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合并左肾静脉重复畸形的混合型胡桃夹综合征:一名18岁男孩的严重罕见病例表现

Mixed nutcracker syndrome with left renal vein duplication: A severe and exceptional presentation in an 18-year-old boy.

作者信息

Mallat Faouzi, Hmida Wissem, Othmen Mouna Ben, Mosbah Faouzi

机构信息

Department of Urology, Hospital of Sahloul, Sousse, Tunisia.

出版信息

Urol Ann. 2015 Apr-Jun;7(2):244-7. doi: 10.4103/0974-7796.150494.

Abstract

The nutcracker syndrome (NCS) is rare and often misdiagnosed because it embraces an extended non-pathognomonic spectrum of symptoms that imply a difficult diagnosis. Ultimately it may be associated with substantial morbidity and even life-threatening events. Mixed NCS with renal vein duplication is an exceptional variety, have previously been reported to the best of our knowledge. We report a rare case of an 18-year-old boy who presented with a long history of abdominal, pelvic and left flank pain, fatigue and higher bilateral varicocele. Computed tomographic angiography, Doppler ultrasonography and venography were performed revealed left renal vein duplication with dilated retroaortic and preaortic branchs, entrapped respectively between the aorta and the vertebral column and in the aortico-mesenteric space, with extensive and complex varices of the deep pelvic venous plexus; promoting the mixed renal NCS. Auto transplantation of the left kidney was suggested, but refused by the patient; and only the varicocele was managed. The patient is still suffering from his severe initial symptoms. Diagnosis is difficult and should be considered in patients with inexplicable flank or abdominal pain. Our purpose is to raise clinician's awareness for this condition so that they will be more likely to diagnose it. This will facilitate prompt diagnosis and treatment.

摘要

胡桃夹综合征(NCS)较为罕见,且常被误诊,因为其症状范围广泛且缺乏特异性,这意味着诊断困难。最终,它可能会导致严重的发病情况,甚至危及生命。据我们所知,伴有肾静脉重复的混合型NCS是一种特殊类型,此前已有相关报道。我们报告了一例罕见的18岁男孩病例,该男孩有长期的腹部、盆腔和左侧胁腹疼痛、疲劳以及双侧高位精索静脉曲张病史。计算机断层血管造影、多普勒超声检查和静脉造影显示左肾静脉重复,伴有扩张的主动脉后支和主动脉前支,分别被困于主动脉和脊柱之间以及主动脉肠系膜间隙,同时伴有盆腔深静脉丛广泛而复杂的静脉曲张;提示为混合型肾NCS。建议对左肾进行自体移植,但患者拒绝;仅对精索静脉曲张进行了处理。患者仍饱受最初的严重症状困扰。诊断困难,对于有无法解释的胁腹或腹痛的患者应考虑此病。我们的目的是提高临床医生对这种疾病的认识,以便他们更有可能做出诊断。这将有助于及时诊断和治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/467c/4374268/eef0f84f1581/UA-7-244-g001.jpg

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