Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia

Sixty-one children who have survived 2.5 years or more after corrective surgery for biliary atresia were prospectively followed by endoscopy. Esophageal varices were detected in 41 patients (67%), 17 of whom (28%) had experienced episodes of variceal hemorrhage. Control of variceal bleeding was achieved by endoscopic injection sclerotherapy in all but one child who died from hemorrhage before the completion of treatment. Complications of the technique comprised episodes of bleeding before variceal obliteration (7), esophageal ulceration (5), and stricture (3). These resolved with conservative management and without long-term sequelae. During a mean follow-up period of 2.8 years after variceal obliteration, rebleeding from recurrent esophageal varices developed in only one child and responded to further sclerotherapy. These results are better than those following surgical procedures for portal hypertension in biliary atresia, and therefore endoscopic sclerotherapy is recommended as the treatment of choice.     

Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality

Background

Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up.

Method

We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction.

Results

Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count.

Conclusion

Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence.     

Portasystemic shunting remains the procedure of choice for control of variceal hemorrhage

We reviewed 108 patients (50% Child's C) who had been admitted with acutely bleeding esophageal varices to compare the efficacy of operative (portasystemic shunt) therapy with the reported efficacy of sclerotherapy. In the patients with shunts the early mortality (preoperative plus operative) was 13% and late mortality was 23%. Survival at five years was 50%. Recurrent variceal bleeding was seen in 4% of the patients. Procedure-related mortality and variceal rebleeding rates for the shunt group were respectively 50% and 8% of that reported for sclerotherapy. Variceal rebleeding in the sclerotherapy group required approximately 7 units of blood per episode. We concluded that immediate attempts at control of hemorrhage followed by portasystemic shunting remains the therapy of choice for these patients.     

A 15-year experience of injection sclerotherapy in adult patients with extrahepatic portal venous obstruction.

OBJECTIVE: The authors report a 15-year experience with injection sclerotherapy in the management of adult and teenage patients with esophageal varices due to extrahepatic portal venous obstruction (EHPVO). SUMMARY BACKGROUND DATA: Extrahepatic portal venous obstruction is an uncommon cause of esophageal varices and is associated with normal liver function. Effective control of variceal bleeding is the major factor influencing survival. The results of surgery have been unsatisfactory, and therefore, more conservative management policies have been adopted. METHODS: Fifty-five patients with proven EHPVO underwent repeated injection sclerotherapy via either a modified rigid esophagoscope under general anaesthesia or a fiber-optic endoscope under light sedation, using ethanolamine oleate as the sclerosant. RESULTS: Esophageal varices were eradicated in 44 patients after a median number 6 injections (range 1-17) over a mean of 12.5 months (range 1-48). The mean follow-up was 6.8 years (range 1.1-14.6 years). Eleven patients were admitted on eighteen occasions with bleeding from esophageal varices before eradication and there were seven bleeding episodes in six patients from recurrent varices after initial eradication. Complications related to sclerotherapy included injection site leak (6), stenosis (11) and mucosal ulceration (32) during 362 injection sclerotherapy episodes. Four patients died during the study period. CONCLUSIONS: Injection scelotherapy is the treatment of choice in most patients with EHPVO.     

Portal hypertension and bleeding esophageal varices

Summary Bleeding from esophageal varices exacts a high mortality and extraordinary societal costs. Prophylaxis—medication, sclerotherapy, or shunt surgery to prevent an initial bleeding episode—is ineffective. In patients who have bled from varices, endoscopic injection sclerotherapy can control acute bleeding in more than 90% of patients. Because recurrent bleeding frequently occurs and survival without definitive therapy is dismal, selection of a permanently effective treatment is mandatory once variceal bleeding has been controlled.Long-term injection sclerotherapy can be performed in compliant patients; it is relatively safe but is associated with a 30–50% rebleeding rate. Betablockers significantly reduce portal pressure and recurrent bleeding but have not been shown to diminish mortality from BEV. Portal decompressive surgery permanently halts bleeding in more than 90% of patients; the risk of operative mortality is high in decompensated cirrhotics, and long-term complications of encephalopathy and accelerated liver failure may limit indications for shunt surgery to good-risk cirrhotics who are not liver transplant candidates. Devascularization procedures have a low operative mortality and encephalopathy rate but unacceptably high rates of recurrent bleeding.Liver transplantation is curative therapy for bleeding esophageal varices and the associated underlying hepatic dysfunction; cost and availability of donor organs generally limit its use in this setting to variceal bleeders with end-stagè liver disease not associated with active alcoholism.     

The endoscopic and surgical management of portal hypertension in children: analysis of 123 cases

Since 1973, 178 children with portal hypertension (PH) have been seen at Instituto da Crian?a of the University of S?o Paulo Medical School. Fifty-five of these children were excluded from this analysis for various reasons, including no treatment required, death before treatment, or incomplete data. From the remaining 123 children with esophageal varices, only 96 (76.1%) of them had at least one episode of upper gastrointestinal hemorrhage. Eighty-eight children were submitted to injection sclerotherapy; 26 treated prophylactically, and 62 for treatment of previous bleeding. Eleven (42.3%) children from the prophylactic group bled from esophageal varices during the treatment. They were all successfully managed thereafter. Satisfactory results were achieved in 53 (85.4%) children in the therapeutic group. Twenty-eight (45.1%) children had at least one episode of bleeding after beginning of sclerotherapy, 19 of whom eventually had successful control of the variceal bleeding. From 1973 to 1984, distal splenorenal shunt (DSS) was the procedure of choice for the treatment of bleeding esophageal varices. Forty-two children have undergone DSS during this period. Only one child was shunted prophylactically. Since 1985, injection sclerotherapy has been the first choice for the treatment and only seven children with sclerotherapy failure have since been treated by DSS. Characteristically these children had very similar splenoportographic pattern with huge esophageal and gastric varices and deviation of portal vein blood flow toward the left gastric vein.(ABSTRACT TRUNCATED AT 250 WORDS)     

Gastric bleeding after endoscopic injection sclerotherapy for esophageal varices may be fatal.

Among 457 Japanese cirrhotic patients with esophageal varices, 28 (6%) bled from the upper gastrointestinal tract after the initial session of endoscopic injection sclerotherapy (EIS); 13 bled during the course of repeated EIS and 15 bled mainly from gastric lesions after eradication of the varices. Of these 28 patients, bleeding from gastritis occurred in 13 (46%), from esophageal varices in 10 (36%), from gastric varices in 4 (14%) and from gastric ulcer in one (4%). Six of 13 patients with gastritis-related bleeding and 3 of 4 patients with gastric variceal bleeding died of uncontrollable hemorrhage complicated liver failure, while 9 of 10 patients with esophageal variceal bleeding were controlled and reinjection was feasible. Ten (36%) of the 28 patients, with Child's grade B or C and severe ascites, died, mainly following bleeding from gastric lesions. This study shows that bleeding from gastric lesions after EIS can be uncontrollable and fatal in patients with poor liver function.     

Variceal recurrence, rebleeding, and survival after endoscopic injection sclerotherapy in 287 alcoholic cirrhotic patients with bleeding esophageal varices

OBJECTIVE: This study tested the validity of the hypothesis that eradication of esophageal varices by repeated injection sclerotherapy would reduce recurrent variceal bleeding and death from bleeding varices in a high-risk cohort of alcoholic patients with cirrhosis. SUMMARY BACKGROUND DATA: Although banding of esophageal varices is now regarded as the most effective method of endoscopic intervention, injection sclerotherapy is still widely used to control acute esophageal variceal bleeding as well as to eradicate varices to prevent recurrent bleeding. This large single-center prospective study provides data on the natural history of alcoholic cirrhotic patients with bleeding varices who underwent injection sclerotherapy. METHODS: Between 1984 and 2001, 287 alcoholic cirrhotic patients (225 men, 62 women; mean age, 51.9 years; range, 24-87 years; Child-Pugh grades A, 39; B, 116; C, 132) underwent a total of 2565 upper gastrointestinal endoscopic sessions, which included 353 emergency and 1015 elective variceal injection treatments. Variceal rebleeding, eradication, recurrence, and survival were recorded. RESULTS: Before eradication of varices was achieved, 104 (36.2%) of the 287 patients had a total of 170 further bleeding episodes after the first endoscopic intervention during the index hospital admission. Rebleeding was markedly reduced after eradication of varices. In 147 (80.7%) of 182 patients who survived more than 3 months, varices were eradicated after a mean of 5 injection sessions and remained eradicated in 69 patients (mean follow-up, 34.6 months; range, 1-174 months). Varices recurred in 78 patients and rebled in 45 of these patients. Median follow-up was 32.3 months (mean, 42.1 months; range, 3-198.9 months). Cumulative overall survival by life-table analysis was 67%, 42%, and 26% at 1, 3, and 5 years, respectively. A total of 201 (70%) patients died during follow-up. Liver failure was the most common cause of death. CONCLUSION: Repeated sclerotherapy eradicates esophageal varices in most alcoholic cirrhotic patients with a reduction in rebleeding. Despite control of variceal bleeding, survival at 5 years was only 26% because of death due to liver failure in most patients.     

The fundal pile: bleeding gastric varices

Bleeding from esophageal varices is a common cause of major upper gastrointestinal tract blood loss in children with portal hypertension but usually ceases spontaneously or is satisfactorily managed by nonoperative measures. Massive hemorrhage from gastric fundal varices may be difficult to control with compression and sclerotherapy; in these cases, a direct surgical approach may be indicated. Since 1984, 27 children have undergone aggressive injection sclerotherapy for bleeding esophageal/gastric varices. Nine (6 with portal vein thrombosis) bled from gastric fundal varices. In 5 of these, medical management and sclerotherapy failed to control the acute bleed. In all 5 there was "rupture" of a large gastric fundal varix or "pile" and bleeding was controlled at emergency laparotomy by underrunning the varices through a high anterior gastrotomy. Four have subsequently been successfully managed by continued sclerotherapy and one patient with cirrhosis has died of liver failure. In 3 of the survivors both esophageal and gastric fundal varices have been completely obliterated. No further life-threatening hemorrhage has occurred in any case during a follow-up period of 1 to 5 years. Bleeding from gastric varices is more common than previously recorded and more difficult to control by nonoperative management, including injection sclerotherapy. In uncontrolled hemorrhage from gastric varices, surgical underrunning offers a means of providing initial control. Thereafter, the inevitable variceal recurrence may be successfully treated with sclerotherapy.     

内镜下硬化与套扎治疗食管静脉曲张破裂出血疗效比较

目的：对比内镜下硬化治疗（EIS）、套扎治疗（EVL）及套扎联合硬化治疗（ESL）3种方法对食管静脉曲张破裂出血的临床疗效。方法：回顾分析中日友好医院消化内科2001—2005年内镜下治疗肝硬化单纯食管静脉曲张破裂出血149例,其中EIS46例、EVL32例、ESL71例,对3种方法的止血率、静脉曲张消失率及再出血率进行比较。结果：3种治疗方法止血率均在90%以上;静脉曲张消失率分别为EIS80.4%、EVL68.8%、ESL87.3%;2年内再出血率分别为EIS52.2%、EVL59.3%、ESL43.6%,差异无统计学意义（P〉0.05）。结论：内镜下EIS、EVL及ESL治疗肝硬化食管曲张静脉出血均可达到较好效果,临床实践中可结合患者实际情况综合考虑后选择。     

The current role of devascularization and transection procedures in portal hypertension]

It is not clear which theory should be used in patients with bleeding esophageal varices that are not controlled by emergency endoscopic sclerotherapy. Definitive hemostasis is the key to successful therapy of variceal bleeding. Recurrence of haemorrhage in patients with portal hypertension is the most feared life threatening complication. Based on our management of 658 patients with esophageal varices and the availability of treatment options at our institution, the strategy of management of uncontrollable variceal haemorrhage by endoscopic sclerotherapy has evolved. Bleeding was controlled in 64 liver cirrhosis (100%) by devascularization and transection procedures and 50 patients (78%) survived to leave the hospital including 43 of 64 patients (67%) with Child grade C liver cirrhosis. Cumulative rebleeding rate at 10 years following emergency surgery was 3% (2/64). It is associated with a lower morbidity and mortality as well as a lower incidence of subsequent encephalopathy. We suggest that emergency transection and devascularization is an effective salvage treatment for the endoscopic sclerotherapy failed group.     

Results of a Modified Sugiura's Devascularisation in the Management of “Unshuntable” Portal Hypertension

The results of a modified Sugiura devascularisation procedure were assessed in 14 patients with thrombosis of the portal and splenic vein requiring surgery for variceal hemorrhage, with no vein suitable for orthodox shunt surgery. The venous anatomy was determined by ultrasonography with Doppler studies and portovenography. Liver biochemistry as well as liver architecture on histopathology was normal in all. The surgery was elective in 9 cases for documented bleed from diffuse fundal gastric varices (FGV) and emergency in 5 cases, 3 having bleeding FGV and 2 for failure of emergency esophageal variceal sclerotherapy. All were subjected to a transabdominal extensive devascularisation of the upper two third of the stomach and lower 7–10cm of the esophagus. Stapled esophageal transection (n=11) or esophageal variceal under-running (n=1) was performed in all with esophageal varices. FGV were underrun. Follow up endoscopies were done six monthly. There were 9 males and 5 females with a mean age of 17.2 years (SD 12.8). There was no operative mortality. Acute variceal bleeding was controlled in all patients. Over a mean follow up of 38 months, all but one remain free of recurrent bleeding. We conclude that a modified Sugiura devascularisation procedure is effective in the immediate and medium term control of variceal bleeding in patients with “unshuntable” portal hypertension.     

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.     

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.     

Transabdominal Extensive Esophagogastric Devascularization with Gastroesophageal Stapling for Management of Noncirrhotic Portal Hypertension: Long-term Results

Outside Japan portosystemic shunts have been favored as the surgical procedure of choice for the management of portal hypertension of noncirrhotic etiology. Devascularization procedures have resulted in high rebleed rates probably owing to a limited extent of devascularization. We performed this study to assess the efficacy of our modification of Sugiura's procedure for long-term control of variceal bleeding in patients with noncirrhotic portal hypertension. Forty-six patients with extrahepatic portal venous obstruction (EHPVO) and 22 with noncirrhotic portal fibrosis (NCPF) were subjected to transabdominal extensive esophagogastric devascularization with esophageal or gastric stapled transection (modified Sugiura's procedure), 38 in an emergency situation and 30 electively. Follow-up endoscopies were performed every 6 months. Operative mortality, morbidity, variceal status, and causes of recurrent bleeding were evaluated. The postoperative mortality was 4%. Early procedure-related complications were seen in 6%, and esophageal strictures formed in 7 of 45 survivors undergoing esophageal transection (15%). Over a mean ± SD follow-up of 53 ± 34 months, 95% of patients were free of varices. Seven survivors (11%) had a rebleed, but only 5% were due to varices (two esophageal, one gastric). Six (9%) patients developed gastropathy. The 5-year survival was 88%. The modified Sugiura's procedure is safe and effective for long-term control of variceal bleeding especially in the emergency setting and in patients with anatomy unsuitable for shunt surgery or if surgical expertise for a shunt operation is not available.     

The surgeon''s role in the management of portal hypertension.

Patients with portal hypertension are referred to surgeons for several reasons. These include the management of continued active variceal bleeding; therapy after a variceal bleed to prevent further recurrent bleeds; consideration for prophylactic surgical therapy to prevent the first variceal bleed; or, rarely, an unusual cause of portal hypertension which may require some specific surgical therapy. Injection sclerotherapy is the most widely used treatment for both acute variceal bleeding and long-term management after a variceal bleed. Unfortunately it has probably been overused in the past. The need to identify the failures of sclerotherapy early and to treat them by other forms of major surgery is emphasized. The selective distal splenorenal shunt is the most widely used portosystemic shunt today, particularly in nonalcoholic cirrhotic patients. The standard portacaval shunt is still used for the management of acute variceal bleeding as well as for long-term management, particularly in alcoholic cirrhotic patients. For acute variceal bleeding the surgical alternative to sclerotherapy or shunting is simple staple-gun esophageal transection, whereas in long-term management the main alternative is an extensive devascularization and transection operation. Liver transplantation is the only therapy that cures both the portal hypertension and the underlying liver disease. All patients with cirrhosis and portal hypertension should be assessed as potential liver transplant recipients. If they are candidates for transplantation, sclerotherapy should be used to treat bleeding varices whenever possible, as this will interfere least with a subsequent liver transplant.     

Partial portacaval shunt: renaissance of an old concept.

BACKGROUND. Partial diversion of the portal system aims to reduce portal pressure sufficiently to prevent variceal hemorrhage but still maintain adequate hepatic portal flow. METHODS. Partial portacaval shunts were performed in 25 patients with cirrhosis with portal hypertension and esophageal varices, either as a primary procedure (n = 16) or for failure of endoscopic sclerotherapy (n = 9), with ringed polytetrafluoroethylene prostheses (8, 10, or 12 mm). RESULTS. All patients have now been followed up for at least 1 year. The operative mortality rate (2 months) was 4%. In 24 patients who survived beyond the initial perioperative period, there was no recurrence of variceal bleeding. Cumulative shunt patency (up to 4 years) is 96%. Acute encephalopathy was detected in two patients (8%), but no patients had signs of chronic encephalopathy. Intraoperative pressure measurements revealed a significant correlation between decreasing diameter of the graft and the percentage reduction of the portacaval pressure gradient. Selective angiography, performed 1 year after surgery, revealed that hepatopetal flow was maintained in 70% of patients with a 10 mm shunt. CONCLUSIONS. It is possible to achieve a partial portacaval shunt, related to the diameter of the prosthesis, that preserves hepatopetal flow in the majority of patients and is associated with a very low incidence of shunt thrombosis. This effectively prevents recurrent variceal bleeding and significant postoperative encephalopathy. The performance of subsequent orthotopic liver transplantation is not compromised. The technique is recommended, either as a primary procedure or when sclerotherapy has failed, in patients with good liver function who are unlikely to require early liver transplantation (grade A and some grade B cirrhosis).     

Evaluation of propranolol for prevention of recurrent bleeding from esophageal varices between sclerotherapy sessions

Forty-one patients admitted with first episode of bleeding from esophageal varices were enrolled in a trial of the efficacy of oral propranolol to prevent rebleeding during the course of endoscopic sclerotherapy until obliteration. Single-blind randomization to sclerotherapy alone or with propranolol was used. At monthly endoscopy the varices were injected with 1% Aethoxysclerol until obliteration. If bleeding recurred, additional sclerotherapy was given. There was no intergroup difference in time to eradication of varices (8.1 vs. 7.7 months). The cumulative number of bleedings from varices and from distal esophageal ulcerations was identical in the two study groups. Five patients in the control group but only one in the propranolol group died of bleeding in the study period, a difference of only borderline significance (chi 2 = 4.08, df = 1). There were no specific side effects of propranolol. Thus propranolol did not significantly reduce the frequency of rebleeding until variceal obliteration, but could have had some influence on the gravity of rebleeding.     

Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial

BACKGROUND/PURPOSE: Experience using endoscopic prophylactic sclerotherapy (PS) is restricted to adult patients and has led to conflicting results. There has not been a randomized, controlled study on the use of PS in children. The purpose of this study is to evaluate prospectively the value of PS to prevent the first hemorrhage from esophageal varices in children with portal hypertension and to assess the effect of PS on survival rate. METHODS: In a controlled, prospective, computer-based randomized trial, the effectiveness of PS was analyzed in 100 consecutive children allocated to a group receiving sclerotherapy (n = 50) or to a control group (n = 50) subjected only to regular clinical and endoscopic examinations. Clinical characteristics in both groups were similar. The minimum follow-up period was at least 18 months after the cessation of the sessions of sclerotherapy. RESULTS: After a median follow-up of 4.5 years, PS eliminated the esophageal varices in 47 of 50 (94%) patients but only 38 (76%) of them do not present upper digestive hemorrhage. Before complete obliteration of the varices, upper gastrointestinal bleeding occurred in 12 patients (24%). Six children (12%) had gastric varices, 3 of 6 of whom (50%) bled. Congestive hypertensive gastropathy was observed to occur in 8 (16%) patients, 4 of 8 of which (50%) had hemorrhagic episodes. Two patients bled from undetermined cause. In the control group, only 29 (58%) children remained free from esophageal variceal bleeding and 26 (52%) from any upper gastrointestinal bleeding (P<.05). During the follow-up period, the development of gastric varices was observed in 5 (10%) patients (P>.05) and of congestive hypertensive gastropathy in only 3 (6%) patients (P<.05), but none of them bled. PS does not improve survival rate. CONCLUSIONS: In children with cirrhotic and noncirrhotic portal hypertension, PS reduces the overall incidence of bleeding from esophageal varices that were eradicated in 94% of cases. The source of bleeding has been different in each group, being predominantly from esophageal varices in the control group and from the stomach in the prophylaxis group. When applied with appropriate technique, PS is a safe procedure with a low incidence of minor complications. PS does not change the incidence of gastric varices but increases the development of congestive hypertensive gastropathy. PS increases the risk of bleeding from the naturally formed gastric varices and from congestive hypertensive gastropathy. PS does not affect survival rate.     

Sugiura procedure in portal hypertensive children

Bleeding from esophageal varices is an important cause of morbidity and mortality in children with portal hypertension. The treatment protocol is planned according to the etiologic factors underlying the portal hypertension, which may be either intrahepatic or extrahepatic. Although portasystemic venous shunt operations were common previously, they are now regarded as nonphysiologic and are rarely used because of their unexpected results and complications. Today, in many centers, endoscopic procedures have become the first-step treatment modality in bleeding esophageal varices. More complicated surgical procedures, such as devascularization procedures in extrahepatic portal hypertension, and liver transplantation in patients with failing liver, should be performed when conservative measures fail. We followed up 69 patients with portal hypertension with endoscopic sclerotherapy in our department. Here we present a retrospective evaluation of the effect of the Sugiura operation on the prognosis of 12 children (6 with extrahepatic and 6 with intrahepatic portal hypertension) who were not responsive to the sclerotherapy program. No rebleeding was seen in 9 of the 12 (75%) patients after the procedure, and the mortality rate in this series was 1 of 12 (8.3%); this patient died of hepatic failure. Received: November 7, 2000 / Accepted: January 25, 2001