ABSTRACT
RATIONALE: Total absence of superior vena cava (ASVC) is a very rare anomaly, and the patient usually suffers from superior vena cava syndrome (SVCS) or conduction disturbances. PATIENT CONCERNS: We report a 36-year-old white male, born and living in Brazil, without comorbidities presented to hematologist thrombotic episodes even under anticoagulant therapy. On his first hematologic appointment, he had no active complaints except by the fullness after meals, and his physical examination presented remarkable collateral circulation in the chest. DIAGNOSES: Congenital ASVC associated with factor V Leiden mutation. OUTCOMES: In his magnetic resonance angiography of the thorax, a great amount of collateral circulation and communication of the azygos and hemiazygos veins with inferior vena cava were evident, as well as the absence of the upper cava vein. Furthermore, heterozygous genetic mutation was found for Leiden factor V. LESSONS: This case gives us the lesson that we need to include ASVC in the differential diagnosis of SVCS. The importance of the V-Leiden factor as a joint risk with this congenital defect for venous thromboembolism episodes was also highlighted.
Subject(s)
Factor V/genetics , Mutation , Vascular Malformations/pathology , Vena Cava, Superior/abnormalities , Venous Thrombosis/diagnosis , Adult , Anticoagulants/therapeutic use , Azygos Vein/abnormalities , Azygos Vein/diagnostic imaging , Brazil , Collateral Circulation , Diagnosis, Differential , Fatal Outcome , Heterozygote , Humans , Magnetic Resonance Angiography/methods , Male , Superior Vena Cava Syndrome/diagnosis , Superior Vena Cava Syndrome/etiology , Thorax/blood supply , Thorax/diagnostic imaging , Thorax/pathology , Tomography, X-Ray Computed/methods , Vascular Malformations/complications , Vascular Malformations/diagnostic imaging , Vena Cava, Superior/pathology , Venous Thrombosis/etiologyABSTRACT
O atendimento odontológico a pacientes com doenças hemorrágicas confere grande responsabilidade ao profissional, para a identificaçäo do problema e adequada atuaçäo, numa atitude multidisciplinar com os demais membros da equipe de saúde. Este aspecto é bem enfatizado na prática da Odontopediatria, em que o bebê ou criança pode estabelecer um primeiro contato sem o diagnóstico prévio de qualquer distúrbio na hemostasia. Por esta razäo, as autoras efetuaram uma revisäo da literatura sobre as patologias mais frequentes observadas, subordinadas ao tema abordado, com base em levantamento piloto das crianças atendidas no setor de Odontologia da Fundaçäo Hemope
