Wunderlich’s syndrome – cohort of patients with spontaneous nontraumatic retroperitoneal hemorrhage

Česká verzia

Authors: Vít Paldus ¹; Vladimír Šámal ^1,2; Jan Mečl ¹
Authors place of work: Urologické oddělení Krajské nemocnice Liberec, a. s., Liberec ¹; Urologická klinika FN a LF UK, Hradec Králové ²
Published in the journal: Ces Urol 2019; 23(3): 230-236
Category: Original Articles

Summary

Objective: Retrospective evaluation of a cohort of patients who suffered from Wunderlich´s syndrome. Assessment of the etiology, clinical manifestation, standard diagnostic tools and treatment options in patients with Wunderlich´s syndrome.

Methods: We performed a retrospective evaluation of a cohort of 8 patients who suffered from spontaneous renal hemorrhage / retroperitoneal bleeding during the 15 years period. The group of patients was divided according to the etiology, clinical manifestation, diagnostic tools and treatment procedures performed in individual patients. Post treatment clinical evaluation was performed as well.

Results: There were 4 cases of renal hemorrhage / retroperitoneal bleeding due to spontaneous rupture of angiolipoma. 2 patients suffered from bleeding due to renal carcinoma rupture. There was 1 patient with tuberous sclerosis and 1 patient with spontaneous rupture of the extra adrenal pheochromocytoma (an extremely rare condition). 5 patients underwent open surgical treatment, while in the remaining 3 patients endovascular procedure was performed (with the aim of preserving the affected organ/kidney). There were no serious adverse events related to the procedure performed / with relationship to the performed procedure. One patient died shortly after the procedure due to tumor progression. The remaining patients are alive.

Conclusion: Spontaneous, nontraumatic renal hemorrhage / retroperitoneal bleeding presented as Wunderlich´s syndrome is a very serious and in some cases life threatening condition. Therapeutic approach is determined by the assessment of the patient´s health status as well as by the possible etiology of bleeding.

Keywords:

angiomyolipoma – Wunderlich´s syndrome – renal carcinoma – extra-adrenal pheochromocytoma – renal lymphangioleiomyomatosis / lymphangioleiomyomatosis of kidney

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