Behcet's disease

Three women aged 30, 38 and 34 years, originating from Morocco, the Dominican Republic and Turkey, had been suffering since several years from, among other things, oral aphthae, vaginal ulcers and skin lesions without specific abnormalities at examination of blood and biopsies. The diagnosis of Behcet's disease was made and immunomodulating treatment instituted, following which the patients improved. Behcet's disease is a chronic inflammatory multisystemic disorder of unknown aetiology. Originally it was characterised as a triad of recurrent oral aphthae, genital ulcers and inflammatory eye disease. Because symptoms manifest themselves over many years the disease can be missed or misdiagnosed easily. There is no laboratory test available to support the diagnosis, so that the diagnosis is based on clinical symptomatology. The incidence is increasing due to migration from areas with higher prevalence.</p

Behcet's disease

Three women aged 30, 38 and 34 years, originating from Morocco, the Dominican Republic and Turkey, had been suffering since several years from, among other things, oral aphthae, vaginal ulcers and skin lesions without specific abnormalities at examination of blood and biopsies. The diagnosis of Behcet's disease was made and immunomodulating treatment instituted, following which the patients improved. Behcet's disease is a chronic inflammatory multisystemic disorder of unknown aetiology. Originally it was characterised as a triad of recurrent oral aphthae, genital ulcers and inflammatory eye disease. Because symptoms manifest themselves over many years the disease can be missed or misdiagnosed easily. There is no laboratory test available to support the diagnosis, so that the diagnosis is based on clinical symptomatology. The incidence is increasing due to migration from areas with higher prevalence.</p

Prevention of implantation metastases after resection of proximal bile duct tumours with pre-operative low dose radiation therapy

AIMS: Pre-operative endoscopic retrograde cholangiopancreatography (ERCP) with biliary drainage is associated with a greater risk of implantation metastases after resection of proximal bile duct tumours. In a previous study among patients who had undergone biliary drainage before resection, eight patients (20%) developed implantation metastases, within 1 year following resection. The aim of this analysis was to evaluate the results of pre-operative irradiation with regard to a possible reduction of implantation metastases. METHODS: Twenty-one patients with proximal bile duct tumours who had undergone resection following pre-operative irradiation were retrospectively analysed. Pre-operative radiation therapy consisted of three fractions of 3.5 Gy external beam irradiation of the hilar area. RESULTS: Pre-operative biliary drainage was performed in 19 patients (90%). All patients received pre-operative radiotherapy during which no complications were noted. None of the patients developed implantation metastases within a follow-up time of 2 to 79 months. CONCLUSION: The results of this study suggest that pre-operative radiotherapy in patients with a resectable proximal bile duct tumour who have undergone pre-operative drainage, decreases the risk of implantation metastases. To be certain about the role of pre-operative radiotherapy, a randomized study is required. Until then, we advocate standard low dose radiotherapy preceding resection in all patients with lesions suggestive of a proximal bile duct tumour who have undergone biliary drainag

Long-term survival after resection of proximal bile duct carcinoma (Klatskin tumors)

This retrospective study in 79 surgically treated patients with a proximal bile duct carcinoma revealed 12 patients with a median age of 59.5 years (range 21-73 years) who survived more than 5 years. These 12 patients were analyzed to identify specific patient characteristics for long-term survival. Fifteen patients died from postoperative complications and were excluded from this survival analysis. In relation with preoperative Bismuth classification, there were 3 (20%) long-term survivors of 15 patients with type I tumors and 9 (35%) long-term survivors of 26 patients with type II tumors. In the group of type III and IV tumors, there were no long-term survivors. Concerning the type of resection, 9 of 51 (18%) patients had long-term survival after local resection and 3 of 13 (23%) patients after local resection combined with hemihepatectomy. Complete tumor-free surgical specimen margins were found in only 4 of 64 cases (6%), among which only one patient survived more than 5 years. Negative proximal bile duct margins, absence of multifocality, and diploid tumors showed a significant correlation with long-term survival. There was no significant correlation between long-term survival and postoperative radiotherapy. Of the 12 long-term survivors, 5 died after 5 years: 2 had developed metastases and 1 a local recurrence; the other 2 died of a metastasis of an ovarian adenocarcinoma and cachexia, respectively. The remaining seven patients were still alive at the completion of this study. The mean survival of the 64 patients analyzed in this study (in which hospital mortality was excluded) was 33.7 months, with a median survival of 18.8 months. In conclusion, the preoperative Bismuth classification of the tumor, absence of multifocality, diploid-type tumors, and negative proximal bile duct margins at histopathologic examination were the only significant prognostic factors for long-term surviva

Unusual cases of acute cholecystitis and cholangitis: Tokyo Guidelines

Unusual cases of acute cholecystitis and cholangitis include (1) pediatric biliary tract infections, (2) geriatric biliary tract infections, (3) acalculous cholecystitis, (4) acute and intrahepatic cholangitis accompanying hepatolithiasis (5) acute biliary tract infection accompanying malignant pancreatic-biliary tumor, (6) postoperative biliary tract infection, (7) acute biliary tract infection accompanying congenital biliary dilatation and pancreaticobiliary maljunction, and (8) primary sclerosing cholangitis. Pediatric biliary tract infection is characterized by great differences in causes from those of adult acute biliary tract infection, and severe cases should be immediately referred to a specialist pediatric surgical unit. Because biliary tract infection in elderly patients, who often have serious systemic conditions and complications, is likely to progress to a serious form, early surgery or biliary drainage is necessary. Acalculous cholangitis, which often occurs in patients with serious concomitant conditions, such as those in intensive care units (ICUs) and those with disturbed cardiac, pulmonary, and nephric function, has a high mortality and poor prognosis. Cholangitis accompanying hepatolithiasis includes recurrent pyogenic cholangitis, an epidemic disease in Southeast Asia. Biliary tract infections, which often occur after a biliary tract operation and treatment of the biliary tract, may have a fatal outcome, and should be carefully observed. The causes of acute cholangitis associated with pancreaticobiliary maljunction differ before and after operation. Direct cholangiography is most useful in the diagnosis of primary sclerosing cholangitis. If cholangiography visualizes a typical bile duct, differentiation from acute pyogenic cholangitis is easy. This article discusses the individual characteristics, diagnostic criteria, treatment guidelines, and prognosis of these unusual types of biliary tract infection