Wunderlich syndrome

Wunderlich syndrome is spontaneous, nontraumatic renal hemorrhage confined to the subcapsular and perirenal space. It may be the first manifestation of a renal angiomyolipoma (AML), or rupture of renal artery or intraparechymal aneurysm.

Cause

Neoplasms are the most common underlying pathology in up to 60% of cases and include renal angiomyolipoma and renal cell carcinoma. Other etiologies include rupture of renal artery or an arteriovenous malformation, polyarteritis nodosa, cystic medial necrosis, segmental arterial mediolysis, and cystic rupture.Few case series suggested angiomyolipoma as the most common cause.

Diagnosis

First symptoms may be subtle such as mild pain, flank tenderness, haematuria. Depending on blood loss, symptoms of hypovolemic shock may develop. Hematoma is usually contained in the retroperitoneum, allowing for a period of haemodynamic stability. Sometimes massive acute hemorrhage is seen when a hematoma ruptures Gerota's fascia and extends into the peritoneum. An ultrasound or CT scan can establish diagnosis, while lab tests may be inconclusive as changes of haematocrit or haemoglobin are not specific to the syndrome, while haematuria is not always present.

Treatment

Treatment varies according to severity, ranging from monitoring of the hematoma (in haemodynamic stability) to emergency surgery (when patients develop hypovolemic shock requiring seminephrectomy or nephrectomy). Vascular causes lead to surgery due to severity of hemorrhage. Robotic-assisted partial nephrectomy has been proposed as a surgical treatment of a ruptured angiomyolipoma causing retroperitoneal hemorrhage, combining the advantages of a kidney preservation procedure and the benefits of a minimally invasive procedure without compromising the safety of the patient.

NIH genetic and rare disease info

• NIH info on Wunderlich syndrome

Wunderlich syndrome is a rare disease.

Wunderlich syndrome Resources
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