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Wunderlich syndrome is a rare condition in which spontaneous non-traumatic renal hemorrhage occurs into the subcapsular and perirenal spaces.
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Wunderlich syndrome is clinically characterized by Lenk's triad:
- acute flank pain
- flank mass
- hypovolemic shock
- F: flank pain (acute)
- F: flank mass
- H: hypovolemic shock
- neoplasms are the most common cause
- among benign neoplasms, angiomyolipoma is the commonest, while among malignancies, renal cell carcinoma is the commonest
- cases of hemorrhage from tuberous sclerosis and pregnancy-related angiomyolipomas have been reported 1,4
- vascular causes: vasculitis (polyarteritis nodosa being the commonest cause), renal artery aneurysms, AV malformations and fistulas, renal vein thrombosis
- cystic renal diseases
- calculus disease
- coagulation disorders
Hemorrhage in the perinephric space, often with a primary lesion seen within the kidney, e.g. angiomyolipoma, renal cell carcinoma.
Treatment and prognosis
If the hemorrhage is self-limiting and the patient is responsive to fluid resuscitation, the patient can be managed conservatively. Angiographic selective embolization may be pursued in haemodynamically stable patients. In haemodynamically unstable patients, emergent nephrectomy (partial or total) is often required 3.
History and etymology
It is named after the German physician Carl Reinhold August Wunderlich (1815-1877), who published the first case description in 1856, and who is most famously remembered for his pioneering work on clinical thermometry 5,6. The term Wunderlich syndrome was first recorded by Coenen in 1910 6.
- 1. Sparks D, Chase D, Thomas D et-al. The Wunderlich's syndrome secondary to massive bilateral angiomyolipomas associated with advanced tuberous sclerosis. Saudi J Kidney Dis Transpl. 2011;22 (3): 534-7. Saudi J Kidney Dis Transpl (link) - Pubmed citation
- 2. Katabathina VS, Katre R, Prasad SR et-al. Wunderlich syndrome: cross-sectional imaging review. J Comput Assist Tomogr. 35 (4): 425-33. doi:10.1097/RCT.0b013e3182203c5e - Pubmed citation
- 3. Morgentaler A, Belville JS, Tumeh SS et-al. Rational approach to evaluation and management of spontaneous perirenal hemorrhage. Surg Gynecol Obstet. 1990;170 (2): 121-5. - Pubmed citation
- 4. Kane D, Abdelrahman M, Looney AT, Eogan M. Wunderlich's syndrome in pregnancy: a shocking triad. (2019) BMJ case reports. doi:10.1136/bcr-2019-229219 - Pubmed
- 5. Mackowiak PA, Worden G. Carl Reinhold August Wunderlich and the evolution of clinical thermometry. (1994) Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 18 (3): 458-67. doi:10.1093/clinids/18.3.458 - Pubmed
- 6. Wang BH, Pureza V, Wang H. A tale of Wünderlich syndrome. (2012) Journal of surgical case reports. doi:10.1093/jscr/rjs015 - Pubmed