肾血管平滑肌脂肪瘤破裂导致的Wunderlich综合征临床误诊分析  

Analysis of Clinical Misdiagnosis of Wunderlich Syndrome Caused by Renal Angiomyolipoma Rupture

作　　者：徐晶晶 丁俊 刘俊强 XU Jingjing;DING Jun;LIU Junqiang(Department of Anesthesiology,No.904 Hospital of the Joint Logistics Support Force of the PLA,Wuxi,Jiangsu 214000,China)

机构地区：[1]联勤保障部队第九〇四医院麻醉科,江苏无锡214000 [2]联勤保障部队第九〇四医院泌尿外科,江苏无锡214000

出　　处：《临床误诊误治》2025年第9期28-32,共5页Clinical Misdiagnosis & Mistherapy

基　　金：无锡市卫生健康委科技成果和适宜技术推广项目(T202451)。

摘　　要：目的 探讨肾血管平滑肌脂肪瘤破裂导致Wunderlich综合征临床特点、诊疗要点,以减少临床误诊误治。方法 回顾性分析2021年7月至2023年8月收治的3例肾血管平滑肌脂肪瘤破裂导致的Wunderlich综合征患者的临床资料,并复习相关文献。结果 1例患者表现为左侧腰腹部疼痛、恶心、呕吐等,初步诊断为腹腔肿瘤破裂;2例患者表现为右侧腰腹部疼痛等,初步诊断为自发性肾破裂出血。病变均为单侧肾,左侧1例,右侧2例;3例患者均给予抗感染、止血、止痛治疗,2例给予抗休克治疗,病情未见明显改善。全腹部增强CT检查均提示肾血管平滑肌脂肪瘤破裂伴大出血。行手术干预治疗,其中行肾切除术1例,肾动脉栓塞介入治疗后行肾部分切除术2例。术后病理证实为肾血管平滑肌脂肪瘤破裂。误诊时间为1~3 d。3例患者术后随访1年,恢复良好,均无复发。结论 肾血管平滑肌脂肪瘤破裂所致的Wunderlich综合征临床少见,疾病较凶险,需早期诊断及治疗。Wunderlich综合征的诊断和治疗需多学科联合会诊,并应重视早期生命体征变化。应根据病情的危重程度和影像学诊断结果制订治疗方案,以避免延误治疗。Objective To explore the clinical features,diagnosis and treatment of Wunderlich syndrome caused by renal angiomyolipoma rupture,so as to reduce clinical misdiagnosis and mistreatment.Methods The clinical data of 3 patients with Wunderlich syndrome caused by renal angiomyolipoma rupture admitted from July 2021 to August 2023 were retrospectively analyzed,and the relevant literature was reviewed.Results One patient presented with pain on the left side of the waist and abdomen,nausea and vomiting,and was initially diagnosed as abdominal tumor rupture.Two patients presented with pain in the right lower back and abdomen,and the initial diagnosis was spontaneous renal rupture hemorrhage.All the lesions were unilateral kidney,with 1 patient on the left side and 2 patients on the right side.All three patients were given anti-infection treatment,hemostasis and analgesia,including 2 patients receiving anti shock treatment,and their condition did not improve significantly.All abdominal contrast-enhanced CT examinations indicated renal angiomyolipoma rupture with massive hemorrhage.Surgical intervention was performed,including nephrectomy in 1 patient,and partial nephrectomy in 2 patients after renal artery embolization.Postoperative pathology confirmed the rupture of renal angiomyolipoma.The misdiagnosis lasted 1-3 d.The 3 patients were followed up for 1 year after operation and recovered well without recurrence.Conclusion Wunderlich syndrome caused by rupture of renal angiomyolipoma is rare and dangerous,requiring early diagnosis and treatment.The diagnosis and treatment of Wunderlich syndrome requires multidisciplinary consultation,and attention should be paid to the changes of early vital signs.The treatment strategy should be developed based on the severity of the condition and imaging diagnosis results to avoid delayed treatment.

关 键 词：Wunderlich综合征 肾血管平滑肌脂肪瘤 误诊 肾肿瘤 经导管动脉栓塞 肾部分切除术 诊断 鉴别 

分 类 号：R692[医药卫生—泌尿科学]