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When a Jehovah’s Witness patient is traumatically injured, the lack of uniform professional consensus guidelines and the cultural knowledge deficit of many clinicians adds an additional layer of legal and ethical complexity to the inherent difficulty of managing a critically ill patient. We present here the case of an incapacitated Jehovah's Witness patient with severe traumatic hemorrhagic shock. We go on to discuss the historical and contemporary case law on proxy refusal of blood transfusion for the incapacitated adult, as well as the ethics of self-determination and the application of the principle of proportionate reason in the management of the critically injured Jehovah's Witness patient.

Background The novel coronavirus (COVID-19) strain has resulted in restrictions potentially impacting patients presenting with acute appendicitis and their disease burden. Methods All acute appendicitis admissions (281 patients) between 1/1/2018-4/30/2020 were reviewed. Two groups were created: 6 weeks before (Group A) and 6 weeks after (Group B) the date elective surgeries were postponed in Massachusetts for COVID-19. Acute appendicitis incidence and disease characteristics were compared between the groups. Similar time periods from 2018 to 2019 were also compared. Results Fifty-four appendicitis patients were categorized in Group A and thirty-seven in Group B. Those who underwent surgery were compared and revealed a 45.5% decrease (CI: 64.2,-26.7) in uncomplicated appendicitis, a 21.1% increase (CI:3.9,38.3) in perforated appendicitis and a 29% increase (CI:11.5,46.5) in gangrenous appendicitis. Significant differences in the incidence of uncomplicated and complicated appendicitis were also noted when comparing 2020 to previous years. Conclusions The significant increase in complicated appendicitis and simultaneous significant decrease in uncomplicated appendicitis during the COVID-19 pandemic indicate that patients are not seeking appropriate, timely surgical care., Highlights • The fear of contracting COVID-19 has affected healthcare access for patients. • Acute appendicitis patients had delayed presentation to the Emergency Room. • Delayed presentation resulted in decrease of uncomplicated, acute appendicitis. • Patients with complicated appendicitis significantly increased during the pandemic.

The response to the COVID-19 pandemic resulted in reallocation of health care resources and removal of barriers to deliver expedited care to those in need. This might be a unique moment in history to reconsider the regulations within our health care system that significantly increase its cost.

The novel coronavirus SARS-CoV-2 (COVID-19) strain has caused a pandemic that affects everyday clinical practice. Care of patients with acute surgical problems is adjusted to minimize exposing health care providers to this highly contagious virus. Our goal is to describe a specific and reproducible perioperative protocol aiming to keep health care providers safe and, simultaneously, not compromise standard of care for surgical patients.

Chemotherapeutic advances have enabled successful cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) expansion in treating metastatic colorectal cancer. The aims of this study were to evaluate the safety of combining liver surgery (LS) with HIPEC and CRS (which remains controversial) and its impact on overall survival (OS) rates. From 2007 to 2015, a total of 77 patients underwent CRS/HIPEC for peritoneal carcinomatosis (PC) of colorectal cancer. Twenty-five of these patients underwent concomitant LS for suspicion of liver metastases (LM; group 2), and were compared with patients who underwent CRS/HIPEC only (group 1). Demographic and clinical data were reviewed retrospectively. Among the group 2 patients, two underwent major hepatectomies, six underwent multiple wedge resections, 16 underwent single wedge resections (one with radiofrequency ablation), and one underwent radiofrequency ablation alone. For groups 1 and 2, median peritoneal cancer index was 6 and 10 (range 0–26; p = 0.08), complication rates were 15.4 and 32.0 % (Dindo–Clavien ≥3; p = 0.15), and median follow-up was 34.2 and 25.5 months (range 0–75 and 3–97), respectively. One group 2 patient died of septic shock after 66 days. Pathology confirmed LM in 21 patients in group 2 (four with benign hepatic lesions were excluded from long-term outcome analysis). Two-year OS rates were 89.5 and 70.2 % (p = 0.04), and 2-year recurrence-free survival rates were 38.3 and 13.4 % (p = 0.01) in groups 1 and 2, respectively. Simultaneous surgery for colorectal LM and PC is both feasible and safe, with low postoperative morbidity. Further longer-term studies would help determine its impact on patient survival.

Background Because of its known malignant potential, precise histologic diagnosis of intraductal papillary mucinous neoplasm of the pancreas (IPMN) during intraoperative pancreatoscopy (IOP) is essential for complete surgical resection. The impact of IOP on perioperative IPMN patient management was reviewed over 20 years of practice at Cliniques universitaires Saint-Luc, Brussels, Belgium. Study design Among 86 IPMN patients treated by pancreatectomy between 1991 and 2013, 21 patients had a dilated main pancreatic duct enabling IOP and were retrospectively reviewed. The IOP was performed using an ultrathin flexible endoscope and biopsy forceps, and specimens of all suspicious lesions underwent frozen section examination. Results Complete IOP with intraductal biopsies was easily and safely performed in 21 patients, revealing 8 occult IPMN lesions. In 5 cases (23.8%), initially planned surgical resection was modified secondary to IOP: 3 for carcinoma in situ and 2 for invasive carcinoma. The postoperative morbidity rate at 3 months was 25.0% (5 of 20); 1 patient died from septic shock postoperatively and was excluded. Median follow-up was 93 months (range 13 to 248 months). Nineteen of 21 patients were still alive and free of disease at last follow-up (90.5%); there was 1 patient with invasive carcinoma at initial pathology (pT3 N1) who died of pulmonary recurrence 21 months after surgery. Conclusions Intraoperative pancreatoscopy of the main pancreatic duct combined with intraductal biopsies plays a significant role in the surgical management of IPMN patients and should be used in all patients presenting a sufficiently dilated main pancreatic duct.

Splenectomy is the only potentially curative treatment for chronic immune thrombocytopenic purpura (ITP) in adults. However, one-third of the patients relapse without predictive factors identified. We evaluate the predictive value of the site of platelet sequestration on the response to splenectomy in patients with ITP. Eighty-two consecutive patients with ITP treated by splenectomy between 1992 and 2013 were retrospectively reviewed. Platelet sequestration site was studied by (111)Indium-oxinate-labeled platelets in 93% of patients. Response to splenectomy was defined at last follow-up as: complete response (CR) for platelet count (PC) ≥100 × 10(9)/L, response (R) for PC≥30 × 10(9)/L and100 × 10(9)/L with absence of bleeding, no response (NR) for PC30 × 10(3)/L or significant bleeding. Laparoscopic splenectomy was performed in 81 patients (conversion rate of 16%), and open approach in one patient. Median follow-up was 57 months (range, 1-235). Platelet sequestration study was performed in 93% of patients: 50 patients (61%) exhibited splenic sequestration, 9 (11%) hepatic sequestration and 14 patients (17%) mixed sequestration. CR was obtained in 72% of patients, R in 25% and NR in 4% (two with splenic sequestration, one with hepatic sequestration). Preoperative PC, age at diagnosis, hepatic sequestration and male gender were significant for predicting CR in univariate analysis, but only age (HR = 1.025 by one-year increase, 95% CI [1.004-1.047], p = 0.020) and pre-operative PC (HR = 0.112 for100 versus=100, 95% CI [0.025-0.493], p = 0.004) were significant predictors of recurrence-free survival in multivariate analysis. Response to splenectomy was independent of the site of platelet sequestration in patients with ITP. Pre-operative platelet sequestration study in these patients cannot be recommended.

Objective and importance:Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder, and liver involvement represents one of its major extra-renal manifestations. Although asymptomatic in most patients, polycystic liver disease (PLD) can lead to organ compression, severe disability and even become life-threatening, thereby warranting early recognition and appropriate management.Clinical presentation:We report the case of a 56-year-old woman with ADPKD and severe weight loss secondary to a giant hepatic cyst compressing the pylorus. Partial hepatectomy was required after failure of cyst aspiration and sclerotherapy, and patient's condition improved rapidly.Discussion and conclusions:We discuss the presentation and classification of compressing liver cysts, and the available therapeutic alternatives for this potentially severe complication of ADPKD.

Assessment of a simple layer peritoneal tube used as an autogenous inferior vena cava replacement. Extensive en-bloc multivisceral resection including major vessels is effective in selected abdominal malignancies, but the need for vascular reconstruction represents a surgical challenge. We describe the use of autologous peritoneum for caval replacement. Autogenous parietal peritoneum without fascial backing was harvested and tubularized to replace the inferior vena cava (IVC) in four patients with complex abdominal tumors. Surgical morbidity was evaluated using the Clavien–Dindo classification, and graft patency was systematically evaluated with ultrasound. All four patients had multiorgan resections for malignancies involving the retro-hepatic IVC, and they all required the replacement of infrarenal and suprarenal IVC segments. Additionally, all four required a right nephrectomy, two had a combined major hepatectomy, and one patient needed a veno-venous bypass. All had an R0 resection. A clinical follow-up took place between 5 and 11 months after surgery for each patient. Four-month graft patency was confirmed by ultra-sound and TDM with no sign of disease recurrence. Autologous peritoneum without fascial backing is a good and safe option for circumferential replacement of IVC after extensive en-bloc tumor resection with IVC involvement.

Background Organ shortage is the greatest challenge facing the field of organ transplantation today. Use of more organs of marginal quality has been advocated to address the shortage. Method We examined the pattern of donation and organ use in the United States as shown in the Organ Procurement and Transplantation Network/United Network for Organ Sharing database of individuals who were consented for and progressed to organ donation between January 2001 and December 2010. Results There were 66,421 living donors and 73,359 deceased donors, including 67,583 (92.1 %) identified as donation after brain death and 5,776 (7.9 %) as donation after circulatory death (DCD).Comparing two periods, era 1 (01/2001-12/2005) and era 2 (01/2006-12/2010), the number of deceased donors increased by 20.3 %from33,300 to 40,059while there was a trend for decreasing living donation. The DCD subgroup increased from 4.9 to 11.7 % comparing the two eras. A significant increase in cardiovascular/cerebrovascular disease as a cause of deathwas also noted, from38.1 %in era 1 to 56.1 %in era 2 (p

Background: Using SRTR/UNOS data, it has previously been shown that increased liver transplant centre volume improves graft and patient survival. In the current era of health care reform and pay for performance, the effects of centre volume on quality, utilization and cost are unknown. Methods: Using the UHC database (2009-2010), 63 liver transplant centres were identified that were organized into tertiles based on annual centre case volume and stratified by severity of illness (SOI). Utilization endpoints included hospital and intensive care unit (ICU) length of stay (LOS), cost and in-hospital mortality. Results: In all, 5130 transplants were identified. Mortality was improved at high volume centres (HVC) vs. low volume centres (LVC), 2.9 vs. 3.4%, respectively. HVC had a lower median LOS than LVC (9 vs. 10 days, P < 0.0001), shorter median ICU stay than LVC and medium volume centres (MVC) (2 vs. 3 and 3 days, respectively, P < 0.0001) and lower direct costs than LVC and MVC ($90 946 vs. $98 055 and $101 014, respectively, P < 0.0001); this effect persisted when adjusted for severity of illness. Conclusions: This UHC-based cohort shows that increased centre volume results in improved long-term post-liver transplant outcomes and more efficient use of hospital resources thereby lowering the cost. A better understanding of these mechanisms can lead to informed decisions and optimization of the pay for performance model in liver transplantation. © 2012 International Hepato-Pancreato-Biliary Association.

Given the complexity of solid organ transplantation, it is reasonable to believe that numerous factors are at play in achieving the enviable outcomes reported. The aim of this study is to examine the role of an organizational structure in maintaining the outcomes of a multiorgan transplant program at a nonacademic center. A retrospective analysis of 2378 solid organ transplants at Nazih Zuhdi Transplant Institute between March 1985 and December 2008 was performed. The 1-year and 3-year patient and graft survival rates, rate of retransplantation, and median length of hospital stay were compared with US national data released by the Scientific Registry of Transplant Recipients in January 2009. The 1-year patient survival rates were 87.5% for heart, 95.1% for kidney, 75.8% for lung, 89.6% for liver, and 100.0% for pancreas. The 3-year patient survival rates were 73.5% for heart, 89.7% for kidney, 57.8% for lung, 87.7% for liver, and 100.0% for pancreas. A well-structured transplant program along with competent medical, administrative, and ancillary support can achieve comparable patient and graft survival rates independent of volume.

Over the last decade the number of patients undergoing transplantation has increased. At the same time, effective peri- and postoperative care and better surgical techniques have resulted in greater numbers of recipients achieving long-term survival. Identification and effective management in the form of adequate treatment is essential, since any delay in diagnosis or treatment may result in graft loss or serious threat to patient's life. Various aspects of endoscopic findings that can be commonly encountered among liver transplant recipients are discussed herein. Topics include: persistent and/or recurrent esophageal varices, reflux, Candida or cytomegalovirus (CMV) esophagitis, esophageal neoplasms, posttransplant peptic ulcer, biliary complications, posttransplant lymphoproliferative disorder (PTLD), Kaposi's sarcoma, CMV colitis and inflammatory bowel disease, colonic neoplasms, Clostridium difficile infection, and graft versus host disease (GVHD).

Approximately 50%-60% of patients with colorectal cancers will develop liver lesions in their life span. Despite the potential of surgical resection to provide long-term survival in this subset of patients, only 15%-20% are found to be resectable. The introduction of new neoadjuvant chemotherapeutic agents and the expanding criteria of resection have enhanced the overall 5-year survival from 30% to 60% in the past decade. The use of technical innovations such as staged resection; portal vein embolization, and repeat resection have allowed higher resection rates in patients with bilobar disease. Extrahepatic primary and liver-exclusive recurrent disease no longer represent an absolute contraindication to resection. The role of regional therapy using hepatic arterial infusion is being redefined for liver-exclusive unresectable disease. Adjuvant chemotherapy in combination with regional therapies is being looked at from fresh perspectives. Ablative approaches have gained a firm role both as an adjunct to surgical resection and in the management of patients who are not surgical candidates. Overall, the management of hepatic metastasis from colorectal cancers requires a multimodal approach.

Biliary complications are still the major source of morbidity for liver transplant recipients. The reported incidence of biliary strictures is 5%-15% after deceased donor liver transplantation and 28%-32% after right-lobe live donor surgery. Presentation is usually within the first year, but the incidence is known to increase with longer follow-up. The anastomotic variant is due to technical factors, whereas the nonanastomotic form is due to immunological and ischemic events, which later may lead to graft loss. Endoscopic management of anastomotic strictures achieves a success rate of 70%-100%; it drops to 50%-75% for nonanastomotic strictures with a higher recurrence rate. Results of endoscopic maneuvers are disappointing for biliary strictures after live donor liver transplantation, and the success rate is 60%-75% for anastomotic strictures and 25%-33% for the nonanastomotic variant. Preventive strategies in the cadaveric donor include the standardization of the type of anastomosis and maintenance of a vascularized ductal stump. In right-lobe live donor livers, donor liver duct harvesting also involves a major risk. The concept of high hilar intrahepatic Glissonian dissection, dissecting the artery and the duct as one unit, use of microsurgical techniques for smaller ducts, use of ductoplasty, and flexibility in the performance of double ductal anastomosis are the critical components of the preventive strategies in the recipient. In the case of live donors, judicious use of intraoperative cholangiograms, minimal dissection of the hilar plate, and perpendicular transection of the duct constitute the underlying principals for obtaining a vascularized duct.

Preoperative, operative, and postoperative factors may all contribute to high rates of anemia in patients undergoing surgery for cancer. Allogeneic blood transfusion is associated with both infectious risks and noninfectious risks such as human errors, hemolytic reactions, transfusion-related acute lung injury, transfusion-associated graft-versus-host disease, and transfusion-related immune modulation. Blood transfusion may also be associated with increased risk of cancer recurrence. Blood-conservation measures such as preoperative autologous donation, acute normovolemic hemodilution, perioperative blood salvage, recombinant human erythropoietin (epoetin alfa), electrosurgical dissection, and minimally invasive surgical procedures may reduce the need for allogeneic blood transfusion in elective surgery. This review summarizes published evidence of the consequences of anemia and blood transfusion, the effects of blood storage, the infectious and noninfectious risks of blood transfusion, and the role of blood-conservation strategies for cancer patients who undergo surgery. The optimal blood-management strategy remains to be defined by additional clinical studies. Until that evidence becomes available, the clinical utility of blood conservation should be assessed for each patient individually as a component of preoperative planning in surgical oncology.

An intra-abdominal pseudotumor is a rare complication of hemophilia. Surgical treatment is associated with high morbidity and mortality rates and reported cases are scarce. We present a 66-year-old Caucasian male suffering from severe hemophilia type A treated for 10 years with Factor VIII. Major complications from the disease were chronic hepatitis B and C, cerebral hemorrhage and disabling arthropathy. Twenty-three years ago, retro-peritoneal bleeding led to the development of a large intra-abdominal pseudotumor, which was followed-up clinically due to the high surgical risk and the lack of clinical indication. The patient presented to the emergency department with severe sepsis and umbilical discharge that had appeared over the past two days. Abdominal computed tomography images were highly suggestive of a bowel fistula. The patient was taken to the operating room under continuous infusion of factor VIII. Surgical exploration revealed a large infected pseudotumor with severe intra-abdominal adhesions and a left colonic fistula. The pseudotumor was partially resected en bloc with the left colon leaving the posterior wall intact. The postoperative period was complicated by septic shock and a small bowel fistula that required reoperation. He was discharged on the 73(rd) hospital day and is well 8 mo after surgery. No bleeding complications were encountered and we consider surgery safe under factor VIII replacement therapy.

IMPORTANCE Choledochal cysts (CCs) are rare, with risk of infection and cancer. OBJECTIVE To characterize the natural history, management, and long-term implications of CC disease. DESIGN, SETTING, AND PARTICIPANTS A total of 394 patients who underwent resection of a CC between January 1, 1972, and April 11, 2014, were identified from an international multi-institutional database. Patients were followed up through September 27, 2014. Clinicopathologic characteristics, operative details, and outcome data were analyzed from May 1, 2014, to October 14, 2014. INTERVENTION Resection of CC. MAIN OUTCOMES AND MEASURES Management, morbidity, and overall survival. RESULTS Among 394 patients, there were 135 children (34.3%) and 318 women (80.7%). Adults were more likely to present with abdominal pain (71.8% vs 40.7%; P < .001) and children were more likely to have jaundice (31.9% vs 11.6%; P < .001). Preoperative interventions were more commonly performed in adults (64.5% vs 31.1%; P < .001), including endoscopic retrograde pancreatography (55.6% vs 27.4%; P < .001), percutaneous transhepatic cholangiography (17.4% vs 5.9%; P < .001), and endobiliary stenting (18.1% vs 4.4%; P < .001)). Type I CCs were more often seen in children vs adults (79.7% vs 64.9%; P = .003); type IV CCs predominated in the adult population (23.9% vs 12.0%; P = .006). Extrahepatic bile duct resection with hepaticoenterostomy was the most frequently performed procedure in both age groups (80.3%). Perioperative morbidity was higher in adults (35.1% vs 16.3%; P < .001). On pathologic examination, 10 patients (2.5%) had cholangiocarcinoma. After a median follow-up of 28 months, 5-year overall survival was 95.5%. On follow-up, 13 patients (3.3%), presented with biliary cancer. CONCLUSIONS AND RELEVANCE Presentation of CC varied between children and adults, and resection was associated with a degree of morbidity. Although concomitant cancer was uncommon, it occurred in 3.0% of the patients. Long-term surveillance is indicated given the possibility of future development of biliary cancer after CC resection.

Background Celiac axis invasion by central and distal pancreatic cancers has been considered a contraindication to resection. Appleby first described en-bloc celiac axis resection with total gastrectomy for locally advanced gastric cancer. We present our experience with a modification of this procedure in central pancreatic cancers involving the celiac trunk. Methods Three patients with central pancreatic cancers invading the celiac axis are reviewed. All patients underwent extended pancreatectomy with en-bloc resection of the celiac axis. Results Margins were grossly clear of tumor in all patients. The mean length of stay was 8.3 ± 1.1 days. There was no evidence of clinically significant gastric or hepatic ischemia. All 3 patients remain disease free at 34, 14, and 14 months from surgery, respectively. Comments Extended pancreatectomy with celiac axis resection can result in prolonged survival and should be considered in central and distal pancreatic cancers invading the celiac trunk.

The chronic shortage of deceased kidney donors has led to increased utilization of donation after cardiac death (DCD) kidneys, the majority of which are procured in a controlled setting. The objective of this study is to evaluate transplantation outcomes from uncontrolled DCD (uDCD) donors and evaluate their utility as a source of donor kidneys. From January 1995 to December 2004, 75,865 kidney-alone transplants from donation after brain death (DBD) donors and 2136 transplants from DCD donors were reported to the United Network for Organ Sharing. Among the DCD transplants, 1814 were from controlled and 216 from uncontrolled DCD donors. The log-rank test was used to compare survival curves. The incidence of delayed graft function in controlled DCD (cDCD) was 42% and in uDCD kidneys was 51%, compared to only 24% in kidneys from DBD donors (p < 0.001). The overall graft and patient survival of DCD donors was similar to that of DBD donor kidneys (p = 0.66; p = 0.88). Despite longer donor warm and cold ischemic times, overall graft and patient survival of uDCD donors was comparable to that of cDCD donors (p = 0.65, p = 0.99). Concerted efforts should be focused on procurement of uDCD donors, which can provide another source of quality deceased donor kidneys.

Summary Background Sirolimus is a potent immunosuppressive agent whose role in liver transplantation has not been well-described. Aim To evaluate the efficacy and side-effects of sirolimus-based immunosuppression in liver transplant patients. Methods Retrospective analysis of 185 patients who underwent orthotopic liver transplantation. Patients were divided into three groups: group SA, sirolimus alone (n = 28); group SC, sirolimus with calcineurin inhibitors (n =56) and group CNI, calcineurin inhibitors without sirolimus (n = 101). Results One-year patient and graft survival rates were 86.5% and 82.1% in group SA, 94.6% and 92.9% in group SC, and 83.2% and 75.2% in group CNI (P = N.S.). The rates of acute cellular rejection at 12 months were comparable among the three groups. At the time of transplantation, serum creatinine levels were significantly higher in group SA, but mean creatinine among the three groups at 1 month was similar. More patients in group SA required dialysis before orthotopic liver transplantation (group SA, 25%; group SC, 9%; group CNI, 5%; P = 0.008), but at 1 year, post-orthotopic liver transplantation dialysis rates were similar. Conclusions Sirolimus given alone or in conjunction with calcineurin inhibitors appears to be an effective primary immunosuppressant regimen for orthotopic liver transplantation patients. Further studies to evaluate the efficacy and side-effect profile of sirolimus in liver transplant patients are warranted.

Background Despite the risks associated with transfusion, the medical community continues to view blood as a safe and abundant product. In this article, we provide an effective strategy to accomplish orthotopic liver transplantation without transfusion. Study design From June 1999 through July 2004, 27 liver transplantations were performed in Jehovah's Witness patients at the USC-University Hospital (24 adults, 3 children). Nineteen of these were living donor (LD) and eight were deceased donor (DD) liver transplants. Preoperative blood augmentation with erythropoietin and iron was achieved. At induction, all LD and six of eight DD recipients underwent acute normovolemic hemodilution (ANH), and the operation was conducted under conditions of moderate anemia. Cell scavenging techniques were used. Acute normovolemic hemodilution and salvaged blood were returned as needed during bleeding or on completion of transplantation. Results The preoperative liver disease severity score was higher in the deceased donor group. We had 100% graft and patient survivals in the LD group, and 75% in the DD recipients. Two DD recipients died. The remaining are all alive and well, with a mean followup of 965 days (range 266 to 1,979 days) in the LD group and 624 days (range 119 to 1,132 days) in the DD group. Conclusions Preoperative blood augmentation and acute normovolemic hemodilution provide a safe cushion against operative blood loss. Elective living donor liver transplantation allows full implementation of a transfusion-free strategy in the setting of early hepatic failure, portal hypertension, and anemia. This feat is an important step toward global standardization of transfusion-free surgical practice and an important response to widespread blood shortages and transfusion risks.

Living donor liver transplantation has come to be an acceptable alternative to deceased donor transplants. Several ethical issues related to living donation have been raised in the face of reported perioperative morbidity and mortality. We report our experience in 13 consecutive Jehovah's Witness (JW) donor hepatectomies. From June 1999 to April 2004, 13 adult JW donors underwent donor hepatectomies at the USC-University Hospital. Nine donors underwent right lobectomy with a 62% mean volume of the liver resected. Four donors underwent a left lateral segmentectomy with a mean volume of 17.8%. Cell scavenging techniques, acute normovolemic hemodilution and fractionated products were used. The mean hospital stay was 6.2 days. All donors are alive and well at a median follow-up time of 3 years and 4 months. Live liver donation can be done safely in JW population if performed within a comprehensive bloodless surgery program.

Background The role of radiofrequency ablation (RFA) in the treatment of hepatic metastases from recurrent ovarian tumors is undefined. Case Three patients with hepatic lesions from recurrent ovarian cancers underwent a combined partial hepatectomy with radiofrequency ablation (RFA) to achieve optimal tumor cytoreduction. Follow-up radiological studies as well as serial tumor markers are consistent with disease-free survival after 39, 13, and 9 months. Conclusion These results demonstrate the feasibility and safety of RFA for metastatic ovarian lesions to the liver in patients previously deemed as poor or non-surgical candidates, and suggest the potential for improvement in survival over unresected patients or in patients resected with residual disease.

Indisputably, liver transplantation is among the most technically challenging operations in current practice and is compounded by significant coagulopathy and portal hypertension. Recombinant human coagulation factor VIIa (rFVIIa) is a new product that was initially described to treat bleeding in hemophilia patients. We present in this paper 10 liver transplants in Jehovah's Witness patients using this novel product at University of Southern California–University Hospital. The subject population included nine males and one female with an average age of 50 years. Six patients underwent cadaveric and four live donor liver transplantation. Surgeries were conducted following our established protocol for transfusion-free liver transplantation, which includes preoperative blood augmentation, intraoperative blood salvage, acute normovolemic hemodilution, and postoperative blood conservation. Factor rFVIIa was used at a dose of 80 μg/kg intravenously just prior to the incision in all patients, and a second intraoperative dose was used in 3 patients. All living donor liver transplantation (LDLT) recipients did well and were discharged uneventfully with normal liver functions. Two of the six cadaveric recipients died. One patient died intraoperatively from acute primary graft nonfunction, and the other died 38 hours postoperatively from severe anemia. This report suggests factor rFVIIa might have a much broader application in surgery in the control of bleeding associated with coagulopathy.

T t n the not-so-distant past, encountering the words “bloodless” and “liver transplant” in the same entence was rare. Also uncommon was seeing liver transplant” and “Jehovah’s Witness” toether—unless in reference to the refusal of a ehovah’s Witness (JW) to allow surgery involving lood transfusions. But today, thanks to advanced echnology and innovative physicians, “bloodless iver transplant” and “Jehovah’s Witness” are beoming associated more frequently. Countless ives have been saved because of advances in the loodless-transfusion process, and the medical alernatives for JWs have broadened amazingly ithin the past several years. In addition to the JWs, who will not have transusions because of religious beliefs, there is a much maller number of other patients who cannot have ransfusions (eg, those with antibodies). Also, ome patients fear the possible transmission of iseases via the transfusion process. Surgery, by its ery nature, involves dealing with the patient’s lood system. Surgical transplants, in turn, can esult in higher blood loss than surgery with less ntricate procedures. And liver transplants are mong the most costly of all in terms of lost blood. ontributing to the difficulty and high risk inolved in liver-transplant operations is the fact that ost patients eligible for a transplant are in exremely poor health by the time surgery begins ecause they have waited so long for a donor. The peration is a complex, time-consuming procedure

Liver transplantation has become mainstream surgical therapy for patient with end-stage liver disease with an operative mortality of less then 5% and 1- and 5-year survival rates 85% and 65%, respectively. 1-5 The disproportionate increase in patients awaiting liver transplant compared to the number of available organs 6,7 has led to the development of new techniques, including “living donor liver transplants” (LDLT), “split liver transplants,” and more recently, the reintroduction of “non-beating-heart liver donors.” 8 In the United States, the total number of liver transplants performed as of today is 58,507, of which 2,065 are from live donors and 56,442 are from cadavers, 9 which is a ratio of 1:27 live-donor to cadaveric liver transplants. The first successful living-donor liver transplantation was performed in 1989, with transplantation of a left hepatic lobe. Because of cultural beliefs discouraging cadaveric transplants in Asia, extensive development in the field of living-donor liver transplant was performed in the early 1990s; almost all liver transplantations performed in Asia involved living donors, whereas in the United States relatively few living-donor liver transplantations were performed until recently. It was mainly performed on children and used a left hepatic lobe (or one or more of its segments) from a parental donor. In the United States, adult lefthepatic-lobe transplantation was attempted in the 1990s, but without much success. The smaller left hepatic lobe provides insufficient hepatic mass for most adult Americans, who are physically larger than most Asians and consequently, the outcome of adult-to-adult left-hepatic-lobe transplantation in the United States was poor, and the procedure was seldom performed. 10 The first successful adult right LDLT was reported by Fan et al in 1997; 11 since then, right LDLT is the preferred adult LDLT.

As part of the spectrum of renal replacement therapies, kidney transplantation has been successful in providing the end-stage renal disease patient with a long-term, medically sound, and economically advantageous alternative to chronic dialysis. 1 The applicability of its success, however, has been limited by the ever-widening disparity between the steadily increasing number of eligible candidates and the relatively fixed number of available donors per year. This trend was partially offset in 2001, when the number of living kidney donors surpassed that of cadaveric donors. 2 In an effort to increase the availability of live donors, new techniques focused on facilitating the donation process through the use of laparoscopy. Adjunctive efforts were also directed toward overcoming previous immunologic barriers between donors and recipients, such as transplantation across “ABO incompatible” pairs. Renal insufficiency and an allocation policy change in the livertransplant recipient impact the supply of cadaveric donor kidneys and directly affect the renal healthcare worker; the perioperative management of these patients is discussed.

Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.

Objectives. To present a series of 5 patients with solitary metastatic renal cell carcinoma (RCC) to the pancreas after radical nephrectomy at our institution and review the published reports of this rare event. Methods. A retrospective review of the records of 5 patients with histologically confirmed RCC metastatic to the pancreas after radical nephrectomy was performed. A total of 5 patients (4 men, 1 woman) with a median age of 56 years (range 54 to 68) underwent radical nephrectomy for primary RCC. The pathologic stage was Robson I (n = 3) or Robson III (n = 2), with a left-sided tumor occurring in 3 patients and a right-sided tumor in 2 patients. The median interval from nephrectomy to the diagnosis of the pancreatic metastasis was 12 years (range 4 to 15). All patients were symptomatic at presentation, including weight loss (n = 3), abdominal pain (n = 3), early satiety (n = 1), steatorrhea (n = 1), and/or hemosuccus pancreaticus (n = 1). Results. All pancreatic metastases were hypervascular on imaging studies, and surgical removal was accomplished by pancreaticoduodenectomy (n = 3), partial pancreatectomy (n = 1), or subtotal pancreatectomy (n = 1). One patient died of disseminated disease 12 months after pancreatic resection. Two other patients had recurrences in the lung (n = 1) at 5 months or the pancreas/liver (n = 1) at 48 months. Both of these patients underwent a second resection and were disease free at 2 and 12 months afterward. The two remaining patients were disease free at 7 and 24 months after pancreatic resection. Conclusions. RCC is an unpredictable tumor that may demonstrate very late metastases even from early-stage lesions. Aggressive surgical management of isolated pancreatic lesions offers a chance of long-term survival.

Objective To evaluate the authors' experience with periduodenal perforations to define a systematic management approach. Summary background data Traditionally, traumatic and atraumatic duodenal perforations have been managed surgically; however, in the last decade, management has shifted toward a more selective approach. Some authors advocate routine nonsurgical management, but the reported death rate of medical treatment failures is almost 50%. Others advocate mandatory surgical exploration. Those who favor a selective approach have not elaborated distinct management guidelines. Methods A retrospective chart review at the authors' medical center from June 1993 to June 1998 identified 14 instances of periduodenal perforation related to endoscopic retrograde cholangiopancreatography (ERCP), a rate of 1.0%. Charts were reviewed for the following parameters: ERCP findings, clinical presentation of perforation, diagnostic methods, time to diagnosis, radiographic extent and location of duodenal leak, methods of management, surgical procedures, complications, length of stay, and outcome. Results Fourteen patients had a periduodenal perforation. Eight patients were initially managed conservatively. Five of the eight patients recovered without incident. Three patients failed nonsurgical management and required extensive procedures with long hospital stays and one death. Six patients were managed initially by surgery, with one death. Each injury was evaluated for location and radiographic extent of leak and classified into types I through IV. Conclusions Clinical and radiographic features of ERCP-related periduodenal perforations can be used to stratify patients into surgical or nonsurgical cohorts. A selective management scheme is proposed based on the features of each type.

To assess the treatment of peripancreatic fluid collections or abscess with percutaneous catheter drainage (PCD).Surgical intervention has been the mainstay of treatment for infected peripancreatic fluid collections and abscesses. Increasingly, PCD has been used, with mixed results reported in the literature.A retrospective chart review of 1993 to 1997 was performed on 82 patients at a tertiary care public teaching hospital who had computed tomography-guided aspiration for suspected infected pancreatic fluid collection or abscess. Culture results, need for subsequent surgical intervention, length of stay, and death rate were assessed.One hundred thirty-five aspirations were performed in 82 patients (57 male patients, 25 female patients) with a mean age of 40 years (range 17-68). The etiologies were alcohol (41), gallstones (32), and other (9). The mean number of Ranson's criteria was four (range 0-9). All patients received antibiotics. Forty-eight patients had evidence of pancreatic necrosis on computed tomography scan. Cultures were negative in 40 patients and positive in 42. Twenty-five of the 42 culture-positive patients had PCD as primary therapy, and 6 required subsequent surgery. Eleven patients had primary surgical therapy, and five required subsequent surgery. Six patients were treated with only antibiotics. The death rates were 12% for culture-positive patients and 8% for the entire 82 patients.Historically, patients with positive peripancreatic aspirate culture have required operation. This series reports an evolving strategy of reliance on catheter drainage. PCD should be considered as the initial therapy for culture-positive patients, with surgical intervention reserved for patients in whom treatment fails.

Wilson's disease is a hereditary defect in copper excretion leading to the accumulation of copper in the tissues, with subsequent tissue damage. The most serious sequela is that of progressive central nervous system involvement. The use of orthotopic liver transplantation (OLT) has been controversial for those patients with neurological symptoms attributed to Wilson's disease. The aim of this study is to determine the effectiveness of OLT for patients with Wilson's disease, including those with neurological involvement attributed to copper accumulation in the central nervous system. OLT was performed in 45 patients (19 men [42.2%], 26 women [57.8%]) with Wilson's disease between 1971 and 1993 who were followed up for at least 4 years. The age at diagnosis of Wilson's disease ranged from 3 to 41 years (mean, 17.7 +/- 7.4 years). The age at OLT ranged from 8 to 52 years (mean, 22.3 +/- 9.4 years). Nineteen patients (42.2%) were aged younger than 18 years at OLT. The indications for OLT included chronic hepatic failure in 15 patients (33.3%) and fulminant (FHF) or subfulminant hepatic failure in 30 patients (66. 6%). All but 1 of the 19 pediatric patients (94.7%) were in the latter group. Twenty-five patients (55.5%) were receiving D-penicillamine, 9 patients for more than 1 year; none of the patients treated long term presented as FHF. Thirty-three patients (73.3%) survived more than 5 years after OLT. Fourteen patients (31%) died during the posttransplantation period; 7 of the 14 patients (50%) were aged younger than 18 years. Twelve patients died during the first 3 months after OLT of complications of disease and surgery, 10 of whom underwent transplantation for FHF. The other 2 patients died 6 and 9 years after transplantation of infectious problems. Eleven patients (24.4%) required retransplantation because of a primary nonfunctioning graft (n = 6), chronic rejection (n = 4), and hepatic artery thrombosis (n = 1). Seventeen patients (37.7%) presented with neurological abnormalities; 14 patients with Wilsonian neurological manifestations and 3 patients with components of increased intracranial pressure. Ten of the 13 surviving patients with hepatic insufficiency and neurological abnormalities at OLT showed significant neurological improvement. Our experience shows OLT is a life-saving procedure in patients with end-stage Wilson's disease and is associated with excellent long-term survival. The neurological manifestation of the disease can improve significantly after OLT. Earlier transplantation in patients with an unsatisfactory response to medical treatment may prevent irreversible neurological deterioration and less satisfactory improvement after OLT.

Thrombocytopenia is frequently present in patients with cirrhosis. The effect of portal decompression on thrombocytopenia using a variety of shunt procedures has been contradictory. Transjugular intrahepatic portosystemic shunt (TIPS) has been proposed as a less invasive procedure for portal decompression, mainly for control of variceal bleeding or intractable ascites. Its effect on thrombocytopenia has not been defined yet. The aim of this review is to define the effect of TIPS on patients with cirrhosis and thrombocytopenia. Sixty-two patients who underwent TIPS at the University of Pittsburgh and survived without transplant for more than two months were included. Platelet count was determined prior to TIPS as well as at one-week, one-month, and three-month intervals after TIPS. The prevalence of thrombocytopenia prior to TIPS was 49%. TIPS had no effect on thrombocytopenia even when the portosystemic gradient was reduced to less than 12 mm Hg. In conclusion, portal decompression after TIPS did not affect the degree of thrombocytopenia.

In the setting of transplant medicine, decision making needs to take into account the multiple clinical and psychosocial case variables, rather than turn to arbitrary rules that cannot be scientifically supported

A quantitative competitive PCR (QC-PCR) assay for Epstein-Barr virus (EBV) has been developed to provide accurate measurement of EBV genome load in pediatric transplant recipients at risk for developing posttransplant lymphoproliferative disorder (PTLD). The assay quantifies between 8 and 5,000 copies of the EBV genome in 10(5) lymphocytes after a 30-cycle amplification reaction. For 14 pediatric patients diagnosed with PTLD, the median EBV genome load was 4,000, and 13 of the 14 patients had values of >500 copies per 10(5) lymphocytes. Only 3 of 12 control transplant recipients not diagnosed with PTLD had detectable viral genome loads (median value, 40). This median was calculated by using the highest value obtained by PCR testing on each of these patients posttransplantation. PCR values of >500 copies per 10(5) lymphocytes appear to correlate with a diagnosis of PTLD. By a modified protocol, the EBV genome copy number in latently infected adults was estimated to be

Congenital intestinal malrotation is a developmental anomaly resulting from interruption of the physiological herniation and return to the abdominal cavity of the midgut during the 6th to 10th week of embryological development. Normal vascular and anatomic relationships used as landmarks during pancreaticoduodenectomy (PD) are altered in patients with congenital malrotation. We present 3 cases of PD in adults with congenital intestinal rotation disorders. Three adult patients with congenital rotational disorders required PD. Two of these patients had bilio-pancreatic tumors, and 1 cadaveric donor underwent total pancreatectomy during pancreas allograft procurement. All patients had arterial and venous anomalies around the celiac trunk and mesenteric vessels, respectively. The midgut and hindgut in each case were shifted toward opposite sides of the abdominal cavity. Modifications to the standard approach to PD were made, and outcomes were favorable in each case. Each patient showed anatomic abnormalities with the need for identifying vascular structures through their expected (or projected) course and location before parenchymal division or ligation of any vessel. This approach becomes crucial in cases of vascular anomalies, such as ones occurring in congenital malformations, and can be used in similar situations encountered during pancreaticoduodenectomy.

Concomitant abdominal aortic aneurysms and cirrhosis that need surgical attention are rare. Currently there are no guidelines with regards to the appropriate timing of the repair of these aneurysms and transplantation. In addition it also raises the issue of which procedure takes precedence. With the advent of endovascular repairs, this issue was resolved with relative ease, by doing the orthotopic liver transplantation (OLT) first and subsequent endovascular stenting on post-operative day 7 during the same hospitalization. This is the first case report of stenting an abdominal aortic aneurysm (AAA) in a liver transplant recipient. The rationale for the OLT and then AAA repair are discussed and formal guidelines are offered.

Although liver biopsy is a very useful procedure used frequently in the diagnosis and management of liver dysfunction occurring after orthotopic liver transplantation, complications can occur with its use. An unusual complication of arterioportal fistula is reported here. Based upon this small series of an unusual event and the knowledge that the posttransplant liver may be more hypervascular than prior to OLTx and that it is uniquely susceptible to hepatic infarction and abscess formation, any attempt at fistula closure should be considered carefully prior to initiating the therapy (15). Unless a serious complication occurs [such as a transient biliary obstruction due to hemobilia as occurred in case 2, portal hypertension as also occurred in case 2, or systemic sepsis or other symptoms develop related directly to the fistula], simple observation may be the best choice of action. Should therapy be required, hepatic arterial embolization should be reserved for adults with intrahepatic fistulas. Primary surgical closure of intrahepatic fistula should be reserved for cases of extrahepatic fistula.

Idiopathic Non-Cirrhotic Portal Hypertension (INCPH) is a rare cause of portal hypertension. Its etiology is still unknown but its prognosis is excellent. Herein, we report a case of a 21 year old gentleman who was found to be thrombocytopenic on routine lab work before right anterior cruciate ligament repair. He had no other symptoms on initial presentation. He denied any history of GI bleeding, encephalopathy, ascites, jaundice, or abdominal distention. His past medical and family history were negative.On examination his blood pressure was 140/80, HR 52/min, and he was afebrile. He was non-icteric. His exam was benign except for soft a palpable spleen 2 cm below the costal margin. He was hemoccult negative. He had a creatinine of 1.12, Bilirubin of 1.4, AST of 26 and ALT of 56. His platelet count was 76,000, hematocrit 48.9, and hemoglobin 16.5. His INR was 1.1. Hepatitis B surface antibody was positive due to vaccination. HBS negative core antibody, Hepatitis A and C serologies were negative. His EBV serologies were negative. A bone marrow biopsy did not reveal any hematological cause of his thrombocytopenia. He also underwent a CT scan of the abdomen which showed splenomegaly, enlarged intra-abdominal varices, and Grade II esophageal varices which required banding. Liver ultrasound showed patent vasculature. He underwent a transjugular liver biopsy with hepatic vein and wedge pressure measurements as well as hepatic vein venograms. His venography showed a normal course, caliber and branching, his hepatic vein and wedge pressures were 12 and 21 mm Hg respectively with a corrected sinusoidal gradient of 8. His biopsy showed vascular abnormalities (megasinusoids) and irregularly distributed vessels without fibrosis associated with focal nodular regenerative change suggestive of INCPH (Figure 1). He underwent varicial banding. He is currently doing very well. Portal hypertension is a clinical syndrome defined by a portosystemic venous pressure gradient exceeding 5 mm Hg.[1] Liver cirrhosis is the most frequent cause of portal hypertension in the western hemisphere. However, in a variety of disorders, portal hypertension develops in the absence of cirrhosis. This condition is referred to as noncirrhotic portal hypertension. [1] The most common cause of noncirrhotic portal hypertension worldwide is schistosomiasis. [2] In the Western world, chronic liver diseases, such as nonalcoholic steatohepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and congenital hepatic fibrosis together with extrahepatic portal vein thrombosis and Budd-Chiari syndrome are common causes of noncirrhotic portal hypertension. Chronic arsenic toxicity is also one of the causes of non cirrhotic portal fibrosis [3]. If all these conditions have been ruled out, the diagnosis of INCPH can be made.[4-6] In the Western world INCPH might be responsible for 3%-5% of cases of portal hypertension.

Ciliated hepatic foregut cyst is a rare foregut cystic developmental malformation. It presents as a solitary cystic lesion in segment four of the liver. Histologically, it consists of four distinct layers; namely, the inner ciliate columnar epithelium, subepithelial connective tissue, smooth muscle layer, and an outer fibrous layer. Usually asymptomatic and detected incidentally, other modes of presentation can include portal hypertension, obstructive jaundice, and development of malignancy. We present a case of a young asymptomatic woman with a complex cyst in segment four of the liver, who underwent a laparoscopic resection, focusing our discussion on the review of the literature and the diagnostic dilemma encountered in these rare cases.