Zollinger-Ellison Syndrome Surgery

Gastrin disease, also known as Zollinger-Ellison syndrome, is characterized by fulminant peptic ulcer, which is the result of high secretion of gastric acid caused by excessive secretion of gastric acid. 1. Incidence: The incidence of gastrinoma in the population is about 1:2.5 million, which is 1% weak in patients with peptic ulcer. 2. Although the disease can occur from child to old age, most patients are between 30 and 60 years old, with an average of 50.5 years. 60% of patients are male. 3. 20% to 40% of patients have MEN-I, the age of the incidence is smaller than that of MEN-I, and 70% are benign, multiple tumors, the distribution can exceed the pancreas. 4. The distribution of gastrinoma is mainly in the pancreas, 6% to 23% can be found in the duodenal wall, and other visceral ectopic occurrences. In the pancreas itself, tumors occur more at the tail of the body. 5. 60% of gastrinomas are malignant, 35% are adenomas, and 5% are islet cell hyperplasia. At the time of diagnosis, 50% to 80% of malignant gastrinomas have metastasized, and the liver is the most common metastatic organ. 6. Most gastrinomas are single-shot, and 20% to 40% may be multi-centered, especially those with MEN-I. 90% of recessive gastrinoma occurs at the junction of the cystic duct and the common bile duct, the margin of the second and third segments of the duodenum, and the triangular region at the junction of the neck and body of the pancreas. 7. The main clinical manifestations of gastrinoma are ulcer disease quality, accounting for 90% to 95%, pain accounting for 90% to 95%, bleeding incidence of 45% to 55%, and perforation 10% to 18%. Another 30% to 31% of patients have diarrhea. 8. 18% of gastrinomas have symptoms of hyperparathyroidism such as osteoporosis, kidney stones and renal calcification. 5% to 19% may be associated with Cushing syndrome. 9. Gastrointestinal barium meal and endoscopy can be seen in the duodenum with multiple ulcers, and can spread to the distal duodenum and jejunum. 10. The level of fasting gastrin in normal adults with high gastrinemia should not exceed 100 ng/L, while patients with gastrinoma can exceed 500 ng/L. In 40% of patients, the gastrin level is between 150 and 500 ng/L. For atypical cases, further diagnosis can be made by a secretin challenge test or a calcium transfusion test. If the fasting gastrin level is above 1500 ng/L, there is a case of metastasis. 11. The classic gastric acid secretion test is not as accurate as the gastrin test for the diagnosis of gastrin, but it still has certain diagnostic value. Basic acid secretion (BAO) in 68% of patients with gastrinomas is higher than 548 mmol/h (15 mEq/h). The ratio of BAO to MAO (maximum acid secretion) is diagnostically significant above 0.6, but up to 50% of cases are below 0.6. The combination of the two results is more diagnostic. 12. Localization diagnosis: 20% to 40% of gastrinomas cannot be located before surgery. Ultrasound diagnosis is only 20% reliable. The sensitivity of CT is 32% to 80%. The sensitivity of selective angiography is 50% to 70%, and the sensitivity to diagnosis of liver metastases can be as high as 86%. Percutaneous transhepatic portal vein blood sampling can detect tumors <1cm in diameter, which is helpful for the early diagnosis of gastrinoma. A group of 27 cases reported that 90% were localized, 43% of them were unable to paralyze tumors during surgery, and micro gastrinoma was confirmed after blind resection of the pancreas. However, the general report believes that the sensitivity of this method is 50% to 74%. Intraoperative ultrasonography is similar to the operation of handcuffs, so it can only be used in conjunction with percussion if the patient fails to locate or re-operate before surgery. Every patient with gastrin should undergo open exploration, but the choice of surgery varies from person to person. Open exploration can determine the presence or absence of tumors and metastatic lesions, which is helpful in estimating the prognosis of patients. If there is no gross tumor visible, the 5-year survival rate is 100%, the 10-year survival rate is 80%, and death itself has nothing to do with the tumor. If the liver has metastasis, the 5-year survival rate is only 40%, and individual cases can survive for more than 10 years. If the tumor is confined to the pancreas, 60% of patients can survive for 5 years, 40% can survive for more than 10 years, and the procedure for gastrinoma is vagus nerve cutting plus pyloric surgery, total gastrectomy, tumor resection, Distal pancreatectomy or semi-pancreatectomy. Treatment of diseases: gastrinoma Indication Zollinger-Ellison Syndrome Surgery applies to: 1. If the laparotomy does not find a specific tumor, the patient has difficulty in long-term drug control, and does not accept total gastrectomy, vagus nerve cutting plus pyloric angioplasty; patients who are willing to undergo total gastrectomy should undergo total gastrectomy It is appropriate. 2. If the tumor is found in the pancreas, duodenum or pancreas, a tumor resection should be performed. In view of the fact that 85% of patients have high gastric acid secretion after tumor resection, if the patient is weak, the body weight is significantly reduced, or other medical conditions, plus vagus nerve cutting plus pyloric or gastric antrum resection. The patient's condition permits, and a total gastrectomy is feasible. 3. For patients with large tumors or extensive lesions, it is feasible to have a distal resection of the pancreas or a semi-pancreatectomy. Due to postoperative complications and high mortality, pancreaticoduodenectomy or pancreatectomy is not recommended for gastrinoma. 4. Patients with metastatic lesions, if possible, should be treated with local lesion resection plus total gastrectomy. 5. Gastrinoma is one of the lesions of MEN-I. No matter whether it is possible to perform tumor resection, a total gastrectomy is required. Contraindications 1. The whole body has serious diseases that cannot tolerate the operation. 2. The patient refuses to perform the operation. Preoperative preparation Patients with severe complication of peptic ulcer have been treated with H2 blockers for a period of time before surgery. The systemic condition is stable, and elective surgery is better than emergency surgery. It is most desirable to use an H2 receptor blocker to control gastric juice above pH 5.5. The method of drug treatment is described later. Surgical procedure 1. Zollinger-Ellison syndrome surgery, generally using the upper abdomen incision. 2. In order to obtain better exposure, it is necessary to perform traction of the xiphoid part; the unobstructed nasogastric tube evacuates the stomach contents; the left hepatic lobe is retracted, and if necessary, the left hepatic triangle ligament is cut off; the hemostasis is completely stopped, and the surgical field is kept clear; Be proficient in the dissection of the upper abdomen and figure out the relationship between various organs and structures. 3. Cut the gastric colon ligament, enter the small omentum space, and explore the tail of the pancreas. 4. Open the duodenal lateral peritoneum, bluntly separate, turn the duodenum to the left, and probe the head of the pancreas. 5. For suspected mass biopsy, a cryosection is performed to determine the nature of the mass. 6. For the treatment of localized gastrinomas, tumors can be performed (refer to insulin tumors), and cryosections should be sent. 7. For patients with large tumors or extensive lesions, distal pancreatectomy is feasible (see the section on insulinoma fistula). 8. Vacotomy Vaginotomy has three surgical procedures, namely vagus nerve stemectomy, selective vagus nerve ablation and proximal gastric vagus nerve ablation. The esophageal plexus in the lower part of the esophagus forms the left and right vagus nerve trunks in the esophageal proximal sac. The left vagus nerve trunk exits the hepatic branch and the Latarjet branch in the part of the cardia in front of the esophageal wall; the right vagus nerve divides the abdominal branch and the posterior Latarjet branch at the same level behind the esophageal wall. The vagus nerve trunk was cut off, the esophagus was dissociated in the lower part of the esophagus, and the left branch of the vagus nerve trunk and its branches were separated in front of the esophagus; the right branch of the vagus nerve trunk and its branches were separated behind the esophagus. Cut off the bilateral vagus nerve trunk. Selective vagus nerve ablation: the left vagus nerve trunk is cut under the vagus nerve branch, and the right vagus nerve is only the stomach branch, and the left gastric artery is ligated. Proximal gastric vagotomy: In order to reduce the secretion of gastric parietal cells without affecting the function of the antrum and pylorus, the anterior and posterior Latarjet branches of the vagus nerve are cut off respectively, and the part of the left vagus nerve is preserved. The Heineke-Mikulicz method is commonly used for pyloricplasty. 9. Total gastrectomy (Total gastrectomy) As mentioned above, the main complications of gastrinoma and the cause of death are caused by the quality of ulcers. The conventional methods for treating ulcer disease are often ineffective. More than 60% of the disease is malignant, and many have been transferred during surgery. Fortunately, the disease progresses slowly. Although the tumor cannot be completely removed, if the target organ is removed (total gastrectomy), not only can the clinical symptoms be alleviated, but the primary lesions of a few patients. May shrink. Therefore, total gastrectomy is the basic method for treating gastrinoma. For the implementation of total gastrectomy, the stomach should be free from large and small curvatures, the blood vessels should be cut, the whole stomach should be free, and the duodenal stump should be closed. Cut off the vagus nerve. In the transverse colon mesal, the proximal jejunum was introduced and the end of the esophagus was end-to-side. In order to reduce the stimulation of bile reflux to the esophagus, jejunum lateral-lateral anastomosis can be performed under the mesenteric membrane. 10. Close the incision: Rinse the abdominal cavity with saline, completely stop the bleeding, and suture the incision layer by layer. 11. Place drainage: Place drainage in the pancreatic resection site and the esophageal jejunal anastomosis, and poke and fix it separately. complication 1. Patients with gastrinoma who have only partial pancreatectomy have similar complications to insulinoma. 2. Patients with vagus nerve cutting surgery may have problems such as poor gastric emptying, abdominal distension, diarrhea, and gallstone formation. If the gastric acid secretion from the stomach wall cells is not controlled, problems such as ulcers, bleeding, and perforation may still occur. 3. After total gastrectomy, there may be anastomotic leakage, abdominal infection, anastomotic stenosis, reflux esophagitis, anemia and dumping syndrome. The emergence of H2 receptor blockers has fundamentally changed the treatment of gastrinoma. Prior to this, even in the case of an emergency, the basic treatment was a total gastrectomy with a higher mortality rate. The earliest clinically applied preparation was cimetidine (cimetidine). This drug can control the clinical symptoms caused by gastrinoma, although there is still hypergastrin. However, 23% to 50% of patients require total gastrectomy or vagotomy to treat ulcer complications even in large doses. The advantage of cimetidine is that it eliminates the risk of emergency surgery. The dosage of cimetidine is more than 2.4g/d, and adverse reactions such as impotence, loss of libido, breast tumor and feminization of male breast can occur. However, some patients need 10g per day. The effect of ranitidine is 3 times stronger than that of cimetidine, and the adverse reactions are also small. With 0.6 to 3 g/d, the symptoms of 66% of patients were controlled without adverse reactions. Famotidine inhibits gastric acid secretion more strongly than cimetidine, and the adverse reactions are smaller than the above two drugs. A further disadvantage of the above three drugs is that the body's tolerance to it increases with time; the greater the dose, the more chances of adverse reactions. For the adverse reactions of H2 receptor blockers, anti-cholinergic drugs can be used, such as isopropamide, glycopyrrolate and pirenzepine. Omeprazole inhibits gastric acid secretion by inhibiting the H+/K+ adenosine triphosphatase system of gastric parietal cells, and is currently another drug for inhibiting gastric acid secretion. The drug has a small adverse reaction, a small dosage, and a good clinical application, but it cannot solve the high gastrinemia. Because the patient's gastric mucosa is still protected by high gastrin, and this effect may also have an effect on gastrin itself. In addition, the experimental animals treated with Omeprazole had the occurrence of gastric fundoid carcinoids, which was also the result of long-term nutritional effects of hypergastrin. Therefore, there are some concerns when using the above drugs. Currently, the best option for long-term medical treatment is to use a somatostatin-like drug, Sandostatin (octreotide acetate). It has been proved by clinical application that it has the effect of inhibiting gastric acid secretion and lowering blood gastrin levels in patients with ZE syndrome, and it is a long-acting drug. The advantage of this drug is to block the secretagogue and reduce its effect on the target organ, inhibit the secondary secretion of high peptides, and make other endocrine tumors of MEN-I patients in a subclinical state. The drug has a small adverse reaction. After a small number of clinical patients, the response rate to gastrinoma treatment is 76%, and it remains in good condition for 41 months. The method of use of the drug is 100 ~ 250g, subcutaneous injection, 3 times a day.