Imaging of extraocular orbital pathology

BY : Ali Hekmatnia M.D

Imaging indications

• Ophthalmologist suspects pathology symptomatically or by sonography not exactly delineated

• In cases of trauma (e.g. foreign body, fractures)

• Posttreatment

Imaging modalities

• US

• CT – MDCT often working horse

• MRI

Imaging techniques CT

MDCT, axial, -/+ CM (depending on pathology), coronal/sagittal reconstructions, soft tissue/bone window level

MRIHeadcoil/surface coils, axial IR, axial T1wSE -/+ CM, coronal/sagittal T1wSE+CM+ FS, matrix 512x512, FOV ~20cm

- Axial and coronal images

- Axial 3mm sections

- Coronal 5mm sections- Coronal sections from the lateral

orbital rim to the posterior aspect of the

optic canals

(anterior clinoid or dorsum sellae)

CT Scan : TECHNIQUE

- Extraocular muscles , optic nerve

sheath , nasal complex ,vessels

and globes , Spread of processes

from surrounding structures

- Windowing : soft tissue as well as

bone-oriented window

Coronal images :

- Multiplanar capability , without ionizing radiation and

bony artifact(especially in the orbital apex,optic canal

and parasellar regions ) .Best soft tissue contrast.

- Protocol of MRI :

coronal and axial T1 and T2W images , coronal T1W

with fat saturation(before and after contrast injection )

MRI :

Anatomy of the Orbit Compartimental anatomy• Extraconal • Conal• Intraconal• Globe• Lacrimal gland

- Bony cavity , the globe, muscle cone,optic

nerve-sheath complex,lacrimal apparatus,orbital

fat,vascular and nerve structures,orbital septum

and lids

Orbital Anatomy :

Muscle Cone :- Superior,medial,lateral and inferior recti,Superior

and inferior obliques, Levator palpebrae superioris.

- Introconal space : Surgical problems

- Extraconal space : Medical management

- Globe : Cornea,lens,anterior chamber,vitreous,

retinal - scleral complex

Optic nerve sheath complex :

- Optic nerve , subarachnoid space , fluid

between dura and nerve , diameter of

complex (4-6mm)

Anatomy of the Orbit

• Intraorbital• ExtraconalConal• Intraconal• Globe

Anatomy of the OrbitCompartimental anatomy

• Intraorbital• Extraconal• Conal• Intraconal• Globe• Lacrimal gland

Supraorbital fissure

Infraorbital fissure and pterygopalatine fossa

Supraorbital fissure

Close relationship to PNS

Variant

Anatomy of the orbit

Muscles

Muscles

Anatomy of the orbit

Close relationship to vascular/nerval structures!!!

Orbita may be easily affected!!!

Anatomic regions

Fossapterygopalatina• Close relation

Orbit-PNS-Oropharynx

• Nerves III, IV, V, VI, VII

• Parasellarregion

Maxillary nerve Greater petrosal nerve

Pathology

• Inflammation orbital-extraorbital• Blastoma orbital-extraorbital

benign-malignant• Trauma• Foreign bodies•

Cave metallic foreign bodies NO MRI !!!

Inflammation

Inflammation of lidCT (Abscess)MRI (Phlegmone)

– Spread of infection from

ethmoid cells– Compression of optic nerve– Thrombosis

Harnsberger R:Head and Neck 2004

Subperiostal abscess CT

-Spread of infection from ethmoid cells

-Compression of optic nerve!!

-Thrombosis!!

Harnsberger R:Head and Neck 2004

Subperiostalabscess MRI

Dacryoadenitis

ORBITAL CELLULITIS IN A 13-YEAR-OLD BOY WITH EXTENSIVE RIGHT ETHMOID SINUS DISEASE

The inflammation involved the medial extraconal portions of the right orbitAxial CT scan shows lateral

displacementof the medial rectus muscle and infiltration ofthe extraconal fat (arrows)

Subperiosteal abscess in a 4-year-old girl with chronic right

ethmoid sinusitis

The inflammation involved the preseptal andextraconal portions of the medial right orbit. Axial CT scan shows the slightly displaced and thickened medial rectus muscle and a small focal fluid collection (arrow), which was confirmed as representing a subperiostealabscess

Orbital pseudotumor

Gross mass-like enlargement of the medial rectus muscle, with characteristic hypointense signal on T1W (a) and T2W (b) sequences. Moderate

heterogeneous enhancement is seen in the post gadolinium image (c)

Orbital pseudotumor (different patients)

Axial CECT shows a diffuse infiltrative right orbital mass involving the globe and causing marked proptosis

Diffuse enlargement of the lacrimal gland is seen with preservation of its shape

There is diffuse thickening of the bilateral medial and lateral rectus muscles including their tendinous insertion (arrows) which is typically spared in thyroid ophthalmopathy

Optic nerve neuritis (MS)

3T Orbital pseudotumorNo diffuse infiltration

Pseudo-inflammation

Endocrine orbitopathy CT

Graves disease / M. Basedow

Endocrine orbitopathy MR

Graves disease / M. Basedow

Endocrine orbitopathy 3T MRThickening and hyperintensity of medial and inferior rectus muscle

Small capillary hemangioma

3T MRLarge capillary hemangioma

Hemangiomatosis

3T MRLarge lymphatic-venousmalformation

LYMPHANGIOMA IN A 4-YEAR-OLD BOY WITH SUDDEN SUPRAORBITAL FULLNESS OF THE RIGHT EYE

Axial CT scan reveals a multilocular intraconal lymphangioma in the right orbit.

Lymphangioma in a patient who experienced sudden proptosis and

discolorationabout his right eye

Axial T2/W MR image demonstrates hemorrhage into a multilocular lymphangioma.The high-signal-intensity methemoglobin is layering anteriorly in each cyst.

VENOLYMPHATIC MALFORMATION

USG reveals a multiseptate cystic mass in the orbit MRI reveals a heterogeneous intraconal mass in the right orbit

displacing the optic nerve. Lesion is heterogeneous in signal intensity with a hyperintense area on T1W image (a) which shows blood-fluid level on T2W sequence (arrows) (b). There is only mild enhancement following contrast administration (c)

Blastoma/Tumor-like Bone

Lacrimal gland

Conus

(Globe)

Nerve

Fibrous dysplasia, Metastasis

Adenoma, Dermoid,pleomorphic Adenoma,Lymphoma

Grave`s, Hemangioma,Lymphoma, Schwannoma,Pseudotu

(Melanoma, Retinoblastoma)

Glioma, Meningeoma

P. Som Head and Neck Imaging 4th ed. 2003

Orbital pathologyPathology bony orbit

Fibrous dysplasia orbit

Blastoma

Melanoma lower lid

Dermoid

Fat

3T MRPleomorphic adenoma

Pleomorphic carcinoma with papilla infiltration (II)

Pleomorphic adenomaparotid gland

MALT-lymphoma lacrimal gland

Orbital lymphoma diffuse infiltration

Rhabdomyosarcoma

3T MRRhabdomyosarcomaM. rectus superior

Rhabdomyosarcoma

Cavernoushemangioma

Axial T1W and T2W MRI reveal an elongated lesion around the optic nerve which is hypointense on T1W and hyperintense on T2W sequence. Note the characteristic “club like” configuration of the lesion in the sagittal T2W

Orbital varix

Orbit Varix :

- Large , tortuous vein or a mass like confluence of

small veins may markedly enlarge with changes in

venous pressure (Valsalva ` maneuver)

ORBITAL VARIX

Axial CECT in a child with intermittent proptosis is almost normal. However, during valsalva maneuver the enhancing mass and the associated tortuous venous channels stand out causing significant proptosis

CAPILLARY HEMANGIOMAIN A 5-MONTH-OLD GIRL WITH DIFFUSELY DILATED CAPILLARIES AND CHEMOSIS OF THE EYELID

• Axial and sagittal Ti-weighted MR images demonstrate a capillary hemangioma superficially and preseptally about the left orbit.

• Several prominent vessels are noted within the mass.

CAPILLARY HEMANGIOMA

Axial CECT shows an intensely enhancing mass in the eyelid and extraconal space of the left orbit causing displacement of the globe

CAVERNOUS HEMANGIOMA IN A 16-YEAR OLD BOY

SAGITTAL T1/W MR IMAGE DEMONSTRATES AN INTRACONAL CAVERNOUS HEMANGIOMA POSTERIORLY(ARROW). CAVERNOUS HEMANGIOMAS MAY BE DIFFERENTIATED FROM LYMPHANGIOMA BECAUSE THEY ENHANCE GREATLY AFTER CONTRAST MATERIAL IS ADMINISTERED.

CAVERNOUS HEMANGIOMA

A homogenous well-defined intraconal mass is seen in the left orbit which is isointense on T1W , hyperintense on T2W sequence and reveals heterogeneous enhancement.

Cavernous hemangiomas are not uncommon in children

Optic nerve meningeoma

Tram-track

Glioma II

3T MRGlioma II

LEFT ORBITAL PLEXIFORM NEUROFIBROMA IN A 10-MONTH-OLD BOY

Axial proton-weighted (a) and coronal Ti-weighted (b) MR images demonstrate extensive involvement of the left eyelid and extraconal region by a plexiform neurofibroma

Optic nerve gliomas

in a teenage girl with neurofibromatosis

Axial Ti-weighted (a) and T2-weighted (b) MR images show diffuse bilateral enlargement ofthe optic nerves by gliomas (arrows)

NF-1

Radiograph of the orbit (a) reveals the characteristic enlarged and “bare” left orbit in a child with NF1

Axial CECT shows the dysplastic left greater wing of sphenoid with anterior herniation of the temporal lobe and an ill-defined infiltrative mass in the temporal fossa invading the orbit suggestive of a plexiform neurofibroma

Multifocal meningioma in an 18-year-old male adolescent with

neurofibromatosis

Axial CT scan shows a calcified meningioma of the right optic nerve

Coronal T1/W MR image demonstrates bilateral isointense intraventricular meningiomas

BILATERAL OPTIC NERVE MENINGIOMAS IN A 15-YEAR-OLD GIRL WITH NO OTHER FINDINGS OF NEUROFIBROMATOSIS

Axial CT scan reveals bilateral calcified meningiomas ofthe optic sheath

Optic nerve glioma in a young boy without neurofibromatosis

Axial CT scan shows diffuse involvement of the right optic nerve by a glioma. Pediatric optic nerve gliomas are frequently associated with neurofibromatosis

Schwannoma III

Extraorbital pathology

Schwannoma V2 with elevationof rectus inf. muscle

Angiofibroma with orbital infiltration

SCC

TRAUMA CT

Le Fort II

Orbital floor-fx with herniation of fat

Hematoma

Orbital floor fracture, Motility disturbance

Foreign bodiesNo MRI metallic !!!

Foreign bodies extraconal intraorbital

Foreign bodies

Foreign body sclera

Foreign bodies

Perforatingintraocular fb

Take home points

• Remind anatomic situation• Imaging technique and characteristics and

localisation of pathology (intraorbital compartments)

• Involvement of adjacent structures• Careful analysis DIAGNOSIS

Intraocular disorders

Calcified retinoblastoma

Axial CT scan demonstrates a calcified mass in the left globe, accompanied by some increased attenuation of the vitreous.

Bilateral retinoblastoma

Coronal CT scan and T1-w axial MR image demonstrate bilateral calcified retinoblastomas

The increased signal intensity of the right globe is likely secondary to hemorrhage

The calcifications so prominent on the CT scan are poorly visualized on the MR image

Trilateral retinoblastoma

AXIAL CECT SHOWS BILATERAL INTRAOCULAR MASSES WITH CALCIFICATION

WITH A SEPARATE INTENSELY ENHANCING MASS IN THE PINEAL LOCATION

Medulloepithelioma

Persistent hyperplastic primary vitreous ( PHPV )in a 3-year-old boy

Axial contrast material-enhanced CT scan shows a coneshaped, noncalcified, central retrolental area of increased attenuation in the right eye

Coronal T2/W MR image better depicts this abnormality. The increased signal intensity in the right globe is due to hemorrhage

PHPV

Transverse color Doppler USG shows an echogenic retrolental structure with a vascular channel within, suggestive of PHPV

RETROLENTAL FIBROPLASIA WITH BILATERAL MEDIAL RETINAL DETACHMENTS IN AN 1 1-MONTH-OLD GIRL WITH BILATERAL LEUKOKORIA

The infant, born prematurely, had received oxygen therapy for respiratory distress syndrome. Axial CT scan clearly shows the high-attenuation detached retinas (arrows).

Sclerosing endophthalmitis

Axial CT scan shows a uniform increased attenuation throughout the right globe. The linear area of high attenuation seen in the middle to lateral aspects of the globe is a detached retina. A classic nematode infection was confirmed at the histopathologic analysis. The lack of a focal mass and of calcification helps differentiate sclerosing endophthalmitis from retinoblastoma.

Coat’s disease

Color doppler USG shows a large retinal detachment with hypoechoic subretinal exudates

CT shows diffuse increase in the intraocular density

Orbital rhabdomyosarcoma in a young child

A large superior right orbital mass compressed and displaced the globe anteriorly and inferiorly. The mass is hypointense on the coronal T1/W image and hyperintense on the T2/W image

Orbital rhabdomyosarcoma

Axial CECT show a homogeneous multicompartmental soft tissue density mass causing orbital expansion and destruction of the medial orbital wall

Dermoid and Epidermoid cyst (different patients)

Axial CECT : A well-defined fat-containing lesion is seen near the outer canthus

Axial CECT : The large well-circumscribed cystic lesion at the inner canthus is suggestive of an epidermoid cyst. The adjacent bone is remodelled

Leukemic involvement of the orbit in a 6-month-old boy with acute lymphocytic leukemia

Axial CT scan shows preseptal swellingand involvement the left orbit, diffusescleral thickening with enhancement, and apoorly defined intraconal mass that envelopsthe optic nerve. There is resultant proptosis.

NEUROBLASTOMA IN A 14-YEAR-OLD BOY

Axial CT scan reveals diffuse sclerotic bone involvement from metastatic neuroblastoma. In addition, there is extraconal involvement about the right orbit with resultant proptosis.

Midline granuloma

Coronal CT scan demonstrates a midline mass with encroachment into the left orbit.

Langerhan’s cell histiocytosis

Axial CECT shows destruction of lateral orbital walls and the greater wing of the sphenoid bone on both sides with associated complex soft tissue mass encroaching on the orbits.

The skull radiograph in the same patient reveals multiple well-defined lytic lesions in the cranial vault with typical bevelled edges

Langerhans ‘cell Histiocytosis

( Orbital LCH )

1953 Lichtenstein

Bone or bone marrow lesions ( Overall incidence 23% )

Most commonly in frontal bone ( superior or superolateral wall

of orbit )

- An osteolytic lesion or multiple lesions

- Well defined or diffuse soft tissue mass , encroaching

lacrimal gland , lateral rectus or even the globe

CT and MRI findings :

Differential diagnosis :

- Rhabdomyosarcoma

- Juvenile fibrosarcoma

- Aggressive fibromatosis

- Lacrimal gland tumor

- Leukemic infiltration

- Metastasis ( Neuroblastoma , Wilm's , Ewing's)

Bilateral Anophthalmia

MICROPHTHALMOS IN A 13-YEAR-OLD BOY

CT scan shows the small right globe

The presence of the radiopaque lens differentiates microphthalmos from an orbital cyst

Sagittal T1/W MR image reveals a small cyst adjacent to the insertion of the optic nerve (arrow)

Microphthalmos and persistent hyperplastic primary vitreous (PHPV) are also present

Posterior optic coloboma in a 2-year-old girl with

seizures

Bilateral coloboma

Axial CECT shows defect of the right globe at the optic nerve head via which the vitreous projects posteriorly (arrow). The

left globe is small, with a retrobulbar (colobomatous) cyst (arrow) and dystrophic calcification at the site of coloboma

Duplication cyst

Congenital cystic eye

Axial CECT shows a large right orbital cyst with absence of the globe. Also note the microphthalmia with coloboma and dysplastic lens on the left side

Septo-optic dysplasia in a 22-month-old deaf, mute, and blind boy

Sagittal midline T1/W MR image demonstrates agenesis of the corpus callosum and a prominent anterior inferior recess ofthe third ventricle (arrow).

Axial T1/W MR image shows bilaterally small globes (left larger than the right), with hemorrhage in the left globe. Since no trauma had occurred, the presence of hemorrhage may indicate PHPV is present

Septo - optic dysplasia :

- A part of a spectrum of developmental and congenital brain

abnormalities

- Absence of the septum pellucidum and pituitary -

hypothalamic endocrine dysfunction and hypoplasia of the

optic nerves

Trigonocephalysecondary to metopic suture synostosis

Axial CT scan obtained at the level of the orbit roofs demonstrates a marked frontal and rbital asymmetry secondary to cranial synostosis

Apert syndrome in a 3-week-old boy

Anterior radiograph of the skull demonstrates the typical features ofApert syndrome

Axial CT scan demonstrates the proptosis and shallow orbits

Orbital encephalocele in a newborn

Axial CT scan demonstrates an encephalocele protruding throughthe left orbit. A left globe was not seen, although retinal remnants were found within the encephalocele atautopsy

Coronal CT scan demonstrates a prominent encephalocele that hasballooned inferiorly into the nasal cavity and medially into both orbits (arrows).

Metastatic disease :

- Most common primary tumor is breast , then lung

- In any part of the orbit , bone or soft tissue

- Findings may be subtle , with small focal areas

of thickening of the globe to large destructive lesion