Ces Urol 2019, 23(3):230-236 | DOI: 10.48095/cccu2019048

Wunderlich's syndrome - cohort of patients with spontaneous nontraumatic retroperitoneal hemorrhage

Vít Paldus1, Vladimír ©ámal1,2, Jan Mečl1
1 Urologické oddělení Krajské nemocnice Liberec, a. s., Liberec
2 Urologická klinika FN a LF UK, Hradec Králové

Paldus V, ©ámal V, Mečl J. Wunderlich´s syndrome - cohort of patients with spontaneous nontraumatic retroperitoneal hemorrhage.

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: Wunderlich´s syndrome, angiomyolipoma, renal carcinoma, extra-adrenal pheochromocytoma, renal lymphangioleiomyomatosis / lymphangioleiomyomatosis of kidney.

Received: July 18, 2019; Accepted: September 5, 2019; Prepublished online: September 6, 2019; Published: September 26, 2019 


References

  1. Michal M, Hes O, Mukenąnabl P. Nádory ledvin dospělého věku. Euroverlag 2000: 10-12, 54-79, 98-112.
  2. Hora M, Hes O, Klecka J, et al. Rupture of papillary renal cell carcinoma. Scand J Urol Nephrol 2004; 38(6): 481-484. Go to original source... Go to PubMed...
  3. Procházková K, Staehler M, Trávníček I, et al. Morphological Characterization of Papillary Renal Cell Carcinoma Type 1, the Efficiency of Its Surgical Treatment. Urol Int. 2017; 98(2): 148-155. Go to original source... Go to PubMed...
  4. Eble JN. Angiomyolipoma of Kidney. Seminars in Diag. Pathol. 1998; 15(1): 21-40.
  5. Vrtěl R, Vodička R, ©antavá A, ©antavý J, Krejčiříková E. Angiomyolipomy a jejich vyąetření v diagnostice tuberózní sklerózy. Čas. Lék. Čes. 2004; 143: 195-197.
  6. Wiliams RD, Dreicer R. Renal Parenchymal Neoplasms. Tanagho EA, McAninch WJ. Smiths General Urology, XIX. ed., Prentice‑Hall Intern. Inc, 1995; 372-374.
  7. Wong K, Waters CM, Hershman MJ, Kaisary VA, Horner J. Angiomyolipoma of the kidney: a clinical enigma in diagnosis and management. Annals of the Royal College of Surg. Of Engl. 1992; 74: 144-148.
  8. Fleischmann J, Huntley HN. Renal Tumors. Clinical Urology. J. B. Lippincot Comp., 1994; 369-37
  9. Klézl P, Richterová R, ©tanc O, Klečka J, Zá»ura F. Objemný angiomyolipom pravé ledviny u mladé ľeny. Urol List 2013; 11(4): 45-48.
  10. Gyimadu AO, Kara O, Basaran D, Esinler I. Conservative management of a retroperitoneal hemorrhage following a ruptured renal angiomyolipoma in pregnancy. J Obstet Gynaecol Res 2011; 37(2): 156-159. Go to original source... Go to PubMed...
  11. Chang SY, Ma CP, Lee SK. Spontaneous retroperitoneal hemorrhage from kidney causes. Eur Urol 1988; 15(3-4): 281-284. Go to original source... Go to PubMed...
  12. Kendall AR, Senay BA, Coll ME. Spontaneous subcapsular renal hematoma: diagnosis and management. Urology 1988; 139(2): 246-250. Go to original source... Go to PubMed...
  13. Moratalla MB. Wunderlich's syndrome due to spontaneous rupture of large bilateral angiomyolipomas. Emerg Med J 2009; 26(1): 72. Go to original source... Go to PubMed...
  14. Hellstrom PA, Mehik A, Talja MT, et al. Spontaneous subcapsular or perirenal haemorrhage caused by renal tumours. A urological emergency. Scand J Urol Nephrol 1999; 33(1): 17-23. Go to original source... Go to PubMed...
  15. Adanur S, Ziypak T, Koç E et al. Renal angiomyolipoma causing retroperitoneal hemorrhage (Wunderlich syndrome): Presentation of our clinical experiences. European Urology Supplements 2013; 12 (4): e1365. Go to original source...
  16. Arabagiu I, Multescu DR, Cozma C, et al. Particular aspect in the diagnosis and treatment of Wunderlich syndrome. European Urology Supplements 2019; 18 (2): e2350. Go to original source...
  17. Ürge T, Hora M, Hes O, Chudáček Z. Renální angiomyolipom, histologie, diagnostika a terapie. Urolog. praxi 2005; 270-272.
  18. Spahn D, Bouillon B, Cerny V, et al. The European guideline on management of major bleeding and coagulopathy following trauma: fifth edition. Critical Care 2019; 23: 98. Go to original source... Go to PubMed...





Web časopisu Česká urologie je určen pouze pro lékaře a odborníky
z oblasti medicíny nebo farmacie.



Beru na vědomí, ľe informace zveřejněné na těchto stránkách
nejsou určeny pro laickou veřejnost.



Odejít Vstoupit