Abstract: TH-PO615

Painful Cyst Hemorrhage with Trivial Trauma in Tuberous Sclerosis Complex with Angiomyolipomas without Nephromegaly – A Case Report

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Baynes-Fields, Jaime A., Drexel University College of Medicine, Philadelphia, Pennsylvania, United States
  • Vela-Ortiz, Myriam C., Drexel University College of Medicine, Philadelphia, Pennsylvania, United States
  • Aggarwal, Sandeep, Drexel University College of Medicine, Philadelphia, Pennsylvania, United States
Background

Tuberous sclerosis complex (TSC) is characterized by renal angiomyolipomas and cysts which have the potential to be complicated by hemorrhagic and malignant conversion. Cyst hemorrhage in such cases may happen spontaneously or by trivial trauma due to their highly vascular nature.

Methods

We present a case of a 25 year old male with TSC with subependymal giant cell astrocytoma, seizure disorder, ash leaf spots, bicuspid aortic valve with cardiac rhabdomyoma, multiple renal angiomyolipomas and cysts, hypertension, and CKD stage 1. On a follow up visit, patient presented with right flank pain of one week duration. Patient’s symptoms were preceded by a motor vehicle accident, characterized by sudden deceleration of his vehicle without bodily impact. Patient denied hematuria, lower urinary tract symptoms, or change in urine output. Pertinent physical examination –blood pressure 126/72 mmHg, pulse 85 per minute, right flank tenderness, remainder of physical exam at baseline. Work up – gadolinium enhanced MRI: hemorrhagic conversion of large cystic lesion (1.4cm) as well as increasing angiomyolipomas with stable renal size (right 11.33cm, left 11.2cm). Patient’s renal function remained stable with baseline creatinine 0.66mg/dL, trace proteinuria, no hematuria on dipstick, and 5mcg/mg microalbumin to creatinine ratio. Conservative treatment was provided with pain control and oral hydration given hemodynamic and laboratory stability. Patient improved clinically in follow up.

Conclusion

Cystic nephropathies with nephromegaly present a high risk of hemorrhagic conversion due to fragility and lack of protection by our thoracic cage. TSC with angiomyolipomas present a higher risk due to pathological vascular aneurysms even without nephromegaly, which is emphasized in this case. Hemorrhagic risk of angiomyolipomas are approximately 25-50% and may lead to circulatory shock. Frequent assessment of patient’s symptoms and history, especially related to physical trauma should be sought during each visit. Emphasis should be laid on seizure control, preventative measures and patient education of avoidable injury with immediate follow up after such an event which could be life threatening.