Pylorus-preserving Total Pancreatectomy Research Articles

A Rare Case of Chronic Diarrhea in a Pediatric Patient

Background: VIPoma, also known as Watery Diarrhea-Hypokalemia- achlorhydria (WDHA) Syndrome is a rare manifestation of multiple endocrine neoplasia syndrome type 1 (MEN1). Vasoactive intestinal peptide, part of the secretin-glucagon family, may be overexcreted in tumors associated with MEN1 and results in diarrhea that persists while fasting, resulting in massive secretion of water and electrolytes. First-line treatment is surgical resection. Clinical Case: We present a 13-year-old male with a past medical history of chronic diarrhea for four years who was transferred from an outside hospital for severe diarrhea and associated electrolyte derangements, including hypokalemia of <1.0 mmol/L (3.5-5.0 mmol/L), sodium of 120 (135-145 mmol/L), and chloride of 84 mmol/L (101-110 mmol/L). Family history was significant for pancreatic, breast, thyroid, stomach, parathyroid, and uterine cancer, as well as hyperparathyroidism and nephrolithiasis. The patient had been admitted to the hospital before for a similar episode of acute on chronic diarrhea but was treated for infectious diarrhea during that admission. CT enterography was obtained during this hospitalization and it revealed multiple solid and heterogeneous appearing pancreatic masses in the head and tail of the pancreas. Work-up was significant for pancreatic polypeptide of 1,523 pg/mL (92-752 pg/mL) and vasoactive intestinal peptide of 1,105 pg/mL (<75 pg/mL). Pancreatic biopsy revealed a grade 2 pancreatic neuroendocrine tumor. Genetic testing revealed a known pathogenic mutation in the menin gene p.R526 (c. 1579>T, pArg527). A pylorus-preserving total pancreatectomy, duodenectomy, cholecystectomy, and splenectomy was performed and surgical pathology revealed a well-differentiated grade 1 neuroendocrine tumor in the head of the pancreas and a well-differentiated grade 2 neuroendocrine tumor in the tail of the pancreas. Since the surgery, the patient currently has no signs of other neuroendocrine tumors associated with MEN1 but continues to follow-up for regular screening for other tumors associated with MEN1. Clinical Lessons: 1. VIPoma, also known as Watery Diarrhea- Hypokalemia- Achlorhydria Syndrome, is characterized by secretory diarrhea that persists while fasting. 2. VIPoma should be considered in patients with a history of chronic diarrhea and a significant family history of neuroendocrine tumors.

Preservation of the Pyloric Ring Confers Little Benefit in Patients Undergoing Total Pancreatectomy

The clinical significance of preservation of the pyloric ring in total pancreatectomy (TP) has not been elucidated. A total of 48 consecutive patients underwent TP and were categorized into two groups based on the absence or presence of pylorus resection: the TP (N = 33) and pylorus-preserving TP (PPTP) (N = 15) groups. Preoperative patient background, intraoperative conditions, postoperative complications, and long-term nutritional status were retrospectively compared between the two groups. Patient background was similar between the groups, with the exception of the prevalence of preoperative diabetes mellitus (55 and 20 %, respectively; p = 0.021). There were no differences between groups with respect to operative times, blood loss, or blood transfusion. The PPTP group was more likely to have postoperative delayed gastric emptying than was the TP group (53 and 21 %, respectively; p = 0.029), and it tended to become increasingly severe. The length of the postoperative fasting period was significantly longer in the PPTP group than in the TP group (mean 15 ± standard deviation [SD] 10.8 and 9 ± 9.7 days, respectively; p = 0.023). The body weights in the TP group started to recover by 1 year postoperatively, whereas those in the PPTP group continued to decrease. Serum hemoglobin levels tended to be higher in the TP group than in the PPTP group at 1 year postoperatively. Preservation of the pyloric ring provided little or no benefit to short-term outcome or long-term nutritional status among patients who underwent TP.

Laparoscopic-Assisted Spleen-Preserving and Pylorus-Preserving Total Pancreatectomy for Main Duct Type Intraductal Papillary Mucinous Tumors of the Pancreas

Minimally invasive and function-preserving pancreatectomy would be the ideal approach for benign and borderline malignant tumors of the pancreas. Total pancreatectomy can be indicated for the main duct type of intraductal papillary mucin-producing tumor (IPMT) to achieve radical resection. Recently, several studies advocating total pancreatectomy in IPMT have been published, but they are all believed to be done by conventional laparotomy. Herein, we report a case of a 72-year-old female patient who successfully underwent laparoscopic-assisted total pancreatectomy with the spleen and pylorus preserved in borderline malignant main duct type IPMT. A marginal ulcer around the duodenojejunostomy was developed, but managed by a proton-pump inhibitor. She was discharged 20 days after surgery. She was followed for more than 2 years without evidence of tumor recurrence. Her blood sugar level was well controlled by insulin pump therapy and image study showed well-preserved spleen function.

Pylorus-preserving total pancreatectomy for an intraductal papillary-mucinous neoplasm of the pancreas

Total pancreatectomy (TP) is rarely performed to treat invasive ductal carcinoma of the pancreas, due to the associated markedly impaired quality of life and poor prognosis after the resection. Intraductal papillary-mucinous neoplasm (IPMN) of the pancreas is characterized by extensive intraductal spread and a favorable outcome even when presenting at an invasive stage. We herein reappraise the role of pylorus-preserving total pancreatectomy (PPTP) as a viable alternative pancreatic resection modality for borderline and malignant IPMN. A total of five patients with IPMN underwent PPTP and their clinical follow-up data were reviewed. TP was performed due to recurrent IPMN in the remnant pancreas after distal pancreatectomy in three patients and due to massive involvement of the entire pancreas in the others. All patients were treated by the pylorus-preserving method, while the spleen was also preserved in one patient. The surgical margins were negative and no metastasis to the resected lymph nodes was evident, based on histological examinations. One patient underwent a re-operation due to postoperative intraabdominal bleeding, while another patient required tubedrainage for left pleural effusion. Three of the four patients who underwent PPTP with a splenectomy experienced postoperative gastric ulcer, which were controlled by medication. One patient died due to suicide 16 months after the PPTP. All the others were doing well without recurrence at periods of 62 to 127 months after the PPTP. PPTP is therefore considered to be indicated as an effective treatment for borderline or malignant IPMN with extensive involvement, when the patient's condition permits, in order to achieve complete resection of the IPMN.

Management of a rare case of fulminant hemobilia due to arteriobiliary fistula following total pancreatectomy

Hemobilia is a rare cause of acute upper gastrointestinal bleeding and is often associated with a history of hepatic or biliary tract injury, tumor growth, hepatic artery aneurysm, cholecystitis, or hepatic abscess. We report a case of a 76-year-old patient with massive hemobilia due to intrahepatic bleeding from the segment 8 hepatic artery without evidence of a true aneurysm, abscess, or metastatic disease 4 weeks following pylorus-preserving total pancreatectomy for pancreatic cancer. Gastroduodenoscopy suggested hemorrhage from the duodenojejunostomy but failed to achieve hemostasis, and the patient underwent exploratory laparotomy. It was realized intraoperatively that the bleeding originated from the intrahepatic biliary tract. Bleeding was controlled by blocking the right hepatic bile duct with a Fogarty catheter and subsequent transarterial embolization. Computed tomography did not reveal any local liver or vascular pathology. Retrospectively, the cause of delayed profuse hemobilia was most likely a traumatic intrahepatic pseudoaneurysm following endoscopic bile duct stenting 3 weeks before the pancreatectomy. The reported case is exceptional and of particular interest because of the absence of a typical history or cause of hemobilia, preoperative misleading diagnostic results in an altered anatomic situation, and the operative management to achieve bleeding control in this emergency setting.

Pylorus-Preserving Total Pancreatectomy

Background/Aims: Preservation of the pylorus is an accepted alternative procedure to the classical Whipple operation for pancreatic head resection but data describing its value for total pancreatectomy are sparse. Methods: A prospective analysis of 22 total pancreatectomies performed in a consecutive series of 436 pancreatic resections from 1.11.93 to 1.5.99. Results: 11 patients underwent total pancreatectomy with preservation of the pylorus. Histopathological examination revealed pancreatic adenocarcinoma in 16 cases and duodenal adenocarcinoma in 1 patient, 5 patients had other types of pancreatic neoplasm. In-hospital mortality was 4.5% (n = 1), cumulative morbidity was 59% and reoperations were performed in 9.1% of cases (n = 2). Median follow-up was 37 months (range 5–66). 62% of patients (n = 13) developed tumor recurrence and 13 patients died during the follow-up period with 10 deaths being cancer related. There was no difference concerning postoperative and follow-up morbidity of survival between patients undergoing pylorus-preserving total pancreatectomy or pancreatectomy with gastrectomy. However, postoperative body weight was increased 3, 6, 9 and 12 months following preservation of the pylorus. Conclusion: Total pancreatectomy with preservation of the pylorus is a feasible type of resection for all types of pancreatic or ampullary tumors, which shows a similar morbidity and long-term survival but improved nutritional recovery compared with standard total pancreatectomy.

The risks of total pancreatectomy and splenic islet autotransplantation.

The intraportal site is the most common site for islet transplantation. Many other sites have been tried experimentally, including the spleen, which has successfully lead to insulin independence in a number of animal models. Nevertheless, there are no detailed reports of total pancreatectomy and splenic islet autotransplantation in humans. Five patients underwent total pancreatectomy and splenic islet autotransplantation for chronic pancreatitis. Four patients had a pylorus-preserving total pancreatectomy and one patient a duodenal-preserving pancreatectomy. In three cases islets were embolized into both the portal vein and spleen. Two patients received splenic islet transplants alone. Islets were transplanted by retrograde venous infusion via the short gastric veins (n = 3), splenic vein stump (n = 1), and the left gastroepiploic vein (n = 1). The total volumes of transplanted pancreatic digest in those receiving combined intraportal and splenic autografts (n = 3) were 15.8, 13.0, and 13.5 ml. The volumes in those receiving a splenic-alone autograft (n = 2) were 12.0 and 5 ml. The mean rise in portal pressure was 18 cm of water. Complications related to the splenic autograft included a wedge splenic infarct, an emergency splenectomy, and a portal vein thrombosis in one patient having a combined intraportal and splenic autograft. Two patients developed insulin independence. two patients were still insulin independent at 1-year follow-up, and all had normal HbA1c levels (mean 5.6, range 5.2-6.3). Splenic islet autotransplantation, after total pancreatectomy, does lead to insulin independence. However, in our experience the combined procedure has a high morbidity because of splenic infarction and venous thrombosis.

PYLORUS-PRESERVING TOTAL PANCREATECTOMY IN CHRONIC PANCREATITIS -A CASE REPORT-

A 62-year-old male who had undergone distal pancreatectomy and splenectomy for chronic pancreatitis 5 years ago was hosptialized complaining of epigastralgia caused by chronic pancreatitis with pancreatic caliculi. He underwent pylorus-preserving total pancreatectomy with duodeno-duodenal end to end anastomosis. Inslulin, 19 units daily, is administered for postoperative days and his body weight was recovered 8 weeks after operation. Oral glucose tolerance tests showed no remarkable change and D-xylose test indicated 2.8 g/5h, a slight disturbance of lipid absorption being suggestd. Postoperative study of gastric juice revealed increases of BAO and MAO. However, endoscopic study showed neither erosion nor ulceration. It is significant that pylorus preserving total pancreatectomy makes the patiens be able to eat enough volume to their satisfaction, with favorable weight-gain after surgery. Standard total pancreatectomy is usually associated with distal gastrectomy, however, recent foreign literatures ahve reported such operative method as the pylorus and whole duodenum are entirely preserved.This seems to be the first case report of pylorus preserving total pancreatectomy in Japan