Risk factors for recurrent primary sclerosing cholangitis after liver transplantation.

BACKGROUND & AIMS: The association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) is well recognised. However, the relationship between IBD and recurrent PSC (rPSC) is less well understood. We assessed the prevalence of rPSC and analysed the factors associated with rPSC post-liver transplantation and its influence on graft and patient survival. METHODS: This is a UK multicentre observational cohort study across six of the seven national liver transplant units. All patients undergoing a first liver transplant for PSC between January 1 1990 and December 31 2010 were included. Prospectively collected liver transplant data was obtained from NHSBT and colitis data was retrospectively collected from individual units. RESULTS: There were 679 (8.8%) first transplants for PSC. 347 patients (61.4%) had IBD, of which 306 (88.2%) had ulcerative colitis (UC). 81 (14.3%) patients developed rPSC and 37 (48.7%) of them developed graft failure from rPSC. Presence of UC post-liver transplant (HR=2.40, 95% CI 1.44-4.02) and younger age (HR=0.78, 95% CI 0.66-0.93) were the only factors significantly associated with rPSC. rPSC was associated with over a 4-fold increase in the risk of death (HR=4.71, 95% CI 3.39, 6.56) with 1, 5, and 10-year graft survival rates of 98%, 84%, and 56% respectively compared to 95%, 88%, and 72% in patients who did not develop rPSC. CONCLUSION: The presence of UC post-liver transplant is associated with a significantly increased risk of rPSC. Furthermore, the presence of rPSC increases the rate of graft failure and death, with higher re-transplantation rates.

Periampullary carcinoma presenting as duodenojejunal intussusception: a diagnostic and therapeutic dilemma.

BACKGROUND: An intussusception is the invagination of one segment of the intestine into another. It is more common in children, but a rare clinical entity in adults, where the condition is almost always caused by tumors. METHODS: A 51-year-old female presented with symptoms of gastric outlet obstruction associated with significant weight loss, but no jaundice. Routine hematological and biochemical investigation, including tumor markers, were normal. Abdominal ultrasound revealed duodenojejunal intussusception, and subsequent CT of the abdomen confirmed it. RESULTS: She underwent a laparotomy, which confirmed duodenojejunal intussusception. On reducing the intussusception and performing a duodenotomy, a periampullary mass was confirmed. Hence, she underwent a pylorus-preserving pancreaticoduodenectomy. Histology confirmed periampullary adenocarcinoma. CONCLUSIONS: Adult intussusceptions are mostly caused by tumors. Contrast CT is the investigation of choice, although ultrasound can be used. One should have a low threshold for suspecting malignancy, obtain frozen section histology, and seek appropriate help at an early stage.

Chylous ascites after pancreatico-duodenectomy cholangiocarcinoma xenografts in nude mice.

BACKGROUND: Chylous ascites (CA) following pancreatico-duodenectomy (PD) is a rare complication secondary to disruption of the lymphatics during extended retroperitoneal lymph node dissection. The majority of cases do not develop CA, possibly due to patency of the proximal thoracic duct and good collaterals. CA may be due to a consequence of occult obstruction of the proximal thoracic duct by malignant infiltration or tumor embolus. This study was to report the incidence of CA and its outcomes of management. METHODS: A retrospective search of our liver database was performed using the key words "pancreatico-duodenectomy", "chylous ascites" from January 2000 to December 2005. The medical records of CA patients and their management and outcome were reviewed. RESULTS: In 138 patients who had undergone PD in our centre for pancreatic malignancy, 3 were identified with CA and managed by abdominal paracentesis. CA resolved in 2 patients with low fat medium chain triglyceride diet alone and 1 patient had total parenteral nutrition (TPN) for persistent CA. Resolution of CA occurred in these 3 patients at a median follow-up of 4 weeks (range 4-12 weeks). Histologically, resected specimen confirmed pancreatic adenocarcinoma in all the patients. Two patients developed loco-regional recurrences at a median follow up of 8 months (range 6-10 months). And the other was currently disease free at a 10-month follow up. CONCLUSIONS: CA as an uncommon postoperative complication requires frequent paracentesis, prolonged hospital stay, and delayed adjuvant chemotherapy. CA is treated with low fat medium chain triglyceride diet or occasionally TPN is required.

Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct: an unusual suspect?

BACKGROUND: Neuroendocrine tumors (NETs) arising in the biliary tree are extremely rare, and 37 cases were identified in the English literature. METHODS: A well-differentiated NET was found arising from the junction of the cystic and common hepatic ducts, in a 51-year-old male presenting with pedal edema and weight loss with abnormal liver enzymes and a normal serum bilirubin level. No mass was seen on radiological imaging and biopsy of the liver was suggestive of an early cholangiopathy. A bile leak complicating the liver biopsy led to an ERCP that demonstrated a filling defect suggestive of a mass in the common bile duct (CBD). RESULTS: He underwent a successful excision of the tumor with a Roux-en-Y hepaticojejunostomy. The diagnosis of NET was made on histological and immunohistochemical analysis of the resected specimen. He remains well and disease free 22 months after surgery. CONCLUSIONS: Recognition of biliary NET continues to be a challenge and an increased awareness of these tumors in rare sites will result in optimal management of these tumors.

Pseudoaneurysm following laparoscopic cholecystectomy.

BACKGROUND: Laparoscopic cholecystectomy (LC) is the operation of choice for removal of the gallbladder. Unrecognized bile duct injuries present with biliary peritonitis and systemic sepsis. Bile has been shown to cause damage to the vascular wall and therefore delay the healing of injured arteries leading to pseudoaneurysm formation. Failure to deal with bile leak and secondary infection may result in pseudoaneurysm formation. This study was to report the incidence and outcomes of pseudoaneurysm in patients with bile leak following LC referred to our hospital. METHODS: A retrospective analysis of our prospectively maintained liver database using key words pseudoaneurysm, bile leak and bile duct injury following laparoscopic cholecystectomy from January 2000 to December 2005 was performed. RESULTS: A total of 86 cases were referred with bile duct injury and bile leak following LC and of these, 4 patients (4.5%) developed hepatic artery pseudoaneurysm (HAP) presenting with haemobilia in 3 and massive intra-abdominal bleed in 1. Selective visceral angiography confirmed pseudoaneurysm of the right hepatic artery in 2 cases, cystic artery stump in one and an intact but ectatic hepatic artery with surgical clips closely applied to the right hepatic artery at the origin of the cystic artery in the fourth case. Effective hemostasis was achieved in 3 patients with coil embolization and the fourth patient required emergency laparotomy for severe bleeding and hemodynamic instability due to a ruptured right hepatic artery. Of the 3 patients treated with coil embolization, 2 developed late strictures of the common hepatic duct (CHD) requiring hepatico-jejunostomy and one developed a stricture of left hepatic duct. All the 4 patients are alive at a median follow up of 17 months (range 1 to 65) with normal liver function tests. CONCLUSIONS: HAP is a rare and potentially life-threatening complication of LC. Biloma and subsequent infection are reported to be associated with pseudoaneurysm formation. Late duct stricture is common either due to unrecognized injury at LC or secondary to ischemia after embolization.

Ruptured hepatocellular carcinoma following chemoembolization: a western experience.

BACKGROUND: Transcatheter arterial chemoembolization (TACE) is a recommended first line therapy for unresectable hepatocellular carcinoma (HCC). Serious complications such as neutropenic sepsis and hepatic decompensation are well known, but rupture of HCC following TACE is a rare and potentially fatal complication. The aim of this study was to identify the incidence of ruptured HCC following TACE and the associated risk factors. METHODS: A retrospective analysis was performed using our liver database with key words "chemoembolization", "ruptured HCC" covering the patients who received chemoembolization from January 1995 to December 2005. There were no exclusions. RESULTS: A total of 294 patients received chemoembolization in 530 sessions during the 10-year period. Of these, 2 ruptured following treatment (incidence 0.68%). The mean age was 65 years and the interval between the treatment and rupture was 2 and 24 days. The common factors were male sex, large tumor size (range 11-13 cm), and exophytic tumor growth. One patient died 2 days after rupture with hepatic decompensation while the second is alive after a 6-month follow up without tumor recurrence. CONCLUSIONS: Ruptured HCC following TACE is a rare but serious complication. Large tumor size, male sex, and exophytic growth of tumor may be predisposing factors for rupture.

Exposure of the thyroid to radiation during routine orthopaedic procedures.

The availability and usage of portable image intensifiers has revolutionised routine orthopaedic practice. Extensive use of fluoroscopy however may result into significant radiation exposure to operating staff. An accumulated dose of 65 microSv per procedure over long exposure has been reported to increase the risk of thyroid cancer. The present prospective study aimed at measuring the scattered dose to the thyroid using an Unfors EDD dosimeter during DHS/IMHS for fractures of the neck of the femur and IM nailing for long bone fractures. In 32 procedures, the dose of 65 microSv was exceeded 13 times; 8 times during DHS/IMHS and 5 times during IMN. The average thyroid dose was 142 microSv during IMN and 55 microSv during DHS. Only 9 of the total 223 (4%) theatre personnel were using a thyroid shield in spite of its availability. These results suggest that the thyroid is frequently exposed to potentially harmful radiation during these procedures. Strict inclusion of a thyroid shield as a part of routine radiation protection is recommended.

Pancreatic trauma--12-year experience from a tertiary center.

UNLABELLED: Pancreatic trauma is rare and often missed during initial assessment of patients with abdominal trauma. This study reviews our experience of managing pancreatic trauma at a tertiary referral center and discusses the diagnostic and therapeutic challenges. METHODS: A retrospective study of our prospectively maintained hepato-pancreatico-biliary database for 12 years preceding December 2007. RESULTS: Twenty-eight patients (23 males, 10 children) with a median age of 11.5 years (range, 6-16 years) in children and 27.5 years (range, 17-54 years) in adults were identified. Nineteen of the 28 had pancreatic duct injury of which 15 were missed on initial evaluation and referred after conservative management (n = 9) or laparotomy (n = 6). Twenty-one patients developed complications including abdominal collections (n = 10), pancreatic fistulae (n = 9), and pseudocysts (n = 2). There were 2 deaths (7%), both of which were associated with multiple intra-abdominal injuries. At a median follow-up of 7.5 months (range, 3-44 months), 19 of 23 patients were asymptomatic and had been discharged from follow-up. CONCLUSIONS: Pancreatic trauma in the United Kingdom is mainly the result of blunt trauma and most commonly affects young males. The presence of pancreatic duct disruption accounts for most of the complications, and in the absence of associated injuries, mortality is rare.

Spontaneous rupture of hepatocellular carcinoma: a Western experience.

BACKGROUND: Spontaneous rupture of hepatocellular carcinoma (HCC) is a life-threatening presentation, with an incidence of <3% of HCC patients in Western countries. The reported overall mortality is < or =50% in Asian countries, where the incidence is 12% to 14%. The aim of this study was to report a single center's experience of patients with ruptured HCC during a 11-year period. METHODS: A retrospective review was performed of all patients who presented with ruptured HCC between 1995 and 2005. Data on clinical features, treatment strategies, and survival outcomes were collected. Statistical methods included univariate analysis and Kaplan-Meier survival estimates with log-rank test. RESULTS: A cohort of 21 patients (15 male and 6 female) was identified. Fourteen (66.6%) patients had histologic evidence of underlying cirrhosis, ad the median age at presentation was 68 years (interquartile range [IQR] 61 to 69). Ten of these patients (71.4%) were hemodynamically unstable at presentation. The mean tumor size was 8.5 cm (range 3 to 13), and there was multifocal disease in 6 (42.8%) patients. The etiology of cirrhosis was hepatitis B infection in 3, hepatitis C in 3, alcohol in 4, and cryptogenic in 4 patients. Initial bleeding control was attempted by transarterial embolization (TAE) in 7 (50%) and by emergency surgery in 7 patients (50%). Four of the operations were performed at referring hospitals, and 3 were performed at our institution. Two patients (14.2%) underwent palliative treatment only. Definitive treatment included resection at emergency surgery in 1, staged hepatectomy in 1, and transarterial chemoembolization in 2 patients. There were 7 patients who were noncirrhotic and had a median age of 51 years (IQR 42 to 60). Of these, 6 (87.5%) were hemodynamically unstable at presentation. Mean tumor size was 9 cm (range 6 to 18) and confined to right lobe in all patients. Primary hemostasis was successfully achieved by TAE in 2 and perihepatic packing in 1 patient. Definitive treatment was provided by emergency hepatectomy in 4 and staged hepatectomy in 3 patients. Patients with cirrhosis (n = 14) had a median survival rate of <30 days. Child-Pugh score at presentation (median 7, IQR 5 to 8) correlated strongly with overall survival (P <.0001). Median survival for noncirrhotic patients was 20 months (IQR 2 to 31). One patient without cirrhosis survived for 122 months without disease recurrence. CONCLUSIONS: Spontaneous rupture of HCC is an uncommon presentation in Western countries. Primary hemostasis, followed by emergency or staged hepatic resection, is the treatment of choice. Median survival in patients initially treated with surgery was better than that observed in patients who underwent initial TAE, although this was not statistically significant. Patients who had no underlying liver disease had better prognosis than those who had cirrhosis.

Staged resection for a ruptured hepatoblastoma: a 6-year follow-up.

Hepatoblastoma (HB) is a rare germ cell tumour of childhood usually presenting with progressive abdominal distention. However, presentation as acute abdomen is a rare occurrence and is secondary to spontaneous rupture. This presentation carries high mortality. To our knowledge, six cases of ruptured hepatoblastoma have previously been reported, although the long-term outcome has not been clear. We report a case of ruptured HB who was managed by initial control of haemorrhage by laparotomy followed by chemotherapy with high-risk hepatoblastoma protocol as per SIOPEL 2 (cisplatin, carboplatin and doxorubicin) and a staged hepatectomy 5 months later. Patient is currently disease free at 6-year follow-up. Staged hepatectomy after initial control of haemorrhage does not preclude a curative resection.