International Journal of Case Reports and Images - IJCRI - Case Reports, Case Series, Case in Images, Clinical Images

   
Case Report
 
A rare presentation of tuberous sclerosis complex: Complicated renal cyst leads to sepsis
Ourania S. Kotsiou1, Konstantinos I. Gourgoulianis2
1Resident in Respiratory Medicine Department, University of Thessaly School of Medicine, Biopolis, 4110 Larissa, Thessaly, Greece.
2Professor in Respiratory Medicine Department, University of Thessaly School of Medicine, Biopolis, 4110 Larissa, Thessaly, Greece.

doi:10.5348/ijcri-201616-CR-10603

Address correspondence to:
Ourania S Kotsiou
Respiratory Medicine Department, University of Thessaly School of Medicine
University Hospital of Larissa, Biopolis
4110 Larissa
Greece

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How to cite this article
Kotsiou OS, Gourgoulianis KI. A rare presentation of tuberous sclerosis complex: Complicated renal cyst leads to sepsis. Int J Case Rep Images 2016;7(2):106–110.


Abstract
Introduction: Tuberous sclerosis complex is a rare autosomal dominant genetic disease, having multi systemic involvement. Kidneys are affected up to 80% of patients, in the form of renal angiomyolipomas, renal cysts or renal cell carcinoma. Renal cysts are presented as single or multiple lesions that are infrequently symptomatic. Rarely, tuberous sclerosis co-exists with polycystic kidney disease with poor prognosis through renal failure or hematuria. The complication of the renal cysts with the form of abscess is extremely rare. Here we present a case of tuberous sclerosis in which complicated renal cyst led to sepsis.
Case Report: A 30-year-old female, with a history of tuberous sclerosis, presented with a week-long fever and a left pleuritic chest pain. Chest radiograph disclosed a left costophrenic angle blunting. Thoracic ultrasound followed, surprisingly revealed a big subdiaphragmatic renal cystic formation pressing against left hemidiaphragm, not previously known. While the patient was septic with no reply to the empirical antibiotic therapy and with a gradually worsening dyspnea, the cystic drainage was decided, resulted in a fast clinical improvement. Proteus mirabilis was cultured from cystic content.
Conclusion: Renal disease is the most common cause of death in population affected by tuberous sclerosis. Thus, monitoring and serial radiologic examination are necessary to improve the prognosis of kidney damage. Abscess following simple renal cyst infection constitute another rare but potentially life-threatening renal complication should be taken under consideration.

Keywords: Abscess, Complicated renal cyst, Sepsis, Tuberous sclerosis complex (TSC)


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Author Contributions
Kotsiou Ourania – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Gourgoulianis Konstantinos – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Kotsiou Ourania et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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