The pathogenesis of the disease is not yet known, but has been thought to be related to chronic biliary ductal inflammation from pancreatic juice reflux, biliary infection or stone disease . The resulting inflammation and duct dilation induces overproliferation of the bile duct epithelium followed by the dysplasia-carcinoma sequence. The obstruction is thought to be due to mucous secretion or the enlarging papilloma.
Treatment for this benign disease has included surgery (including liver transplantation), palliative stenting, drainage or ablation. In general, if the disease is localized, lobectomy offers the best chance for cure. Small, localized tumour masses may be ablated using radiofrequency when resection is not possible. Choledochotomy, tumour curettage and T-tube drainage have been used for tumours involving the common bile duct, which often prove to be the most challenging surgically. With diffuse and bilateral disease, liver transplantation may offer the only chance of cure . Given that this is a new therapeutic option for BP, long-term follow-up data are not available. In cases where resection or transplantation is not possible, palliative stenting or bilioen-teric drainage with a Hutson access loop may be valuable for preventing recurrent cholangitis. buy asthma inhalers
BP is associated with high recurrence rates and poor prognosis secondary to septic complications . Survival rates in the published case series are poorly reported, with incomplete follow-up. Without resection, the prognosis is poor, with median survival of 11 months. Lee et al found that 20 of 24 patients (83%) with BP who underwent curative resection were alive after five years. Also, in a review of 76 patients, Yeung et al found that the pooled mean survival time after resection for both malignant and benign BP was 28 months. The shorter survival time is likely due to the inclusion of malignant disease. In the same study, patients undergoing percutaneous drainage or laser ablation had survival rates ranging from six to 17 months.
Although BP is a rare condition, it is not without consequence. A high index of suspicion is required to diagnose BP, because it is prone to recurrence, has a high malignant potential and may be a diffuse process. The natural history of this benigndisease usually commits patients to an intermittent course of recurrent abdominal pain, cholangitis and sepsis. Death is most often due to sepsis and/or liver failure. Given these potential complications,treatment recommendations involve resection, ablation, transplantation or palliative stent-ing and drainage. Furthermore, clinicians must be cognizant of the high likelihood of malignant transformation to cholangio-carcinoma.