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[A human pancreatic glucagonoma, ultrastructural, immunocytochemical and radioimmunological investigations (author's transl)].

The authors report a case of glucagonoma in a 52 years old man presenting a migratory necrolytic erythema. By conjugated means of arteriography and splenoportography with plasma glucagon assays the tumour was localized in the tail of the pancreas. Surgical excision was easy but hepatic metastases revealed the malignant nature of the tumor. This glucagonoma has been investigated by several approaches including electron microscopy, immunocytochemistry and radioimmunological techniques. The tumor contained scattered glucagon and pancreatic polypeptide immunoreactive cells; insuline, glucagon, somatostatin, pancreatic polypeptide, gastrin and VIP antisera gave negative results. Ultrastructurally, these cells showed atypical secretory granules different from A granules of the normal glucagon cell. Radio immunological determinations carried out after gel permeation chromatography of plasma revealed high molecular weight (4 000, 9 000, 14 000) immunoreactive glucagon peptides. They have been thought to be proglucagon forms which did not react with specific antiglucagon sera used in cytological studies. Reported data are consistent with the classification of this tumor in the category of glucagonoma with the "glucagonoma syndrome".

Adenoma, Islet Cell↗

Splenic vein thrombosis with bleeding gastroesophageal varices. Reports of two splenectomized cases and review of the literature.

Two cases of bleeding gastroesophageal varices due to isolated splenic vein thrombosis are reported. The patients were aged 51 and 50 years. The respective etiologies were pancreatic pseudocyst and previous gastric surgery. The diagnosis was confirmed prior to splenectomy by splenoportography. There was no recurrence of bleeding during 2 and 5 years of observation. The 49 cases of splenic vein thrombosis published since 1970 are briefly reviewed and their treatment is discussed.

Esophageal and Gastric Varices↗

[A nonselective proximal end-to-side shunt between the gastric vein and the inferior vena cava (coronario-caval shunt). Indication, technic and results].

From 1976 to 1982, 104 patients with bleeding esophageal varices were treated surgically. In three patients a wide left gastric vein without portal flow was demonstrated by means of arterial splenoportography. Ascites estimated between 4 to 61 was observed in two patients. Anemia, leukopenia, platelet counts inferior to 85 X 10(9)/l were shown in all of them. Liver panangiography was done in the three patients. According to the absence of portal flow, the presence of intractable ascites and the demonstration of a wide left gastric vein, we dissect this vein disconnecting it from the stomach, and performing the anastomosis between the proximal end of this vessel and the inferior caval vena (proximal unselective left gastric caval shunt). Ascites and esophageal varices disappeared in the three patients between 1 and 3 weeks after the operation. The shunts were confirmed open between eight months to eight and a half years after operation. All the three patients are still living.

Esophagus↗

[Diagnosis of cavernomatous transformation of the portal vein].

In 10 patients at the age of 17 to 59 years the diagnosis of a cavernomatous transformation of the portal vein primarily was made by sonography and was confirmed by computer tomography and angiography, respectively. These findings were seen by chance when an abdominal ultrasound was performed in order to clarify splenomegaly or esophageal varices. The characteristics of the disease are the positive proof of a convoluted agglomeration of racemose venous structures that have replaced the normal single portal vein and signs of portal hypertension. The sonographic figures are so typical that ultrasound is the decisive procedure in its diagnosis and that direct or indirect splenoportography is not necessary. The present results show that clinical manifestations of cavernomatous transformation of the portal vein are delayed sometimes in to adolescence and that invasive diagnostic methods are only necessary when shunt operation is planned.

Adolescent↗

[Diagnosis of primary and secondary tumors of the liver].

A brief epidemiological and clinical introduction is followed by an examination of the most useful methods for the diagnosis of primary and secondary tumours of the liver: alpha protein values, scintiscanning, arteriography of the coeliac artery, splenoportography, and laparotomy with directed biopsy. The diagnostic specificity and percent positivity of each method are illustrated.

Angiography↗

Left gastric arteriovenous fistula after selective distal splenorenal shunt.

We present the case of a 64-year-old alcoholic who had suffered two episodes of hemorrhage from esophageal varices. For control of variceal hemorrhage, he underwent a distal splenorenal shunt. His immediate postoperative course was complicated by the development of marked ascites and intermittent episodes of encephalopathy. Routine postoperative angiography was performed after 4 months and demonstrated a fistula between the left gastric artery and vein. Patency of the shunt was demonstrated by direct percutaneous splenoportography. Two months after this admission, the patient was readmitted with the complaints of anorexia and nausea. Marked encephalopathy was noted. Eight hours following admission, he developed acute abdominal distention and hypotension. An abdominal tap revealed bloody fluid, and the patient was immediately prepared for transport to the operating room. He suffered cardiac arrest during transport, and all efforts at resuscitation were unsuccessful. Although a postmortem examination was not performed, it is suspected the arteriovenous fistula resulted in severe portal venous hypertension leading to intraperitoneal rupture of one of the affected veins, producing a massive hemoperitoneum.

Arteriovenous Fistula↗

Caroli's disease: role of endoscopic retrograde cholangiopancreatography.

Several modalities have been used in the diagnosis of congenital dilatation of the intrahepatic bile ducts (Caroli's disease) with varying degrees of reliability and morbidity. We report two patients with Caroli's disease where endoscopic retrograde cholangiopancreatography was used to define the anatomy of the extra and intrahepatic biliary trees. In one of the patients (BT) esophageal varices were easily visualized on endoscopy which had been previously demonstrated by a more invasive procedure (splenoportography). There were no complications of retrograde cholangiopancreatography in these two patients. We believe that retrograde cholangiopancreatography is a safe and effective method of evaluating the biliary tree and status of portal hypertension in patients with Caroli's disease as well as providing a reliable means of following the progression of their disease.

Adolescent↗

[Diagnostic significance of the "streaming phenomenon" in interpreting splenoportograms in the portal hypertension syndrome].

The "phenomenon of streaming" has been found by the author in splenoportography in 9,4% of patients. The variants of the phenomenon are described. The phemenon seen as a conoid shade superimposed on the shade of the portal vein trunk can suggest possible stenosis of the mouth of the splenic vein. Under these conditions it is inexpedient to fulfill distal splenorenal anastomosis.

Diagnosis, Differential↗

[Portal hypertension of extra-hepatic origin in children (author's transl)].

From 1960 to 1981, 31 children, 18 boys and 13 girls, have been treated at Ste-Justine Hospital for extra-hepatic portal hypertension. Age at the onset of gastro-intestinal bleeding 3 1/2 months and 13 years. Splenomegaly with hypersplenism, hematemesis and melena have been the most frequent clinical manifestations. Percutaneous splenoportography be coelio-mesenteric arteriography confirmed the diagnosis of cavernomatous transformation of the portal vein. Among the 31 children, 28 bled from their varices, the 3 others did not to date. Among the 28 patients with active bleeding complication, 7 have been treated conservatively, 11 had sclerotherapy (sclerosing injections of varices). On the surgical point of view, 7 had ligation of varices with intra-thoracic transposition of the spleen in 5 of them. Portosystemic shunts were performed in 10 patients, 3 central spleno-renal and 7 cavo-mesenteric shunt. Each of these 28 patients had an average of 9,5 episodes of gastro-intestinal bleeding, 8.7 hospitalizations, and received 7.5 liter of blood. Medical treatment, sclerotherapy, ligature of varices and intra-thoracic transposition of the spleen are palliative measures. Nevertheless the procedures are time and life savers, allowing improvement and development of natural porto-systemic shunts. Follow up of unshunted patients on a period of 8 to 17 years revealed a decreasing frequency and intensity of the hemorrhagic manifestations. Operative risks and morbidity of recurrent bleeding should be the guidelines for surgery. Natural course of illness and possible neuro-psychiatric consequences of a porto-caval shunt should also be considered.

Adolescent↗

Portopulmonary shunt by splenopneumopexy as a surgical treatment of Budd-Chiari syndrome.

Portopulmonary shunting by splenopneumopexy was devised as a new surgical procedure for treatment of portal hypertension accompanied by Budd-Chiari syndrome. Technique of this procedure is simple and is safely performed. The remarkable portopulmonary shunts were visualized by splenoportography postoperatively. Splenic pulp pressure was reduced to a postoperative mean value of 258.3 mm H2O from a preoperative mean value of 336.8 mm H2O. Hemorrhage from esophageal varices and ascites were well controlled after operation. Postoperative liver function tests were essentially unchanged from the preoperative values. Any respiratory and circulatory complications after this shunting have not been observed so far. It is concluded that portopulmonary shunt by splenopneumopexy is an effective procedure for portal hypertension caused by Budd-Chiari syndrome.

Adult↗

[Differential surgical treatment of liver cirrhosis].

The work analyzes the data of treatment of 144 patients with the cirrhotic process in the liver. The patients were subjected to 196 different operations. Surgical interventions were fulfilled depending on the stage of cirrhosis, chronic hepatitis--precirrhosis included. To diagnoze and determine the stage of the cirrhotic process the authors used a complex of investigations including the summary assessment of the liver function by the formula developed in the clinic, radioisotopic diagnosis, laparoscopy with target puncture biopsy, splenoportography, lymphography. The cirrhosis stage and the degree of hepatic insufficiency determined the choice of periarterial hepatic neurectomy. Marginal resection of the liver was performed in chronic hepatitis and the initial stage of cirrhosis.

Chronic Disease↗

The "pseudo-cholangiocarcinoma sign" in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels.

OBJECTIVES: The goal of this study was to identify the underlying disorder responsible for portal venous thrombosis and cavernous transformation of the portal vein (CTPV). All patients with this finding underwent a thorough medical examination with intent to determine the cause and biochemical consequences of CTPV. METHODS: During an 8-yr period, a total of 1247 patients with clinical evidence of portal hypertension were examined using ultrasonography. Forty four of these 1247 patients were found to have CTPV. In each case, the finding of CTPV was confirmed by portography using either splenoportography or arterial portography, with digital subtraction angiography. These 44 patients were studied in an effort to determine the etiology of the cavernous transformation. In addition, the specific reason for the increased serum bilirubin and alkaline phosphatase levels in 35 of the 44 cases was evaluated by endoscopic retrograde cholangiopancreatography (ERCP) (34 patients), percutaneous transhepatic cholangiography (one patient), and by CT in 19. The surgical findings in 10 of these 44 patients, who ultimately underwent splenectomy and portal venous decompression for bleeding, were reviewed in light of the ultrasonographic, portographic, and ERCP findings in the same 10 patients. RESULTS: The underlying disorder responsible for cavernous transformation was found to be Behcet's disease in seven patients, chronic liver disease in four, congenital hepatic fibrosis in five, congenital protein C deficiency in one, and a prior abdominal operation for cholelithiasis in one patient. Despite a full clinical, radiological, hematological, and chemical evaluation, no etiology for CTPV was found in the remaining 26 patients. All of these later cases had no indication for liver biopsy or evidence for parenchymal liver disease. In these 26 patients, the serum levels of bilirubin and alkaline phosphatase ranged from mild to moderately increased compared with the moderately to markedly increased levels present in the 18 patients having an identifiable underlying liver disease. Irregular, undulating narrowing and nodular extrinsic defects, the so-called "pseudo-cholangiocarcinoma sign" was present in 33 of the 35 patients who underwent either ERCP or percutaneous transhepatic cholangiography. No such findings were observed in 10 control cirrhotic patients with portal hypertension but without CTPV, who also underwent ERCP. CONCLUSION: The results of this study indicate that mildly increased serum alkaline phosphatase and direct reacting bilirubin levels occur in cases with CTPV associated with a pseudo-cholangiocarcinoma sign. Presumably, these enzyme elevations are a result of compression of the biliary tree by the venous collaterals that run along the extrahepatic biliary tree. None of the 33 cases with this sign had cholangiolar carcinoma. Thus, when a patient with splenomegaly but without documentable parenchymal liver disease demonstrates an increase in the serum direct reacting bilirubin and alkaline phosphatase levels, CTPV and the presence of large extrahepatic venous collaterals partially obstructing the biliary tree should be suspected.

Adult↗

[Surgical aspects of thrombosis in the portal system (author's transl)].

The Authors present and discuss clinical and therapeutic aspects of several cases of chronic portal thrombosis, troncular and radicular, either isolated or variously associated. Splenoportography and selective arteriography do not always provide a clear-cut picture of the vascular situation, particularly in the presence of mural thrombi. Surgery may be needed as an emergency measure in cases of hemorrhage, but the best results are obtained if it can be done electively. Indications, however, must be evaluated very carefully in each individual case, especially for thrombosis not associated with cirrhosis of the liver, in which the tendency to a more favorable natural evolution may invite a more conservative approach. The choice of surgical procedures is dictated essentially by the site of obstruction in the portal system. After discussing the indications for various methods, the authors present some cases of thrombosis involving only the superior mesenteric vein, managed successfully by disobliteration and mesenterocaval anastomosis.

Adolescent↗

Complete obstruction of the inferior vena cava due to chronic relapsing pancreatitis: a case report.

A woman aged 62 with long history of chronic relapsing pancreatitis presented with swelling and ulcer in the lower limbs and occasional gastrointestinal bleeding. The radiological imaging showed complete obstruction of Inferior Vena Cava (IVC) at the level of the pancreas and well developed collateral vessels. Portal vein and splenic vein were also obstructed and superior mesenteric venous blood drained into the liver via coronary vein. She was originally found to have pancreas head tumor, which was not resectable. A palliative operation was performed, but histological examination of pancreatic specimen suggested only chronic inflammation and no evidence of malignancy. She was diagnosed as tumor-forming type chronic pancreatitis. Although SPV or SMV-PV obstruction has been recognized as a complication of chronic pancreatitis, IVC obstruction can occur by the same mechanism. This is the only case but one ever reported. Not only splenoportography but IVC-graphy will contribute to more precise understanding of patient's condition with chronic pancreatitis.

Chronic Disease↗

[Portal hypertension--current status of ultrasound diagnosis].

Splenomegaly, ascites, and anatomy of intra- and extrahepatic portal vessels can reliably be detected by ultrasound in case of portal hypertension. The increased diameter of the portal vein and its roots is a not sufficient sensitive and specific finding in portal hypertension. However a marked variation of diameter at the superior mesenteric vein during in- and expiration is nearly exclusive. With the help of colour flow imaging or duplex sonography additional finding of blood flow in the portal system can be detected noninvasively and continuously. Quantitative blood-flow measurement in routine examinations is unnecessary and reserved to special questions. Reverse flow or significantly decreased blood flow velocity or the detection of portocaval collaterals are reliable findings in portal hypertension. In addition thrombosis of portal vessels and its hemodynamic consequences can be seen. Because underlying diseases e.g. liver cirrhosis or tumours are diagnosed in the same procedure ultrasound techniques are used in first line when portal hypertension is suspected. The findings are complementary to endoscopy of upper g.i.tract and lead on the one hand to a well-aimed use of CT scanning or x-ray splenoportography, and--on the other hand--make them dispensable in a low of cases.

Blood Flow Velocity↗

[Progress in pancreatic X-ray diagnosis in the last 30 years].

Proceeding from the pre-war state of the radiology of the pancreas in temporary order those investigations are discussed which essentially furthered the diagnosis of pancreatic diseases. The pneumopancreatotomography allowed the first direct proof of the pancreas; it has essentially enriched the anatomic conceptions. 10 to 15 years ago the splenoportography was the most evident examination of vessels. Today it is substituted by the coeliaco-mesentericography which in the venous phase delivers a splenoportogram and which allows to prove tumours rich in vessels with sufficient certainty. The investigation of the duodenum in hypotension improves the diagnostic of space-demanding processes on the surface of the head of the pancreas. The main value of scintigraphy consists in the exclusion of pancreatic diseases. The main value of scintigraphy consists in the exclusion of pancreatic diseases. The endoscopic retrograde cholangio-pancreaticography allows to shift the operative phase into the preoperative phase. Trijodated contrast remedies and developments concerning the technics of the apparatuses resulted in essential advantages. The pancreatic space which was empty 30 years ago is very much detected nowadays, though a contrast remedy which can be secreted by the pancreas needs still the detection.

Cholangiography↗

[Transcutaneous transsplenic catheterization of the splenic vein].

The authors have developed a method for transcutaneous transsplenic catheterization of the portal vein basin and used this method in clinical studies. This method permits a higher quality of spot splenoportography, selective catheterization and embolization of the gastric veins in bleedings from varicose veins of the esophagus in the patients in whom catheterization of the portal vein bain via the transcutaneous transhepatic approach is impossible because of liver tumors of occlusive stenotic deformation of the portal vein.

Adult↗

[Our experience in the management of portal cavernoma in children].

The authors present 14 patients affected with portal cavernoma, diagnosed and controlled over the last 12 years. The splenomegaly (60%) and the digestive hemorrhages (40%) were the initial study causes. The cavernoma appeared idiopathic in 10 patients or secondary either to a catheterization of umbilical vein (3 cases) or onfalitis (1 case). The first clinical manifestation oscillated between the ages of 3 months to nine years. The diagnosis was performed in the initial cases (4 cases) using splenoportography (3 cases) and superior mesenteric arteriography (SMA) in the other. In the remaining cases the ultrasonography together with the endoscopic evidence of the varies backed up the diagnosis. Before the intervention, a SMA was performed in all cases. The surgical decision was taken with regards to the persistence of the digestive bleeding after the start of medical treatment with propranolol, cimetidine, and sucralfate, along with the existence of dangerous varices. Eight patients were operated on, two by the Warren technique, four by mesocaval jugular graft interposition (MJI) and two by azigo portal disconnection (APD). The shunts initially controlled the bleeding in all cases. There was one rebleeding instance at eleven months in a patient operated on by MJI, and in another by APD. Four patients continue medical treatment without problems, and two are free of treatment. Our experience with varies sclerosis is limited to two cases, although since the beginning of the treatment, one year ago, neither of them has bled.

Angiography↗