42ORBIT, EYELIDS, AND OCULAR ADNEXA

Lacrimal sac lies between the anterior (strongest) and posterior (most functionally and structurally important) arms of the MCT. The sac is anterior to the septum and is thus not intraorbital. The posterior lacrimal crest is lacrimal bone, and the anterior lacrimal crest is maxillary bone.

Classically, the fundus of the sac (above the MCT) enlarges with malignancy, and the body of the sac (below the MCT) enlarges with inflammation.

Lacrimal pump: mostly from the deep head of the pretarsal muscle

(Horner’s tensor tarsi), which inserts onto the posterior lacrimal crest and also encircles the canaliculi. The anterior head of the pretarsal orbicularis muscle inserts onto the anterior lacrimal crest. The preseptal orbicularis also has two medial heads, and the deep portion ( Jones’ muscle) is adherent to the lacrimal sac. Thus, with each blink and orbicularis contraction, the pretarsal portion of the muscle squeezes the canaliculi, and the preseptal portion of the orbicularis pulls open the lacrimal sac, pumping tears through the drainage system.

Signs and Symptoms

ABAXIAL GLOBE DISPLACEMENT

Upward: maxillary sinus tumor, lymphoma, capillary hemangioma, and rhabdomyosarcoma

Nasal: sphenoid wing meningioma

Downward: dermoid, encephalocele, arteriovenous shunt, varix, and mucocele

Temporal: lacrimal sac mass and ethmoid mucocele

AIDS-ASSOCIATED EYELID LESIONS Keratoacanthoma, molluscum contagiosum, and Kaposi’s sarcoma. All often have multiple lesions.

‘‘BIG, RED’’ EYELID LESIONS Capillary hemangioma, Kaposi’s sarcoma, hemangiopericytoma, Merkel cell CA (golf ball size), and pilomatricoma (hair cell tumor)

CALCIFIED LESIONS Phlebolith, varix, lymphangioma, chronic inflammation, pleomorphic adenoma, old hemangioma, meningioma, and dermoid. Calcification usually indicates chronicity.

CANALICULAR STENOSIS Infection (herpes simplex virus, trachoma, mononucleosis), inflammation (SJS, OCP), trauma (lacerations, chemical or thermal injury, probings), allergy, radiation therapy, tumors (rare), canaliculitis, and eyedrops (antivirals, strong miotics, epinephrine-containing)

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CHORISTOMA Normal tissue (albeit disorganized) in abnormal location (chorista if single-cell type), such as dermoid and lymphangioma (although some believe that there are lymphatics in the orbit)

CLASSIC MASS LOCATIONS

Central/intraconal orbit: ON glioma, meningioma, hemangioma, and neurolemmoma

Peripheral orbit: dermoid cysts, neurofibroma, pseudotumor, lymphoma, rhabdomyosarcoma, lacrimal gland tumors, and metastatic cancer

Subperiosteal: hematoma, mucocele, abscess, and eosinophilic granuloma

CYSTIC LESIONS Dermoid cyst (most common cause), lacrimal cysts, degenerated neoplasms, lymphangioma (‘‘chocolate cysts’’), and benign mixed cell tumor (cystic changes helps to differentiate from adenomatous neoplasm)

DEEP SUPERIOR SULCUS Anophthalmos, levator dehiscence, and fat atrophy

DEGENERATIVE DISEASES OF THE ORBIT Fat atrophy (50% of degenerative disease), fat prolapse (27%), myopia (11%), and others

DESTRUCTIVE BONE LESIONS Metastatic cancer (in children usually metastatic neuroblastoma), reparative granuloma, aneurysmal bone cysts, Ewing’s and osteogenic sarcoma, fibrosarcoma, histiocytosis X, (Langerhans’ cells histiocytoses, especially eosinophilic granuloma), leukemia, lymphoma, and sarcoidosis.

DISTICHIASIS Extra row of short, soft lashes posterior to normal lashes usually in meibomian orifices. May be congenital (autosomal dominant) or secondary to inflammatory conditions (SJS, OCP, chronic blepharitis, etc.).

DYSMOTILITY EOM palsy, myasthenia, CPEO, CN III (rule out aneurysm or tumor) or CN IV or VI palsies

ECTROPION Outward rotation of lid margin, typically lower lid. Signs and symptoms: tearing (no punctal apposition), ocular irritation, canthal dehiscence, inferior scleral show, punctal eversion, lid margin keratinization, and possible keratopathy.

Involutional: most common, age-related lid laxity with defect of lateral canthal tendon/medial canthal tendon, not tarsus. Treat with horizontal lid tightening or shortening (lateral tarsal strip procedure, fullthickness wedge resection), medial spindle, or two-snip procedure.

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44ORBIT, EYELIDS, AND OCULAR ADNEXA

Cicatricial: relative deficit of anterior lamella, often from UV-damaged tight skin, chemical/thermal injury, skin tumors, or inflammatory disease. Treat with skin graft, Z-plasty (changes vertical tension to horizontal), transposition flap, mid-face-lift, or lid shortening if mild.

Paralytic: CN VII palsy, botulinum toxin. Treat with lower lid recession and LTS with spacer to stiffen the lower lid and gold weight upper lid, lateral tarsorrhaphy, or Arion sling (silicone ocular cerclage).

Mechanical: tumor, edema. Treat underlying cause.

Congenital: rare; associated with blepharophimosis.

ENTROPION Inward rotation of lid margin, usually lower lid. Signs and symptoms: foreign body sensation, entropion worse in downgaze or with forcible closure. Check snap back, lateral commissure position, distraction, punctal position, fat atrophy, and orbicularis override (thickening), and evert the lid (scarring, fornix shortening, trachoma).

Involutional: may have several contributing factors, including CPF dehiscence (inferior cul-de-sac deepened or visible white line), retractor disinsertion or redundancy, horizontal laxity, overriding of preseptal orbicularis, fat atrophy enophthalmos. Involutional entropion of the lower lid is much like involutional ptosis of the upper lid. Often has a spastic component clinically from sustained squeezing or overriding orbicularis. Treat by reattaching the CPF (suture to inferior border of tarsus), lid shortening (LTS), tape applied to the skin, Quickert-Rathbun rotational sutures, and Bick or Wies procedure.

Cicatricial: deficient posterior lamella (conjunctiva or tarsus) as from chemical injury, OCP, SJS, or trauma. Treat underlying problem, then consider conjunctiva graft (hard palate gives stiffer graft; may also

harvest from mouth, vagina, or nasal mucosa, or use amniotic membrane, alloderm, or Gortex).

Congenital, developmental: usually posterior lamella shortening and anterior lamellar excess, with overriding of preseptal orbicularis onto pretarsus orbicularis. Tarsal kink is rare; more often from epiblepharon.

Epiblepharon: extra skinfold, usually in Asians, and usually worse in downgaze. Pulling eyelid down manually helps break spasm. Patients often grow out of it; otherwise, may reform eyelid crease.

Mechanical: treat underlying cause.

ENLARGING ORBITAL MASS Capillary or cavernous hemangioma, lymphangioma, hemangiopericytoma, varix, and rhabdomyosarcoma

ENOPHTHALMOS Scirrhous breast CA, trauma, floor fracture, congenital absence of sphenoid wing, postradiation therapy and postinflammatory orbital cicatrization

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EPICANTHUS Fold of skin covering medial canthus. Classified based on position of the fold:

Tarsalis: upper lid fold larger (Down syndrome)

Inversus: lower lid larger (blepharophimosis)

Palpebralis: equal, upper and lower

Supraciliaris: from brow to medial canthus

EPIPHORA NLDO, poor punctal position, reflex lacrimation from external disease or CN V irritation, pseudodeficiency of basic secretions, ‘‘crocodile tears’’ (tearing when hungry or eating) from aberrant regeneration of CN VII

EYELID EDEMA (MNEMONIC: CRAB HAD FISH) Chalazion, renal disease, angioneurotic edema, blepharochalasis, heart disease, allergy, dacryoadenitis, fat herniation, infection (conjunctivitis), superior vena cava syndrome, hypothyroidism

EYELID RETRACTION Thyroid eye disease is most common cause of unilateral and bilateral retraction (from stimulation of Mu¨ller’s muscle); may also be caused by Parinaud’s dorsal midbrain syndrome (Collier’s sign), recession of SR or IR, congenital retraction, surgical overcorrection of ptosis, Marcus Gunn jaw wink, hydrocephalus (such as convergence-retraction nystagmus in periaqueductal stenosis), sympathomimetic drugs, aberrant regeneration, cirrhosis, basal ganglia disease (Parkinson’s), or pseudoretraction from ptosis of other lid.

FACIAL ASYMMETRY Fibrous dysplasia, NF, osteosarcoma, congenital hypoplasia, craniofacial dysostoses, and silent sinus syndrome

FIVE ORBITAL COMPARTMENTS Subperiosteal, extraconal, intraconal, subtenons, and subarachnoid spaces

FOUR ORBITAL TUMORS TO EXCISE COMPLETELY (BECAUSE OF RISK OF RECURRENCE WITH MALIGNANT POTENTIAL) Benign mixed tumor (pleomorphic adenoma), hemangiopericytoma, fibrous histiocytoma, and schwannoma

FROZEN GLOBE Metastatic cancer, orbital phycomycosis, and sclerosing pseudotumor

HAMARTOMA Abnormal tissue ‘‘at home’’ in normal location (called hamartia if from single-cell line), such as cavernous hemangioma.

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46 ORBIT, EYELIDS, AND OCULAR ADNEXA

HYPEROSTOSIS OF BONE Prostate CA, orbital osteoma (associated with familial adenomatous polyposis and CHRPE lesions; refer for colonoscopy), meningioma (sphenoid wing 10x more common than ON sheath meningiomas), and fibrous dysplasia

INFILTRATIVE LESIONS Plexiform neurofibroma, lymphangioma, capillary hemangioma, metastatic cancer, malignant fibrous histiocytoma, lymphoma, leukemia, and amyloid

INFLAMMATORY LESIONS OF THE ORBIT 85% are thyroid orbitopathy; of the remaining nonthyroid lesions, most are pseudotumor (46%), infectious (36%), vasculitic (7%), ocular inflammation (5%), Tolosa-Hunt (2.6%), sarcoid (1.3%), or others.

MARCUS GUNN JAW WINKING Eye opens with jaw movement to the contralateral side; associated with aberrant regeneration, congenital ptosis, Duane’s syndrome, double-elevator palsy.

MULTIPLE ERUPTIVE SKIN OR EYELID LESIONS Such as seborrheic keratosis, fibroepithelial polyps or acanthoma; consider occult visceral cancer (such as Muir-Torre syndrome).

MUSCLE LESIONS, ENLARGED MUSCLES (MNEMONIC: MASSIVE MUSCLES ARE LIKELY TO CAUSE GIANT TROUBLE) Myositis, metastatic cancer, amyloid/acromegaly, lymphoma/lymphangioma, thyroid, carotidcavernous sinus fistula (arteriovenous shunts), granulomatosis lesions (such as Wegener’s granulomatosis), and trichinosis/trauma

NEUROGENIC TUMORS Wing meningiomas (30%), glioma (20%), neurofibroma (17%), schwannoma (12%), optic nerve sheath meningioma (9%), and others

ON MASS LESIONS Glioma, meningioma, neurofibroma, metastatic cancer, leukemia

ORBITAL HEMORRHAGE Trauma is primary cause; also varix, lymphangioma, bleeding diathesis, rickets or scurvy, barotrauma, or idiopathic.

ORBITAL LESIONS IN CHILDHOOD (0–20 YEARS OLD) Structural/ dermoid (11%), inflammatory (14%), thyroid (10%), and capillary hemangioma (9%), and others

ORBITAL LESIONS IN MIDDLE-AGED PATIENTS (20–60 YEARS OLD)

Thyroid (61%), neoplastic (12%), and others

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ORBITAL LESIONS IN OLDER ADULTS (>60 YEARS OLD) Thyroid (40%), neoplastic (27%), inflammatory (12%), and lymphoproliferative (11%), and others

PARASELLAR OR SPHENOCAVERNOUS SYNDROME Constellation of cranio-orbital signs from many etiologies encompassing:

Superior orbital fissure syndrome: CN III, IV, V1, VI palsies with spared CN II and CN V2 function. Etiology is similar to orbital apex syndrome.

Orbital apex syndrome: SOF syndrome plus ON involved. Causes include tumors (most commonly sphenoid wing meningioma or pituitary adenoma, also glioma), trauma, infection, Tolosa-Hunt (name is commonly used as pseudotumor of the apex but is actually a specific granulomatous inflammation around the carotid siphon), sarcoid, sphenoid mucocele, or posterior dermoid.

Cavernous sinus syndrome: variable signs of SOF or apex syndrome, especially if a Horner’s syndrome is present with CN III or VI

dysfunction. If CN V2 is involved, then lesion is in posterior cavernous sinus. Caused by carotid-cavernous sinus or dural fistula, carotid artery aneurysm, infection, or inflammation. Imitators include botulinum and Miller Fisher syndrome.

PRIMARY ORBITAL TUMORS PRESENTING BY AGE Childhood (51%), middle age (41%), older age (8%)

PROPTOSIS Graves’ disease (leading cause in adults), cellulitis (leading cause in children); also pseudotumor, tumors, and carotid–cavernous sinus fistulas. Bilateral: Graves’ disease (leading cause of bilateral proptosis), pseudotumor, Wegener’s granulomatosis, and neoplasm

PROPTOSIS, RAPID Orbital cellulitis, rhabdomyosarcoma, hemorrhage, and lymphangioma with hemorrhage

PSEUDOPROPTOSIS Ptosis of other lid, lid retraction, buphthalmos, myopia, familial, EOM palsy, contralateral enophthalmos

PSEUDOPTOSIS Dermatochalasis, brow ptosis, eyelid edema, chalazion or other tumor, contralateral lid retraction, hypotropia (e.g., double-elevator palsy), enophthalmos (e.g., blow-out fracture), phthisis bulbi, and microphthalmos

PTOSIS DIFFERENTIAL DIAGNOSIS Congenital (myogenic), CPEO, levator dehiscence, Horner’s syndrome, botulinum toxin, CN III palsy, trauma

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48 ORBIT, EYELIDS, AND OCULAR ADNEXA

PTOSIS, BLEPHAROPTOSIS Drooping eyelids, which may interfere with superior visual field. Surgical options depend on levator function. If unilateral, remember that surgery on one side may cause the contralateral lid to become more ptotic due to Herring’s law because the levator is innervated bilaterally by the caudate CN III nucleus.

Aponeurotic, involutional: acquired dehiscence of levator aponeurosis with age or from postoperative or other trauma, chronic edema, or blepharochalasis. Signs and symptoms include high lid crease, excellent levator function, thinning of lid superior to the upper tarsal plate. Treat with levator advancement.

Neurogenic: CN III palsy with levator paresis may be acquired (vascular, aneurysm, trauma, tumors, botulinum toxin) or present congenitally (ptosis is stationary; consider surgery after age 5; monitor for amblyopia). Horner’s syndrome affecting Mu¨ller’s muscle causes a minimal ptosis.

Aberrant regeneration of CN III: inferior division of CN III abnormally innervates superior division, and lid may be ptotic in primary gaze but retracts in medial or inferior gaze as the levator is stimulated with the MR or IR (pseudo-Graefe’s sign: the lid goes up when eye looks down).

Marcus Gunn jaw winking: aberrant levator muscle innervation with the ipsilateral lateral pterygoid muscle (CN V) causes a

synkinetic ptosis.

Myogenic: usually congenital (from poor levator development with a fatty infiltration and fibrosed levator muscle) but rarely acquired in CPEO, oropharyngeal dystrophy, muscular dystrophy, or myasthenia gravis (worse after prolonged upgaze). Poor muscle contraction and relaxation. Do surgery (usually frontalis sling) before school age.

Mechanical: levator hematoma, trauma to the levator, aponeurosis, or Mu¨ller’s muscle.

Others: anterior segment disease (e.g., corneal abrasion) or prolonged topical or subcutaneous steroid, pseudoptosis (contralateral retraction, etc.)

PULSATILE PROPTOSIS Carotid–cavernous sinus fistula or arteriovenous malformations (abnormal vascular flow), mucocele or encephalocele (transmission of normal intracranial pulsations), surgical removal of bone or neurofibromatosis (NF) 1 (absence of sphenoid wing)

PUNCTAL STENOSIS Many causes including infection, trauma, congenital, and secondary to drug reaction such as with chemotherapy (Taxol) and miotics (especially phospholine iodide). Consider two-snip procedure or silicone stent intubation.

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