Wünderlich Syndrome: A Narrative Review and Case Report
DOI:
https://doi.org/10.17533/udea.iatreia.286Keywords:
Abdomen, Acute, Acute Kidney Injury, Hemorrhage, KidneyAbstract
Introduction: Wünderlich syndrome is a rare condition with fewer than 300 reported cases. It is characterized by the presence of Lenk's triad: abdominal pain, flank mass, and hypovolemic shock. Rapid treatment is required due to the risk of potentially lethal hemorrhagic shock.
Methods: A case is presented along with a narrative review of the state of the art on spontaneous renal hemorrhage.
Results: We report a patient who arrived at the emergency department with sudden, severe abdominal pain associated with prolonged capillary refill time and tachycardia. The FAST-E protocol was applied, documenting free fluid. Due to the absence of macrohemodynamic compromise, a multiphase computed tomography was performed, which revealed a left renal laceration with extensive hemoperitoneum. The patient underwent laparotomy, where a 1500 mL hemoperitoneum and left perirenal and pararenal hematoma were found. Partial nephrectomy and renal packing were performed. During the postoperative period, a selective arteriography of abdominal vessels was conducted, revealing a cortical perfusion defect in the middle third. Following surgical intervention, the patient had an optimal clinical evolution.
Conclusions: Wünderlich syndrome, or spontaneous perirenal hemorrhage, is a rare condition. The most common causes are neoplasms, vascular injuries, and coagulopathies. Upon identifying the hemorrhage, a multiphase computed tomography or FAST-E should be performed. Active extravasation indicates the need for intervention through angiography and embolization, reserving surgery for emergencies or refractory cases.
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References
(1) Parmar N, Langdon J, Kaliannan K, Mathur M, Guo Y, Mahalingam S. Wunderlich Syndrome: Wonder What It Is. Vol. 51, Curr Probl Diagn Radiol [Internet]. 2022;52(2):270-281. https://doi.org/10.1067/j.cpradiol.2020.12.002
(2) Zhang JQ, Fielding JR, Zou KH. Etiology of spontaneous perirenal hemorrhage: A meta-analysis. J Urol [Internet]. 2002;167(4). https://doi.org/10.1016/S0022-5347(05)65160-9
(3) Roxo MI, Pepe B, Birne R. Wunderlich syndrome in a patient on hemodialysis. Braz J Nephrol [Internet]. 2023;45(3). https://doi.org/10.1590/2175-8239-jbn-2023-0011en
(4) Kim JW, Kim JY, Ahn ST, Park TY, Oh MM, Moon DG, et al. Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases. Am J Emerg Med [Internet]. 2019;37(1):45-47. https://doi.org/10.1016/j.ajem.2018.04.045
(5) Giovini M, Poggiali E, Zocchi P, Bianchi E, Antonucci E, Barbera M. A Case of Spontaneous Renal Haemorrhage (Wunderlich Syndrome) in an Anticoagulated Patient. Eur J Case Rep Intern Med [Internet]. 2022;9(4). https://doi.org/10.12890/2022_003269
(6) Shah JN, Gandhi D, Prasad SR, Sandhu PK, Banker H, Molina R, et al. Wunderlich Syndrome: Comprehensive Review of Diagnosis and Management. Vol. 43, Radiographics [Internet]. 2023;43(6). https://doi.org/10.1148/rg.220172
(7) Vercelli A, Pagani L, Christodoulakis K, Marcello G, Petrini M, Michieletti E, et al. Hypovolemic shock due to Wunderlich syndrome (spontaneous renal haemorrhage): a case report and literature review. Acta Biomed [Internet]. 2023;94(3). Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10308475/
(8) Triana GJJ, García CA, Fernández JN. Síndrome de Wünderlich en una paciente con 10 semanas de gestación tratado satisfactoriamente con embolización arterial selectiva. Rev Urol Colomb [Internet]. 2015;24(3):177-80. https://doi.org/10.1016/j.uroco.2015.09.006
(9) Rued L, Torres P, Ballestas C, González A. Renal Angiomyolipoma with Spontaneus Rupture (Wünderlich Syndrome): A Case Report. Rev Colomb Radiol [Internet]. 2015;26(3):4279-82. Available from: https://contenido.acronline.org/Publicaciones/RCR/RCR26-3/08_Angiomiolipoma.pdf
(10) Godbey EA, Schwartz J. "Massive transfusion protocols and the use of tranexamic acid." Curr Opin Hematol [Internet]. 2018;25(6):482-485. https://doi.org/10.1097/MOH.0000000000000457
(11) McDougal WS, Kursh ED, Persky L. Spontaneous rupture of the kidney with perirenal hematoma. J Urol [Internet]. 1975;114(2):181-184. https://doi.org/10.1016/S0022-5347(17)66981-7
(12) Cinman AC, Farrer J, Kaufman JJ. Spontaneous perinephric hemorrhage in a 65-year-old man. J Urol [Internet]. 1985;133(5):829-832. https://doi.org/10.1016/S0022-5347(17)49243-3
(13) Ahn T, Roberts MJ, Navaratnam A, Chung E, Wood S. Changing etiology and management patterns for spontaneous renal hemorrhage: a systematic review of contemporary series. Int Urol Nephrol [Internet]. 2017;49:1897–1905. https://doi.org/10.1007/s11255-017-1694-8
(14) Mao Y, De Oliveira IS, Hedgire S, Prapruttam D, Harisinghani M. Aetiology, imaging features, and evolution of spontaneous perirenal haemorrhage. Clin Radiol [Internet]. 2017;72(2):175.e19-175.e26. https://doi.org/10.1016/j.crad.2016.08.010
(15) Linehan WM, Ricketts CJ. The Cancer Genome Atlas of renal cell carcinoma: findings and clinical implications. Nat Rev Urol [Internet]. 2019;16:539-552. https://doi.org/10.1038/s41585-019-0211-5
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