Grebeldinger Slobodan P, Balj Svetlana S, Adic Oto
Institute for Children and Youth Health Care of Vojvodina, Clinic of Pediatric Surgery.
Vascular. 2011 Jun;19(3):170-4. doi: 10.1258/vasc.2010.cr0232.
Hypoplasia of the thoracic and abdominal aorta is an extremely rare vascular pathology. The most common clinical manifestation is severe uncontrolled hypertension in adolescents and young adults. Medical treatment alone can decrease blood pressure, but often very high doses of antihypertensive drugs are needed. When hypertension is refractory to the antihypertensive medications, surgical revascularization is considered as the treatment of choice. We report the case of a severe and diffuse hypoplasia of the aorta, beginning with the aortic isthmus, to the aortic bifurcation, associated with an aberrant celiac trunk and superior mesenteric artery, and with other multiple vascular abnormalities. Unlikely, the only manifestation of this extensive vascular malformation was medicamentously controllable hypertension. To our knowledge, this severe vascular anomaly, with such a minimal clinical manifestation, has not been previously described in the English literature.
胸主动脉和腹主动脉发育不全是一种极其罕见的血管病变。最常见的临床表现是青少年和青年中严重的难治性高血压。仅药物治疗可降低血压，但往往需要非常高剂量的抗高血压药物。当高血压对抗高血压药物难治时，手术血管重建被视为首选治疗方法。我们报告一例严重弥漫性主动脉发育不全病例，病变始于主动脉峡部，延伸至主动脉分叉，伴有腹腔干和肠系膜上动脉异常以及其他多种血管异常。不太常见的是，这种广泛血管畸形的唯一表现是药物可控的高血压。据我们所知，这种具有如此轻微临床表现的严重血管异常在英文文献中此前尚未有描述。
