H-type shunt with an autologous venous graft for treatment of portal hypertension in children

From 1981 to 1987, 86 children aged 16 months to 16 years underwent a portosystemic shunt procedure using an autologous venous graft (internal jugular vein in 80 cases). Fifty-five mesocaval, 20 splenorenal, 4 portacaval, and 7 makeshift shunts were constructed. The indication for shunting was an extrahepatic portal obstruction in 59 cases, intrahepatic portal obstruction in 23 cases (including 6 cases of congenital hepatic fibrosis), and Budd-Chiari syndrome in 4 cases. One patient of the latter group died early from intractable ascites with a nonfunctioning shunt, and a second child died 2 months after operation from unknown reasons with a patent shunt. With a follow-up over 1 year for 58 of the 84 survivors, 78 successes and 6 failures were recorded according to the clinical outcome and the findings of ultrasonic and endoscopic examinations. Three of the six children with a failed shunt have been submitted to a second successful H-type shunt operation. No case of encephalopathy was recorded in this series. Thus, with an approximate success rate of 95%, the H-type shunt with a venous graft should be recommended for treatment of portal hypertension of extrahepatic origin, especially in young children.     

Portosystemic shunts in children: a 15-year experience

BACKGROUND: The role of portosystemic shunt (PSS) in children with portal hypertension has changed because of acceptance of liver transplantation and endoscopic hemostasis. We report our experience with PSS, mainly the distal splenorenal shunt, to define its role in the management of variceal bleeding. STUDY DESIGN: From 1987 to 2002, 20 children with variceal bleeding after endoscopic therapy underwent PSS. Patient and database records were reviewed. RESULTS: There were 14 boys and 6 girls; mean age was 11 years (range 3 to 18 years). Seventeen distal splenorenal and three mesocaval venous interposition shunts were performed. There was no operative mortality, 19 patients were alive at a median followup of 31 months (range 4 to 168 months) without evidence of recurrent gastrointestinal bleeding. One patient underwent transplantation 2 years after PSS and 1 patient died of hepatic failure while awaiting transplantation. The cause of portal hypertension was portal vein thrombosis (n = 13), biliary atresia (n = 3), congenital hepatic fibrosis (n = 2), hepatitis C cirrhosis (n = 1), and Budd-Chiari syndrome (n = 1). Eighteen children were Child-Turcotte-Pugh class A and the remaining two were class B. One patient had two episodes of hematemesis after PSS. Two patients had worsening ascites. One patient had mild encephalopathy and one patient had shunt stenosis requiring angioplasty. CONCLUSIONS: PSS is a safe and durable therapy for pediatric patients with portal hypertension. Liver transplantation should be reserved for children with poor synthetic function associated with variceal bleeding. PSS may also serve as a bridge to transplantation in patients with preserved hepatic function. PSS, in particular the distal splenorenal shunt, has produced excellent results. This experience challenges the need for alternative forms of portal decompression.     

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.     

联合手术治疗儿童门静脉海绵样变性肝前性门静脉高压症

目的评估联合手术治疗儿童门静脉海绵样变性引起的肝前性门静脉高压症的疗效。方法2例门静脉海绵样变性和反复上消化道出血的患儿行脾切除脾肾静脉分流加贲门周围血管离断术;1例因断流术后再出血,行肠系膜上-腔静脉分流加贲门周围血管离断术,分析这3例的临床疗效。结果手术并发症和手术死亡率为0,随访13~31个月,肝功能正常,贫血纠正。无再出血、门静脉高压性胃病和肝性脑病。结论联合手术,特别是脾切除脾肾静脉分流加贲门周围血管离断术,安全、有效,适用于各种血流动力学状态,应成为治疗儿童门静脉海绵样变性引起的肝前性门静脉高压症的首选术式。     

间接门静脉造影对诊治肝前型门静脉高压症的意义

目的：探讨间接门静脉造影对诊断肝前型门静脉高压症以及对手术治疗的指导意义。方法：回顾分析近3年来收治的肝前型门脉高压症11例患者的临床资料，依照间接门静脉造影和/或彩超结果，全部确定为肝前型门脉高压症。施肠系膜上静脉-下腔静脉转流术5例，门静脉-下腔静脉转流术1例，脾切除、脾静脉-肾静脉转流术2例，肠系膜上静脉门静脉探查术、门奇静脉断流术1例，未行手术2例。结果：经转流手术治疗的患者术后脾亢消失，未再发生上消化道出血。结论：间接门静脉造影检查是诊断肝前型门脉高压症的金标准，对选择手术治疗方法有指导意义。     

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.     

Long-term results of side-to-side and H-graft mesocaval shunt

From January, 1966 to January, 1988, mesocaval shunt was performed on 47 patients with the variceal hemorrhage secondary to portal hypertension. Of these, 30 patients underwent side-to-side mesocaval shunt, the remaining 17 had H-graft mesocaval shunt. Postoperative follow-up averaged 6 years and 11 months for the patients surviving operation. The rebleeding rate was 21.1%, and the shunt related encephalopathy rate 25.5%. The 5-, 10-, 15- and 20-year survival rates of the whole series were 65.8%, 51.8%, 37.5% and 20.0% respectively. We conclude that mesocaval shunt is the procedure of choice for treatment of variceal bleeding, especially for the control of postoperatively recurrent variceal bleeding.     

Hyperdynamic circulation in the left gastric venous area in patients with portal hypertension: angiological assessment

The time of appearance of the left gastric vein on serial celiac arteriograms in patients with portal hypertension and esophageal varices was compared with that of the portal vein to assess regional hemodynamics in the left venous portion of the stomach, an area located in close proximity to the varices. In two thirds of all the patients with cirrhosis or non-cirrhotic idiopathic portal hypertension (IPH), the left gastric vein was visualized earlier or simultaneously than or with the portal vein, while in all but one patient with prehepatic portal obstruction, there was a delayed opacification of the left gastric vein. These results suggest the presence of a hyperdynamic circulatory state which promotes venous hypertension in the left gastric venous area of the stomach of a considerable number of patients with cirrhosis or IPH. In such a hemodynamic state, selective decompression of varices can be achieved by a left gastric venous caval shunt.     

肝前性门脉高压症

目的 探讨肝前性门脉高压症的临床特点及治疗方法的选择。方法 ２例非肝硬化门脉纤维化,８例肝外门脉阻塞,隔膜切除１例,自体血管钎肠系膜上静脉→门静脉搭桥１例,肠腔分 ３例,脾切除脾脏脾肾分流５例。结果 １例死亡,９例治愈。结果 肝前生门脉高压症在临床表面方面有许多不同,分流术可作为基本手术,病变血管的矫正术更合理。     

Hyperdynamic circulation in the left gastric venous area in patients with portal hypertension: Angiological assessment

The time of appearance of the left gastric vein on serial celiac arteriograms in patients with portal hypertension and esophageal varices was compared with that of the portal vein to assess regional hemodynamics in the left venous portion of the stomach, an area located in close proximity to the varices. In two thirds of all the patients with cirrhosis or non-cirrhotic idiopathic portal hypertension (IPH), the left gastric vein was visualized earlier or simultaneously than or with the portal vein, while in all but one patient with prehepatic portal obstruction, there was a delayed opacification of the left gastric vein. These results suggest the presence of a hyperdynamic circulatory state which promotes venous hypertension in the left gastric venous area of the stomach of a considerable number of patients with cirrhosis or IPH. In such a hemodynamic state, selective decompression of varices can be achieved by a left gastric venous caval shunt.     

The long-term effect of distal lienorenal shunt surgery on portal venous pressure

To assess whether portal venous pressure remains elevated following distal lienorenal shunt surgery, 11 patients with alcoholic cirrhosis were assessed from 0.5 to 59 months following the creation of a distal lienorenal shunt. These were compared with five patients following mesocaval shunt surgery and nine with cirrhosis alone. Portal pressure was measured by direct transhepatic catheterization of the portal vein or by determining intrahepatic pulp pressure. Splenic pulp pressure was also measured in the shunt groups at the time of assessment of shunt patency by scintisplenoportography. All shunts were patent. Four of five patients studied within 10 months of distal lienorenal shunt surgery had persisting portal hypertension (comparable with that in the cirrhosis alone group) and persisting portasplenic gradient. In contrast, five of six patients studied more than 10 months following surgery had portal pressures similar to that following mesocaval shunt surgery and no portasplenic gradient. Thus, with time, selective decompression tends to become total decompression. Whether this correlates with deterioration of hepatic function requires further study.     

Mesocaval shunt for portal hypertensive small bowel bleeding documented with intraoperative enteroscopy

INTRODUCTIONBesides upper gastrointestinal tract, small bowel has also been implicated as a potential source of hemorrhage in patients with portal hypertension.PRESENTATION OF CASEWe report an interesting case of recurrent massive small intestinal bleeding in a patient with portal hypertension secondary to liver cirrhosis treated with a mesocaval shunt. Endoscopic assessment with gastroscopy and colonoscopy failed to identify the source of hemorrhage. An intraoperative enteroscopy was conducted which revealed a diffuse bleeding pattern from the small bowel mucosa.DISCUSSIONAn interposition mesocaval shunt procedure was successfully carried out on an emergency basis that eventually managed to control bleeding.CONCLUSIONIn cases, where a diffuse pattern of hemorrhage exist or non-operative measures fail emergency mesocaval shunting seems to be an efficacious alternative treatment approach for portal hypertension related intestinal bleeding.     

Portal diversion for portal hypertension in early childhood.

Twenty-three children under 6 years of age with portal hypertention were treated by portal diversion. Fourteen had cavernomatous transformation of the portal vein and 9 had an intrahepatic block due to cirrhosis (8) or congenital hepatic fibrosis (1). Portal-systemic shunts were central splenorenal in 20 patients, side-to-side portacaval in 2 and mesocaval in one. In 20 of the 21 peripheral shunts, the veins used for the anastomosis were less than 10 mm in diameter. There was no operative mortality. Thrombosis of the shunt occurred in 3 children (13%) and was responsible for recurrent bleeding in one who was treated later with success by a mesocaval shunt. The two other children with a thrombosed shunt are waiting, at the present time, for a mesocaval anastomosis. The volume of blood flowing through the shunt was small initially and the fall in pressure gradient was slight: therefore intraoperative angiography appeared to be a better way to assess the patency of shunts done at an early age than pressure or flow measurements. The figures recently reported by Clatworthy, with a mortality rate of 12% directly or indirectly related to repeated hemorrhage, are for us a forceful argument for early adequate management of portal hypertension in children. Until now, portal-systemic shunts have been complicated by a high frequency of thrombosis and have given discouraging results. Our results suggest that it is possible to perform portal diversion successfully on diminutive veins (down to 4 mm). From this experience early portal diversion appears to represent the treatment of choice for portal hypertension in childhood.     

Portal hypertension in infants and children with histiocytosis X.

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.     

Portal diversion for portal hypertension in children. The first ninety patients.

Ninety children with portal hypertension were treated by portal diversion. Fifty-two had cavernous transformation of the portal vein and 38 had an intrahepatic block from various causes. There were 59 central splenorenal shunts, 19 mesocaval, 11 portacaval and one distal splenorenal. In 61 peripheral shunts the veins used for the anastomosis were less than 10 mm in diameter. There was no operative mortality in children with extrahepatic block. One child with cystic fibrosis died postoperatively. Thrombosis of the shunt occurred in five children (5.6 per cent) and was responsible for recurrent bleeding in two. Four children with a thrombosed shunt underwent succesful reoperation and one is awaiting another anastomosis. No late complications occurred in the 52 children with extrahepatic block, while encephalopathy developed in four children with intrahepatic block. These figures confirm our earlier results in the management of portal hypertension in childhood and suggest that portal diversion is the treatment of choice. Several precautions have permitted lowering of the rate of thrombosis whichever shunt is performed. Portal diversion should be indicated following the first episode of hemorrhage in children with extrahepatic block. In patients with intrahepatic block, congenital hepatic fibrosis and cystic fibrosis are good indications as are in general the hepatic diseases with no or mild activity.     

Idiopathic extrahepatic portal hypertension in adults

Nine adults with bleeding gastroesophageal varices secondary to portal vein thrombosis of unknown cause are presented. Their average age was 51.6 years. Splenomegaly and secondary hypersplenism were the most common signs that portal hypertension was present. Liver function studies and histology were essentially within normal limits. The diagnosis was not suspected at the time of initial variceal hemorrhage in four patients. In only two patients could a conventional splenorenal or mesocaval shunt be fashioned. Surprisingly all five patients in whom a shunt could not be constructed and who survived operation remain alive for an average of 47.8 months. Only two of these patients have had rebleeding. Complete angiographic evaluation of the portal venous system with portograms, splenoportograms, or tolazoline mesenteric arteriograms is essential for proper management of these patients.     

Duodenal varicose veins

Summary Duodenal varices (DV) are rare. We present a review of published cases with emphasis on the management and outcome, as illustrated by our own case, which reflects the experience reported in the literature. The diagnosis of DV must be considered in patients with gastrointestinal bleeding. Two-thirds of all reported cases have portal venous hypertension caused by hepatic cirrhosis. In the remaining one-third prehepatic portal hypertension as a consequence of either a compromised portal venous circulation (caused by perivenous tumor or inflammation) or a primary haematological disease is the underlying cause. Previously, duodenoscopy has often failed to detect and correctly interpret DV, and was similarily unsuccessful in our case. This case report demonstrates the problems and shortcomings in the management of DV and documents a hither to unreported cause. Treatment depends on the severity of bleeding. When conservative measures cannot control the haemorrhage, emergency laparotomy may be indicated. The type of surgery should be chosen according to the aetiology, site and extent of the bleeding DV. Among 112 reported cases of DV, information on outcome exists for only 35 patients who presented with haemorrhage. The aetiology was liver cirrhosis in 26 of these patients, 10 of whom had a fatal outcome, and prehepatic portal hypertension in the remaining 9, 1 of whom had a fatal outcome.     

Role of surgical portosystemic shunts in the era of interventional radiology and liver transplantation

BACKGROUND: In the present era of liver transplantation and transjugular intrahepatic portosystemic shunts, the role and choice of shunt surgery for portal hypertension was reviewed. METHODS: This retrospective study analysed the management of patients with portal hypertension in a tertiary liver transplant unit between June 1993 and May 2002. During this 9-year interval, 394 patients underwent endoscopic control of varices, 235 transjugular intrahepatic portosystemic shunts were inserted, 1142 liver transplants were performed, while only 29 patients needed a surgical portosystemic shunt. RESULTS: Twenty-nine shunt operations were performed in nine patients with cirrhosis, one patient with congenital hepatic fibrosis and 19 without parenchymal liver disease. There were 12 side-to-side lienorenal, nine mesocaval, three proximal lienorenal, two distal lienorenal, two portacaval and one mesoportal shunts. Encephalopathy was seen in five of 11 patients with a non-selective shunt, but did not occur after side-to-side or selective lienorenal shunt procedures. At a median follow-up of 42.5 months, one mesocaval shunt had thrombosed and one portacaval shunt had stenosed; both were successfully managed by percutaneous intervention. To date, six patients have died; two succumbed to postoperative complications, one of which was related to the shunt. CONCLUSION: Patients with Budd-Chiari syndrome and cirrhosis can nearly always be managed by a combination of endoscopy, interventional radiology and liver transplantation. In the rare instances when these therapies fail in patients with cirrhosis, a side-to-side lienorenal shunt is a good option.     

Pathological abnormalities in splenic vasculature in non-cirrhotic portal hypertension:Its relevance in the management of portal hypertension

BACKGROUND Portal hypertension(PH) is associated with changes in vascular structure and function of the portosplenomesenteric system(PSMS).This is referred to as portal hypertensive vasculopathy.Pathological abnormalities of PSMS has been described in the literature for cirrhotic patients.Raised portal pressure and hyperdynamic circulation are thought to be the underlying cause of this vasculopathy.In view of this,it is expected that pathological changes in splenic and portal vein similar to those reported in cirrhotic patients with PH may also be present in patients with non-cirrhotic PH(NCPH).AIM To investigate pathological abnormalities of splenic vein in patients with NCPH,and suggest its possible implications in the management of PH.METHODS A prospective observational study was performed on 116 patients with NCPH[Extrahepatic portal vein obstruction(EHPVO):53 and non-cirrhotic portal fibrosis(NCPF):63] who underwent proximal splenorenal shunt(PSRS),interposition shunt or splenectomy with devascularization in JIPMER,Pondicherry,India,a tertiary level referral center,between 2011-2016.All patients were evaluated by Doppler study of PSMS,computed tomography portovenogram and upper gastrointestinal endoscopy.An acoustic resonance forced impulse(ARFI) scan and abdomen ultrasound were done for all cases to exclude cirrhosis.Intraoperative and histopathological assessment of the harvested splenic vein was performed in all.The study group was divided into delayed and early presentation based on the median duration of symptoms(i.e.108 mo).RESULTS The study group comprising of 116 patients [77(66%) females and 39(34%)males] with NCPH had a median age of 22 years.Median duration of symptoms was 108 mo.The most common presentation in both EHPVO and NCPF patients was upper gastrointestinal bleeding(hematemesis and melena).The ARFI scan revealed a median score of 1.2(1.0-1.8) m/s for EHPVO and 1.5(0.9-2.8) m/s for NCPF.PSRS was performed in 84 patients(two of whom underwent interposition PSRS using a 10 mm Dacron graft);splenoadrenal shunt in 9;interposition mesocaval shunt in 5;interposition 1 st jejunal to caval shunt in 1 patient and devascularization with splenectomy in 17 patients.Median presplenectomy portal pressure was 25(range:15-51) mm Hg.In 77% cases,the splenic vein was abnormal upon intraoperative assessment.Under macroscopic examination,wall thickening was observed in 108(93%),venous thrombosis in 32(28%) and vein wall calcification in 27(23%) cases.Upon examination under a surgical magnification loupe,21(18%) patients had intimal defects in the splenic vein.Histopathological examination of veins was abnormal in all cases.Medial hypertrophy was noted in nearly all patients(107/116),while intimal fibrosis was seen in 30%.Ninety one percent of patients with intimal fibrosis also had venous thrombosis.Vein wall calcification was found in 22%,all of whom had intimal fibrosis and venous thrombosis.The proportion of patients with pathological abnormalities in the splenic vein were significantly greater in the delayed presentation group as compared to the early presentation group.CONCLUSION Pathological changes in the splenic vein similar to those in cirrhotic patients with PH are noted in NCPH.We recommend that PH in NCPH be treated as systemic and pulmonary hypertension equivalent in the gastrointestinal tract,and that early aggressive therapy be initiated to reduce portal pressure and hemodynamic stress to avoid potential lethal effects.     

Graft interposition splenocaval shunt for total or selective decompression of portal hypertension

A new operation for selective or total decompression of the portal venous system in cases of intrahepatic portal hypertension is described. It involves interposition of a large-caliber Dacron graft between the splenic vein and the inferior vena cava. The graft-interposition splenocaval shunt is performed readily and quickly, satisfying the variable hemodynamic needs of patients with portal hypertension. It can be either selective (S-SCS) or total (T-SCS) from the beginning, or a T-SCS may be converted subsequently to a S-SCS should surgically induced hepatic decompensation supervene. It is less demanding technically than distal splenorenal shunt (D-SRS). The S-SCS conserves portal venous perfusion of the liver, preserves hepatocellular function and architecture at the preoperative levels, avoids precipitation of postshunt portal-systemic encephalopathy, and decompresses gastric-esophageal varices with prevention of further variceal bleeding even better than D-SRS. One hundred percent graft patency has been obtained, and the surgical results have been superior to those following portacaval shunt in patients with large liver blood flow and relative benignity of the liver disease, be it cirrhosis or noncirrhotic portal fibrosis. In patients with advanced cirrhosis, variceal bleeding, and small liver blood flows, T-SCS would be indicated. Patients of this category obtained inferior surgical results and had operative deaths (16.7%) following S-SCS. The concept of the operation has merits and deserves further evaluation.