Pancreatic arteriovenous malformation (PAVM) is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.
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http://dx.doi.org/10.1155/2011/612657 | DOI Listing |
Rev Esp Enferm Dig
January 2025
Gastroenterology and Hepatology, Hospital Universitario Virgen de las Nieves, España.
Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic disease characterised by mucocutaneous telangiectasias and arteriovenous malformations that can affect multiple organs. Although rare, ischaemic cholangiopathy can occur, a serious complication that can even lead to death. We present the case of a patient with HHT disease with previous mucocutaneous and gastrointestinal manifestations in whom 8 weeks after cholecystectomy a saccular dilatation of the intrahepatic bile duct was observed.
View Article and Find Full Text PDFJ Cardiol Cases
October 2024
Pediatric Cardiology Unit, University of Campania "Luigi Vanvitelli", AORN "Ospedali dei Colli", Monaldi Hospital, Naples, Italy.
Unlabelled: Right-to-left shunt may be a cause of paradoxical embolization (and cryptogenic cerebral ischemic events). Patent foramen ovale and pulmonary arterio-venous malformations are the most common cause of paradoxical embolization; however, some rare cardiovascular lesions may cause a right-to-left shunt with consequent paradoxical embolization. We describe a case of a young child with superior sinus venosus atrial septal defect who underwent surgical closure and developed a cryptogenic cerebral ischemic attack due to a rare cause of paradoxical embolization, during a long-term follow-up.
View Article and Find Full Text PDFHereditary hemorrhagic telangiectasia is an autosomal dominant disorder caused by mutations in the bone morphogenetic protein signaling pathway, leading to arteriovenous malformations. While previously thought to share molecular and cellular dysregulation, this study reveals highly distinct mechanisms depending on whether mutations occur in Alk1 or SMAD4. Loss of SMAD4 enhances endothelial cell responses to flow, including flow-regulated transcription and cell migration against blood flow, causing excessive pruning of capillaries and the formation of single large shunts.
View Article and Find Full Text PDFInt J Surg Case Rep
January 2025
Department of Gynecology-Obstetrics, Mohammed V Military University Hospital, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.
Introduction And Importance: Uterine arteriovenous malformations (UAVMs) are rare vascular anomalies caused by abnormal connections between uterine arteries and veins. They can lead to severe bleeding, requiring prompt and accurate diagnosis. Historically treated with hysterectomy, transcatheter vascular embolization has emerged as a fertility-sparing alternative.
View Article and Find Full Text PDFActa Neurol Belg
January 2025
Intensive Care Department, Cliniques Universitaire Saint-Luc (CUSL), Université Catholique de Louvain (UCL), Brussels, Belgium.
Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare vascular disorder characterized by arteriovenous malformations (AVMs) in various organs, including the lungs. Pulmonary AVMs (PAVMs) are especially worrisome due to their potential to form right-to-left shunts, resulting in life-threatening complications such as paradoxical embolism and stroke . We present a case of fatal air embolism in a young patient with a known history of HHT and recurring hemoptysis.
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