A histologically proven case of progressive liver sarcoidosis with variceal rupture

Sarcoidosis is a chronic multi-systemic granulomatous disease,and liver involvement frequently occurs.in most cases,no evidence of liver dysfunction is ob-served,and portal hypertension due to sarcoid liver diseases is a rareoccurrence.Moreover,no case of liver sarcoidosis has ever been reported with confirma-tion of the disease progression.Herein we describe a patient having hepatic sarcoidosis with severe portal hypertension and liver dysfunction.The diagnosis was histologically confirmed from granulomatous status to established liver cirrhosis over 10 years.A 46-year-old woman developed massive hematemesis due to the rupture of gastric cardial varices.She underwent emer-gency endoscopic injection sclerotherapy,and clear evi-dence of chronic hepatic failure.Twelve years ago,she was diagnosed as having sarcoidosis with respiratoryclinicalsymptoms.Liver biopsy revealed asymptomatic incidental granulomas without fibrosis development.After a couple of years,features of liver dysfunction were manifest and progressed.Ten years after the first biopsy,a second liver biopsy was performed,and well established dense fibrosis was revealed.Although significant liver dysfunction with portal hypertension is rarely seen in sarcoidosis,this case indicates that we have to consider the possibility that sarcoidosis may cause end-stage liver cirrhosis.     

Splenic arteriovenous fistula and sudden onset of portal hypertension as complications of a ruptured splenic artery aneurysm： Successful treatment with transcatheter arterial embolization. A case study and review of the literature

Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension. A case of a 50-year-old multiparous female who developed suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of haematemesis and melaena during the past twelve days and thus was emergently admitted to hospital for management. Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT) and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The aforementioned vascular abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed.     

Splenic artery ligature associated with endoscopic banding for schistosomal portal hypertension

AIM:To propose a less invasive surgical treatment for schistosomal portal hypertension.METHODS:Ten consecutive patients with hepatosplenic schistosomiasis and portal hypertension with a history of upper gastrointestinal hemorrhage from esophageal varices rupture were evaluated in this study.Patients were subjected to a small supraumbilical laparotomy with the ligature of the splenic artery and left gastric vein.During the procedure,direct portal vein pressure before and after the ligatures was measured.Upper gastrointestinal endoscopy was performed at the 30th postoperative day,when esophageal varices diameter were measured and band ligature performed.During follow-up,other endoscopic procedures were performed according to endoscopy findings.RESULTS:There was no intra-operative mortality and all patients had confirmed histologic diagnoses ofschistosomal portal hypertension.During the immediate postoperative period,two of the ten patients had complications,one characterized by a splenic infarction,and the other by an incision hematoma.Mean hospitalization time was 4.1 d(range:2-7 d).Pre-and post-operative liver function tests did not show any significant changes.During endoscopy thirty days after surgery,a decrease in variceal diameters was observed in seven patients.During the follow-up period(57-72mo),endoscopic therapy was performed and seven patients had their varices eradicated.Considering the late postoperative evaluation,nine patients had a decrease in variceal diameters.A mean of 3.9 endoscopic banding sessions were performed per patient.Two patients presented bleeding recurrence at the late postoperative period,which was controlled with endoscopic banding in one patient due to variceal rupture and presented as secondary to congestive gastropathy in the other patient.Both bleeding episodes were of minor degree with no hemodynamic consequences or need for blood transfusion.CONCLUSION:Ligature of the splenic artery and left gastric vein with supraumbilical laparotomy is a promising and less invasive method for treating presinusoidal schistosomiasis portal hypertension.     

Association of liver cirrhosis related IgA nephropathy with portal hypertension

A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.     

Indocyanine green retention is a potential prog-nostic indicator after splenectomy and pericar-dial devascularization for cirrhotic patients

BACKGROUND: Splenectomy and pericardial devasculariza-tion (SPD) is an effective treatment of upper gastrointestinal bleeding and hypersplenism in cirrhotic patients with portal hypertension. Indocyanine green retention at 15 minutes (ICGR15) was reported to offer better sensitivity and speciifc-ity than the Child-Pugh classiifcation in hepatectomy, but few reports describe ICGR15 in SPD. The present study was to evaluate the prognostic value of ICGR15 for cirrhotic patients with portal hypertension who underwent SPD.
METHODS: From January 2012 to January 2015, 43 patients with portal hypertension and hypersplenism caused by liver cirrhosis were admitted in our center and received SPD. The ICGR15, Child-Pugh classiifcation, model for end-stage liver disease (MELD) score, and perioperative characteristics were analyzed retrospectively.
RESULTS: Preoperative liver function assessment revealed that 34 patients were Child-Pugh class A with ICGR15 of 13.6%-43.0% and MELD score of 7-20; 8 patients were class B with ICGR15 of 22.8%-40.7% and MELD score of 7-17; 1 patient was class C with ICGR15 of 39.7% and MELD score of 22. The optimal ICGR15 threshold for liver function com-pensation was 31.2%, which offered a sensitivity of 68.4% and a speciifcity of 70.8%. Univariate analysis showed preopera-tive ICGR15, MELD score, surgical procedure, intraoperative blood loss, and autologous blood transfusion were signiifcant-ly different between postoperative liver function compensated and decompensated groups. Multivariate regression analysis revealed that ICGR15 was an independent risk factor of post-operative liver function recovery (P=0.020).
CONCLUSIONS: ICGR15 has outperformed the Child-Pugh classiifcation for assessing liver function in cirrhotic patients with portal hypertension. ICGR15 may be a suitable prognos-tic indicator for cirrhotic patients after SPD.     

Laparoscopic splenectomy for hypersplenism secondary to liver cirrhosis and portal hypertension

Since the first laparoscopic splenectomy(LS)was reported in 1991,LS has become the gold standard for the removal of normal to moderately enlarged spleens in benign conditions.Compared with open splenectomy,fewer postsurgical complications and better postoperative recovery have been observed,but LS is contraindicated for hypersplenism secondary to liver cirrhosis in many institutions owing to technical difficulties associated with splenomegaly,well-developed collateral circulation,and increased risk of bleeding.With the improvements of laparoscopic technique,the concept is changing.This article aims to give an overview of the latest development in laparoscopic splenectomy for hypersplenism secondary to liver cirrhosis and portal hypertension.Despite a lack of randomized controlled trial,the publications obtained have shown that with meticulous surgical techniques and advanced instruments,LS is a technically feasible,safe,and effective procedure for hypersplenism secondary to cirrhosis and portal hypertension and contributes to decreased blood loss,shorter hospital stay,and less impairment of liver function.It is recommended that the dilated short gastric vessels and other enlarged collateral circulation surrounding the spleen be divided with the LigaSure vessel sealing equipment,and the splenic artery and vein be transected en bloc with the application of the endovascular stapler.To support the clinical evidence,further randomized controlled trials about this topic are necessary.     

Evaluation of surgical procedure selection based on intraoperative free portal pressure measurement in patients with portal hypertension

BACKGROUND:Various surgical procedures can be used to treat liver cirrhosis and portal hypertension.How to select the most appropriate procedure for patients with portal hypertension has become a difficult problem.This study aimed to analyze the relationship between the value of intraoperative free portal pressure(FPP)and postoperative complications,and to explore the significance of intraoperative FPP measurement with respect to surgical procedure selection.METHODS:The clinical data of 187 patients with portal hypertension who received pericardial devascularization and proximal splenorenal shunt combined with devascularization(combined operation)at the Department of General Surgery in our hospital from January 2001 to September 2008 were retrospectively analyzed.Among the patients who received pericardial devascularization,those with a postoperative FPP ≥22 mmHg were included in a high-pressure group(n=68), and those with FPP22 mmHg were in a low-pressure group(n=49).Seventy patients who received the combined operation comprised a combined group.The intraoperative FPP measurement changes at different times,and the incidence of postoperative complications in the three groups of patients were compared.RESULTS:The postoperative FPP value in the high-pressure group was 27.5±2.3 mmHg,which was significantly higher than that of the low-pressure(20.9±1.8 mmHg)or combined groups(21.7±2.5 mmHg).The rebleeding rate in the high-pressure group was significantly higher than that in the low-pressure and combined groups.The incidence rates of postoperative hepatic encephalopathy and liver failure were not statistically different among the three groups.The mortality due to rebleeding in the low-pressure and combined groups(0.84%) was significantly lower than that of the high-pressure group.CONCLUSIONS:The study demonstrates that FPP is a critical measurement for surgical procedure selection in patients with portal hypertension.A FPP value≥22 mmHg after splenectomy and devascularization alone is an important indicator that an additional proximal splenorenal shunt needs to be performed.     

Simultaneous partial splenectomy during liver transplantation for advanced cirrhosis patients combined with severe splenomegaly and hypersplenism

BACKGROUND The most effective treatment for advanced cirrhosis and portal hypertension is liver transplantation(LT).However,splenomegaly and hypersplenism can persist even after LT in patients with massive splenomegaly.AIM To examine the feasibility of performing partial splenectomy during LT in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism.METHODS Between October 2015 and February 2019,762 orthotopic LTs were performed for patients with end-stage liver diseases in Tianjin First Center Hospital.Eighty-four cases had advanced cirrhosis combined with severe splenomegaly and hypersplenism.Among these patients,41 received partial splenectomy during LT(PSLT group),and 43 received only LT(LT group).Patient characteristics,intraoperative parameters,and postoperative outcomes were retrospectively analyzed and compared between the two groups.RESULTS The incidence of postoperative hypersplenism(2/41,4.8%)and recurrent ascites(1/41,2.4%)in the PSLT group was significantly lower than that in the LT group(22/43,51.2%;8/43,18.6%,respectively).Seventeen patients(17/43,39.5%)in the LT group required two-stage splenic embolization,and further splenectomy was required in 6 of them.The operation time and intraoperative blood loss in the PSLT group(8.6±1.3 h;640.8±347.3 mL)were relatively increased compared with the LT group(6.8±0.9 h;349.4±116.1 mL).The incidence of postoperative bleeding,pulmonary infection,thrombosis and splenic arterial steal syndrome in the PSLT group was not different to that in the LT group,respectively.CONCLUSION Simultaneous PSLT is an effective treatment and should be performed in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism to prevent postoperative persistent hypersplenism.     

Ectopic liver: Different manifestations,one solution

Developmental abnormalities are rare in the liver.This study presents two case reports of ectopic liver.The first case was a 31-year-old male with clinical indication for laparoscopic appendectomy.Laparoscopy identified a perforated appendix and an unknown tumorous lesion in the ligamentum hepato umbilicalis.The patient underwent a laparoscopic appendectomy,intraoperative lavage of the peritoneal cavity,and extirpation of the lesion in the ligamentum hepato umbilicalis.Histpathological examination of the excised tumor revealed that it comprised liver tissue with fibrinous changes.The tumor was completely separate from the liver with no connection.It was classified as an ectopic liver.No further therapy was required.The second case was a59-year-old male with a tumor on the upper pole of the spleen,incidentally diagnosed in an ultrasound examination.The biopsy raised suspicion of hepatocellular carcinoma.A positron emission tomography-computed tomography examination revealed accumulation of F-18 fluorodeoxyglucose only in the tumor.The patient underwent a splenectomy with a resection and reconstruction of diaphragm.After the hepatocellular carcinoma was confirmed,adjuvant therapy(sorafenib)was initialized.The operations and postoperative recoveries were uncomplicated in both cases.Despite the low incidence of ectopic liver and rare complications,it is necessary to maintain awareness of this possibility.The potential malignancy risk for ectopic liver tissue is the basis for radical surgical removal.Therapy for hepatocellular carcinoma in an ectopic liver follows the same guidelines as those followed for treating the"mother"liver.     

Cirrhosis and portal hypertension: The importance of risk stratification,the role of hepatic venous pressure gradient measurement

Portal hypertension is the main prognostic factor in cirrhosis. The recent emergence of potent antiviral drugs and new algorithm of treatment for the management of complications due to portal hypertension have sensibly changed our perception of cirrhosis that can be now considered as a multistage liver disease whose mortality risk can be reduced by a tailored approachfor any stage of risk. Experts recommend to move toward a pathophysiological classification of cirrhosis that considers both structural and functional changes. The hepatic venous pressure gradient HVPG, is the reference gold standard to estimate the severity of portal hypertension in cirrhosis. It correlates with both structural and functional changes that occur in cirrhosis and carries valuable prognostic information to stratify the mortality risk. This article provides a general overview of the pathophysiology and natural course of cirrhosis and portal hypertension. We propose a simplified classification of cirrhosis based on low, intermediate and high mortality stage. The prognostic information provided by HVPG is presented according to each stage. A comparison with prognostic models based on clinical and endoscopic variables is discussed in order to evidence the additional contribute given by HVPG on top of other clinical and instrumental variables widely used in clinical practice.     

Splenogastrorenal shunt in portal hypertension: a little known entity. Study of 6 cases and review of the literature

The authors report 6 cases of portal hypertension with gastrorenal shunt. This shunt did not arise from the left gastric vein, but from the splenic vein. Portal hypertension was related to alcoholic cirrhosis in 3 cases, to extensive portal thrombosis in 2 cases, and to nodular regenerative hyperplasia of the liver in one case. A gastrointestinal hemorrhage revealed portal hypertension and the liver disease in the 3 cases of alcoholic cirrhosis and complicated the course of the disease in the other cases. Hemorrhage was either massive and life-threatening or often recurred. It was related to a rupture of fundic varices in all cases. The fundic varices were not associated with esophageal varices in the 3 cases of cirrhosis. The degree of portal hypertension was above 20 mm Hg, as assessed by the portohepatic gradient (one case), or the pressure gradient between a tributary portal system vein and the inferior vena cava during laparotomy (5 cases). Definitive control of hemorrhage could not be achieved by endoscopic variceal sclerotherapy (2 cases) or percutaneous transhepatic embolization (one case). Portacaval shunt or splenectomy was performed in 5 cases. These findings suggest that spontaneous splenogastrorenal shunt is a clinical and hemodynamic entity which requires specific treatment when associated with gastric variceal bleeding.     

部分脾动脉栓塞术对肝硬化门静脉高压症血流动力学的影响

目的探讨部分脾动脉栓塞术（PSE）治疗肝硬化门静脉高压症的临床疗效及应用价值。方法 23例不同原因肝硬化门静脉高压患者行PSE治疗,PSE术前及术后1周、1、3、6个月彩色多普勒分别检测患者门静脉和脾静脉的内径、血流速度,计算血流量,并与PSE术前配对分析。结果术后各期患者脾静脉、门静脉血流速度及血流量较术前降低（P〈0.05）;脾静脉内径术后各期与术前相比明显缩小（P〈0.05）;而门静脉主干内径术后6个月内缩小不明显（P〉0.05）。结论 PSE能有效的降低门静脉压力。     

Natural course of splenic artery aneurysm with associated spontaneous splenorenal shunt in non-cirrhotic liver: An 18-year observational follow-up and review of literature

Through a review of the literature, a splenic artery aneurysm (SAA) with associated spontaneous splenorenal shunt (SSRS) was only reported in patients with liver cirrhosis and portal hypertension. However, a natural course of a SAA with associated SSRS was found in a non-cirrhotic male patient during an 8-year observational follow-up, and thus reported. Initially, splenomegaly and thrombocytopenia were noted; SSRS was observed later with a tortuous dilated splenic artery, and a SAA was then progressively formed and found. The patient received splenectomy with aneurysm resection and SSRS was preserved. Post-operative follow-up revealed that the size of the SSRS was reduced. Through the course, no abnormalities of liver enzymes, portal hypertension, or esophageal-gastric varicose were found in the patient. No positive association was demonstrated between the formation of SSRS and the severity of liver cirrhosis in patients, implying some other factors, e.g., vascular endothelial growth factor (VEGF) mentioned in the literature, might be involved.     

Spontaneous splenic rupture in a patient with large hepatocellular carcinoma

We report a 61-yr-old woman with acute circulatory failure from spontaneous splenic rupture with decompensated liver cirrhosis complicating large hepatocellular carcinoma exposed on the right liver surface. On admission, the patient was tentatively diagnosed as having rupture of the hepatocellular carcinoma, and she died 15 hours after admission despite blood transfusion. Autopsy revealed that the origin of the intraperitoneal hemorrhage was a ruptured spleen. According to a MEDLINE search of the English-language literature published between 1966 and March 1998, this is the first reported case of spontaneous splenic rupture in a patient with hepatocellular carcinoma without splenic metastasis.     

Ectopic Jejunal Variceal Rupture in a Liver Transplant Recipient Successfully Treated With Percutaneous Transhepatic Coil Embolization: A Case Report

Here we present the rupture of ectopic jejunal varices developing in a liver transplant recipient without portal hypertension, which was successfully treated with percutaneous transhepatic coil embolization.A 48-year-old man with massive melena was admitted to our department. He had undergone liver transplantation for hepatitis B virus-related liver cirrhosis 8 months before, and his postoperative course was satisfactory except for an acute cellular rejection. No evidence of bleeding was detected by upper endoscopy or colonoscopy, but dynamic multidetector computed tomography of the whole abdomen revealed an intestinal varix protruding into the lumen of the jejunum with suspected extravasation. There was no evidence of portal venous stenosis or thrombosis. Immediately upon diagnosis of the ruptured ectopic jejunal varix, percutaneous transhepatic coil embolization was performed, achieving complete hemostasis. The portal venous pressure measured during the procedure was within normal limits. He was discharged from the hospital 11 days after embolization and remained in stable condition without re-bleeding 6 months after discharge.This is the first report of an ectopic intestinal variceal rupture in an uneventful liver transplant recipient that was successfully treated with interventional percutaneous transhepatic coil embolization. Clinicians encountering liver transplant recipients with melena should be aware of the possibility of late-onset rupture of ectopic varices, even in those having an uneventful post-transplant course without portal hypertension.     

Alpha-1 antitrypsin deficiency and splenic artery aneurysm rupture: an association?

OBJECTIVE: Theoretically, patients with alpha 1-antitrypsin deficiency may be vulnerable to the development of splenic artery aneurysms. alpha-1 antitrypsin deficiency can induce cirrhosis with portal hypertension, and resulting protease-antiprotease imbalances may exaggerate arterial wall weakness due to proteolysis of arterial structural proteins. A splenic artery aneurysm rupture 7 days after liver transplantation provoked a reassessment of the incidence of this phenomenon in a liver transplant population. METHODS: Case records from three institutions and the results of a survey sent to 126 liver transplantation programs in the United Network for Organ Sharing database were reviewed. The incidence of splenic artery aneurysm rupture in the peritransplantation period, etiology of liver disease associated with this phenomenon, and recommendations regarding management of splenic artery aneurysms was assessed. RESULTS: Twenty-one cases of splenic artery aneurysm rupture were identified. alpha-1 antitrypsin deficiency was the most common cause of cirrhosis in the majority of identified patients who presented with splenic artery aneurysm rupture, which was associated with a mortality rate of 57%. Respondents to the survey indicated that a preoperative evaluation was warranted if a splenic artery aneurysm was suspected; however, no consensus regarding management exists. CONCLUSIONS: The presence and risk of rupture of splenic artery aneurysms may be greater in patients with alpha-1 antitrypsin deficiency. If identified before rupture, an aggressive approach to diagnosing and treating these aneurysms should be initiated. At present, no consensus exists regarding the management of splenic artery aneurysms.     

Spontaneous splenic rupture during Pringle maneuver in liver surgery

During liver resection clamping of the hepato-duodenal ligament (the Pringle maneuver) is performed to reduce intraoperative blood-loss. During this maneuver acute portal hypertension may lead to spontaneous splenic rupture requiring rapid splenectomy in order to control blood loss. We present 2 case of patients with hemorrhage from the spleen during clamping for liver surgery. A review of the literature with an emphasis on the pathophysiology of splenic hemorrhage is presented.     

A case of gastric adenocarcinoma presenting as portal hypertension

Portal vein thrombus has been detected in patients with liver cirrhosis, pancreatitis, ulcerative colitis, septicemia, myeloproliferative disorder, and neoplasm. The formation of portal tumor thrombus by hepatocellular carcinoma is well recognized, because of its high incidence, and subsequent development of portal hypertension such as rupture of varices, ascites and liver failure indicates the poor prognosis. In gastric cancer, portal hypertension as an initial presentation is extremely rare. Herein we report a case presenting as portal hypertension caused by tumor thrombus without invasion of liver parenchyma. It is presumed to be intraluminal tumor thrombus originating from primary foci of gastric adenocarcinoma. Tumor thrombus in the portal vein is demonstrated on the PET-CT.     

Formation, hemodynamics and new management options for gastric varices

Abstract   Gastric varices develop in patients with portal hypertension, including liver cirrhosis, idiopathic portal hypertension as well as left sided-local portal hypertension such as splenic vein thrombosis or splenic AV malformation. The inflow vein is the left gastric vein, posterior vein, or short gastric vein, while the outflow vein is the gastro-renal shunt in most of the patients with gastric varices. The form of the gastric varices is classified into three types of venous dilatation; tortuous type, notched type and tumor type according to the shape and size of the varices. The location is classified into five sites; the posterior site, anterior site, greater curvature site and lesser curvature site of the cardiac area, and the fundic area. The risk of the rupture depends on the mucosal factor of the varices as well as the location and the form. The hemostasis rate has been improved to 94–97% with the usage of the endoscopic occlusive agent such as Histoacryl. It is absolutely necessary to eradicate the gastric varices within a few weeks after rupture of the gastric varices. There are new management options such as laparoscopic Hassab's operation or balloon-occluded retrograde transvenous obliteration of the varices (B-RTO). The 5-year cumulative rate of the non-variceal bleeding is more than 90% after the B-RTO as well as after surgery. Further prospective clinical trials are to be investigated for an evidence-based medicine.