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在一名成年1型神经纤维瘤病患者中发现的肌源性膀胱功能障碍和输尿管梗阻:病例报告

Myogenic bladder dysfunction and ureteral obstruction discovered in an adult patient with neurofibromatosis type 1: A case report.

作者信息

Evans Daniel D, Fuchs Molly, Baradaran Nima

机构信息

Department of Urology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.

Department of Pediatric Urology, Nationwide Childrens Hospital, Columbus, OH, USA.

出版信息

Urol Case Rep. 2023 Mar 3;47:102367. doi: 10.1016/j.eucr.2023.102367. eCollection 2023 Mar.

Abstract

We present a case of a 44-year-old male with cutaneous manifestations of neurofibromatosis type 1 presenting with long-standing urologic symptoms of uncertain etiology including urinary retention from myogenic bladder failure, chronic kidney disease with evidence of bilateral ureteral obstruction and presenting signs of an obstructing left ureterocele. This patient had a complete urologic evaluation and underwent ileocecocystoplasty with a continent catheterizable channel and bilateral ureteral reimplantation. Surgical excision of a left ureteral mound of tissue demonstrated the presence of a neurofibroma involving the bladder that led to obstruction. To our knowledge, this is the first report of such a presentation.

摘要

我们报告一例44岁男性,患有1型神经纤维瘤病的皮肤表现,并伴有病因不明的长期泌尿系统症状,包括因肌源性膀胱功能衰竭导致的尿潴留、有双侧输尿管梗阻证据的慢性肾病以及左侧输尿管囊肿梗阻的表现体征。该患者接受了全面的泌尿系统评估,并接受了回盲部膀胱扩大术,构建可控性储尿囊通道及双侧输尿管再植术。手术切除左侧输尿管肿物组织显示存在累及膀胱的神经纤维瘤,导致梗阻。据我们所知,这是此类病例的首例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9654/10015184/cd97c18b2bb4/gr1.jpg

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