Department of Nephrology, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK.
Am J Kidney Dis. 2010 Dec;56(6):1197-201. doi: 10.1053/j.ajkd.2010.04.023. Epub 2010 Jul 29.
We describe the case of a 23-year-old white woman with a long history of hypertension. She was referred to us 7 years after her initial diagnosis of hypertension when her blood pressure control worsened during pregnancy. Clinical examination showed an abdominal bruit and weak femoral pulses. Imaging showed midaortic syndrome with bilateral renal artery stenosis as the cause of her hypertension, and further investigations showed neurofibromatosis type 1 as the underlying disorder. Midaortic syndrome, a rare disorder of the abdominal aorta that is different from classic coarctation, typically is associated with neurofibromatosis. Renal artery stenosis is common, as are weak femoral pulses and impaired development of the lower limbs. Because of the rarity of this syndrome, only anectodal evidence exists with regard to treatment. Surgery and interventional treatment with stent placement in the abdominal aorta have been reported, as well as good outcomes with long-term medical management. Our patient continues to be healthy without intervention, with reasonable blood pressure control and normal kidney function on a 4-drug antihypertensive regimen. We discuss midaortic syndrome with a focus on diagnosis, differential diagnosis, associated conditions, and management. Nephrologists, radiologists, and ultrasonographers should be aware of this rare cause of renovascular hypertension.
我们描述了一位 23 岁的白人女性的病例，她患有长期高血压病史。她在最初被诊断为高血压 7 年后因怀孕期间血压控制恶化而被转介给我们。临床检查显示腹部杂音和股动脉搏动减弱。影像学检查显示中主动脉综合征，双侧肾动脉狭窄是导致她高血压的原因，进一步的检查显示她患有 1 型神经纤维瘤病，这是导致她高血压的潜在疾病。中主动脉综合征是一种罕见的腹主动脉疾病，与经典缩窄不同，通常与神经纤维瘤病有关。肾动脉狭窄很常见，股动脉搏动减弱和下肢发育不良也很常见。由于这种综合征非常罕见，因此仅存在关于治疗的偶发证据。已经报道了手术和介入治疗用支架置入腹主动脉，以及长期药物治疗的良好结果。我们的患者继续保持健康，无需干预，在 4 种降压药物治疗方案下血压控制合理，肾功能正常。我们讨论了中主动脉综合征，重点是诊断、鉴别诊断、相关疾病和治疗。肾脏病学家、放射科医生和超声科医生应该意识到这是一种罕见的肾血管性高血压的原因。
