老年性Abernethy畸形致门脉系统性脑病2例并文献复习

Abernethy畸形是门-体静脉瘘（分流）造成的一种先天或后天形成的疾病,患病率极低,且目前无明确诊疗指南,早期常无症状,晚期通常因其他疾病通过影像学检查意外发现而确诊。而Abernethy畸形引起的门脉系统性脑病易误诊为肝硬化导致的肝性脑病、神经病等。本次收治的2例病例均有慢性肾衰竭病史、反复高氨血症,伴有精神症状,完善影像学检查考虑门体分流,予以降氨等保守治疗。临床上遇见神经精神症状提示可疑肝性脑病,但肝功能检测的异常值不足以提示肝硬化时,应怀疑门脉系统性脑病,尤其是Abernethy 畸形导致的门脉系统性脑病,避免延误治疗时间。     

Spontaneous porto-systemic shunts in liver cirrhosis: Clinical and therapeutical aspects

Spontaneous porto-systemic shunts(SPSS) are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates, probably as a consequence of worsening portal hypertension, but without achieving an effective protection against cirrhosis' complications. Several types of SPSS have been described in the literature, each one associated with different clinical manifestations. In particular,recurrent or persistent hepatic encephalopathy is more frequent in patients with splenorenal shunt, while the presence of gastric varices and consequently the incidence of variceal bleeding is more common in gastrorenal shunt. In the advanced stage, the presence of large SPSS can lead to the so called"portosystemic shunt syndrome", characterized by a progressive deterioration of hepatic function, hepatic encephalopathy and, sometimes, portal vein thrombosis. The detection of SPSS in patients with liver cirrhosis is recommended in order to prevent or treat recurrent hepatic encephalopathy or variceal bleeding.     

Analysis of hepatic hemodynamics by a new method of per-rectal portal scintigraphy using 99mTcO4- (direct intramural administration of radioisotope)

To depict of porto-systemic collaterals clearly, and to analyze of hemodynamics of liver, we developed new method of per-rectal portal scintigraphy (direct intramural administration of 99mTcO4- by 23G needle). And we used this method in patient with liver diseases (acute hepatitis: 5, chronic hepatitis: 7, liver cirrhosis: 25 cases). From time activity curve of the liver and the heart, liver/heart ratio; index of porto-systemic shunt via inferior mesenteric vein (IMV) and first flow ratio(k); index of portal blood flow from IMV pathway/index of hepatic total blood flow were calculated. In our method, the images of portal vein, liver, heart, especially porto-systemic collaterals were visualized more clearly than enema methods. The liver/heart ratio was significantly lower in patients with liver cirrhosis than that in non-cirrhotic patients (p less than 0.01), which indicated that patients with liver cirrhosis had more porto-systemic collaterals than non-cirrhotic diseases. The k was more lower in liver cirrhosis than in acute hepatitis (p less than 0.01). And the k was also more lower in chronic hepatitis than in acute hepatitis (p less than 0.1), which indicated that portal blood flow via IMV reduced in early stage of chronic liver diseases. In conclusion, new method of per-rectal portal scintigraphy has more advantage for analysis of hepatic hemodynamics than enema methods.     

Post-surgical shunt hepatopulmonary syndrome in a case of non-cirrhotic portal hypertension: lack of efficacy of shunt reversal

Hepatopulmonary syndrome, a consequence of significant liver disease and portal hypertension, is thought to be secondary to the effects of vasoactive substances, normally inactivated in the liver, on the pulmonary vasculature. We report a patient with preserved hepatic function who underwent a decompressive surgical porto-systemic shunt for non-cirrhotic portal hypertension. This patient developed hepatopulmonary syndrome with dyspnoea and oxygen desaturation 2 years post-surgical shunt. Over the next 7 years, the patient's respiratory function became increasingly impaired although hepatic function remained preserved. Because of the hypothesized role of porto-systemic shunting in the aetiology of this syndrome, the surgical shunt was successfully reversed angiographically. No improvement in dyspnoea or oxygen saturation occurred and liver transplantation was undertaken. Six months post-transplant, the patient has decreased his oxygen requirements and is free of dyspnoea. Our experience supports the causal role of porto-systemic shunting in the pathogenesis of hepatopulmonary syndrome but suggests that merely decreasing the extent of porto-systemic shunting is not beneficial. Liver transplantation remains the only reliable therapeutic modality available to these patients.     

Porto-hepatic venous shunt via portal vein aneurysm with splenomegaly

A 59-year-old woman presented with consciousness disturbance with flapping tremor. Laboratory examinations revealed normal liver functions, and imaging studies, ultrasonography, magnetic resonance imaging, and angiography, showed portal vein aneurysm communicating from the portal vein to the hepatic vein, splenomegaly, and splenic artery aneurysm. These examinations confirmed porto-systemic shunt leading to hepatic encephalopathy. Porto-hepatic venous shunt via portal vein aneurysm is extremely rare, and there are few reports that it causes encephalopathy; therefore, little is known about the entity and this association. The literature in English is reviewed and the etiology, clinical features, and prognosis discussed.     

Encephalopathy following portal shunting in nodular regenerative hyperplasia of the liver

Two patients with nodular hyperplasia of the liver developed a chronic disabling encephalopathy after an interposition mesocaval shunt. Both had a low total hepatic blood flow-rate postoperatively. Encephalopathy disappeared following surgical occlusion of the shunt. These observations emphasize the risk of postshunt encephalopathy in patients with non-cirrhotic intrahepatic portal hypertension.     

肝硬化非常见侧支循环临床特点研究

目的 探讨肝硬化患者门体循环之间非常见侧支循环形成的临床特点及意义。方法 对临床确诊为肝硬化的患者运用64排螺旋CT和三维血管成像结合电子胃镜检查,观察其门体循环之间非常见侧支循环的形成。结果 ①700例肝硬化患者中118例(16.86%)存在非常见侧支循环,依次为脾肾静脉分流、胃肾静脉分流、椎旁静脉分流、腹膜后静脉分流、胃脾分流和心膈角静脉分流。②非常见侧支循环形成与肝硬化Child-Pugh分级相关(P<0.01)。③与常见侧支循环形成组比较,非常见侧支循环组较少出现重度食管和(或)胃底静脉曲张、重度门静脉高压性胃病及大量腹水(P<0.01)。④非常见侧支循环组中肝性脑病和慢性血氨升高的发生率高于常见侧支循环组(P<0.01)。结论 ①肝硬化患者中非常见侧支循环并不"非常见";②非常见侧支循环形成与肝功能Child-Pugh分级有关;③非常见侧支循环形成可缓解门静脉高压引起的相关并发症,但增大了肝性脑病和慢性血氨升高的发病率。     

Spinal cord involvement in patients with cirrhosis

A severe spinal cord involvement may rarely occur in patients with cirrhosis and other chronic liver diseases;this complication is usually associated with overt liver failure and surgical or spontaneous porto-systemic shunt.Hepatic myelopathy(HM)is characterized by progressive weakness and spasticity of the lower extremities,while sensory and sphincter disturbances have rarely been described and are usually less important.The diagnosis is assigned in the appropriate clinical setting on clinical grounds after the exclusion of other clinical entities leading to spastic paraparesis.Magnetic resonance imaging is often unremarkable;however,also intracerebral corticospinal tract abnor-malities have been reported recently.The study of motor evoked potentials may disclose central conduction abnormalities even before HM is clinically manifest.HM responds poorly to blood ammonia-lowering and other conservative medical therapy.Liver transplantation represents a potentially definitive treatment for HM in patients with decompensated cirrhosis of Child-Pugh B and C grades.Other surgical treatment options in HM include surgical ligation,shunt reduction,or occlusion by interventional procedures.     

Development of chronic hepatocerebral degeneration eight years after a distal splenorenal (Warren) shunt.

It is well known that chronic encephalopathy may be a major complication after the establishment of a surgical portal caval shunt for an episode of variceal haemorrhage. In an effort to minimise this problem Warren and colleagues developed the distal splenorenal shunt where the portal and mesenteric blood flow to the liver was left intact. It is now recognised, however, that the longterm incidence of encephalopathy may be no different with this type of shunt compared with conventional surgical portal systemic shunts. Acquired chronic hepatocerebral degeneration has not been reported after such a selective shunt. A patient with primary biliary cirrhosis is reported who developed the clinical features of this syndrome eight years after a successful distal splenorenal shunt.     

Laparoscopic division of a portosystemic shunt to treat chronic hepatic encephalopathy

In the event of liver cirrhosis with severe portal hypertension, voluminous portosystemic shunt may lead to refractory encephalopathy. Obliteration of the shunt has been described as a satisfactory therapeutic solution, and reported procedures are mainly endovascular embolization and surgical shunt ligation through laparotomy. The former procedure is less invasive and seems to be as efficient. Laparoscopy, which is widely recognized to minimize mortality and morbidity in cirrhotic patients undergoing surgery, has never been used for such a procedure. Shunt division can therefore be considered using this modern approach to good effect and reduced morbidity. In support of this view, we report a case of severe chronic encephalopathy cured by laparoscopic surgical division of a large shunt after failure of the percutaneous technique.     

Clinical and Portal Hemodynamic Features in Cirrhotic Patients Having a Large Spontaneous Splenorenal and/or Gastrorenal Shunt

Clinical and portal hemodynamic features in 28 cirrhotic subjects with a large spontaneous spleno- and/or gastrorenal shunt were studied in comparison with 30 control cirrhotic cases without such collaterals. Forty-six percent of the former had chronic hepatic encephalopathy, but none of the latter was encephalopathic. These patients with large renal shunts were divided into those with and those without encephalopathy. Large esophageal varices were significantly less common in patients with a large shunt and encephalopathy compared with those who had a large shunt but no encephalopathy, and the control. But there was no significant difference of past variceal bleeding among these three groups. In all those with encephalopathy, part of superior mesenteric venous blood was shunting through these collaterals into the left renal vein or inferior vena cava, but the same was not demonstrable in patients with a large shunt and no encephalopathy and control cirrhotics. In the chronic encephalopathic, portal venous flow was estimated to be less than one-half of that in control cirrhotics, and the portion of superior mesenteric venous blood that was flowing hepatofugally through a large shunt into the left renal vein seemed about the same or greater than the portal venous flow. Thus, a large spontaneous spleno- and/or gastrorenal shunt might prevent development of large esophageal varices but not variceal hemorrhage and it increased a risk of chronic hepatic encephalopathy.     

Usefulness of multi-detector helical CT with multiplanar reconstruction for depicting the duodenal varices with multiple collateral shunt vessels

Duodenal varices are a rare complication in patients with portal hypertension. Bleeding from duodenal varices often results in a severe prognosis. Diagnosis of the disease is usually based on findings obtained by endoscopy or angiography. However, it occasionally fails to detect the lesion and demonstrate its porto-systemic shunt vessels which are necessary information to decide an appropriate treatment. Recent advances in CT may make it possible for us to reveal duodenal varices with complicated porto-systemic shunt vessels. We report the case of a 58-year-old man with liver cirrhosis with repeated bleeding from duodenal varices. Esophagogastroduodenoscopy (EGD) revealed multinodular varices in the third portion of the duodenum. Then we conducted a capsule endoscopy (CE) and found fresh blood in the duodenum, suggesting duodenal variceal hemorrhage. Angiography depicted the varices with one afferent and two efferent vessels. Abdominal CT examination was conducted using a four-channel multi-detector row CT scanner. The multiplanar reconstructed images revealed not only the varices, but also three afferent and two efferent vessels. The patient was treated by surgical ligation and sclerotherapy, because of its complicated porto-systemic shunt and reserved liver function. No gastrointestinal bleeding has been seen after the surgery. Our case suggests the usefulness of multi-detector CT with multiplanar reconstruction (MPR) for the diagnosis and therapeutic decision of duodenal varices.     

Opiate receptors and β-endorphin levels in brain areas of dogs with portal-systemic encephalopathy

Clinical observation has indicated a supersensitivity to morphine in patients with hepatic encephalopathy. With the aim of clarifying the issue, radioreceptor binding studies of opiate receptors were performed in frontal cortex and hypothalamus of 6 dogs with mild portal-systemic encephalopathy induced by chronic treatment with dimethylnitrosamine followed by porto-caval shunt end-to-side. beta-Endorphin assays were performed in the same areas with radioimmunoassay. Opiate receptors labeled with [3H]naloxone in both areas showed a significant increase in the receptor densities (Bmax) without changes in the dissociation constant (KD). In parallel beta-endorphin levels showed a decline during the development of encephalopathy in both areas. The increased densities of opiate receptors in the mild stage of encephalopathy may explain the supersensitivity to morphine in patients with liver diseases.     

Reversal of hepatic encephalopathy and alteration in amino acid profiles after blocking a surgical splenorenal shunt by interventional radiological techniques

Portal-systemic shunts are effective in preventing haemorrhage from varices in portal hypertension, but at the price of hepatic encephalopathy. We describe the blockage of a splenorenal shunt using interventional radiology, in a patient with incapacitating chronic encephalopathy. The procedure successfully reversed encephalopathy, without haemodynamic disturbances or haemorrhage from recurrent varices and was associated with a return of the plasma amino acid profile towards normal, with a reduction in aromatic amino acids and an increase in branch chain amino acids. This observation supports the hypothesis that changes in plasma amino acid profiles in patients with portal-systemic shunting are due to the diversion of portal blood rather than the underlying chronic liver disease.     

Prevention of recurrent variceal bleeding

Patients surviving a first episode of variceal bleeding have a risk of over 60% of experiencing recurrent haemorrhages within 1 year from the index episode. Because of this, all patients surviving a variceal bleeding should receive active treatments for the prevention of rebleeding. beta-Blockers+/-isosorbide-5-mononitrate and band ligation are effective in preventing recurrent bleeding and both can be used. Combination of beta-blockers+/-isosorbide-5-mononitrate and band ligation may be the best treatment to prevent rebleeding but more studies are needed to confirm this issue. In patients with recurrent variceal bleeding despite appropriate medical and endoscopic treatment, transjugular intrahepatic porto-systemic shunt is highly effective in controlling bleeding. The efficacy is not significantly different from that of shunt surgery (distal splenorenal shunt or 8mm H-graft shunt), especially since the introduction of polytetrafluoroethylene-covered stents. Therefore, in this situation, transjugular intrahepatic porto-systemic shunt using polytetrafluoroethylene stents should be the treatment of choice.     

Budd-Chiari syndrome and Beh?et's disease. A case treated by mesenterico-atrial prosthesis

A 36 year-old North African man, with Beh?et's syndrome complicated by an inferior vena caval thrombosis, developed a chronic Budd-Chiari syndrome associated with bleeding esophageal varices. He was treated by an emergency mesoatrial shunt. Results at 2 years were good. Analysis of this case and the 13 other similar cases with associated Budd-Chiari syndrome and Beh?et's syndrome found in the literature showed that hepatic veins thrombosis: a) is often due to inferior vena caval thrombosis or membranous obstruction; b) has a high spontaneous mortality rate by acute liver failure; c) remains a potential indication for porto-systemic shunt, as are other causes of Budd-Chiari syndrome.     

Inflammation and hepatic encephalopathy: ibuprofen restores learning ability in rats with portacaval shunts

One of the neurological alterations in patients with minimal or overt hepatic encephalopathy is cognitive impairment. This impairment is reproduced in rats with chronic liver failure due to portacaval shunt (PCS). These rats show decreased ability to learn a conditional discrimination task in a Y-maze, likely due to reduced function of the glutamate-nitric oxide (NO)-cyclic guanosine monophosphate (cGMP) pathway in brain. It has been proposed that inflammation exacerbates the neuropsychological alterations induced by hyperammonemia, suggesting that inflammation-associated alterations may contribute to cognitive impairment in hepatic encephalopathy. This study assessed whether treatment with an anti-inflammatory drug, ibuprofen, is able to restore the function of the glutamate-NO-cGMP pathway in cerebral cortex in brain in vivo and/or learning ability in PCS rats. We show that PCS rats have increased levels of interleukin-6 and increased activities of cyclooxygenase and of inducible NO synthase in cerebral cortex, indicating the presence of inflammation. Chronic treatment with ibuprofen normalizes cyclooxygenase and inducible NO synthase activities but not interleukin-6 levels. Moreover, ibuprofen normalizes the function of the glutamate-NO-cGMP pathway in cerebral cortex in vivo and completely restores the ability of rats with chronic liver failure to learn the Y-maze task. This supports that inflammation contributes to the cognitive impairment in hepatic encephalopathy. CONCLUSION: the results reported point to the possible therapeutic utility of decreasing inflammation in the treatment of the cognitive deficits in patients with minimal or overt hepatic encephalopathy.     

Assessment of portosystemic shunt by summation of radioactivity during 201thallium chloride portal scintigraphy in patients with chronic liver disease

BACKGROUND/AIMS: Portal scintigraphy is a useful non-invasive method for the determination of portosystemic shunts in patients with liver cirrhosis. Several procedures have been reported for its execution in clinical practice but most of them failed to show sufficient sensitivity for the diagnosis of portosystemic shunt. In the present study, we evaluated whether summation of radioisotope counts obtained during intrarectal or intraduodenal administration of 201thalium chloride is useful for increasing the diagnostic yield of porto-systemic shunts in patients with chronic liver disease. METHODOLOGY: Seven patients with chronic viral hepatitis and 8 with liver cirrhosis secondary to viral hepatitis were enrolled in this study. Following the conventional protocol, 201thalium chloride was administered per rectum and the 60-second-heart-to-liver uptake (conv-H/L-R) ratio was calculated after 20 min. Continuous measurement of the radioactivity signals during 20 min were also done and the summated heart-to-liver uptake (sum-H/L-R) ratio from the total radioactivity count were calculated. Measurement of the conventional heart-to-liver uptake (conv-H/L-D) ratio and the summated (sum-H/L-D) ratio were also done as described above after the intraduodenal administration of 201thalium chloride by endoscopy. RESULTS: All ratios (conv-H/L-R, conv-H/L-D, sum-H/D-R, sum-H/L-D) were significantly higher in patients with liver cirrhosis than in those with chronic hepatitis. Among all heart/liver ratios, only the sum-H/L-R ratio was significantly different between patients with and without esophageal varices. Serum hyaluronate level and other liver function tests were found to be significantly correlated with all heart-to-liver ratios, but they were more strongly correlated with the sum-H/D-R and sum-H/L-D ratios than with the conv-H/L-R and conv-H/L-D ratios. CONCLUSIONS: The results of this study showed that the heart-to-liver ratio calculated by summation of radioactivity is better than the conventional method for the diagnosis of portosystemic shunt in patients with chronic liver disease.     

RECURRING ENCEPHALOPATHY ABOLISHED BY GASTRORENAL SHUNT LIGATION IN A DIABETIC HEMODIALYSIS PATIENT

A 57-year-old man was admitted to our hospital for hepatic encephalopathy. He previously had undergone a partial gastrectomy for gastric ulcer, and also had been on maintenance hemodialysis because of diabetic nephropathy. Despite treatment with branched-chain amino acids and lactulose, encephalopathy occurred repeatedly. The findings of his laboratory examinations, computed tomography, and liver biopsy were not suggestive of chronic liver damage. Angiography revealed a portal-systemic shunt from the superior mesenteric vein via the left gastric vein to the left renal vein. A ligation of the gastrorenal shunt was performed. After the shunt ligation, hepatic encephalopathy no longer recurred, and no medication was required to prevent it. The insulin requirements also decreased, the plasma ammonia concentration then decreased, and serum concentration of several amino acids related to the ammonia metabolism also decreased. The molar ratio of branched-chain amino acids to aromatic amino acids increased. The ligation of the portal-systemic shunt was thus considered to be the key to the successful treatment of hepatic encephalopathy in this unusual case.     

Disappearance of hepatic encephalopathy and improvement of liver function after surgical treatment of portal-systemic shunt in a patient with liver cirrhosis

Large portal-systemic shunts in cirrhotic patients often cause recurrent hepatic encephalopathy and might promote liver dysfunction because of the reduced portal blood flow. We report a case of liver cirrhosis in which hepatic encephalopathy disappeared and liver function improved together with an increase of hepatopetal portal blood flow and liver volume after shunt resection. A 70-year-old woman with liver cirrhosis was admitted because of recurrent disorientation. Serum ammonia levels ranged from 174 to 321 micrograms/dL. Computed tomography demonstrated an atrophic liver and a large shunt. Portography disclosed that this shunt originated from the superior mesenteric vein and flowed into the inferior vena cava, common iliac vein and ovarian vein. Portal blood flow was poor because of the deviation into this shunt. After the surgical resection of the shunt, ammonia levels were normalized and hepatic encephalopathy no longer occurred. Portography and computed tomography after surgery demonstrated that hepatopetal portal blood flow evidently improved and the liver volume increased (before 369; after 574 cm3). Two years after surgery, hepaplastin test and serum albumin level improved from 41 to 76% and from 2.7 to 3.4 g/dL, respectively. This case supports the effectiveness of shunt resection for hepatic encephalopathy and the deteriorated liver function in cirrhotic patients with large portal-systemic shunt.