Wunderlich syndrome is a rare condition in which spontaneous non-traumatic renal hemorrhage occurs into the subcapsular and perirenal spaces.
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Clinical presentation
Wunderlich syndrome is clinically characterized by Lenk's triad:
acute flank pain
flank mass
hypovolemic shock
Mnemonic
F: flank pain (acute)
F: flank mass
H: hypovolemic shock
Pathology
Etiology
-
neoplastic
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
-
non-neoplastic
vascular causes: vasculitis (polyarteritis nodosa being the commonest cause), renal artery aneurysms, AV malformations and fistulas, renal vein thrombosis
calculus disease
nephritis
coagulation disorders
Radiographic features
Hemorrhage in the perinephric space, often with a primary lesion seen within the kidney, e.g. angiomyolipoma, renal cell carcinoma.
Plain radiograph
On plain abdominal X-rays; indirect features of a retroperitoneal mass including effacement of the psoas outline, loss of renal fat outline, displacement of bowel.
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.