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Symptoms to Diagnosis

An 86-year-old man with unexplained right-sided headache and vision loss

Marc Ohlhausen, BS, Eden Bernstein, MD and Craig D. Nielsen, MD, FACP
Cleveland Clinic Journal of Medicine September 2021, 88 (9) 494-501; DOI: https://doi.org/10.3949/ccjm.88a.20077
Marc Ohlhausen
Medical Student, Case Western Reserve School of Medicine, Cleveland, OH
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Eden Bernstein
Internal Medicine Resident, Department of Internal Medicine, Cleveland Clinic, Cleveland, OH
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Craig D. Nielsen
Department of Internal Medicine, Cleveland Clinic; Associate Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH; Governor, Ohio Chapter, American College of Physicians; Deputy Editor, Cleveland Clinic Journal of Medicine
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    Figure 1

    The anatomy of the orbital apex, the most posterior aspect of the orbit. The unilateral cranial nerve (CN) deficits in this patient point to disruption of structures of the orbital apex including the optic, oculomotor, trochlear, and abducens nerves. The optic foramen contains the optic nerve, ophthalmic artery, and associated sympathetic nerves. The nasociliary, frontal, and lacrimal branches of the ophthalmic nerve, superior ophthalmic vein, and cranial nerves III, IV, and VI pass through the superior orbital fissure.

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    Figure 2

    Magnetic resonance imaging of the orbit showed lesions (arrows) in axial T1-weighted fat-suppressed series (A) and coronal multiplanar reformation (B) views.

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    TABLE 1

    Causes of orbital apex syndrome

    Inflammatory
    Sarcoidosis, systemic lupus erythematosus, antineutrophil cytoplasmic antibody vasculitis (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis), Tolosa-Hunt syndrome, giant cell arteritis, orbital inflammatory pseudotumor, thyroid orbitopathy, immunoglobulin G4-related disease
    Infectious
    Fungi: Aspergillosis, mucormycosis
    Bacteria: Streptococcus species, Staphylococcus species, Actinomyces species, gram-negative bacilli, anaerobes, Mycobacterium tuberculosis
    Spirochetes: Treponema pallidum
    Viruses: Herpes zoster ophthalmicus
    Neoplastic
    Head and neck tumors: nasopharyngeal carcinoma, adenoid cystic carcinoma, squamous cell carcinoma
    Neural tumors: neurofibroma, meningioma, ciliary neurinoma, schwannoma
    Metastases: lung, breast, renal cell, malignant melanoma
    Hematologic: non-Hodgkin lymphoma, leukemia
    Perineural invasion of cutaneous malignancy
    Iatrogenic
    Sinonasal surgery, orbital-facial surgery
    Traumatic
    Orbital apex fracture, retained foreign body, penetrating or nonpenetrating injury
    Vascular
    Carotid cavernous aneurysm, carotid cavernous fistula, cavernous sinus thrombosis, sickle cell anemia
    Other
    Mucocele
    • Adapted from reference 8.

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Cleveland Clinic Journal of Medicine: 88 (9)
Cleveland Clinic Journal of Medicine
Vol. 88, Issue 9
1 Sep 2021
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An 86-year-old man with unexplained right-sided headache and vision loss
Marc Ohlhausen, Eden Bernstein, Craig D. Nielsen
Cleveland Clinic Journal of Medicine Sep 2021, 88 (9) 494-501; DOI: 10.3949/ccjm.88a.20077

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An 86-year-old man with unexplained right-sided headache and vision loss
Marc Ohlhausen, Eden Bernstein, Craig D. Nielsen
Cleveland Clinic Journal of Medicine Sep 2021, 88 (9) 494-501; DOI: 10.3949/ccjm.88a.20077
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  • Article
    • EVALUATING AND MANAGING SUSPECTED GIANT CELL ARTERITIS
    • CASE CONTINUED: SYMPTOMS PROGRESS DESPITE STEROIDS
    • CRANIAL NERVE PATTERN PROVIDES CLUES
    • EVALUATING THE CAUSE OF ORBITAL APEX SYNDROME
    • CASE CONTINUED: FURTHER EVALUATION
    • IMAGING THE ORBITAL APEX
    • CASE CONTINUED: DIAGNOSTIC IMAGING AND BIOPSY
    • ORBITAL FUNGAL INFECTIONS
    • TREATING INVASIVE ASPERGILLOSIS
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