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以休克为表现的肾血管平滑肌脂肪瘤自发性破裂:来自坦桑尼亚的一例病例报告

Spontaneous rupture of renal angiomyolipoma presenting with shock; a case report from Tanzania.

作者信息

Chhatbar Nishi, Ismail Allyzain, Panjwani Sajida, Datoo Adil, Uddin Hussam, Zehri Aliakbar

机构信息

The Aga Khan Hospital, Dar-es-Salaam, Tanzania.

The Aga Khan University, East Africa Medical College, Tanzania.

出版信息

Int J Surg Case Rep. 2023 Dec;113:109073. doi: 10.1016/j.ijscr.2023.109073. Epub 2023 Nov 23.

Abstract

INTRODUCTION AND IMPORTANCE

Renal angiomyolipoma (AML) are benign tumors, often incidentally diagnosed with rupture being the commonest complication and cause of mortality. These tumors are rare with a higher prevalence among patients with tuberous sclerosis and female predominance. Management is dependent on tumor size and whether or not the tumor has ruptured.

CASE PRESENTATION

32-year-old female presenting with sudden right flank pain with shock without history of prior trauma or surgeries. Underwent imaging revealing a suspected ruptured AML thus underwent emergent nephrectomy with admission to intensive care.

CLINICAL DISCUSSION

Wunderlich syndrome manifests as the Lenk triad, which includes acute flank pain, flank mass, and hypovolemic shock with signs of internal bleeding such as hematuria. It is a rare manifestation signifying spontaneous renal hemorrhage. Due to the instability of the patient had to undergo emergency laparotomy and nephrectomy.

CONCLUSION

Renal AML occur as a rare benign phenomenon which when ruptured are associated with high mortality rate if not treated promptly in a setting with specialized treatment and intensive unit care. We hope that through our experience patients presenting with Lenk's triad are identified early for adequate intervention.

摘要

引言与重要性

肾血管平滑肌脂肪瘤(AML)是良性肿瘤,常为偶然诊断,破裂是最常见的并发症和死亡原因。这些肿瘤罕见,在结节性硬化症患者中患病率较高,且女性居多。治疗取决于肿瘤大小以及肿瘤是否破裂。

病例介绍

一名32岁女性,突发右侧胁腹疼痛并伴有休克,无既往外伤或手术史。影像学检查显示疑似破裂的AML,因此接受了急诊肾切除术,并入住重症监护病房。

临床讨论

温德利希综合征表现为Lenk三联征,包括急性胁腹疼痛、胁腹肿块以及伴有血尿等内出血迹象的低血容量性休克。这是一种罕见的表现,提示自发性肾出血。由于患者病情不稳定,不得不接受急诊剖腹手术和肾切除术。

结论

肾AML是一种罕见的良性病变,破裂后若在缺乏专科治疗和重症监护的情况下未及时治疗,死亡率很高。我们希望通过我们的经验,能早期识别出出现Lenk三联征的患者,以便进行充分干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a1a/10709492/961bed9dc3d7/gr1.jpg

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