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一例以神经源性膀胱为表现的罕见脊髓空洞症病例。

An Unusual Case of Syringohydromyelia Presenting with Neurogenic Bladder.

作者信息

Geljic Antonella, Abdovic Slaven, Stampalija Fran, Loncar Lana, Tripalo Batos A, Cuk Martin

机构信息

Department of Paediatrics, Klinika za djecje bolesti Zagreb, Zagreb, Croatia.

Department of Pediatric Nephrology, Klinika za djecje bolesti Zagreb Klinika za pedijatriju, Zagreb, Croatia.

出版信息

European J Pediatr Surg Rep. 2019 Jan;7(1):e79-e82. doi: 10.1055/s-0039-1697925. Epub 2019 Nov 22.

Abstract

We report the case of a 4-year-old boy who first presented with acute pyelonephritis at the age of 6 months. Diagnostic workup revealed high-grade bilateral vesicourethral reflux (VUR). At the age of 18 months, a bulking agent was used to treat bilateral VUR. Since the VUR persisted, an open bilateral Lich-Gregoir procedure was done at the age of 3 years. Immediately after surgery, he developed acute urinary retention with hydronephrosis that resolved with the placement of dwelling urinary catheter. After removal of the catheter urinary retention relapsed so placement of suprapubic urinary catheter was indicated since he did not have sensory loss. He was started with tamsulosin (α - 1-blocker) and prophylactic antibiotics. Urodynamics were performed and suggested bladder outlet obstruction. On the basis of previous urethroscopy and the absence of neurological sequelae, the differential diagnosis of Hinman syndrome was made. After removal of the suprapubic catheter, clean intermittent catheterization was started and α-blocker continued. However, magnetic resonance imaging of the brain and the spinal cord revealed syringohydromyelia extending from thoracic spine (Th5) to conus medullaris with 6 to 7 mm in diameter. Electromyoneurogram was normal. After a follow-up of 3 years, the hydronephrosis has resolved. The patient is on clean intermittent catherization and has no urinary tract infections.

摘要

我们报告了一名4岁男孩的病例,他在6个月大时首次出现急性肾盂肾炎。诊断检查发现双侧重度膀胱输尿管反流(VUR)。18个月大时,使用填充剂治疗双侧VUR。由于VUR持续存在,在3岁时进行了开放性双侧利奇-格雷戈尔手术。术后立即出现急性尿潴留伴肾积水,留置导尿管后症状缓解。拔除导尿管后尿潴留复发,由于他没有感觉丧失,因此需要留置耻骨上导尿管。开始使用坦索罗辛(α-1阻滞剂)和预防性抗生素。进行了尿动力学检查,提示膀胱出口梗阻。基于之前的尿道镜检查和无神经后遗症,做出了欣曼综合征的鉴别诊断。拔除耻骨上导尿管后,开始进行清洁间歇性导尿,并继续使用α阻滞剂。然而,脑部和脊髓的磁共振成像显示脊髓空洞症从胸椎(Th5)延伸至脊髓圆锥,直径为6至7毫米。肌电图正常。经过3年的随访,肾积水已消退。患者进行清洁间歇性导尿,没有尿路感染。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/edf2/6874505/5b9254d8a1f4/10-1055-s-0039-1697925-i190441cr-1.jpg

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