Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage.

A 53-year-old woman was diagnosed with liver dysfunction in August 20XX. Computed tomography (CT) revealed multiple hepatic AV shunts, and she was placed under observation. In March 20XX + 3, she developed back pain, and CT performed during an emergency hospital visit showed evidence of intrahepatic bile duct dilatation. She was referred to our gastroenterology department in May 20XX + 3. We conducted investigations on suspicion of hereditary hemorrhagic telangiectasia (HHT) with hepatic AV shunting based on contrast-enhanced CT performed at another hospital. HHT is generally discovered due to epistaxis, but there are also cases where it is diagnosed during examination of liver damage.

Fetal intrahepatic Umbilical-Porto-Systemic venous shunts (IHUPSVS): In-utero anatomic classification.

OBJECTIVE: Congenital intrahepatic shunts divert highly oxygen and nutrients rich placental blood flow from the liver into the systemic flow having a negative influence on normal fetal growth and postnatal development. The ability to recognize this anomaly helps assess the possible clinical impact, counseling, and management of pregnancy. The present study aimed to propose in utero classification for the Intrahepatic Umbilical-Porto-Systemic Venous Shunt (IHUPSVS) based on our experience. STUDY DESIGN: A prospective study. Grayscale ultrasound with two and three-dimensional high-definition Doppler modalities was used. IHUPSVS was defined as a diversion of blood from the liver tissue by abnormal communication between a branch of the intrahepatic Umbilical vein or the Portal veins with the systemic circulation (the Hepatic veins or the Sub-Diaphragmatic Vestibulum). RESULTS: Twenty-five fetuses were diagnosed with IHUPSVS. We identified three main anatomic types: I) Porto-hepatic shunt which was divided into Ia) regular single shunt (15/25, 60%) and Ib) regular multiple shunts, (6/25, 24%) II) Umbilical-Porto-Hepatic shunt divided into a) Umbilical or b) Umbilical combined with Portal hepatic shunt (2/25, 8%) and III) Cavernous- aneurysmatic shunt (2/25, 8%). All the shunts were verified by postnatal targeted sonography. CONCLUSIONS: This study creates the anatomic basis for common nomenclature and future probable updating for this anomaly.

A Case of High-Output Heart Failure.

CASE PRESENTATION: A 55-year-old woman with a medical history of hereditary hemorrhagic telangiectasia (HHT) complicated by recurrent nosebleeds, severe blood loss anemia, hepatic arterial-venous malformation (AVM), pulmonary hypertension, and severe tricuspid regurgitation presented to the HHT specialty clinic with acute hypoxic respiratory failure (new 3-L O2 requirement), weight gain, and volume overload. She was directly admitted to the pulmonary hypertension unit of our hospital. She had two recent admissions for similar symptoms thought to be due to worsening pulmonary arterial hypertension. In prior admissions, she had undergone right heart catheterization demonstrating mild pulmonary hypertension (pulmonary arterial pressure, 29 mm Hg, cardiac output by Fick 5.76, and cardiac index 3.22, mildly elevated pulmonary vascular resistance to 5.5 woods units). She would undergo diuresis with symptomatic improvement; however, after discharge she would rapidly develop recurrent heart failure symptoms. She reported compliance with guideline-directed medications, diuretics, and dietary restrictions and was still suffering severe symptoms. Notably she had previously elevated liver enzymes concerning for cirrhosis and had begun a workup to evaluate for causes of cirrhosis; she had a history of mild alcohol use, negative hepatitis viral serology, and no known history of liver disease.

Situs ambiguous and hepatic vein anomaly: a case report / Situs ambiguous y anomalía de la vena hepática: informe de un caso

SUMMARY: Situs ambiguous is the placement of vessels and organs in the thoracoabdominal space that are anatomically located outside its normal position in a certain order. This condition is a broad definition that includes many variations. In this case report, we reported a patient with Situs ambiguus with an abnormal hepatic vein who was diagnosed incidentally during medical imaging (computed tomography, sonography and MRI study).

RESUMEN: El Situs ambiguous es la colocación de vasos y órganos en el espacio toracoabdominal que anatómicamente se encuentran fuera de su posición normal en un cierto orden. Esta condición es una definición amplia que incluye muchas variaciones. En este reporte de caso, reportamos un paciente con Situs ambiguous con una vena hepática anormal que fue diagnosticado incidentalmente durante un estudio de imagen médica (tomografía computarizada, ecografía y resonancia magnética).

Congenital Hepatic Arteriovenous Malformation Presenting as Isolated Massive Hepatomegaly in an Otherwise Healthy Neonate: A Case Report.

Congenital hepatic arteriovenous malformations (HAVMs), though rare, carry high morbidity and mortality rates if left undiagnosed. The usual clinical presentation is in infancy with congestive heart failure, anaemia and hepatomegaly. There are reports of presentation as persistent pulmonary hypertension in newborns and reports of their spontaneous regression as well. We describe a healthy full-term neonate with HAVM who was presented with isolated massive hepatomegaly and underwent surgical ligation.

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.

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.

Clinical management of type C hepatic encephalopathy.

Type-C hepatic encephalopathy is a complex neurological syndrome, characteristic of patients with liver disease, causing a wide and complex spectrum of nonspecific neurological and psychiatric manifestations, ranging from a subclinical entity, minimal hepatic encephalopathy, to a deep form in which a complete alteration of consciousness can be observed: overt hepatic encephalopathy. Overt hepatic encephalopathy occurs in 30-40% of patients. According to the time course, hepatic encephalopathy is subdivided into episodic, recurrent and persistent. Diagnostic strategies range from simple clinical scales to more complex psychometric and neurophysiological tools. Therapeutic options may vary between episodic hepatic encephalopathy, in which it is important to define and treat the precipitating factor and hepatic encephalopathy and secondary prophylaxis, where the standard of care is non-absorbable disaccharides and rifaximin. Grey areas and future needs remain the therapeutic approach to minimal hepatic encephalopathy and issues in the design of therapeutic studies for hepatic encephalopathy.

Intra-hepatic arterio-venous fistula after penetrating liver injury.

Arterio-portal fistula occurs relatively frequently after liver trauma; its treatment is well codified. We present here in the case of a patient with an intraparenchymal fistula between a hepatic artery branch and the right hepatic vein. Embolization via interventional radiology was considered at high risk. However, hepatic necrosis developed, leading to the performance of a right lobectomy. The literature on this type of arterio-venous fistula is scanty making it difficult to propose specific management recommendations.

Endovascular Transjugular Occlusion of Congenital Intrahepatic Portosystemic Venous Shunt Using Simultaneous Fluoroscopy and Transabdominal Ultrasound Guidance: Report of 2 Cases.

Congenital intrahepatic portosystemic venous shunts (CIPVS) are rare anomalies that can be detected before birth or in early infancy or later in life. Symptomatic shunts are treated as they carry high risk of complications like hepatic encephalopathy. Various treatment options include surgery, endovascular embolization, and percutaneous closure devices. We treated 2 infants with CIPVS successfully by endovascular embolization of the shunt using vascular plug through transjugular route. Transabdominal ultrasound guidance in addition to fluoroscopy was used at the time of vascular plug placement. We emphasize that the use of transabdominal ultrasound during endovascular occlusion enhances the safety and technical success rate.

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.

Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava.

BACKGROUND: Patients with heterotaxy, single ventricle and interrupted inferior vena cava are at risk of developing significant pulmonary arteriovenous malformations and cyanosis, and inequitable distribution of hepatic factor has been implicated in their development. We describe our experience with a technique for hepatic vein incorporation that reliably provides resolution of cyanosis and presumably equitable hepatic factor distribution. METHODS: A retrospective review of a single-surgeon experience was conducted for patients who underwent this modified Fontan operation utilizing an extracardiac conduit from the hepatic veins to the dominant superior cavopulmonary connection. Preoperative characteristics and imaging, operative details, and postoperative course and imaging were abstracted. RESULTS: Median age at operation was 5 years (2-10 years) and median weight was 19.6 kg (11.8-23 kg). Sixty percent (3/5) of patients had Fontan completion without cardiopulmonary bypass, and follow-up was complete at a median of 14 months (range 1-20 months). Systemic saturations increased significantly from 81% ± 1.9% preoperatively to 95% ± 3.5% postoperatively, P = .0008. Median length of stay was 10 days (range: 7-14 days). No deaths occurred. One patient required reoperation for bleeding and one was readmitted for pleural effusion. Postoperative imaging suggested distribution of hepatic factor to all lung segments with improved pulmonary arteriovenous malformation burden. CONCLUSIONS: Hepatic vein incorporation for patients with heterotaxy and interrupted inferior vena cava should optimally provide equitable pulmonary distribution of hepatic factor with resolution of cyanosis. The described technique is performed through a conventional approach, is facile, and improves cyanosis in these complex patients.

Impact of the Inferior Right Hepatic Veins on Right Liver Lobe Regeneration in Living-Donor Liver Transplant: 3-Dimensional Computed Tomography Scan Analyses in Donors and Recipients.

OBJECTIVES: The presence of an inferior right hepatic vein significantly modifies the outflow pattern of the right liver and may have an impact on graft regeneration. MATERIALS AND METHODS: Study patients were donors and recipients with 3-dimensional computed tomography scan simulation. We studied the physiologic impact of the inferior right hepatic veins on right liver regeneration in donors of left lobe grafts (step1; n = 60 patients) and the efficacy of a inferior right hepatic vein reconstruction policy based on a vessel caliber > 5 mm criteria in recipients of right liver grafts (step 2; n = 38 patients). RESULTS: In step 1, the regeneration rate at 1 month was comparable between future liver remnants with inferior right hepatic vein (regeneration rate of 28.3%) and without inferior right hepatic vein (regeneration rate of 30.2%). In step 2, at 3 months after living-donor liver transplant, we observed no significant differences in regeneration rates among grafts anatomically without an inferior right hepatic vein (regeneration rate of 60.7%), grafts with an inferior right hepatic vein but no reconstruction (regeneration rate of 64.9%), and grafts with an inferior right hepatic vein and its reconstruction (regeneration rate of 68.1%). Within the latter subgroup, grafts with a dominant inferior right hepatic vein showed a greater regeneration of the anterior sector with inversion of the anterior-to-posterior sector regeneration ratio compared with right hepatic vein-dominant grafts (inferior right hepatic vein-dominant vs right hepatic vein-dominant showed anterior sector regeneration rates of 85.7% vs 52.1%; P = .02; anterior/posterior sector regeneration ratio was 2.3 vs 0.6, respectively; P = .04). CONCLUSIONS: The reconstruction policy for inferior right hepatic vein with caliber > 5 mm is effective but should be implemented with computed tomography scan simulation. Right lobe grafts with a dominant inferior right hepatic vein are at higher risk of impaired regeneration due to expected large volumes of hepatic venous congestion if the middle hepatic veins tributaries or the inferior right hepatic veins are not reconstructed and an increased rate of right hepatic vein complications.

Medical management of haemorrhagic hereditary telangiectasia in adult patients. / Tratamiento de la telangiectasia hemorrágica hereditaria en el paciente adulto.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited Rare Disease that causes a systemic anomalous vascular overgrowth. The approach and follow-up of these patients should be from multidisciplinary units. Its diagnosis is carried out according to Curaçao clinical Criteria. Telangiectasia in the nasal mucosa cause recurrent epistaxis, the main symptom of HHT and difficult to control. The three types of hepatic shunting, hepatic artery to hepatic vein, hepatic artery to portal vein or to portal vein to hepatic vein, can cause high-output heart failure, portal hypertension or porto-systemic encephalopathy, respectively. These types of vascular involvement can be established using computerised tomography. Pulmonary arteriovenous fistula should be screened for all HHT patients by contrast echocardiography. The main objective is to review the management of epistaxis, liver and lung involvement of the adult patient with HHT.

Additional superior vena cava combined with abnormal inflow of the hepatic vein.

We represent a case of successful surgical treatment of a rare congenital heart disease: abnormal inflow of an additional superior vena cava into the left atrium, combined with atrial septal defect, mitral and tricuspid valve regurgitation, and abnormal inflow of the left hepatic vein into a roofless coronary sinus.