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. 2011 Jun;73(3):227-9.
doi: 10.1007/s12262-010-0231-0. Epub 2011 Jan 27.

Wunderlich's Syndrome in a Tuberous Sclerosis Patient

Surya Prakash Vaddi  1 , Vedamurthy Pogula Reddy, Rahul Devraj
Affiliations

Wunderlich's Syndrome in a Tuberous Sclerosis Patient

Surya Prakash Vaddi et al. Indian J Surg. 2011 Jun.

Abstract

Spontaneous non-traumatic renal hemorrhage known as Wunderlich's syndrome is known to occur in renal angiomyolipoma and may be the first manifestation of the disease. Angiomyolipomas in tuberous sclerosis are usually bilateral and multicentric. A 25yr old female, a case of Tuberous sclerosis with bilateral renal angiomyolipomas presented with right loin pain of increasing severity. On evaluation she had acute abdomen with increasing abdominal distension. Investigations revealed large right perinephric hematoma with bilateral renal angiomyolipomas. Exploratory laparotomy and right nephrectomy was done. Spontaneous hemorrhage in renal angiomyolipoma in a case of tuberous sclerosis and management are discussed.

Electronic supplementary material: The online version of this article (doi:10.1007/s12262-010-0231-0) contains supplementary material, which is available to authorized users.

Keywords: Renal angiomyolipomas; Tuberous sclerosis syndrome (TS); Wunderlich's syndrome.

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Figures

Fig. 1
Fig. 1
Tuberous sclerosis patient with adenoma sebaceum over the face
Fig. 2
Fig. 2
CECT scan of abdomen showing bilateral renal angiomyolipomas with large right perinephric hematoma
See this image and copyright information in PMC

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