Reply

We thank Drs. Albayram, Yilmaz, and Salvolini for taking an interest in our case report of a patient with Marfan syndrome who presented with symptomatic enlargement of the anterior epidural venous plexus. Drs. Albayram and Yilmaz bring to our attention several other reports that document an association of spontaneous intracranial hypotension and Marfan syndrome. Although we do not have proof that this syndrome was present in our patient, it was among our differential diagnoses, as our article emphasized (p 623). Nonetheless, it is important to consider CSF hypovolemia as a cause of compensatory enlargement of the dural and epidural venous structures, which may appear very prominent on MR images of patients with spontaneous intracranial hypotension. We were unable to elicit a history of postural headache from our patient, and unfortunately, the patient did not undergo CSF pressure analysis; neither, however, rules this possibility out.

Dr. Salvolini also wrote to support the position of CSF hypotension as the probable cause of our patient’s findings. We do not argue this point, and as we have mentioned, we did consider it to be a possible cause. He presents another case of a patient with Marfan syndrome in whom a large sacral meningocele resulted in intracranial findings of spontaneous intracranial hypotension. We thank him for the letter.

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