[Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report].
Spontaneous intracranial hypotension is a rare complication of connective tissue disorders. One of them is Marfan syndrome. It predisposes the patient to meningeal diverticula. Possibly after minor unrecognised head trauma or secondary to Valsalva manoeuvre cerebrospinal fluid leak from meningeal diverticula can happen. It causes postural headache. We describe a 15 year old adolescent female newly diagnosed with Marfan syndrome, who presented with intractable postural headache. Our patient's brain MRI showed bilateral frontal subdural effusion, narrow ventricles, downward displacement of the brainstem, enlarged cervical venous plexi, dural ectasias and wide nerve root sleeves. Radionuclide cisternography demonstrated CSF leaks at multiple sites. She could not be treated conservatively, but was successfully treated by epidural saline injections. Control brain and cervical MRI confirmed her healing, too. At the two and half year follow up visit, she was completely well. So we recommend this easy technique to be used before epidural autologous blood patches.
Rosdy
Beáta
B
Fovárosi Onkormányzat Heim Pá1 Gyermekkórháza, Neurológia Osztá1y, Budapest. rosbe@heimpalkorhaz.hu
Kollár
Katalin
K
Móser
Judit
J
Várallyay
György
G
Kordas
Mariann
M
D000293:Adolescent; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D007268:Injections, Epidural; D019585:Intracranial Hypotension / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D007552:Isotonic Solutions / Q000008:administration & dosage; D008279:Magnetic Resonance Imaging; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D012965:Sodium Chloride / Q000008:administration & dosage; D013353:Subdural Effusion / Q000150:complications / Q000209:etiology*
D007552:Isotonic Solutions; D012965:Sodium Chloride