Cruveilhier-Baumgarten disease in Japan--on the basis of our own case

The article reports on a patient with Cruveilhier-Baumgarten disease. The anamnesis of the 59-year-old housewife revealed previous anemia, splenomegaly and abnormal liver function 21 years ago. Three years ago, esophageal varices were found. A tortuously distended vein was seen in the falciform ligament at the time of laparoscopy, and this finding was confirmed by angiography and ultrasonography. Esophageal varices and hypersplenism were also noted. Despite these findings, liver biopsy specimens of both lobes showed only slight fibrosis with minimal lymphocyte infiltration in some portal areas, and no evidence of cirrhosis. Patency of the umbilical vein and portal hypertension without significant histologic change of the liver, are both in keeping with the features of this disease. Dilatation of the umbilical vein seemed to be congenital and did not contribute to active blood flow of portal hypertension in this patient. In Japan, development of "caput medusae" in portal hypertension is rather rare, whereas esophageal varices and splenomegaly are more frequent. Venous hum is also seldom found. 14 cases of Cruveilhier-Baumgarten disease and 28 cases of Cruveilhier-Baumgarten syndrome have been reported from Japan in the literature since 1911.     

Extrahepatic portal venous thrombosis: frequent recognition of associated diseases

Twelve patients with obstruction of the extrahepatic portal venous system were seen at the Tufts-New England Medical Center between 1970 and 1979; a cause for the portal vein thrombosis was detected in 11. These included pancreatic disease (4); hematologic disorders (2); postoperative complications of laparotomy (3); transhepatic gelfoam embolization of the portal vein (1); and exchange transfusion via the umbilical vein (1). Clinical features included frequent self-limited episodes of bleeding from esophageal or gastric varices; and no characteristic or clinically helpful laboratory findings. The diagnosis was usually made in patients by identifying clots in the portal vein on selective angiography of the celiac and/or superior mesenteric arteries in which the venous phase was examined. Attempts at surgical correction were largely unsuccessful. Further thrombotic episodes occurred in three patients, and led to death in one. Two patients were given chronic anticoagulation with Coumadin and Persantin for 1 and 11/2 years, respectively without further thrombosis or gastrointestinal bleeding. However, it not yet possible to assess the risks and benefits of such therapy.     

Hepatic radionuclide angiography and Doppler ultrasonography in the detection and assessment of vascular disturbances in the portal system

BACKGROUND/AIMS: The aim of the study was evaluation of the morphology of the blood vessels, blood flow velocity and direction with Doppler ultrasound (D-US) and correlation with the relative liver parenchymal perfusion assessed by hepatic radionuclide angiography (HRA). METHODOLOGY: Real-time, D-US and HRA were performed in 108 patients. RESULTS: In patients with portal venous aneurysm, hepatopetal blood flow was increased, while portal perfusion did not differ from controls. In portal hypertensive patients, D-US detected dilatation of the portal system veins, with decreased blood flow. In comparison to the portal perfusion in controls and portal venous aneurysm, values were significantly (p < 0.01) lower in chronic active hepatitis and liver cirrhosis and differed between themselves (p < 0.01). In the groups of cirrhotic patients with esophageal varices, sclerosed esophageal varices, recanalized umbilical vein, partial portal thrombosis and cavernous portal vein with hepatofugal, hyperkinetic or slow blood flow, and very low velocities beside the thrombi, portal perfusion was lower (p < 0.01) than in controls, portal venous aneurysm, chronic active hepatitis and liver cirrhosis without collaterals. In complete thrombosis, minimal collateral flow was found with D-US, while HRA proved no portal supply. CONCLUSIONS: D-US and HRA are complementary for the estimation of various liver vascular disorders.     

门静脉收缩器建立犬门静脉高压食管静脉曲张模型的实验研究

目的 探讨建立稳定的门脉高压食管静脉曲张犬模型的可行性.方法 12只杂交犬,采用门静脉.下腔静脉侧侧分流术+门静脉主干环置amemid收缩器+下腔静脉头侧双线交叉缝合结扎的方法制作动物模型.6周后,胃镜和门静脉造影检查观察食管曲张静脉形成情况.结果 造模手术前门静脉压力为(11.0 ±1.1)mm Hg,造模后门静脉压力升高至(22.9±1.2)mm as(P=0.010).胃镜发现所有的杂交犬均形成了轻-中度食管曲张静脉,门静脉造影检杳证实食管中下段有曲张静脉形成,同时腹壁形成了肉眼可见的曲张静脉.结论 门静脉收缩器法能成功地建立门脉高压食管静脉曲张动物模型.     

A case of mesenteric venous thrombosis after endoscopic variceal band ligation

A rare case of isolated superior mesenteric venous thrombosis (MVT) after endoscopic variceal band ligation (EVL) is reported. A 64-year-old woman with a history of idiopathic portal hypertension presented at the emergency room with vomiting, increasing cramping abdominal pain, and low-grade fever. She had undergone EVL for esophageal varices 4 months before and had had intermittent attacks of mild abdominal pain after the EVL. Ultrasonogram of the abdomen demonstrated marked concentric wall thickening of the ileal loop. Enhanced computed tomographic (CT) scan revealed a central lucency in the lumen of the superior mesenteric vein, surrounded by a high-density vein wall, corresponding to a thrombus. An isolated MVT and venous collateral network in the splanchnic area were confirmed by angiography. Supportive therapy, i.e., water and electrolyte replacement, and anticoagulation improved the clinical condition and radiologic status. This case of MVT after EVL suggests a possible relationship between EVL and MVT. It is necessary for clinicians to be aware of this relationship for the early diagnosis of MVT.     

CURATIVE TREATMENT OF BLEEDING ESOPHAGEAL VARICES SECONDARY TO A SPLENIC ARTERIO-VENOUS FISTULA

A 59 year old female, who presented with abdominal pain, diarrhea, and ascites, developed major bleeding from esophageal varices. Celiac angiography demonstrated a splenic arterio-venous fistula with early filling of an enlarged splenic vein and esophageal varices (pre-sinusoidal extra hepatic portal hypertension). The patient underwent splenectomy and resection of the fistula with resultant disappearance of the varices and presenting symptoms.     

Percutaneous paraumbilical embolization as an unconventional and successful treatment for bleeding jejunal varices

A 48-year-old Indian male with alcoholic liver cirrhosis was admitted after being found unresponsive. He was hypotensive and had hematochezia. Esophagogastroduodenoscopy (EGD) showed small esophageal varices and a clean-based duodenal ulcer. He continued to have hematochezia and anemia despite blood transfusions. Colonoscopy was normal. Repeat EGD did not reveal any source of recent bleed. Twelve days after admission, his hematochezia ceased. He refused further investigation and was discharged two days later. He presented one week after discharge with hematochezia. EGD showed non-bleeding Grade 1 esophageal varices and a clean-based duodenal ulcer. Colonoscopy was normal. Abdominal computed tomography (CT) showed liver cirrhosis with mild ascites, paraumbilical varices, and splenomegaly. He had multiple episodes of hematochezia, requiring repeated blood transfusions. Capsule endoscopy ident i f ied the bleeding s i te in the jejunum. Concurrently, CT angiography showed paraumbilical varices inseparable from a loop of small bowel, which had herniated through an umbilical hernia. The lumen of this loop of small bowel opacified in the delayed phase, which suggested variceal bleeding into the small bowel. Portal vein thrombosis was present. As he had severe coagulopathy and extensive paraumbilical varices, surgery was of high risk. He was not suitable for transjugular intrahepatic porto-systemic shunt as he had portal vein thrombosis. Percutaneous paraumbilical embolization via caput medusa was performed on day 9 of hospitalization. Following the embolization, the hematochezia stopped. However, he defaulted subsequent follow-up.     

Percutaneous paraumbilical embolization as an unconventional and successful treatment for bleeding jejunal varices

A 48-year-old Indian male with alcoholic liver cirrhosis was admitted after being found unresponsive. He was hypotensive and had hematochezia. Esophagogastroduodenoscopy （EGD） showed small esophageal varices and a clean-based duodenal ulcer. He continued to have hematochezia and anemia despite blood transfusions. Colonoscopy was normal. Repeat EGD did not reveal any source of recent bleed. Twelve days after admission, his hematochezia ceased. He refused further investigation and was discharged two days later. He presented one week after discharge with hematochezia. EGD showed non-bleeding Grade 1 esophageal varices and a clean-based duodenal ulcer. Colonoscopy was normal. Abdominal computed tomography （CT） showed liver cirrhosis with mild ascites, paraumbilical varices, and splenomegaly. He had multiple episodes of hematochezia, requiring repeated blood transfusions. Capsule endoscopy identified the bleeding site in the jejunum. Concurrently, CT angiography showed paraumbilical varices inseparable from a loop of small bowel, which had herniated through an umbilical hernia. The lumen of this loop of small bowel opacified in the delayed phase, which suggested variceal bleeding into thesmall bowel. Portal vein thrombosis was present. As he had severe coagulopathy and extensive paraumbilical varices, surgery was of high risk. He was not suitable for transjugular intrahepatic porto-systemic shunt as he had portal vein thrombosis. Percutaneous paraumbilical embolization via caput medusa was performed on day 9 of hospitalization. Following the embolization, the hematochezia stopped. However, he defaulted subsequent follow-up.     

Pancreatic arteriovenous malformation combined with portal thrombosis

We encountered a case of portal vein thrombosis (PVT) after treatment for portal hypertension due to pancreatic arteriovenous malformation (PAVM). A 75-year-old man was admitted for the treatment of esophageal varices. Diffuse PAVM and aneurysm in the celiac and superior mesenteric arteries were detected via abdominal computed tomography and angiography. Although endoscopical sclerotherapy was performed, PVT was identified after the treatment and variceal bleeding continued. Autopsy was performed and the thrombus and malformation were pathologically confirmed. This case indicates that PVT can be associated with PAVM.     

肝硬化食管静脉曲张出血内镜治疗后早期再出血的危险因素分析

目的探讨内镜治疗肝硬化食管静脉曲张破裂出血(EVB)后早期再出血的危险因素。 方法回顾分析2016年8月至2018年8月因肝硬化食管静脉曲张(EV)首次出血就诊于包头医学院二附院并采用内镜下治疗的患者资料,依据术后6周内是否再出血分再出血组和未出血组,对两组患者的一般资料、肝功能、血常规、凝血、门静脉血栓、门静脉异常分流等情况进行单因素分析,探讨内镜治疗EV术后早期再出血的危险因素。 结果(1)入组患者共450例,治疗后6周内出血27例,止血成功率94%;(2)单因素分析AST、GGT、TBIL、ALB、PTA、TG、肝功能、Child-Pugh分级、EV程度、门静脉血栓、门静脉异常分流在出血和未出血组之间的差异具有统计学意义;(3)多因素Logistic回归分析结果显示AST等是影响EV术后再出血的危险因素;ALB、门静脉异常分流是影响EV术后再出血的保护因素(P<0.05)。 结论AST、GGT、PTA、TG、肝功能Child-Pugh分级、EV程度、门静脉血栓是影响EV术再出血的危险因素;ALB、门静脉异常分流是影响EV术后早期再出血的保护因素。     

Etiology and consequences of thrombosis in abdominal vessels

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.     

Portal vein thrombosis following sclerotherapy.

We present the case of a woman with idiopathic portal hypertension who underwent sclerotherapy for bleeding esophageal varices. She had a rebleed 27 months after complete eradication of esophageal varices. Endoscopy showed bleeding gastric varices. Ultrasonography, and later splenoportography, revealed a large thrombus in the right branch of the portal vein causing gross dilation of the portal and splenic vein. A proximal splenorenal shunt was done to decompress the portal system and hence gastric varices. Repeat endoscopy 4 weeks after surgery revealed complete disappearance of the gastric varices, while ultrasonography at 38 weeks showed marked decompression of the portal system with complete disappearance of the thrombus from the right branch of the portal vein. No new thrombus formation was seen.     

Ileal varices associated with recurrent bleeding in a patient with liver cirrhosis

We report a rare case of massive and recurrent bleeding from ileal varices in a patient with hepatitis C virus-positive liver cirrhosis. A 66-year old woman, who had undergone laparotomy and blood transfusion 36 years before (because of an extrauterine pregnancy) and endoscopic sclerotherapy for esophageal varices 1 year previously, was admitted to our hospital with loss of bright red blood per rectum. The bleeding was massive and recurrent, and frequent blood transfusions were required. Endoscopic studies failed to find the bleeding site. In the venous phase of selective superior mesenteric angiography, mesenteric varices in the lower part of the abdominal cavity were observed. Laparotomy was performed to control the repeated bleeding which had lasted for more than 1 month. Varices communicating with the right ovarian vein were found on the ileal wall and segmental resection of the ileum was performed. Histological examination demonstrated a massive varicose vein and several dilated veins in the submucosa. The patient's postoperative course was favorable, with no hemorrhagic events during a follow-up of more than 6 months after surgery. Ileal varices should be considered in the diagnosis of a patient who presents with lower gastrointestinal bleeding and portal hypertension. (Received: July 9, 1998; accepted: Oct. 23, 1998)     

"Downhill" esophageal varices and occlusion of superior and inferior vena cavas due to a systemic venulitis

A patient with a 10-year history of episodes of venous thrombosis, a high ESR, an elevated plasma polyclonal 1gG concentration, and bleeding from esophageal varices had obstruction of both superior and inferior vena cavas with "downhill" esophageal varices and a normal portal vein with normal portal pressure. Extensive investigations revealed no predisposing factor for the venous thromboses, but the patient made a good clinical response to steroids and dapsone, with no further episodes of bleeding nor evidence of major venous thrombosis. The causes and outcome of "downhill" varices are discussed.     

Diagnosis of cavernomatous transformation of the portal vein

In 10 patients at the age of 17 to 59 years the diagnosis of a cavernomatous transformation of the portal vein primarily was made by sonography and was confirmed by computer tomography and angiography, respectively. These findings were seen by chance when an abdominal ultrasound was performed in order to clarify splenomegaly or esophageal varices. The characteristics of the disease are the positive proof of a convoluted agglomeration of racemose venous structures that have replaced the normal single portal vein and signs of portal hypertension. The sonographic figures are so typical that ultrasound is the decisive procedure in its diagnosis and that direct or indirect splenoportography is not necessary. The present results show that clinical manifestations of cavernomatous transformation of the portal vein are delayed sometimes in to adolescence and that invasive diagnostic methods are only necessary when shunt operation is planned.     

Portal and mesenteric venous thrombosis as a complication of endoscopic sclerotherapy

We report on the occurrence of portal and mesenteric venous thrombosis in two patients who underwent endoscopic injection sclerotherapy (EIS) for bleeding esophageal varices. One patient was diagnosed at autopsy, and portal and mesenteric venous thrombosis was not suspected during life. The second patient was suspected on ultrasound examination and was diagnosed by angiography. In the second case, therapy was effective and symptoms disappeared. He was treated by a distal splenorenal shunt after he had stabilized for 1 month. During the operation, no evidence of mesenteric infarction was found. The venous phase of the superior mesenteric arteriogram 1 month after surgery revealed an organized thrombosis in the right intrahepatic portal branches. No other thrombi were seen. This patient is the first reported who has survived portal and mesenteric venous thrombosis after sclerotherapy.     

门-体分流程度评估血吸虫病肝硬化上消化道出血的应用

目的探讨门-体静脉分流程度在评估血吸虫病肝硬化上消化道出血中的应用。方法以金山医院经临床证实的33例血吸虫病肝硬化上消化道出血患者,及29例血吸虫病肝硬化非出血患者为研究对象,对其进行上腹部128层螺旋CT扫描。采用薄层块最大强度投影(TSMIP)、多平面重建(MPR)对门静脉系进行血管重建,对两组患者门-体静脉分流程度进行评分和比较,分析各侧支血管分流程度与血吸虫病肝硬化上消化道出血的关系。结果 33例上消化道出血患者中,侧支血管发生率如下:胃左静脉曲张86.4%、胃短静脉曲张68.2%、食管静脉曲张50.0%、食管旁静脉曲张50.0%、胃底静脉曲张37.9%、胃肾静脉69.7%、脾肾静脉51.5%、腹壁静脉曲张25.8%、网膜静脉曲张15.2%、脾周静脉曲张63.6%、附脐静脉曲张34.8%、腹膜后-椎旁静脉40.9%、肠系膜静脉曲张36.4%。出血组食管静脉、食管旁静脉、胃左静脉和胃底静脉的发生率和分流程度均明显大于非出血组(P值均0.05)。结论 CT门静脉系成像可精确显示各类侧支血管的部位、程度及走向。食管静脉、食管旁静脉、胃左静脉和胃底静脉能较准确地预测血吸虫病肝硬化上消化道出血的风险情况,上述侧支血管分流程度越高,上消化道出血危险性就越大。     

Sonographic Patent Umbilical Vein: Lack of Specificity for Portal Hypertension

The sonographically patent umbilical vein is commonly considered a sign of portal hypertension and is held to be specific by some authors. We found sonoagraphically patent umbilical veins in 11 patients with hepatic cirrhosis and esophageal varices, and in three subjects without liver disease and without esophageal varices. All subjects were submitted to Doppler flowmetry, which, with the investigation of the direction of venous flow, proved to be a simple discriminatory test.     

Extensive calcifications in portal venous system in a patient with hepatocarcinoma

Calcification of the portal venous system is a rare entity that can be incidentally discovered during computed tomography(CT).We describe a case of extensive calcifications in the portal venous system in a middleaged male patient with hepatocellular carcinoma(HCC).This patient presented with epigastric pain that had no obvious origin prior to admission.Laboratory examinations were positive for hepatitis B surface antigen andα-fetoprotein,and severe esophageal and gastric varices were detected during gastroscopy.Abdominal X-ray plain film showed well-defined linear and tracklike calcification,with irregular margins directed along the course of the portal venous system.CT revealed extensive calcifications along the course of the portal,splenic,superior mesenteric and gastroesophageal veins.He underwent splenectomy 22 years ago due to splenomegaly and partial hepatectomy seven months before because of HCC of low-grade differentiation,confirmed by pathology.Finally,the patient was diagnosed with postoperative recurrent HCC and extensive portal venous system calcification after selective hepatic angiography under digital subtraction angiography.     

Isolated sigmoid varicose vein rupture and hemorrhage: A case report

Rationale:Ectopic varices are the collateral circulation of portal vein located anywhere in the gastrointestinal tract other than the esophageal and gastric regions. Rupture of these varices often results in life-threatening hemorrhage. Management guidelines for ectopic variceal bleeds are not yet standardized because cases are rare and treatment approaches described in the literature vary considerably.Patient concerns:A 53-year-old woman with a 20-year history of chronic hepatitis C cirrhosis came to our hospital for treatment due to intermittent black stools for 4 days. After admission, the patient developed hemorrhagic shock, with hemodynamic instability.Diagnosis:Postoperative histological examination confirmed the diagnosis of sigmoid varicose veins.Intervention:Emergency colonoscopy showed that a varicose vein mass in the sigmoid colon wall 30 cm from the anus was ruptured and bleeding. Percutaneous transhepatic inferior mesenteric venography revealed the presence of a varicose mass of sigmoid colon veins. After embolization of the sigmoid varicose veins with spring coils, angiography showed that the hemorheology of the distal varicose vein mass was slow but not completely blocked. Three days after embolization, the patient had hematochezia again. Splenectomy and sigmoid colon resection were performed immediately.Outcomes:Follow-up computed tomography showed no residual varices were observed after sigmoid colon resection.Lessons:Ectopic varices, which are rare sequelae of portal hypertension, need to be taken seriously because bleeding from these varices can be catastrophic. We report a case of isolated sigmoid variceal rupture and hemorrhage due to portal hypertension in cirrhosis. The patient experienced failure of endoscopic hemostasis and sigmoid colon venous coil embolization. She was eventually successfully brought to hemostasis by surgery.