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Williams JM, Racadio JM, Johnson ND, Donnelly LF, Bissler JJ. (2006). Embolization of renal angiomyolipomata in patients with tuberous sclerosis complex. Am J Kidney Dis 47: 95-102.

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Article

Wunderlich’s Syndrome in Advanced Tuberous Sclerosis Complex: A Rare Case of Perinephric Hematoma

1Cardiology Department, Interventional Cardiologist, Case Western Reserve University MetroHealth Medical Center, Cleveland, Ohio, USA

2Department of Cardiology, the University of Arkansas for Medical Science, Little Rock, Arkansas, USA


American Journal of Medical Case Reports. 2019, Vol. 7 No. 12, 334-336
DOI: 10.12691/ajmcr-7-12-8
Copyright © 2019 Science and Education Publishing

Cite this paper:
Aisha Siraj, Negar Salehi. Wunderlich’s Syndrome in Advanced Tuberous Sclerosis Complex: A Rare Case of Perinephric Hematoma. American Journal of Medical Case Reports. 2019; 7(12):334-336. doi: 10.12691/ajmcr-7-12-8.

Correspondence to: Aisha  Siraj, Cardiology Department, Interventional Cardiologist, Case Western Reserve University MetroHealth Medical Center, Cleveland, Ohio, USA. Email: aishasiraj@gmail.com

Abstract

Tuberous sclerosis complex (TSC) is a rare, autosomal dominant disease, associated with mutations in either the TSC gene. The incidence of TSC is estimated as 1 in 5,000-10,000 live births and angiomyolipomas (AML) is one of the characteristic findings of tuberous sclerosis. A 36-year-old Caucasian female admitted to the hospital after two episodes of pre-syncope in March 2016. The patient was tachycardic on examination, and laboratory studies revealed acute anemia with hemoglobin of 8.2 g/dL on admission with a decrease to a nadir of 6.2 g/dL. Imaging studies revealed bilateral fat-rich renal masses with distortion of the renal anatomy consistent with angiomyolipomas (AML). Further as a part of treatment embolization of the right renal artery was performed. However, everolimus is also approved for the treatment of AMLs not requiring surgery. Each patient should have an individualized treatment plan based on the clinical situation as determined by the physician.

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