Portal and pancreatic vein catheterization with radioimmunologic determination of insulin.

Percutaneous transhepatic catheterization of the portal vein along with the catheterization of the aorta and the vena cava was performed in six patients, one of whom had hypoglycemic attacks due to Whipple's triad. Blood samples from different branches in the different vessel systems were withdrawn for radioimmunologic determination of insulin. By this method, preoperative localization of the insulin producing pancreatic islet cell tumor was su-cessfully performed. Multiple tumors as well as liver metastases were preoperatively excluded. Recatheterization and blood sampling for radioimmunologic determination of insulin was performed postoperatively to verify the radicality of the operation. Insulin concentrations from the hypoglycemic patients were compared with those of the patients without insulinoma and found to be significantly higher. The procedure is proposed as a useful method for localizing all types of gastrointestinal hormone producing primary or secondary tumors in which a method for hormone determination is available. The postoperative investigation is useful as a check of the operation performed and as a follow-up examination for the early diagnosis of recurrence.     

Surgical management of islet cell dysmaturation syndrome in young children

Islet cell dysmaturation syndrome (ICDS) encompasses the causes of infantile hyperinsulinemic hypoglycemia histologically described as islet cell hyperplasia, pancreatic adenomatosis and nesidioblastosis. Eleven infants underwent 14 pancreatic resections for ICDS from 1965 to 1990 at the University of California at Los Angeles Medical Center for severe hypoglycemia unresponsive to medical therapy. Seizures were the presenting symptoms of hypoglycemia in eight infants. Six patients had nesidioblastosis, four had islet cell hyperplasia and one patient had an adenoma with histologically normal pancreatic islet cells. Four neonates underwent 80 per cent pancreatic resection; three with nesidioblastosis required reoperation (90 to 95 per cent resection). Four older infants underwent 80 per cent pancreatic resection but required diazoxide for less than six months postoperatively. Three infants underwent 90 to 95 per cent pancreatic resection. None have required reoperation or postoperative medications. All infants are normoglycemic without pancreatic exocrine insufficiency and none had postoperative complications. Five infants had preoperative neurologic impairment, with three having severe retardation; all showed some improvement postoperatively, but only one infant now has normal findings on neurologic examination. Early diagnosis and aggressive surgical resection should minimize neurologic complications of the ICDS.     

Surgical treatment for carcinoma at the confluence of the major hepatic ducts

During the period of November 1976 through October 1984, we have treated 46 patients with carcinoma at the confluence of the major hepatic ducts. Curative resection of the tumor (where no carcinoma cells at the margin of the resected portion were found macroscopically and microscopially), was performed upon ten patients, palliative resections were done in 11, palliative resection and intraoperative radiotherapy (IOR) in ten, bile duct drainage and IOR in three, and percutaneous transhepatic cholangiodrainage in 12. Among the ten patients who underwent curative operation, five are living six to 104 months after operation while the remaining five died four to 72 months postoperatively. Of the 11 patients who underwent palliative resection, five are alive two to 22 months after operation and six died between four and 20 months after operation. For the ten patients with carcinoma in the advanced stages, palliative resection with IOR was performed. Eight patients died 20 days to 16 months after operation while two patients are alive 13 and 14 months after the procedure. Of the three patients who underwent drainage of the bile duct and IOR, two died three months after operation and one patient survived 34 months. Twelve patients underwent drainage of the bile duct only and all died after 13 months. To perform a curative operation for carcinoma located at the confluence of major hepatic ducts, it is necessary to resect the right or left lobe, including the caudate lobe, in many instances. For the patients with carcinoma in the advanced stages, the possibility of long term survival period after operation exists through the use of the combined techniques of IOR and palliative resection of the tumor.     

Cystic neoplasms of the pancreas

Eight patients with cystic neoplasms of the pancreas were seen at four Northern California hospitals between the years 1978 and 1986. Three of the tumors were benign and five were malignant. Three females, whose average age was 61 years, had cystadenomas. Three females and two males, whose average age was 48 years, had mucinous cystadenocarcinomas. Clinical presentations were similar among all patients. Abdominal pain was a prominent feature. Anorexia, weight loss, nausea and vomiting with a palpable abdominal mass were seen in five of eight patients. Obstructive jaundice was seen in two of eight patients. Among patients with benign lesions, one lesion was in the head and two lesions were in the tail of the pancreas. The malignant lesions were in the head of the pancreas in three patients and in the tail or body in two. A presumptive diagnosis was made preoperatively on the basis of the clinical, laboratory and roentgenographic findings in seven of eight patients. Of the patients with benign tumors, two are alive and well at seven years and four months and one patient was lost to follow-up study at four years. Among the patients with a malignant condition who underwent operation, resection for cure was performed upon four patients. One patient died postoperatively and the other three patients are alive and well without evidence of a recurrence at three and one-half, four and four years after resection. Pancreaticoduodenectomy was performed upon two patients and distal pancreatectomy in another. Palliation was attempted in one critically ill patient with an unresectable tumor by longitudinal pancreaticojejunostomy. This procedure was not effective in providing pain relief because of obstruction of the pancreatic duct by the viscous mucoid secretion of the tumor. The preoperative diagnosis of these very rare tumors is usually possible roentgenographically, especially with the use of the computed tomography scan. The presence of a thick mucoid secretion of high viscosity is diagnostic of mucinous cystadenocarcinoma. Cystic neoplasms of the pancreas should always be resected, if possible, with the expectation of long term survival.     

Total cavopulmonary connection for surgical treatment of complex congenital heart disease.

Diversion of vena caval flow directly into the pulmonary circulation--total cavopulmonary connection--has been tried at our hospital for two years as an alternative to the modified Fontan procedure for surgical treatment of complex congenital heart disease other than tricuspid atresia in 26 cases with six operative mortalities. The causes of death were low cardiac output in four, uncontrollable paroxysmal supraventricular tachycardia in one and airway obstruction in one. Except for one late death which occurred three months after surgery due to sepsis, the 19 patients who survived the operation were followed up for four to 27 months (mean 15 months). All of them improved clinically (NYHA Class 1). Graded bicycle exercise tests were performed in five of them at three to 12 months after surgery, and their exercise tolerance was comparable to that of patients with an atriopulmonary connection. In conclusion, total cavopulmonary connection is an acceptable alternative to the modified Fontan operation for surgical treatment of complex congenital heart disease, although longer follow-up is necessary.     

Chemoinfusion of the hepatic artery for metastases to the liver

During a five year period, 69 patients were treated for carcinoma of the liver (seven primary tumors and 62 metastatic tumors) with 5-fluorodeoxyuridine (5-FUDR) administered through a hepatic artery (n = 62) or portal vein (n = 3) implantable infusion pump. Ten patients proved to have previously unsuspected extrahepatic nodal metastases at laparotomy for pump insertion. 5-FUDR was given in 14 day cycles for three months. At the end of that period and at three month intervals thereafter (mean follow-up time of 18 months, a range of one to 60 months), patients were evaluated with roentgenograms of the chest, liver function tests, carcinoembryonic antigen levels, radionuclide scans and computed tomography. Thirty-five patients had a partial response, nine had stabilization of the disease and 25 had progression of the disease (five during therapy, who were given mitomycin C). Median regression was 6.8 months (a range of three to 18 months). Six of the 35 partial responders, three of the nine patients with stabilization and ten of the 25 patients with progression had extrahepatic visceral disease. Survival time averaged 18.4 months (a range of five to 60 months) for the partial responders, 12.6 months (a range of two to 40 months) for patients with stabilization and seven months (a range of one to 17 months) for those with progression of the disease.     

Clinical and hemodynamic results after Fontan operation.

Twenty-six patients, ranging in age from 2 to 14 years (mean, 6 years and 8 months), with tricuspid atresia or other complex cyanotic cardiac diseases underwent a modified Fontan operation between 1980 and 1990. In 13 patients, palliative operations had been previously performed. There were 7 mortalities within 1 month after the operation. Seventeen of the 19 survivors were followed up for 5-122 months (mean, 27) with no late deaths. Fifteen patients were in the New York Heart Association functional class 1. Two patients required reoperations at 2 and 57 months postoperatively: the former for atrio-ventricular valvular regurgitation, and the latter for late-developing conduit obstruction and residual atrial right to left shunting. Fourteen patients underwent cardiac catheterization after 1-57 months (mean, 9.8) postoperatively. Two patients had a residual right to left shunt at the atrial level, while the oxygen saturation in the remaining 12 patients was above 90%. Thus, the modified Fontan operation offers an alternate surgical approach for patients with complex congenital heart disease for whom no other palliative procedure can be expected to obtain significant improvement. It can be performed with good clinical and hemodynamic results in selected patients.     

Regional intra-arterial infusion of adriamycin in the treatment of cancer.

Forty patients with otherwise untreatable advanced carcinomas received arterial infusions of adriamycin. Significant responses were seen in five of nine tumors of the bladder, two of five hypernephromas, two of two islet cell tumors, three of five sarcomas and one of two metastatic tumors of the breast. One patient with advanced transitional cell carcinoma of the bladder had no tumor at the time of a subsequent resection. There were no responses seen in two oat cell tumors metastatic to the liver, one seminoma of the retroperitoneal space metastatic to the liver, one squamous cell tumor metastatic to the liver, one squamous cell of the vulva, one carcinoma of the cervix uteri and one cloacogenic carcinoma. these responses in advanced tumors are exciting. The use of this technique to reduce tumor bulk prior to operation, irradiation, adjuvant chemotherapy and immunotherapy may allow a significant breakthrough for such tumors as sarcomas, hypernephromas and transitional cell carcinomas of the bladder. Further studies will be pursued using this concept.     

Major amputation for advanced malignant melanoma

The Memorial Sloan-Kettering Cancer Center experience with major amputation for advanced malignant melanoma from 1965 to 1984 is reported. This is a retrospective review of 58 patients who underwent hemipelvectomy, disarticulation of the hip and above knee or forequarter amputation for advanced or recurrent malignant melanoma. Major amputation with curative intent was performed upon 43 patients. There were three deaths that occurred 30 days postoperatively (7 per cent). Intransit metastasis was one of the indications for amputation in 33 patients, and local control of disease was achieved in 30 of 43 patients. The median time to recurrence in those patients who did have a recurrence was 12 months (a range of 18 days to 16 years). Fifteen of 40 patients who survived the operation had no evidence of disease five years after undergoing a major amputation. Age, sex, primary site, stage of disease at diagnosis and amputation, disease-free interval and positive node status at any time during the course of the disease were not predictive of the outcome. Neurovascular involvement with melanoma and positive margins of resection predicted early failure. A palliative amputation for impending major vascular disruption or extensive, fungating disease was performed upon 15 patients. There was one death 30 days after the operation, and generally, hospitalization did not exceed 30 days. Median survival time was five months. All of the patients died of local or distant disease within 33 months of treatment. Local recurrence preceded death in nine of 15 patients.     

卵巢支持莱狄细胞瘤11例临床分析

目的探讨卵巢支持莱狄细胞瘤的临床特征、治疗和预后。方法 回顾性分析了我院1962—2002年治疗的11例卵巢支持莱狄细胞瘤患者的临床和病理资料。结果11例患者中,病理分化程度:高分化7例,中分化3例,低分化1例。临床分期:Ⅰ a期9例,Ⅱc期及Ⅲc期各1例。临床症状:腹部包块8例;去女性化及男性化表现共6例,其中3例行血清睾酮水平测定,均不同程度升高;月经增多、绝经后阴道出血等女性化表现者5例,其中1例同时具有女性化及男性化表现。合并症:5例合并与雌激素相关的疾病,包括子宫肌瘤、子宫内膜增生等,2例患者先后患乳腺癌。治疗:11例患者均行手术治疗,其中5例肿瘤分化不良或Ⅱ～Ⅲ期者,术后行系统性巩固化疗。随诊:随诊时间为6个月至34年,中位数随诊时间为7年,无一例死于本病。3例患者保留生育功能,均于术后1—3个月恢复月经,其中1例已生育。结论卵巢支持莱狄细胞瘤预后良好。早期、高分化者,可单纯手术治疗。分化不良或晚期肿瘤患者,术后应予巩固化疗。有生育要求者,可保留生育功能。     

Bilateral ovarian lipid cell hyperplasia in a young hirsute patient

Virilizing lipid cell ovarian hyperplasia occurrd in a 24-year-old woman referred with secondary amenorrhea, hirsutism, and weight gain. Hormone analyses revealed abnormal androgen production shown in a suppression test to be autonomic and of ovarian origin.Ovarian vein catheterization revealed abnormally increased androgen levels in the left ovarian vein and in the caval vein where it was entered by the right ovarian vein. As the patient wanted to become pregnant, three-quarters of her ovarian tissue was resected. Menstruation and ovulation returned. Two years after the operation the patient conceived.Histopathological and electron microscopic examinations revealed bilateral lipid cell hyperplasia.     

Transhepatic portal sampling for preoperative localization of insulinomas

The data of 72 patients with pancreatic insulinomas were analyzed. Twenty-one were obtained from personal experience and 51 from a review of the literature. In all instances, detailed information about insulin levels in the portal tree as obtained by means of a transhepatic portal sampling (THPS) and localization of the tumor as found during the surgical procedure was available. Five different criteria were compared for defining the peak insulin concentrations regarded as indicative of tumor localization: 1, peak above 500 milliunits per liter; 2, peak above 200 milliunits per liter; 3, peak 2.5 times higher than the lowest portal value; 4, peak 2.3 time higher than the distal mesenteric value, and 5, peak higher than mean portal concentration plus or minus 2 standard deviation. Criterion 5, associated with sample numbers larger than 15 and catheterization of the cephalic veins, provided the best results for obtaining valuable information from THPS.     

Granulosa cell, Sertoli-Leydig cell, and unclassified sex cord-stromal tumors associated with pregnancy: a clinicopathological analysis of thirty-six cases

Seventeen granulosa cell, thirteen Sertoli-Leydig cell and six unclassified sex cord-stromal tumors diagnosed during pregnancy or the puerperium were reviewed. Eleven patients presented with abdominal pain or swelling, five in shock, two with virilization, and one with vaginal bleeding. Three asymptomatic patients were explored because of a palpable mass and one because of an adnexal mass found on ultrasound examination. In thirteen patients the tumor was discovered during a cesarean section; five of them had had dystocia and in eight of them the tumor was an incidental finding. All the tumors were Stage I but 13 of them had ruptured; all but one were unilateral. Hemoperitoneum was present in seven cases. On microscopical examination many of the tumors differed from tumors in the same diagnostic categories occurring in the absence of pregnancy by having a disorderly arrangement of their cells, lacking recognizable differentiation in many areas, showing prominent edema, and containing unusually large numbers of lutein or Leydig cells. The last two features were most obtrusive in tumors removed at term. With one exception the patients were initially treated by conservative surgical procedures. Two of them received chemotherapy and two radiation therapy postoperatively. A hysterectomy and salpingo-oophorectomy was performed at a second operation in eight cases; no residual tumor was found in any of the specimens. Only one patient had a recurrence, which was treated surgically. Follow-up for an average of 4.7 years is available for 30 of the 36 patients; all of them were alive and free of disease at the time of the last examination.     

Clinical and experimental study of pancreatic exocrine function after pancreaticoduodenectomy for periampullary carcinoma

The residual pancreatic exocrine function before and after pancreaticoduodenectomy (PD) for periampullary carcinoma was studied clinically as well as experimentally. In clinical instances (n = 35), the N-Benzoyl-L-Tyrosil-p-Aminobenzoic Acid test (BTPABA test) before and within two months after PD revealed reduction of the function compared with those in the control study. However, the test result one year after PD was improved compared with those before and within two months after operation, without differences from that of the control group. In new canine PD models in which drainage of the pancreatic duct, 50 per cent pancreatectomy and duodenojejunectomy were performed after three months of pancreatic duct obstruction, fibrosis surrounding the pancreatic duct was disclosed. However, the results of examination five months after PD revealed a milder degree of pancreatic fibrosis without aggravation of the lesion. The aforementioned findings indicated that the exocrine pancreas before PD was impaired due to obstructive pancreatitis and that the postoperative pancreatic function was well preserved at the level close to that in the control group even after approximately 50 per cent resection of the pancreas, if pancreatic duct drainage was effectively performed.     

Surgical treatment of hilar carcinoma of the bile duct

The operative results of hilar carcinoma of the bile duct are extremely poor and there are few long term survivors. During the past seven years and six months at our clinic, 26 of 32 patients with hilar carcinoma were operated upon and 24 of these patients underwent resection with a resectability rate of 92.3 per cent. There was one operative death and the mortality was 3.8 per cent in 26 patients operated upon and 4.2 per cent in 24 patients who underwent resection. Seven are still alive postoperatively. The longest survival time is five years and seven months without a recurrence after right trisegmentectomy for carcinoma of the intrahepatic bile duct with hilar invasion. Curative resection was performed upon ten of 26 patients who underwent operation. Of the patients who were operated upon, 12 had invasion of the parenchyma of the liver at the hilum and 11, invasion of the caudate lobe, including direct invasion in three and invasion of the bile ducts in eight. Therefore, the caudate lobe should be resected for radical operation for hilar carcinoma. In this study, the anatomy of the hilar area, including vascular structures of the caudate lobe, was evaluated in 106 cadavers, concerning radical operation for hilar carcinoma.     

The influence of portal blood upon lipid metabolism in normal and diabetic dogs and baboons

Complete diversion of portal blood in dogs caused sustained falls in serum cholesterol and phospholipid concentrations an declines in hepatic cholesterol and triglyceride synthesis. The hepatocytes in these canine livers were deglycogenated, and they atrophied to about half of their original size within two months. At the same time, there was evidence of increased mitoses. Ultrastructurally, the dominant change in the hepatocytes was in the rough endoplasmic reticulum which decreased in amount, underwent marked dilatation, and became depleted of ribosomes. There was also marked loss of glycogen granules, variable mitochondrial abnormalities, and widespread accumulation in the hepatocyte cytoplasm of lipid vacuoles. Bypass of intestinal venous return around the liver through a mesenteric caval shunt did not influence the serum lipid concentrations in dogs and baboons, although cholesterol synthesis was depressed in the canine livers and significant morphologic changes, including atrophy, were produced. In both species, the addition of a second stage central portacaval shunt which diverted venous return from the pancreaticogastroduosplenic area caused declines in serum cholesterol and phospholipid concentrations. After the second operation, hepatic cholesterol synthesis in the dogs was further reduced, and triglyceride synthesis was markedly depressed. The eventual ultrastructural changes were similar to those after one stage portal diversion. In other experiments on dogs, discrete regions of the liver were provided with portal perfusion from different splanchnic sources during a two month period. When the right lobes received pancreatiogastroduodenosplenic venous blood and the left lobes received intestinal venous effluent, in vivo cholesterol and triglyceride synthesis were higher in the hormone-enriched right lobes. This advantage was eliminated with pre-existing alloxan-induced diabetes or by the concomitant performance of total pancreatectomy in dogs that were treated during the ensuing two months with subcutaneously administered insulin. The nutrient-enriched left lobes had the higher lipid synthesis. In a final series of experiments, the right lobes of dogs were given the total splanchnic flow, and the left lobes were perfused with systemic venous blood by anastomosing the left portal vein to the suprarenal vena cava. The right lobar advantage in lipid synthesis could not be eliminated in this preparation with alloxan-induced diabetes or total pancreatectomy. These results indicate that a reduction of hepatic lipid synthesis is an important, although not necessarily the sole, factor in the antilipidemic influence of portacaval shunt. The effects upon synthesis and blood lipids apparently are due more to the diversion of endogenous hormones than to the bypass of intestinal nutrients. The substances in portal venous blood that subserve hepatic lipid metabolism are presumably largely the same as the hepatotropic factors which have been described before as profoundly affecting hepatic structure, function, and the capacity for regeneration. These portal blood factors are multiple and interrelated, but the single most important one seems to be insulin.     

Carcinoma of the duodenum

Primary carcinoma of the duodenum is an uncommon tumor of the gastrointestinal tract. We reviewed the histories of ten patients seen between 1976 and 1986. Some of the patients with tumors in the second part of the duodenum presented with clinically evident jaundice. The symptom complex of all other patients was compatible with many benign diseases and made the diagnosis difficult. At laparotomy, seven patients had resectable disease. Two patients had advanced disease so that no curative resection could be done. In one patient, a resection was technically impossible. A modified Whipple procedure (in which the pylorus is saved) is the method of choice for tumors of the second part of the duodenum. We perform a segmental resection for other tumors. In five patients, there was no involvement of the lymph nodes and these patients are well--two more than 30 months postoperatively and one patient almost ten years postoperatively. In two patients, one or more lymph nodes were involved, but they are still well 30 months postoperatively. As the prognosis of carcinoma of the duodenum, once metastasized, is poor, a greater awareness of the possibility of a duodenal tumor must accompany aggressive diagnostic and surgical procedures. That will be the only way to a higher percentage of cures.     

Repeat hepatectomy for recurrent malignant tumors of the liver

The results of 34 repeat hepatectomies for recurrent malignant tumors of the liver in 28 patients are reported herein. There was no operative mortality, and the morbidity rate was 15 per cent. No instances of postoperative hepatic insufficiency were observed. Only five of the repeat hepatectomies were extensive (three or more hepatic segments). Thirteen resections were performed upon 11 patients for recurrent hepatocellular carcinoma. Four of these patients are still alive, one patient with a known recurrence (a mean survival time of 33 months with a range of four to 54 months). Ten resections were performed upon nine patients for recurrent metastases from the colon and rectum. Five of these patients are alive, four without apparent recurrence (a mean survival time of 13 months and a range of one to 35 months). Eleven resections were performed upon eight patients for recurrent miscellaneous malignant tumors, and four patients are alive, of whom three have a recurrence. The results of this study demonstrate that some patients benefit from repeat hepatectomy for recurrence of malignant hepatic tumors. Repeat hepatectomy is technically highly feasible. Economic hepatic resection with a sound oncologic basis (segmentectomy) is the operation of choice.     

Results of Senning operation for transposition of great arteries.

We report our results with 10 infants and children who underwent atrial repair using the Senning operation between 1985 and 1990. All cases had abdominal situs solitus, levocardia and atrio-ventricular concordance (D-bulboventricular loop). Nine patients had simple D-transposition of the great arteries without ventricular septal defects (VSD) or left ventricular outflow tract obstruction. The other patient had a double outlet right ventricle with subpulmonic VSD and pulmonary hypertension (PHT) and underwent a palliative Senning procedure. All patients had a balloon atrial septostomy (BAS) before surgery, except for one with Taussig-Bing syndrome. One patient had a Blalock-Hanlon operation after BAS. The age at the time of surgery ranged from two months to four years seven months (mean: 22 months) and weight ranged from 4.3-12 kg. There were two hospital mortalities including the patient with VSD and PHT. All of the patients had echocardiographic examinations and six of the eight survivors received cardiac recatheterization four to 19 months (mean: 7.4 months) postoperatively. No baffle leaks were noted in the survivors. Two patients had both mild tricuspid regurgitation and slightly decreased right ventricular contractility, and one patient had a pressure gradient of 6 mmHg between the superior vena cava and neo-right atrium. The clinical follow-up interval was eight to 64 months (mean: 31.4 months). All survivors showed a sinus rhythm on their latest electrocardiogram and were participating in normal daily activities without medication.     

Intestinalization of pancreatic fragments in dogs

A new operative procedure for the preservation of pancreatic fragments is introduced ("intestinalization") in which vascularized fragments are placed inside a jejunal pouch. This step is done to manage all secretions, lymph and potential debris coming from the pancreas; to maintain the primary portal drainage of insulin; to avoid vascular operations, and to keep the fragment in a denervated state and still preserve its endocrine function to prevent diabetes. The procedure was compared with conventional end to end pancreaticojejunostomy. Sixteen dogs were studied. Group 1 consisted of four dogs that had intestinalization of the pancreatic tail. Group 2 is made up of eight dogs that had complete resection of the remaining duodenal portion of the gland as well as intestinalization of the pancreatic tail. Group 3 consisted of four dogs that had end to end pancreaticojejunostomy to the isolated tail and complete resection of the duodenal portion of the gland as those dogs in group 2. No dog became diabetic during the time of observation (up to one year)--fasting glucose levels and K values in glucose tolerance tests remained normal. Insulin peak values at ten minutes were lower postoperatively. However, return to fasting levels occurred equally fast postoperatively. There was no difference among the groups. The most important morphologic finding in the intestinalized fragments was a firm capsule surrounding the tissue after two weeks. Exocrine atrophy was present in both groups 2 and 3 after one year. No reinnervation of intestinalized pancreas was seen. Intestinalization may offer an alternative to pancreatic resection especially when heterotopic autotransplantation is considered.