Wunderlich Syndrome - A Rare Cause of Hypovolemic Shock- Case Report

Retroperitoneal haemorrhage (Wunderlich syndrome) is a  rare cause of hypovolemic shock in emergency patients; however,  it is associated with a high morbidity and presents a diagnostic challenge in the Emergency Department. It is more common in female patients older than 50 years, and one of the leading causes is a benign renal tumour angiomyolipoma (AML). Angiomyolipoma predominantly affects only one kidney. Bilateral  AML is strongly associated with a rare autoimmune disorder-tuberous sclerosis and occur in  80%  of cases of  TS.  Symptoms caused by growing renal AML are absent to mild and non-specific  (intermittent loin,  lower back pains, haematuria). Rupture with retroperitoneal bleeding is a frequent complication of AML and occurs  in 20%  of all cases. Initial presentation of retroperitoneal haemorrhage is remarkably similar to a ruptured abdominal aortic aneurysm (AAA). Most of the cases remain clinically silent during the growth phase and present to the hospital with a  complication in the form of retroperitoneal bleeding. Risk of bleeding increases with the size of the tumor and AML bigger than  4cm are treated with selective embolization if found on routine scanning. Many cases of  AML are diagnosed when a patient is being scanned for other medical problems. Even in an emergency when AML ruptures  is still possible to treat it either with selective embolization or selective nephrectomy, and the majority of patients are making a successful recovery.

Author(s): JaroslawNowacki

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