Value of clinical classification of adult polycystic liver disease for selection of treatments / 第二军医大学学报

Objective: To study the role of clinical classification of adult polycystic liver disease (APLD) in guiding the selection of treatments for the patients. Methods: The clinical data of 43 APLD patients who were treated in our hospital from May 1995 to Oct. 2007 were retrospectively analyzed. The patients were divided into 3 stages according to the clinical manifestations and imaging examination: non-symptom or slight symptom stage, obvious symptom stage, and lethal symptom stage. Seventeen patients in our group received outpatient clinical follow-up; 26 were treated by partial hepatectomy combined with fenestration. Results: Among the 15 cases at non-symptom or slight symptom stage, one had primary hepatic carcinoma simultaneously and died 4 mouths after diagnosis; the rest 14 case were followed up for a mean of (42.8±37.9) months; 12 of them had slow progression and 2 had rapid progression of APLD; the latter 2 cases received operatioa The 24 cases at obvious symptom stage were treated by partial hepatectomy combined with fenestration. There were no perioperation deathes and the mean follow-up period was (61.4±43.0) months; 2 cases died of renal failure and 3 had post-operation recurrence. Two of the 4 cases at the lethal symptom stage were treated by partial hepatectomy combined with fenestration: one had no symptom relieve after operation and one developed hepatic dysfunction and received liver transplantation 8 months after operation; the other 2 patients received percutaneous cyst aspiration at the outpatient department, one patient died of upper gastrointestinal hemorrhage 5 months later, and one had the symptom slightly relieved. Conclusion: Our classification of APLD can help to select treatments for patients: patients at non-symptom or slight symptom stage should be followed up, those at the obvious symptom stage can be treated surgically, and those at the lethal symptom stage are not suitable for routine operation and liver transplantation may be the best choice for them.

Surgical Treatment of Unilobar Adult Polycystic Liver Disease

Adult polycystic liver disease (APLD) is an inherited, benign rare condition, often associated with polycystic kidney disease. Liver failure is unusual, but some patients may require therapy. Surgery appears to be more effective in relieving the symptoms of APLD for an extended period than nonsurgical therapies. We report on the successful surgical treatment of a case of APLD located in the left lobe of the liver.