Resection of biliary mucinous cystic neoplasms of the liver: a prospective cohort series of 13 consecutive patients.

BACKGROUND: Biliary mucinous cystic neoplasms (BMCNs) are uncommon neoplastic septated intrahepatic cysts which are often incorrectly diagnosed and have the potential for malignant transformation. OBJECTIVE: To assess the outcome of surgical resection of BMCNs. METHOD: A prospective liver surgery database was used to identify patients who underwent surgery at Groote Schuur Hospital Complex for BMCN from 1999 to 2015. Demographic variables including age and gender were documented as well as detailed preoperative imaging, location and size, operative treatment, extent of resection, histology, postoperative complications and outcome. RESULTS: Thirteen female patients (median age 45 years) had surgery. Eleven were diagnosed by imaging for symptoms. Two were jaundiced. One cyst was found during an elective cholecystectomy. Five cysts were located centrally in the liver. Before referral three cysts were treated with percutaneous drainage and two were treated with operative deroofing. Six patients had anatomical liver resections and seven patients had non anatomical liver resections of which two needed ablation of residual cyst wall. One patient needed a biliary-enteric reconstruction to treat a fistula. Median operative time was 183 minutes (range: 130-375). No invasive carcinoma was found. There was no operative mortality. One surgical site infection and one intra-abdominal collection were treated. Two patients developed recurrent BMCN after 24 months. CONCLUSION: BMCNs should be considered in middle aged women who have well encapsulated multilocular liver cysts. Treatment of large central BMCNs adjacent to vascular and biliary structures may require technically complex liver resections and are best managed in a specialised hepato-pancreatico-biliary unit.

Resection of biliary mucinous cystic neoplasms of the liver: a prospective cohort series of 13 consecutive patients

Background: Biliary mucinous cystic neoplasms (BMCNs) are uncommon neoplastic septated intrahepatic cysts which are often incorrectly diagnosed and have the potential for malignant transformation.Objectives:To assess the outcome of surgical resection of BMCNs.Methods:A prospective liver surgery database was used to identify patients who underwent surgery at Groote Schuur Hospital Complex for BMCN from 1999 to 2015. Demographic variables including age and gender were documented as well as detailed preoperative imaging, location and size, operative treatment, extent of resection, histology, postoperative complications and outcome.Results:Thirteen female patients (median age 45 years) had surgery. Eleven were diagnosed by imaging for symptoms. Two were jaundiced. One cyst was found during an elective cholecystectomy. Five cysts were located centrally in the liver. Before referral three cysts were treated with percutaneous drainage and two were treated with operative deroofing. Six patients had anatomical liver resections and seven patients had non anatomical liver resections of which two needed ablation of residual cyst wall. One patient needed a biliary-enteric reconstruction to treat a fistula. Median operative time was 183 minutes (range: 130­375). No invasive carcinoma was found. There was no operative mortality. One surgical site infection and one intra-abdominal collection were treated. Two patients developed recurrent BMCN after 24 months.Conclusion:BMCNs should be considered in middle aged women who have well encapsulated multilocular liver cysts. Treatment of large central BMCNs adjacent to vascular and biliary structures may require technically complex liver resections and are best managed in a specialised hepato-pancreatico-biliary unit

Incidence and management of postoperative bile leaks: A prospective cohort analysis of 467 liver resections.

BACKGROUND: Bile leaks from the parenchymal transection margin are a major cause of morbidity following major liver resections. The aim of this study was to benchmark the incidence and identify the risk factors for postoperative bile leakage after hepatic resection. PATIENTS AND METHODS: A prospective database of 467 consecutive liver resections performed by the University of Cape Town HPB surgical unit between January 1990 and January 2016 was analysed. The relationship of demographic, clinical and perioperative factors to the development of bile leakage was determined. Bile leak and postoperative complications severity were graded using the International Study Group of Liver Surgery and Accordion classifications. RESULTS: Overall morbidity was 24% (n = 112), with bile leaks occurring in 25 (5.4%) patients. Significantly more bile leaks occurred in patients who had major resections (≥ 3 segments) and longer total operative times (p < 0.05). There were 5 Grade A bile leaks which stopped spontaneously. Seventeen Grade B leaks required a combination of percutaneous drainage (n = 15), endoscopic biliary stenting (n = 8) and percutaneous transhepatic biliary drainage (n = 3). All 3 Grade C leaks required laparotomy for definitive drainage. Median hospital stay in the 442 patients without a bile leak was 8 days (IQR 1-98) compared with 12 days (IQR 6-30) for the 25 with bile leaks (p < 0.05) with no mortality. Major resections (≥ 3 segments) and total operative time (> 180mins) were significantly associated with bile leaks. CONCLUSION: The incidence of bile leakage was 5.4% and occurred after major liver resections with longer operative times and resulted in significantly extended hospitalisation. Most were effectively treated nonoperatively by percutaneous drainage of the collection and/or endoscopic or percutaneous biliary drainage without mortality.

A cost analysis of operative repair of major laparoscopic bile duct injuries.

BACKGROUND: Major bile duct injuries occur infrequently after laparoscopic cholecystectomy, but may result in life-threatening complications. Few data exist on the financial implications of duct repair. This study calculated the costs of operative repair in a cohort of patients who underwent reconstruction of the bile duct after major ductal injury. OBJECTIVE: To calculate the total in-hospital cost of surgical repair of patients referred with major bile duct injuries. METHODS: A prospective database was reviewed to identify all patients referred to the University of Cape Town Private Academic Hospital, South Africa, between 2002 and 2013 for assessment and repair of major laparoscopic bile duct injuries. The detailed clinical records and billing information were evaluated to determine all costs from admission to discharge. Total costs for each patient were adjusted for inflation between the year of repair and 2013. Results. Forty-four patients (33 women, 11 men; median age 48 years, range 30 - 78) underwent reconstruction of a major bile duct injury. First-time repairs were performed at a median of 24.5 days (range 1 - 3,662) after initial surgery. Median hospital stay was 15 days (range 6 - 86). Mean cost of repair was ZAR215,711 (range ZAR68,764 - 980,830). Major contributors to cost were theatre expenses (22%), admission to intensive care (21%), radiology (17%) and specialist fees (12%). Admission to a general ward (10%), consumables (7%), pharmacy (5%), endoscopy (3%) and laboratory costs (3%) made up the balance. CONCLUSIONS: The cost of repair of a major laparoscopic bile duct injury is substantial owing to prolonged hospitalisation, complex surgicalintervention and intensive imaging requirements.

Solid pseudopapillary epithelial neoplasm--a rare but curable pancreatic tumour in young women.

BACKGROUND: Solid pseudopapillary epithelial neoplasms (SPENs) of the pancreas are rare but curable tumours that have a low-grade malignant potential and occur almost exclusively in young women, with an excellent prognosis after complete resection. This study examines the clinicopathological characteristics of these tumours and evaluates the role of surgery in relation to their size and location. STUDY DESIGN: We reviewed the pre-, intra- and postoperative data on 21 patients with SPENs who underwent resection during a 30-year period. Data including demographic information, presenting symptoms and signs, extent of operation, histology, tumour markers and postoperative complications were evaluated to establish the optimal surgical management. RESULTS: All 21 tumours occurred in women (mean age 24.6 years, range 13-51 years). Sixteen patients presented with nonspecific abdominal complaints and a palpable abdominal mass, in 1 patient the tumour was found during emergency laparotomy for a complicated ovarian cyst, 1 patient presented with severe abdominal pain and shock due to a ruptured tumour, and in 3 patients the tumour was detected incidentally during imaging. The correct pre-operative diagnosis of SPEN was made in 10 patients. Incorrect preoperative diagnoses included hydatid cyst (3 patients), mesenteric cyst (2), pancreatic cystadenoma (2), ovarian cysts (1), islet cell tumour of the pancreas (1), and cavernous haemangioma of the liver (1). The mean diameter of the tumours was 12.5 cm (range 8 - 20 cm), and they occurred in the head (8), neck (5), body (2), and tail (6) of the pancreas. All SPENs were resected. Five patients had a pylorus-preserving pancreaticoduodenectomy, 4 a central pancreatectomy with distal pancreaticogastrostomy, 8 a distal pancreatectomy, 3 a local resection and one a total pancreatectomy and portal vein graft. In 1 patient, 2 liver metastases were resected in addition to the pancreatic primary tumour. The patient who presented in shock with tumour rupture and bleeding into the lesser sac later died of multiple organ failure after successful resection. Postoperative complications included a stricture at the hepaticojejunostomy after pancreaticoduodenectomy, which resolved after stenting, and a pancreatic duct fistula after local tumour resection, which required a distal pancreatectomy. Other complications were bleeding (2 patients) requiring re-operation and intraabdominal fluid collections requiring percutaneous drainage (3) or operation (1). Mean postoperative hospital stay was 16 days (range 6 - 40 days). Twenty patients are alive and well without recurrence, including the patient with metastases, with a mean follow-up of 6.6 years (range 6 months-15 years). CONCLUSIONS: This study demonstrated that SPENs of the pancreas are uncommon, but should be considered in the differential diagnosis of a cystic mass of the pancreas in a young woman. Despite the indolent biological behaviour of SPENs, most patients required major pancreatic resection. Surgery is curative regardless of the size or location of the tumour. Metastases are rare, as is recurrence after complete surgical resection.

Self-expanding metal stents as an alternative to surgical bypass for malignant gastric outlet obstruction.

BACKGROUND: Gastroduodenal obstruction due to malignancy can be difficult to palliate. Self-expanding metal stents (SEMS) are gaining acceptance as an effective alternative to surgical bypass. METHODS: Patients not suitable for surgical bypass, with complete gastric outlet obstruction as a result of malignancy, were offered palliation with SEMS from November 2004 to December 2008. The procedure was performed under fluoroscopic guidance and conscious sedation. Data were collected prospectively. RESULTS: Seventy patients underwent SEMS placement (hepatobiliary and pancreatic malignancy, 44; antral gastric carcinoma, 19; other, seven). Follow-up was complete in 69 patients (99 per cent). Technical and clinical success rates were 93 and 95 per cent respectively. Median hospital stay was 2 (range 1-18) days, median survival was 1.8 (0.1-19.0) months, and 87 per cent had improved intake after SEMS placement, as determined by Gastric Outlet Obstruction Severity Score before and after stenting (P < 0.001). Complications included two episodes of minor bleeding. CONCLUSION: The use of SEMS to alleviate complete malignant gastric outlet obstruction in patients with limited life expectancy is successful in re-establishing enteral intake in most patients, with minimal morbidity, no mortality and a short hospital stay.

Guideline for the diagnosis and treatment of chronic pancreatitis.

BACKGROUND: Chronic pancreatitis (CP) is defined as a continuing inflammatory disease of the pancreas characterised by irreversible morphological changes, often associated with pain and with the loss of exocrine and/or endocrine function that may be clinically relevant. Alcohol is the predominant cause of CP in the western world and is particularly prevalent in South Africa, especially in the indigent patient. CP ranks high among intractable diseases of the gastrointestinal tract. The tendency for substance abuse in the alcohol-induced group poses major psychological and socio-economic problems. OBJECTIVE: CP is a disease with significant clinical and pathological heterogeneity. Level 1 evidence to support definitive guidelines for diagnosis, medical management and interventional therapy is lacking. Despite this paucity of robust scientific evidence, it is important to provide some assistance based on the best available evidence as to the current standard of care for CP in the South African context; this will aid all involved in the management of the disease, and includes clinicians, health care managers and funders. SCOPE: The guidelines were developed as recommendations addressing the diagnosis, medical management and interventions, both endoscopic and surgical, for the management of a very complex and heterogeneous disease of the pancreas. The recommendations are particularly relevant in the South African context where the predominant patho-aetiological agents are alcohol-associated with smoking. RECOMMENDATIONS: The guidelines provide clear recommendations regarding the diagnostic modalities available, both imaging (which includes MRI and endoscopic ultrasound (EUS)) and pancreatic function tests. The section on medical management makes recommendations on the use of analgesics, enzyme replacement and other therapeutic options in the non-interventional management of the majority of patients with CP. The section on interventional procedures identifies the indications and options available for the interventional management of both uncomplicated and complicated CP. The role of endoscopic and surgical modalities is defined, but it is in this context especially that the best available evidence, combined with the experience of the group, influenced the recommendations put forward. Owing to the lack of evidence and the complexity of the disease, it is recommended that, where possible, CP is managed in the context of a multidisciplinary team. VALIDATION: The guidelines are based on best practice principles determined by the available evidence and the opinions of the group, which comprised 7 medical and surgical gastroenterologists with significant experience in dealing with patients with chronic pancreatitis in the South African context. The group convened between May 2009 and August 2010 under the auspices of the Hepato-Pancreatico-Biliary Association of South Africa (HPBASA) and the South African Gastroenterology Society (SAGES), and the guidelines are the result of broad consensus within this group. The draft was presented to other experts in this field of endeavour to ensure broader participation and consensus. PLANS FOR GUIDELINE REVISION: HPBASA and SAGES will publish a revised modification of the recommendations when new levels 1 and 2 evidence data are published.

Early rebleeding and death at 6 weeks in alcoholic cirrhotic patients with acute variceal bleeding treated with emergency endoscopic injection sclerotherapy.

BACKGROUND: This study evaluated the incidence of rebleeding and death at 6 weeks after a first episode of acute variceal haemorrhage (AVH) treated by emergency endoscopic sclerotherapy in a large cohort of alcoholic cirrhotic patients. METHODS: From January 1984 to December 2006, 310 alcoholic cirrhotic patients (242 men, 68 women; mean age 51.7 years) with AVH underwent 786 endoscopic variceal injection treatments (342 emergency, 444 elective) during 919 endoscopy sessions in the first 6 weeks after the first variceal bleed. Endoscopic control of initial bleeding, variceal rebleeding and survival at 6 weeks were recorded. RESULTS: Endoscopic intervention controlled AVH in 304 of 310 patients (98.1%). Seventy-five patients (24.2%) rebled, 38 (12.3%) within 5 days and 37 (11.9%) within 6 weeks. No patient scored as Child-Pugh A died. Seventy-seven (24.8%) Child-Pugh B and C patients died, 29 (9.3%) within 5 days and 48 (15.4%) between 6 and 42 days. Mortality increased exponentially as the Child-Pugh score increased, reaching 80% when the score exceeded 13. CONCLUSION: Despite initial control of variceal haemorrhage, 1 in 4 patients (24.2%) rebled within 6 weeks. Survival at 6 weeks was 75.2% and was influenced by the severity of liver failure, with most deaths occurring in Child-Pugh grade C patients.

Clinical trial: intravenous pantoprazole vs. ranitidine for the prevention of peptic ulcer rebleeding: a multicentre, multinational, randomized trial.

BACKGROUND: Controlled pantoprazole data in peptic ulcer bleeding are few. AIM: To compare intravenous (IV) pantoprazole with IV ranitidine for bleeding ulcers. METHODS: After endoscopic haemostasis, 1256 patients were randomized to pantoprazole 80 mg+8 mg/h or ranitidine 50 mg+13 mg/h, both for 72 h. Patients underwent second-look endoscopy on day 3 or earlier, if clinically indicated. The primary endpoint was an overall outcome ordinal score: no rebleeding, rebleeding without/with subsequent haemostasis, surgery and mortality. The latter three events were also assessed separately and together. RESULTS: There were no between-group differences in overall outcome scores (pantoprazole vs. ranitidine: S0: 91.2 vs. 89.3%, S1: 1.5 vs. 2.5%, S2: 5.4 vs. 5.7%, S3: 1.7 vs. 2.1%, S4: 0.19 vs. 0.38%, P = 0.083), 72-h clinically detected rebleeding (2.9% [95% CI 1.7, 4.6] vs. 3.2% [95% CI 2.0, 4.9]), surgery (1.9% [95% CI 1.0, 3.4] vs. 2.1% [95% CI 1.1, 3.5]) or day-3 mortality (0.2% [95% CI 0, 0.09] vs. 0.3% [95% CI 0, 1.1]). Pantoprazole significantly decreased cumulative frequencies of events comprising the ordinal score in spurting lesions (13.9% [95% CI 6.6, 24.7] vs. 33.9% [95% CI 22.1, 47.4]; P = 0.01) and gastric ulcers (6.7% [95% CI 4, 10.4] vs. 14.3% [95% CI 10.3, 19.2], P = 0.006). CONCLUSIONS: Outcomes amongst pantoprazole and ranitidine-treated patients were similar; pantoprazole provided benefits in patients with arterial spurting and gastric ulcers.

Hydatid disease of the liver.

Echinococcus granulosus remains a clinical problem in sheep and subsistence farming communities in South Africa. The most commonly affected organs are the liver and the lung. Most cysts remain clinically silent and are diagnosed incidentally or when complications occur. Clinical examination is unreliable in making the diagnosis. Serological testing has a broad range of sensitivity and specificity and is dependent on the purity of the antigens utilised. Ultrasound examination of the abdomen is gens utilised. Ultrasound examination of the abdomen is both sensitive and cost effective. Computed tomography and endoscopic retrograde cholangiopancreatography (ERCP) are reserved for complicated cases. The differential diagnosis includes any cystic lesion of the liver. Liver hydatid cysts can be treated by medical or minimally invasive (laparoscopic and percutaneous) means or by conventional open surgery. The most effective chemotherapeutic agents against the parasite are the benzimidazole carbamates, albendazole and mebendazole. Albendazole is more efficacious, but recommended treatment regimens differ widely in terms of timing, length of treatment and dose. Medical treatment alone is not an effective and durable treatment option. PAIR (puncture, aspiration, injection, reaspiration) is the newest and most widely practised minimally invasive technique with encouraging results, but it requires considerable expertise. Open surgery remains the most accessible and widely practised method of treatment in South Africa. The options are either radical (pericystectomy and hepatic resection) or conservative (deroofing and management of the residual cavity). Various scolicidal agents are used intraoperatively (Eusol, hypertonic saline and others), although none have been tested in a formal randomised controlled trial. Laparoscopic surgery trials are small and unconvincing at present and should be limited to centres with expertise. Complicated cysts (intrabiliary rupture and secondary infection) may require ERCP to obtain biliary clearance before surgery, and referral to a specialist centre may be indicated.

Pancreatic pseudocysts.

Improvements in imaging studies and a better understanding of the natural history of pancreatic fluid collections (PFCs) have allowed the different types to be clarified. Stratification of PFCs into subgroups should help in selecting from the increasing current available treatment options, which include percutaneous, endoscopic and surgical drainage. Percutaneous catheter drainage is safe and effective and should be the treatment of choice in poor-risk patients, and for infected pseudocysts related to acute pancreatitis. Endoscopic drainage should be the first management option in suitable pseudocysts related to chronic pancreatitis, if the necessary expertise is available. The high success rate and current low morbidity of elective open surgery mean that it is still the standard of management in this disease. Laparoscopic approaches are gaining favour, predominantly in drainage of collections in the lesser sac, and long-term data are awaited. The precise application of this modality will need to be critically compared with the low morbidity of mini-laparotomy, which is the current standard after non-operative treatment fails in these patients. It is essential to clearly stratify the different types of pancreatic pseudocysts, in particular with relation to acute or chronic pancreatitis, and perform a valid comparison of the different treatment modalities within groups. In this capacity a precise and transparent classification may provide valuable answers, in particular relating to optimal management according to pseudocyst type.

Hydatid disease of the liver

Echinococcus granulosus remains a clinical problem in sheep and subsistence farming communities in South Africa. The most commonly affected organs are the liver and the lung. Most cysts remain clinically silent and are diagnosed incidentally or when complications occur. Clinical examination is unreliable in making the diagnosis. Serological testing has a broad range of sensitivity and specificity and is dependent on the purity of the antigens utilised. Ultrasound examination of the abdomen is both sensitive and cost effective. Computed tomography and endoscopic retrograde cholangiopancreatography (ERCP) are reserved for complicated cases. The differential diagnosis includes any cystic lesion of the liver. Liver hydatid cysts can be treated by medical or minimally invasive (laparoscopic and percutaneous) means or by conventional open surgery. The most effective chemotherapeutic agents against the parasite are the benzimidazole carbamates; albendazole and mebendazole. Albendazole is more efficacious; but recommended treatment regimens differ widely in terms of timing; length of treatment and dose. Medical treatment alone is not an effective and durable treatment option. PAIR (puncture; aspiration; injection; reaspiration) is the newest and most widely practised minimally invasive technique with encouraging results; but it requires considerable expertise. Open surgery remains the most accessible and widely practised method of treatment in South Africa. The options are either radical (pericystectomy and hepatic resection) or conservative (deroofing and management of the residual cavity). Various scolicidal agents are used intraoperatively (Eusol; hypertonic saline and others); although none have been tested in a formal randomised controlled trial. Laparoscopic surgery trials are small and unconvincing at present and should be limited to centres with expertise. Complicated cysts (intrabiliary rupture and secondary infection) may require ERCP to obtain biliary clearance before surgery; and referral to a specialist centre may be indicated

Primary hydatid cysts of the pancreas.

Pancreatic involvement by hydatid disease is uncommon. Establishing a precise diagnosis may be difficult because the presenting symptoms and findings of investigations may be similar to other more commonly encountered cystic lesions of the pancreas. We report 4 patients with primary hydatid cysts in the head of the pancreas. The records of all patients treated for hydatid disease from 1980 to 2000 were reviewed. During the study period a total of 280 patients were treated, 4 of whom had hydatid disease involving only the pancreas. The 4 patients (3 women, 1 man) ranged in age from 17 to 60 years. Three patients presented with jaundice, abdominal pain and weight loss, 2 with hepatomegaly and 1 with an epigastric mass. All 4 lesions involved the head of the pancreas and ranged in size from 3 to 10 cm in diameter. In 2 patients the investigations incorrectly suggested a cystic tumour and both underwent pancreaticoduodenectomy. In 2 patients the correct diagnosis allowed local excision to be performed. Hydatid cyst is a rare cause of a cystic mass in the head of the pancreas, but should be included in the differential diagnosis of cystic lesions of the pancreas, especially in endemic areas.

The evolving role of endoscopic treatment for bleeding esophageal varices.

The treatment of acute and recurrent variceal bleeding is best accomplished by a skilled, knowledgeable, and well-equipped team using a multidisciplinary integrated approach. Optimal management should provide the full spectrum of treatment options including pharmacologic therapy, endoscopic treatment, interventional radiologic procedures, surgical shunts, and liver transplantation. Endoscopic therapy with either band ligation or injection sclerotherapy is an integral component of the management of acute variceal bleeding and of the long-term treatment of patients after a variceal bleed. Variceal eradication with endoscopic ligation requires fewer endoscopic treatment sessions and causes substantially less esophageal complications than does injection sclerotherapy. Although the incidence of early gastrointestinal rebleeding is reduced by endoscopic ligation in most studies, there is no overall survival benefit relative to injection sclerotherapy. Simultaneous combined ligation and sclerotherapy confers no advantage over ligation alone. A sequential staged approach with initial endoscopic ligation followed by sclerotherapy when varices are small may prove to be the optimal method of reducing variceal recurrence. Overall, current data demonstrate clear advantages for using ligation in preference to sclerotherapy. Ligation should therefore be considered the endoscopic treatment of choice in the treatment of esophageal varices.

Primary hydatid cysts of the pancreas : general surgery

Pancreatic involvement by hydatid disease is uncommon. Establishing a precise diagnosis may be difficult because the presenting symptoms and findings of investigations may be similar to other more commonly encountered cystic lesions of the pancreas. We report 4 patients with primary hydatid cysts in the head of the pancreas. The records of all patients treated for hydatid disease from 1980 to 2000 were reviewed. During the study period a total of 280 patients were treated; 4 of whom had hydatid disease involving only the pancreas. The 4 patients (3 women; 1 man) ranged in age from 17 to 60 years. Three patients presented with jaundice; abdominal pain and weight loss; 2 with hepatomegaly and 1 with an epigastric mass. All 4 lesions involved the head of the pancreas and ranged in size from 3 to 10 cm in diameter. In 2 patients the investigations incorrectly suggested a cystic tumour and both underwent pancreaticoduodenectomy. In 2 patients the correct diagnosis allowed local excision to be performed. Hydatid cyst is a rare cause of a cystic mass in the head of the pancreas; but should be included in the differential diagnosis of cystic lesions of the pancreas; especially in endemic areas