Orbital apex disorders: Imaging findings and management

Abstract

Orbital apex disorders include orbital apex syndrome, superior orbital fissure syndrome and cavernous sinus syndrome. These disorders result from various etiologies, including trauma, neoplastic, developmental, infectious, inflammatory as well as vascular causes. In the past, these have been described separately based on anatomical locations of disease process; however, these three disorders share similar causes, diagnostic evaluation and management strategies. The etiology is diverse and management is directed to the causative process. This imaging review summarizes the pertinent anatomy of the orbital apex and illustrates representative pathological processes that may affect this region. The purpose of this review is to provide an update on the current status of diagnostic imaging and management of patients with orbital apex disorders.

Keywords: Orbital apex; cavernous sinus; ophthalmoplegia; proptosis; ptosis; superior orbital fissure.

Figure 1.

Orbital apex anatomy: CT. Axial and coronal CT slices demonstrate the useful clinical anatomy of the orbital apex. Red arrow: SOF, yellow arrow: greater wing of sphenoid, blue arrow: optic strut, green arrow: anterior clinoid process, white arrow: optic canal. CT: computed tomography; SOF: superior orbital fissure.

Figure 2.

Orbital apex anatomy: MRI. Top images: Axial T2W MR images (top) through the orbits demonstrate the useful clinical anatomy of the orbital apex. Red arrow: ICA, with arrow: cavernous sinus, blue arrow: SOF, green arrow: optic canal, white arrow: greater wing of sphenoid, pink arrow: optic nerve. Bottom image: Enlarged coronal T2W MR image though the orbital apex well demonstrates the anatomy. Yellow arrow: optic canal, red arrow: anterior clinoid process, blue arrow: SOF, white arrow: ethmoid sinus. MRI: magnetic resonance imaging; T2W: T2-weighted; ICA: internal carotid artery; SOF: superior orbital fissure.

Figure 3.

Diagrammatic representation of the right superior orbital fissure with its content. (Image modified from Rai S and Rattan V. Natl J Maxillofac Surg 2012; 3: 222–225, copyright 2012, Wolters Kluwer).

Figure 4.

Facial fractures. A 47-year-old male status post-motor vehicle collision. Axial computed tomography (CT) images in bone (a) and brain (b) windows demonstrate a comminuted fracture of the greater wing of the left sphenoid bone (red and yellow arrow), left lateral orbital wall (blue arrow), and lamina papyracea (green arrowhead) causing deformity of the face and irregularity of the optic canal. A small subarachnoid hemorrhage is seen more superiorly (curved white arrow). (c) CT image demonstrates comminuted fractures of the inferior orbital wall (green and blue arrows) and left maxillary sinus (red and yellow arrows) with resultant cranial caudal narrowing of the optic canal (curved white arrow).

Figure 5.

Metastases: parotid adenoid cystic carcinoma. A 63-year-old female with parotid adenoid cystic carcinoma presenting with diplopia and visual field deficit. Axial (a) and coronal (b) post-contrast images demonstrate an enhancing lesion centered at the orbital apex (yellow arrow). Coronal images show extension of the lesion into the cavernous sinus (red arrow). (c) Axial post-contrast image through the brain (a) demonstrates an enhancing dural metastatic lesion (white arrow). Sagittal T2 (b), T1 (c), and post-contrast (d) images of the spine demonstrate abnormally low T1 signal throughout all vertebral bodies corresponding to widespread enhancing metastatic lesions.

Figure 6.

Perineural tumoral spread. A 59-year-old female with history of left forehead squamous cell carcinoma. Coronal post-contrast images through the orbits (a) and sphenoid sinus (b) demonstrate a thickened and enhancing right supraorbital nerve (red arrow) abutting the superior rectus with posterior extension to the orbital apex and superior orbital fissure (red and blue arrows). Axial post-contrast (c) is confirmatory.

Figure 7.

Perineural tumoral spread. Same patient as Figure 6, axial (a) and coronal (b) images through the level of pituitary in 2011 demonstrate normal left cavernous sinus and Meckel’s cave. In 2012, axial (c) and coronal (d) post-contrast images through the same region demonstrate enhancing tumor infiltrating the left cavernous sinus (yellow arrows) and left Meckel’s cave (red arrows).

Figure 8.

An 84-year-old female with transformed diffuse B-cell lymphoma refractory to therapy presenting with left vision loss and syncope. Axial T2 (a) and axial post-contrast (b) images through the brain demonstrate a dural-based enhancing lesion that extends into the orbital apex and optic nerve (red arrows). (c) Axial fluid-attenuated inversion recovery image through the basifrontal lobes (a) demonstrates associated white matter changes (red arrow). An axial post-contrast image (d) through the sphenoid air cells shows a peripherally enhancing fluid collection (red arrow). Biopsy of the sphenoid sinus revealed diffuse large B-cell lymphoma.

Figure 9.

Meningioma: computed tomography. A 54-year-old female with right-sided diminution of vision. (a) axial, (b) coronal and (c) sagittal images through the right orbit show an enlarged left optic nerve with tram-track calcification around the right optic nerve (black arrows in (a) and (b), white arrow in (c)). This calcification is characteristic for optic nerve sheath meningioma.

Figure 10.

Meningioma: magnetic resonance imaging. A 61-year-old male with history of prior subtotal sphenoidal meningioma resection presenting with left eye blindness. Axial post-contrast images at the level of the superior orbital fissure (a) and cavernous sinus (b) demonstrate an extra-axial enhancing lesion (red arrows) related to the sphenoid bone extending into the superior orbital fissure (blue arrow), orbital apex, and cavernous sinus (curved yellow arrow). Coronal post-contrast image (c) through the orbits demonstrates the large enhancing mass (*) encircling and compressing the left optic nerve (red arrow). The normal right optic nerve is seen for comparison (yellow arrow in image (c)).

Figure 11.

A 56-year-old male with sudden-onset ophthalmoplegia and decreased facial sensation in the left V1 distribution. Axial T2 (a) and post-contrast (b) images demonstrate a cystic lesion in the left cavernous sinus (yellow arrow) with a fluid level (red arrow). No enhancement is seen (yellow arrow). Surgery revealed a hemorrhagic V1 schwannoma.

Figure 12.

Coronal (a), axial (b) T2W MR image shows a hyperintense lesion medial to the medial rectus muscle (yellow arrow) extending to the medial orbital wall forming sub-periosteal abscess and extending laterally into the intraconal fat. Post-contrast coronal T1W image (c) shows the enhancement (yellow arrow). This was a case of orbital cellulitis. T2W: T2-weighted; MR: magnetic resonance; T1W: T1-weighted.

Figure 13.

An 87-year-old male presenting with right eye pain, vision loss, ophthalmoplegia, and ptosis of the right eyelid. Axial T1 (a) and coronal T2 (b) images through the cavernous sinus demonstrate intermediate signal soft tissue (yellow arrows) expanding into the right cavernous sinus. Note lack of normal flow voids on the right compatible with internal carotid artery (ICA) thrombosis. A normal ICA flow void is seen on the left (red arrows in (a) and (b)). Axial (c) and coronal (d) post-contrast T1 images through the cavernous sinus demonstrate enhancement of the expansile right cavernous sinus soft tissue (yellow arrows). Again, note lack of normal right ICA flow voids and normal left-sided ICA flow voids (red arrows). Surgery revealed invasive fungal hyphae compatible with aspergillus.

Figure 14.

A 64-year-old male presenting with right-sided vision loss and cranial neuropathy. The patient has a history of sarcoidosis. Axial (a) and coronal (b) post-contrast T1 images at the level of the cavernous sinus demonstrate enhancing extra-axial soft tissue along the greater wing of the right sphenoid (red arrow) with intracranial extension into the cavernous sinus (yellow arrows). Coronal computed tomography through the chest in soft tissue (c) and lung (d) window demonstrates anteroposterior window lymphadenopathy (yellow arrows) as well as upper lobe predominant fibrosis (red arrows) compatible with sarcoidosis.

Figure 15.

Orbital pseudotumor. A 42-year-old male with left eye exophthalmos, lid swelling, and facial swelling. Axial (a) and coronal (b) noncontrast computed tomography images through the orbits demonstrate enlargement and infiltrative changes of the lacrimal gland (yellow arrows) as well as of the extra-ocular muscles (red arrows). Axial short inversion time inversion recovery (STIR) (c) and post-contrast (d) images through the orbits show enlargement and increased signal in the left lateral rectus muscle (red arrow). Additionally, there is nodular extension of disease into the superior orbital fissure and orbital apex (thick yellow arrow). Symptoms resolved after steroid therapy.

Figure 16.

Tolosa Hunt syndrome. A 44-year-old female with waxing and waning ophthalmoplegia of the left eye for two years. The patient is treated episodically with mycophenolate mofetil and steroids. Post-contrast axial (a) and coronal (b) post-contrast images through the brain demonstrate an enhancing lesion extending from the orbital apex to the cavernous sinus (red arrows). The patient underwent external beam radiation for persistent symptoms. Follow-up contrast-enhanced magnetic resonance imaging ((c) and (d)) demonstrates normal cavernous sinuses bilaterally (red arrows). The enhancing lesion previously seen in the left cavernous sinus is no longer present.

Figure 17.

A 68-year-old male with Graves’ disease presenting with proptosis and diplopia. Coronal short inversion time inversion recovery (a) and axial post-contrast images through the orbits demonstrate an enlarged inferior rectus muscle with tendinous sparing (yellow arrows), which is characteristic of thyroid orbitopathy.

Figure 18.

A 67-year-old female presenting with proptosis, conjunctival arteriolization and cranial nerve VI palsy. Coronal three-dimensional contrast-enhanced magnetic resonance (MR) angiogram (a) through the cavernous sinuses demonstrates abnormal early enhancement and enlargement of the left cavernous sinus (yellow arrows). Axial post-contrast T1 (b) image demonstrates an enlarged left superior ophthalmic vein (curved red arrow). Frontal (c) and sagittal (d) angiographic images again demonstrate an abnormal early blush in the cavernous sinus (yellow arrows) as well as an enlarged draining superior ophthalmic vein (red arrows) compatible with a carotid cavernous fistula.

Figure 19.

A 48-year-old female presenting with question of left facial cellulitis. Axial post-contrast CT image through the cavernous sinus (a) demonstrates lack of normal cavernous sinus enhancement bilaterally (thin yellow arrows) compatible with cavernous sinus thrombosis. The internal carotid arteries (ICAs) show appropriate enhancement (thick blue arrows). More superiorly (b) an enlarged left superior ophthalmic vein is seen (curved red arrow). Note congestive changes in the overlying scalp and face (*). Axial post-contrast T1 (c) image through the cavernous sinuses again demonstrate lack of normal cavernous sinus enhancement (thin blue arrows) and normal ICA flow voids (thick yellow arrows). More superiorly (d), a distended right superior ophthalmic vein is seen (curved red arrow) secondary to back pressure.

Figure 20.

Optic nerve glioma. (a) Axial T2WI and (b) T1WI MR in a patient with NF1 shows diffuse enlargement of the optic nerves bilaterally (yellow ovals in (a) and red ovals in (b)). The lesions extend posteriorly to involve the optic chiasm and optic tracts. These show heterogeneous enhancement on post-contrast T1W fat-suppressed images (blue ovals in (c)). T2WI: T2-weighted imaging; T1WI: T1-weighted imaging; MR: magnetic resonance; NF1: neurofibromatosis type 1.

Figure 21.

A 71-year-old female with progressive right visual loss and proptosis. Axial computed tomography through the orbit (a) demonstrates an expansile ground-glass lesion (yellow arrow) in the right greater wing of the sphenoid. Note narrowing of the optic canal (red arrow) and proptosis (curved blue arrow). Axial (b) and coronal (c) post-contrast magnetic resonance images demonstrate mild enhancement (yellow arrow). Although having classic appearance for fibrous dysplasia, this case was confirmed with surgery.

Figure 22.

Dermoid cyst. Axial T1WI (a), axial GRE (b), axial post-contrast T1WI (c), axial T2WI (d), coronal T2WI (e) MR images demonstrate a circumscribed T1 and T2 hyperintense, nonenhancing mass in the antero-lateral cavernous sinus (yellow arrows). (f) CT shows fat density in the posterior aspect of the mass (red circle). T1WI: T1-weighted imaging; GRE: gradient echo; T2WI: T2-weighted imaging; MR: magnetic resonance; CT: computed tomography.

Figure 23.

Epidermoid cyst. (a) Axial T2WI shows a high-signal intensity oval lesion (red arrow) in the anterior left cavernous sinus, and with no enhancement on post-contrast T1WI (yellow arrow in (b)). This lesion is hyperintense on DWI (blue circle in (c)) and shows restricted diffusion on ADC map (green circle in (d)), compatible with orbital apex epidermoid. T2WI: T2-weighted imaging; T1WI: T1-weighted imaging; DWI: diffusion-weighted imaging; ADC: apparent diffusion coefficient.

Figure 24.

Sphenoid mucocele. Axial (a) and coronal (b) CT images show an isodense expansile lesion (yellow ovals) in the sphenoid sinus causing narrowing of the right superior orbital fissure (red arrow). Axial (c) and sagittal (d) T1W MR images show the lesion to be T1 hyperintense (green oval). Note right superior orbital fissure (blue arrow). The lesion is isointense (pink ovals) on T2W images ((e) and (f)). CT: computed tomography; T1W: T1-weighted; MR: magnetic resonance; T2W: T2-weighted.