OPHTHALMOPLEGIA

Lesions of 1. Oculomotor nerve (III) innervates medial rectus, superior rectus, inferior rectus, inferior oblique muscle, pupilloconstrictor, levator palpebrae 2. Trochlear nerve (IV) innervates superior oblique muscle 3. Abducens nerve (VI) innervates lateral rectus muscle

ANOPIA
[numbers refer to drawing] A. MONOCULAR DEFECTS 1 = monocular blindness (optic nerve lesion in fracture of optic canal, amaurosis fugax) B. BILATERAL HETERONYMOUS DEFECTS 2 = bitemporal hemianopia (chiasmatic lesion) C. BILATERAL HOMONYMOUS DEFECTS 3 = homonymous hemianopia 4 = upper right-sided quadrantanopia 5 = central hemianoptic scotoma 3,4,5 = most common type of hemianopia (CVA, brain tumor)

Monocular Blindness In Adulthood

1. Optic neuritis 2. Vascular ischemia

(a) Amaurosis fugax = cholesterol emboli from internal carotid artery occluding central retinal artery and its branches (b) Occult cerebrovascular malformation affecting the optic nerve 3. Temporal arteritis 4. Malignant optic glioma of adulthood

ORBIT
Spectrum of Orbital Disorders A. INFLAMMATORY DISEASE 1. Tissue-specific inflammation: orbital cellulitis, optic neuritis, scleritis, myositis, Graves disease 2. Panophthalmitis 3. Pseudotumor of orbit B. CYSTIC DISEASE 1) Dermoid cyst 2) Mucocele 3) Retro-ocular cyst (developmental)

C. VASCULAR DISEASE

Cavernous angioma 2. Capillary angioma 3. Lymphangioma 4. Varix 5. Carotid-cavernous fistula D. TUMORS 1. Rhabdomyosarcoma 2. Optic nerve glioma 3. Meningioma 4. Lymphoma 5. Metastasis
1.

Intraconal Lesion
mnemonic: Mel Met Rita Mending Hems On Poor Charlies Grave

Melanoma Metastasis Retinoblastoma Meningioma Hemangioma Optic glioma Pseudotumor Cellulitis Grave disease

Intraconal Lesion with Optic Nerve Involvement

1. 2. 3.

4.
5. 6. 7.

Optic nerve glioma Optic nerve sheath meningioma (10% of orbital neoplasm) Optic neuritis Inflammatory pseudotumor (may surround optic nerve) Sarcoidosis Intraorbital lymphoma (may surround optic nerve, older patient) Elevated intracranial pressure
= distension of optic sheath bilateral tortuous enlarged optic nerve-sheath complex

OPTIC NERVE TRAM-TRACK SIGN

1. Optic nerve sheath meningioma 2. Orbital pseudotumor 3. Preoptic neuritis

4. Perioptic hemorrhage
5. Sarcoidosis 6. Lymphoma / leukemia

7. Metastasis
8. Erdheim-Chester disease = systemic

xanthogranulomatosis

Intraconal Lesion without Optic Nerve Involvement Cavernous hemangioma 2. Orbital varix 3. Carotid-cavernous fistula 4. Arteriovenous malformation least common of orbital vascular malformations (congenital, idiopathic, traumatic) irregularly shaped intensely enhancing mass of enlarged vessels associated with dilated superior / inferior ophthalmic vein
1.

Intraconal Lesion without Optic Nerve Involvement

Hematoma 6. Lymphangioma 7. Neurilemoma commonly adjacent to superior orbital fissure local bone erosion
5.

Extraconal Lesion
Extraconal-intraorbital Lesion A. BENIGN TUMOR 1. Dermoid cyst 2. Teratoma <1% of all pediatric orbital tumors areas of fat, cartilage, bone expansion of boney orbit bone defect 3. Capillary hemangioma 4. Lymphangioma 5. Plexiform neurofibroma 6. Inflammatory orbital pseudotumor 7. Histiocytosis X lesion usually arises from bone

B.

MALIGNANT TUMOR 1. Lymphoma / leukemia 2. Metastasis 3. Rhabdomyosarcoma mnemonic : MOLD Metastasis Others (rhabdomyosarcoma, lymphangioma, sinus lesion) Lymphoma, Lacrimal gland tumor Dermoid

Extraconal-extraorbital Lesion A. FROM SINUS maxillary / sphenoid sinuses are rare locations of origin 1. Tumor: squamous cell carcinoma (80%), lymphoma, adenocarcinoma, adenoid cystic carcinoma 2. Mucocele

3. Paranasal sinusitis:

Most common cause of orbital infection! Origin: from ethmoid sinuses (in children), from frontal sinus (in adolescence) Organism: Staphylococcus, Streptococcus, Pneumococcus preseptal / orbital edema / cellulitis subperiosteal / orbital abscess mucormycosis (in diabetics) destroys bone + extends into cavernous sinus

Cx: (1) epidural abscess (2) subdural empyema (3) cavernous sinus thrombosis (4) meningitis (5) cerebritis (6) brain abscess B. FROM SKIN 1. Orbital cellulitis C. FROM LACRIMAL GLAND mass arising from superolateral aspect of orbit

Orbital Mass
Orbital Mass in Childhood
1. Dermoid cyst............................ 46% 2. Inflammatory lesion................ 16% 3. Dermolipoma............................ 7% 4. Capillary hemangioma............ 4% 5. Rhabdomyosarcoma................. 4% 6. Leukemia / lymphoma............. 2% 7. Optic nerve glioma................... 2% 8. Lymphangioma........................ 2% 9. Cavernous hemangioma.......... 1%

mnemonic: LO VISHON Leukemia, Lymphoma Optic nerve glioma Vascular malformation: hemangioma, lymphangioma Inflammation Sarcoma: ie, rhabdomyosarcoma Histiocytosis Orbital pseudotumor, Osteoma Neuroblastoma

Primary Malignant Orbital Tumors

1. Retinoblastoma 2. Rhabdomyosarcoma 3. Uveal melanoma 4. Sarcoma 86.0% 8.1% 2.3% 1.7%

Secondary Malignant Orbital Tumors

1. Leukemia...................................... 2. Sarcoma........................................ 3. Hodgkin lymphoma ..................... 4. Neuroblastoma............................. 5. Wilms tumor................................. 6. Non-Hodgkin lymphoma ...... 7. Histiocytosis................................. 8. Medulloblastoma.......................... 36.7% 14.3% 11.0% 9.2% 6.7% 5.6% 3.9% 3.5%

Orbital Cystic Lesion

1. Abscess 2. Intraorbital hematoma 3. Dermoid cyst 4. Lacrimal cyst 5. Lymphangioma 6. Hydatid cyst

Orbital Vascular Tumors

1. Orbital varix 2. Arteriovenous malformation 3. Carotid-cavernous fistula 4. Hemangioma: capillary /cavernous 5. Blood cyst 6. Arterial malformation 7. Glomus tumor 8. Hemangiopericytoma

Mass in Superolateral Quadrant of Orbit

1. 2. 3. 4. 5. 6. 7. 8. 9. Lacrimal gland tumor Dermoid cyst Metastasis (breast, prostate, lung) Lymphoma Leukemic infiltration of lacrimal gland Sarcoidosis Wegener granulomatosis Pseudotumor Frontal sinus mucocele

Extraocular Muscle Enlargement

A. ENDOCRINE 1. Graves disease (50%) 2. Acromegaly B. INFLAMMATION 1. Myositis rapid onset of proptosis, erythema of lids, conjunctival injection Location: single muscle (in adults); multiple muscles (in children) enlarged extraocular muscle positive response to steroids 2. Orbital cellulitis 3. Sjgren disease, Wegener granulomatosis, lethal midline granuloma, SLE 4. Sarcoidosis 5. Foreign-body reaction

Extraocular Muscle Enlargement

C. TUMOR 1. Pseudotumor 2. Rhabdomyosarcoma 3. Metastasis, lymphoma, leukemia D. VASCULAR 1. Spontaneous / traumatic hematoma 2. Arteriovenous malformation 3. Carotid-cavernous sinus fistula

GLOBE
Spectrum of Ocular Disorders
A. CONGENITAL 1. Persistent hyperplastic primary vitreous 2. Coats disease 3. Coloboma 4. Congenital cataract

Spectrum of Ocular Disorders

B. VITREORETINAL 1. Vitreous hemorrhage 2. Retinal detachment 3. Choroidal detachment 4. Endophthalmitis 5. Retinoschisis 6. Retrolental fibroplasia

Spectrum of Ocular Disorders

C. TUMOR 1. Retinoblastoma 2. Choroidal hemangioma 3. Retinal angiomatosis 4. Melanocytoma 5. Choroidal osteoma D. TRAUMA

Microphthalmia
= congenital underdevelopment / acquired diminution of globe

A. BILATERAL with cataract

1. Congenital rubella 2. Persistent hyperplastic vitreous 3. Retinopathy of prematurity 4. Retinal folds

5. Lowe syndrome
small globe + small orbit

Microphthalmia
B. UNILATERAL

1. Trauma / surgery / radiation therapy

2. Inflammation with disorganization of eye (phthisis bulbi) shrunken calcified globe + normal orbit

Macrophthalmia
= enlargement of globe A. WITHOUT INTRAOCULAR MASS (a) generalized enlargement 1. Axial myopia (most common cause) enlargement of globe in AP direction thinning of sclera 2. Buphthalmos 3. Juvenile glaucoma 4. Connective tissue disorder: Marfan syndrome, Ehlers-Danlos syndrome, Weill-Marchesani syndrome (congenital mesodermal dysmorphodystrophy), homocystinuria "wavy" contour of sclera

Macrophthalmia
(b) focal enlargement 1. Staphyloma 2. Apparent enlargement due to contralateral microphthalmia B. WITH INTRAOCULAR MASS (rare cause for enlargement) (a) with calcifications: 1. Retinoblastoma (b) without calcifications: 1. Melanoma 2. Metastasis

Ocular Lesion
Intraocular Calcifications
1. Retinoblastoma (>50% of all cases) 2. Astrocytic hamartoma 3. Choroidal osteoma 4. Optic drusen 5. Scleral calcifications (a) in systemic hypercalcemic states (HPT, hypervitaminosis D, sarcoidosis, secondary to chronic renal disease) (b) in elderly: at insertion of extraocular muscles 6. Retrolental fibroplasia

Ocular Lesion
7. Phthisis bulbi secondary to trauma or infection small contracted calcified disorganized nonfunctioning globe mnemonic: NMR CT Neurofibromatosis Melanoma (hyperdense melanin) Retinoblastoma Choroidal osteoma Tuberous sclerosis

Ocular Lesion
Noncalcified Ocular Process
1. Uveal melanoma 2. Metastasis 86% of ocular lesions within globe; usually in vascula choroid Origin: breast, lung, GI tract, GU tract, cutaneous melanoma, neuroblastoma 7 bilateral in 30% 3. Choroidal hemangiom

Noncalcified Ocular Process

4. Vitreous lymphoma diffuse ill-defined soft-tissue density 5. Developmental anomalies (a) Primary glaucoma = enlargement of eye seconda to narrowing of Schlemm canal (b) Coloboma (c) Staphyloma

Vitreous Hemorrhage
Cause: trauma, surgical intervention, arterial hypertension, retinal detachment, ocular tumor. Coats disease visual loss frequent US: numerous irregular, poorly defined low-intensity echoe; echogenic material moving freely within vitreous chamber during eye movement voluminous hyperechoic fibrin clots not fixed to optic nerve (DDx to retinal detachment) Prognosis: complete absorption / development of vitreous membranes (repetitive episodes) Cx: retinal detachment (vitreous traction secondary to fibrovascular ingrowth following hemorrhage)

Dense Vitreous in Pediatric Age Group

1. Retinoblastoma 2. Persistent hyperplastic primary vitreous 3. Coats disease 4. Norrie disease 5. Retrolental fibroplasia 6. Sclerosing endophthalmitis

Leukokoria
= abnormal white / pinkish / yellowish pupillary light reflex [leuko, Greek = white and koria, Greek = pupil] A. TUMOR 1. Retinoblastoma (most common cause - 58%) 2. Retinal astrocytic hamartoma (3%): associated with tuberous sclerosis + von Recklinghausen disease 3. Medulloepithelioma (rare)

B. DEVELOPMENTAL 1. Persistent hyperplastic primary vitreous (2nd most common cause - 28%) 2. Coats disease (16%) 3. Retrolental fibroplasia (3-5%) 4. Coloboma of choroid / optic disc C. INFECTION 1. Uveitis 2. Larval granulomatosis (16%) D. DEGENERATIVE 1. Posterior cataract

E. TRAUMA 1. Retinopathy of prematurity (5%) 2. Organized vitreous hemorrhage 3. Long-standing retinal detachment Leukokoria in Normal-sized Eye A. CALCIFIED MASS 1. Retioblastoma 2. Retinal astrocytoma B. NONCALCIFIED MASS 1. Toxocaral endophthalmitis 2. Coats disease

Leukokoria with Microphthalmia A. UNILATERAL 1. Persistent hyperplastic primary vitreous (PHPV) B. BILATERAL 1. Retinopathy of prematurity 2. Bilateral PHPV

OPTIC NERVE
Optic Nerve Enlargement A. TUMOR: 1. Optic nerve glioma 2. Optic nerve sheath meningioma 3. Infiltration by leukemia / lymphoma B. FLUID: 1. Perineural hematoma 2. Papilledema of intracranial hypertension 3. Patulous subarachnoid space

INFLAMMATION: 1. Optic neuritis 2. Sarcoidosis fusiform thickening = lens-shaped thickening of nerve-sheath complex (a) with central lucency: meningioma (b) without central lucency: optic nerve glioma
C.

 excrescentic thickening = single / multiple nodules along nerve-sheath complex usually due to tumor tubular enlargement = uniform enlargement of nerve-sheath complex (a) with central lucency: subarachnoid process (metastases, perineuritis, meningioma, perineural homorrhage) (b) without central lucency: papilledema leukemia, lymphoma, sarcoid, optic nerve glioma

LACRIMAL GLAND
Lacrimal Gland Lesion A. INFLAMMATION 1. Dacryoadenitis 2. Milulicz syndrome = nonspecific enlargement of lacrimal + salivary glands Associated with: sarcoidosis, lymphoma, leukemia

Sjgren syndrome = lymphocytic infiltration of lacrimal + salivary glands decreased lacrimation, xerostomia Often associated with: rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis 4. Sarcoidosis B. TUMOR (a) benign: granuloma, cyst, benign mixed tumor (= pleomorphic adenoma) (b) malignant: malignant mixed tumor (= pleomorphic adenocarcinoma), adenoid cysitic, carcinoma, lymphoma, metastasis (rare)
3.

Lacrimal Gland Enlargement mnemonic: MELD Metastasis Epithelial tumor Lymphoid tumor Dermoid Bilateral Lacrimal Gland Masses mnemonic: LACS Lymphoma And Collagen-vascular disease Sarcoidosis

ORBITAL CONNECTIONS

Superior Orbital Fissure Boundaries (Grays Anatomy):

-

medial above below lateral

: : : :

sphenoid body lesser wing of sphenoid = optic strut greater wing of sphenoid small segment of frontal bone

Contents: (a) nerves: III oculomotor n. IV trochlear n. V1 ophthalmic branch of trigeminal n.: (a) lacrimal nerve (b) frontal nerve VI abducens n. sympathetic filaments of internal carotid plexus (b) veins: superior + inferior ophthalmic vein (c) arteries: 1. meningeal branch of lacrimal artery 2. orbital branch of middle meningeal artery

Inferior Orbital Fissure Location: between floor + lateral wall of orbit; connects with pterygopalatine + infratemporal fossa Contents: (a) nerves: infraorbital + zygomatic nn. branches from pterygopalatine ganglion (b) veins: connection between inferior orbital v. + pterygoid plexus

Optic Canal
completely formed by lesser wing of sphenoid Contents: (a) nerve: optic nerve (I) (b) vessel: ophthalmic a.

NORMAL ORBIT MEASUREMENTS Muscles medial rectus muscle Inferior rectus muscle superior rectus muscle lateral rectus muscle superior oblique muscle Superior ophthalmic vein axial CT coronal CT Optic nerve sheath retrobullar waist Globe position behind interzygomatic line

4.1 0.5mm 4.9 0.8mm 3.8 0.7mm 2.9 0.6mm 2.4 0.4mm

1.8 0.5mm 2.7 1.0mm 5.5 0.8mm 4.2 0.6mm

9.9 1.7mm

ORBITAL COMPARTMENTS
the orbital septum + globe divide orbit into A. ANTERIOR COMPARTMENT lids, lacrimal apparatus, anterior soft tissues B. P0STERIOR COMPARTMENT = RETROBULBAR SPACE = the cone consisting of extraorbital muscles + envelope of fascia divides retrobulbar space into (a) intraconal space (b) extraconal space

Orbital Spaces
globe: subdivided into anterior + posterior segments by lens

optic nervesheath complex: optic nerve surrounded by meningeal sheath as extension from cerebral meninges intraconal space: orbital fat, ophthalmic a., superior ophthalmic v., nerves I, III, IV, V1 , VI

conus:

incomplete fenestrated musculofascial system extending from bony orbit to anterior third of globe,consists of extraocular muscles + interconnecting fascia extraconal space: between muscle cone + body orbit containing fat, lacrimal gland, lacrimal sac, portion of superior ophthalmic v.