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Washington University Experience | VASCULAR | Vasculitis - Giant Cell Arteritis | 1A0 Case 1 History
Case 1 History ---- The patient was a 5 year old previously healthy male who presented to SLCH ER with a one week history of emesis following most meals and fevers of around 99°F. The cause was clinically suspected to be an enterovirus and his symptoms resolved 3 days prior to admission. On the morning of admission the child's parents were awakened by the child's screaming, finding he had fallen out of his bed and was complaining of a very severe headache. His bed is approximately 3 feet above a carpeted floor and his parents didn't notice any evidence of trauma and were able to console him and he went back to sleep. Approximately 5 minutes later he awakened again screaming and incoherent and was brought to the SLCH ER. He was unresponsive in the ER and had upper extremity rhythmic jerking following which he became limp and totally unresponsive. His right pupil was dilated and unresponsive to light. The patient was intubated and taken to CT which showed subarachnoid hemorrhage with blood in the ventricles; a ventriculostomy was placed. The patient was transferred to the ICU where he was unresponsive to vocal stimuli and was only responsive to noxious stimuli, withdrawing to pain but not showing evidence of localization. Neither pupil was responsive to light. The right fundi displayed massive papilledema, venous engorgement and retinal hemorrhage. The patient was placed on mannitol, Decadron and mechanical hyperventilation to control intracranial pressure. Dilantin was also started for seizure control. The patient was taken for angiography which showed a large basilar aneurysm. Immediately following angiography the patient was noted to have gradually decreasing skin perfusion. Labs revealed he was acidotic with a pH of 7.2. A heart monitor detected no electrical activity and heart sounds were absent. A code was called and heart rate and perfusion improved with therapy but the patient's right and left pupils were fixed and dilated and he was not arousable with loss of all brainstem reflexes. Head CT at this time showed a massive right cerebral infarction with no cisterns. A second ventriculostomy was placed on the left side at this time. The patient's parents were appraised of the grim prognosis and they wished to have their child placed on DNR status and desired organ donation upon brain death. The patient's heart, lung, liver and bilateral kidneys were retrieved for donation. ---- At autopsy a subarachnoid hematoma was present over the base of the brain and involved the left and right temporal and parietal lobes. A basilar artery aneurysm, 2 cm in maximal dimension was focally ruptured near the origin of the right posterior cerebral artery. Microscopic examination showed giant cell arteritis of the basilar artery with secondary aneurysmal dilatation. A large acute infarct involved the distribution of the right middle cerebral artery with small hemorrhagic lesions in the right basal ganglia, right thalamus and left anterior temporal lobe. Transverse sections through the brainstem showed grossly normal brainstem configuration and no focal lesions.
