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与血管平滑肌脂肪瘤相关的温德利希综合征。

Wunderlich Syndrome Associated With Angiomyolipomas.

作者信息

Ramirez-Limon David Antonio, Gonzaga-Carlos Nezahualcoyotl, Angulo-Lozano Juan Carlos, Miranda-Symes Olivia, Virgen-Gutierrez Francisco

机构信息

Department of Urology, Hospital General de Xalapa, Xalapa, MEX.

Department of Urology, Hospital General de Mexico, Mexico City, MEX.

出版信息

Cureus. 2022 Apr 5;14(4):e23861. doi: 10.7759/cureus.23861. eCollection 2022 Apr.

DOI:10.7759/cureus.23861
PMID:35530872
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9072293/
Abstract

Wünderlich syndrome (WS) is a spontaneous retroperitoneal hemorrhage confined to the subcapsular or perinephric space without a history of trauma. Since it is a rare condition with a significant mortality rate if not treated timely, it is essential to identify its risk factors and early clinical manifestations for a favorable outcome. Various conditions are associated, but the most common causes are benign and malignant renal neoplasms. We present a 26-year-old female with a history of tonic-clonic seizures who presented to the ED with intense abdominal pain located on the right flank with a palpable mass. Management included IV fluids and blood transfusion. She underwent a right total nephrectomy. She was later diagnosed with tuberous sclerosis. A 44-year-old female with a three-year history of right costovertebral pain and recurrent urinary tract infections that presented to the ED with acute right flank pain was diagnosed with WS secondary to an angiomyolipoma and underwent right total nephrectomy.WS is a very rare pathology that represents a diagnostic challenge for the physician. The treatment will depend on the hemodynamic condition of the patient. Active follow-up should be reserved for those who have small tumors, are asymptomatic, and have hemodynamic stability. Surgical or radiology intervention is reserved for those who are hemodynamically unstable or who have a suspicion of renal cell carcinoma.

摘要

温德利希综合征(WS)是一种自发性腹膜后出血,局限于肾包膜下或肾周间隙,无外伤史。由于它是一种罕见疾病,若不及时治疗死亡率很高,因此识别其危险因素和早期临床表现对于取得良好预后至关重要。多种情况与之相关,但最常见的病因是良性和恶性肾肿瘤。我们报告一名26岁女性,有强直阵挛性癫痫病史,因右下腹剧痛伴可触及肿块就诊于急诊科。治疗包括静脉补液和输血。她接受了右侧全肾切除术。后来她被诊断为结节性硬化症。一名44岁女性,有三年右肋脊角疼痛和反复尿路感染病史,因急性右下腹疼痛就诊于急诊科,被诊断为血管平滑肌脂肪瘤继发的WS,并接受了右侧全肾切除术。WS是一种非常罕见的病理情况,对医生来说是一个诊断挑战。治疗将取决于患者的血流动力学状况。对于肿瘤小、无症状且血流动力学稳定的患者,应进行积极随访。对于血流动力学不稳定或怀疑有肾细胞癌的患者,则应进行手术或放射学干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/08988c599227/cureus-0014-00000023861-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/a57afa971d30/cureus-0014-00000023861-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/dffacd85ac71/cureus-0014-00000023861-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/887fe1854b95/cureus-0014-00000023861-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/2b5539607aa4/cureus-0014-00000023861-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/38a411dfc166/cureus-0014-00000023861-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/f1b96aeff0c6/cureus-0014-00000023861-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/08988c599227/cureus-0014-00000023861-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/a57afa971d30/cureus-0014-00000023861-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/dffacd85ac71/cureus-0014-00000023861-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/887fe1854b95/cureus-0014-00000023861-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/2b5539607aa4/cureus-0014-00000023861-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/38a411dfc166/cureus-0014-00000023861-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/f1b96aeff0c6/cureus-0014-00000023861-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/061e/9072293/08988c599227/cureus-0014-00000023861-i07.jpg

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本文引用的文献

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The Herlyn-Werner-Wunderlich (HWW) syndrome - A case report with radiological review.赫林-韦纳-温德利希(HWW)综合征——一例病例报告及影像学回顾
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