Orbital apex syndrome

Orbital apex syndrome

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  Orbital syndromes
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  Cavernous sinus syndrome (CSS) OculomotorNerve (III), Trochlear Nerve (IV), Abducens Nerve (VI), Ophthalmic Branch Of Trigeminal Nerve (V1) Maxillary branch of the trigeminal nerve (V2) Oculosympathetic fibers . Orbital apex syndrome (OAS) Oculomotor Nerve (III), Trochlear Nerve (IV), Abducens Nerve (VI), Ophthalmic Branch Of Trigeminal Nerve (V1) Optic Nerve dysfunction. Superior orbital fissure syndrome (SOFS) Oculomotor Nerve (III), Trochlear Nerve (IV), Abducens Nerve (VI), Ophthalmic Branch Of Trigeminal Nerve (V1) OR Nasociliary nerve
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  • Cavernous sinuslesions are also more commonly bilateral • The superior orbital fissure, orbital apex, and cavernous sinus are all contiguous, and etiologies are similar. • Sometimes, pts with SOFS may subsequently develop orbital apex and cavernous sinus pathology.
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  Symptoms and signs •Visual loss and ophthalmoplegia • Periorbital or facial pain, involvement of the ophthalmic (V1) or maxillary (V2) branch of the trigeminal nerve. • It is important to test the periorbital skin and the corneal reflexes to detect asymmetry in sensation. • Infectious, inflammatory, and neoplastic conditions may be associated with proptosis. • Vascular causes of a CSS, such as a carotid–cavernous fistula, classically are associated with pulsatile proptosis.
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  • To assessfor optic nerve dysfunction, BCVA, afferent pupillary defect, and color vision . • Visual field testing with kinetic or static perimetry may reveal subtle visual field deficits when visual acuity is normal. • Optic atrophy typically develops over weeks to months in patients with an OAS.
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  • Diplopia maybe the presenting symptom in SOFS, OAS, or CSS. • The pattern of an ocular deviation is especially important in the evaluation of a single ocular motor nerve palsy • However, because multiple cranial nervesmay be involved, a distinct pattern may be difficult to detect.
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  Causes Orbital apex syndromesmay be caused by • Inflammatory, • Infectious, • Neoplastic, • Iatrogenic/Traumatic, • Vascular Processes
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  Inflammatory • Sarcoidosis • Systemiclupus erythematosus • Churg–Strauss syndrome • Wegener granulomatosis • THS • Giant cell arteritis • Orbital inflammatory pseudotumor • Thyroid orbitopathy
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  Inflammatory • painful ophthalmoplegiawith or without associated optic neuropathy . • Neurosarcoidosis • Elevated ESR and a normal serum angiotensin- converting enzyme, chest radiograph, and gallium scan. • Definitive diagnosis was made by cavernous sinus biopsy, which revealed noncaseating granulomas with multinucleated giant cells.
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  • Churg–Strauss syndrome •Autoimmune condition that causes inflammation of small and medium sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).
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  • The THSrefers to a condition of unknown cause involving painful ophthalmoplegia resulting from granulomatous inflammation within the cavernous sinus or orbital apex . • Diagnostic criteria of THS to include episodes of unilateral orbital pain persisting for weeks if untreated; associated paralysis of one or more of the third, fourth, or sixth cranial nerves; and/or demonstration of a granuloma by MRI or biopsy.
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  Infectious • Fungi: Aspergillosis,Mucormycosis • Bacteria: Streptococcus spp., Staphylococcus spp., Actinomyces spp., Gram-negative bacilli, anaerobes, Mycobacterium tuberculosis • Spirochetes: Treponema pallidum • Viruses: Herpes zoster
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  • Mucormycosis andAspergillosis should be suspected in individuals with predisposing conditions including DM, alcoholism, hematologic malignancies, and immunosuppression. • A fungal cause should be considered in any patient requiring immunomodulatory, antineoplastic, or long-term corticosteroid therapy.
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  • Tuberculosis hasalso been reported to cause both unilateral and bilateral CSS. • Bacterial infection may result in cavernous sinus thrombosis, most commonly from contiguous spread of infections from the paranasal sinuses. Organisms most common include Staphylococcus aureus, Streptococcus pneumoniae, other streptococci, Gram- negative bacilli, and anaerobes.
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  Neoplastic • Head andneck tumors: nasopharyngeal carcinoma, adenoid cystic carcinoma, squamous cell carcinoma • Neural tumors: neurofibroma, meningioma, ciliary neurinoma, schwannoma • Metastatic lesions: lung, breast, renal cell, malignant melanoma • Hematologic: Burkitt lymphoma, non-Hodgkin lymphoma, leukemia • Perineural invasion of cutaneous malignancy
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  • Lymphoma maybe found in the paranasal sinuses or within the cavernous sinus alone. • Neural tumors of the orbital apex are most commonly benign and include meningiomas, schwannomas, neurofibromas, and, rarely, ciliary neurinomas . • Metastatic disease to the cavernous sinus has been reported from the breast, lung , kidney , and from malignant melanoma.
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  Iatrogenic/traumatic • Iatrogenic – Sinonasalsurgery – Orbital/facial surgery • Traumatic – Penetrating injury – Nonpenetrating injury – Orbital apex fracture – Retained foreign body
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  • Vascular – Carotidcavernous aneurysm – Carotid cavernous fistula – Cavernous sinus thrombosis – Sickle cell anemia • Other – Mucocele
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  • Cavernous carotidaneurysms may cause a unilateral or bilateral cavernous sinus syndrome via compression of adjacent cranial nerves. • A carotid– cavernous fistula may cause pulsatile proptosis, severe conjunctival injection, and glaucoma from elevated episcleral venous pressure. • Septic thrombosis of the cavernous sinus may occur in the setting of systemic or, more commonly, periorbital infections, but aseptic cavernous sinus thromboses have also been noted. Cavernous sinus thrombosis may be visualized on contrast-enhanced high-resolution CT or with MRI.
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  Evaluation and management •High-resolution MRI of the brain and orbits with contrast and fat suppression sequences. • CT also plays an important role in the setting of trauma or in suspected magnetic foreign bodies, surgical clips • CT is superior to MRI for the depiction of bony anatomy and is especially helpful if an orbital apex fracture is suspected clinically. • Vascular lesion suspected, MRA or CT angiography.
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  • Laboratory testingfor inflammation and infection (eg, ESR, CBC with differential, rapid plasma reagin, microhemagglutination assay for antibody to Treponema pallidum, ACE, p-ANCA, c-ANCA, ANA, purified protein derivative, chest Xray, HIV, LP)should also be considered if the clinical findings are suggestive of these processes.
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  • When aspecific cause cannot be determined, • Primary management options in a patient with an OAS include observation, an empiric trial of corticosteroids, and surgical biopsy. • In the absence of systemic signs of infection and when inflammatory disease is likely, we offer corticosteroids and a period of close observation. • If visual loss or ophthalmoplegia progresses, repeat neuroimaging and a biopsy are then pursued. • Neurosurgical, otolaryngologic, and radiologic consultation, Internal medicine or neurologic consultation
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  • Management ofan OAS is aimed at the underlying cause. • The distinction between inflammation, infection, and neoplasia is often difficult, and all may respond initially to corticosteroids. • Methotrexate and azathioprine have provided clinical benefit in a limited number of patients with THS. • Radiotherapy also has reportedly alleviated symptoms of THS in one patient refractory to immunosuppressive therapy and in another patient who became steroid dependent. • Following traumatic or iatrogenic operative injury, corticosteroids may be instituted while surgical intervention is considered