Marfan syndrome and symptomatic sacral cyst: Report of two cases

Abstract

Context: Meningeal abnormalities such as dural ectasia are seen in Marfan syndrome, but spinal meningeal cysts are rarely seen. These cysts usually asymptomatic and often found incidentally on magnetic resonance imaging, large cysts may cause neurological deficits and pain secondary to nerve root compression. Design: Case reports. Findings: Two patients with Marfan syndrome presented with urinary symptoms secondary to dural ectasia and sacral cysts. Patient 1 had a history of low back pain, erectile dysfunction, and occasional urinary incontinence and groin pain with recent symptom worsening. He underwent L5 partial laminectomy and S1-S2 laminectomy with sacral cyst decompression. Nine weeks later, he underwent drainage of a sacral pseudomeningocele. Pain and urinary symptoms resolved, and he remains neurologically normal 2 years after surgery. Patient 2 presented after a fall on his tailbone, complaining of low back pain and difficulty urinating. Physical therapy was implemented, but after 4 weeks, urinary retention had not improved. He then underwent resection of the sacral cyst and S1-S3 laminectomy. Pain and paresthesias resolved and bowel function returned to normal. Other than needing intermittent self-catheterization, all other neurologic findings were normal 30 months after surgery. Conclusion/clinical relevance: Surgical goals for sacral cysts include resection as well as closure of the dura, which can be challenging due to thinning from ectasia. Neurosurgical intervention in Marfan syndrome is associated with a high risk of dural tears and osseous complications, and should be performed only when symptoms are severe.

Multiple Large Perineural (Tarlov) Cysts in the Sacrum of a Cadaver: A Case Report and Review

“…In the study by Marino, et al, examination of family histories suggested that Tarlov cysts have both vertical and lateral inheritance, in some cases. A report connecting Tarlov cysts with Marfan syndrome supports a genetic hypothesis for some patients [8]. Hydrostatic pressure might also be a contributing factor and potentially the reason the S2 nerve root is so commonly involved [9].…”

Pelvic pain from a giant presacral Tarlov cyst successfully obliterated using aneurysm clips in a patient with Marfan syndrome

“…24 Many of the Marfan syndrome pathologies related to the spinal meninges, such as dural ectasia, arachnoid cysts, and meningeal cysts, often do not produce any symptoms; however, radicular, myelopathic, and urinary symptoms from such pathologies have been reported in patients with this syndrome. 1,5,14,17,21,23 In addition, spontaneous spinal CSF leaks are much more commonly observed in patients with Marfan syndrome.…”

“…A direct surgical approach is associated with a high risk of dural tears and osseous complications. 1 The advantage of our approach over other surgical methods is that it is less likely to tear the dura while accomplishing direct obliteration of the connection between the thecal sac and cyst. Other approaches may accomplish the same goal without using an aneurysmal clip, but are more likely to cause dural tearing, especially in patients with Marfan syndrome.…”

Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient

“…The pathology may be asymptomatic,[ 24 ] but can cause postural headaches due to a “cerebrospinal fluid sink” mechanism, as well as back pain, leg pain, or rectal pain. [ 25 21 ] If significant arachnoid cysts are present, the compression of lumbosacral nerve roots may lead to neurological deficits, including cauda equina syndrome (CES).…”