Wunderlich Syndrome: Comprehensive Review of Diagnosis and Management

Acute presentation of Wunderlich syndrome in a pregnant woman treated with supra-selective cannulation of renal segmental arteries and nephrectomy: A case report

“…Although Bonet was the first to describe spontaneous perirenal hemorrhage,[ 1 ] Carl Wunderlich is credited with the detailed description of the entity and coined the term “spontaneous renal capsular stroke.”[ 2 ] Signs and symptoms described by Carl Wunderlich, which would later be known as the Lenk triad, present in 20% of patients, consist of pain and a flank mass in the presence of hypovolemic shock. [ 1 2 ] Angiomyolipoma (~40%) and clear cell renal cell carcinoma (~30%) represent the most common causes of Wunderlich syndrome, followed by other etiologies such as vascular anomalies (~20%) and renal cystic disease.…”

“…Although Bonet was the first to describe spontaneous perirenal hemorrhage,[ 1 ] Carl Wunderlich is credited with the detailed description of the entity and coined the term “spontaneous renal capsular stroke.”[ 2 ] Signs and symptoms described by Carl Wunderlich, which would later be known as the Lenk triad, present in 20% of patients, consist of pain and a flank mass in the presence of hypovolemic shock. [ 1 2 ] Angiomyolipoma (~40%) and clear cell renal cell carcinoma (~30%) represent the most common causes of Wunderlich syndrome, followed by other etiologies such as vascular anomalies (~20%) and renal cystic disease. [ 2 5 ] Angiomyolipoma is characterized by the presence of dysmorphic blood vessels with smooth muscle cells and mature adipose tissue, whereas clear cell renal carcinoma is the most common histologic subtype linked to this syndrome due to its rapid growth and vascular nature with thin-walled sinusoidal vessels.…”

“…[ 1 2 ] Angiomyolipoma (~40%) and clear cell renal cell carcinoma (~30%) represent the most common causes of Wunderlich syndrome, followed by other etiologies such as vascular anomalies (~20%) and renal cystic disease. [ 2 5 ] Angiomyolipoma is characterized by the presence of dysmorphic blood vessels with smooth muscle cells and mature adipose tissue, whereas clear cell renal carcinoma is the most common histologic subtype linked to this syndrome due to its rapid growth and vascular nature with thin-walled sinusoidal vessels. [ 1 2 ] The theory of increased pressure of the renal vessels, changes in tumor perfusion pressure, and subsequent vascular damage is perhaps the one that best explains the development of spontaneous perirenal hemorrhage.…”

“…[ 6 ] Ultrasound is perhaps a very sensitive instrument for quickly identifying the presence of a perirenal hematoma in the emergency department, whereas at the same time, it can be useful for percutaneous drainage, and monitoring. [ 2 ] The perirenal hematoma is visualized as an avascular isoechoic or hyperechoic collection in the acute stage, and as an anechoic collection in the advanced stages. CT usually is the preferred diagnostic approach, because, like ultrasound, it contributes to differential diagnosis evaluation and to the treatment of the syndrome.…”

“…Wunderlich syndrome is a rare entity whose exact incidence is unknown,[ 1 ] characterized by the presence of abdominal pain, hematuria, and hypovolemic shock. Symptoms attributed to the occurrence of spontaneous perirenal hemorrhage, which has its origin in the development of angiolipomas or renal cell carcinoma, among other etiologies,[ 2 3 ] share a common causal mechanism, namely, increase in vascular pressure resulting in necrosis and spontaneous bleeding. [ 4 ] Since Wunderlich syndrome is a potentially lethal entity, high diagnostic suspicion and prompt recognition are required to provide timely management.…”