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Background: The gastric cancer AJCC/UICC staging system recently underwent significant revisions, but studies on Asian patients have reported a lack of adequate discrimination between various consecutive stages. We sought to validate the new system on a U.S. population database., Methods: California Cancer Registry data linked to the Office of Statewide Health Planning and Development discharge abstracts were used to identify patients with gastric adenocarcinoma (esophagogastric junction and gastric cardia tumors excluded) who underwent curative-intent surgical resection in California from 2002 to 2006. AJCC/UICC stage was recalculated based on the latest seventh edition. Overall survival probabilities were calculated using the Kaplan-Meier method., Results: Of 1905 patients analyzed, 54 % were males with a median age of 70 years. Median number of pathologically examined lymph nodes was 12 (range, 1-90); 40 % of patients received adjuvant chemotherapy, and 31 % received adjuvant radiotherapy. The seventh edition AJCC/UICC system did not distinguish outcome adequately between stages IB and IIA (P = 0.40), or IIB and IIIA (P = 0.34). By merging stage II into 1 category and moving T2N1 to stage IB and T2N2, T1N3 to stage IIIA, we propose a new grouping system with improved discriminatory ability, Conclusions: In this first study validating the new seventh edition AJCC/UICC staging system for gastric cancer on a U.S. population with a relatively limited number of lymph nodes examined, we found stages IB and IIA, as well as IIB and IIIA to perform similarly. We propose a revised stage grouping for the AJCC/UICC staging system that better discriminates between outcomes.

The vast majority of choledochal cysts occur as either saccular or diffuse fusiform dilatation of the extrahepatic bile duct. We describe the complete resection of a rare single intrahepatic choledochal cyst communicating with the extrahepatic biliary tree. While previous reports describe partial resection with enteral drainage, we performed a complete resection of this rare choledochal cyst., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Hospital readmission has attracted attention from policymakers as a measure of quality and a target for cost reduction. The aim of the study was to evaluate the frequency and patterns of rehospitalization after a pancreaticoduodenectomy (PD)., Methods: The records of all patients undergoing a PD at an academic medical centre for malignant or benign diagnoses between January 2006 and September 2011 were retrospectively reviewed. The incidence, aetiology and predictors of subsequent readmission(s) were analysed., Results: Of 257 consecutive patients who underwent a PD, 50 (19.7%) were readmitted within 30 days from discharge. Both the presence of any post-operative complication (P = 0.049) and discharge to a nursing/rehabilitation facility or to home with health care services (P = 0.018) were associated with readmission. The most common reasons for readmission were diet intolerance (36.0%), pancreatic fistula/abscess (26.0%) and superficial wound infection (8.0%). Nine (18.0%) readmissions had lengths of stay of 2 days or less and in four of those (8.0%) diagnostic evaluation was eventually negative., Conclusion: Approximately one-fifth of patients require hospital readmission within 30 days of discharge after a PD. A small fraction of these readmissions are short (2 days or less) and may be preventable or manageable in the outpatient setting., (© 2012 International Hepato-Pancreato-Biliary Association.)

Objective: The study aimed to better define the epidemiology of idiopathic acute pancreatitis (IAP)., Methods: We identified admissions with primary diagnosis of acute pancreatitis (AP) in Nationwide Inpatient Sample between 1998 and 2007. Idiopathic AP was defined as all cases after excluding International Classification of Diseases, Ninth Revision, codes for other causes of AP (including biliary, alcoholic, trauma, iatrogenic, hyperparathyroidism, hyperlipidemia, etc)., Results: Among the primary admissions for AP, 26.9% had biliary pancreatitis, 25.1% alcoholic, and 36.5% idiopathic. Idiopathic AP had estimated 81,8025 admissions with a mean hospitalization of 5.6 days. Patients with IAP accounted for almost half of the fatalities among the cases of AP (48.2%) and had a higher mortality rate than both patients with biliary pancreatitis and patients with alcoholic pancreatitis (1.9%, 1.5%, and 1.0%, respectively, P < 0.01). Forty-six percent of patients with biliary pancreatitis underwent cholecystectomy during the index hospitalization, compared with 0.42% of patients with IAP. Patients with IAP had a demographic distribution similar to that of patients with biliary AP (female predominant and older), which was distinct from patients with alcoholic pancreatitis (male predominant and younger). There was a gradual but steady decrease in the incidence of IAP, from 41% in 1998 to 30% in 2007., Conclusions: Despite improving diagnostics, IAP remains a common clinical problem with a significant mortality. Standardization of the clinical management of these patients warrants further investigation.

Aim: To identify and describe changes in spleen volume occurring in patients with colorectal metastases to the liver after partial hepatectomy., Materials and Methods: Forty-one consecutive patients (20 men, 21 women) with histopathology-proven colorectal liver metastases who underwent partial hepatectomy between August 2007 and April 2011 were included. Liver and spleen volumes were measured by computed tomography (CT) volumetry on the most recent CT prior to surgery and on all CTs obtained within a year after partial hepatectomy. Patients were carefully evaluated for and excluded if they had co-morbid conditions known to cause splenomegaly or risk factors for portal hypertension such as underlying liver disease and portal vein thrombosis., Results: Thirty-two (78%) patients demonstrated an increase in spleen volume on the first post-operative CT, with more than a double increase in volume amongst five patients. Spleen volume increased by an average of 43% within 3 months of partial hepatectomy (p < 0.0001) and remained increased through 6 months after surgery, returning to near baseline thereafter. In the remaining nine (22%) patients, the spleen was observed to decrease an average of 11% in volume on first postoperative CT (p < 0.005)., Conclusions: Splenic enlargement after partial hepatectomy of colorectal metastases is a common finding on CT. Increased familiarity amongst radiologists of this phenomenon as likely reflecting physiological changes is important in order to avoid unnecessary evaluation for underlying conditions causing interval enlargement of the spleen., (Copyright © 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Aberrancy of the hepatic arterial anatomy is common. Because of its course directly adjacent to the head of the pancreas, a replaced right hepatic artery (RHA) is vulnerable to invasion by peri-pancreatic malignancies. Division of the RHA at the time of pancreaticoduodenectomy, however, may result in hepatic infarction and/or bilioenteric anastomotic complications. We report two cases of patients undergoing preoperative embolization of tumor encased replaced RHAs to allow for sufficient collateralization prior to pancreaticoduodenectomy., (Copyright © 2012 Wiley Periodicals, Inc.)

Hypothesis: Lymph node metastases decrease survival in patients with pancreatic neuroendocrine tumors (pNETs)., Design: Prospective database searches., Setting: National Institutes of Health (NIH) and Stanford University Hospital (SUH)., Patients: A total of 326 patients underwent surgical exploration for pNETs at the NIH (n = 216) and SUH (n = 110)., Main Outcome Measures: Overall survival, disease-related survival, and time to development of liver metastases., Results: Forty patients (12.3%) underwent enucleation and 305 (93.6%) underwent resection. Of the patients who underwent resection, 117 (35.9%) had partial pancreatectomy and 30 (9.2%) had a Whipple procedure. Forty-one patients also had liver resections, 21 had wedge resections, and 20 had lobectomies. Mean follow-up was 8.1 years (range, 0.3-28.6 years). The 10-year overall survival for patients with no metastases or lymph node metastases only was similar at 80%. As expected, patients with liver metastases had a significantly decreased 10-year survival of 30% (P < .001). The time to development of liver metastases was significantly reduced for patients with lymph node metastases alone compared with those with none (P < .001). For the NIH cohort with longer follow-up, disease-related survival was significantly different for those patients with no metastases, lymph node metastases alone, and liver metastases (P < .001). Extent of lymph node involvement in this subgroup showed that disease-related survival decreased as a function of the number of lymph nodes involved (P = .004)., Conclusions: As expected, liver metastases decrease survival of patients with pNETs. Patients with lymph node metastases alone have a shorter time to the development of liver metastases that is dependent on the number of lymph nodes involved. With sufficient long-term follow-up, lymph node metastases decrease disease-related survival. Careful evaluation of number and extent of lymph node involvement is warranted in all surgical procedures for pNETs.

Introduction: There are little data available regarding compliance with the National Comprehensive Cancer Network (NCCN) guidelines. We investigated variation in the management of pancreatic cancer (PC) among large hospitals in California, USA, specifically to evaluate whether compliance with NCCN guidelines correlates with patient outcomes., Methods: The California Cancer Registry was used to identify patients treated for PC from 2001 to 2006. Only hospitals with ≥ 400 beds were included to limit evaluation to centres possessing resources to provide multimodality care (n= 50). Risk-adjusted multivariable models evaluated predictors of adherence to stage-specific NCCN guidelines for PC and mortality., Results: In all, 3706 patients were treated for PC in large hospitals during the study period. Compliance with NCCN guidelines was only 34.5%. Patients were less likely to get recommended therapy with advanced age and low socioeconomic status (SES). Using multilevel analysis, controlling for patient factors (including demographics and comorbidities), hospital factors (e.g. size, academic affiliation and case volume), compliance with NCCN guidelines was associated with a reduced risk of mortality [odds ratio (OR) for death 0.64 (0.53-0.77, P < 0.0001)]., Conclusions: There is relatively poor overall compliance with the NCCN PC guidelines in California's large hospitals. Higher compliance rates are correlated with improved survival. Compliance is an important potential measure of the quality of care., (© 2012 International Hepato-Pancreato-Biliary Association.)

Background: Studies to identify preoperative prognostic variables for pancreatic neuroendocrine tumor (PNET) have been inconclusive. Specifically, the prevalence and prognostic significance of radiographic calcifications in these tumors remains unclear., Methods: From 1998 to 2009, a total of 110 patients with well-differentiated PNET underwent surgical resection at our institution. Synchronous liver metastases present in 31 patients (28%) were addressed surgically with curative intent. Patients with high-grade PNET were excluded. The presence of calcifications in the primary tumor on preoperative computed tomography was recorded and correlated with clinicopathologic variables and overall survival., Results: Calcifications were present in 16% of patients and were more common in gastrinomas and glucagonomas (50%), but never encountered in insulinomas. Calcified tumors were larger (median size 4.5 vs. 2.3 cm, P=0.04) and more commonly associated with lymph node metastasis (75 vs. 35%, P=0.01), synchronous liver metastasis (62 vs. 21%, P<0.01), and intermediate tumor grade (80 vs. 31%, P<0.01). On multivariate analysis of factors available preoperatively, calcifications (P=0.01) and size (P<0.01) remained independent predictors of lymph node metastasis. Overall survival after resection was significantly worse in the presence of synchronous liver metastasis (5-year, 64 vs. 86%, P=0.04), but not in the presence of radiographic calcifications., Conclusions: Calcifications on preoperative computed tomography correlate with intermediate grade and lymph node metastasis in well-differentiated PNET. This information is available preoperatively and supports the routine dissection of regional lymph nodes through formal pancreatectomy rather than enucleation in calcified PNET.

Because of technical complexity, concern for vascular control, and uncertainty in regard to oncologic outcome, the application of minimally invasive techniques to liver surgery has been slower than in most other abdominal procedures. This is despite well-known advantages with respect to postoperative pain, length of hospitalization, and recovery time. Although laparoscopic liver surgery has recently become more common, the majority of laparoscopic liver resections comprise anterolateral wedge resections and left lateral sectorectomies. Laparoscopic resections of the posterosuperior segments are more difficult and few reports are available in the literature. Compared to laparoscopy, gaining access to tumors in the dome of the liver may be more easily obtained via thoracoscopy, thereby preserving the benefits of minimally invasive surgery. This technical report describes two cases of hepatocellular carcinoma in segments VII and VIII resected via a video-assisted thoracoscopic transdiaphragmatic approach.

Purpose: To report the outcomes and toxicities in patients treated with intensity-modulated radiotherapy (IMRT) for pancreatic adenocarcinoma., Methods and Materials: Forty-seven patients with pancreatic adenocarcinoma were treated with IMRT between 2003 and 2008. Of these 47 patients, 29 were treated adjuvantly and 18 definitively. All received concurrent 5-fluorouracil chemotherapy. The treatment plans were optimized such that 95% of the planning target volume received the prescription dose. The median delivered dose for the adjuvant and definitive patients was 50.4 and 54.0 Gy, respectively., Results: The median age at diagnosis was 63.9 years. For adjuvant patients, the 1- and 2-year overall survival rate was 79% and 40%, respectively. The 1- and 2-year recurrence-free survival rate was 58% and 17%, respectively. The local-regional control rate at 1 and 2 years was 92% and 80%, respectively. For definitive patients, the 1-year overall survival, recurrence-free survival, and local-regional control rate was 24%, 16%, and 64%, respectively. Four patients developed Grade 3 or greater acute toxicity (9%) and four developed Grade 3 late toxicity (9%)., Conclusions: Survival for patients with pancreatic cancer remains poor. A small percentage of adjuvant patients have durable disease control, and with improved therapies, this proportion will increase. Systemic therapy offers the greatest opportunity. The present results have demonstrated that IMRT is well tolerated. Compared with those who received three-dimensional conformal radiotherapy in previously reported prospective clinical trials, patients with pancreatic adenocarcinoma treated with IMRT in our series had improved acute toxicity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Accurate tests to diagnose adenocarcinoma and high-grade dysplasia among mucinous pancreatic cysts are clinically needed. This study evaluated the diagnostic utility of amphiregulin (AREG) as a pancreatic cyst fluid biomarker to differentiate non-mucinous, benign mucinous, and malignant mucinous cysts., Methods: A single-center retrospective study to evaluate AREG levels in pancreatic cyst fluid by ELISA from 33 patients with a histological gold standard was performed., Results: Among the cyst fluid samples, the median (IQR) AREG levels for non-mucinous (n = 6), benign mucinous (n = 15), and cancerous cysts (n = 15) were 85 pg/ml (47-168), 63 pg/ml (30-847), and 986 pg/ml (417-3160), respectively. A significant difference between benign mucinous and malignant mucinous cysts was observed (p = 0.025). AREG levels greater than 300 pg/ml possessed a diagnostic accuracy for cancer or high-grade dysplasia of 78% (sensitivity 83%, specificity 73%)., Conclusion: Cyst fluid AREG levels are significantly higher in cancerous and high-grade dysplastic cysts compared to benign mucinous cysts. Thus AREG exhibits potential clinical utility in the evaluation of pancreatic cysts.

Objectives: To define current use of surgical therapies for hepatocellular carcinoma (HCC) and evaluate the correlation of various patient and hospital characteristics with the receipt of these interventions., Design: Retrospective cohort., Setting: California Cancer Registry data linked to the Office of Statewide Health Planning and Development patient discharge abstracts between 1996 and 2006., Patients: Patients with primary HCC., Main Outcome Measures: Receipt of liver transplant, hepatic resection, or local ablation., Results: Of 12,148 HCC cases, 2390 (20%) underwent surgical intervention. Three hundred eleven (2.56%) received a liver transplant, 1307 (10.8%) underwent resection, and 772 (6.35%) had local ablation. There were wide variations in treatment by race and hospital type. African American and Hispanic patients were less likely than white patients to undergo transplant (P < .05). African American and Hispanic patients were less likely than white and Asian/Pacific Islander patients to have hepatectomy or ablation (P < .05). In multivariable analysis, the apparent differences in surgical intervention by race/ethnicity were decreased when adjusting for the patients' socioeconomic and insurance statuses. Patients with lower socioeconomic status and no private insurance were less likely to receive any surgery (P < .01). Hospital characteristics also explained some variations. Disproportionate Share Hospitals and public, rural, and nonteaching hospitals were less likely to offer surgical treatment (P < .01)., Conclusion: There are significant racial, socioeconomic, and hospital-type disparities in surgical treatment of HCC.

Background: Surgery for pancreatic endocrine tumors (PETs) with blood vessel involvement is controversial., Hypothesis: Resection of PETs with major blood vessel involvement can be beneficial., Design: The combined databases of the National Institutes of Health and Stanford University hospitals were queried., Main Outcome Measures: Operation, pathologic condition, complications, and disease-free and overall survival., Results: Of 273 patients with PETs, 46 (17%) had preoperative computed tomography evidence of major vascular involvement. The mean size for the primary PET was 5.0 cm. The involved major vessel was as follows: portal vein (n = 20), superior mesenteric vein or superior mesenteric artery (n = 16), inferior vena cava (n = 4), splenic vein (n = 4), and heart (n = 2). Forty-two of 46 patients had a PET removed: 12 (27%) primary only, 30 (68%) with lymph nodes, and 18 (41%) with liver metastases. PETs were removed by either enucleation (n = 7) or resection (n = 35). Resections included distal or subtotal pancreatectomy in 23, Whipple in 10, and total in 2. Eighteen patients had concomitant liver resection: 10 wedge resection and 8 anatomic resections. Nine patients had vascular reconstruction: each had reconstruction of the superior mesenteric vein and portal vein, and 1 had concomitant reconstruction of the superior mesenteric artery. There were no deaths, but 12 patients had complications. Eighteen patients (41%) were immediately disease free, and 5 recurred with follow-up, leaving 13 (30%) disease-free long term. The 10-year overall survival was 60%. Functional tumors were associated with a better overall survival (P < .001), and liver metastases decreased overall survival (P < .001)., Conclusion: These findings suggest that surgical resection of PETs with vascular abutment/invasion and nodal or distant metastases is indicated.

Objective: To delineate the genetic and phenotypic features of Carney complex in a family with multiple cases of primary pigmented nodular adrenocortical disease (PPNAD)., Methods: Detailed clinical, laboratory, genetic, radiologic, and pathologic findings are presented, and the pertinent literature is reviewed., Results: A 17-year-old girl presented with symptoms and physical findings suggestive of hypercortisolemia, in addition to facial lentigines. She was found to have adrenocorticotropic hormone (ACTH)-independent Cushing syndrome. The adrenal glands appeared normal on computed tomographic scanning. Bilateral surgical adrenalectomy revealed PPNAD. Evaluation of her 14-year-old sister revealed ACTH-independent Cushing syndrome as well as facial lentigines, and adrenalectomy revealed PPNAD as well. Genetic testing of the 2 sisters and their mother (who also had multiple facial lentigines but did not have Cushing syndrome) revealed a novel mutation in the PRKAR1A gene., Conclusion: We describe a novel mutation in the PRKAR1A gene in a family with Carney complex and multiple members with PPNAD. PPNAD should be suspected in cases of ACTH-independent Cushing syndrome, and screening for Carney complex and its complications is recommended in all cases of PPNAD, including first-degree relatives.

Background: Clinical outcomes are increasingly subject to objective assessment and professional accountability. Informed consent relies on accurate estimation of operative risk. Current scoring systems for assessment of operative mortality after colorectal surgery (CRS) almost uniformly report 30-day mortality and may not represent true risk., Design: Prospective cohort., Setting: University-affiliated Veterans Affairs Medical Center., Patients: All patients who underwent resections of the colon and/or rectum (as the principal operation) at a single hospital whose data are captured in the Veterans Affairs National Surgical Quality Improvement Program (VA-NSQIP) database from January 1, 2000, through December 31, 2006., Main Outcome Measures: Mortality at 30 days and 90 days., Results: The VA-NSQIP cohort included 186 patients who underwent CRS, including 148 patients who underwent elective procedures (79.6%) and 38 patients who underwent emergency procedures (20.4%). All but 8 patients were men, with a median age of 67 years (range, 26-92 years). Laparoscopic operations comprised 24.2% and open operations comprised 75.8%. Most (60.8%) were performed for neoplasms. The actual 30-day mortality rates (all, elective, and emergency procedures) were 4.3%, 1.4%, and 15.8%, respectively. These rates closely mirrored the calculated VA-NSQIP risk-adjusted observed-to-expected ratio for 30-day mortality (4.8%, 1.8%, and 18.2%, respectively). However, mortality at 90 days increased substantially to 9.1%, 4.1%, and 28.9%, respectively., Conclusion: The 30-day mortality significantly underreports the true risk of death after CRS. The 90-day mortality rate should be included as a standard outcome measure after CRS because it serves as a better estimation of risk for counseling patients.

Laparoscopic cholecystectomy has all but replaced the traditional open approach. Hence open cholecystectomy (OC) is principally reserved for cases in which laparoscopy fails, leaving fewer surgeons with experience in the procedure required for the most challenging cases. This review of OC includes discussion of the indications for a primary open approach, conversion from laparoscopy, technical aspects of OC, and alternatives (cholecystostomy and subtotal cholecystectomy). Strategies for safe OC must be formally addressed in residency programs.

Background: Cystic pancreatic neoplasms (CPNs) present a unique challenge in preoperative diagnosis. We investigated the accuracy of diagnostic methods for CPN., Material and Methods: This retrospective cases series includes 70 patients who underwent surgery at a university hospital for presumed CPNs between 1997 and 2003, and for whom a definitive diagnosis was established. Variables examined included symptoms, preoperative work-up (including endoscopic retrograde cholangiopancreatography (ERCP) in 22 cases and endoscopic ultrasound (EUS) in 12), and operative and pathological findings. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) scans (n=50 patients; CT=48; MRI=13) were independently reviewed by two blinded GI radiologists., Results: The final histopathologic diagnoses were mucinous cystic neoplasm (n=13), mucinous cystadenocarcinoma (10), serous cystadenoma (11), IPMN (14), simple cyst (3), cystic neuroendocrine tumor (5), pseudocyst (4), and other (10). Overall, 25 of 70 were malignant (37%), 21 premalignant (30%), and 24 benign (34%). The attending surgeon's preoperative diagnosis was correct in 31% of cases, incorrect in 29%, non-specific "cystic tumor" in 27%, and "pseudocyst vs. neoplasm" in 11%. Eight had been previously managed as pseudocysts, and 3 pseudocysts were excised as presumed CPN. In review of the CT and MRI, a multivariate analysis of the morphologic features did not identify predictors of specific pathologic diagnoses. Both radiologists were accurate with their preferred (no. 1) diagnosis in <50% of cases. MRI demonstrated no additional utility beyond CT., Conclusions: The diagnosis of CPN remains challenging. Cross-sectional imaging methods do not reliably give an accurate preoperative diagnosis. Surgeons should continue to err on the side of resection.

Objective: The objective of our study was to determine the role and relative accuracy of CT and MRI in the characterization of cystic pancreatic masses., Materials and Methods: We retrospectively identified 58 patients with histopathologically proven cystic pancreatic masses at our institution who underwent preoperative CT (n = 40), MRI (n = 6), or both (n = 12). Two radiologists independently recorded their leading diagnoses with levels of diagnostic certainty (0-100%), their estimates of overall likelihood of malignancy (0-100%), and the morphologic characteristics of the tumors. Data were analyzed to determine relative accuracy in the diagnosis of malignancy, relationship between diagnostic certainty and accuracy, and frequency of malignancy in unilocular thin-walled cysts smaller than 4 cm., Results: Twenty-one (36%) of 58 masses were malignant. CT and MRI were equally accurate in establishing the diagnosis of malignancy (area under the receiver operating characteristic curve [A(z)] = 0.91 and 0.85 for reviewers 1 and 2 at MRI vs 0.82 and 0.76 at CT, respectively; p > 0.05). The leading diagnosis given by reviewers 1 and 2 was correct in 46% (32/70) and 43% (30/70) of the studies, respectively. When reviewer diagnostic certainty was 90% or more, the corresponding values were not significantly (p > 0.05) improved at 55% (12/22) and 48% (10/21), respectively. Two (15%) of 13 unilocular thin-walled cysts smaller than 4 cm were frankly malignant., Conclusion: CT and MRI are reasonably and similarly accurate in the characterization of cystic pancreatic masses as benign or malignant; limitations include a substantial rate of misdiagnosis even when reviewer certainty is high and a moderate frequency of malignancy in small morphologically benign-appearing cysts.

After portoenterostomy (PE) for biliary atresia (BA), many patients suffer progressive deterioration of liver function and ultimately require liver transplantation. We retrospectively reviewed a single center's experience with pediatric liver transplantation for BA from 1988 to 2002. Sixty-six patients underwent 69 liver transplants for BA. Forty-two (63%) patients had previously undergone Kasai PE, 11 (17%) biliary appendicoduodenostomy (BAD), and 13 (20%) had no prior biliary drainage (NBD). The BAD procedure offered only short-term biliary drainage--the mean interval between PE and transplant was more than twice that for Kasai patients than for BAD patients (132 versus 49 weeks). The transplants included 11 cadaveric partial, 27 cadaveric whole, and 31 living related transplants. Three patients required retransplant. Prior PE did not increase the incidence of major perioperative complications or unplanned reexploration. After transplant, the 1-, 5-, and 10-year actuarial graft survival rates were 87%, 86%, and 80%, respectively. The 1-, 5-, and 10-year actuarial patient survival rates were 91%, 89%, and 83%. PE remains an important bridge to transplant. In conclusion, transplantation for BA offers excellent long-term graft and patient survival.

Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date., Design: Retrospective case series and literature review., Setting: Tertiary care university hospital., Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004., Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome., Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct., Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.

Cystic pancreatic neoplasms are being diagnosed with growing frequency due to improving imaging technologies and increasing clinician awareness. Distinguishing cystic neoplasms from pseudocysts and discriminating among the various cystic neoplasms is essential to appropriate management. The backbone of diagnosis of these tumors continues to be cross-sectional imaging by CT and MRI. Despite refinements in technology and significant progress in characterizing these lesions, the overall accuracy of CT and MR is limited. EUS, especially as means of FNA, will have an increasing role in the evaluation of selected cases as experience grows. No radiologic investigation can reliably distinguish cystic neoplasms from pseudocysts nor differentiate among cystic neoplasms in all cases. For uncertain lesions, surgeons should favor either careful observation with serial imaging or surgical resection.

Despite important refinements of surgical technique and significant progress in perioperative care, esophageal cancer remains highly lethal. Therefore, hope for improvement in the prognosis of esophageal cancer lies largely in the use of additional therapy. Promising data from numerous Phase II trials and a single Phase III trial led to the widespread adoption of neoadjuvant chemoradiotherapy. However, subsequent randomized trials did not conclusively demonstrate a survival benefit with any of the current neoadjuvant protocols for patients with resectable esophageal cancer. Benefit, if any, exists only for complete pathologic responders. Neoadjuvant chemoradiation should not be used in patients with resectable esophageal cancer outside of the clinical trials. Future investigation must focus on the development of new biologic or chemotherapeutic agents, and the identification of biologic markers that might predict response to chemoradiation.

In selected patients with early rectal cancer, local therapy is an effective alternative to radical resection and offers minimal morbidity and the avoidance of a colostomy. Several techniques are described: transanal excision, dorsal approaches (York-Mason or Kraske procedures), transanal endoscopic microsurgery, endocavitary radiation, and transanal fulguration. Among these, transanal excision is favored for the low rate of complications, promising outcomes, and ability to secure tissue for pathology. Patients with T1 lesions with favorable histologic features may undergo local excision alone, while those with T2 lesions require adjuvant chemoradiation. The data currently available do not support the use of local therapy with curative intent for tumors that are advanced (T3 or T4), poorly differentiated, or have other negative pathologic characteristics. In carefully selected patients for local excision, local recurrence and survival rates are similar to traditional radical resection. Following local excision, patients require close observation for recurrence. Most patients with local recurrence can be salvaged by radical resection, though the long-term outcome is unknown.

Background/aims: Abdominal disorders occurring during pregnancy pose special difficulties in diagnosis and management to the obstetrician and surgeon. The advisability of nonobstetric abdominal surgery during pregnancy is uncertain. Our objective was to evaluate the safety and timing of abdominal surgery during pregnancy., Methods: We retrospectively reviewed 77 consecutive gravid patients undergoing nonobstetric abdominal surgery from 1989 to 1996 at an urban academic medical center and a large affiliated community teaching hospital. Medical records were evaluated for clinical presentation, perioperative management, preterm labor, and maternal and fetal morbidity and mortality., Results: The rate of nonobstetric abdominal surgery during pregnancy was 1 in every 527 births. Among the 77 patients, the indications for surgery were adnexal mass (42%), acute appendicitis (21%), gallstone disease (17%) and other (21%). There was no maternal or fetal loss or identifiable neonatal birth defect. Preterm labor occurred in 26% of the second-trimester patients and 82% of the third-trimester patients. Preterm labor was most common in patients with appendicitis and after adnexal surgery. Preterm delivery occurred in 16% of the patients, but appeared to be directly related to the abdominal surgery in only 5%., Conclusion: Surgery during the first or second trimester is not associated with significant preterm labor, fetal loss or risk of teratogenicity. Surgery during the third trimester is associated with preterm labor, but not fetal loss., (Copyright 2001 S. Karger AG, Basel)

Background: Symptomatic gallstones may be problematic during pregnancy. The advisability of laparoscopic cholecystectomy (LC) is uncertain. The objective of this study is to define the natural history of gallstone disease during pregnancy and evaluate the safety of LC during pregnancy., Methods: Review of medical records of all pregnant patients with gallstone disease at the University of California, San Francisco, from 1980 to 1996., Results: Of approximately 29,750 deliveries, 47 (0.16%) patients were treated for gallstone disease, including biliary colic in 33, acute cholecystitis in 12, and pancreatitis in two. Conservative treatment was attempted in all patients but failed in 17 (36%) cases. Two patients required combined preterm Cesarean-section cholecystectomy and 10 required surgery in the early postpartum period for persistent symptoms. Seventeen patients required cholecystectomy during pregnancy for biliary colic (10), acute cholecystitis (six), and pancreatitis (one). Three patients were treated with open cholecystectomy. Fourteen patients underwent LC at a mean gestational age of 18.6 weeks, mean OR time of 74 min, and mean length of stay of 1.2 days. Hasson cannulation was utilized in 11 patients. Reduced-pressure pneumoperitoneum (6-10 mmHg) was used in seven patients. Prophylactic tocolytics were used in seven patients, with transient postoperative preterm labor observed in one. There were no open conversions, preterm deliveries, fetal loss, teratogenicity, or maternal morbidity., Conclusions: In past years, symptomatic gallstones during pregnancy were managed conservatively or with open cholecystectomy. LC is a feasible and safe method for treating severely symptomatic patients.

In all normal tissues, cell proliferation and cell death are balanced. The physiology of normal cell death, which has become generally known as apoptosis or programmed cell death, has been intensely investigated in recent years. In this review the cell biology and biochemistry of apoptosis are discussed. Although apoptotic cells can be morphologically recognized, characteristic molecular features such as internucleosomal DNA fragmentation, and histochemical techniques such as in situ end labeling, facilitate the recognition of apoptosis. Many of the genes involved in the regulation of apoptosis, which include cell growth associated genes such as c-myc and p53, have been identified. It has become clear that the bcl-genes (more explicitly bcl-2 and bax) are important apoptosis regulators. The details of the mechanism of programmed cell death are, however, not completely unraveled. It has become clear that apoptosis plays an important role in organ and tissue development during embryogenesis. Examples are the morphogenesis of limbs from limb buds, the development of the central nervous system and the maturation of the hematopoietic and lymphatic systems. Hormonal regulation of cells and tissues is also partly executed through apoptosis. In a variety of disease apoptosis plays a role. In cancer, apoptosis is a crucial feature, and in the resolution of inflammatory reactions, apoptosis is essential. In neurodegenerative diseases, dysregulation of the cell death programme may play a role. Further elucidation of the role of apoptosis in these diseases may lead to new possibilities for treatment.