Primary angiosarcoma of the Pancreas - A case Report and review of the literature.

BACKGROUND: Angiosarcomas are malignant neoplasms that originate from endothelial cells. The symptoms exhibit a non-specific nature, and achieving a preoperative diagnosis is frequently challenging. They are seldom encountered in the abdomen, and their occurrence in the pancreas is even rarer. METHODS: Here we document a 67-year-old man with pancreatic angiosarcoma and analyse the literature to outline the clinicopathologic characteristics of this rare phenomenon. RESULTS: This patient with family history of pancreas cancer presented with abdominal pain, and the CT-scan revealed a 4 cm mass at the neck of the pancreas but CA19-9 was normal. Radiologic findings were unusual for ordinary pancreas cancer. Fine-needle aspiration biopsy through endoscopic ultrasound revealed "undifferentiated malignant cells for which the diagnosis of "carcinoma" was favoured. Total pancreatectomy, splenectomy and portal vein reconstruction were performed and epithelioid angiosarcoma were diagnosed. Despite an uneventful postoperative period, discharge on postoperative day 8 without any complications, as well as diligent post-discharge clinical care, the patient died 65 days postoperatively, attributed to the presence of extensive metastasis. A comprehensive literature search has identified a limited number of documented cases of primary pancreatic angiosarcoma, with only ten cases reported to date. CONCLUSIONS: Pancreatic angiosarcomas are very rare and prone to misdiagnosis. The formation of a more demarcated but high-grade tumour with necrosis is a feature that distinguishes angiosarcomas from ordinary carcinomas of this organ. Pathologic diagnosis is also highly challenging closely resembling undifferentiated carcinomas. Angiosarcomas are highly aggressive when they occur in the pancreas. Prompt diagnosis at an early stage is crucial as surgery with curative intent serves as the primary treatment approach.

Surgery with curative intent is the mainstay treatment for pancreatic angiosarcoma when diagnosed at an early stage.Oncological treatment options should be taken into consideration according to the follow-up data.Why does this paper matter?This article is important in that it is the most comprehensive review of the literature on pancreatic angiosarcoma, which is a very rare pathology, from the perspective of radiology, pathology and surgery.

Pancreatic surgery in elderly patients: results of 329 consecutive patients during 10 years.

Introduction: There is a progressive shift from a younger population to an older population throughout the world. With the population age shift, surgeons will be more encountered with older patient profiles. We aim to determine age-related risk factors of pancreatic cancer surgery and the effect of patient age on outcomes after pancreatic surgery. Materials and methods: A retrospective review was conducted with data obtained from consecutive 329 patients whose pancreatic surgery was performed by a single senior surgeon between January 2011 and December 2020. Patients were divided into three groups based on age: patients younger than 65 years old, between 65 and 74 years old, and older than 74 years old. Demographics and postoperative outcomes of the patients were evaluated and compared between these age groups. Results: The distribution of a total of 329 patients into the groups was 168 patients (51.06%) in Group 1 (age <65 years old), 93 patients (28.26%) in Group 2 (age ≥65 and <75 years old), and 68 patients (20.66%) in Group 3 (age ≥75 years old). The overall postoperative complications were statistically significantly higher in Group 3 than in Group 1 and Group 2 (p = 0.013). The comprehensive complication index of the patients in each group was 23.1 ± 6.8, 20.4 ± 8.1, and 20.5 + 6.9, respectively (p = 0.33). Fisher's exact test indicated a significant difference in morbidity in patients with ASA 3-4 (p = 0.023). In-hospital or 90-day mortality was observed in two patients (0.62%), one from Group 2 and one from Group 3. The 3-year survival rates for each group were 65.4%, 58.8%, and 56.8%, respectively (p = 0.038). Conclusion: Our data demonstrate that comorbidity, ASA score, and the possibility of achieving a curative resection do have significantly more impact than age alone.

Optimizing the Personalized Care for the Management of Rectal Cancer: A Consensus Statement.

Colorectal cancer is the third most common cancer in Turkey. The current guidelines do not provide sufficient information to cover all aspects of the management of rectal cancer. Although treatment has been standardized in terms of the basic principles of neoadjuvant, surgical, and adjuvant therapy, uncertainties in the management of rectal cancer may lead to significant differences in clinical practice. In order to clarify these uncertainties, a consensus program was constructed with the participation of the physicians from the Acibadem Mehmet Ali Aydinlar and Koç Universities. This program included the physicians from the departments of general surgery, gastroenterology, pathology, radiology, nuclear medicine, medical oncology, radiation oncology, and medical genetics. The gray zones in the management of rectal cancer were determined by reviewing the evidence-based data and current guidelines before the meeting. Topics to be discussed consisted of diagnosis, staging, surgical treatment for the primary disease, use of neoadjuvant and adjuvant treatment, management of recurrent disease, screening, follow-up, and genetic counseling. All those topics were discussed under supervision of a presenter and a chair with active participation of related physicians. The consensus text was structured by centralizing the decisions based on the existing data.

Risk Factors for Delayed Gastric Emptying After Pancreaticoduodenectomy.

OBJECTIVES: Delayed gastric emptying (DGE) is a complication that affects the length of hospitalization and associated cost after pancreaticoduodenectomy (PD). The reported risk factors for DGE were controversial. This study aimed to identify risk factors for the development of DGE after PD. METHODS: The patients who underwent PD between October 2010 and October 2020 were retrospectively examined. Multivariate analysis was performed to predict the variables causing DGE. RESULTS: In total, 225 patients underwent PD. The pylorus preserving PD was applied to 151 patients (67%), whereas standard PD to 74 (33%). The DGE was detected in 26 patients (11.5%). The majority of cases were classified as grade A (57.7%), whereas 38.4% as grade B and 3.9% as grade C. In the multivariate analysis, diabetes mellitus (odds ratio [OR], 3.48; 95% confidence interval [CI], 1.45-8.34; P = 0.05), the preoperative biliary stent (OR, 2.5; 95% CI, 1.04-5.99; P = 0.039), and the pylorus resection (OR, 3.05; 95% CI, 1.28-7.25; P = 0.012) were independently associated with DGE. CONCLUSIONS: We demonstrated that implementation of the preoperative stent, pylorus resection, and diabetes mellitus are independent risk factors for DGE. Pylorus preservation should remain the standard of care in PD.

Effects of the Largest Metastatic Lymph Node Size on the Outcomes of Patients who Underwent Pancreaticoduodenectomy for Pancreatic Ductal Adenocarcinoma.

Objectives: Prognostic importance of metastatic lymph nodes in pancreatic cancer is always garnered attention due to dismal prognosis, with some quantitative factors drawing attention for significantly predicting outcomes. Size is one of the easy approach morphological characteristics of the lymph node, and data for effect of largest metastatic lymph node (LMLN) size on survival outcomes are lacking in pancreatic cancer. We aim to evaluate the effect of LMLN size on the prognosis of patients with pancreatic ductal adenocarcinoma (PDAC). Methods: This retrospective study evaluates the effect of LMLN size on survival outcomes by grouping the patients who were surgically treated for PDAC, according to their lymph node stage and calculated cutoff value for LMLN size, between February 2015 and May 2020. Results: In the study cohort of 131 patients, the mean age was 63.9±10.8 years and 77 patients were female. Ninety-nine of the patients had pN1, 32 had pN2 stage disease. The optimal cutoff point of LMLN size for predicting the prognosis was calculated as 7.5 mm (sensitivity = 81% and specificity = 81%). 34 (34.3%) of pN1 and 7 (21.9%) of pN2-staged patients had lymph node smaller than 7.5 mm. Three-year survival was significantly longer for patients whose LMLN size was <7.5 mm (56.2-18.2%, p<0.001). Whereas, the patients with LMLN size <7.5 mm had statistically significant longer median survival rate in the subgroup of patients with pN1 lymph node stage, no significant difference in median survival rates was observed between subgroups of pN2 patients (p=0.237). Conclusion: The present study demonstrated that the LMLN size was one of the potential predictors of survival in patients with PDAC.

Is elective cancer surgery feasible during the lock-down period of the COVID-19 pandemic? Analysis of a single institutional experience of 404 consecutive patients.

BACKGROUND: We aimed to assess the feasibility and short-term clinical outcomes of surgical procedures for cancer at an institution using a coronavirus disease 2019 (COVID-19)-free surgical pathway during the peak phase of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic. MATERIALS AND METHODS: This was a single-center study, including cancer patients from all surgical departments, who underwent elective surgical procedures during the first peak phase between March 10 and June 30, 2020. The primary outcomes were the rate of postoperative SARS-CoV-2 infection and 30-day pulmonary or non-pulmonary related morbidity and mortality associated with SARS-CoV-2 disease. RESULTS: Four hundred and four cancer patients fulfilling inclusion criteria were analyzed. The rate of patients who underwent open and minimally invasive procedures was 61.9% and 38.1%, respectively. Only one (0.2%) patient died during the study period due to postoperative SARS-CoV2 infection because of acute respiratory distress syndrome. The overall non-SARS-CoV2 related 30-day morbidity and mortality rates were 19.3% and 1.7%, respectively; whereas the overall SARS-CoV2 related 30-day morbidity and mortality rates were 0.2% and 0.2%, respectively. CONCLUSIONS: Under strict institutional policies and measures to establish a COVID-19-free surgical pathway, elective and emergency cancer operations can be performed with acceptable perioperative and postoperative morbidity and mortality.

Surgical site infections after pancreaticoduodenectomy: Preoperative biliary system interventions and antimicrobial prophylaxis.

BACKGROUND: The rate of surgical site infection (SSI) after pancreaticoduodenectomy (PD) is high and insertion of preoperative bile duct catheterization (PBDC) predispose a high risk of SSI with multidrug resistant (MDR) microorganisms. AIM: To describe the effects of PBDC and the prophylaxis in development of SSI. METHODS: We conducted a retrospective study between January 01, 2010 and December 2018 including the patients with PD and total pancreatectomy. FINDINGS: In total 214 consecutive patients were included. The PBDC was inserted to 63 (29%) patients. The rate of intraoperative bile fluid culture positivity was higher among the patients with PBDC (84% vs. 17% respectively, p<0.001). The SSI was detected in 52 patients (24%). In multivariate analysis, the rate of SSI was found to be higher among the patients with PBDC (OR: 2.33, 95% Cl: 1.14-4.76, p=0.02). As the etiologic agents of SSI, Pseudomonas spp. and MDR pathogens were mainly detected in PBDC group. The resistance to ampicillin-sulbactam was significantly higher in the PBDC group (87.5% vs. 25%, p=0.012). The similar bacterial species both in bile fluid and the surgical site were detected in 11 (21%) patients with SSI. Among 8 patients (15%), antimicrobial susceptibility was the same. Only in five out of 52 (10%) patients, the SSI pathogens was susceptible to the agent that was used for surgical prophylaxis. CONCLUSION: Unnecessary catheterizations should be avoided. By considering the increasing prevalence of resistant bacteria as the cause of SSI, the clinicians should closely follow-up their patients for choosing the proper antimicrobials.

Transplantation for Large Liver Tumors in the Setting of Abernethy Malformation.

In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus. The Stanford-Binet intelligence scale showed moderate mental retardation (IQ 39); however, the patient showed good ability for caring for himself. His cognitive defect was ascribed partially to chronic encephalopathy. The patient received a right hepatic lobe from his older brother. The congenital portacaval shunt was disconnected to provide inflow to the graft. Pathologic examination of the explanted liver revealed no evidence of malignancy. His IQ improved to 75 at 29 months posttransplant. The hyperintensity of the globus pallidus on magnetic resonance imaging disappeared. The patient has maintained a normal life during 9 years of follow-up. Patient 2 was a 17-year-old girl who was referred for multiple hepatic masses; she had no symptoms at admission. Magnetic resonance imaging showed type 1 Abernethy malformation and multiple hepatic masses (largest was 10 cm), which appeared to be hyperplastic lesions. Because malignancy could not be definitely excluded, she received a right lobe without the middle hepatic vein from her uncle. Pathologic examination of the explanted liver showed localized nodular hyperplasia; there was no evidence of malignancy. She has maintained normal life activities during 3 years of follow-up. Liver transplant is a curative treatment option for patients with large liver tumors, replacing the hepatic parenchyma in the setting of Abernethy malformation.

Hepatic hydatid disease requiring urgent treatment during pregnancy.

BACKGROUND: Pregnant women may experience an acute presentation of hepatic hydatid disease. The available literature is limited to case reports. METHODS: The charts of 7 patients who underwent urgent treatment for hepatic hydatid disease during pregnancy between 1992 and 2010 were reviewed. RESULTS: The median patient age was 27 (range 23-39) years and median gestational age was 18 (range 13-24) weeks. The symptoms were severe abdominal pain (4), vomiting (2), jaundice (2), pruritus (2) and severe dyspepsia (1); in the asymptomatic patient, a closed intraperitoneal rupture had been detected during gynecologic ultrasonography. Surgical drainage of the cysts was performed in all cases. The two patients with frank biliary rupture underwent choledochoduodenostomy or Roux-Y hepaticojejunostomy. Four patients required postoperative tocolysis. Albendazole was not used. All mothers gave birth to healthy babies at term. The patients were followed for a median of 9 (range 4-19) years. Two patients developed recurrences at 2 and 7 years; these were treated with surgical drainage and albendazole. CONCLUSION: This entity entails the responsibility of two human beings. Although it imposes limitations on the routine diagnostic and therapeutic options due to risk of premature labor or teratogenicity, acceptable results can be obtained in collaboration with the department of obstetrics and gynecology.

Curative surgery for locally advanced retroperitoneal mature teratoma in an adult. Case report.

INTRODUCTION: Primary teratomas of retroperitoneum are not usual in the adult population. These tumors most commonly seen at the gonadal and sacrococcygeal regions. Herein we describe a case of an 18-year-old female who had a benign cystic teratoma at the retroperitoneum. PRESENTATION OF CASE: The patient underwent an operation at another hospital following a misdiagnosis of hydatid cyst. The patient was referred to our hospital because of the detection of an unresectable tumor during her operation. A computerized tomography (CT)-angiography revealed a cystic mass, with a diameter of 14cm which was invaded into the retrohepatic suprarenal inferior vena cava and also extended to the posterior aspect of the liver. Additionally the mass invaded the posterior wall of the inferior vena cava and the right renal vein. The tumor was completely resected with a vascular resection. The inferior vena cava was reconstructed with a 12cm Dacron(®) graft and the renal vein was implanted. The patient's postoperative period was uneventful. DISCUSSION: Germ cell tumors of retroperitoneum are usually seen in children, but there are also some reports of adult cases in the literature. Adult cases are especially seen in females. Imaging studies are paramount for diagnosis, preoperative strategy and safe surgical excision. CT scans and MRIs can identify various components of these tumors. CONCLUSION: Even though primary retroperitoneal teratomas are quite rare in adults. Preoperative radiology imaging and strategy is critical for performing a safe surgery. The gold standard treatment strategy for this neoplasm is the surgical resection.

Hepatic adenomatosis may mimic metastatic lesions of liver with 18F-FDG PET/CT.

Hepatic adenomatosis is an uncommon benign neoplasm, with the presence of multiple adenomas (generally more than 4) within the liver. A 52-year-old woman presented with multiple (>10) solid liver lesions detected with abdominal ultrasonography and verified with magnetic resonance imaging (MRI). Subsequently, F-18 FDG PET/CT demonstrated increased uptake in these lesions. Histology revealed hepatic adenomatosis. F-18 FDG PET/CT cannot reliably differentiate hepatic adenomas from malignant processes on the basis of uptake.

Intrahepatic biliary cystic neoplasms: Surgical results of 9 patients and literature review.

AIM: To investigate the eligible management of the cystic neoplasms of the liver. METHODS: The charts of 9 patients who underwent surgery for intrahepatic biliary cystic liver neoplasms between 2003 and 2008 were reviewed retrospectively. Informed consent was obtained from the patients and approval was obtained from the designated review board of the institution. RESULTS: All patients were female with a median (range) age of 49 (27-60 years). The most frequent symptom was abdominal pain in 6 of the patients. Four patients had undergone previous laparotomy (with other diagnoses) which resulted in incomplete surgery or recurrences. Liver resection (n = 6) or enucleation (n = 3) was performed. The final diagnosis was intrahepatic biliary cystadenoma in 8 patients and cystadenocarcinoma in 1 patient. All symptoms resolved after surgery. There has been no recurrence during a median (range) 31 (7-72) mo of follow up. CONCLUSION: In spite of the improvement in imaging modalities and increasing recognition of biliary cystadenoma and cystadenocarcinoma, accurate preoperative diagnosis may be difficult. Complete surgical removal (liver resection or enucleation) of these lesions yields satisfying long-term results.

A New Era in Minimally Invasive Liver Resection (MILR) Single-Incision Laparoscopic Liver Resection (SIL-LR): The First Two Cases.

Laparoscopy has gained a place in everyday surgical routine as an alternative surgical approach that decreases morbidity and postoperative hospitalization. Single port laparoscopic surgery has been introduced as a further development of laparoscopy. The feasibility and safety of single port laparoscopy is under extensive evaluation in specialized laparoscopic centers. Nevertheless, wide acceptance of the technique requires adequate documentation of the advantages of the approach over conventional laparoscopy and further refinement of surgical instrumentation to overcome intraoperative ergonomic problems.

Our early experiences with single-incision laparoscopic surgery: the first 32 patients.

BACKGROUND: Most of the laparoscopic surgeons have been attempting to reduce incisional morbidity and improve cosmetic outcomes by using fewer and smaller ports. Single-incision laparoscopic surgery (SILS) is a new laparoscopic procedure. Herein we would like to present our experiences. PATIENTS AND METHODS: Between January 2009 and October 2009, data of the 32 patients who were operated through SILS are evaluated prospectively. There were 22 females and 10 males with a mean age of 45.1±14.8 years. Ten splenectomies for idiopathic thrombocytopenic purpura, 16 cholecystectomies for acute cholecystitis or asymptomatic gallstones, 3 appendectomies for acute appendicitis, one distal pancreatosplenectomy for Renal Cell Cancer metastases, 1 adrenalectomy for Conn Syndrome and 1 case of liver resection for hepatic adenoma were carried out. The most common splenectomy indication was idiopathic thrombocytopenic purpura, cholecystectomy indication was acute cholecystitis (n=8), and asymptomatic cholelithiasis (n=8), appendectomy indication was acute appendicitis, distal pancreatosplenectomy indication was renal cell cancer metastases, adrenalectomy indication was Conn syndrome and liver resection indication was left hepatic mass in between segments 1 and 2. SILS was carried out successfully in 31 patients. Only in 1 patient conversion was required owing to bleeding. RESULTS: All procedures were carried out through a 2-cm umbilical incision. There was no mortality and morbidity recorded. CONCLUSIONS: In experienced hands of minimally invasive surgeons, SILS incision laparoscopic surgery could be carried out successfully in a wide range of surgical operations. However, to be able to show the differences between Standard laparoscopic surgery and single SILS, prospective randomized comparative studies are required.

Somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation.

We report our success with somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation. A 48-year-old woman with cirrhosis due to autoimmune hepatitis underwent living-donor liver transplant; her graft-to-body weight ratio of the right lobe was 0.91%. After arterial reperfusion, portal pressure and flow were 24 cm H20 and 2.22 L/min (ie, 360 mL/100g graft/min), respectively. Following splenic artery ligation, the portal pressure decreased to 16 cm H20 and portal flow to 1.74 L/min (ie, 282 mL/100g graft/min). On the second postoperative day, small-for-size syndrome was diagnosed based on the marked prolongation of prothrombin time (international normalized ratio, 4.4), hyperbilirubinemia (359.1 micromol/L), rapid escalation of transaminases (alanine aminotransferase 2488 U/L, aspartate aminotransferase 1075 U/L) and very high portal flow rate (> 90 cm/sec). Oral propranolol (40 mg/day b.i.d.) and somatostatin infusion (250-microgram bolus followed by perfusion at a rate of 250 microgram/h for 5 days) were started. Prothrombin time and transaminase levels began to decrease the following day, although the bilirubin level increased to 495.9 micromol/L before returning to normal. The patient was discharged in excellent health 5 weeks after surgery. Despite reduction of portal pressure by splenic artery ligation, small-for-size syndrome may develop in patients with persistent high portal flow. To the best of our knowledge, this is the first report of the successful treatment of small-for-size syndrome by somatostatin and propranolol in the clinical setting.

Bile duct injury during cholecystectomy requiring delayed liver transplantation: a case report and literature review.

Major bile duct injury during cholecystectomy represents potentially severe complications with unpredictable long-term results. If these lesions are not treated adequately, they can lead to hepatic failure or secondary biliary cirrhosis therefore requiring liver transplantation. We report a patient who required liver transplantation 15 years after open cholecystectomy. A l0-year old girl underwent open cholecystectomy and duodenal repair for cholelithiasis and cholecystoduodenal fistula. She required two surgical interventions, hepaticojejunostomy which was performed in another center and portoenterostomy for biliary stricture at our institution seven years after the cholecystectomy. Eight years after the third operation, she required recurrent hospitalization for treatment of hepatic abscesses. The extremely short intervals between the three life threatening episodes and the rapid progression to severe sepsis were taken into consideration and liver transplantation was performed at the age of 25. She is leading a healthy life at 4 years post transplantation. Although iatrogenic biliary injury can usually be treated successfully by a combination of surgery, radiological and endoscopic techniques, patients with severe injuries develop irreversible liver disease. This case report and review of the literature suggest that liver transplantation is a treatment modality for a selected group of patients with end-stage liver disease secondary to bile duct injury.

Hepatic atrophy-hypertrophy complex due to Echinococcus granulosus.

Obstruction of a major hepatic vein, or major portal vein, or biliary tree branch causes atrophy of the related hepatic region, and frequently, hypertrophy in the remaining liver-the atrophy-hypertrophy complex (AHC). Whether hydatid cysts can cause AHC is controversial. The records of 370 patients who underwent surgery for hepatic hydatid disease between August 1993 and July 2002 were evaluated retrospectively. Excluding six patients with previous interventions on the liver, AHC had been recorded in the operative notes of 16 patients (4.4%); for all patients, a cyst located in the right hemiliver had caused atrophy of the right hemiliver and compensatory hypertrophy of the left hemiliver. The computed tomography images of seven patients were suitable for volumetric analysis. The median (range) right and left hemiliver volumes were 334 (0-686) ml and 1084 (663-1339) ml, respectively. The median (range) cyst volume was 392 (70-1363) ml. AHC due to Echinococcus granulosus was confirmed by objective volumetric analysis. The presence of AHC should alert the surgeon to two implications. First, pericystectomy may be hazardous due to association with major vascular and biliary structures. Second, in patients with AHC, the hepatoduodenal ligament rotates around its axis; this should be considered to avoid vascular injury if a common bile duct exploration is to be performed.

Successful living-donor liver transplantation and retransplantation with cavoportal hemitransposition: a case report.

An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. End-to-end anastomosis of the recipient suprarenal vena cava to the graft portal vein (a left lateral section from the patient's mother) was performed. An end-to-side portocaval shunt with the recipient portal vein was constructed to mitigate portal hypertension. The early postoperative course was relatively uneventful. However, persistent hepatitis caused by infection with Cytomegalovirus and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, a living-donor retransplantation (a left lateral section from the patient's grandmother) was performed. One month after retransplantation, severe acute rejection that eventually required OKT3 treatment developed. The patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed. Cavoportal hemitransposition should be included in the armamentarium of the transplant surgeon for the management of extensive portal system thrombosis and portal vein hypoplasia. An additional shunt may be useful in mitigating portal hypertension.

Endoscopic and radiologic interventions as the leading causes of severe cholangitis in a tertiary referral center.

BACKGROUND: Iatrogenic factors became the leading mechanisms of severe cholangitis in a referral center. PATIENTS AND METHODS: The records of the 58 patients treated for severe cholangitis between 1996 and May 2004 (inclusive) were evaluated. RESULTS: The most frequent underlying diseases were periampullary tumors and mid-bile duct carcinomas (22), followed by proximal cholangiocarcinomas (14). The triggering mechanism was an incomplete endoscopic retrograde cholangiopancreatography (ERCP) in 32 patients, incomplete or inappropriate percutaneous transhepatic biliary drainage (PTBD) in 6, apparently successful ERCP and stenting in 1, and percutaneous transhepatic cholangiography in 1. PTBD was the treatment of choice (38). Mortality was 29% (17/58); the major causes were refractory sepsis (8) and incomplete biliary drainage (advanced tumor, technical failure, or hemobilia) (8). CONCLUSIONS: In this series composed predominantly of patients referred after development of sepsis, ERCP and PTBD complications were the leading mechanisms of severe cholangitis. Nonoperative biliary manipulations are invasive procedures with potentially fatal complications. The decisions to perform such procedures and periprocedural management are responsibilities of an experienced multidisciplinary team.