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伴有先天性异常的罕见病例——左肾异位伴盆腔位及胸降主动脉缩窄。

Rare case of associated congenital anomalies--ectopic left kidney with pelvic presentation and descending thoracic aorta coarctation.

作者信息

Slobodan Tanaskovic, Goran Vucurevic, Slobodan Micovic, Dario Jocic, Srdjan Babic, Vladimir Kovacevic, Djordje Radak

机构信息

Vascular Surgery Clinic, "Dedinje" Cardiovascular Institute, Belgrade, Serbia.

Vascular Surgery Clinic, "Dedinje" Cardiovascular Institute, Belgrade, Serbia.

出版信息

Ann Vasc Surg. 2014 Feb;28(2):493.e15-9. doi: 10.1016/j.avsg.2013.04.027. Epub 2013 Dec 19.

Abstract

We present the case of a patient with extremely rare associated congenital anomalies: thoracic aorta coarctation and left ectopic kidney with pelvic presentation. A 45-year-old male patient was admitted to our institution for multidetector computed tomography (MDCT) angiography. On admission, he complained of intermittent claudication after 10 meters of walking, frequent chest pain, and hypertension crisis. Femoral pulse was absent in the left leg. At 14 years of age, he underwent insertion of a 10-mm Dacron tubular graft at the descending aorta level for isthmic coarctation. MDCT arteriography revealed significant stenosis (>90%) at the proximal anastomosis site of a previous graft in the form of a floating thrombus (pseudocoarctation‒re-coarctation) as well as an ectopic pelvic left kidney with the left renal and accessory renal artery arising from the left common iliac artery. One year ago we attempted percutaneous angioplasty of the aforementioned in-graft stenosis. At the time, the procedure was partly successful and the patient had been doing well, but all of the aforementioned symptoms reappeared and CT angiography showed no signs of improvement. Therefore, ascending-to-descending aortic bypass was done with an 18-mm Dacron tubular graft with preserved postoperative renal function and palpable peripheral pulses. MDCT angiography showed normal postoperative findings and the patient was doing well. In the case presented, MDCT angiography played a significant role in this rare aortic anomaly detection with incidental discovery of a rare developmental kidney complication.

摘要

我们报告一例伴有极其罕见先天性异常的患者

胸主动脉缩窄和左侧异位肾伴盆腔异位。一名45岁男性患者因多排螺旋计算机断层扫描(MDCT)血管造影入住我院。入院时,他诉说行走10米后出现间歇性跛行、频繁胸痛和高血压危象。左腿股动脉搏动消失。14岁时,他因峡部缩窄在降主动脉水平植入了一个10毫米的涤纶人工血管。MDCT血管造影显示,先前人工血管近端吻合部位存在严重狭窄(>90%),表现为漂浮血栓(假性缩窄-再缩窄),以及左侧盆腔异位肾,左肾及副肾动脉发自左髂总动脉。一年前,我们尝试对上述人工血管内狭窄进行经皮血管成形术。当时,该手术部分成功,患者情况良好,但上述所有症状再次出现,CT血管造影显示无改善迹象。因此,采用18毫米涤纶人工血管进行升主动脉至降主动脉旁路移植术,术后肾功能保留,外周脉搏可触及。MDCT血管造影显示术后结果正常,患者情况良好。在本病例中,MDCT血管造影在这种罕见的主动脉异常检测中发挥了重要作用,同时偶然发现了一种罕见的肾脏发育并发症。

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