Orbit	Bones	Related structures/miscellaneous
Roof	Sphenoid (lesser wing)	Lacrimal gland fossa
	Frontal	Trochlea
		Supraorbital notch (medial)
Lateral wall	Sphenoid (greater wing)	Lateral orbital tubercle of Whitnall
	Zygomatic	Strongest orbital wall
		Lateral orbital rim at equator of globe
Floor	Maxilla	Contains infraorbital nerve and canal
	Palatine	Forms roof of maxillary sinus
	Zygomatic
Medial wall	Sphenoid	Lacrimal sac fossa
	Maxilla	Adjacent to ethmoid and sphenoid sinuses
	Ethmoid	Posterior ethmoidal foramen
	Lacrimal	Weakest orbital wall

Optic nerve

orbital length = 25–30 mm; length from globe to optic foramen = 18 mm; width = 1.5 mm in globe, 3.5 mm posterior to lamina cribrosa (due to myelin), 5.0 mm with the addition of the optic nerve sheath (see Figure 4-2)

Proptosis

measure with Hertel exophthalmometer (Table 6-2)

Table 6-2 Hertel exophthalmometry measurements

	Mean (mm)	Upper Limit of Normal (mm)
Caucasian male	16.5	21.7
Caucasian female	15.4	20
African American male	18.5	24.7
African American female	17.8	23.0

Apertures

(Figures 6-1 to 6-3)

Figure 6-1 Bony anatomy of the orbit in frontal view.

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Figure 6-2 Orbital apex, superior and inferior orbital fissure. MR, medial rectus; IR, inferior rectus; LR, lateral rectus; SR, superior rectus; L, levator; SO, superior oblique. Note that the trochlear nerve lies outside the muscle cone.

Figure 6-3 Schematic representation of the landmarks, temporal view.

(From Bajandas FJ, Kline BK: Neuro-Ophthalmology Review Manual, Thorofare, NJ, Slack, 1988.)

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Figure 6-5 Orbital veins. Venous drainage from the orbit, in coronal view.

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Figure 6-6 Vascular supply to the eye. All arterial branches originate with the ophthalmic artery. Venous drainage is through the cavernous sinus and the pterygoid plexus.

Innervation of Eye

Sensory innervation provided by ophthalmic and maxillary division of trigeminal nerve (CN 5) (Figure 6-7)

Figure 6-7 Sensory nerves of the orbit, in lateral view.

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Maxillary nerve (V₂)

Passes through foramen rotundum, then passes through inferior orbital fissure

Divides into infraorbital nerve, zygomatic nerve, and superior alveolar nerve

Parasympathetic innervation

Controls accommodation, pupillary constriction, and lacrimal gland stimulation

Enters eye as short posterior ciliary nerves after synapsing in the ciliary ganglion

Sympathetic innervation

Controls pupillary dilation, vasoconstriction, smooth muscle function of eyelids and orbit, and hidrosis

Dysfunction: Horner’s syndrome (ptosis, miosis, anhidrosis, and vasodilation)

Nerve fibers follow arterial supply as well as long ciliary nerves

Sinuses

(Figure 6-8)

Figure 6-8 Relationship of the orbits to the paranasal sinuses: FS, frontal sinus, ES, ethmoid sinus; MS, maxillary sinus; SS, sphenoid sinus.

(Reprinted with permission from Slamovits TL: Basic and Clinical Science Course, Section 7: Orbit, Eyelids and Lacrimal System. American Academy of Ophthalmology, San Francisco, 1993.)

Frontal

Not radiographically visible before age 6

Drains into anterior portion of the middle meatus

Ethmoid

Multiple thin-walled cavities

First sinus to aerate

Anterior and middle air cells drain into the middle meatus

Posterior air cells drain into the superior meatus

Ethmoidal sinusitis is the most common source of infection that leads to orbital cellulitis

Sphenoid

Rudimentary at birth

Reaches full size after puberty

Optic canal is superior and lateral to sphenoid sinus

Drains into sphenoethmoid recess of each nasal fossa

Maxillary

Largest sinus

Roof contains the infraorbital nerve

Drains into middle meatus

Soft Tissues

(Figure 6-9)

Figure 6-9 Sagittal section of orbital tissues through the vertical recti.

(Adapted from Parks MM: Extraocular muscles. In Duane TD Clinical Ophthalmology, Philadelphia, Harper and Row, 1982.)

Periorbita

Periosteum is attached firmly at the orbital rim and suture lines

Arcus marginalis: fusion of periosteum and orbital septum at orbital rim

Fuses with the dura covering the optic nerve

Annulus of Zinn

Fibrous tissue ring arising from periorbita that surrounds the optic canal

Origin of the 4 recti muscles

Fuses with dura covering ON at apex

Adipose tissue

Surrounds most of orbital contents; divided by fine fibrous septa

Preaponeurotic fat pads: lie immediately posterior to orbital septum

2 FAT PADS IN UPPER LID: anterior to levator aponeurosis; nasal pad smaller and paler than central pad; with aging, nasal pad moves anteriorly and may cause bulging of upper nasal orbit

3 FAT PADS IN LOWER LID: anterior to capsulopalpebral fascia; inferior oblique muscle separates the nasal and middle pads; lateral pad is small and more inferior

Lacrimal gland

2 lobes: orbital (larger) and palpebral, separated by levator aponeurosis

Ducts from both lobes pass though the palpebral lobe and empty into the superior fornix

Innervation: secretomotor from superior salivatory nucleus via CN 7; sensory from CN 5; sympathetic from superior cervical ganglion via deep petrosal nerve, pterygopalatine ganglion, and zygomatic nerve

Blood supply: lacrimal artery

Accessory lacrimal glands: glands of Wolfring located in tarsus and glands of Krause located in the conjunctival fornices (10% of production)

Eyelid

Lamellae of upper eyelid: (Figure 6-10)

Anterior: skin, orbicularis

Posterior: tarsus, levator aponeurosis, Müller’s muscle, palpebral conjunctiva

Figure 6-10 Cross section of upper eyelid. Note position of cilia, tarsal gland orifices, and mucocutaneous junction.

(Reprinted with permission from Grand MG: Basic and Clinical Science Course, Section 2: Fundamentals and Principles of Ophthalmology. American Academy of Ophthalmology, San Francisco, 1993.)

Orbicularis oculi: (Figure 6-11)

Main protractor of the eyelid; acts as lacrimal pump; innervated by CN 7

3 anatomic parts: palpebral portion (pretarsal and preseptal) involved with involuntary blinking; orbital portion involved with voluntary, forced lid closure

PRETARSAL: overlies tarsus, originates from lateral orbital tubercle, forms muscle of Riolan (seen as gray line at lid margin)

SUPERFICIAL HEAD: medially inserts into anterior lacrimal crest, contributes to medial canthal tendon, laterally inserts into zygomatic bone

DEEP HEAD: medially inserts into posterior lacrimal crest, called Horner’s muscle, surrounds canaliculi facilitating tear drainage, laterally inserts into lateral orbital tubercle, contributes to lateral canthal tendon

PRESEPTAL: overlies orbital septum

Originates from anterior limb of the medial canthal tendon, and from posterior lacrimal crest and lacrimal sac fascia

Forms lateral palpebral raphe overlying the lateral orbital rim

ORBITAL: lies beneath skin

Thickest portion of orbicularis

Inserts at medial canthal tendon

Interdigitates with the frontalis muscle superiorly at eyebrow

Figure 6-11 The orbicularis and frontalis muscles.

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Other muscles of forehead and eyebrow

Frontalis: moves scalp anteriorly and posteriorly; raises eyebrows; innervated by CN 7

Corrugator: pulls medial eyebrow inferiorly and medially producing vertical glabellar wrinkle; originates from nasal process of frontal bone; inserts laterally into subcutaneous tissue; innervated by CN 7

Procerus: pulls forehead and medial eyebrow inferiorly producing horizontal lines in nose; interdigitates with inferior edge of frontalis; innervated by CN 7

Upper eyelid retractors

(Figure 6-13)

Whitnall’s ligament (superior transverse ligament): condensation of levator muscle sheath

Visible as horizontal white line 10 mm above superior edge of tarsus

Arises from the sheath of anterior portion of levator muscle

Medially attaches to trochlea

Laterally forms septa through stroma of lacrimal gland and attaches to inner aspect of lateral orbital wall at the frontozygomatic suture (10 mm above orbital tubercle [Whitnall’s tubercle]) and at the orbital tubercle

Acts to change direction of pull of levator and serves as check ligament to prevent excessive lid elevation

Müller’s muscle (superior tarsal muscle):

Posterior to levator aponeurosis

Sympathetic innervation

Originates from undersurface of levator approximately at level of Whitnall’s ligament

Inserts into upper border of tarsus

12–15 mm long; raises eyelid 2 mm

Peripheral arterial arcade found between aponeurosis and Müller’s muscle

Figure 6-13 The orbital septum inserts into the levator aponeurosis (arrows). The preaponeurotic fat pads are located posterior to the septum. In downgaze, the lid crease becomes attenuated (weakened), and in a normal young eyelid, the fold is absent.

(From Zide BW, Jelks BW: Surgical Anatomy of the Orbit. New York, Raven Press, 1985.)

Figure 6-14 The levator aponeurosis, and the medial and lateral canthal tendons.

(From Dutton JJ: Atlas of Clinical and Surgical Orbital Anatomy, Philadelphia, WB Saunders, 1994.)

Lower eyelid retractors

(Figure 6-15)

Capsulopalpebral fascia: analogous to levator aponeurosis

Originates from inferior rectus muscle sheath, divides as it encircles inferior oblique muscle, and then joins to form Lockwood’s ligament

Fuses with septum and inserts into inferior tarsus

Lockwood’s suspensory ligament: analogous to Whitnall’s ligament

Arises posteriorly from fibrous attachments to inferior side of the IR muscle and continues anteriorly as capsulopalpebral fascia

Medial and lateral horns attach to retinacula forming a suspensory hammock for the globe

Inferior tarsal muscle: analogous to Müller’s muscle

Sympathetic innervation

Arises from capsulopalpebral fascia

Figure 6-15 Anatomy of the lower eyelid retractors.

(Adapted from Hawes MJ, Dortzbach RK: The microscopic anatomy of the lower eyelid retractors, Arch Ophthalmol 100:1313-1318, 1982.)

Tarsus

Dense connective tissue plate in each eyelid; not cartilage

Contains meibomian glands

29 mm long and 1 mm thick; tapered at ends

Rigid attachments to periosteum medially and laterally

Superior tarsal plate: 10 mm in height

Inferior tarsal plate: 4 mm in height

Medial canthal tendon

Fusion of tendinous insertions of pretarsal and preseptal orbicularis muscles

Attaches to tarsal plates

Anterior limb: attaches to frontal process of maxillary bone and serves as origin of superficial head of pretarsal orbicularis; passes in front of lacrimal sac and attaches to anterior lacrimal crest

Posterior limb: attaches to posterior lacrimal crest; passes behind lacrimal sac. More important than anterior limb for normal medial canthal appearance and function (maintaining apposition of lids to globe)

Lateral canthal tendon

Broad band of connective tissue from lateral borders of upper and lower tarsal plates

Inserts onto lateral orbital tubercle of Whitnall; 3 mm superior to medial canthal tendon insertion

Eyelid margin

3 distinguishing landmarks

Lash line: 2–3 rows of lashes; 100 in upper lid, 50 in lower lid; 10-week growth, 5-month resting phase

Gray line: border of pretarsal orbicularis (muscle of Riolan); junction of anterior and posterior lamellae; vascular watershed

Meibomian gland orifices: 30 in upper lid, 20 in lower lid

Innervation

Sensory: CN V₁ (upper lid), CN V₂ (lower lid)

Motor: CN 3, CN 7, sympathetics

Lymphatic drainage

Submandibular nodes: drain medial

of upper lid, medial

of lower lid

Preauricular nodes: drain lateral

of upper lid, lateral

of lower lid

No lymphatic vessels or nodes are found within the orbit

Lymphatic vessels are found in the conjunctiva

Glands of the eyelids

(Table 6-3)

Eccrine: secretion by simple exocytosis

Holocrine: secretion by release of entire cellular contents (disruption of cell)

Apocrine: secretion by pinching or budding off of a portion of cellular cytoplasm

Table 6-3 Glands of the eyelids

Nasolacrimal System

(Figure 6-17)

Figure 6-17 Excretory lacrimal system.

(From Grand MG: Basic and Clinical Science Course, Section 2, Fundamentals and Principles of Ophthalmology, San Francisco, American Academy of Ophthalmology, 1993.)

Puncta

Upper and lower; 6 mm from medial canthus, lower is slightly more temporal

Slightly inverted against globe

Open into ampulla (2 mm long) oriented perpendicular to eyelid margin

Canaliculi

10 mm long (2 mm vertical segment [ampulla] and 8 mm horizontal portion parallel to lid margin)

Combine to form single common canaliculus in 90% of individuals

Valve of Rosenmüller prevents reflux from lacrimal sac into canaliculi

Nasolacrimal sac

10 mm long, occupies the lacrimal fossa

Lies between anterior and posterior crura of the medial canthal tendon, anterior to orbital septum

Lateral to middle meatus of nose

Nasolacrimal duct

15 mm long

Passes inferiorly, posteriorly, and laterally within canal formed by maxillary and lacrimal bones

Extends into inferior meatus, which opens under inferior turbinate (2.5 cm posterior to naris)

Partially covered by valve of Hasner

Lacrimal pump

Lids close: pretarsal orbicularis contracts, compresses ampulla, shortens canaliculus; punctum moves medially; lacrimal sac expands, creates negative pressure, draws fluid from canaliculus into sac

Lids open: muscles relax, lacrimal sac collapses, tears forced into nose, punctum moves laterally, tears enter canaliculus

Imaging

Ultrasound

Optimal sound wave frequency is 10 MHz

Higher frequencies give better resolution; lower frequencies provide better penetration (Table 6-4)

Table 6-4 Ultrasound characteristics of lesions

Good sound transmission	Poor sound transmission
Cavernous hemangioma	Metastatic cancer
Lymphangioma	Orbital pseudotumor
Mucocele	Glioma
Dermoid	Neurofibroma
High Reflectivity	Low Reflectivity
Neurofibroma	Metastatic cancer
Fresh hemorrhage	Orbital pseudotumor
Hemangioma	Cyst
Thyroid eye disease	Mucocele
	Varix
	Dermoid
	Lymphoma

MRI

Strong magnetic field results in alignment of nuclei of atoms with odd numbers of protons or neutrons

Radiofrequency pulse disturbs the alignment by energizing protons or neutrons

When pulse terminates, protons return to previous alignment and emit absorbed energy as radiofrequency signal, generating an image

Can provide axial, coronal, and sagittal views

Relaxation times

Longitudinal relaxation time (T1): time required for net bulk magnetization to realign itself along original axis

Blood, orbital fat, melanin, and mucus appear bright

Acute hemorrhage, vitreous, optic nerve sheath, and extraocular muscles appear dark

Most orbital tumors are hypointense to fat on T1-weighted images

EXCEPTIONS: mucinous lesions (orbital dermoid cyst, mucocele), lipoma, liposarcoma, melanoma, subacute hemorrhage

Gadolinium: bright white on T1-weighted images

Must perform fat suppression on T1-weighted images when gadolinium is used

Fat suppression renders orbital fat hypointense and permits better visualization of optic nerve and extraocular muscles

Transverse relaxation time (T2): mean relaxation time

Based on interaction of hydrogen nuclei within a given tissue

Provides more information about pathologic processes

Helps differentiate melanotic lesions from hemorrhagic process

Vitreous is bright on T2-weighted images

Blood and fat appear dark

Demyelinating plaques of MS are best seen with T2-weighted images (hyperintense foci, usually in periventricular white matter)

Advantages

better for soft tissues and posterior fossa, sagittal sections, no artifacts from bone or teeth, no ionizing radiation

Disadvantages

poor bony detail, longer imaging time, more expensive, certain contraindications

Contraindications

patients with retained metallic foreign body, pacemaker, aneurysm clip

CT Scan

Use with orbital trauma to detect foreign body or calcification, or for evaluation of orbital soft tissue lesion with suspicion of bony erosion

Bone and metal appear bright

Advantages

better bony detail and for acute hemorrhage, sinuses, and trauma; shorter imaging time; less expensive

Disadvantages

poor posterior fossa detail; no sagittal sections, ionizing radiation

(Table 6-5)

Table 6-5 CT and MRI characteristics of lesions

Most common orbital lesions with well-circumscribed appearance on CT and MRI	Most common orbital lesions with Ill-defined appearance on CT and MRI
Children:	Children:
Dermoid cyst	Capillary hemangioma
Lymphangioma	Orbital pseudotumor
Rhabdomyosarcoma	Plexiform neurofibroma
ON glioma	Leukemic infiltrate
	Eosinophilic granuloma
Adults:	Adults:
Cavernous hemangioma	Orbital pseudotumor
Neurofibroma	Metastasis
Neurilemmoma	Leukemic infiltrate
Fibrous histiocytoma	Primary malignant tumor
(Lymphoproliferative disorders)	Lymphoproliferative disorders

Radiographs

Specific views can be helpful, especially when CT is unavailable or to quickly rule out a metallic foreign body

Plain film radiography also detects sinus pathology, calcification, hyperostosis, and lytic lesions

Canthomeatal line

radiographic baseline of skull (line between lateral canthal angle and tragus of ear)

Waters view

occipitomental; head extended until canthomeatal line lies at 37° to the central beam; best view of orbital roof and floor

Caldwell view

posteroanterior view with central beam tilted 25° toward feet; best view of superior and lateral orbital rim, medial wall, and ethmoid and frontal sinuses

Submental vertex view

basal or Hirtz view with head tilted back until canthomeatal line is perpendicular to central beam; best view of sphenoid and ethmoid sinuses, nasal cavities, and zygomatic arch

Oblique (×2) views

PA view with central beam tilted 32° toward feet and 37° from perpendicular; best views of optic foramina

Orbital Disorders

Congenital and Developmental Anomalies

(See Ch. 5, Pediatrics / Strabismus)

Trauma

Orbital Contusion

Bruising from blunt trauma

Pain, decreased vision

Often have associated lid, orbit, and ocular injuries

Orbital Fractures

Fractures of orbital bones, often associated with ocular or intracranial injuries

Orbital floor (blow-out)

Maxillary bone

Posterior medial floor is weakest area

Findings: may have enophthalmos, diplopia, infraorbital hypesthesia, orbit or lid emphysema, and medial wall fracture

Complications: orbital contents may become entrapped in maxillary sinus with resultant restriction of ocular motility, diplopia, and globe ptosis

Medial wall

Maxillary, lacrimal, and ethmoid bones

Findings: may have depressed nasal bridge, telecanthus, floor fracture

Complications: epistaxis from injury of anterior ethmoidal artery, CSF rhinorrhea, lacrimal drainage injury, orbit or lid emphysema

Orbital apex

Can involve optic canal and superior orbital fissure

Complications: CSF rhinorrhea, carotid-cavernous fistula

Orbital roof

Uncommon

May affect frontal sinus, cribriform plate, and brain

Complications: CSF rhinorrhea, pneumocephalus

Zygomatic (tripod)

Fracture of zygoma at 3 sites (zygomaticomaxillary suture, zygomaticofrontal suture, and zygomatic arch) and orbital floor

Findings: discontinuity of orbital rim, flattening of malar eminence, enophthalmos, infraorbital hypesthesia, trismus, orbit or lid emphysema, downward displacement of lateral canthus

LeFort (I, II, III)

I: low transverse fracture of maxillary bone above teeth; no orbital involvement

II: pyramidal fracture of nasal, lacrimal, and maxillary bones; involves medial orbital floors

III: craniofacial dysjunction; involves orbital floor, lateral and medial walls; may involve optic canal

CT scan: identify and localize fracture and coexisting injuries

Treatment

Systemic steroids and antibiotics

Surgical repair depends on type, location, and severity of fracture and associated findings

Intraorbital Foreign Bodies

Commonly associated with intraocular and / or optic nerve injury

Inert material can be well tolerated and may be observed

Copper and organic material are poorly tolerated and must be removed

Often asymptomatic, but may have pain or decreased vision

CT scan (MRI is contraindicated for metallic foreign bodies)

Treatment

systemic antibiotics, tetanus booster, consider surgical removal

Orbital (Retrobulbar) Hemorrhage

Compartment syndrome due to bleeding in orbit with globe and nerve compression

Ocular emergency

Findings

pain, decreased vision, subconjunctival hemorrhage, proptosis, restriction of ocular motility, increased IOP, tense orbit

Degeneration

Atrophia Bulbi

Progressive degeneration and decompensation of globe following severe injury

3 stages: atrophia bulbi without shrinkage, atrophia bulbi with shrinkage, atrophia bulbi with disorganization (phthisis bulbi)

Increased risk of intraocular malignancy

Annual B scan to rule out malignancy

Findings

cataract, retinal detachment, hypotony, corneal edema, globe shrinkage, intraocular hemorrhage, inflammation, calcification, and disorganization

Treatment

topical steroid and cycloplegic for pain; consider retrobulbar alcohol injection or enucleation for severe pain

Infections

Bacterial

Preseptal cellulitis, orbital cellulitis (see Ch. 5, Pediatrics / Strabismus)

Fungal

Most commonly Phycomycetes

Aspergillosis

Septated branching hyphae

Disseminated form: occurs in immunosuppressed hosts; widespread necrotizing angiitis, thrombosis, endophthalmitis

Local form: sclerosing granulomatous infiltrative mass that originates in the sinus; proptosis, periorbital pain, visual loss

Treatment: surgical débridement, amphotericin B, flucytosine, rifampin (IV and oral)

Viral

Dengue fever

Flavivirus

75% with ocular soreness and pain on eye movement

Inflammation

Idiopathic Orbital Inflammation (Orbital Pseudotumor)

Idiopathic inflammatory disease of orbital tissues

Findings

acute orbital pain, lid erythema and edema, lacrimal gland enlargement, restricted eye movements, proptosis, diplopia, increased IOP; may have impaired vision from optic nerve involvement

Adults

usually unilateral; bilateral cases need workup for systemic vasculitis and lymphoproliferative disorders

Children

bilateral in 33%; commonly have headache, fever, vomiting, and lethargy; may have associated papillitis or iritis; work up usually not needed (see Ch. 5, Pediatrics / Strabismus)

Pathology

enlargement of extraocular muscles and tendons; patchy infiltrate of lymphocytes, plasma cells, and eosinophils

DDx

Diagnosis

Lab tests: eosinophilia, elevated ESR, positive ANA, CSF pleocytosis

CT scan: enlargement of extraocular muscles and tendons, ring sign (contrast enhanced sclera), enlarged lacrimal gland

B-scan ultrasound: may show acoustically hollow area corresponding to edematous Tenon’s capsule

Treatment

systemic NSAIDs, systemic steroids (60–120 mg oral prednisone, taper slowly over months); consider radiation if unresponsive to steroids, perform biopsy first; chemotherapy (cyclophosphamide)

Orbital myositis

localized to extraocular muscles MR and LR most commonly involved (33% each), IR 10%

Findings: diplopia, pain, proptosis, ptosis, conjunctival injection, and chemosis

Complications: fibrosis with strabismus

Tolosa-Hunt syndrome

localized to superior orbital fissure, optic canal, and cavernous sinus

Findings: painful ophthalmoplegia, decreased vision

Sclerosing orbital pseudotumor

fibrosis of orbit and lacrimal gland

Insidious onset

More steroid resistant

Pain is less common, and eye is often white and quiet

Associated with retroperitoneal fibrosis

A-scan ultrasound: low reflectivity (seen with both orbital pseudotumor and lymphoma)

Thyroid-Related Ophthalmopathy

Autoimmune disease with spectrum of ocular manifestations

Most common cause of unilateral or bilateral proptosis in adults

Most common cause of acquired diplopia in adults

Women affected 8–10× more often than men

Patient can be hyperthyroid (90%), euthyroid (5–10%), or hypothyroid (1%); most commonly associated with Graves’ disease; increased incidence in Graves’ patients who smoke (7× more likely)

Associated with myasthenia gravis (in 5% of patients with Graves’ disease)

Usually asymptomatic

Findings

eyelid retraction (90%), proptosis (63%), restrictive myopathy with diplopia; overaction of levator (secondary to restrictive myopathy of inferior rectus muscle); pseudoretraction (secondary to proptosis); lagophthalmos; lid lag on downgaze (von Graefe’s sign); dry eye (due to corneal exposure and infiltration of lacrimal glands); conjunctival injection and chemosis; corneal exposure (from combination of eyelid retraction, proptosis, and lagophthalmos); increased IOP on upgaze; choroidal folds or disc hyperemia; compressive optic neuropathy (<5%) with decreased acuity and color vision, RAPD, and VF defect

Werner Classification of Eye Findings in Graves’ Disease (NO SPECS):

No signs or symptoms

Only signs

Soft tissue involvement (signs and symptoms)

Proptosis

Extraocular muscle involvement

Corneal involvement

Sight loss (optic nerve compression)

Pathology

enlargement of extraocular muscles; patchy infiltrates of lymphocytes, monocytes, mast cells, and fibroblasts; fibroblasts produce mucopolysaccharides, which leads to increased water content of muscles; inflammation spares tendons (Figure 6-18)

Figure 6-18 Thyroid-related ophthalmopathy demonstrating thickened extraocular muscles with inflammatory cell infiltrate and fluid.

(Courtesy of Dr. RC Eagle, Jr. Reported in Hufnagel TJ, Hickey WF, Cobbs WH et al: Immunohistochemical and ultrastructural studies on the exenterated orbital tissues of a patient with Graves’ disease, Ophthalmology 91:1411-9, 1984.)

CT scan

enlargement of extraocular muscles sparing tendons; inferior rectus > medial rectus > superior rectus > lateral rectus > obliques (IMSLO)

Treatment

control underlying thyroid abnormality; ocular lubrication; consider lid taping or tarsorrhaphy; systemic steroids; radiation (effects take 2–4 weeks)

Surgery:

ORBITAL DECOMPRESSION: perform before strabismus surgery; 2, 3, or 4 walls; 80% will experience postoperative diplopia

STRABISMUS SURGERY: perform before lid surgery

INDICATIONS: diplopia, abnormal head position, large-angle strabismus

Strabismus must be stable for at least 6 months

Avoid if anterior inflammatory signs present

Recession of affected muscles is mainstay of treatment, avoid resections (can exacerbate restrictions already present)

Vertical muscle surgery can affect eyelid position (recession of inferior rectus can cause increased lid retraction due to connection of IR to lower lid retractors; recession of IR can decrease upper lid retraction in that the SR now has to work less against the tight IR [thus, the associated levator muscle is less stimulated, causing less eyelid retraction])

Beware late slippage of inferior rectus

Consider adjustable sutures

LID SURGERY: eyelid retraction repair; perform after orbital decompression and strabismus surgery

Vascular Abnormalities

Varix

(See Ch. 5, Pediatrics / Strabismus)

AV Fistula

Direct or indirect communication between previously normal carotid artery and venous structures of the cavernous sinus

Direct carotid-cavernous sinus fistula

high flow; associated with head trauma, especially basal skull fracture; due to spontaneous rupture of aneurysm

Findings: dilated corkscrew scleral / episcleral vessels, conjunctival injection and chemosis, elevated IOP on involved side, proptosis (may be pulsatile), orbital bruit that may be abolished by ipsilateral carotid compression, dilated tortuous retinal veins; may develop ischemic maculopathy or retinal artery occlusion, enlarged cup-to-disc ratio, ophthalmoplegia (often CN 6), anterior segment ischemia, blood in Schlemm’s canal

CT / MRI scan: dilated superior ophthalmic vein

Treatment (for severely affected patients): embolization, surgical ligation

Dural-sinus fistula

low-flow communication between meningeal branches of carotid artery and dural walls of cavernous sinus; often asymptomatic; associated with hypertension, atherosclerosis, and connective tissue diseases; may close spontaneously; need MRI / MRA

Tumors

(Table 6-6)

Table 6-6 Common orbital tumors

In Children (see Chapter 5, Pediatrics/Strabismus)	In Adults
90% are benign; 10% are malignant	Mucocele
Rhabdomyosarcoma (most common primary orbital malignancy)	Cavernous hemangioma
Capillary hemangioma (most common benign orbital tumor)	Meningioma
Lymphangioma	Fibrous histiocytoma
Neuroblastoma (most common metastatic orbital tumor)	Neurilemmoma
Dermoid (most common orbital mass)	Pleomorphic adenoma (BMT)
Teratoma	Lymphoid tumors
ON glioma	Metastatic tumors
Granulocytic sarcoma (‘chloroma’)
Burkitt’s lymphoma
Histiocytic tumors

Hamartomas

growth arising from tissue normally found at that site (e.g., nevus, neurofibroma, neurilemmoma, schwannomma, glioma, hemangioma, hemangiopericytoma, lymphangioma, trichoepithelioma)

Choristomas

growth arising from tissue not normally found at that site (e.g., dermoid cyst, dermatolipoma, ectopic lacrimal gland)

Cystic Tumors

(See Ch. 5, Pediatrics / Strabismus)

Vascular Tumors

Lymphangioma

(See Ch. 5, Pediatrics/Strabismus)

Capillary Hemangioma

(See Ch. 5, Pediatrics / Strabismus)

Cavernous Hemangioma

Most common benign orbital tumor in adults (usually middle-aged women)

Findings

slowly progressive proptosis; may induce hyperopia, may also have retinal striae, IOP elevation, strabismus, or optic nerve compression

Growth may accelerate during pregnancy

Pathology

encapsulated lesion composed of blood-filled cavernous spaces, lined by endothelial cells (Figure 6-19)

Figure 6-19 Cavernous hemangioma demonstrating large, blood-filled spaces and fibrous septa.

(Case presented by Dr. WC Frayer to the meeting of the Verhoeff Society, 1989. From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

well-demarcated, encapsulated, intraconal mass

MRI

hypointense to fat on T1-weighted images, hyperintense to fat and equivalent to vitreous on T2-weighted images

A-scan ultrasound

high internal reflectivity

Treatment

observation, surgical excision

Neural Tumors

Optic Nerve Glioma

(See Ch. 5, Pediatrics / Strabismus)

Neurofibroma

(See Ch. 5, Pediatrics / Strabismus)

Neurilemmoma (Schwannoma)

Encapsulated tumor consisting of benign proliferation of Schwann cells

No malignant potential

Occurs in middle-aged individuals

Lesion can be painful due to perineural spread and compression of nerve

Usually located in the superior orbit causing gradual proptosis and globe dystopia

Schwannomas can grow along any peripheral or cranial nerve, most commonly CN 8 (acoustic neuroma)

Rarely associated with neurofibromatosis

Pathology

stains with S-100

Antoni A: spindle cells arranged in interlacing cords, whorls, or palisades; contains Verocay bodies (collections of cells resembling sensory corpuscles) (Figure 6-20)

Antoni B: loose, myxoid; stellate cells with a mucoid stroma (Figure 6-21)

Figure 6-20 Antoni A pattern demonstrating palisading spindle cells with Verocray bodies (areas that appear acellular).

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Figure 6-21 Antoni B pattern demonstrating necrosis with inflammatory cells.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

well-circumscribed, fusiform mass

Treatment

complete surgical excision; can recur after complete surgical removal

Meningioma

Sphenoid wing meningioma

Most common tumor to spread to orbit from intracranial space

Findings: temporal fullness, proptosis, lid edema

CT scan: hyperostosis and calcifications

Treatment: observation, surgical excision

Rhabdomyosarcoma

(See Ch. 5, Pediatrics / Strabismus.)

Lymphoid Tumors

Spectrum of disorders characterized by abnormal proliferation of lymphoid tissue

20% of all orbital tumors; 90% are non-Hodgkins B-cell lymphoma

Usually occur in adults 50–70 years old; rare in children

Conjunctival or lacrimal gland lesions

Cause painless proptosis

Tissue biopsy with immunohistochemical studies required for diagnosis

All lymphoid lesions of orbit must have a workup for systemic lymphoma, including CBC with differential, serum protein electrophoresis (SPEP), physical examination for lymphadenopathy, CT of thoracic and abdominal viscera, and bone scan

Perform every 6 months for 2 years

Bone marrow biopsy (better than bone marrow aspirate)

Monoclonal proliferations do not all progress to systemic disease; some polyclonal proliferations do progress

Benign Reactive Lymphoid Hyperplasia

Findings

bilateral painless lacrimal gland enlargement, limitation of ocular motility, visual disturbances

Pathology

mature lymphocytes with reactive germinal centers; T cells, 60–80% with scattered polyclonal B cells; high degree of endothelial cell proliferation

Atypical Lymphoid Hyperplasia

Low-grade lymphoma, no mitotic activity

Pathology

follicles and polymorphous response consistent with benign process

40% develop systemic disease within 5 years

Orbital Lymphoma

Orbital lymphoma occurs primarily in adults and usually involves superior orbit (only Burkitt’s occurs in children); increased risk of systemic disease

Findings

limitation of ocular motility, painless lacrimal gland swelling, conjunctival salmon patches, visual changes; 17% bilateral

Pathology

atypical immature lymphocytes with mitoses; diffuse or follicular growth; monoclonal B-cell proliferations 60–90% with scattered or reactive T cells; involves reticuloendothelial system, including retroperitoneal lymph nodes (Figure 6-22)

Figure 6-22 A, Nodular lymphoid infiltrate. B, Higher magnification shows uniform lymphocyte infiltrate with mitotic figures.

(From Yanoff M, Scheie HG: Malignant lymphoma of the orbit – difficulties in diagnosis, Surv Ophthalmol 12:133-140, 1967.)

CT scan

putty-like molding of tumor; orbital tissues not displaced

Treatment

radiotherapy for localized orbital disease, chemotherapy for systemic involvement

Prognosis

location appears more important than histopathology for determining systemic involvement: lymphoma of eyelids (67% have systemic involvement) > orbit (35%) > conjunctiva (20%)

Malignant lymphoma

60% risk of systemic lymphoma

Plasmacytoma

Tumor composed of plasma cells

Pathology

plasmacytoid lymphocytes, immunoblasts, mature plasma cells

DDx

Waldenström’s macroglobulinemia, multiple myeloma, lymphoma with immunoglobulin production

Granulocytic Sarcoma (‘Chloroma’)

(See Ch. 5, Pediatrics / Strabismus)

Burkitt’s Lymphoma

(See Ch. 5, Pediatrics / Strabismus)

Systemic Lymphoma and Waldenström’s Macroglobulinemia

Solid infiltrating tumor with putty-like molding of tumor to pre-existing structures

Pathology

Dutcher bodies (intranuclear PAS-positive inclusions of immunoglobulin)

Fibro-osseous Tumors

Fibrous Dysplasia

(see Chapter 5, Pediatrics / Strabismus)

Ossifying Fibroma

Variant of fibrous dysplasia

Occurs in second and third decades of life

More common in women

Well-circumscribed, slow-growing, monostotic lesion

Pathology

vascular stroma containing lamellar bone with a rim of osteoid and osteoblasts

Osteoma

Dense bony lesions originating in the frontal and ethmoid sinus

Well-circumscribed, slow-growing mass

Symptoms secondary to sinus obstruction and intracranial or intraorbital extension

Pathology

lamellar bone with variable amounts of fibrous stroma

Histiocytosis X

(Langerhans’ cell histiocytosis) (see Ch. 5, Pediatrics / Strabismus)

Juvenile Xanthogranuloma

(JXG; nevoxanthoendothelioma) (see Ch. 5, Pediatrics / Strabismus)

Epithelial Lacrimal Gland Tumors

50% of lacrimal gland lesions are inflammatory and lymphoproliferative; contour around the globe

50% of lacrimal gland tumors are of epithelial origin

50% of epithelial tumors are benign pleomorphic adenomas

50% of malignant tumors are adenoid cystic carcinomas

Pleomorphic Adenoma (Benign Mixed Tumor)

Most common epithelial tumor of the lacrimal gland

Occurs in 4th–5th decade of life

More common in men

Slow onset (6–12 months)

Firm mass in lacrimal fossa with painless proptosis; globe often displaced medially and downward

Progressive expansile growth may indent bone of lacrimal fossa

Tumor growth stimulates periosteum to deposit a thin layer of new bone (cortication)

Pathology

proliferation of epithelial cells into a double layer, forming lumina with ductal and secretory elements; ductal inner cells secrete mucus; outer stromal cells give rise to fibrous stroma and osteoid and cartilaginous metaplasia, pseudoencapsulated with surface bosselations (Figure 6-24)

Figure 6-24 BMT demonstrating ductal structures in a myxoid stroma.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

well-circumscribed but may have nodular configuration

Treatment

complete en bloc excision without biopsy; incomplete excision may result in recurrence and malignant transformation into pleomorphic adenocarcinoma

Pleomorphic Adenocarcinoma (Malignant Mixed Tumor)

Long history of mass in lacrimal fossa

Occurs in elderly individuals

Findings

rapid, progressive, painful proptosis

Pathology

Treatment

radical orbitectomy and bone removal

Sinus Tumors

Sinus Mucocele

Cystic, slowly expanding sinus lesion

Entrapment of mucus in aerated space due to obstruction of sinus ostia

Exerts pressure on surrounding bony structures

May become infected (mucopyocele)

Causes of ostial obstruction: inflammation with scarring, congenital narrowing of ostia, trauma, osteomas, polyps, septal deviation, mucus retention cysts

Associated with cystic fibrosis

Must rule out encephalocele and meningocele

Findings:

Frontoethmoid sinus (most common location): outward and downward displacement of globe, fullness in superonasal and medial canthal regions, above the medial canthal tendon

Sphenoid and posterior ethmoid sinus: visual symptoms, retrobulbar pain; may have cranial nerve palsies; 50% have nasal symptoms

Maxillary sinus (rare): upward displacement of globe, erosion of orbital floor may cause enophthalmos

CT scan

homogeneous opacified cyst with bowing of sinus wall and attenuation/erosion of bone

MRI

highly variable signal intensities

Treatment

surgical excision with IV antibiotics; reestablish normal drainage; obliteration of sinus (only if frontal)

Sinus Carcinoma

Usually squamous cell

Most commonly from maxillary sinus

No early signs except for sinusitis

Late findings

nonaxial proptosis, epiphora, epistaxis, infraorbital anesthesia

Eyelid Disorders

Congenital Anomalies

(See Ch. 5, Pediatrics / Strabismus)

Inflammation

Hordeolum

Obstruction and infection of Zeiss (external hordeolum, stye) or meibomian (internal hordeolum, chalazion) glands

Painful erythematous eyelid swelling

Accumulation of secretions causes acute inflammatory response

Lipogranuloma formation (chalazion) often evolves from internal hordeolum

Pathology

epithelioid and giant cells surrounding empty lipid vacuoles, zonal granulomatous inflammation (zonal lipogranuloma) (Figure 6-26)

Figure 6-26 Chalazion demonstrating epithelioid and giant cells surrounding clear areas that contained lipid.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Treatment

warm compresses, topical antibiotic ointment, lid scrubs (for associated blepharitis); if no response, consider local steroid injection, or incision and curettage

Acne Rosacea

Idiopathic, chronic skin disorder affecting sebaceous glands of face (including meibomian glands)

Type IV hypersensitivity may play a role

Common between ages 40 and 60 years

Ocular involvement in >50%

Findings

chronic blepharitis, meibomitis, lid margin telangiectasia, conjunctival injection, abnormal tear film (foam, mucin particles, excess oil), decreased tear breakup time, meibomian gland plugging, recurrent chalazions; may develop keratitis with peripheral corneal infiltrates, vascularization, scarring, thinning, ulceration, and rarely perforation

Other findings

acne-like skin changes, telangiectatic vessels, papules, and rhinophyma

Pathology

granulomatous inflammation

Treatment

warm compresses and lid scrubs, oral doxycycline or erythromycin, oral flaxseed oil and omega-3 fatty acids, topical azithromycin, lubrication (for associated dry eye); topical metronidazole to facial skin; consider topical steroid

Contact Dermatitis

Inflammation of lid skin due to exogenous irritant or allergic hypersensitivity reaction

Symptoms

red, swollen, itchy periorbital skin

Findings

erythematous, scaling lesions; may have vesicles or weeping areas

Treatment

eliminate inciting agent; use mild steroid cream or tropical tacrolimus 0.1% (Protopic)

Infections

Molluscum Contagiosum

Shiny, white-yellow papule with central umbilication

Infection by poxvirus

Spread by direct contact; consider HIV in healthy adult

Usually asymptomatic

Can cause follicular conjunctivitis and punctate keratitis

Pathology

lobular acanthosis, large basophilic poxviral intracytoplasmic inclusions composed of nucleic acids from DNA virus (Figure 6-27)

Figure 6-27 Intracytoplasmic, eosinophilic molluscum bodies in epidermis; they become larger and more basophilic near the surface.

(Courtesy of Dr. WC Frayer. From Skin and lacrimal drainage system. In Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Treatment

surgical excision, cryo, incision and curettage

Phthiriasis Palpebrum / Pediculosis

Lice infection of lashes

Spread by direct contact, usually sexually transmitted

Produces blepharoconjunctivitis

Findings

follicles, injection, small white eggs (nits) and lice attached to lashes

Treatment

remove nits and lice, topical ointment to suffocate lice, delousing creams and shampoo

Verruca Vulgaris (Papilloma)

Pink, pedunculated or sessile mass

Associated with papillomavirus (HPV)

Usually asymptomatic

Often multiple and resolve spontaneously

Leprosy

Organism is acid-fast bacillus Mycobacterium leprae

Tuberculoid and lepromatous leprosy can affect lids

Findings

loss of lashes, trichiasis, ectropion, and exposure keratitis

Treatment

systemic antibiotics (dapsone, rifampin)

Malposition and Other Disorders

Blepharospasm

Bilateral, intermittent, involuntary contractions of orbicularis and facial muscles causing uncontrolled blinking; may cause functional blindness

Etiology unknown but may be due to abnormality of basal ganglia

Usually occurs in 5th–7th decade of life; female > male (3 : 1); associated with Parkinson’s disease

Absent during sleep

Meige’s syndrome

essential blepharospasm with facial grimacing

DDx

secondary blepharospasm (due to ocular irritation), hemifacial spasm (usually unilateral, due to compression of CN 7, can be caused by cerebellopontine angle [CPA] tumor, present during sleep), myokymia, Tourette’s syndrome, trigeminal neuralgia, basal ganglia disease, tardive dyskinesia

Consider CT / MRI scan to rule out posterior fossa lesion

Treatment

Botox (botulinum toxin type A) injections, surgery (excision of lid protractors or differential section of CN 7), medications (tetrabenazine, lithium, carbidopa, clonazepam)

Botox: derived from Clostridium botulinum, freeze-dried solution; subcutaneous injection, no more than 200 units/month

MECHANISM: blocks neuromuscular conduction by binding to receptors on nerve terminals and stops release of ACh by disrupting calcium metabolism; does not cross blood–brain barrier

INDICATIONS: blepharospasm (effective in 90%, lasts 3 months), hemifacial spasm (lasts 4 months), strabismus (lasts 1–8 weeks)

ADVERSE EFFECTS (no systemic toxicity): exposure keratopathy, ptosis, diplopia, flaccid lower lid ectropion

Blepharochalasis

Idiopathic inflammatory edema of the eyelids

Familial, most often in young women

Recurrent attacks of transient, painless eyelid edema resulting in atrophy, wrinkling, and redundancy of eyelid skin

May develop ptosis and herniation of orbital lacrimal gland

Dermatochalasis

Redundancy of eyelid skin with orbital fat prolapse due to involutional changes

Blepharoptosis (Acquired)

Eyelid malposition characterized by drooping upper eyelid

Myogenic

uncommon; due to local or systemic muscular disease (e.g., myotonic dystrophy, chronic progressive external ophthalmoplegia, myasthenia gravis)

Treat underlying condition; usually requires frontalis sling

Involutional (aponeurotic)

most common form of ptosis; disinsertion of levator aponeurosis due to aging or chronic inflammation; may be exacerbated by eye surgery or trauma

High eyelid crease with good levator function; ptotic lid in all positions of gaze (no lid lag on downgaze as in congenital cases); may have thinning of eyelid above tarsal plate

Neurogenic

due to CN 3 palsy, aberrant regeneration of CN 3 (Marcus Gunn jaw-winking ptosis), Horner’s syndrome, multiple sclerosis, ophthalmoplegic migraine

Treatment for Horner’s: shorten Müller’s muscle (Putterman [conjunctival-Müller’s muscle resection]) or Fasanella-Servat [tarsoconjunctival resection] procedure)

Mechanical

due to mass effect of orbital or eyelid tumors, dermatochalasis, blepharochalasis, cicatrix

Traumatic

due to trauma to levator aponeurosis; may have lagophthalmos from cicatricial changes

May improve spontaneously, therefore observe for 6 months

Examination

Palpebral fissure height (PF): normally 10–11 mm in primary gaze

Marginal reflex distance (MRD): distance between corneal light reflex and upper lid margin in primary gaze; normally 4 mm

Levator function (LF): distance of upper lid excursion while frontalis is immobilized; normal >12 mm, fair if 6–11 mm, poor if <5 mm

2.5% phenylephrine test: activates sympathetic fibers of Müller’s muscle; resulting lid elevation simulates position after Müller’s muscle resection

DDx

pseudoptosis (nanophthalmos, microphthalmos, phthisis bulbi, hypotropia, dermatochalasis, enophthalmos, contralateral eyelid retraction or proptosis)

Treatment

eyelid crutches, levator aponeurosis advancement, levator muscle resection, Müller’s muscle resection, frontalis suspension with autogenous fascia lata, banked fascia lata, or synthetic materials

Surgical complications

overcorrection or undercorrection, lid lag, lagophthalmos, exposure keratopathy

Eyelid Retraction

Upper eyelid at or above superior limbus

Lower eyelid exposing sclera

DDx

Treatment

lubrication; surgical repair (levator aponeurosis recession, levator myotomy, spacer insertion, full-thickness skin graft for lower eyelid, hard palate grafts for lower eyelid)

Ectropion

Eversion of eyelid margin; may cause keratinization and hypertrophy of conjunctiva

Symptoms

tearing, foreign body sensation, redness

Involutional

most common cause of ectropion; often in lower eyelid

Etiology: horizontal laxity, disinsertion of lower eyelid retractors

Diagnosis: snap-back test

Treatment: horizontal eyelid shortening, lateral canthoplasty, repair of lower eyelid retractors

Paralytic

occurs after CN 7 palsy

Frequent complaints of tearing from chronic reflex secretion

Treatment: lubrication, taping of temporal lower eyelid, moisture chamber goggles, lateral tarsorrhaphy, horizontal tightening procedure, hard palate mucosal graft for lower eyelid elevation, gold weight implantation to aid in closure of upper eyelid

Cicatricial

shortening of anterior lamella

Etiology: burns, trauma, tumor, infection, chronic inflammation causing secondary anterior lamellar contraction (acne rosacea, atopic dermatitis, eczema, herpes zoster dermatitis, scleroderma, epidermolysis bullosa, porphyria, xeroderma pigmentosum)

Treatment: revision and relaxation of cicatrix, horizontal tightening procedure, may require vertical lengthening with full-thickness graft

Mechanical

Etiology: tumors of eyelid, herniated orbital fat, poorly fitted spectacles, chronic edema

Treat underlying condition

Entropion

Inversion of eyelid margin

Lower eyelid entropion usually involutional

Upper eyelid entropion usually cicatricial

Symptoms

tearing, foreign body sensation, redness

Congenital

very rare; due to epiblepharon or tarsal kink (see Ch. 5, Pediatrics / Strabismus)

Usually does not require treatment

Spastic

associated with ocular inflammation, trauma, and prolonged patching

Squeezing of eyelids causes inward rolling of eyelid margin; ocular surface irritation perpetuates cycle

Treatment: taping of eyelid, cautery, Botox injection, Quickert suture

Involutional

Etiology: canthal tendon laxity (horizontal lid laxity, diagnose with snap-back test), eyelid retractor dehiscence (vertical lid laxity), overriding preseptal orbicularis muscle, involutional enophthalmos

Treatment: Quickert suture, horizontal lid-shortening procedure (Bick, lateral tarsal strip, marginal wedge resection), vertical lid-shortening procedure (Jones, Hotz, Wies marginal rotation), retractor advancement, excision of preseptal orbicularis

Cicatricial

shortening of posterior lamella

Etiology: ocular cicatricial pemphigoid, Stevens-Johnson syndrome, trachoma, herpes zoster dermatitis, surgery, trauma, chemical burns, miotics

Digital pressure on inferior border of tarsus corrects eyelid position in involutional entropion but not cicatricial entropion

Treatment: lubrication, avoid surgery during acute phase of autoimmune disease, remove lashes in contact with cornea, use tarsal fracture operation, apply tarsoconjunctival grafts or hard palate mucosal grafts to replace scarred tarsus; may require symblepharon ring and amniotic membrane graft to prevent recurrent scarring

Trichiasis

Misdirection of eyelashes and contact with ocular surface

May be associated with posterior lamellar scarring

Associated with distichiasis, entropion, OCP, Stevens-Johnson syndrome, chronic blepharitis, chemical burns

Symptoms

tearing, foreign body sensation, redness

Treatment

lubrication, epilation, electrolysis, cryodestruction with double-freeze/thaw technique, argon laser, full-thickness wedge resection

Floppy Eyelid Syndrome

Easily everted upper eyelids

Chronic papillary conjunctivitis due to lid autoeversion during sleep with resultant mechanical irritation from bed sheets

Associated with obesity, keratoconus, eyelid rubbing, and sleep apnea

Treatment

lubrication, tape/patch/shield lids during sleep; consider horizontal eyelid tightening

Madarosis

Loss of eyelashes and/or eyebrows due to local or systemic disorders

DDx

eyelid neoplasms, chronic blepharitis, trauma, burns, trichotillomania, alopecia, seborrheic dermatitis, chemotherapy agents, malnutrition, lupus, leprosy

Poliosis / Vitiligo

Premature whitening of the eyelashes / eyebrows (poliosis) or skin (vitiligo) due to local systemic disorders

Associated with Vogt-Koyanagi-Harada syndrome, sympathetic ophthalmia, Waardenburg syndrome, tuberous sclerosis, radiation, and dermatitis

Eyelid Tumors

Benign Epithelial Tumors

Squamous Papilloma

Keratinized epidermal fronds with fibrovascular cores

Most common benign lesion of eyelid

Associated with papovavirus (HPV) infection

May be sessile or pedunculated

Pathology

papillary configuration, proliferating fibrovascular tissue covered by hyperplastic prickle cell layer of the epidermis, hyperkeratosis and parakeratosis may be present, vacuolated cells containing virus particles may be seen in the upper squamous layer (Figure 6-28)

Figure 6-28 Squamous papilloma demonstrating acanthosis and hyperkeratosis.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Treatment

surgical excision, cryo or laser ablation

Seborrheic Keratosis

Papillomatous proliferation of suprabasalar (prickle) cells

Pigmented keratin crust with greasy ‘stuck-on’ appearance

Occurs in elderly

Pathology

acanthosis, hyperkeratosis, parakeratosis, and squamous eddies may be present (Figure 6-29)

Figure 6-29 Seborrheic keratosis appears as blue lesion above skin surface with blue basaloid cells and keratin cysts.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Inverted Follicular Keratosis

Nodular or verrucous lesion

Looks like a cutaneous horn

Inflamed seborrheic keratosis

Pathology

acanthosis, squamous and basal cell proliferation

Cysts

Epithelial lined chambers filled with debris

Epidermal Inclusion Cyst (Epidermoid Cyst)

Single-lumen, round or oval lesion lined by keratinized, stratified squamous epithelium (epidermis) and filled with cheesy keratin debris

Occurs after a fragment of epidermis is carried into the subepithelium by trauma

Trapped but viable epithelium proliferates and produces keratin

Rupture can cause a foreign body granulomatous reaction

Dermoid Cyst

Lined by keratinized squamous epithelium and dermal appendages such as hair shafts and sebaceous glands

Contains keratin, cilia, and sebum

Often congenital; most common orbital tumor in children

Can be found in eyelid or orbit

Hydrocystoma (Sudiforous Cyst)

Multilocular, branching lumen that appears empty or contains serous fluid

Lined by a double row of cuboidal epithelium (resembling sweat duct)

Most are eccrine hydrocystomas (can be apocrine if arising from the glands of Moll)

Occur most commonly at eyelid margin or lateral canthus

Sebaceous Gland Tumors

Congenital Sebaceous Gland Hyperplasia

Proliferation of normal sebaceous glands

20% degenerate into basal cell carcinoma

Acquired Sebaceous Gland Hyperplasia

Multiple well-circumscribed yellow nodules

Lobules of mature sebaceous glands around a dilated duct

Muir-Torre syndrome

multiple sebaceous neoplasms, keratoacanthomas, and visceral tumors (especially GI)

Sweat Gland Tumors

Syringoma

Waxy, yellow nodules on lower lid

Usually occur in young women

Benign proliferation of eccrine ductal structures

Hydrocystoma

Translucent, bluish cyst

Arises from eccrine or apocrine glands

Tumors of Hair Follicle Origin

Trichoepithelioma

Firm, skin-colored nodule

More common in women

Usually occurs on forehead, eyelids, nasolabial fold, and upper lip

Pathology

basaloid cells surrounding a keratin center

Multiple lesions

Brooke’s tumor (AD)

Trichofolliculoma

A keratin-filled dilated cystic hair follicle, surrounded by immature hair follicles

Appears as a small umbilicated nodule usually with central white hairs

Tricholemmoma

Small crusty lesion with rough ulcerated surface

Usually occurs on face

Arises from glycogen-rich clear cells of the outer hair sheath

May resemble basal, squamous, or sebaceous cell carcinoma

Cowden’s disease (AD)

multiple facial tricholemmomas; marker for breast (40%) or thyroid cancer

Pilomatrixoma (‘Calcifying Epithelioma of Malherbe’)

Solitary, firm, deep nodule with overlying normal, pink, or bluish skin

Freely movable subcutaneous pink-purple nodule

Most common cystic lesion of childhood

In young adult, arises from hair matrix of upper lid or brow

Occurs on the eyelid, face, neck, or arms

Can range from 5 to 30 mm in diameter

May resemble an epidermal cyst

Associated with myotonic dystrophy and Gardner’s syndrome

Precancerous Lesions

Nevus

Congenital or acquired hamartoma

Can be flat, but is usually elevated and pigmented

Arises from neural crest cells

Pigmentation and size tend to increase during puberty

With time, nevi tend to move deeper, migrating into the dermis

Contains benign-appearing dermal melanocytes

Malignant transformation is rare

Classified by location

Junctional nevus

occurs at epidermal/dermal junction; flat

Greatest malignant potential

Compound nevus

both intradermal and junctional components (Figure 6-31); slightly elevated or papillomatous, often pigmented

Junctional component gives malignant potential

Figure 6-31 Compound nevus with cells at junction and in dermis.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Intradermal nevus

most common, most benign; can be papillomatous, dome-shaped, or pedunculated

Slightly pigmented or amelanotic; hair shafts indicate intradermal variety

Kissing nevus

congenital; involves both upper and lower lids, secondary to fusion of lids during embryonic development

Spindle cell nevus

compound nevus of childhood; bizarre cellular components

No malignant potential

Giant hairy nevus of the face and scalp

congenital; thickened eyelids can cause amblyopia

Nevus of Ota (Oculodermal Melanocytosis)

(See Ch. 5, Pediatrics / Strabismus.)

Lentigo Maligna (Melanotic Freckle of Hutchinson)

Acquired cutaneous pigmentation

Often periocular

Occurs in middle-aged or older individuals

Conjunctival pigmentation may be noted

No episcleral pigmentation

Melanoma arises in approximately 30%

Cutaneous counterpart of primary acquired melanosis (PAM) of the conjunctiva

Xeroderma Pigmentosa (AR)

Defect in DNA repair (UV light endonuclease)

Freckles and scaling at early age

Susceptible to a variety of malignant tumors (basal cell carcinoma, squamous cell carcinoma, malignant melanoma, sarcoma)

3% incidence of skin malignant melanoma

Malignant Epithelial Tumors

Risk factors

increased age, sun exposure, fair skin, previous history of skin cancer, positive family history

Characteristics

irregularity, induration, and altered lid anatomy

Basal Cell Carcinoma (BCC)

Most common malignancy of the eyelid (90%)

40 times more common than squamous cell carcinoma

Develops on sun-exposed skin in elderly patients; smoking is also a risk

Location (in order of frequency)

lower lid (50–60%), medial canthus (20–30%), upper lid (15%), outer canthus (5%)

Poorest prognosis when found in medial canthus because tumor often extends deeper and can involve lacrimal drainage system

Morbidity and mortality occur from local invasion of skull and CNS

Rarely metastasizes

Pathology

blue basaloid tumor cells arranged in nests and cords (H&E stain), peripheral palisading commonly seen

(Figure 6-32)

Figure 6-32 A, BCC appears as blue nests of basal cells proliferating over pale pink desmoplasia (dermal fibroblast proliferation). B, Basal cell nests with peripheral palisading and mitotic figures. (Courtesy of HG Scheie. From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)