Vein Agenesis Research Articles

Right jugular vein agenesis. a challenging case for experienced endocrine surgeons

Right jugular vein agenesis. a challenging case for experienced endocrine surgeons

Treatment of external iliac veins agenesis: Case report

Iliac vein agenesis is a rare condition, which can lead to symptoms of CVI of lower limbs and to DVT. CTV, MRV and phlebography are effective methods on diagnosis. The treatment of these patients consists of anticoagulation in cases of DVT and venous grafts in cases of CVI. The patient is a 28-year-old female, with history of varicose veins in her face when she was a baby, varicose veins in lower limbs, edema and pain in adolescence and varicose veins in the abdominal wall and thorax in adulthood.

Double superior vena cava and left brachiocephalic vein agenesis: a rare systemic vein anomaly and potential source of cardiac implantable electronic device and central venous catheter placement complications.

Abnormal systemic vein development produces anomalous veins, which - in the case of persistent left superior vena cava and/or left brachiocephalic vein - exhibit considerable topographic and morphometric differences in comparison with their usual anatomy. The nature and extent of those developmental anomalies - detected during intravenous procedures, such as cardiac implantable electronic device (CIED) lead insertion or central venous catheter placement - may hinder the procedure itself and/or adversely affect its outcome, both at the stage of cardiac lead advancement through an abnormally shaped vessel and lead positioning within the heart. This may lead to problems in achieving optimal sensing and pacing parameters and in ensuring that the patient cannot feel the pacing impulses. These events accompanied a de novo CIED implantation procedure in the patient with a double superior vena cava and left brachiocephalic vein agenesis, who ultimately required reoperation.

Treatment of external iliac veins agenesis: Case report

Iliac vein agenesis is a rare condition, which can lead to symptoms of CVI of lower limbs and to DVT. CTV, MRV and phlebography are effective methods on diagnosis. The treatment of these patients consists of anticoagulation in cases of DVT and venous grafts in cases of CVI. The patient is a 28-year-old female, with history of varicose veins in her face when she was a baby, varicose veins in lower limbs, edema and pain in adolescence and varicose veins in the abdominal wall and thorax in adulthood. She underwent Venous Ultrasound of lower limbs that identified saphenous and tributary insufficiency and no signs of DVT. She then had MRV of thorax, abdomen and pelvis that identified external iliac vein agenesis and collateral veins in all three areas.

A226 INCIDENTAL FINDING OF A LARGE GASTRIC VARIX ASSOCIATED WITH NEONATAL UMBILICAL VEIN CANALIZATION

Abstract Results A 41-year old Asian male, who immigrated to Canada many years ago, and who had previously been successfully treated for Helicobacter pylori infection underwent gastroscopy for investigation of dyspepsia. His gastroscopy was normal except for a large subepithelial abnormality that was noted close to the gastroesophageal junction. Routine gastric biopsies from the antrum and body were normal. Subsequent endoscopic ultrasound revealed flow through the anechoic tortuous lesion and confirmed it was a very large isolated gastric varix type 1. Abdominal CT scan revealed chronic occlusion of the portal vein, splenic vein, and the portal confluence with extensive collateralization in the upper abdomen. There was complete cavernous transformation of the portal vein. Of the numerous varices in the upper abdomen, a very large varix drained into the left renal vein and indented into the posterior wall of the fundus of the stomach which accounted for the endoscopic finding. Multiple mesenteric veins were identified that connected to varices adjacent to the inferior aspect of the pancreas and duodenum. Notably, there was no evidence of cirrhosis or chronic pancreatitis. Liver enzymes, albumin, and INR were normal. Further collateral history revealed that he was hospitalized as a neonate for pneumonia with catheterization of the umbilical vein, which is known to be associated with thrombosis of the portal vein. Conclusions Detection of congenital absence of the portal vein (CAPV) is recognized more often due to advances in diagnostic imaging. Radiologically, the absence of the portal vein in CAPV is distinguished from portal vein thrombosis by the lack of venous collaterals or sequalae of portal hypertension, such as ascites or splenomegaly. A more gradual thrombosis of the portal vein may permit collaterals to develop without acute changes and is not equivalent to portal vein aplasia or agenesis as intrahepatic bile ducts are normal. The gold standard for diagnosis of CAPV is histologic absence of the portal vein in the liver on catheter angiography. CAPV is associated with abnormal embryologic development of the portal vein and frequently presents with complications of portal hypertension or portosystemic encephalopathy or the sequalae of venous shunts, hepatic or cardiac abnormalities found on imaging. Our case is an incidentally discovered absence of the portal venous system due to chronic thrombosis with extensive collateralization and an enlarged gastric varix protruding into the proximal stomach. It is well documented that canalization of the umbilical vein in infancy is associated with portal vein thrombosis, with incidences up to 68%. This case highlights the importance of eliciting a childhood hospitalization history in cases of non-cirrhotic portal hypertension. Funding Agencies None

Clinical outcomes and risk factors of hepatopulmonary syndrome in children

Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA). We performed a retrospective cohort study of all children who were diagnosed with HPS between 2000 and 2018 at Seoul National University Hospital. The clinical features and outcomes of the 10 patients diagnosed with HPS were reviewed. To clarify the risk factors of HPS in patients with BA, we reviewed 120 patients diagnosed with BA. Underlying liver disease was BA in 8 patients, portal vein agenesis in 1 patient, and portal vein thrombosis in 1 patient. A total of 7 patients underwent liver transplantation (LT). Currently, all seven patients, including 3 patients with severe HPS, survived after LT. The prevalence of HPS in children with BA was 7%. Polysplenia/interrupted inferior vena was the only risk factor for HPS in BA patients in multivariate analysis. The Pediatric End-Stage Liver Disease score was not associated with the development of HPS. Children with severe HPS undergoing LT had excellent outcomes. Screening for HPS in children with BA is required regardless of the severity of liver diseases.

Whole liver deceased donor liver transplantation for pediatric recipients: single-center experience for 20 years.

BackgroundWe investigated the incidence and outcomes of pediatric deceased donor liver transplantation (DDLT) using whole liver grafts in a high-volume liver transplantation (LT) center.MethodsThe study was a retrospective single-center analysis of whole LT in pediatric recipients. The study period was set as 20 years between January 2000 and December 2019. We defined pediatric recipients and donors to be aged ≤18 years.ResultsDuring the study period, there were 98 cases of pediatric DDLT, and 34 patients (34.7%) received whole liver grafts. The age range of the deceased donors was 3 months to 56 years and that of pediatric recipients was 7 months to 17 years. Common primary diseases for LT were biliary atresia in 13, acute liver failure in four, Wilson disease in four, congenital portal vein agenesis in three, and genetic metabolic diseases in three. Pediatric-to-pediatric and adult-to-pediatric whole LTs were 22 (64.7%) and 12 (35.3%), respectively. A good correlation was noted between the donor and the recipient’s body weight, and the recipient’s body weight and allograft’s weight. Graft and overall patient survival rates were 91.2% and 91.2% at 1 year, 88.0% and 88.0% at 3 years, and 88.0% and 88.0% at 5 years, respectively.ConclusionsThe results of this study revealed that Korean Network for Organ Sharing (KONOS) regulations with donor-recipient body weight matching exhibited good performance. Considering the reciprocal trades of liver organs among pediatric and adult donors and recipients, it is necessary to establish a policy for pediatric donor liver grafts to pediatric recipients on a priority basis.

Whole liver deceased donor liver transplantation for pediatric recipients: single-center experience for 20 years

Background: We investigated the incidence and outcomes of pediatric deceased donor liver transplantation (DDLT) using whole liver grafts in a high-volume liver transplantation (LT) center. Methods: The study was a retrospective single-center analysis of whole LT in pediatric recipients. The study period was set as 20 years between January 2000 and December 2019. We defined pediatric recipients and donors to be aged ≤18 years. Results: During the study period, there were 98 cases of pediatric DDLT, and 34 pa tients (34.7%) received whole liver grafts. The age range of the deceased donors was 3 months to 56 years and that of pediatric recipients was 7 months to 17 years. Common primary diseases for LT were biliary atresia in 13, acute liver failure in four, Wilson dis ease in four, congenital portal vein agenesis in three, and genetic metabolic diseases in three. Pediatric-to-pediatric and adult-to-pediatric whole LTs were 22 (64.7%) and 12 (35.3%), respectively. A good correlation was noted between the donor and the re cipient’s body weight, and the recipient’s body weight and allograft’s weight. Graft and overall patient survival rates were 91.2% and 91.2% at 1 year, 88.0% and 88.0% at 3 years, and 88.0% and 88.0% at 5 years, respectively. Conclusions: The results of this study revealed that Korean Network for Organ Sharing (KONOS) regulations with donor-recipient body weight matching exhibited good perfor mance. Considering the reciprocal trades of liver organs among pediatric and adult do nors and recipients, it is necessary to establish a policy for pediatric donor liver grafts to pediatric recipients on a priority basis.

Rare anatomical variation of the azygos system

This study presents a rare case of azygos vein agenesis associated with hemiazygos vein agenesis. The observed anatomical variation consists in the existence of a large venous trunk located entirely to the left of the midline that receives drainage from the right and left posterior intercostal veins and drains into the superior vena cava by means of an arch. No vein in typical azygos or hemiazygos vein topography was observed. This anatomical variation may lead to clinical and hemodynamic repercussions, while also presenting anatomical and radiological importance regarding surgical planning concerning posterior mediastinal structures.

Portal-to-right portal vein bypass for extrahepatic portal vein obstruction

ObjectiveRex shunt (mesenteric-to-left portal vein bypass) is considered a more physiologically rational treatment for EHPVO than other portosystemic systemic shunts in children. However, about 13.6% of children with EHPVO do not have usable left portal veins and up to 28.1%. Rex operations in children are not successful. Hence, a Rex shunt in these children was impossible. This study reports a novel approach by portal-to-right portal vein bypass for treatment of children with failed Rex shunts. Material and methodsEight children (age 6.1years, range 3.5–8.9years) who underwent Rex shunts developed recurrent gastrointestinal bleeding and hypersplenism 13months (11–30months) postoperatively. After ultrasound confirmation of blocked shunt, they underwent exploration. Three patients were found to have right portal vein agenesis. Five patients (62.5%) were found to have the patent right portal vein, with the diameter of 3–6mm. Four patients underwent bypass between the main portal vein in the hepatoduodenal ligament and the right portal vein by interposing an inferior mesenteric vein autograft, whereas the remaining patient underwent a bypass using ileal mesenteric vein autograft. ResultsThe operations took 2.3h (1.9–3.5h). The estimated blood loss was 50ml (30–80ml), with no complication. The portal venous pressure dropped from 34.6cmH2O (28–45 cmH2O) before the bypass to 19.6cmH2O (14–24cmH2O) after the bypass. The 5 patients were followed up for 10.2months (4–17months) and the post-operative ultrasound and CT angiography confirmed the patency of all the grafts and disappearance of the portal venous cavernova in all five patients. ConclusionThe portal-to-right portal vein bypass technique is feasible and safe for treatment of children with EHPVO who have had failed Rex shunts. Our preliminary result indicates that this technique extends the success of Rex shunt from left portal vein to right portal vein and open a new indication of physiological shunt for some of the children who not only have had failed Rex shunts or but also are not suitable for the Rex shunts. Type of studyTreatment study. Level of evidenceLevel IV.

Liver transplant due to hepatic adenoma in patient with portal vein agenesis: Anastomosis of the donor portal vein to the recipient left gastric vein

Liver transplant due to hepatic adenoma in patient with portal vein agenesis: Anastomosis of the donor portal vein to the recipient left gastric vein

Hepatocellular nodules expressing markers of hepatocellular adenomas in Budd-Chiari syndrome and other rare hepatic vascular disorders

A broad range of hepatocellular nodules has been reported in hepatic vascular disorders. It is not clear whether hepatocellular adenoma (HCA) in this context share the same characteristics as conventional HCA. The aim of this study was to carry out a retrospective multicenter survey of hepatocellular nodules associated with hepatic vascular disorders. Forty-five cases were reviewed, including 32 Budd-Chiari syndrome (BCS). Benign nodules were subtyped using the HCA immunohistochemical panel. Nodules with a HCA morphology were observed in 11 cases. Six originated in BCS: two were liver fatty acid binding protein (LFABP) negative (one with malignant transformation); two expressed glutamine synthetase (GS) and nuclear b-catenin, two expressed C reactive protein (CRP). Among three cases with portal vein agenesis, one nodule was LFABP negative, two expressed GS and nuclear b-catenin, both with malignant transformation. In a Fallot tetralogy case, there were multiple LFABP negative nodules with borderline features and in a hepatoportal sclerosis case, the nodule looked like an inflammatory HCA. Two additional cases had nodules expressing CRP, without typical characteristics of inflammatory HCA. HCA of different immunohistochemical phenotype can develop in hepatic vascular disorders; they may have a different behavior compared to conventional HCA and be more at risk of malignant transformation.

Epidural venous plexus engorgement: what lies beneath?

Epidural venous plexus engorgement may occur due to several conditions that prevent the normal venous circulation. Inferior vena cava agenesis is a very rare cause of epidural venous enlargement. We present a case with a very thin inferior vena cava and left iliac vein agenesis who presented with back pain due to epidural vein engorgement and lacked other venous problems such as deep vein thrombosis.

Benign Hepatocellular Tumors: A Multidisciplinary Approach

Benign Hepatocellular Tumors: A Multidisciplinary Approach

Portal vein agenesis: a case report

Portal vein agenesis is a rare malformation. It is seen particularly with cardiovascular and gastrointestinal system anomalies. Radiologic studies of the portal venous system include color Doppler ultrasonography (US), computed tomography (CT), magnetic resonance imaging, and arterial or direct portography. A ten year old child with recurrent abdominal pain because of portal vein agenesis is presented. Keyword: Portal vein agenesis, Abdominal pain

Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein

Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and splenic venous drainage bypass the liver and drain directly into the systemic veins through various porto-systemic shunts. In this article, we illustrate a case of multiple focal nodular hyperplasia (FNH) with congenital absence of the portal vein in a male, which, to our knowledge, is the third reported case in the literature since its first description in 1793. Furthermore, we discuss the embryology of the portal vein and the Morgan and Superina classification of portosystemic anomalies, the association between portal vein agenesis and multiple FNHs, and, lastly, the use of a hepatocellular-specific MRI contrast agent as an important diagnostic tool in the confirmation of FNH.

Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt?

Background Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients. Methods We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post–umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT. Results It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post–umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now. Conclusions The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post–umbilical catheterization EHPVT is yet to be clearly evaluated.

Anatomic variation: absence of portal vein bifurcation

To describe in CT scan the absence of a portal vein bifurcation. We described the contrast-enhanced CT scan appearance of an absence of portal vein bifurcation in an "asymptomatic" 39year old woman. It was a single intrahepatic portal vein which crossed the entire liver parenchyma from the right to the left and with a gradually decreasing diameter. The vascularization of the hepatic parenchyma was not ensured by third order branches, but directly made by second order ramifications which were regularly originated from the single IHPV along its course. This unusual portal vein mimicking a left portal vein agenesis, appeared in fact to be more in relation with a right portal vein agenesis. Distribution of hepatic veins and hepatic volume was normal. This very rare anatomic variation is easily detected on CT scan and appears important to describe for surgeon and interventional radiologists.

Hypoplastic Right Lung Associated with Right Pulmonary Vein Stenosis and Systemic Collateral

We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidetector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities.

Orthotopic Liver Transplantation for Hepatic Adenoma in a Patient with Portal Vein Agenesis

Congenital portal vein agenesis, known as the Abernathy malformation, was first described in 1793. It is associated with focal nodular hyperplasia and adenomas due to abnormal hepatic regeneration secondary to abnormal or absent flow to the liver. We report a case of a 25-year-old Caucasian female with a complicated medical history including idiopathic membranoproliferative glomerulonephritis, hypertension, hyperlipidemia, Sweet's syndrome, acute febrile neutrophilic dermatosis, who incidentally was diagnosed with multiple liver nodules, the largest 14 cm in diameter, arising at the junction of the left and right hepatic lobes. She was also noted to have a congenital absence of the portal vein. There was no evidence of cirrhosis or portal hypertension. Liver function tests and  - fetoprotein were within normal limits. A biopsy of the largest mass revealed hepatocellular adenoma. Because of the size, location, and multiplicity of the lesions, we elected to proceed with orthotopic liver transplantation. She subsequently underwent deceased donor liver transplantation using the piggyback technique. Despite dividing the portal vein shunt after completion of the caval anastomosis and minimizing the portal vein clamp time, her spleen spontaneously ruptured, for which splenectomy was performed. The patient did well postoperatively with good liver function.