TY - JOUR
T1 - Wunderlich Syndrome
T2 - Comprehensive Review of Diagnosis and Management
AU - Shah, Jignesh N.
AU - Gandhi, Darshan
AU - Prasad, Srinivasa R.
AU - Sandhu, Preet K.
AU - Banker, Hiral
AU - Molina, Ryan
AU - Khan, Muhammad Salman
AU - Garg, Tushar
AU - Katabathina, Venkata S.
N1 - Publisher Copyright:
© RSNA, 2023.
PY - 2023/6
Y1 - 2023/6
N2 - Wunderlich syndrome (WS), which was named after Carl Wunderlich, is a rare clinical syndrome characterized by an acute onset of spontaneous renal hemorrhage into the subcapsular, perirenal, and/or pararenal spaces, without a history of antecedent trauma. Patients may present with a multitude of symptoms ranging from nonspecific flank or abdominal pain to serious manifestations such as hypovolemic shock. The classic symptom complex of flank pain, a flank mass, and hypovolemic shock referred to as the Lenk triad is seen in a small subset of patients. Renal neoplasms such as angiomyolipomas and clear cell renal cell carcinomas that display an increased proclivity for hemorrhage and rupture contribute to approxi-mately 60%–65% of all cases of WS. A plethora of renal vascular diseases (aneurysms or pseudoan-eurysms, arteriovenous malformations or fistulae, renal vein thrombosis, and vasculitis syndromes) account for 20%–30% of cases of WS. Rare causes of WS include renal infections, cystic diseases, calculi, kidney failure, and coagulation disorders. Cross-sectional imaging modalities, particularly multiphasic CT or MRI, are integral to the detection, localization, and characterization of the underlying causes and facilitate optimal management. However, large-volume hemorrhage at patient presentation may obscure underlying causes, particularly neoplasms. If the initial CT or MRI examination shows no contribu-tary causes, a dedicated CT or MRI follow-up study may be warranted to establish the cause of WS. Renal arterial embolization is a useful, minimally invasive, therapeutic option in patients who present with acute or life-threatening hemorrhage and can help avoid emergency radical surgery. Accurate diagnosis of the underlying cause of WS is critical for optimal patient treatment in emergency and nonemergency clinical settings.
AB - Wunderlich syndrome (WS), which was named after Carl Wunderlich, is a rare clinical syndrome characterized by an acute onset of spontaneous renal hemorrhage into the subcapsular, perirenal, and/or pararenal spaces, without a history of antecedent trauma. Patients may present with a multitude of symptoms ranging from nonspecific flank or abdominal pain to serious manifestations such as hypovolemic shock. The classic symptom complex of flank pain, a flank mass, and hypovolemic shock referred to as the Lenk triad is seen in a small subset of patients. Renal neoplasms such as angiomyolipomas and clear cell renal cell carcinomas that display an increased proclivity for hemorrhage and rupture contribute to approxi-mately 60%–65% of all cases of WS. A plethora of renal vascular diseases (aneurysms or pseudoan-eurysms, arteriovenous malformations or fistulae, renal vein thrombosis, and vasculitis syndromes) account for 20%–30% of cases of WS. Rare causes of WS include renal infections, cystic diseases, calculi, kidney failure, and coagulation disorders. Cross-sectional imaging modalities, particularly multiphasic CT or MRI, are integral to the detection, localization, and characterization of the underlying causes and facilitate optimal management. However, large-volume hemorrhage at patient presentation may obscure underlying causes, particularly neoplasms. If the initial CT or MRI examination shows no contribu-tary causes, a dedicated CT or MRI follow-up study may be warranted to establish the cause of WS. Renal arterial embolization is a useful, minimally invasive, therapeutic option in patients who present with acute or life-threatening hemorrhage and can help avoid emergency radical surgery. Accurate diagnosis of the underlying cause of WS is critical for optimal patient treatment in emergency and nonemergency clinical settings.
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U2 - 10.1148/rg.220172
DO - 10.1148/rg.220172
M3 - Review article
C2 - 37227946
AN - SCOPUS:85160455480
SN - 0271-5333
VL - 43
JO - Radiographics
JF - Radiographics
IS - 6
ER -