Wunderlich syndrome with pseudoaneurysm due to angiomyolipoma rupture
Presentation
A postpartum patient with left flank pain and mass feeling on physical exam, hypovolemia shock, and gross hematuria.
Patient Data
An 85 × 70 mm hetero-dense fat-containing mass is seen in the anterior aspect of the left kidney, accompanied by moderate subcapsular and perinephric hyperdense fluid accumulation. After contrast media injection, the lesion shows heterogeneous enhancement with a 33 mm contrast-filled space within, inferring a pseudoaneurysm.
Diastasis recti is present up to 68 mm, accompanied by a small fat-containing umbilical hernia.
Case Discussion
A spontaneous non-traumatic perinephric hemorrhage with acute flank pain, flank mass feeling, and hypovolemic shock is known as Wunderlich syndrome. It can be due to neoplastic etiologies such as angiomyolipoma and renal cell carcinoma rupture or non-neoplastic causes like vasculitis, AVM, and AVF rupture. The process is rarely complicated by pseudoaneurysm formation that should be reported in detail.
Angiomyolipomas are the most common benign neoplastic mass and the most common fat-containing lesion of kidneys. About 95% of angiomyolipomas show macroscopic fat, and only 5% may have microscopic fat, which is more common in patients with tuberous sclerosis.
Some texts and literature have described that angiomyolipomas may increase in size during pregnancy and predispose to spontaneous rupture, which is more common in the third trimester or first three months after delivery.
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