Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults(with video)

Background:Extrahepatic portal vein obstruction(EHPVO)results in severe portal hypertension(PHT)leading to severely compromised quality of life.Often,pharmacological and endoscopic management is unable to solve this problem.Restoring hepatic portal flow using meso-Rex bypass(MRB)may solve it.This procedure,uncommon in adult patients,is considered the treatment of choice for EHPVO in children.Methods:From 1997 to 2018,8 male and 6 female adults,with a median age of 51 years(range 22-66)underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels,Belgium.Symp-toms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings,sepsis due to portal biliopathy,and/or severe abdominal discomfort.The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein.Results:Median operative time was 500 min(range 300-730).Median follow-up duration was 22 months(range 2-169).One patient died due to hemorrhagic shock following percutaneous transluminal interven-tion for early graft thrombosis.Major morbidity,defined as Clavien-Dindo score≥III,was 35.7%(5/14).Shunt patency at last follow-up was 64.3%(9/14):85.7%(6/7)of pure venous grafts and only 42.9%(3/7)of prosthetic graft.Symptom relief was achieved in 85.7%(12/14)who became asymptomatic after MRB.Conclusions:Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications.In such patients,MRB represents the only and last resort to restore physiological portal vein flow.Although successful in a majority of patients,this procedure is associated with major morbidity and mortality and should be done in tertiary centers expe-rienced with vascular liver surgery to get the best results.

Management of splenic artery aneurysm associated with extrahepatic portal vein obstruction

BACKGROUND: Splenic artery aneurysms although rare are clinically significant in view of their propensity for spontaneous rupture and life-threatening bleeding. While portal hypertension is an important etiological factor, the majority of reported cases are secondary to cirrhosis of the liver. We report three cases of splenic artery aneurysms associated with extrahepatic portal vein obstruction and discuss their management. METHODS: The records of three patients of splenic artery aneurysm associated with extrahepatic portal vein obstruction managed from 2003 to 2010 were reviewed retrospectively. The clinical presentation, surgical treatment and outcome were analyzed. RESULTS: The aneurysm was >3 cm in all patients. The clinical symptoms were secondary to extrahepatic portal vein obstruction (hematemesis in two, portal biliopathy in two) while the aneurysm was asymptomatic. Doppler ultrasound demonstrated aneurysms in all patients. A proximal splenorenal shunt was performed in two patients with excision of the aneurysm in one patient and ligation of the aneurysm in another one. The third patient had the splenic vein replaced by collaterals and hence underwent splenectomy with aneurysmectomy. All patients had an uneventful post-operative course. CONCLUSIONS: Splenic artery aneurysms are associated with extrahepatic portal vein obstruction. Surgery is the mainstay of treatment. Although technically difficult, it can be safely performed in an experienced center with minimal morbidity and good outcome.

Non-Hodgkin lymphoma as a cause of obstructive jaundice with simultaneous extrahepatic portal vein obstruction:A case report

Non-Hodgkin lymphoma is a rare cause of biliary ob-struction. To the best of our knowledge, non-Hodgkin lymphoma in the peripancreatic region causing obstruc-tive jaundice with simultaneous portal vein (PV) inva-sion has not yet been reported. We present a 50-year-old patient with obstructive jaundice whose extrahepatic portal vein was obstructed by the invasion of a peripan-creatic non-Hodgkin lymphoma. The patient denied any other symptoms such as recurrent fever, night sweat and loss of body weight. Computed tomography (CT) revealed a 10 cm mass in the retroperitoneal space behind the head of the pancreas causing obstruction of the distal bile duct and the PV. A pylorus-preserving pancreaticoduodenectomy combined with a PV resection was performed. The PV was reconstructed using an au-tologous right internal jugular vein graft. The resected specimen showed endoluminal invasion of both the bile duct and the PV. Histological examination showed the mass consisting of diffuse sheets of large malignant lymphoid cells. These cells were positive for CD20 and CD79a, partially positive for CD10, and negative for CD3, CD4, CD5, CD8 and CD30. The pathologic diagno-sis was diffuse large B-cell type non-Hodgkin lymphoma and the patient was transferred to the Department of Hematology and Oncology for chemotherapy. He re-ceived four cycles of combined chemotherapy including cyclophosphamide, doxorubicin, vincristine and pred-nisone plus rituximab, and three cycles of intrathecal chemoprophylaxis including methotorexate, cytosine arbinoside and prednisone. The patient is alive with noevidence of the disease for 7 mo after operation and will receive additional courses of chemotherapy.

Treatment of Retinal Vein Obstruction with Acupuncture and Chinese Medicinal Herbs

We have treated thirty-two cases (52 eyes) of retinal vein obstruction by acupuncture and oral administration of Huo Xue Ming Mu Decoction. The total effective rate of 90.38% demonstrated that the treatment was definitely effective.

Successful upgrade to cardiac resynchronization therapy for cardiac implantation-associated left subclavian vein occlusion:A case report

BACKGROUND Subclavian vein stenosis or occlusion may be caused by a transvenous pacemaker,which makes the reimplantation of a new pacemaker lead difficult.Transvenous pacemaker lead implantation-related subclavian vein occlusion may present difficulty with regard to cardiac resynchronization therapy(CRT)upgrade.CASE SUMMARY We report the case of a 46-year-old man who was admitted with total subclavian vein occlusion caused by a permanent pacemaker that had been implanted 2 years previously.We successfully treated this patient with an upgrade to a CRT pacemaker by utilizing transferable interventional coronary and radiological techniques.The patient recovered uneventfully during the follow-up period.CONCLUSION CRT upgrade is still a viable technique for the treatment of subclavian vein obstruction caused by previous pacemaker implantation.

Current approaches to the management of jejunal variceal bleeding at the site of hepaticojejunostomy after pancreaticoduodenectomy

Jejunal variceal bleeding at the site of hepaticojejunostomy after pancre-aticoduodenectomy due to portal hypertension caused by extrahepatic portal vein obstruction is a life-threatening complication and is very difficult to treat.Pharma-cotherapy,endoscopic methods,transcatheter embolization of veins supplying the jejunal afferent loop,portal venous stenting,and surgical procedures can be used for the treatment of jejunal variceal bleeding.Nevertheless,the optimal mana-gement strategy has not yet been established,which is due to the lack of ran-domized controlled trials involving a large cohort of patients necessary for their development.

Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.

Hepatobiliary and pancreatic disorders in celiac disease

A variety of hepatic and biliary tract disorders may complicate the clinical course of celiac disease. Some of these have been hypothesized to share common genetic factors or have a common immunopathogenesis, such as primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune forms of hepatitis or cholangitis. Other hepatic changes in celiac disease may be associated with malnutrition resulting from impaired nutrient absorption, including hepatic steatosis. In addition, celiac disease may be associated with rare hepatic complications, such as hepatic T-cell lymphoma. Finally, pancreatic exocrine function may be impaired in celiac disease and represent a cause of treatment failure.

Diagnosis and treatment of portal biliopathy

Background: Portal biliopathy producing intrahepa- tic and extrahepatic biliary ductai abnormalities from portal hypertension, particularly with extrahepatic portal vein obstruction (EHPVO) is common. A majority of these patients are asymptomatic, but oc- casionally there is symptomatic biliary obstruction, and cholangitis and choledocholithiasis. Objective: To explore the principles of diagnosis and treatment of portal biliopathy. Data sources: To review the literature of portal bili- opathy. Conclusions: Endoscopic sphincterotomy, stone ex- traction and supportive drainage could effectively re- lieve cholangitis when jaundice is associated with common bile duct stones. Definitive decompressive portal-system vein shunting operation and choledo- cho-jejunostomy are sometimes required when biliary obstruction is recurrent and progressive.

Pediatric non-cirrhotic portal hypertension:Endoscopic outcome and perspectives from developing nations

Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in children.Variceal bleed and splenomegaly are their predominant presentation.Laboratory features show cytopenias(hypersplenism)and preserved hepatic synthetic functions.Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases.After variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO.Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate.Shunt surgeries prevent rebleed,improve growth and QoL.Non-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade,massive splenomegaly and patent portal vein are discriminating features of NCPF.Shunt surgery is required in severe cases when endotherapy is insufficient for the varices.Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly.Ductal plate malformation forms the histological hallmark of CHF.CHF is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy.Liver transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s syndrome.Combined liver-kidney transplantation is indicated when both liver and renal issues are present.

Redefining Budd-Chiari syndrome:A systematic review

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

Important Newborn Cardiac Diagnostic Dilemmas for the Neonatologist and Cardiologist-A Clinical Perspective

Most congenital heart disease(CHD)is readily recognisable in the newborn.Forewarned by previous fetal scanning,the presence of a murmur,tachypnoea,cyanosis and/or differential pulses and saturations all point to a cardiac abnormality.Yet serious heart disease may be missed on a fetal scan.There may be no murmur or clinical cyanosis,and tachypneoa may be attributed to non-cardiac causes.Tachypnoea on day 1 is usually non-cardiac except arising from ventricular failure or a large systemic arteriovenous fistula.A patent ductus arteriosus(PDA)may support either pulmonary or systemic duct dependent circulations.The initially high pulmonary vascular resistance(PVR)limits shunts so that murmurs even from large communications between the systemic and pulmonary circulations take days/weeks to develop.At times despite expert input,serious CHD maybe difficult to diagnose and warrants close interaction between the neonatologist and cardiologist to reach a timely diagnosis.Such conditions include obstructed total anomalous pulmonary venous connections(TAPVC)and the need to distinguish it from persistent pulmonary hypertension in the newborn(PPHN)–the treatment of the former is surgical the latter medical.A large duct shunting right to left may overshadow a suspected hypoplastic aortic isthmus and/or coarctation.Is the right to left shunting because of severe aortic obstruction or resulting from a high PVR with little obstruction.The diagnosis of pulmonary vein stenosis(PVS)remains problematic often developing in premature infants with ongoing bronchopulmonary dysplasia(BPD),still being cared for by the neonatologist.While there are other diagnostic dilemmas including deciding the contribution of a recognised CHD in a sick neonate,this paper will focus on the above-mentioned conditions with suggestions on what may be done to arrive at a timely diagnosis to achieve optimal outcomes.

Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas

Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.