Disclosures: Lindsay A. Burke, MD: No financial relationships or conflicts of interest

Case Diagnosis: Thoracic myelopathy secondary to arachnoid cyst in a patient with Marfan syndrome

Case Description or Program Description: A 73-year-old man with a history of Marfan syndrome and non-healing ischial ulcer presented to the hospital with progressive leg weakness plus, bowel and bladder incontinence over several months and was found on MRI to have a thoracolumbar junction arachnoid cyst causing spinal cord compression and signal change. He underwent emergent L1 laminectomy with cyst fenestration and post-operatively was transferred to acute rehabilitation where his functional independence improved significantly enabling a safe discharge home.

Setting: Urban Academic University Hospital

Assessment/Results: On admission to acute rehabilitation, he was determined to have T12 ASIA Impairment Scale (AIS) C paraplegia. He required maximal assistance of one person with bowel and bladder care and transfers. Throughout his course, he developed independence with bowel and bladder management and transfers. His mobility improved such that he could ambulate household distances with standby assistance and independently navigate community distances at the wheelchair level.

Discussion (relevance): Marfan syndrome is a connective tissue disease with wide-ranging clinical manifestations including dural ectasia. Weakness in the dura due to poorly formed elastic tissue has led to reports of meningeal spinal cysts. Arachnoid cysts, however, are not commonly reported likely because they are typically asymptomatic. Additionally, comprehensive literature review resulted in reports only of sacral arachnoid cysts but failed to yield any documented cases of thoracic arachnoid cysts in Marfan syndrome. Our patient’s presentation of a severely symptomatic thoracic arachnoid cyst is unique.

Conclusions: This appears to be the first documented case of thoracic arachnoid cyst in a patient with Marfan syndrome causing spinal cord compression and myelopathy.  This diagnosis should be considered on the differential of progressively worsening weakness in a patient with Marfan syndrome as a treatable cause of spinal cord compression.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/thoracic-myelopathy-secondary-to-arachnoid-cyst-in-a-patient-with-marfan-syndrome-a-case-report/