| Background:Autosomal dominant polycyst liver disease (APLD) is a genetic heterogeneous disorder characterized by progressive development of multiple fluid-filled, biliary epithelial liver cysts, and frequently associated with automsomal dominant polycystic kidney disease. Most cases of APLD are asymptomatic and do not require surgical treatment. However with more effectivetreatment of renal disease, increasing numbers of patients are living long enough to experience symptoms from their associated polycystic liver disease. Significant symptoms or complications from liver involvement can occur in up to 20 percent of cases. In symptomatic PCLD patients, surgical therapy is the mainstay of therapy including laparoscopic or open fenestration with or without hepatic resection and orthotopic liver transplantation. The surgical therapy should be tailored to the extent of disease in each patient. In this artical, we analysized 43 patients treated in our hospital, and a new classification was developed as a stratification scheme to conceptualize which treatment to offer to patients with adult polycystic liver disease.1. Research on the significance of clinical classification for the treatment of autosomal dominant polycystic liver diseaseObjective The aim of this study was to develop a new classification of adult polycystic liver disease(APLD) as a stratification scheme to help us conceptualize which treatment to offer to patients with APLD. Methods From May 1995 to Oct 2007, 43 patients suffering from APLD were referred to our hospital, 17 patients were received outpatient clinical visitation, 26 patients were treated by combined partial hepatectomy and fenestration. According to their clinical symptoms and imaging examinations, We classified them into three stages: no symptomatic or minimally symptomatic stage, obviously symptomatic stage, lethal symptomatic stage. Results 15 cases were in no symptomatic or minimally symptomatic stage. 1 patient of APLD suffered from primary hepatic carcinoma simultaneously, died of 4 mouths after diagnosis. The mean follow up time for the present was 35.8 mouths. Symptoms of 12 patients developed slowly, and they were accepted visitation in outpatient department. 2 patients symptoms developed rapidly, and accepted surgical treatment. 24 cases in obviously symptomatic stage were treated by combined partial hepatectomy and fenestration, which were discharged free of preoperative symptoms. The mean follow up time for the present was 55.4 mouths. Three patient had symptoms recurrence 81,68,43 mouths after operation. Symptom recurrence rate was 13.0 %. 2 patients died of renal failure due to polycystic kidney disease 137 and 85 mouths after operation. 4 cases were in lethal symptomatic stage. 2 cases were treated by combined partial hepatectomy and fenestration, 1 of which did not alleviate symptoms after operation; and 1 patient received liver transplantation 8 mouths after operation because of liver disfunction. 2 patients visited in outpatient accepted percutaneous cyst aspiration with alcohol sclerotherapy, 1 patient died of upper gastrointestinal hemorrhage caused by portal hypertension; 1 patient had to accept percutaneous cyst aspiration with alcohol sclerotherapy repeatedly. Conclusion Our classification of APLD can be a stratification scheme to help doctors conceptualize which treatment to offer to patients with APLD, patients in no symptomatic or minimally symptomatic stage should received outpatient visitation; patients in obviously symptomatic stage can be treated by combined hepatic resection and fenestration; surgical treatment is contraindication in lethal symptomatic stage, liver transplantation is the best choice for patients in this stage.2. Combined hepatic resection-fenestration for treatment of highly symptomatic adult polycystic liver diseaseObjective The aim of this study was to evaluate immediate and long-term outcomes in a series of patients with highly symptomatic autosomal dominant polycyst liver disease (APLD) treated by combined hepatic resection and fenestration technique. A new classification of APLD was recommended to presume postoperative complications and long outcome of each patient. Methods Between May 1995 and May 2007, 24 patients with highly symptomatic APLD were treated by combined hepatic resection and fenestration technique in our surgery team. All patients were reviewed retrospectively, and clinical symptoms, performance status and morbidity were recorded before surgery and after surgery. Follow-up was made by clinical and CT examinations in all patients. A new classification of patients with APLD that is different from Giot classification1 was recommended. According to the number of deep site cysts in the liver parenchyma that could not been treated during operation by preoperative evaluation, each grade was classified into two types: class A and B. And in each types, according to the location of cysts diffused in the liver, they were further classified into three grades: Gradeâ… ,â…¡andâ…¢. Results No patient died during the operation. All 24 patients were discharged from the hospital free of the preoperative symptoms. The mean follow-up time was 55.7 months (median 54, range 6-151). Three patients had recurrence of the symptoms 81, 68 and 43 mouths after operation with a symptom recurrence rate of (3/24) 12.5%. Two patients died of renal failure due to polycystic kidney disease 137 and 85 mouths after operation. The overall morbidity rate was79.2% (19/24) . Two patients with Type B-â…¡and TypeB-â… developed right biliary leakage. Seven patients had severe ascites, including three patients with Type B-â…¢and four patients with Type B-â…¢. Eleven patients had pleural effusion, including one patient with Type A-â… and one with Type B-â… ; five patients with Type B-â…¡; four with Type B-â…¢. Three patients with Type B had recurrence of the symptoms, while no patient with Type A had severe complications. Conclusions Our experience demonstrates that combined hepatic resection and fenestration is a safe and acceptable procedure for the treatment of highly symptomatic APLD. Although the morbidity rate seems high, this procedure still provides patients with severe symptoms with an opportunity for symptomatic and clinical improvement with an acceptable recurrence rate. According to our classification, postoperative complications and long outcome can be predicted before surgery.3. Research on the feasibility of surgical treatment of autosomal dominant polycystic liver disease associated with autosomal dominant polycystic kidney disease.Objective To research the feasibility of autosomal dominant polycystic liver disease(APLD) associated with autosomal dominant polycystic kidney disease(APKD) treated by procedures of partial hepatectomy and fenestration combined with renal cyst decortication. Methods From July 2007 to Oct 2007, three patients with polycystic liver disease associated with autosomal dominant polycystic kidney disease treated by partial hepatectomy and fenestration combined with renal cyst decortication were analyzed retrospectively. Symptoms before operation, operation time,complications and following outcomes were recorded. Results No mortality in our series, operation time of the 3 patients were 475, 402 and 430 minutes, pleural effusion and ascites occurred in all patients, after 9, 6 and 5 mouths visitation, discomfort symptoms disappeared in all patients, renal function and blood pressure of 2 patients recovered to normal. Conclusion Our experience demonstrates that this surgical technique can be a safe and acceptable procedure for treatment polycystic liver disease associated with autosomal dominant polycystic kidney disease. But there morbidity were high, long term outcomes should be evaluated in the future.4. Research on the complications of prevention and treatment of autosomal dominant polycystic liver disease undergoing partial hepatectomy and fenestration.Objective The aim of this study was to research complications of prevention and treatment of autosomal dominant polycystic liver disease (APLD) undergoing partial hepatectomy and fenestration. Methods Retrospectively analyzed 24 patients suffering from APLD in our hospital, all patients were treated with partial hepatectomy and fenestration. argon beam coagulator were used in 20 cases to destroy epithelium of the cysts and 4 patients cysts fluid composition were examined during operation Their complications, crystal and blood transfusion were recorded. Results No mortality in our series,20 cases received blood transfusion, Morbidity is 79.2%,including hemorrhage in 1 case, biliary leakage in 2 cases, ascites in 19 cases, severe ascites in 9 cases, pleural effusion in 14 cases, abdominal infection in 2 cases. The mean draining fluid after operation is 2886.1 millilitres. Conclusion Morbidity of patients with APLD undergoing partial hepatectomy and fenestration is high, more attention should be paid to the prevention during operation and treatment of complications after operation. |
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