[Hypoplasias of the thoracic and abdominal aorta: presentation of two cases, with evaluation 11 and 20 years after surgical management].
Two cases of hypoplasia of the thoracic and abdominal aorta are reported, diagnosed in two individuals, a girl 13 years old and a boy aged 16 years. The malformation involved the upper abdominal aorta and the visceral arteries in the first case, and simply the descending thoracic aorta in the remainder. Arterial hypertension was the principal manifestation who lead to the diagnosis, which was confirmed through the conventional angiography. Both patients underwent surgical management, which consisted in the aortic revascularization associated to complete visceral revascularization in the first case, and in the single aortic revascularization in the second patient. Surgical therapy course was uneventful in both cases and blood pressure returned to normal values following the operation. Reviewed 11 and 20 years after the procedure, they were found in good condition, with normal blood pressure without any medication; angio CT studies disclosed the prosthetic grafts working in excellent condition. The main features of etiopathogeny, clinical presentation, diagnosis and surgical management are subjected to a discussion, based on an extensive review of the literature dedicated to this clinical entity.
da Gama
A Dinis
AD
Clinica Universitária de Cirurgia Vascular, Hospital de Santa Maria, Lisboa.
D000293:Adolescent; D001012:Aorta, Abdominal / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011859:Radiography; D013997:Time Factors