A 23-year-old critically-ill male with history of Burkitt's lymphoma, with graft-versus-host disease (GVHD) requiring high-dose corticosteroids, was admitted to the Emergency Department following a 2 days history of somnolence and confusion with evolving respiratory distress requiring endotracheal intubation. On admission neurological examination, he would open eyes to painful stimuli and continued to follow commands.
Initial CT head revealed a flame hemorrhage in the left gyrus rectus suspicious for vascular origin and a hypodensity in the left thalamus suspicious for an established infarction. An angiogram performed the next day demonstrated six fusiform aneurysms: three at the frontopolar branch and one at the orbitofrontal branch of the left anterior cerebral artery (ACA); 1 at the frontopolar branch of the right ACA; and one at the anterior temporal artery of the left middle cerebral artery (MCA); [Figure 1]. Body imaging revealed cavitary lung lesions. The patient was started on broad spectrum antibiotics, antifungals, and antiviral medications.
The location of hemorrhage pointed to the left ACA branch aneurysms as the source. Surgery was chosen to allow for inspection of all lesions under direct vision for ruptured status as well as obtain tissue for microbiological purposes. A bicoronal craniotomy, parent vessel sacrifice, and excision of the three aneurysms arising from the left ACA were performed on postbleed day 2. Intraoperatively, the aneurysms were friable, thin walled, and prone to bleeding. The right ACA aneurysms were managed conservatively. Microbiology confirmed angioinvasive S. apiospermum infection. Vericonazole, amphotericin B, and caspofunging were started empirically and continued throughout the disease process.
CT scan obtained due to neurological deterioration on postbleed day 4 revealed new left frontal intracerebral hemorrhage and intraventricular hemorrhage (IVH). An emergency external ventricular drain was placed and a follow-up angiogram was obtained revealing interval growth of the left orbitofrontal artery aneurysm. Since microbiological diagnosis was available, endovascular parent vessel occlusion was recommended and preceded uneventfully. An interval angiogram on postbleed day 7 demonstrated three new fusiform aneurysms of the right ACA, a new aneurysm of the left anterior choroidal artery (AChA), and the left posterior communicating artery (PCOMA) and confirmed a stable appearing left anterior temporal artery aneurysm. Due to the rapid development of new aneurysms, as well as the hemorrhagic presentation, the treatment was recommended. A3-A3 revascularization was attempted to avoid ACA sacrifice; however, due to the friable nature of the diseased vessels, this was unsuccessful and parent vessel sacrifice had to be performed. An intraoperative angiogram demonstrated a new anterior communicating artery (ACOMA) aneurysm, which was left untreated at the time of surgery. On further follow-up imaging, the ACOMA aneurysm continued to enlarge and, therefore, was treated with endovascular A1 occlusions on postbleed day 9. Finally, continued surveillance imaging demonstrated interval growth of the previously identified left AChA and PCOMA aneurysm, which were ultimately treated with parent vessel sacrifice.
Despite multiple parent vessel sacrifice, the patient recovered neurologically, continued to follow commands with upper extremities with expected, severe paraparesis in the lower extremities. His follow-up MRI confirmed a thalamic infarct which was already seen on admission imaging and new, incomplete ACA territory infarctions in keeping with the previous surgeries and parent vessel sacrifice of the bilateral ACAs. Due the severe systemic and pulmonary disease, the patient remained intubated and ventilated and underwent a tracheostomy procedure. His subsequent course was complicated with septic shock from multidrug resistant pseudomonas superinfection of the cavitary lung lesions and the patient died 6 months after the hemorrhagic presentation.