Recurrent pneumoencephalus associated with transclival cerebrospinal fluid fistula in a patient with Marfan syndrome

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  • 気脳症を反復した Marfan 症候群
  • 症例報告 気脳症を反復したMarfan症候群
  • ショウレイ ホウコク キノウショウ オ ハンプク シタ Marfan ショウコウグン

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Abstract

<p>　Marfan syndrome is a dominantly inherited multi-system disorder of the connective tissue. Herein, we report a patient with Marfan syndrome who presented with recurrent pneumoencephalus.</p><p>　A 31-year-old female patient visited our emergency room with severe headache, and brain computed tomography revealed the presence of pneumoencephalus. Magnetic resonance imaging revealed the presence of a meningocele posterior to the sphenoid sinus due to a bone wall defect. She was admitted to the neurosurgery department and treated conservatively by intravenous administration of antimicrobial agents. The treatment was effective and the patient was discharged. Four years later, she presented with severe headache, as before, was admitted, and was again successfully treated conservatively. 111In-DTPA cisternography revealed transclival cerebrospinal fluid rhinorrhea. On day 17 of the second admission, she was discharged without any neurological sequelae. However, 3 days after the second hospital discharge, she presented again with the same headache and was admitted. This time, endonasal endoscopic surgery was performed via a transsphenoidal approach, and no pneumocephalus or cerebrospinal fluid rhinorrhea was observed for at least 18 months after this operation. In this patient, it is presumed that the bone wall defect associated with Marfan syndrome and excessive pneumatization of the sphenoid sinus had led to weakness and thinning of the cranial bone, and that continuous pressure pulses of cerebrospinal fluid to the skull base caused a bony defect of the clivus and weakness of the dura mater. Thus, presumably, fragile meningeal diverticula developed, leading to recurrent spontaneous cerebrospinal fluid leakage and pneumoencephalus.</p>

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