自发性血管肌脂肪瘤破裂:一例失血性休克和紧急栓塞手术

Q4 Medicine
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引用次数: 0

摘要

本病例报告讨论了一名 64 岁男性结节性硬化症复合体(TSC)患者的病例,TSC 是一种遗传性疾病,其特征是在多个器官系统中形成良性肿瘤。该患者因右肾血管脂肪瘤自发性出血而就诊,这是TSC的常见表现,尤其是在TSC2基因突变的患者中,有可能危及生命。患者患有高血压和血脂异常等严重并发症,使其临床病程更加复杂。最初的治疗包括积极的液体复苏和血制品输注,随后进行紧急栓塞以控制活动性出血。尽管患者出现了输血相关循环负荷过重(TACO)等并发症,但病情还是成功稳定了下来。本病例强调了对 TSC 患者进行仔细监测和及时干预的必要性,因为血管肌脂肪瘤,尤其是大于 3 厘米的血管肌脂肪瘤引起出血的风险很高。考虑到临床表现的多变性和晚发症状的可能性,我们还强调了将TSC相关血管肌脂肪瘤与其他肾脏肿块区分开来的重要性。此外,它还强调了多学科方法在管理 TSC 患者中的关键作用,既要解决急性并发症,又要进行长期监测,以防止疾病复发和出现其他系统性表现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Spontaneous angiomyolipoma rupture: A case of hemorrhagic shock and urgent embolization
This case report discusses a 64-year-old male with tuberous sclerosis complex (TSC), a genetic disorder characterized by benign tumor formation across multiple organ systems. The patient presented with a spontaneous hemorrhage from a right renal angiomyolipoma, a common and potentially life-threatening manifestation of TSC, particularly in patients with TSC2 mutations. The patient's significant comorbidities, including hypertension and dyslipidemia, further complicated his clinical course. Initial management involved aggressive fluid resuscitation and blood product transfusion, followed by urgent embolization to control active bleeding. Despite developing complications such as transfusion-associated circulatory overload (TACO), the patient was successfully stabilized. This case highlights the necessity for careful monitoring and prompt intervention in patients with TSC, given the high risk of hemorrhage from angiomyolipomas, especially those larger than 3 cm. We also emphasize the importance of differentiating TSC-associated angiomyolipomas from other renal masses, considering the variability in clinical presentation and the potential for late-onset symptoms. Additionally, it highlights the critical role of a multidisciplinary approach in managing TSC patients, addressing both acute complications and long-term surveillance to prevent recurrence and other systemic manifestations of the disease.
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来源期刊
Radiology Case Reports
Radiology Case Reports Medicine-Radiology, Nuclear Medicine and Imaging
CiteScore
1.10
自引率
0.00%
发文量
1074
审稿时长
30 days
期刊介绍: The content of this journal is exclusively case reports that feature diagnostic imaging. Categories in which case reports can be placed include the musculoskeletal system, spine, central nervous system, head and neck, cardiovascular, chest, gastrointestinal, genitourinary, multisystem, pediatric, emergency, women''s imaging, oncologic, normal variants, medical devices, foreign bodies, interventional radiology, nuclear medicine, molecular imaging, ultrasonography, imaging artifacts, forensic, anthropological, and medical-legal. Articles must be well-documented and include a review of the appropriate literature.
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