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).

Predicting the presence of an accessory hepatic vein using abdominal computed tomography / Predicción de la presencia de una vena hepática accesoria mediante tomografía computarizada abdominal

The incidence of detection of accessory hepatic vein (AHV) using MRI or CT has been reported. However, previous studies had a small sample size or only reported on the incidence of hepatic vein variants. To the best of our knowledge, there has been no previous report evaluating the factors predictive of the presence of an AHV. To evaluate the incidence and morphology of the accessory hepatic vein (AHV) using multidetector row computed tomography (MDCT) and to investigate the factors which may be helpful in predicting the presence of an AHV. We enrolled 360 patients who underwent abdominal MDCT. We investigated whether the AHV was present and evaluated the frequency of AHVs greater than 5 mm in diameter. We classified the morphology of the AHV entering the inferior vena cava (IVC). We also examined the factors that predicted the presence of an AHV by comparing the diameter of the middle hepatic vein (MHV) and the right hepatic vein (RHV). We identified an AHV in 164 of the 360 patients (45.6 %). Among the 164 AHVs, 56.7 % were larger than 5 mm in diameter. The most common morphologies of the inferior RHV were a single main trunk (58.5 %), followed by two main trunks with a V-shape (19.5 %) and two trunks entering the IVC separately (17.0 %). The possibility that an AHV will be present was significantly higher when the diameter of the RHV was smaller than that of the MHV. MDCT can provide important information regarding AHV incidence and morphology. The possibility of an AHV being present was significantly higher when the diameter of the RHV was smaller than that of the MHV.

Se ha informado de la incidencia de la detección de la vena hepática accesoria (VHA) mediante RM o TC. Sin embargo, estudios previos tenían un tamaño muestral pequeño o solo informaban sobre la incidencia de variantes de las venas hepáticas. Hasta donde sabemos, no ha habido ningún informe previo que evalúe los factores predictivos de la presencia de una VHA. El objetivo del estudio fue evaluar la incidencia y morfología de la vena hepática accesoria (VHA) mediante tomografía computarizada multidetector (TCMD) e investigar los factores que pueden ser útiles para predecir la presencia de un VHA. Se evaluaron 360 pacientes que se sometieron a TCMD abdominal. Se investigó si la VHA estaba presente y se evaluó la frecuencia de VHA mayores de 5 mm de diámetro. Se clasificó la morfología del VHA que drenaba en la vena cava inferior (VCI). Además, se examinaron los factores que predijeron la presencia de una VHA mediante la comparación del diámetro de la vena hepática media (VHM) y la vena hepática derecha (VHD). Se identificó un VHA en 164 de los 360 pacientes (45,6%). Entre las 164 VHA, el 56,7% tenía más de 5 mm de diámetro. Las morfologías más frecuentes del VHD inferior fueron un tronco principal único (58,5%), seguido por dos troncos principales con forma de V (19,5%) y dos troncos que drenaban en la VCI por separado (17,0%). La posibilidad de que una VHA esté presente fue significativamente mayor cuando el diámetro de la VHD era menor que la de la VHM. La MDCT puede proporcionar información importante sobre la incidencia de la VHA y su morfología. La posibilidad de que un VHA estuviera presente era significativamente mayor cuando el diámetro del VHD era menor que la VHM.

Decreased C-reactive protein induces abnormal vascular structure in a rat model of liver dysfunction induced by bile duct ligation

BACKGROUND/AIMS: Chronic liver disease leads to liver fibrosis, and although the liver does have a certain regenerative capacity, this disease is associated with dysfunction of the liver vessels. C-reactive protein (CRP) is produced in the liver and circulated from there for metabolism. CRP was recently shown to inhibit angiogenesis by inducing endothelial cell dysfunction. The objective of this study was to determine the effect of CRP levels on angiogenesis in a rat model of liver dysfunction induced by bile duct ligation (BDL). METHODS: The diameter of the hepatic vein was analyzed in rat liver tissues using hematoxylin and eosin (H&E) staining. The expression levels of angiogenic factors, albumin, and CRP were analyzed by real-time PCR and Western blotting. A tube formation assay was performed to confirm the effect of CRP on angiogenesis in human umbilical vein endothelial cells (HUVECs) treated with lithocholic acid (LCA) and siRNA-CRP. RESULTS: The diameter of the hepatic portal vein increased significantly with the progression of cirrhosis. The expression levels of angiogenic factors were increased in the cirrhotic liver. In contrast, the expression levels of albumin and CRP were significantly lower in the liver tissue obtained from the BDL rat model than in the normal liver. The CRP level was correlated with the expression of albumin in hepatocytes treated with LCA and siRNA-CRP. Tube formation was significantly decreased in HUVECs when they were treated with LCA or a combination of LCA and siRNA-CRP. CONCLUSION: CRP seems to be involved in the abnormal formation of vessels in hepatic disease, and so it could be a useful diagnostic marker for hepatic disease.

Intrahepatic Portosystemic Venous Shunt: Successful Embolization Using the Amplatzer Vascular Plug II

A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate.

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14- month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112μmol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Transcatheter coil occlusion of hepatic arteriovenous malformation in a neonate.

A 3-day-old neonate was diagnosed to have severe congestive heart failure due to a large shunt through a hepatic arteriovenous malformation. Percutaneous transcatheter delivery of two detachable coils resulted in complete abolition of the shunt. The patient showed dramatic clinical improvement and resolution of the heart failure, which was sustained on follow-up. This case represents a novel use of detachable occluding spring coils designed primarily for occluding patent ductus arteriosus.

Conexión anómala total de las venas pulmonares. Estudio clínico de 92 casos / Total anomalous pulmonary venous connection. Clinical study of 92 cases

Los autores revisaron 92 expedientes de 92 niños con conexión anómala total de venas pulmonares (CATVP). Los pacientes se diagnosticaron invariablemente con estudio hemodinámico. Se dividieron en cinco grupos: a)CATVP supracardiaca, a la vena cava superior, 43 casos. 2) Intracardiaca, 37 de los cuales 28 al seno coronario y nueve a la aurícula derecha. 3) Infracardiaca, a las venas suprahepáticas, cuatro casos. 4) Mixta, a la vena cava superior y al seno coronario, cuatro casos. 5) Malformaciones cardiacas complejas con CATVP acompañante: tres a la vena capa superior y una al seno coronario. Se discuten los datos clínicos, radiológicos y hemodinámicos y se señala que en un alto porcentaje de los casos al cuadro clínico y la silueta cardiaca permiten hacer el diagnóstico presuntivo sin estudios cruentos. La mortalidad quirúrgica general se relaciona con la gravedad del enfermo, lo que a su vez se expresa por insuficiencia cardiaca resistente a tratamiento, a edades tempranas, en pacientes que en esta serie tenían menos de cuatro meses de edad. La gravedad depende en gran medida, de la pequeñez de la comunicación interauricular invariablemente presente; si es pequeña o "restritiva", la situación es grave. Los pacientes que en esta serie alcanzaron nueve a 13 años de edad tuvieron grandes defectos auriculares y esto explica la supervivencia. Si bién es deseable la cirugía temprana para corrección completa, tiene alto riesgo pero no tanto como el tratamiento conservador, el cual puede causar muertes repentinas. Siempre debe intentarse septostomía con globo en el momento de lograr el diagnóstico con cateterismo. Esta septostomía sin duda es benéfica y muchas veces permite que se realice la cirugía correctora tiempo después, sin embargo, el beneficio de la septostomía a menudo es temporal.El tratamiento médico de la insuficiencia cardiaca siempre se emplea pero también es una medida paliativa temporal que debe ir seguida de cirugía correctora