Treatment of esophageal varices by sclerotherapy in children

Bleeding from esophageal varices may be a serious or lethal complication of portal hypertension in children. The standard therapy over the past 30 years has been to create a portosystemic shunt. In children physiologic complications leading to high rates of perioperative morbidity and early and late thrombosis with recurrence and encephalopathy have been common. Over a 42 month period, we treated six patients aged 5 to 18 years, with endoscopic injection of 3 percent sodium tetradecyl sulfate into the varix. Five patients required only injection, whereas one underwent direct oversewing of gastric varices followed by endoscopic sclerosis of the esophageal varices which remained. A total of 38 endoscopic procedures were performed. There has been complete cessation of bleeding in two patients, minimal subsequent bleeding in two others, and anemia requiring transfusion in the last two treated. These last two patients, although still requiring occasional transfusions, have been free from hypotensive or exsanguinating hemorrhage since beginning sclerotherapy. No deaths or serious complications were encountered in this series. Follow-up has ranged from 18 to 42 months (mean 26 months). The results of this trial suggest that repeated endoscopic sclerotherapy of varices, combined with operative oversewing of gastric varices when necessary, offered a viable alternative therapy for patients with esophageal varices.     

Surgical management of portal hypertension and esophageal varices: 10 year experience

The results of 157 operations performed for portal hypertension and esophageal varices on 148 patients at the Cleveland Clinic in the 10 year period between 1970 and 1980 are reported. One hundred four shunt procedures and 53 ligation procedures were performed. The overall operative mortality rate of 13 percent did not differ significantly from the 11 percent rate reported from this institution in 1971. A comparatively higher rate of recurrent variceal hemorrhage and a lower rate of encephalopathy reflected our increased use of selective shunts and ligation procedures. There was no improvement in overall long-term survival, which was approximately 50 percent.The two most important factors in predicting the results of all operations for esophageal varices continue to be assessment of preoperative liver function and the timing of the operation. The best results were obtained in patients with good liver function who had an elective operation. Our data suggest that the portacaval shunt is associated with a higher incidence of late mortality, largely as a result of liver failure; therefore, our preference now is to perform a distal selective splenorenal shunt procedure whenever possible. If a selective shunt procedure cannot be performed, we advocate either a mesocaval shunt or a ligation procedure, depending on patient risk and the suitability of veins for a shunt procedure.     

Management of esophageal varices in the Western world--the state of the art

The treatment by Western countries of bleeding from esophageal varices was reviewed from three definite viewpoints: prevention of first bleeding or prophylactic treatment, control of acute bleeding or emergency treatment, and prevention of rebleeding or elective treatment. Even though prophylactic surgery has been abandoned on the basis of several randomized studies, some authors still perform esophageal transection and report encouraging results. In emergency situations, the role of surgery has been limited by the prohibitive hospital mortality and by the introduction of vasoactive drugs and endoscopic sclerotherapy. Nevertheless, good immediate and long term results have been obtained in specialized centers in which bleeding patients undergo surgery no later than 8 hours after their admission. As regards the prevention of rebleeding, non selective portal decompression gives adequate protection against rebleeding, however, hepatoencephalopathy follows in considerable incidence. In order to avoid this complication, direct operations on varices have been performed, largely with good results. The Warren shunt offers results showing advantage over the non-selective shunt in the first postoperative period but later on, it behaves hemodynamically as a total shunt and the advantage is then cancelled. We report herein a review of the literature and also describe our personal experience with treating bleeding esophageal varices.     

Hyperparathyroidism, cirrhosis, and portacaval shunt. A new clinical syndrome

This is a report of six patients with cirrhosis of the liver in whom primary hyperparathyroidism occurred due to a solitary parathyroid adenoma 3 months to 9 years after undergoing emergency portacaval shunt for hemorrhage from esophageal varices. The presenting symptoms in all six patients were weakness and bone pain. Three patients had a bone fracture after insignificant trauma, one and probably two passed kidney stones, and a duodenal ulcer developed in two. Bone x-ray films showed generalized osteoporosis in all patients. Renal function and arterial blood pH were within normal limits in every patient. The diagnosis of primary hyperparathyroidism in each patient was based on repeated demonstrations of hypercalcemia, hypophosphatemia, and markedly elevated serum immunoreactive parathyroid hormone concentrations. In all six patients, removal of the parathyroid adenoma resulted in disappearance of symptoms; normalization of serum calcium, phosphorus, and immunoreactive parathyroid hormone levels; and in four of the six, improvement in radiographic evidence of osteoporosis during follow-up of from 1 to 6 years. The association of cirrhosis, portacaval shunt, and primary hyperparathyroidism has not been documented previously. Our six patients with primary hyperparathyroidism constitute 3.4 percent of 174 survivors of emergency portacaval shunt in a series of 264 unselected, consecutive patients with cirrhosis and bleeding esophageal varices. Hepatic osteodystrophy is known to have occurred in only 11 of these 174 survivors. Primary hyperparathyroidism may be a more common cause of hepatic osteodystrophy than has been previously recognized, and should be considered in patients with cirrhosis in whom weakness, bone pain, and bone demineralization develop, particularly if they have a portacaval anastomosis.     

Management of variceal hemorrhage in the potential liver transplant candidate

The increased utilization of liver transplantation raises new issues regarding the management of bleeding esophageal varices in patients who are or may become transplant candidates. Since December 1982, 53 patients were referred from a university hospital to distant liver transplant centers for transplantation. Transplants were performed in 37 patients; at last follow-up, 6 died before transplantation, 7 were awaiting transplantation, and 3 were declined. Of the 53 patients referred for transplantation, 22 (42 percent) had a history of variceal hemorrhage. Sclerotherapy was required in nine patients and portosystemic shunt in four patients. Variceal hemorrhage contributed to the deaths of three of the six patients who died before transplantation could be performed. Endoscopic sclerotherapy has become the mainstay of invasive therapy in most patients with bleeding esophageal varices. If sclerotherapy is unsuccessful in the arrest or control of variceal hemorrhage, the decision must be made whether to proceed with urgent liver transplantation or portosystemic shunt. Factors which influence this choice include the ability to stabilize an acutely bleeding patient, the hepatic reserve and general clinical stature of a patient, and the availability of a liver transplant center.     

Injection sclerotherapy of esophageal varices using ethanolamine oleate: A pilot study

Fifty-three patients with upper gastrointestinal bleeding and proven esophageal varices were treated by intravascular injection sclerotherapy of the varices using a mixture of ethanolamine oleate, bovine thrombin and cephalothin. An intraesophageal balloon was used to impede craniad flow during the injection. Except in three patients who failed to stop bleeding from nonvariceal lesions, sclerotherapy was 94 percent successful in controlling bleeding. The mortality rate was 21 percent, or less than half that in historical controls. The hospital mortality rate in sclerotherapy patients with ascites was 25 percent compared with 54 to 75 percent reported elsewhere. There has been no rebleeding from varices after the third treatment week in patients followed up for up to 14 months.     

Correlation between endoscopic and angiographic findings in patients with esophageal and isolated gastric varices.

BACKGROUND/AIM: The correlation between angiographic vascular patterns and endoscopic findings in portal hypertension is not sufficiently known, and knowledge of the vascular anatomy may contribute to an improvement in endoscopic embolization and transjugular retrograde obliteration procedures. We propose a new vascular map that should prove useful for this purpose. METHODS: Between April 1985 and December 1997 we performed percutaneous transhepatic portography in a selected group of 75 patients (16 women and 59 men), aged 43-71 years, from whom informed consent was obtained. All patients had been diagnosed endoscopically as having either esophageal or isolated gastric varices. According to the Child-Pugh classification, class A, B, and C cirrhosis was seen in 19, 40, and 16 patients, respectively. We created a vascular map of esophageal and isolated gastric varices, based on the opacification of the portal venous collaterals on percutaneous transhepatic portography. We compared the patients in both variceal groups in terms of portal venous pressure, main blood supply, and drainage routes. RESULTS: We found that the portal collateral system was divided into two systems: the portoazygos venous system and the portophrenic venous system. The former contributed to the formation of esophageal and cardiac varices and the latter to the formation of isolated gastric varices located at the fundus or at both the cardia and fundus. The left gastric vein participated as blood supply in 70% of the isolated gastric varices and in 100% of the esophageal varices (p < 0.01). The posterior gastric vein participated as blood supply in 70% of the isolated gastric varices and in 24% of the esophageal varices (p < 0.01). We classified the main blood drainage routes of isolated gastric varices functionally into three types: gastrorenal shunt (85%), gastrophrenic shunt (10%), and gastropericardiac shunt (5%). The portal venous pressure in patients with esophageal varices was 358 +/- 66 mm H(2)O, whereas in patients with isolated gastric varices it was 262 +/- 44 mm H(2)O (p < 0.01). CONCLUSION: We suggest that this new vascular map will be useful in endoscopic embolization and transjugular retrograde obliteration procedures for esophageal and isolated gastric varices.     

Transthoracic suturing of bleeding esophageal varices without esophagotomy. Preliminary report.

Suturing of bleeding esophageal varices by transthoracic esophagotomy is frequently followed by leakage in the suture line. To avoid this complication we suggest transesophageal suturing without esophagotomy. The technique was evaluated in five normal pigs and applied to two patients with bleeding esophageal varices. Esophagus both in the pigs and the patients tolerated the procedure well and the bleeding from the esophageal varices stopped. We suggest application of this procedure for bleeding esophageal varices instead of emergency portocaval shunt operation.     

Selective distal splenorenal shunt versus side-to-side portacaval shunt. Clinical results of a prospective, controlled study

A prospective, controlled study comparing the clinical results of the selective distal splenorenal shunt procedure and the side-to-side portacaval shunt procedure was undertaken in 1980. Ninety-three cirrhotic patients with previous episodes of bleeding from esophageal varices underwent a distal splenorenal shunt procedure (47 patients). The operative mortality rate was 2 percent in both groups. The intraoperative decrease of portal hypertension after the portacaval shunt procedure was higher than after the distal splenorenal shunt procedure (p less than 0.05), and in those with patent shunts, there was a 0 percent incidence of early variceal rebleeding after the portacaval shunt procedure compared with a 9 percent incidence after the distal splenorenal shunt procedure (p less than 0.05). Both shunts, however, had similarly satisfactory results in preventing long-term variceal rebleeding (portacaval shunt 2 percent and distal splenorenal shunt 0 percent). Postoperative ascites was more common after the distal splenorenal shunt procedure (58 percent versus 24 percent; p less than 0.01). Analysis of actuarial survival curves showed no difference between the two procedures. The incidences of long-term episodes of chronic encephalopathy were not statistically different after both procedures. The only three instances of severe encephalopathy occurred in patients with the portacaval shunt (p less than 0.05). The distal splenorenal shunt also seemed to have a less negative effect on postoperative liver function than the portacaval shunt. These data suggest that the selective shunt should be viewed as a first choice strategy in the treatment of portal hypertension.     

Management of esophageal varices in the western world —The state of the art—

The treatment by Western countries of bleeding from esophageal varices was reviewed from three definite viewpoints; prevention of first bleeding or prophylactic treatment, control of acute bleeding or emergency treatment, and prevention of rebleeding or elective treatment. Even though prophylactic surgery has been abandoned on the basis of several randomized studies, some authors still perform esophageal transection and report encouraging results. In emergency situations, the role of surgery has been limited by the prohibitive hospital mortality and by the introduction of vasoactive drugs and endoscopic sclerotherapy. Nevertheless, good immediate and long term results have been obtained in specialized centers in which bleeding patients undergo surgery no later than 8 hours after their admission. As regards the prevention of rebleeding, non selective portal decompression gives adequate protection against rebleeding, however, hepatoencephalopathy follows in considerable incidence. In order to avoid this complication, direct operations on varices have been performed, largely with good results. The Warren shunt offers results showing advantage over the non-selective shunt in the first postoperative period but later on, it behaves hemodynamically as a total shunt and the advantage is then cancelled. We report herein a review of the literature and also describe our personal experience with treating bleeding esophageal varices. This report is the gist of a paper read by S.S. at the 20th Annual Meeting of the Japanese Research Society for Portal Hypertension, Sendai, Japan, 1987     

The fate of esophageal varices following selective distal splenorenal shunt.

Esophagographic evaluation of the fate of esophageal varices after distal splenorenal shunt was obtained. The radiologist-observer was blinded as to the surgical status of the films under study. The results indicate that varices are likely to persist after surgery. However, the sizes of the varices are clearly diminished following selective distal splenorenal shunt. The incidence of postoperative bleeding has been low, 3.7% (2/54) or one episode for each 441 months of postoperative survival.     

Correlation between gastric microcirculation and mucosal injury after surgical therapy of esophageal varices

The correlation between gastric microcirculation and mucosal injury was studied in patients who underwent surgery for esophageal varices. Both mucosal and submucosal blood flow at the lower esophagus, gastric body and antrum was measured using hydrogen gas clearance method through endoscopy in 55 patients including 33 cirrhotics, 10 idiopathic portal hypertensive patients and 12 controls. In 20 cases with esophageal varices, 10 patients were treated with transabdominal esophageal transection (transection group) and 10 with left gastric vena caval shunt (shunt group). The patients with portal hypertension, showed a reduced blood flow in gastric mucosa but increased flow in the submucosa, as compared with the controls. When comparing the postoperative changes in gastric mucosal flow between the two groups, the transection group showed a reduction of mucosal flow by approximately 30% during surgery, and 20% for 4 weeks after operation. In shunt group, the mucosal flow was well preserved with reduction rate less than 10%. Postoperative mucosal injury was endoscopically and histologically found in almost all patients who showed a reduction rate of more than 20%. This study suggests that active protection against possible gastric mucosal lesion should be kept in mind in the setting of surgical therapy for esophageal varices.     

Esophageal transection may well be the approach of choice for patient with portal venous obstruction and esophageal varices

Thirty patients with esophageal varices, portal venous obstruction and a histologically proven normal liver underwent either one of 2 different types of surgery. Shunt surgery was performed on 20 patients: 9 had a mesocaval shunt, 3, a splenorenal shunt, 4, a left gastric venacaval shunt, and 4, a distal splenorenal shunt. Conversely, direct interruption was performed on the other 10 patients; 6 underwent an esophageal transection, and 4 underwent a resection of the proximal stomach. Re-hemorrhage occurred in 7 of the former 20 patients but not in any of the 10 on whom the direct interruption method was used. In 6 of these 7 patients who experienced rebleeding, subsequent direct interruption surgery led to control of the bleeding. One patient died of a variceal hemorrhage one month postoperatively. The total 10 year cumulative survival rate was 86.3 per cent. In the light of these findings, we believe that methods of direct interruption, such as esophageal transection, may well be the approach of choice for patients with esophageal varices caused by extrahepatic portal venous obstruction.     

Surgical versus non-surgical treatment in patients with esophageal varices--a prospective randomized study]

In Japan, non-shunting procedures and selective shunt such as esophageal transection (ET), and distal splenorenal shunt (DSRS) have been widely performed. A prospective randomized trial was done to assess the effects of EIS and DSRS for treating patients with esophageal varices. Ninety-six Japanese with good liver function (Child A or B) and large esophageal varices were randomly assigned to one of three groups given different treatments; (EIS, n = 32), (ET, n = 32) and (DSRS, n = 32). Five patients (15.6%) of the DSRS group has to be excluded from this study, because of severe chronic pancreatitis. No patient died within 30 days of the treatments. The 5-year cumulative bleeding rates were 0%, 4.3% and 12.1% in the EIS, ET and DSRS groups, respectively, with no statistical significances. In no case in the three groups did the death occur because of variceal bleeding. Nineteen patients died mainly due to the underlying liver disease; 5 in the EIS, 5 in the ET and 9 in the DSRS group. There was no statistically significant difference in the survival rates among the three groups. We conclude that EIS is a satisfactory alternative to ET or DSRS for the management of patients with large esophageal varices.     

Experience with 49 consecutive dacron interposition mesocaval shunts. A unified approach to portasystemic decompression procedures

In 1973, a plan was developed to manage all patients with bleeding esophageal varices who required portasystemic decompression with a Dacron interposition mesocaval shunt procedure. This paper has analyzed 7 years of such experience in 49 consecutive patients. Forty-eight were cirrhotic, 26 (53 percent) required emergency shunting, and 6 were in Child's class A, 13 were in class B and 30 were in class C. Overall, operative mortality was 11 of 49 patients (22.4 percent). Ten of the 11 deaths were of patients in class C and all but one of the patients (90.9 percent) had undergone an emergency operation. Sixteen patients had episodes of significant postshunt recurrent bleeding. Such bleeding occurring within 30 days of operation was a function of severe hepatic, hematologic, and general metabolic derangements. Recurrent hemorrhage occurring after discharge was a function of shunt thrombosis (four patients) or alcoholic recidivism. Twelve patients (31.6 percent) had significant postshunt encephalopathy. Cumulative 5 year survival was 49.3 percent. These data emphasize the high risk of mortality in class C patients operated on an emergency basis. Postoperative encephalopathy is a significant problem with this shunting procedure.     

Esophageal transection may well be the approach of choice for patients with portal venous obstruction and esophageal varices

Thirty patients with esophageal varices, portal venous obstruction and a histologically proven normal liver underwent either one of 2 different types of surgery. Shunt surgery was performed on 20 patients: 9 had a mesocaval shunt, 3, a splenorenal shunt, 4, a left gastric venacaval shunt, and 4, a distal splenorenal shunt. Conversely, direct interruption was performed on the other 10 patients: 6 underwent an esophageal transection, and 4 underwent a resection of the proximal stomach. Re-hemorrhage occurred in 7 of the former 20 patients but not in any of the 10 on whom the direct interruption method was used. In 6 of these 7 patients who experienced rebleeding, subsequent direct interruption surgery led to control of the bleeding. One patient died of a variceal hemorrhage one month postoperatively. The total 10 year cumulative survival rate was 86.3 per cent. In the light of these findings, we believe that methods of direct interruption, such as esophageal transection, may well be the approach of choice for patients with esophageal varices caused by extrahepatic portal venous obstruction.     

Results of esophageal transection for esophageal varices: Experience in 100 cases

The results of transthoracic esophageal transection in 100 patients with esophageal varices are described. There were 11 operative deaths in this series, and the majority of patients died from hepatic failure. Esophageal varices disappeared completely in 81 percent of the patients and faded in 18 percent. Post-transection rebleeding was observed in six cases. There were 16 late deaths, caused mainly by hepatic failure and hepatoma. The 3 year and 5 year survival rates including operative deaths were 70 and 58 percent, respectively. Based on the low operative mortality rate, the efficacy in eliminating varices and the sufficient survival rate, it is presumed that esophageal transection is the most suitable operation for esophageal varices, even in poor risk patients.     

Hepatofugal portal blood flow in hepatic cirrhosis.

A variety of indirect techniques has been claimed to provide evidence of spontaneous reversal of portal blood flow in hepatic cirrhosis but the existence of the phenomenon has been doubted by some who do not accept the validity of the indirect evidence. There are few reports of the demonstration of hepatofugal portal flow by selective hepatic arteriography, which is the only acceptable technique. We report three patients with histologically confirmed cirrhosis in whom hepatofugal portal blood flow was unequivocally demonstrated by arteriography, in whom no surgical portosystemic shunt had been performed and in whom there was no evidence of the Budd-Chiari Syndrome or hepatoma, situations accepted as associated with reversed portal blood flow. Theoretical considerations suggest that shunt surgery for bleeding esophageal varices should not be ruled out on the grounds of hepatofugal portal flow. However, end-to-side portacaval anastomosis and distal splenorenal shunt might predispose to the early redevelopment of esophageal varices when reversed portal flow is present. Side-to-side portacaval and conventional splenorenal shunts might be preferable in having less effect on hepatic parenchyma perfusion than when orthograde portal flow in the case.     

Long-term survival after emergency portacaval shunting for bleeding varices in patients with alcoholic cirrhosis

Since 1963, a prospective evaluation of the emergency portacaval shunt procedure has been conducted in 264 unselected patients with cirrhosis and bleeding varices who underwent operation within 8 hours of admission to the emergency department. Of 153 patients who underwent operation 10 or more years ago, 45 (29 percent) have survived from 10 to 22 years and their current status is known. On admission, 40 percent of the long-term survivors had jaundice, 44 percent had ascites, 13 percent had encephalopathy (with an additional 9 percent with a history of encephalopathy), 29 percent had severe muscle wasting, and 82 percent had a hyperdynamic state. There were 9 Child's class A patients, 33 Child's class B patients, and 3 Child's class C patients. At operation, all patients had portal hypertension which was reduced by the shunt to a mean corrected free portal pressure of 18 mm saline solution. The emergency portacaval shunt procedure permanently controlled variceal bleeding. None of the patients bled again from varices, and the shunt remained patent throughout life in every patient. Encephalopathy did not affect 91 percent of the patients, but was a recurrent problem in 9 percent, usually related to the use of alcohol. Lifelong abstinence from alcohol occurred in 58 percent of the long-term survivors, but 11 percent resumed regular drinking and 31 percent consumed alcohol occasionally. Liver function declined compared with preoperative function in only 18 percent of the patients, almost always because of alcohol use. Ten years after operation, 73 percent of the patients were in excellent or good condition, and 68 percent were gainfully employed or engaged in full-time housework. Comparison of the 10 to 22 year survivors with our early group of 180 patients reported previously and our recent group of 84 patients showed no significant differences in preoperative or operative data. The single factor that appeared to influence long-term survival was resumption of regular use of alcohol. We conclude that the emergency portacaval shunt procedure, by preventing hemorrhage from varices, results in prolonged survival and an acceptable quality of life for a substantial number of patients with advanced alcoholic cirrhosis.     

Enhanced survival in patients with variceal bleeding after elective portal decompression

In a retrospective study of 49 patients who had bled from esophageal varices but who survived and did not require emergency portal decompression, 24 patients were discharged without surgical consultation and 25 underwent portal decompression using a small-stoma portacaval shunt. Shunted and nonshunted patients were similar both demographically and in clinical and chemical criteria for preoperative hepatocellular function. Among nonshunted patients, survival was 33 percent during a follow-up period of up to 39 months, whereas shunted patients had an 83 percent survival during a follow-up period of up to 43 months. This difference in survival was statistically significant (p = 0.03). Shunted patients showed a 10.5 percent incidence of hepatic encephalopathy. We conclude that survival after variceal hemorrhage may be significantly increased by a portacaval shunt procedure, especially if it is carried out semielectively in stable patients.