Symptomatic Enlarged Cervical Anterior Epidural Venous Plexus in a Patient with Marfan Syndrome

To the Editor: We read with interest the article by Chun et al (1) in the April 2002 issue of the AJNR. The authors described the symptomatic enlarged cervical anterior epidural venous plexus in a patient with Marfan syndrome. They concluded that Marfan syndrome might predispose the patient to enlargement of cervical anterior epidural venous plexus secondary to a vessel wall abnormality. We would like to take this opportunity to emphasize the following point.

We draw the authors’ attention to the previously published reports related to spontaneous intracranial hypotension from CSF leaks in patients with Marfan syndrome. We found five reports about this topic by conducting a MEDLINE search. However, we cannot cite all of them because of the restriction on the number of references. Schrijver et al (2) worked on 20 consecutive patients and found CSF leaks in all, four (20%) of whom exhibited minor skeletal features of Marfan syndrome. Fukutake et al (3) reported the case of a 30-year-old woman with Marfan syndrome who experienced chronic intractable headaches and spontaneous intracranial hypotension. Myelography showed multiple, large, lumbosacral arachnoid diverticula. Radioisotope cisternography revealed a halo-like accumulation in the lumbosacral region and rapid uptake of isotope in the urinary bladder, indicating CSF leakage. Epidural blood patching brought immediate relief from the headaches. Currently, anterior internal vertebral venous plexus dilation secondary intracranial hypotension is well reported in the neuroradiologic literature (4, 5).

Chun et al concluded that in their case, Marfan syndrome might have predisposed the patient to an enlargement of the anterior epidural venous plexus because of the disorder of the venous connective tissue phenomenon. However, we think that the enlargement of the anterior epidural venous plexus in their case was due to an intracranial hypotension syndrome. The patient’s clinical history also supports our hypothesis.

• Copyright © American Society of Neuroradiology