Key result
Urinary disorders in Marfan syndrome stem from dural ectasia or aortic surgery complications.
Why the study?
Urinary disorders are uncommon in Marfan syndrome and their pathophysiology and clinical presentation are not well characterized.
Case Report (n=4)
Urinary disorders in Marfan syndrome are rare but can occur due to surgical complications from aortic surgery or sacral nerve root compression, requiring medical care to prevent uro-nephrological complications.
Supports evaluation for these causes in Marfan patients with urinary disorders; hypothesis-generating case data leave open prevalence and management needs.
Marfan syndrome is a genetic disease responsible for causing cardiovascular, eye and musculoskeletal damages. Urinary disorders are not common. We present 4 cases of chronic urinary tract symptoms, with 2 different pathophysiological processes. Three patients presented with spinal cord infarct following aortic dissection surgery. They were affected by an overactive bladder with detrusor overactivity and detrusor-sphincter dyssynergia. One patient complained of voiding dysfunction, possibly related to dural ectasia. Although a rare outcome, urinary disorders may appear in Marfan syndrome, by the occurrence of surgical complications in aortic surgery or possibility of sacral nerve root compression. If so, medical care is necessary to prevent uro-nephrological complications.
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Hentzen et al. (2017) conducted a case report in Marfan syndrome with urinary disorders (n=4). Spinal cord infarct or dural ectasia was evaluated on Pathophysiological processes of urinary disorders. Urinary disorders in Marfan syndrome may arise from surgical complications in aortic surgery (spinal cord infarct) or sacral nerve root compression (dural ectasia).
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