Wunderlich Syndrome: Comprehensive Review of Diagnosis and Management

Jignesh N. Shah, Darshan Gandhi, Srinivasa R. Prasad, Preet K. Sandhu, Hiral Banker, Ryan Molina, Muhammad Salman Khan, Tushar Garg, Venkata S. Katabathina

Research output: Contribution to journalReview articlepeer-review

4 Scopus citations

Abstract

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.

Original languageEnglish (US)
JournalRadiographics
Volume43
Issue number6
DOIs
StatePublished - Jun 2023

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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