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.

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.

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.

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.

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 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.

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.

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.

Subtotal cholecystectomy: for the difficult gallbladder in portal hypertension and cholecystitis.

An easy, safe, and definitive operation for the "difficult gallbladder" is described and has been termed subtotal cholecystectomy. Eighteen patients underwent subtotal cholecystectomy during a 30-month period, which constitutes approximately 7% of cholecystectomies performed at our institution. The indications were cholecystitis with severe fibrosis or inflammatory changes that prevented safe dissection in Calot's triangle in 11 patients and portal hypertension in seven patients (liver cirrhosis [two patients] and segmental portal hypertension caused by chronic pancreatitis [five patients]) to prevent massive blood loss from the gallbladder bed. The operation entails leaving the posterior wall of the gallbladder attached to the liver and securing the cystic duct at its origin from within the gallbladder with a purse string technique. The latter obviates the need for dangerous dissection in Calot's triangle. Control of bleeding from the remaining gallbladder edge is greatly facilitated by the use of a running suture after each stage of piecemeal excision of the gallbladder. All patients survived the operation and wound infection occurred in only two patients (11%). One patient required a laparotomy 1 month after surgery for adhesive small bowel obstruction related to the remaining gallbladder wall and site of a liver biopsy. No patients have so far developed postcholecystectomy symptoms (median follow-up 12.2 months; range 3 to 31 months). Subtotal cholecystectomy is a definitive operation that prevents recurrent gallstone formation, as no residual diseased gallbladder mucosa is left in continuity with the biliary system. It provides a simple, safe option in patients in whom cholecystectomy could be hazardous.

The role of U tube palliative treatment in high bile duct carcinoma.

Twenty-one patients with cholangiocarcinoma at the confluence of the main right and left hepatic ducts were referred to our professorial surgical unit between 1968 and 1982. All were evaluated, treated, and documented prospectively with follow-up to mid 1986. No lesion was deemed resectable. The U tube palliative bypass developed during the course of the study was used in 14 patients, and its role in treating high bile duct carcinoma was evaluated. Histologic confirmation of the diagnosis was obtained in 71% of patients. Seven patients received additional treatment with radical radiotherapy. The 30-day overall hospital mortality rate was 19%. The 1- and 2-year survival rates were 57% and 33%, respectively. The quality of survival was usually good. The need for centralized referral and treatment of these difficult patients is stressed. The case against radical resection for this lesion is presented. It is concluded that radical resection is seldom possible, and therefore the U tube palliative procedure is advocated in most patients.

Incidence and management of complications after injection sclerotherapy: a ten-year prospective evaluation.

The incidence and management of complications of injection sclerotherapy are reviewed in 304 consecutive patients with esophageal varices followed up prospectively for a 10-year period. The 304 patients were injected on 1336 occasions. Three hundred eleven local esophageal complications occurred in 140 patients (complication rate, 23% per injection and 46% per patient). Esophageal mucosal slough, which was diagnosed by endoscopy, occurred on 250 occasions in 126 patients but did not require specific treatment. An injection site leak occurred in 25 patients, was managed conservatively, and was associated with a mortality rate of 28%. Stenosis of the esophagus was found in 32 patients, but only five patients required dilatation for relief of symptoms. Rupture of the esophagus occurred in four patients, three of whom had surgical treatment, and was associated with a mortality rate of 50%. Serious complications were more frequent with the rigid esophagoscope. An injection site leak occurred more frequently after acute sclerotherapy via the rigid esophagoscope. All four patients with rupture of the esophagus were injected electively via the rigid esophagoscope. Although the incidence of serious complications after injection sclerotherapy in this series appears acceptable, complications have been noted to be cumulative with time.

Giant hepatic hemangiomas: diagnostic and therapeutic dilemmas.

This report describes four cases of surgically treated giant hepatic hemangiomas which illustrate some diagnostic and therapeutic difficulties encountered in the management of this condition. An important diagnostic triad has emerged, which should alert the physician to the possibility of a complicated hepatic hemangioma: the clinical signs of an acute inflammatory liver process contrasted with a normal white blood cell count and liver function tests. Hemangiomas of the left lobe were either missed or poorly demonstrated on selective hepatic angiographic examination, and in two patients the diagnosis was made only at the time of laparotomy. Hepatic resection was successfully performed in all patients; there was minimal morbidity and none of the patients died. In two patients with multiple hemangiomas, only symptomatic or easily resectable lesions were removed. All patients are alive and well; three have been followed up for more than 5 years. We conclude that resection in asymptomatic cases should be carried out only in those cases that require a diagnostic laparotomy and in those where the lesion is easily resectable. The majority of patients with symptomatic and complicated tumors should undergo resection, but even in these patients continued conservative treatment is appropriate when the risk of major resection outweighs the small risk of live-threatening bleeding.

Simplified hepatic resection with the use of prolonged vascular inflow occlusion.

Ten consecutive patients scheduled to undergo liver resection were studied prospectively with the use of a standard protocol, which included routine vascular inflow occlusion to reduce blood loss and blood transfusion requirements. Fibrin sealant was sprayed on the raw liver surface, and abdominal drainage was not performed. No deaths occurred, and the postoperative course was remarkably smooth. The normothermic liver ischemic times of 30 to 122 minutes (mean, 73 minutes) were well tolerated. The amount of blood transfused was reduced to a mean of 2 U (range, 0 to 4 U). The occurrence of infected intraabdominal bile collections in two patients with preexisting biliary tract infection suggested that abdominal drainage should be performed in such patients. Vascular inflow occlusion is recommended for all liver resections.

Variceal rebleeding and recurrence after endoscopic injection sclerotherapy: a prospective evaluation in 204 patients.

HYPOTHESIS: Eradication of esophageal varices by repeated injection sclerotherapy and maintenance of eradication using continued surveillance endoscopy may reduce recurrent variceal bleeding and death from esophageal varices. DESIGN: A prospective study of consecutive adult patients with endoscopically proved esophageal variceal bleeding. SETTING: A tertiary care university hospital in a metropolitan area. PATIENTS: Two hundred four patients (127 men and 77 women; mean age, 50.1 years; age range, 16-82 years) underwent 993 emergency and elective variceal endoscopic injection treatments with 5% ethanolamine oleate during 1992 endoscopy sessions. Most (166 [81.4%]) had cirrhosis, mainly due to alcohol abuse (131 [78. 9%]). The number of patients with each modified Pugh-Child risk grade was as follows: A, 30; B, 91; and C, 83. (The modified Pugh-Child classification comprises ascites, encephalopathy, serum albumin and bilirubin levels, and prothrombin time. Each variable is given a value of 1 to 3 with increasing impairment of liver function. Addition of the values leads to the Pugh-Child risk grades for each patient, with 5 and 6 giving grade A; 7 through 9, grade B; and 10 through 15, grade C, respectively.) RESULTS: Ninety-five patients (46.6%) rebled at a median of 17 days (range, 0-2583 days). Seventy-four patients (36.3%) had a total of 112 further bleeding episodes before eradication of varices. Varices were eradicated in 99 (87.6%) of 113 patients who survived longer than 3 months after a median of 5 injections and remained eradicated in 43 (mean follow-up after eradication, 38 months; range, 4-125 months). Rebleeding was markedly reduced after eradication of varices. Varices recurred in 56 patients, of whom only 10 rebled from recurrent esophageal varices. Cumulative survival by life table analysis was 55%, 41%, and 30% at 1, 3, and 5 years, respectively. One hundred thirty-seven patients (67.2%) died during follow-up. Liver failure was the most common cause of death. Minor complications (mucosal ulceration) occurred in 105 patients. Major complications, including a localized injection site leak (n = 9), esophageal stenosis (n = 25), and esophageal perforation (n = 5), occurred in 39 patients. CONCLUSIONS: Repeated injection sclerotherapy eradicated esophageal varices in most long-term patients. Complications related to injection sclerotherapy were mostly minor. Complete eradication of varices reduced rebleeding and death from esophageal varices.

Surgery in South Africa.

The practice of surgery in South Africa ranges from full-time service in state-funded and academic hospitals serving a largely indigent population to a private sector for medically insured patients. Surgical training occurs at eight medical schools, and specialist registration is obtained after 4 to 5 years with either a university-conferred degree or a fellowship from the College of Surgeons of South Africa. The wide spectrum of First- to Third-World diseases and the high incidence of trauma provide comprehensive experience for practical training. Surgical standards are uniformly high, matching and sometimes pioneering the very best of Western medicine. The health care system is undergoing radical change to correct the imbalances of the apartheid era. Academic institutions are under pressure, and with incipient major financial cutbacks, there is concern that the proud record of service, teaching, and research excellence may be compromised. To facilitate the mission of broadening health care services, diploma training in surgery for rural practitioners is being developed. Outreach programs and closer liaisons with surgical societies in sub-Saharan African countries have also been initiated.

Primary sclerosing cholangitis. Requiem for biliary drainage operations?

OBJECTIVE: To review the outcome of a consecutive number of patients with primary sclerosing cholangitis (PSC) treated at one institution to define prognostic variables and determine the influence of surgery on outcome. DESIGN: Case series of patients with PSC seen in Cape Town, South Africa, between 1981 and 1991. SETTING: Tertiary referral center. PATIENTS: Thirty-six patients with PSC were studied. Diagnosis was based on cholangiographic findings of multiple strictures of the bile ducts together with compatible clinical and biochemical features. Thirty-two patients were followed up prospectively for up to 9 years. MAIN OUTCOME MEASURES: Patient outcome was defined as good (stable or slowly progressive disease) or poor (death or liver transplantation). RESULTS: During the follow-up period, seven patients with PSC died and two underwent liver transplantation. Actuarial survival at 5 years was 52%. An increased serum bilirubin concentration was the only variable at presentation that independently predicted a poor outcome. Cholangiography was unhelpful in predicting patient outcome. Six patients who developed obstructive jaundice associated with advanced liver disease underwent biliary drainage operations for surgically correctable strictures, but this did not seem to prevent progression of the disease. Two patients who progressed to end-stage liver disease went on to have liver transplantation and were alive with functioning grafts at 7 and 14 months, respectively. CONCLUSIONS: Symptomatic PSC is a progressive disorder with a poor prognosis. Our experience suggests that patients with advanced liver disease caused by PSC should be considered directly for liver transplantation rather than biliary bypass operations.

Choledochal cyst in pregnancy: a therapeutic dilemma.

BACKGROUND: Choledochal cysts occurring in pregnant women represent a diagnostic and therapeutic challenge to a broad spectrum of the medical profession. Not only is the association rare, but the clinical signs and symptoms are obscured by physiological changes that occur during pregnancy. As a result, diagnosis is often delayed until patients present with life-threatening complications. STUDY DESIGN: This report describes three cases of choledochal cysts occurring during pregnancy. RESULTS: Although the diagnosis was initially missed in two patients, delayed treatment was not associated with an adverse outcome. In a third patient, conservative management was complicated by rupture of the cyst which resulted in fetal loss and a protracted hospital course. Definitive cyst surgery resulted in a good long-term result in all three patients. CONCLUSIONS: Although choledochal cysts rarely occur in pregnancy, clinicians need to be aware of the condition, as delayed or inappropriate therapy may be catastrophic for both mother and child. Once the diagnosis is established, patients should be referred to specialized centers where treatment can be carefully planned, bearing in mind maternal and fetal well-being, as well as the likelihood of cyst-related complications both in the short- and long-term period. Excision with reconstruction is the procedure of choice to treat this type of cyst in nonpregnant patients. In pregnancy, however, a more conservative approach may have to be adopted until the second trimester or after delivery, when the surgical risk is lowest. Elective cesarean section should be undertaken in patients in whom the cyst has not been decompressed so as to avoid the complication of cystic rupture postpartum.

Operative management of papillary cystic neoplasms of the pancreas.

BACKGROUND: Papillary cystic neoplasm (PCN) is a rare malignant tumor of the pancreas that typically occurs in young females and has an excellent prognosis. STUDY DESIGN: We report a retrospective review of 12 patients treated during a 16-year period. Pre-, intra-, and postoperative data were evaluated in all patients to determine optimal management with specific reference to surgical strategy. RESULTS: All 12 tumors occurred in young women (mean age 22 years, range 14-36 years). Six patients presented with an epigastric mass, and three with severe abdominal pain. The correct diagnosis was made preoperatively in only five patients. Incorrect diagnoses included hepatoma, pancreatic pseudocyst, and hydatid cyst. The PCNs had a mean diameter of 12.5 cm (range 8-20 cm), and occurred in the head (four), neck (three), body (three), and tail (two) of the pancreas. All were resected. Operations performed were pylorus-preserving pancreaticoduodenectomy (three), central pancreatectomy with pancreaticogastrostomy (three), distal pancreatectomy (three), and local resection (three). In one patient two liver metastases were resected in addition to the pancreatic primary. One patient presented with tumor rupture and a major bleed into the lesser sac and died of multiple organ failure after resection. Postoperative complications included a stricture at the hepaticojejunostomy after pancreaticoduodenectomy, which resolved after temporary stenting, and a pancreatic duct fistula after local tumor resection, which required a distal pancreatectomy. Eleven patients are well at followup (mean 6.6 years; range 6 months to 15 years). CONCLUSIONS: PCN should be considered in the differential diagnosis of large pancreatic masses, especially in young females. Conservative resection, where technically feasible, is safe and effective and represents the therapy of choice.