Distal splenorenal shunts for the treatment of severe thrombocytopenia from portal hypertension in children

Profound thrombocytopenia resulting from portal hypertension may exacerbate gastrointestinal bleeding, precipitate spontaneous bleeding, preclude surgical intervention for associated disorders, and severely limit life-style because of the danger of splenic injury. Although splenectomy can reverse the thrombocytopenia, the procedure should be avoided in children. We reviewed our experience with distal splenorenal shunting (DSRS) in children, particularly when performed for the sole purpose of reversing severe thrombocytopenia resulting from portal hypertension. DSRS was performed in 11 children between the ages of 7 and 15 years: five for severe thrombocytopenia (group 1), four for advanced hypersplenism and congenital hepatic fibrosis prior to renal transplantation (group 2), and two for esophageal bleeding (group 3). One child in group 1 with severe heart disease and Child’s class C cirrhosis due to hepatitis C died of progressive cardiac failure and was excluded from further analysis. Of the eight remaining patients in groups 1 and 2, four children had congenital hepatic fibrosis, two had portal vein thrombosis, one had hepatitis B, and one had Wilson’s disease. After DSRS, the mean platelet count increased from 37,000 ±18,000 to 137,600 ±81,000 (P = 0.01). The platelet count improved significantly in all seven children with presinusoidal portal hypertension or stable cirrhosis but did not increase in the child with hepatitis Band Child’s class B cirrhosis. The white blood cell count increased from an average of 3.3 ±1.1 to 5.4 ± 2.6 (P= 0.02). There were no postoperative complications in this group. The improved platelet count allowed the four children with congenital hepatic fibrosis and renal failure to undergo renal transplantation with full posttransplant immunosuppression including azathioprine. Postoperative Doppler ultrasound examination demonstrated shunt patency at 6 months in all cases. Spleen size decreased appreciably in all children in groups 1 and 2. All children were able to resume full activity including contact sports. In summary, DSRS effectively controls profound thrombocytopenia resulting from presinusoidal portal hypertension or stable cirrhosis without sacrificing the spleen and should be the treatment of choice for this condition. Presented at the British Association of Pediatric Surgeons Annual International Congress, Istanbul, Turkey, July 22–25, 1997.     

Results of Surgical Treatment (Modified Sugiura-Futagawa Operation) of Portal Hypertension Associated to Complete Splenomesoportal Thrombosis and Cirrhosis

Background Hemorrhagic portal hypertension, secondary to both intrahepatic and extrahepatic portal hypertension, is an uncommon entity. In this condition, the extrahepatic and the intrahepatic obstruction of the portal vein, due to chronic liver disease, produce a more severe form of hemorrhagic portal hypertension that is more difficult to control. The results of surgical treatment (modified Sugiura- Futagawa operation) in this subset of patients is analyzed.Methods Among 714 patients with a history of hemorrhagic portal hypertension, 14 cases were found with histologically proven liver cirrhosis and complete splenomesoportal thrombosis demonstrated by means of preoperative angiography. Patients with incomplete (partial) splenomesoportal thrombosis were excluded. There were nine males and 5 females with a mean age of 51 years. Alcoholic cirrhosis was demonstrated in 50% of the cases, post hepatitic cirrhosis in 28%, primary biliary cirrhosis in 7%, and cryptogenic cirrhosis in 14%. There were nine Child-Pugh A and 5 B cases. All cases were treated by means of our modified Sugiura-Futagawa procedure.Results Bleeding recurrence from esophagogastric varices was shown in one case, colonic varices in one case and hypertensive gastropathy in another of the survivors. Post operative encephalopathy was shown in 3 of the cases. The thirty-six month survival rate was 30% (Kaplan-Meier).Conclusions The combination of intrahepatic plus extrahepatic portal hypertension has a worse prognosis. Treatment options are limited (sclerotherapy and/or devascularization), because shunt surgery, TIPS and liver transplantation have a very restricted role and postoperative outcome is poor.     

经颈静脉肝内门体分流术与肝细胞癌的相关性

经颈静脉肝内门体分流术（TIPS）可有效治疗肝硬化患者门静脉高压，但可能影响患者血流动力学和肝功能。TIPS与肝硬化及肝癌具有潜在相关性，可能与肝脏血流动力学改变和肠道群菌失调等有关，目前对于其间关系仍不明确。本文就TIPS与肝细胞癌相关性研究进展进行综述。     

Significance of corrected sinusoidal pressure (CSP) in patients with cirrhosis and portal hypertension.

The relationship between the degree of portal hypertension measured by the corrected sinusoidal pressure (CSP), the size of varices graded endoscopically, the risk of variceal hemorrhage and the prognosis following portal decompression were studied in 32 patients with cirrhosis demonstrated by examination of a biopsy specimen. The CSP was no different in patients with large-sized versus small or moderate-sized varices. CSP was of no discriminant value in patients with bleeding versus nonbleeding varices and, furthermore, was of no prognostic value in patients requiring operative portal decompression. The risk of variceal bleeding was highest in patients with large-sized varices. We conclude, therefore, that CSP measurements have little if any therapeutic or prognostic importance in cirrhotic patients with portal hypertension or gastrointestinal bleeding. CSP may, however, be useful in establishing extrahepatic or presinusoidal causes of portal hypertension.     

Liver transplantation in a 7-month-old girl with Caroli's disease

Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.     

Arterioportal fistulas: introduction of a novel classification with therapeutic implications

Arterioportal fistulas (APFs) are arteriovenous communications between the splanchnic arteries and the portal vein that represent an infrequent cause of presinusoidal portal hypertension. They can be acquired or congenital. Penetrating hepatic trauma, including liver biopsies, represent the most common etiology. They can be asymptomatic or manifest with portal hypertension. An abdominal bruit is a valuable physical finding. Persistence of an APF can cause hepatoportal sclerosis and possibly portal fibrosis. A detailed radiologic evaluation is mandatory. One must differentiate between small peripheral intrahepatic APFs (type 1) and large central APFs (type 2). The former usually resolve spontaneously, whereas the latter can cause portal hypertension and hepatic parenchymal changes. Type 1 APFs caused by needle injury can be followed by Doppler ultrasound. All other fistulas need treatment. Arterioportal fistulas are first treated by transcatheter embolization. Surgical approaches are reserved for complex cases. Congenital APFs (type 3) are diffuse and intrahepatic and can be difficult to manage. Overall, the prognosis is good. Herein, we propose a novel classification for arterioportal fistulas with therapeutic implications.     

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     

特发性门静脉高压症的诊治现状

特发性门静脉高压症(IPH)非常罕见,其特点是有门静脉高压,但没有肝硬化,有门静脉小分支堵塞或狭窄,却没有肝静脉和门静脉主干的堵塞。该疾病尚有其它命名,如:特发性非硬化性门静脉高压症、非肝硬化性门静脉纤维化等。IPH病因及发病机制未明确,可能与遗传、免疫、感染等因素相关。临床表现上IPH以脾功能亢进、胃食管静脉曲张为主,而少有肝功能不全、肝性脑病。诊疗上尚有不少争议,目前IPH的诊断为排除性诊断,治疗上多参考肝硬化性门静脉高压的指南。IPH预后资料不多,目前认为较肝硬化性门静脉高压者预后更佳,只有少数患者进展至肝衰竭。     

Surgical experience in children with biliary atresia treated with portoenterostomy

OBJECTIVE: Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow. METHODS: This retrospective analysis includes 30 consecutive patients undergoing portoenterostomy for biliary atresia at our hospital. RESULTS: The 5-year actuarial survival of the 30 patients was 68%. Thirteen patients (43.3%) died 3 days to 7 years after portoenterostomy. Four patients (13.3%) underwent liver transplantation 3 to 24 months after the Kasai procedure with a 100% survival. In 65% of patients without presence of cirrhosis, the portoenterostomy was successful, compared with 35% of cases with liver cirrhosis (p = 0.0148). Liver cirrhosis with extrahepatic biliary atresia alone was present in 5 of 17 patients (29%) as compared with 8 of 12 patients (66%) with intrahepatic biliary hypoplasia in addition to extrahepatic biliary atresia and cirrhosis. CONCLUSIONS: Portoenterostomy remains the treatment of choice for patients with extrahepatic biliary atresia. However, the presence of cirrhosis portends a poorer prognosis and may be an indication for early transplantation. Cirrhosis is more commonly present in the setting of intrahepatic biliary hypoplasia and may account for the lower success rates of portoenterostomy in this group of patients. Five-year survival of the female patients was 88% as compared with 55% of the male patients.     

Surgical treatment of choledochal cysts

Biliary cystic disease is uncommon in Asia and very rare in Europe and the Americas. Patients with biliary cysts may present as infants, children, or adults. When patients present as adults, they are more likely to have stones in the gallbladder, common duct, or intrahepatic ducts and to present with biliary colic, acute cholecystitis, cholangitis, or gallstone pancreatitis. With increasing age at presentation, the risks of intrahepatic strictures and stones, segmented hepatic atrophy/hypertrophy, secondary biliary cirrhosis, portal hypertension, and biliary malignancy all increase significantly. Factors to be considered when performing surgery on patients with biliary cystic disease include: (1) age, (2) presenting symptoms, (3) cyst type, (4) associated biliary stones, (5) prior biliary surgery, (6) intrahepatic strictures, (7) hepatic atrophy/hypertrophy, (8) biliary cirrhosis, (9) portal hypertension, and (10) associated biliary malignancy. In general, regardless of age, presenting symptoms, biliary stones, prior surgery or other secondary problems, surgery should include cholecystectomy and excision of extrahepatic cyst(s). With respect to the distal bile duct, the surgical principle should be excision of a portion of the intrapancreatic bile duct with care to not injure the pancreatic duct or a long common channel. Resection of the pancreatic head should be reserved for patients with an established malignancy. With respect to the intrahepatic ducts, surgery should be individualized depending on whether (1) both lobes are involved, (2) strictures and stones are present, (3) cirrhosis has developed, or (4) an associated malignancy is localized or metastatic. When the liver is not cirrhotic, hepatic parenchyma should be preserved even when strictures and stones are present. If cirrhosis is advanced, hepatic transplantation may be indicated, but this sequence of events is unusual. If a malignancy has developed, oncologic principles should be followed. Whenever possible, resection of a localized tumor including adjacent hepatic parenchyma and regional lymph nodes should be performed.     

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.     

Portal decompression in infants and children with the interposition mesocaval shunt.

The mesocaval graft for portal decompression is applicable in infants and children with portal hypertension secondary to extrahepatic or intrahepatic causes. It is recommended in patients in whom extensive previous surgery in the portahepatis (Kasai procedure) would make dissection of the portal vein difficult and endanger the integrity of the functioning biliary conduit. It can be performed in patients who have had previous splenectomy or in whom portacaval or splenorenal shunts have failed. Autogenous jugular vein is favored for creation of the shunt.     

Hydatid cysts of liver and portal hypertension

Two cases of portal hypertension due to hydatid cysts of the liver are reported. In one of the patients, symptoms were secondary to obstruction of inferior vena cava and hepatic outflow tract. The other patient was operated on with a diagnosis of extrahepatic presinusoidal portal hypertension caused by extrinsic compression of the liver by an hydatid cyst. Although hydatidosis is a benign disease, it can produce serious complications as in these reported cases. Therefore hydatidosis should be remembered amongst the causes of portal hypertension in countries where the disease is endemic.     

Controlled splenorenal shunt for esophageal varices with the long term clinical observations

Conclusion Late results of our newly devised controlled splenorenal shunt were mentioned. This procedure involves special technique which confirms patency of the shunt and controls the postshunt blood volume in order to maintain consistently considerable portal blood flows into the liver even after the shunt. Our clinical analysis on 40 cases included 12 of Laennec or septal cirrhosis of the liver (Nagayo B-type), 7 of postnecrotic cirrhosis (Nagayo A-type), 13 of intrahepatic presinusoidal portal hypertension and 8 of prehepatic obstruction and schistosomiasis japonica, and among these 24 elapsed longer than 3 years and 14 longer than 5 years after the operation. Operative mortality, rate of postoperative bleeding, 5-year survival rate ware 15%, 7.5% and 67.5% respectively in over all cases, and should A-type of cirrhosis were omitted from the statistics, the operative mortality was 3% and long term survival rate 82% respectively. Since A-type of liver cirrhosis is apt to fall into circulatory insufficiency of the liver even by the controlled shunting, it is considered not to be adopted as indication of this operation.     

肝硬化门脉高压症治疗的新途径——干细胞移植研究进展

肝干细胞是多源性的,可分为肝源性肝干细胞和非肝源性肝干细胞两大类。多种成体组织来源的干细胞可在受者(包括鼠和人)肝脏内分化为肝细胞和胆管上皮细胞,为成体干细胞移植治疗肝硬化门脉高压提供了新思路。在临床应用中,肝干细胞移植具有移植技术简单、价格相对低廉,免疫源性小、易于低温保存,具有广泛的扩展性,体外基因转染率高,并能稳定高效地表达外源基因等优点;但肝内干细胞移植是否会引起肝脏肿瘤等问题尚未明确。     

Transjugular intrahepatic portosystemic shunt in non-cirrhotic portal hypertension related to cystic fibrosis in a lung transplant patient

Liver involvement is not uncommon in patients with cystic fibrosis (CF). Even if serious complications as non-cirrhotic portal hypertension, cirrhosis and liver failure rarely occur, they are associated with impaired survival and reduced quality of life. Herein, we have reported the first case of a patient with CF and non-cirrhotic portal hypertension who underwent transjugular intrahepatic portosystemic shunt placement for recurrent variceal bleeding after bilateral lung transplantation, and we have reviewed the available literature pertaining to this field.     

精准外科时代门静脉高压症的外科治疗策略

门静脉高压症最危重的并发症是食管静脉曲张破裂出血。药物治疗和内镜套扎是食管静脉曲张出血的一线治疗方法,非手术治疗失败或再出血时可考虑外科手术治疗。应根据患者的病因、病期和肝功能代偿状况,依据循证医学和精准外科治疗的理念,个体化选择分流术、断流术、肝脏移植等外科手术治疗。对于某些特殊类型的非肝硬化造成的肝外型门静脉高压症,Meso—Rex转流术可作为治愈性手术方法;而对于区域性的门静脉高压症,也可能通过外科手术治愈。     

Hyperkinetic portal hypertension. Arterioportal fistula: problems--case reports--review of the literature

Hyperkinetic portal hypertension is caused by pathological arterioportal shunts. Clinical differentiation is necessary between extrahepatic fistulas, splenoportal hypertension (arteriovenous anastomoses at the level of the pre-penicilary arteries) and intrahepatic fistulas in "active" cirrhosis and malignant tumors. This paper reports the clinical and angiographic features of eight patients with this type of fistula. A review of the literature is also presented (144 cases). Because of the severity of this disease, surgical intervention is necessary. The surgical technique depends on the organ-related necessity of vascular preservation.     

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.     

Portal hypertension and ascites

Portal hypertension is secondary to increased resistance to blood flow and increased blood flow through the portal system. The most common cause is liver cirrhosis. The most severe and life-threatening presentation of portal hypertension is acute variceal bleeding. Pharmacotherapy with vasoactive agents (terlipressin or somatostatin), endoscopic band ligation and radiological treatment with transjugular intrahepatic portosystemic shunt (TIPSS) are the most common treatment options for variceal bleeding. However, where surgical expertise exists, portosystemic shunts can be considered for refractory bleeding in patients without significant liver failure, especially when TIPSS is unavailable or contraindicated. Diuretic therapy with spironolactone and furosemide are the basis for the management of ascites. If ascites becomes refractory, repeat large volume paracentesis and TIPSS are potential treatment options. Liver transplantation offers the definitive treatment for portal hypertension secondary to cirrhosis as it cures the underlying liver disease.