Liver cirrhosis in a patient with hepatic hereditary hemorrhagic telangiectasia and Budd-Chiari syndrome: a case report.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) often involves the liver, and belongs to abnormal blood vessel disease. The etiology of Budd-Chiari syndrome (BCS) is not clear, but congenital vascular dysplasia is considered to be one of the causes. Liver cirrhosis due to hepatic hereditary hemorrhagic telangiectasia concomitant with BCS has not been reported. Here, we report a case of cirrhosis with hepatic hereditary hemorrhagic telangiectasia (HHHT) and BCS. CASE PRESENTATION: A 58-year-old woman with hepatic hereditary hemorrhagic telangiectasia showed decompensated liver cirrhosis, and abdominal imaging revealed Budd-Chiari syndrome. Disease has progressed considerably during 2.5 years after hospital discharge despite subsequent transjugular intrahepatic portosystemic shunting (TIPS). One hypothesis that might explain the coexistence of hepatic hereditary hemorrhagic telangiectasia and Budd-Chiari syndrome in this patient is ischemia and thrombosis of hepatic veins. CONCLUSIONS: Further studies are required to evaluate the relationship between HHHT and BCS. Our observations already challenged the TIPS therapeutic strategy in BCS secondary to HHHT patients.

Laparoscopic ligation of portosystemic shunt for the treatment of congenital intrahepatic portosystemic shunt in one newborn infant.

BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare congenital portal malformation. 35.5% of CPSS is intrahepatic. Certain intrahepatic CPSS cases can be spontaneously closed within 2 years after birth. However, the intrahepatic CPSS with clinical symptoms or large shunt requires immediate treatment. In the present study, we used a laparoscopic ligation of portosystemic shunt to treat one newborn case. MATERIALS AND METHODS: An abnormal shunt between the portal and the hepatic vein was initially detected by a prenatal ultrasound in one newborn infant. The postnatal ultrasound and CT indicated a connection between the left portal and hepatic vein and a venous cyst at the anterior edge of the left liver. Owing to the hyperammonemia and the large shunt from the portal into the hepatic vein, the infant was treated with a laparoscopic ligation of the shunt. During the operation, the left portal vein and its branches were dissected and the anterior branch of the left portal vein was clamped using a Hem-o-lok. The venous cyst was sutured and ligated using a prolene suture. RESULTS: The surgery was completed successfully. The duration of the operation time was 60 min. The postoperative hospital stay was 3 days. The preoperative portal angiography indicated an intrahepatic shunt between the left portal and the left hepatic vein and a venous cyst. Postoperative portal angiography indicated the disappearance of the abnormal shunt and the venous cyst. The portal pressure was increased from the preoperative 6 cmH2O to the postoperative 12 cmH2O. The duration of the follow-up was 22 months. Following surgery, the level of serum ammonia returned to normal. The postoperative CT demonstrated that the abnormal shunt had disappeared. No symptoms of portal hypertension were noted following surgery, such as splenomegaly, hypothrombocytopenia, and upper gastrointestinal bleeding. CONCLUSION: The laparoscopic ligation of portosystemic shunt is suitable for the treatment of the intrahepatic CPSS in the newborn patient.

Vascular Anomalies of the Pediatric Liver.

The liver is a unique organ as it receives afferent blood supply from the umbilical vein, portal vein, and hepatic artery in the developing embryo but has only one efferent drainage method, through the hepatic veins. In the postnatal period, about 70% of the afferent blood flow into the liver is from the portal venous system, unique vessels that begin and end in a capillary system. Vascular anomalies of the hepatic artery, hepatic veins, portal vein, and/or umbilical vein can be congenital or acquired secondary to inflammation and/or infection, trauma, systemic disorders, or iatrogenic causes. The vascular anomalies can be incidental findings at imaging, or the infant or child can present with symptoms such as abdominal pain and ascites, be diagnosed with gastrointestinal bleeding, and have abnormal liver function test results. Imaging can demonstrate vascular findings such as shunts, thrombosis, or collaterals; secondary parenchymal findings such as diffuse or focal abnormal enhancement patterns; and parenchymal lesions such as regenerative nodules. This article discusses and illustrates vascular disorders of the liver that may be encountered in the pediatric population. These include (a) normal vascular variants; (b) congenital anomalies (preduodenal portal vein and infradiaphragmatic total anomalous pulmonary venous return); (c) acquired thromboses (extrahepatic portal venous thrombosis); (d) inflammatory vascular conditions, which can result in hepatic artery aneurysms or pseudoaneurysms; (e) hepatic venous outflow disorders (veno-occlusive disease); and shunt lesions. Liver transplantation and associated vascular complications are a large topic and will not be reviewed in this article. Knowledge of the vascular and parenchymal changes seen with these entities can aid imaging diagnosis and guide appropriate management. ©RSNA, 2019.

Comparison of 3.0T MRI with 3D LAVA sequence and digital subtraction angiography for the assessment of accessory hepatic veins in Budd-Chiari syndrome.

PURPOSE: To compare 3D liver acceleration volume acquisition (LAVA) and digital subtraction angiography (DSA) for evaluating the presence of accessory hepatic veins (AHV) in Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: This was a retrospective study in 228 patients with BCS who underwent 3.0T magnetic resonance imaging (MRI) with the 3D LAVA sequence. Two reviewers noted AHV: openings located in the inferior vena cava (IVC), caliber, and the angle of entering into the IVC. MRI results were compared to DSA. Kappa statistics were calculated to quantify intrareader variability in detecting AHVs. RESULTS: On MRI, 63 patients demonstrated no AHV on LAVA images, 70 had one AHV, 62 had two AHVs, 26 patients had three AHVs, six patients had four AHVs, and one patient had five AHVs (P < 0.05 vs. DSA). The mean caliber of the AHVs was 8.3 ± 4.0 mm compared to 9.9 ± 3.2 for DSA (P < 0.001). Among the 301 AHVs, there were 140 with acute angles (46.5%), 71 with right angles (23.6%), and 90 with obtuse angles (29.8%). The prevalence of AHVs on DSA was 54.8% (125/228), while MRI demonstrated 301 AHVs in 165 patients, for a prevalence of 72.4% (165/228) compared to 54.8% for DSA (P = 0.001). The two methods were concordant in only 116/228 (50.9%) patients. The kappa coefficient demonstrated good intrareader consistency for all documented MRI findings of AHVs (κ = 0.626 for caliber and κ = 0.65 for angles). CONCLUSION: More AHVs were visible on MRI LAVA sequences than on conventional DSA. LEVEL OF EVIDENCE: 4 J. Magn. Reson. Imaging 2017;45:401-409.

Coronary sinus aneurysm associated with multiple venous anomalies.

BACKGROUND: Congenital anomalies of the venous system are rare, involve the inferior vena cava (IVC), a persistent left superior vena cava (PLSVC), and the left hepatic vein (LHV), and can make cardiac diagnostic and therapeutic procedures difficult. CASE PRESENTATION: We present a 67-year-old woman without heterotaxy syndrome associated with interruption of the left IVC that continued with the hemiazygos vein system, a PLSVC, and an anomalous LHV draining the into coronary sinus (CS). The venous anomalies caused a CS aneurysm. The anomalies were demonstrated by echocardiography and the diagnosis was established by contrast-enhanced computed tomography. Three days later, a coronary artery bypass graft was performed, which confirmed the diagnosis. Half a month after surgery, the pain had been relieved and the patient was discharged from the hospital. CONCLUSION: Echocardiography is a useful modality to diagnose and assess anomalies of the CS, including CS aneurysms. Congenital anomalies of the venous system in this case were all due to embryonic development abnormalities. Contrast-enhanced computed tomography provides a more comprehensive view of the entire course of abnormal veins.

Fontan operation with bilateral conduits for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia after reconstruction of a non-confluent pulmonary artery.

We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery.

Anomalous biliary and vascular anatomy-Potential pitfalls during cholecystectomy.

Laparoscopic cholecystectomy is usually a low-risk procedure associated with a short stay and a low rate of conversion to open surgery. Complications are sometimes associated with anomalous vascular or biliary anatomy. Outlined below are the variations in vascular and biliary anatomy which may result in complications either due to involvement in the inflammatory process or inadvertent division during dissection. Clin. Anat. 30:1103-1106, 2017. © 2017 Wiley Periodicals, Inc.

Congenital Vascular Malformations of the Liver: An Association With Trisomy 21.

A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.

Liver resection for the treatment of a congenital intrahepatic portosystemic venous shunt.

Intrahepatic portosystemic shunts (IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis. It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications. When recognized, early intervention to occlude the shunt will reverse the associated complications. Literature review reports of surgical and radiological occlusion of the shunt, but due to its rarity, a standard therapeutic protocol has not been established. A case of a 38-year-old woman with abdominal pain and low grade encephalopathy, diagnosed with an IPSS and treated by right hepatectomy was reported.

Classification and techniques of en bloc venous reconstruction for pancreaticoduodenectomy.

BACKGROUND: Surgical resection is the only cure for hepato-pancreato-biliary (HPB) malignancy. In the era of multidisciplinary approaches and neoadjuvant therapies for locally advanced, borderline resectable tumors, the feasibility and efficacy of en bloc vascular resection has been validated across multiple studies. However, the variability of venous anatomy within the perihepatic and peri-portal regions necessitates familiarity with alternative resection and reconstruction techniques appropriate to the specific region of tumor invasion. METHODS: To organize these paradigms, the venous system has been divided into five zones: 1) hepatic hilum; 2) hepatoduodenal ligament; 3) portal vein/splenic vein confluence, which is further subdivided into right (3a) and left (3b); 4) infra-confluence; and 5) splenic vein. RESULTS: This study systematically analyzes the anatomic considerations and clinical scenarios specific to each zone to organize the necessary preparative maneuvers, surgical procedures, and vascular reconstruction techniques to achieve an R0 resection. The anatomic and tumor-specific factors which deem a specimen unresectable are also explored. Surgical videos demonstrating these techniques are presented. DISCUSSION: Preparation and familiarity with venous reconstruction maneuvers is essential for an oncologically effective operation, and can be safely achieved by utilizing this logical anatomic and procedural framework.

"Single Oval Ostium Technique" using Polytetrafluoroethylene Graft for Outflow Reconstruction in Right Liver Grafts with venous Anomalies in Living Donor Liver Transplantation.

Right lobe living donor liver transplantation form a major source of liver allografts in Asia because of the scarcity of deceased donation. However, the transplant surgeons often face challenges while managing right lobe liver allografts due to variations in vascular anatomy. Such variations have led the transplant team to adopt modifications in existing techniques of inflow and outflow reconstruction. One of such variations is presence of multiple draining inferior right hepatic veins (IRHVs). This hepatic venous anomaly pose a lot of technical difficulties in the outflow reconstruction as second and/or third anastomosis to inferior vena cava is not always possible in limited retrohepatic space. Herein, we describe the "Single oval ostium technique" using dual synthetic vascular grafts ensuring a common outflow channel for all the hepatic veins.

Drainage of the Left Hepatic Vein into the Coronary Sinus, a Rare Intraoperative Finding.

In a 76-year-old female undergoing coronary artery bypass surgery, a persistent left hepatic vein was observed. Draining of this vein into the coronary sinus is an extremely rare embryological malformation and this is the first case to be reported as a solitary malformation in absence of other cardiac malformations.

Thoracoscopic Surgery for Partial Anomalous Pulmonary Venous Connection with Dual Drainage.

We report our technique for thoracoscopic surgery for a 15-year-old female (body weight, 59 kg) diagnosed with partial anomalous pulmonary venous connection with dual drainage. A large anomalous right lower pulmonary vein (RLPV) was drained into the inferior vena cava and left atrium, along with thoracoscopic ligation and clipping of RLPV and some anomalous hepatic veins.

Persistent primitive hepatic venous plexus with Scimitar syndrome: description of a case and review of the literature.

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature.

Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

Single ventricle and single atrium with anomalous hepatic venous drainage and azygos continuation of inferior vena cava in an adult patient.

In a 37-year-old patient, ultrasonography showed and CT confirmed the presence of single ventricle, single atrium, azygos continuation of inferior vena cava, anomalous hepatic vein drainage, and persistent left superior vena cava. Such a constellation of cardiovascular anomalies is exceptionally diagnosed in adulthood.

Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy.

OBJECTIVE: To propose an anatomical classification of congenital portosystemic shunts (CPSs) correlating with conservative surgery. BACKGROUND: CPSs entail a risk of life-threatening complications because of poor portal inflow, which may be prevented or cured by their closure. Current classifications based on portal origin of the shunt are not helpful for planning conservative surgery. METHODS: Twenty-three patients who underwent at least 1 surgical procedure to close the CPSs were included in this retrospective study (1997-2012). We designed a classification according to the ending of the shunt in the caval system. We analyzed the results and outcomes of surgery according to this classification. RESULTS: Two patients had an extrahepatic portosystemic shunt, 17 had a portacaval shunt [subdivided in 5 end-to-side-like portal-caval, 7 side-to-side-like portal-caval, and 5 H-shaped (H-type portal-caval)], 2 had portal-to-hepatic vein shunts (portohepatic), and 2 had a persistent ductus venosus. All extrahepatic portosystemic shunts, H-type portal-caval, portohepatic, and patent ductus venosus patients had a successful 1-stage ligation. All 5 end-to-side-like portal-caval patients had a threadlike intrahepatic portal venous system; a 2-stage complete closure was successfully achieved for 4 and a partial closure for 1. The first 2 side-to-side-like portal-caval patients had a successful 2-stage closure whereas the 5 others had a 1-stage longitudinal caval partition. All patients are alive and none needed a liver transplantation. CONCLUSIONS: Our classification correlates the anatomy of CPSs and the surgical strategy: outcomes are good provided end-to-side-like portal-caval shunts patients have a 2-stage closure, side-to-side portal-caval shunts patients have a 1-stage caval partition, and the others have a 1-stage ligation.

Selection of a right posterior sector graft for living donor liver transplantation.

Right posterior sector (RPS) grafts have been used to overcome graft size discrepancies, the major concern of living donor liver transplantation. Previous studies have reported the volumetry-based selection of RPS grafts without anatomical exclusion. We reviewed our data and established selection criteria for RPS grafts. The procurement of RPS grafts [conventional (n = 3) and extended (n = 5)] was performed for 8 of 429 recipients at our center. Extended RPS grafts contained the drainage area of the right hepatic vein. The mean graft weight (GW) according to 3-dimensional computed tomography volumetry was 488 g, and the GW/standard liver weight (SLW) ratio was 42.6%. The mean actual GW was 437 g, and the GW/SLW ratio was 38.4%. One donor exhibited standard bifurcation of the right portal vein (PV) and the left PV, and 2 donors exhibited trifurcation of the left PV, the right anterior portal vein (APV), and the posterior PV. The remaining 5 donors exhibited APV branching from the left PV, which is the most suitable anatomy for RPS grafts. Two recipients died of sepsis or small-for-size graft syndrome. One underwent retransplantation because of an intractable bile leak and fibrosing cholestatic hepatitis. Intractable bile duct (BD) stenosis developed in 4 of the 6 survivors. In conclusion, with the significant complications and potential concerns associated with RPS grafts, these grafts should be used very rarely and with extreme caution. Donors with the standard bifurcation of the PV and the posterior BD running through the dorsal side of the posterior PV are not suitable candidates for RPS grafts. Extended RPS graft procurement is recommended for easier parenchymal transection.