Clinical and Radiological Effect of Epidural Blood Patch in a Patient with Marfan Syndrome, Chiari I Malformation, Intracranial Hypertension, and Dural Ectasia: Case Report and Systematic Review

Abstract

Background and Purpose— Patients with Marfan syndrome can develop headaches and upright posture
intolerance due to dural ectasia and/or cerebrospinal fluid (CSF) leakage. The role of epidural blood patch in such
patients remains unclear.
Methods— We present a case of a 26 year-old woman with Marfan syndrome, hydrocephalus, sacral dural ectasia,
and tonsillar herniation, presenting with headache and upright posture intolerance. Cerebrospinal fluid pressure
monitoring demonstrated elevated pressure in supine and upright positions with no evidence of a CSF leak on
computed tomography (CT) myelogram. Two epidural injections of autologous blood and fibrin were performed 5
months apart using caudal and lumbar approaches, respectively. A systematic review of the literature was performed
to determine outcomes of epidural blood patch in patients with Marfan syndrome.
Results— After the first epidural blood patch, the patient had marked clinical improvement that persisted for 1
month and partial improvement that persisted for 2 months. After the second epidural blood patch, the patient had
marked clinical improvement that persisted for 2 months and partial improvement that persisted from last follow-up
to 18 months. The tonsillar descent was measured at 10 mm at baseline evaluation and demonstrated reduction in
magnitude of descent after epidural blood patch; 2.3 mm and 3 mm reduction after first and second epidural blood
patches, respectively. A total of 9 patients with Marfan syndrome who were treated a total of twelve epidural blood
patches (nine performed in the lumbar region, two in the thoracic region, and one in the sacral region) were identified.
The follow-up period ranged from 3-months to 18-months post-treatment. Of the seven patients with long-term fol
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low up, complete resolution of symptoms was seen in four and partial resolution of symptoms seen in three patients.
Conclusions— Our report suggests that patients with Chiari malformation and dural ectasia can experience clinical
and radiological improvement even without evidence of intracranial hypotension or CSF leak.