Liver transplantation in the setting of a spontaneous shunt between superior mesenteric vein and right renal vein.

Shunts between the superior mesenteric vein (SMV) and the right renal vein (RRV) are very rare. Here, we describe and depict the rare case of a liver transplant (LT) in the setting of shunt between SMV and RRV. A 67-year-old white man presenting with Child C cirrhosis secondary to hemochromatosis and persistent encephalopathy was listed for LT. Preoperative abdominal angiotomography revealed the presence of a large spontaneous shunt between the SMV and the RRV. The patient underwent LT by receiving a liver from a 17-year-old brain-dead deceased donor victim of trauma. A large shunt between the SMV and the RRV was confirmed intraoperatively. Although there was no portal vein (PV) thrombosis, the PV was atrophic and had a reduced flow. PV pressure was 22mmHg (an arterial line was inserted inside the PV stump, and this line was connected to a common pressure transducer, the pressure readings was expressed in the anesthesia monitor). After shunt ligation PV pressure increased to 32mmHg. There were no post-transplant vascular complications, and the patient was discharged home in good health. Preoperative study of all LT candidates with angio CT scan is mandatory. Whenever there is PV thrombosis, an attempt to remove the entire thrombus is warranted. After thrombectomy or whenever there is not PV thrombosis, all large shunts should be ligated. PV pressure and flow should be measured before and after shunt ligation. In the absence of PV thrombosis, ligation of the shunt should enable an increase in PV flow and pressure, as reported herein.

Vascular alterations underlie developmental problems manifested in cloned cattle before or after birth.

Although assisted reproductive techniques are commonly applied in humans and animals, they are frequently associated with major developmental deficits and reduced viability. To explore abnormalities associated with cloning or nuclear transfer (NT) as the most invasive of these methods, we used a bovine model to characterize abnormalities. Detailed necropsy examinations were done on 13 calves that died soon after birth; in addition, we included data from embryos and fetuses (produced by NT) that terminated prematurely. Bovine clones that survived until the neonatal period differed quantitatively and qualitatively from in-vivo-derived cattle. Although alterations affected a variety of organs (e.g. heart, lung and liver), there was a clear association with abberant vascular developmental during the early intrauterine phase. Therefore, we concluded that vascular problems were key alterations induced by cloning (presumably via epigenetic modifications).

Study of the etiopathogenesis and differential diagnosis of oral vascular lesions by immunoexpression of GLUT-1 and HIF-1α.

BACKGROUND: To investigate whether the immunohistochemical expression of GLUT-1 and HIF-1α is related to the diagnosis and pathogenesis of oral vascular lesions. STUDY METHODS: Thirty cases each of pyogenic granuloma (PG) and hemangioma were studied. Antibodies against GLUT-1 and HIF-1α were detected by immunoperoxidase staining in 3-µm histological sections, and the results were analyzed qualitatively and quantitatively, respectively. Positive and negative cells were counted, and the mean number of positive cells was calculated for each case. RESULTS: The initial diagnosis of hemangioma was maintained in only 7 (23%) of the 30 cases studied, which were positive for GLUT-1. The remaining 23 cases were reclassified as vascular malformation (VM) (n = 13) and PG (n = 10) due to the absence of staining. The endothelium of blood vessels was negative for GLUT-1 in all cases initially diagnosed as PG (n = 30). The percentage of HIF-1α-positive cells was higher in cases of PG, followed by hemangiomas and VMs (P = 0.005). CONCLUSIONS: Histological features are not sufficient to establish the correct diagnosis of oral hemangiomas, and an accurate anamnesis is essential in these cases. In addition, these findings demonstrate that vascular lesions express mediators of angiogenesis, HIF-1α, and suggest that his process may play a role in the pathogenesis of vascular.

[Blue rubber bleb nevus syndrome: A case report]. / Síndrome de nevos azules ahulados: reporte de un caso.

Blue Rubber Bleb Nevus Syndrome (BRBS) is a rare disease, characterized by multiple vascular malformations in the skin and gastrointestinal tract. Other organs can also be affected, presenting different clinical manifestations such as arthralgia, epistaxis, hemoptysis, hematuria, hemothorax, mild thrombocytopenia, consumptive coagulopathy, and bone deformities, among others. We present a case of BRBS in a nine-year-old boy with the characteristic clinical manifestations of punctated purplish-blue skin lesions that vary in size and gastrointestinal vascular malformations with upper digestive tract bleeding.

Interrupted left aortic arch and isolated right subclavian artery from the right pulmonary artery.

We report a 10-day-old newborn, weighing 2.9 kg with an interrupted left aortic arch type B, a large subarterial ventricular septal defect and a right ductus connecting the right pulmonary artery to an isolated right subclavian artery. The patient underwent successful total surgical repair and the isolated right subclavian artery was ligated. He was discharged from hospital without complication and maintains excellent perfusion to the right arm via collaterals.

Achados de imagem e alternativas terapêuticas das malformações vasculares periféricas / Imaging findings and therapeutic alternatives for peripheral vascular malformations

As malformações vasculares periféricas compreendem um espectro de lesões que se tornam aparentes no decorrer da vida e podem ser encontradas em praticamente todo o corpo. São pouco comuns e frequentemente confundidas com o hemangioma infantil. Estas doenças são completamente distintas tanto em relação à história clínica como ao prognóstico e às formas de tratamento. Nestas lesões, a história evolutiva e as características do exame físico são de extrema importância para o adequado diagnóstico clinicorradiológico, que guiará a melhor alternativa terapêutica. As classificações mais recentes dividem as malformações vasculares periféricas levando em consideração o fluxo sanguíneo (alto e baixo) e os componentes vasculares envolvidos (arteriais, capilares, linfáticos e venosos). As malformações vasculares periféricas representam um desafio diagnóstico e terapêutico, e exames complementares como tomografia computadorizada, ultrassonografia com Doppler e ressonância magnética, em conjunto com a história clínica, podem trazer informações quanto às características de fluxo e à extensão das lesões. Arteriografia e flebografia confirmam o diagnóstico, avaliam a sua extensão e orientam a decisão terapêutica. Malformações de baixo fluxo geralmente são tratadas por abordagem percutânea e injeção de agente esclerosante, enquanto para as malformações de alto fluxo o acesso é endovascular com uso de agentes embolizantes permanentes líquidos ou sólidos.

Peripheral vascular malformations represent a spectrum of lesions that appear through the lifetime and can be found in the whole body. Such lesions are uncommon and are frequently confounded with infantile hemangioma, a common benign neoplastic lesion. In the presence of such lesions, the correlation between the clinical and radiological findings is extremely important to achieve a correct diagnosis, which will guide the best therapeutic approach. The most recent classifications for peripheral vascular malformations are based on the blood flow (low or high) and on the main vascular components (arterial, capillary, lymphatic or venous). Peripheral vascular malformations represent a diagnostic and therapeutic challenge, and complementary methods such as computed tomography, Doppler ultrasonography and magnetic resonance imaging, in association with clinical findings can provide information regarding blood flow characteristics and lesions extent. Arteriography and venography confirm the diagnosis, evaluate the lesions extent and guide the therapeutic decision making. Generally, low flow vascular malformations are percutaneously treated with sclerosing agents injection, while in high flow lesions the approach is endovascular, with permanent liquid or solid embolization agents.

Malformaciones Vasculares / Vascular malformation

Las malformaciones vasculares son anomalías presentes siempre desde el nacimiento que, al contrario de los hemangiomas, nunca desaparecen; pueden crecer durante toda la vida por hipertrofia. Según la clasificación de la ISSVA, las malformaciones vasculares se dividen en función del vaso afectado en capilares o venulares, venosas, linfáticas, arterio-venosas y combinadas o complejas. Cada una de ellas, con unas peculiaridades clínicas y hemodinámicas definitorias.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress; and may hypertrophy during lifetime. According to the ISSVA classification, vascular malformations are divided, depending on the affected vessel, into capillary or venular, venous, lymphatic, arteriovenous and combined or complex; each with certain defining clinical and haemodynamic peculiarities.