Ruptured Mycotic Aneurysm of the Distal Circulation in a Patient with Mucormycosis Without Direct Skull Base Extension: Case Report

Ruptured Mycotic Aneurysm of the Distal Circulation in a Patient with Mucormycosis Without Direct... Abstract BACKGROUND AND IMPORTANCE Infectious intracranial aneurysms are a rare subset of intracranial aneurysms caused by bacterial, fungal, or viral sources. Intracranial aneurysms of fungal etiology carry a high mortality risk and typically occur in immunocompromised patients via direct extension of skull base infections, or more rarely, after intracranial surgery. CLINICAL PRESENTATION We present the case of a 27-yr-old female with systemic lupus erythematous and primary pulmonary mucormycosis, who suffered a subarachnoid hemorrhage from a ruptured fusiform distal middle cerebral artery aneurysm. Despite undergoing a successful extracranial-to-intracranial bypass and aneurysm excision, the patient ultimately died following progressive disseminated infection and a secondary intracranial hemorrhage of unknown etiology. Pathological examination of the excised artery confirmed Mucor infection. CONCLUSION To the best of our knowledge, this case represents one of the first mycotic cerebral aneurysms from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. Despite optimal surgical management, clinical outcomes for mycotic cerebral aneurysms are largely dependent on the success of medical therapies at controlling systemic disease. Mucormycosis, Revascularization, Extracranial-to-intracranial bypass, Graft patency, Mycotic Aneurysm ABBREVIATIONS ABBREVIATIONS CNS central nervous system CMV cytomegalovirus CT computed tomography CTA CT angiogram EC-IC extracranial-to-intracranial GMS Gomori–Metheneamine silver H&E Hematoxylin and eosin IIAs infectious intracranial aneurysms IPH intraparenchymal haemorrhage MCA middle cerebral artery STA superficial temporal artery Mycotic or infectious intracranial aneurysms (IIAs) account for less than 6% of all intracranial aneurysms.1,2 IIAs are characterized by a bacterial, fungal, or viral invasion of cerebral vessel walls, which causes a local inflammatory response and subsequent vessel dilatation and aneurysm formation.2,3 For bacterial IIAs, there is commonly an underlying intravascular pathology, such as infective endocarditis that leads to aneurysm formation in the distal cerebral vasculature.2,4 “True” mycotic aneurysms from fungal infections are rare, and tend to occur in the proximal vasculature following direct arterial invasion from an extravascular cranial infection.5 Although typical IIA management requires antimicrobial therapy against the causative organism, in the event of aneurysm rupture, neurosurgical intervention is required. We present a unique case of a 27-yr-old female with a primary pulmonary mucormycosis who developed a ruptured mycotic intracranial aneurysm of the distal middle cerebral artery (MCA) that was treated with excision and distal revascularization with an extracranial-to-intracranial (EC-IC) bypass. To the best of our knowledge, this is one of the first reported cases of a “true” mycotic cerebral aneurysm from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. CLINICAL PRESENTATION A 27-yr-old female with systemic lupus erythematous treated with Cyclophosphamide and steroids was transferred to our institution after progressively worsening shortness of breath, anemia, and fatigue. Initial workup demonstrated a necrotic abscess of the right lower lobe, managed by chest tube drainage, broad-spectrum antibiotic, and antifungal therapy. She was stabilized with conservative management, however, prior to discharge developed severe headaches and blurred vision. Her neurological exam was lethargic but arousable and able to intermittently follow simple commands at time of neurosurgical consult. Head computed tomography (CT) and CT angiogram (CTA) identified an intraparenchymal hemorrhage (IPH) of the left temporoparietal lobe measuring 2.6 × 2.6 × 3.7 cm, with an underlying multilobulated aneurysm of the distal left MCA suspicious for a fusiform mycotic aneurysm (Figure 1). A second smaller left temporo-occipital IPH did not have an identifiable associated vascular lesion. Blood cultures and a transthoracic echocardiogram were unrevealing. Given the patient's tenuous clinical status and adequacy of cerebral vessel imaging with CTA, a formal angiogram was foregone and the patient was taken to the operating room for open microsurgical aneurysm excision and revascularization. FIGURE 1. View largeDownload slide Preoperative CTA demonstrating a left temporoparietal intraparenchymal hemorrhage with an associated multi-lobulated aneurysm of the distal MCA in a 27-year-old female with primary pulmonary mucormycosis with new neurologic deficits. A, Axial and B, coronal images. C, Three-dimensional reconstructions of the aneurysm, demonstrating its circumferential arterial involvement. FIGURE 1. View largeDownload slide Preoperative CTA demonstrating a left temporoparietal intraparenchymal hemorrhage with an associated multi-lobulated aneurysm of the distal MCA in a 27-year-old female with primary pulmonary mucormycosis with new neurologic deficits. A, Axial and B, coronal images. C, Three-dimensional reconstructions of the aneurysm, demonstrating its circumferential arterial involvement. All patients undergoing this technique consented as per university protocol. Institutional Review Board approval was not needed for the single patient data included in this report. Operation The superficial temporal artery (STA) was mapped and dissected (Figure 2A), prior to performing a craniotomy over the IPH/aneurysm. The involved MCA branch was circumferentially dissected, and an end-to-side microvascular anastomosis of the STA to the MCA distal to the lesion was completeds (Figure 2B and 2C). Intraoperative indocyanine green imaging demonstrated anastomosis patency. The aneurysm and all grossly diseased segments of the parent artery were excised and sent for pathological examination. The IPH was then evacuated before the surgical cavity was closed in a layered fashion. FIGURE 2. View largeDownload slide STA-MCA bypass and excision of distal MCA mycotic aneurysm. A, The STA was dissected prior to performing a craniotomy over the left temporal IPH/aneurysm. B, The affected MCA branch was circumferentially dissected in preparation for clipping/excision, and C, an end-to-side STA-MCA anastomosis to an uninvolved artery segment distal to the lesion was performed (diseased MCA segment marked with a black arrow in panels B and C). The aneurysm and all diseased MCA segments were then excised (not shown). FIGURE 2. View largeDownload slide STA-MCA bypass and excision of distal MCA mycotic aneurysm. A, The STA was dissected prior to performing a craniotomy over the left temporal IPH/aneurysm. B, The affected MCA branch was circumferentially dissected in preparation for clipping/excision, and C, an end-to-side STA-MCA anastomosis to an uninvolved artery segment distal to the lesion was performed (diseased MCA segment marked with a black arrow in panels B and C). The aneurysm and all diseased MCA segments were then excised (not shown). FIGURE 3. View largeDownload slide Histologic analysis of resected aneurysm. A, H&E staining demonstrated inflamed tissue with fibrin deposition (40× magnification). B, 40× and C, 400× magnification of GMS silver stain for fungal organisms, demonstrating numerous broad, degenerate appearing hyphae, consistent with mucormycosis. D, Immunohistochemical staining also identified rare CMV positive cytomegalic cells (400× magnification; black arrow). FIGURE 3. View largeDownload slide Histologic analysis of resected aneurysm. A, H&E staining demonstrated inflamed tissue with fibrin deposition (40× magnification). B, 40× and C, 400× magnification of GMS silver stain for fungal organisms, demonstrating numerous broad, degenerate appearing hyphae, consistent with mucormycosis. D, Immunohistochemical staining also identified rare CMV positive cytomegalic cells (400× magnification; black arrow). Histopathology Aneurysm histopathology showed inflamed tissue with fibrin deposition on Hematoxylin and eosin (H&E) staining (Figure 3A). Gomori–Metheneamine silver (GMS) stain for fungal organisms demonstrated numerous broad, degenerate appearing hyphae, consistent with mucormycosis (zygomycosis; Figure 3B and 3C). In addition, immunohistochemical staining highlighted rare Cytomegalovirus (CMV) positive cells (Figure 3D). Postoperative Course Postoperatively, the patient improved neurologically to being alert and oriented, and following commands with full strength in all extremities, with her only deficit being mild aphasia. CTA demonstrated a patent anastomosis and IPH decompression (Figure 4). Endocarditis as a source of septic emboli was excluded with a negative transesophageal echocardiogram. As per infectious diseases, the patient was treated with long-term amphotericin B for disseminated mucormycosis, and was discharged home neurologically stable during her second postoperative week. She nonetheless required multiple readmissions for pulmonary complications and overwhelming infectious burden. One month postoperatively, she was admitted obtunded with a large left-sided IPH involving the deep white matter without an associated vascular lesion on CTA (Figure 5). Her neurological exam was consistent with a brain death at this time, and the patient's family withdrew care. Of note, the patient was consented for all described care as per university protocol. Institutional Review Board approval was not needed for the single patient data included in this report. FIGURE 4. View largeDownload slide Postoperative CTA. A, Axial view demonstrating adequate clot evacuation. B, Coronal view with visualization of stable perfusion distal to the bypass graft. FIGURE 4. View largeDownload slide Postoperative CTA. A, Axial view demonstrating adequate clot evacuation. B, Coronal view with visualization of stable perfusion distal to the bypass graft. FIGURE 5. View largeDownload slide Head CT upon re-presentation in obtunded state. Despite a well-tolerated aneurysm resection and bypass, the patient had progressive disseminated infection and presented 1 mo postoperatively with a large left-sided intraparenchymal hemorrhage involving the deep white matter of unknown etiology (axial noncontrast scan shown). CTA demonstrated decreased perfusion adjacent to the hemorrhage, but no underlying vascular lesion (not shown). The patient's neurological exam was consistent with brain death, and the decision to withdraw care was made by the patient's family. FIGURE 5. View largeDownload slide Head CT upon re-presentation in obtunded state. Despite a well-tolerated aneurysm resection and bypass, the patient had progressive disseminated infection and presented 1 mo postoperatively with a large left-sided intraparenchymal hemorrhage involving the deep white matter of unknown etiology (axial noncontrast scan shown). CTA demonstrated decreased perfusion adjacent to the hemorrhage, but no underlying vascular lesion (not shown). The patient's neurological exam was consistent with brain death, and the decision to withdraw care was made by the patient's family. DISCUSSION Mucormycosis infections are a rare pathology with high mortality in immunocompromised patients, even if diagnosed and treated early.6,7 We present the unique case of an IIA from disseminated mucormycosis from a primary pulmonary source, without evidence of associated skull base involvement. Candida species, Aspergillus species, Pseudallerscheria boydii, and Coccidioides are the most common causative pathogens of fungal IIAs,8 which typically occur as opportunistic infections in immunocompromised patients and/or following prolonged steroid use.2,3,5 Mechanistically, fungal IIAs are thought to destroy the elastic lamina of the arterial wall, resulting in a decreased resistance to intra-arterial pressures and aneurysm formation.9 While fungal IIAs are most often the result of extravascular spread from skull base infections,9–14 IIAs associated with intracranial abscesses, following intracranial surgery, or from hematogenous seeding have also been reported with Candida and Aspergillus species, and Pseudallerscheria boydii.15–19 Although published data are limited, rupture of fungal IIAs is associated with nearly 100% mortality.9,10,12–15,20 IIAs from Mucor species are a rare subset of fungal aneurysms that to date have almost uniformly been described in association with skull base infections.5,21–24 Although a significant number of disseminated mucormycosis cases involving the central nervous system (CNS) have been reported,7,18,25–27 the manifestations of these infections are typically meningitis, abscesses, or infarctions secondary to vasculitis, rather than aneurysms. Upon our review of the literature, only 2 other cases of Mucor IIAs without an associated skull base infection have been reported.20,28 In the first case, a 61-yr-old man on steroids following clipping of a ruptured anterior communicating artery aneurysm died after a hemorrhagic cerebral infarction.20 On autopsy, 4 aneurysms of the pericollosal artery were noted, with Mucor infiltration seen on vessel wall histology. In the second case, a 4-yr-old female on a prolonged steroid course for a lung infection of unknown etiology presented with focal seizures.28 Workup was concerning for subarachnoid hemorrhage, and the patient died several hours after presentation. On autopsy, she was found to have a ruptured left anterior cerebral artery aneurysm with Mucor infiltration of the aneurysm wall on histology. While no primary source of Mucor was identified in either of these cases, steroid use (and the previous craniotomy in the first case) were identified as risk factors for fungal IIA development. A summary of all previously reported IIAs from Mucor species is provided in Table. With this background, we believe the present case represents the second report of hematogenous seeding of mucormycosis resulting in IIA formation in the distal cerebral vasculature, in the absence of a contiguous skull base infection or previous intracranial surgery. TABLE. Previously Reported Infectious Intracranial Aneurysms From Mucor Species Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Abbreviations: ACA, anterior cerebral artery; Acomm, anterior communicating artery; DM, diabetes mellitus; ICA, internal carotid artery; SAH, subarachnoid hemorrhage; yr, year View Large TABLE. Previously Reported Infectious Intracranial Aneurysms From Mucor Species Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Abbreviations: ACA, anterior cerebral artery; Acomm, anterior communicating artery; DM, diabetes mellitus; ICA, internal carotid artery; SAH, subarachnoid hemorrhage; yr, year View Large While the patient's immunocompromised state from systemic lupus erythematous and Cyclophosphamide/steroid treatments almost certainly contributed to the development of her initial mucormycosis infection, the concomitant presence of Mucor and CMV within the aneurysm wall represents an additional unique component of this case. Although CMV has not been previously associated with IIA or noninfectious cerebral aneurysm formation, other viruses, such as the human immunodeficiency virus and varicella–zoster virus, have been linked to IIAs.8,29 CMV has also been reported as a cause of CNS vasculitis that results from an inflammatory response to viral invasion of the vascular wall.30 The dual inflammatory response to CMV and Mucor in this case may have thus synergistically increased arterial wall vulnerability to aneurysm formation. Despite the high mortality of fungal IIAs, aggressive surgical management was pursued due to the patient's young age and relatively high neurological function upon neurosurgery evaluation. Although conservative management of nonruptured IIAs can cause aneurysm resolution or decrease rupture risk,2,31 the ruptured status of the patient's aneurysm required treatment.1,32,33 The distal location and fusiform morphology with downstream eloquent cortex nonetheless limited endovascular and nonreconstructive surgical options. We thus chose to excise the aneurysm and all grossly involved vessel segments after an EC-IC bypass for distal revascularization. Even with successful aneurysm treatment and initial neurological improvement, the patient's prognosis worsened as a result of her progressive infectious burden, and she ultimately died from a secondary intracranial hemorrhage of unknown etiology. Although CTA at this time was negative for vascular disease, there was poor visualization of the distal MCA vessels on the affected side due to presumed mass effect from the hemorrhage. Progression of microscopically diseased arteries proximal to the resected segment (or even within the bypass graft itself following hematogenous seeding) is thus a possible hemorrhagic source. Hemorrhagic conversion of a large ischemic event, the latter described with cerebral mucormycosis from both skull base and disseminated infections,20,22,27 is another possible explanation for this secondary bleed. This case highlights the challenges of treating fungal IIAs, and serves as a reminder of the potential for abnormal presentations of infectious cerebrovascular pathologies. Important technical considerations when performing aneurysm and bypass surgery for IIAs include the need for meticulous microsurgical dissections due to the increased friability of the cerebrum and involved vasculature. Choosing a healthy recipient vessel site is also critical to the technical success of the bypass. CONCLUSION While typically associated with skull base lesions, IIAs from mucormycosis can form following hematogenous seeding from disseminated infections. Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article. REFERENCES 1. Ding D , Bok AP . Acute subdural hematoma from a ruptured aneurysm of the distal middle cerebral artery . J Neurosci Rural Pract . 2017 ; 8 ( 1 ): 152 - 154 . Google Scholar CrossRef Search ADS PubMed 2. Winn HR , Mayer SA , Bederson JB . Infectious intracranial aneurysms . In: Winn HR , ed. Youmans Neurological Surgery . 6th ed . Philadelphia, PA : Saunders , 2011 ; 3972 - 3977 . 3. Morris NA , Matiello M , Lyons JL , Samuels MA . Neurologic complications in infective endocarditis . Neurohospitalist . 2014 ; 4 ( 4 ): 213 - 222 . Google Scholar CrossRef Search ADS PubMed 4. Ducruet AF , Hickman ZL , Zacharia BE et al. 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Iihara K , Makita Y , Nabeshima S , Tei T , Keyaki A , Nioka H . Aspergillosis of the central nervous system causing subarachnoid hemorrhage from mycotic aneurysm of the basilar artery . Neurol Med Chir (Tokyo) . 1990 ; 30 ( 8 ): 618 - 623 . Google Scholar CrossRef Search ADS PubMed 14. Watson JC , Myseros JS , Bullock MR . True fungal mycotic aneurysm of the basilar artery: a clinical and surgical dilemma . Cerebrovasc Dis . 1999 ; 9 ( 1 ): 50 - 53 . Google Scholar CrossRef Search ADS PubMed 15. Messori A , Lanza C , De Nicola M et al. Mycotic aneurysms as lethal complication of brain pseudallescheriasis in a near-drowned child: a CT demonstration . AJNR Am J Neuroradiol . 2002 ; 23 ( 10 ): 1697 - 1699 . Google Scholar PubMed 16. Piotrowski WP , Pilz P , Chuang IH . Subarachnoid hemorrhage caused by a fungal aneurysm of the vertebral artery as a complication of intracranial aneurysm clipping. Case report . J Neurosurg . 1990 ; 73 ( 6 ): 962 - 964 . Google Scholar CrossRef Search ADS PubMed 17. Visudhiphan P , Bunyaratavej S , Khantanaphar S . Cerebral aspergillosis. Report of three cases . J Neurosurg . 1973 ; 38 ( 4 ): 472 - 476 . Google Scholar CrossRef Search ADS PubMed 18. Gavito-Higuera J , Mullins CB , Ramos-Duran L , Olivas Chacon CI , Hakim N , Palacios E . Fungal infections of the central nervous system: a pictorial review . J Clin Imaging Sci . 2016 ; 6 1 : 24 . Google Scholar CrossRef Search ADS PubMed 19. Ho CL , Deruytter MJ . CNS aspergillosis with mycotic aneurysm, cerebral granuloma and infarction . Acta Neurochir . 2004 ; 146 ( 8 ): 851 - 856 . Google Scholar CrossRef Search ADS PubMed 20. Kikuchi K , Watanabe K , Sugawara A , Kowada M . Multiple fungal aneurysms: report of a rare case implicating steroid as predisposing factor . Surg Neurol . 1985 ; 24 ( 3 ): 253 - 259 . Google Scholar CrossRef Search ADS PubMed 21. Ho KL . Acute subdural hematoma and intracerebral hemorrhage. Rare complications of rhinocerebral mucormycosis . Arch Otolaryngol . 1979 ; 105 ( 5 ): 279 - 281 . Google Scholar CrossRef Search ADS PubMed 22. Thajeb P , Thajeb T , Dai D . Fatal strokes in patients with rhino-orbito-cerebral mucormycosis and associated vasculopathy . Scand J Infect Dis . 2004 ; 36 ( 9 ): 643 - 648 . Google Scholar CrossRef Search ADS PubMed 23. Alvernia JE , Patel RN , Cai DZ , Dang N , Anderson DW , Melgar M . A successful combined endovascular and surgical treatment of a cranial base mucormycosis with an associated internal carotid artery pseudoaneurysm . Neurosurgery . 2009 ; 65 ( 4 ): 733 - 740 ; discussion 740 . Google Scholar CrossRef Search ADS PubMed 24. Price DL , Wolpow ER , Richardson EP . Intracranial phycomycosis: a clinicopathological and radiological study . J Neurol Sci . 1971 ; 14 ( 3 ): 359 - 375 . Google Scholar CrossRef Search ADS PubMed 25. Ma J , Jia R , Li J et al. Retrospective clinical study of Eighty-One cases of intracranial mucormycosis . J Glob Infect Dis . 2015 ; 7 ( 4 ): 143 - 150 . Google Scholar CrossRef Search ADS PubMed 26. Royer M , Cervera P , Kahan A , Menkès CJ , Puéchal X . Mucormycosis cerebral arteritis mimicking a flare in ANCA-associated vasculitis . Lancet Infect Dis . 2013 ; 13 ( 2 ): 182 . Google Scholar CrossRef Search ADS PubMed 27. Ermak D , Kanekar S , Specht CS , Wojnar M , Lowden M . Looks like a stroke, acts like a stroke, but it's more than a stroke: a case of cerebral mucormycosis . J Stroke Cerebrovasc Dis . 2014 ; 23 ( 8 ): e403 - e404 . Google Scholar CrossRef Search ADS PubMed 28. Glass EC , Stadalnik RC , Barnett CA . Ventricular visualization on brain scan with intracranial hemorrhage in disseminated phycomycosis . Clin Nucl Med . 1978 ; 3 ( 11 ): 429 - 431 . Google Scholar CrossRef Search ADS PubMed 29. Ake JA , Erickson JC , Lowry KJ . Cerebral aneurysmal arteriopathy associated with HIV infection in an adult . Clin Infect Dis . 2006 ; 43 ( 5 ): e46 - e50 . Google Scholar CrossRef Search ADS PubMed 30. Koeppen AH , Lansing LS , Peng SK , Smith RS . Central nervous system vasculitis in cytomegalovirus infection . J Neurol Sci . 1981 ; 51 ( 3 ): 395 - 410 . Google Scholar CrossRef Search ADS PubMed 31. Molinari GF , Smith L , Goldstein MN , Satran R . Pathogenesis of cerebral mycotic aneurysms . Neurology . 1973 ; 23 ( 4 ): 325 - 332 . Google Scholar CrossRef Search ADS PubMed 32. Neto S , Flores JC , Figueiredo EG , Caldas JG , Teixeira MJ . Mycotic aneurysm treated with aneurysm trapping. Case report . J Neurol Surg Rep . 2016 ; 77 ( 1 ): e013 - e016 . Google Scholar PubMed 33. Singla V , Sharma R , Nagamani AC , Manjunath CN . Mycotic aneurysm: a rare and dreaded complication of infective endocarditis . BMJ Case Rep . 2013 ; 2013 : bcr2013200016 . Google Scholar PubMed COMMENTS In this report, the authors presented a case of a 27-year-old female with systemic lupus erythematosus and primary pulmonary mucormycosis who suffered from an intraparenchymal hemorrhage due to a ruptured fusiform distal MCA aneurysm. Pathologic examination of the excised artery confirmed Mucor infection. the authors believe that this case represents the first mycotic cerebral aneurysm from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. This case underscores the prognosis of ruptured infectious aneurysms in general and “true” mycotic aneurysms in particular, which is poor. Managing infectious aneurysms is one of the challenges in neurosurgery that is better approached in a multidisciplinary way. As this patent was demed unstable to do cerebral aniography, we remind the physicians managing these patient about its importance and advantages. Cerebral angiography will not only show the characteristics of the aneurysm, the status of the artery harboring the aneurysm, presence of other small aneurysms that may be missed by CTA, it will most importantly assess if this affected artery is amenable for endovascular treatment (especially if there is no mass effect) as it becomes the primary choice in the many cases due to its versatility and the ability to deliver both reconstructive and deconstructive treatments to these tenacious lesions. Abdulrahman Y. Alturki Christopher S. Ogilvy Boston, Massachusetts The authors present a rather unique case report describing the many technical challenges faced when performing bypass surgery on a patient with a ruptured mycotic aneurysm. The authors report the surgical treatment of a 27-year-old woman with systemic lupus erythematous and pulmonary mucomycosis presenting with subarachnoid hemorrhage from a distal middle cerebral artery mycotic aneurysm. The authors performed a technically successful superficial temporal artery to middle cerebral artery bypass, successfully excising the infected vessel segment and achieving a successful patent bypass. The concomitant cytomegalovirus infection may have contributed to the ultimate rehemorrhage and demise of this patient though it will remain unclear. Though a difficult case presentation with an unfortunate outcome, the authors present a very well-described report that present to the reader important nuances in the management of patients with mycotic aneurysms. As with every bypass operation, we are reminded of the need for meticulous preparation of the tissue and a most-careful inspection of the donor and recipient artery in preparation for the bypass. In particular, vessels harboring mycotic aneurysms must be bypassed in locations where the tissue is at the very least macroscopically normal. Additional follow-up and image monitoring might also be required to determine interval recurrence of infectious lesions, especially in the setting of on-going infection and seeding. Charles J. Prestigiacomo Cincinnati, Ohio Copyright © 2018 by the Congress of Neurological Surgeons This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Operative Neurosurgery Oxford University Press

Ruptured Mycotic Aneurysm of the Distal Circulation in a Patient with Mucormycosis Without Direct Skull Base Extension: Case Report

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Copyright © 2018 by the Congress of Neurological Surgeons
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Abstract

Abstract BACKGROUND AND IMPORTANCE Infectious intracranial aneurysms are a rare subset of intracranial aneurysms caused by bacterial, fungal, or viral sources. Intracranial aneurysms of fungal etiology carry a high mortality risk and typically occur in immunocompromised patients via direct extension of skull base infections, or more rarely, after intracranial surgery. CLINICAL PRESENTATION We present the case of a 27-yr-old female with systemic lupus erythematous and primary pulmonary mucormycosis, who suffered a subarachnoid hemorrhage from a ruptured fusiform distal middle cerebral artery aneurysm. Despite undergoing a successful extracranial-to-intracranial bypass and aneurysm excision, the patient ultimately died following progressive disseminated infection and a secondary intracranial hemorrhage of unknown etiology. Pathological examination of the excised artery confirmed Mucor infection. CONCLUSION To the best of our knowledge, this case represents one of the first mycotic cerebral aneurysms from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. Despite optimal surgical management, clinical outcomes for mycotic cerebral aneurysms are largely dependent on the success of medical therapies at controlling systemic disease. Mucormycosis, Revascularization, Extracranial-to-intracranial bypass, Graft patency, Mycotic Aneurysm ABBREVIATIONS ABBREVIATIONS CNS central nervous system CMV cytomegalovirus CT computed tomography CTA CT angiogram EC-IC extracranial-to-intracranial GMS Gomori–Metheneamine silver H&E Hematoxylin and eosin IIAs infectious intracranial aneurysms IPH intraparenchymal haemorrhage MCA middle cerebral artery STA superficial temporal artery Mycotic or infectious intracranial aneurysms (IIAs) account for less than 6% of all intracranial aneurysms.1,2 IIAs are characterized by a bacterial, fungal, or viral invasion of cerebral vessel walls, which causes a local inflammatory response and subsequent vessel dilatation and aneurysm formation.2,3 For bacterial IIAs, there is commonly an underlying intravascular pathology, such as infective endocarditis that leads to aneurysm formation in the distal cerebral vasculature.2,4 “True” mycotic aneurysms from fungal infections are rare, and tend to occur in the proximal vasculature following direct arterial invasion from an extravascular cranial infection.5 Although typical IIA management requires antimicrobial therapy against the causative organism, in the event of aneurysm rupture, neurosurgical intervention is required. We present a unique case of a 27-yr-old female with a primary pulmonary mucormycosis who developed a ruptured mycotic intracranial aneurysm of the distal middle cerebral artery (MCA) that was treated with excision and distal revascularization with an extracranial-to-intracranial (EC-IC) bypass. To the best of our knowledge, this is one of the first reported cases of a “true” mycotic cerebral aneurysm from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. CLINICAL PRESENTATION A 27-yr-old female with systemic lupus erythematous treated with Cyclophosphamide and steroids was transferred to our institution after progressively worsening shortness of breath, anemia, and fatigue. Initial workup demonstrated a necrotic abscess of the right lower lobe, managed by chest tube drainage, broad-spectrum antibiotic, and antifungal therapy. She was stabilized with conservative management, however, prior to discharge developed severe headaches and blurred vision. Her neurological exam was lethargic but arousable and able to intermittently follow simple commands at time of neurosurgical consult. Head computed tomography (CT) and CT angiogram (CTA) identified an intraparenchymal hemorrhage (IPH) of the left temporoparietal lobe measuring 2.6 × 2.6 × 3.7 cm, with an underlying multilobulated aneurysm of the distal left MCA suspicious for a fusiform mycotic aneurysm (Figure 1). A second smaller left temporo-occipital IPH did not have an identifiable associated vascular lesion. Blood cultures and a transthoracic echocardiogram were unrevealing. Given the patient's tenuous clinical status and adequacy of cerebral vessel imaging with CTA, a formal angiogram was foregone and the patient was taken to the operating room for open microsurgical aneurysm excision and revascularization. FIGURE 1. View largeDownload slide Preoperative CTA demonstrating a left temporoparietal intraparenchymal hemorrhage with an associated multi-lobulated aneurysm of the distal MCA in a 27-year-old female with primary pulmonary mucormycosis with new neurologic deficits. A, Axial and B, coronal images. C, Three-dimensional reconstructions of the aneurysm, demonstrating its circumferential arterial involvement. FIGURE 1. View largeDownload slide Preoperative CTA demonstrating a left temporoparietal intraparenchymal hemorrhage with an associated multi-lobulated aneurysm of the distal MCA in a 27-year-old female with primary pulmonary mucormycosis with new neurologic deficits. A, Axial and B, coronal images. C, Three-dimensional reconstructions of the aneurysm, demonstrating its circumferential arterial involvement. All patients undergoing this technique consented as per university protocol. Institutional Review Board approval was not needed for the single patient data included in this report. Operation The superficial temporal artery (STA) was mapped and dissected (Figure 2A), prior to performing a craniotomy over the IPH/aneurysm. The involved MCA branch was circumferentially dissected, and an end-to-side microvascular anastomosis of the STA to the MCA distal to the lesion was completeds (Figure 2B and 2C). Intraoperative indocyanine green imaging demonstrated anastomosis patency. The aneurysm and all grossly diseased segments of the parent artery were excised and sent for pathological examination. The IPH was then evacuated before the surgical cavity was closed in a layered fashion. FIGURE 2. View largeDownload slide STA-MCA bypass and excision of distal MCA mycotic aneurysm. A, The STA was dissected prior to performing a craniotomy over the left temporal IPH/aneurysm. B, The affected MCA branch was circumferentially dissected in preparation for clipping/excision, and C, an end-to-side STA-MCA anastomosis to an uninvolved artery segment distal to the lesion was performed (diseased MCA segment marked with a black arrow in panels B and C). The aneurysm and all diseased MCA segments were then excised (not shown). FIGURE 2. View largeDownload slide STA-MCA bypass and excision of distal MCA mycotic aneurysm. A, The STA was dissected prior to performing a craniotomy over the left temporal IPH/aneurysm. B, The affected MCA branch was circumferentially dissected in preparation for clipping/excision, and C, an end-to-side STA-MCA anastomosis to an uninvolved artery segment distal to the lesion was performed (diseased MCA segment marked with a black arrow in panels B and C). The aneurysm and all diseased MCA segments were then excised (not shown). FIGURE 3. View largeDownload slide Histologic analysis of resected aneurysm. A, H&E staining demonstrated inflamed tissue with fibrin deposition (40× magnification). B, 40× and C, 400× magnification of GMS silver stain for fungal organisms, demonstrating numerous broad, degenerate appearing hyphae, consistent with mucormycosis. D, Immunohistochemical staining also identified rare CMV positive cytomegalic cells (400× magnification; black arrow). FIGURE 3. View largeDownload slide Histologic analysis of resected aneurysm. A, H&E staining demonstrated inflamed tissue with fibrin deposition (40× magnification). B, 40× and C, 400× magnification of GMS silver stain for fungal organisms, demonstrating numerous broad, degenerate appearing hyphae, consistent with mucormycosis. D, Immunohistochemical staining also identified rare CMV positive cytomegalic cells (400× magnification; black arrow). Histopathology Aneurysm histopathology showed inflamed tissue with fibrin deposition on Hematoxylin and eosin (H&E) staining (Figure 3A). Gomori–Metheneamine silver (GMS) stain for fungal organisms demonstrated numerous broad, degenerate appearing hyphae, consistent with mucormycosis (zygomycosis; Figure 3B and 3C). In addition, immunohistochemical staining highlighted rare Cytomegalovirus (CMV) positive cells (Figure 3D). Postoperative Course Postoperatively, the patient improved neurologically to being alert and oriented, and following commands with full strength in all extremities, with her only deficit being mild aphasia. CTA demonstrated a patent anastomosis and IPH decompression (Figure 4). Endocarditis as a source of septic emboli was excluded with a negative transesophageal echocardiogram. As per infectious diseases, the patient was treated with long-term amphotericin B for disseminated mucormycosis, and was discharged home neurologically stable during her second postoperative week. She nonetheless required multiple readmissions for pulmonary complications and overwhelming infectious burden. One month postoperatively, she was admitted obtunded with a large left-sided IPH involving the deep white matter without an associated vascular lesion on CTA (Figure 5). Her neurological exam was consistent with a brain death at this time, and the patient's family withdrew care. Of note, the patient was consented for all described care as per university protocol. Institutional Review Board approval was not needed for the single patient data included in this report. FIGURE 4. View largeDownload slide Postoperative CTA. A, Axial view demonstrating adequate clot evacuation. B, Coronal view with visualization of stable perfusion distal to the bypass graft. FIGURE 4. View largeDownload slide Postoperative CTA. A, Axial view demonstrating adequate clot evacuation. B, Coronal view with visualization of stable perfusion distal to the bypass graft. FIGURE 5. View largeDownload slide Head CT upon re-presentation in obtunded state. Despite a well-tolerated aneurysm resection and bypass, the patient had progressive disseminated infection and presented 1 mo postoperatively with a large left-sided intraparenchymal hemorrhage involving the deep white matter of unknown etiology (axial noncontrast scan shown). CTA demonstrated decreased perfusion adjacent to the hemorrhage, but no underlying vascular lesion (not shown). The patient's neurological exam was consistent with brain death, and the decision to withdraw care was made by the patient's family. FIGURE 5. View largeDownload slide Head CT upon re-presentation in obtunded state. Despite a well-tolerated aneurysm resection and bypass, the patient had progressive disseminated infection and presented 1 mo postoperatively with a large left-sided intraparenchymal hemorrhage involving the deep white matter of unknown etiology (axial noncontrast scan shown). CTA demonstrated decreased perfusion adjacent to the hemorrhage, but no underlying vascular lesion (not shown). The patient's neurological exam was consistent with brain death, and the decision to withdraw care was made by the patient's family. DISCUSSION Mucormycosis infections are a rare pathology with high mortality in immunocompromised patients, even if diagnosed and treated early.6,7 We present the unique case of an IIA from disseminated mucormycosis from a primary pulmonary source, without evidence of associated skull base involvement. Candida species, Aspergillus species, Pseudallerscheria boydii, and Coccidioides are the most common causative pathogens of fungal IIAs,8 which typically occur as opportunistic infections in immunocompromised patients and/or following prolonged steroid use.2,3,5 Mechanistically, fungal IIAs are thought to destroy the elastic lamina of the arterial wall, resulting in a decreased resistance to intra-arterial pressures and aneurysm formation.9 While fungal IIAs are most often the result of extravascular spread from skull base infections,9–14 IIAs associated with intracranial abscesses, following intracranial surgery, or from hematogenous seeding have also been reported with Candida and Aspergillus species, and Pseudallerscheria boydii.15–19 Although published data are limited, rupture of fungal IIAs is associated with nearly 100% mortality.9,10,12–15,20 IIAs from Mucor species are a rare subset of fungal aneurysms that to date have almost uniformly been described in association with skull base infections.5,21–24 Although a significant number of disseminated mucormycosis cases involving the central nervous system (CNS) have been reported,7,18,25–27 the manifestations of these infections are typically meningitis, abscesses, or infarctions secondary to vasculitis, rather than aneurysms. Upon our review of the literature, only 2 other cases of Mucor IIAs without an associated skull base infection have been reported.20,28 In the first case, a 61-yr-old man on steroids following clipping of a ruptured anterior communicating artery aneurysm died after a hemorrhagic cerebral infarction.20 On autopsy, 4 aneurysms of the pericollosal artery were noted, with Mucor infiltration seen on vessel wall histology. In the second case, a 4-yr-old female on a prolonged steroid course for a lung infection of unknown etiology presented with focal seizures.28 Workup was concerning for subarachnoid hemorrhage, and the patient died several hours after presentation. On autopsy, she was found to have a ruptured left anterior cerebral artery aneurysm with Mucor infiltration of the aneurysm wall on histology. While no primary source of Mucor was identified in either of these cases, steroid use (and the previous craniotomy in the first case) were identified as risk factors for fungal IIA development. A summary of all previously reported IIAs from Mucor species is provided in Table. With this background, we believe the present case represents the second report of hematogenous seeding of mucormycosis resulting in IIA formation in the distal cerebral vasculature, in the absence of a contiguous skull base infection or previous intracranial surgery. TABLE. Previously Reported Infectious Intracranial Aneurysms From Mucor Species Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Abbreviations: ACA, anterior cerebral artery; Acomm, anterior communicating artery; DM, diabetes mellitus; ICA, internal carotid artery; SAH, subarachnoid hemorrhage; yr, year View Large TABLE. Previously Reported Infectious Intracranial Aneurysms From Mucor Species Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Authors Year Age (yr) Sex Underlying disease process Organism Source of intracranial infection Aneurysm location Aneurysm status Surgical management of aneurysm Clinical outcome Price et al24 1971 38 Male DM Phycomycetes (Mucor) Spontaneous infection of paranasal sinuses with extension to anterior skull base Proximal right ICA Ruptured N/a Death Ho21 1979 48 Female DM Mucor Spontaneous infection of paranasal sinuses with extension to anterior skull base Acomm Ruptured Surgical clipping Death Thajeb et al22 2004 62 Male DM Mucor Spontaneous infection of left orbital apex and cavernous sinus Not imaged Presumed ruptured (SAH) N/a Death Alvernia et al23 2009 38 Male DM Mucor Spontaneous infection of paranasal sinuses, left cavernous sinus, temporal fossa, and petrous bone Petrous segment of left ICA Unruptured Endovascular sacrifice of left ICA Neurologically intact at 1-yr follow-up Kasliwal et al5 2009 61 Male DM Mucor Postoperative infection of anterior skull base following trans-sphenoidal pituitary surgery Bilateral proximal (A1) ACAs Ruptured Surgical clipping of right A1 aneurysm Death Glass et al28 1978 4 Female Prolonged steroid use for lung infection of unknown etiology Phycomycetes (Mucor) Unknown Left anterior cerebral artery Ruptured N/a Death Kikuchi et al20 1985 61 Male Prolonged steroid use for vascular changes and edema after clipping of a ruptured Acomm aneurysm Phycomycetes (Mucor) Unknown Pericollosal artery (4 aneurysms) Unruptured N/a Death Abbreviations: ACA, anterior cerebral artery; Acomm, anterior communicating artery; DM, diabetes mellitus; ICA, internal carotid artery; SAH, subarachnoid hemorrhage; yr, year View Large While the patient's immunocompromised state from systemic lupus erythematous and Cyclophosphamide/steroid treatments almost certainly contributed to the development of her initial mucormycosis infection, the concomitant presence of Mucor and CMV within the aneurysm wall represents an additional unique component of this case. Although CMV has not been previously associated with IIA or noninfectious cerebral aneurysm formation, other viruses, such as the human immunodeficiency virus and varicella–zoster virus, have been linked to IIAs.8,29 CMV has also been reported as a cause of CNS vasculitis that results from an inflammatory response to viral invasion of the vascular wall.30 The dual inflammatory response to CMV and Mucor in this case may have thus synergistically increased arterial wall vulnerability to aneurysm formation. Despite the high mortality of fungal IIAs, aggressive surgical management was pursued due to the patient's young age and relatively high neurological function upon neurosurgery evaluation. Although conservative management of nonruptured IIAs can cause aneurysm resolution or decrease rupture risk,2,31 the ruptured status of the patient's aneurysm required treatment.1,32,33 The distal location and fusiform morphology with downstream eloquent cortex nonetheless limited endovascular and nonreconstructive surgical options. We thus chose to excise the aneurysm and all grossly involved vessel segments after an EC-IC bypass for distal revascularization. Even with successful aneurysm treatment and initial neurological improvement, the patient's prognosis worsened as a result of her progressive infectious burden, and she ultimately died from a secondary intracranial hemorrhage of unknown etiology. Although CTA at this time was negative for vascular disease, there was poor visualization of the distal MCA vessels on the affected side due to presumed mass effect from the hemorrhage. Progression of microscopically diseased arteries proximal to the resected segment (or even within the bypass graft itself following hematogenous seeding) is thus a possible hemorrhagic source. Hemorrhagic conversion of a large ischemic event, the latter described with cerebral mucormycosis from both skull base and disseminated infections,20,22,27 is another possible explanation for this secondary bleed. This case highlights the challenges of treating fungal IIAs, and serves as a reminder of the potential for abnormal presentations of infectious cerebrovascular pathologies. Important technical considerations when performing aneurysm and bypass surgery for IIAs include the need for meticulous microsurgical dissections due to the increased friability of the cerebrum and involved vasculature. Choosing a healthy recipient vessel site is also critical to the technical success of the bypass. CONCLUSION While typically associated with skull base lesions, IIAs from mucormycosis can form following hematogenous seeding from disseminated infections. Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article. REFERENCES 1. Ding D , Bok AP . Acute subdural hematoma from a ruptured aneurysm of the distal middle cerebral artery . J Neurosci Rural Pract . 2017 ; 8 ( 1 ): 152 - 154 . Google Scholar CrossRef Search ADS PubMed 2. Winn HR , Mayer SA , Bederson JB . Infectious intracranial aneurysms . In: Winn HR , ed. Youmans Neurological Surgery . 6th ed . Philadelphia, PA : Saunders , 2011 ; 3972 - 3977 . 3. Morris NA , Matiello M , Lyons JL , Samuels MA . Neurologic complications in infective endocarditis . Neurohospitalist . 2014 ; 4 ( 4 ): 213 - 222 . Google Scholar CrossRef Search ADS PubMed 4. Ducruet AF , Hickman ZL , Zacharia BE et al. Intracranial infectious aneurysms: a comprehensive review . Neurosurg Rev . 2010 ; 33 ( 1 ): 37 - 46 . Google Scholar CrossRef Search ADS PubMed 5. Kasliwal MK , Reddy VS , Sinha S , Sharma BS , Das P , Suri V . Bilateral anterior cerebral artery aneurysm due to mucormycosis . J Clin Neurosci . 2009 ; 16 ( 1 ): 156 - 159 . Google Scholar CrossRef Search ADS PubMed 6. Mok CC , Que TL , Tsui EY , Lam WY . Mucormycosis in systemic lupus erythematosus . Semin Arthritis Rheum . 2003 ; 33 ( 2 ): 115 - 124 . Google Scholar CrossRef Search ADS PubMed 7. Spellberg B , Edwards J , Ibrahim A . Novel perspectives on mucormycosis: pathophysiology, presentation, and management . Clin Microbiol Rev . 2005 ; 18 ( 3 ): 556 - 569 . Google Scholar CrossRef Search ADS PubMed 8. Kannoth S , Thomas SV . Intracranial microbial aneurysm (infectious aneurysm): current options for diagnosis and management . Neurocrit Care . 2009 ; 11 ( 1 ): 120 - 129 . Google Scholar CrossRef Search ADS PubMed 9. Hurst RW , Judkins A , Bolger W , Chu A , Loevner LA . Mycotic aneurysm and cerebral infarction resulting from fungal sinusitis: imaging and pathologic correlation . AJNR Am J Neuroradiol . 2001 ; 22 ( 5 ): 858 - 863 . Google Scholar PubMed 10. Ahsan H , Ajmal F , Saleem MF , Sonawala AB . Cerebral fungal infection with mycotic aneurysm of basilar artery and subarachnoid haemorrhage . Singapore Med J . 2009 ; 50 ( 1 ): e22 - e25 . Google Scholar PubMed 11. Watanabe T , Okada T , Okada C et al. An aspergillotic aneurysm of the internal carotid artery following allogeneic bone marrow transplantation: successful management with catheter coil embolization and long-term antifungal agents . Transpl Infect Dis . 2009 ; 11 ( 1 ): 49 - 53 . Google Scholar CrossRef Search ADS PubMed 12. Ahuja GK , Jain N , Vijayaraghavan M , Roy S . Cerebral mycotic aneurysm of fungal origin. Case report . J Neurosurg . 1978 ; 49 ( 1 ): 107-110 . Google Scholar CrossRef Search ADS 13. Iihara K , Makita Y , Nabeshima S , Tei T , Keyaki A , Nioka H . Aspergillosis of the central nervous system causing subarachnoid hemorrhage from mycotic aneurysm of the basilar artery . Neurol Med Chir (Tokyo) . 1990 ; 30 ( 8 ): 618 - 623 . Google Scholar CrossRef Search ADS PubMed 14. Watson JC , Myseros JS , Bullock MR . True fungal mycotic aneurysm of the basilar artery: a clinical and surgical dilemma . Cerebrovasc Dis . 1999 ; 9 ( 1 ): 50 - 53 . Google Scholar CrossRef Search ADS PubMed 15. Messori A , Lanza C , De Nicola M et al. Mycotic aneurysms as lethal complication of brain pseudallescheriasis in a near-drowned child: a CT demonstration . AJNR Am J Neuroradiol . 2002 ; 23 ( 10 ): 1697 - 1699 . Google Scholar PubMed 16. Piotrowski WP , Pilz P , Chuang IH . Subarachnoid hemorrhage caused by a fungal aneurysm of the vertebral artery as a complication of intracranial aneurysm clipping. Case report . J Neurosurg . 1990 ; 73 ( 6 ): 962 - 964 . Google Scholar CrossRef Search ADS PubMed 17. Visudhiphan P , Bunyaratavej S , Khantanaphar S . Cerebral aspergillosis. Report of three cases . J Neurosurg . 1973 ; 38 ( 4 ): 472 - 476 . Google Scholar CrossRef Search ADS PubMed 18. Gavito-Higuera J , Mullins CB , Ramos-Duran L , Olivas Chacon CI , Hakim N , Palacios E . Fungal infections of the central nervous system: a pictorial review . J Clin Imaging Sci . 2016 ; 6 1 : 24 . Google Scholar CrossRef Search ADS PubMed 19. Ho CL , Deruytter MJ . CNS aspergillosis with mycotic aneurysm, cerebral granuloma and infarction . Acta Neurochir . 2004 ; 146 ( 8 ): 851 - 856 . Google Scholar CrossRef Search ADS PubMed 20. Kikuchi K , Watanabe K , Sugawara A , Kowada M . Multiple fungal aneurysms: report of a rare case implicating steroid as predisposing factor . Surg Neurol . 1985 ; 24 ( 3 ): 253 - 259 . Google Scholar CrossRef Search ADS PubMed 21. Ho KL . Acute subdural hematoma and intracerebral hemorrhage. Rare complications of rhinocerebral mucormycosis . Arch Otolaryngol . 1979 ; 105 ( 5 ): 279 - 281 . Google Scholar CrossRef Search ADS PubMed 22. Thajeb P , Thajeb T , Dai D . Fatal strokes in patients with rhino-orbito-cerebral mucormycosis and associated vasculopathy . Scand J Infect Dis . 2004 ; 36 ( 9 ): 643 - 648 . Google Scholar CrossRef Search ADS PubMed 23. Alvernia JE , Patel RN , Cai DZ , Dang N , Anderson DW , Melgar M . A successful combined endovascular and surgical treatment of a cranial base mucormycosis with an associated internal carotid artery pseudoaneurysm . Neurosurgery . 2009 ; 65 ( 4 ): 733 - 740 ; discussion 740 . Google Scholar CrossRef Search ADS PubMed 24. Price DL , Wolpow ER , Richardson EP . Intracranial phycomycosis: a clinicopathological and radiological study . J Neurol Sci . 1971 ; 14 ( 3 ): 359 - 375 . Google Scholar CrossRef Search ADS PubMed 25. Ma J , Jia R , Li J et al. Retrospective clinical study of Eighty-One cases of intracranial mucormycosis . J Glob Infect Dis . 2015 ; 7 ( 4 ): 143 - 150 . Google Scholar CrossRef Search ADS PubMed 26. Royer M , Cervera P , Kahan A , Menkès CJ , Puéchal X . Mucormycosis cerebral arteritis mimicking a flare in ANCA-associated vasculitis . Lancet Infect Dis . 2013 ; 13 ( 2 ): 182 . Google Scholar CrossRef Search ADS PubMed 27. Ermak D , Kanekar S , Specht CS , Wojnar M , Lowden M . Looks like a stroke, acts like a stroke, but it's more than a stroke: a case of cerebral mucormycosis . J Stroke Cerebrovasc Dis . 2014 ; 23 ( 8 ): e403 - e404 . Google Scholar CrossRef Search ADS PubMed 28. Glass EC , Stadalnik RC , Barnett CA . Ventricular visualization on brain scan with intracranial hemorrhage in disseminated phycomycosis . Clin Nucl Med . 1978 ; 3 ( 11 ): 429 - 431 . Google Scholar CrossRef Search ADS PubMed 29. Ake JA , Erickson JC , Lowry KJ . Cerebral aneurysmal arteriopathy associated with HIV infection in an adult . Clin Infect Dis . 2006 ; 43 ( 5 ): e46 - e50 . Google Scholar CrossRef Search ADS PubMed 30. Koeppen AH , Lansing LS , Peng SK , Smith RS . Central nervous system vasculitis in cytomegalovirus infection . J Neurol Sci . 1981 ; 51 ( 3 ): 395 - 410 . Google Scholar CrossRef Search ADS PubMed 31. Molinari GF , Smith L , Goldstein MN , Satran R . Pathogenesis of cerebral mycotic aneurysms . Neurology . 1973 ; 23 ( 4 ): 325 - 332 . Google Scholar CrossRef Search ADS PubMed 32. Neto S , Flores JC , Figueiredo EG , Caldas JG , Teixeira MJ . Mycotic aneurysm treated with aneurysm trapping. Case report . J Neurol Surg Rep . 2016 ; 77 ( 1 ): e013 - e016 . Google Scholar PubMed 33. Singla V , Sharma R , Nagamani AC , Manjunath CN . Mycotic aneurysm: a rare and dreaded complication of infective endocarditis . BMJ Case Rep . 2013 ; 2013 : bcr2013200016 . Google Scholar PubMed COMMENTS In this report, the authors presented a case of a 27-year-old female with systemic lupus erythematosus and primary pulmonary mucormycosis who suffered from an intraparenchymal hemorrhage due to a ruptured fusiform distal MCA aneurysm. Pathologic examination of the excised artery confirmed Mucor infection. the authors believe that this case represents the first mycotic cerebral aneurysm from mucormycosis in a patient without an underlying skull base infection or previous intracranial surgery. This case underscores the prognosis of ruptured infectious aneurysms in general and “true” mycotic aneurysms in particular, which is poor. Managing infectious aneurysms is one of the challenges in neurosurgery that is better approached in a multidisciplinary way. As this patent was demed unstable to do cerebral aniography, we remind the physicians managing these patient about its importance and advantages. Cerebral angiography will not only show the characteristics of the aneurysm, the status of the artery harboring the aneurysm, presence of other small aneurysms that may be missed by CTA, it will most importantly assess if this affected artery is amenable for endovascular treatment (especially if there is no mass effect) as it becomes the primary choice in the many cases due to its versatility and the ability to deliver both reconstructive and deconstructive treatments to these tenacious lesions. Abdulrahman Y. Alturki Christopher S. Ogilvy Boston, Massachusetts The authors present a rather unique case report describing the many technical challenges faced when performing bypass surgery on a patient with a ruptured mycotic aneurysm. The authors report the surgical treatment of a 27-year-old woman with systemic lupus erythematous and pulmonary mucomycosis presenting with subarachnoid hemorrhage from a distal middle cerebral artery mycotic aneurysm. The authors performed a technically successful superficial temporal artery to middle cerebral artery bypass, successfully excising the infected vessel segment and achieving a successful patent bypass. The concomitant cytomegalovirus infection may have contributed to the ultimate rehemorrhage and demise of this patient though it will remain unclear. Though a difficult case presentation with an unfortunate outcome, the authors present a very well-described report that present to the reader important nuances in the management of patients with mycotic aneurysms. As with every bypass operation, we are reminded of the need for meticulous preparation of the tissue and a most-careful inspection of the donor and recipient artery in preparation for the bypass. In particular, vessels harboring mycotic aneurysms must be bypassed in locations where the tissue is at the very least macroscopically normal. Additional follow-up and image monitoring might also be required to determine interval recurrence of infectious lesions, especially in the setting of on-going infection and seeding. Charles J. Prestigiacomo Cincinnati, Ohio Copyright © 2018 by the Congress of Neurological Surgeons This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

Operative NeurosurgeryOxford University Press

Published: May 24, 2018

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