has increased the uncertainties concerning the proper management of these patients. Eight patients with solitary cysts and nine with polycystic liver and kidney disease (PLD) were seen during a period of 2 years. After a careful review of the symptoms, 6 patients were excluded from surgical treatment and 11 (4 solitary cysts and 7 PLD) were treated by laparoscopic fenestration. Postoperative morbidity and mortality rates, hospital stay, and clinical early and late results were evaluated. In the solitary cyst group, there was no surgical morbidity or deaths, and a complete regression of symptoms occurred in all patients. No recurrences were observed. In the PLD group, two patients had to be converted to laparotomic fenestration (28%). There were no deaths, and the surgical morbidity was limited to two cases of postoperative ascites. Symptomatic relief was obtained in 80% of patients, but the symptoms recurred in 60%. A subgroup of PLD at high risk for recurrence was identified. The best indications for laparoscopic fenestration seem to be solitary cyst and PLD characterized by large cysts mainly located on the liver surface (type 1), whereas PLD characterized by numerous small cysts all over the liver (type 2) should be considered a contraindication to laparoscopic fenestration.

This study describes the natural history of patients with polycystic liver disease, a rare disorder characterized by multiple hepatic cysts. Cases were identified through review of charts from a hepatology practice, a hepatobiliary surgery practice and a retrospective chart review of inpatient charts from 1990 to 2002. All patients had greater than four simple liver cysts without infectious etiology. Medical records were reviewed for history, physical examination, imaging, and laboratory data. Patients' family practitioners provided follow-up. Fifty-three cases (62.3% female, 37.7% male) were identified. The mean age at diagnosis was 56.4 yr. Thirty-eight cases (71.7%) had associated polycystic kidney disease. The minority of patients were symptomatic at diagnosis (pain in 19 (36.5%), dyspnea in 5 (9.6%), and restricted mobility in 5 (9.6%) with hepatomegaly in 23 (45.1%). Follow-up information was attainable for 40 patients with a mean follow-up duration of 4.69 yr (range 0.1-15 yr). Within this subgroup, 9 patients (22.5%) had cyst bleeding, 5 (12.5%) had cyst rupture, 5 (12.5%) had cyst infection, 12 (30%) required an intervention. One patient (2.5%) developed portal hypertension, and two (5%) received a liver transplant. One patient (2.5%) died due to complications from liver cysts. Most patients in this highly selected cohort were asymptomatic with normal hepatic function. Pain was the most common symptom. The natural history is variable however, with some patients developing complications including portal hypertension. Minimally invasive interventions are appropriate initially, with hepatic resection and liver transplantation reserved for those with severe symptoms or life-threatening complications.