Marfan syndrome and symptomatic sacral cyst: report of two cases.
BACKGROUND
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.


METHODS
Case reports.


RESULTS
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.


CONCLUSIONS
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.
University of Kansas Medical Center, Kansas City, KS, USA. parnold@kumc.edu
Paul M
PM
Arnold
Jan
J
Teuber
D000328:Adult; D020863:Central Nervous System Cysts / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D019299:Decompression, Surgical; D006801:Humans; D007796:Laminectomy; D017116:Low Back Pain / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D012447:Sacrum / Q000000981:diagnostic imaging* / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome