7 Oculoplastics and Orbital Disease



Orbital Dimensions

Volume: 30 mL.

Height: 35 mm.

Width: 40 mm.

Medial wall length: 45 mm.

Globe to optic canal: 18 mm.

Optic nerve: 5 cm.

Intraocular: 1 mm.

Intraorbital: 25 mm.

Intracanalicular: 10 mm.

Intracranial: 15 mm.

Orbital Bones

See Figure 7.1.

Orbital Apertures

See Figures 7.2 and 7.3, and Table 7.1.

Evaluation of Orbital Disorders

Axial displacement (Table 7.2).

Nonaxial displacement (Table 7.3).

Imaging (Table 7.4).

Destructive lesions of bone (Table 7.5).

Classification of orbital disorders (Tables 7.6 and 7.7).


Orbital Cellulitis


Bacterial and fungal infections of the orbit may occur in children (usually monomicrobial) and adults (polymicrobial) with vision-threatening and life-threatening sequelae. Preseptal cellulitis occurs only in the periorbital structures anterior to the orbital septum, unlike orbital cellulitis, which involves more posterior structures. Orbital cellulitis most commonly occurs in the setting of active paranasal sinus disease or trauma with associated infected facial wounds.


Periorbital structures: paranasal sinus, face and eyelid, lacrimal sac (dacryocystitis), or dental infections.

Intraorbital structures: endophthalmitis, dacryoadenitis.

Exogenous: trauma, surgery.

Endogenous: septic embolization.

Most common organisms: Staphylococcus aureus, Streptococcus sp., Haemophilus influenzae (in children), Gram-negative rods, Mucor (in diabetics), Aspergillus.

Signs and Symptoms

See Table 7.8 and Figure 7.4.

Special Tests

images  Cultures and/or Gram stain: blood, conjunctiva, nasopharynx, external wounds, cerebrospinal fluid, as necessary.

images  Complete blood cell count (CBC) with differential.

Table 7.2 Axial displacement


aAll causes listed below may cause unilateral proptosis as well.

Table 7.3 Nonaxial displacement


Table 7.5 Destructive lesions of bone


Table 7.6 Common orbital disorders


Table 7.7 Craniofacial deformities


images  Computed tomography (CT) scan: Order with contrast, evaluate preseptal versus postseptal location, look for paranasal sinus disease, subperiosteal abscess.

images  Biopsy for microscopy.

images  Fungus: Mucor—nonseptate hyphae, Aspergillus—septate branching hyphae.


images  Severe infection with genera Rhizopus, Mucor, Absidia belonging to the family Mucoraceae, a subset of the class Phycomycetes.

images  Blood vessel invasion, vascular occlusion, necrosis, black eschar (late finding) on roof of mouth or nose.

images  Begins in sinus—invades orbit.

images  Risk factors: diabetics in ketoacidosis or with antecedent bacterial infection, alcoholics, and immunocompromised patients.

images  Histopathology: nonseptate, large branching filamentous hyphae seen on periodic acid-Schiff and Gomori methenamine-silver stains.

images  Treatment: extensive debridement of necrotic tissues with irrigation with amphotericin B.

images  Intravenous amphotericin B (nephrotoxic, aplastic anemia).

Disease Course

images  May progress from preseptal to orbital cellulitis, orbital cellulitis to cavernous sinus thrombosis or intracranial abscess, without appropriate treatment. Must follow patients closely for development of deeper infection, decreased vision or ocular motility, or afferent pupillary defect.

Table 7.8 Signs and symptoms of orbital cellulitis


images  Cavernous sinus thrombosis: bilateral orbital involvement, decreased skin sensation affecting ophthalmic (V1) or maxillary (V2) divisions of the trigeminal nerve, complete ophthalmoplegia, headache, nausea, vomiting, and fever.

images  Subperiosteal abscess formation (commonly superomedial or inferomedial orbit) can result in sudden loss of vision with posterior extension of the abscess toward the orbital apex.

Treatment and Management

images  After cultures and blood work are obtained, the patient should be immediately placed on a regimen of antibiotics. Infectious disease consultation may be obtained.

images  Patients with preseptal cellulitis are occasionally treated as outpatients with oral antibiotics; otherwise, intravenous antibiotics are given.

images  Patients with sinus disease should be evaluated by an otolaryngologist.

images  Mucormycosis, a rare, rapidly progressive fungal orbital infection, should be suspected in all diabetics and immunosuppressed patients; definitive diagnosis and treatment should be performed immediately; treatment consists of orbital debridement, possible exenteration and local irrigation, and systemic treatment with amphotericin B.

images  A lag time of 24 to 48 hours commonly occurs between initiation of antibiotics and clinical response.

images  If there is no improvement or if new signs such as decreased vision or development of an afferent pupillary defect develop, repeat CT scan and suspect abscess formation or resistant organisms.

images  Abscesses in adults should be surgically drained, particularly in the setting of visual compromise; abscesses in children may be followed closely if under the age of 9, with surgical drainage for any deterioration.

images  Aspergillosis, a fungal infection that may be acute or chronic and local or disseminated, is treated with orbital debridement, possible exenteration and local irrigation, and systemic treatment with amphotericin.


Antibiotic choice depends on origin or cause of cellulitis.

images  Trauma: third-generation cephalosporin and clindamycin for Gram negative organisms Gram-positive skin flora, and anaerobes, respectively.

images  Sinus: second-generation cephalosporin for sinus bacteria.

images  Children: single aerobe, second-generation cephalosporin for H. influenzae.

images  Adults (>14 y/o): polymicrobial (average five species). Third-generation cephalosporin, nafcillin, or vancomycin (if methicillin-resistant S. aureus suspected), and metronidazole (crosses blood–brain barrier) or clindamycin for anaerobes.

images  Fungus: amphotericin B (liposomal amphotericin B reserved for immunocompromised host with renal failure), voriconazole (more effective for invasive Aspergillus).


images  Patients should be followed extremely closely until signs of improvement are evident, such as decreased lid edema and erythema, decreased chemosis, improved ocular motility, improved visual acuity, or decreased proptosis.

images  Intravenous antibiotics should be continued for approximately 1 week before switching to oral antibiotics.

Differential Diagnosis

images  Idiopathic orbital inflammation: no signs of infection.

images  Carotid cavernous fistula: no signs of infection.

images  Orbital vasculitis.


Bilyk J. Periocular infection. Curr Opin Ophthalmol 2007;18: 414–423.

Epstein VA, Kern RC. Invasive fungal sinusitis and complications of rhinosinusitis. Otolaryngol Clin N Am 2008;41(3): 497–524.

Harris GJ. Subperiosteal abscess of the orbit. Age as a factor in the bacteriology and response to treatment. Ophthalmology 1994;101(3):585–595.

Necrotizing Fasciitis


images  Periocular necrotizing fasciitis is a rapidly progressive infection that spreads along the subcutaneous soft tissue planes and that can eventually lead to shock and organ failure.


images  Idiopathic but has been associated with blunt or surgical trauma (blepharoplasty) in healthy patients.

images  Group β-hemolytic Streptococcus.

Signs and Symptoms

images  Mimics preseptal cellulitis initially but results in necrosis of adjacent tissue and cyanosis (subcutaneous hemorrhage and thrombosis).

images  Painful, erythematous, and/or blue eyelids.

images  Possible retinal artery occlusion due to thrombosis or ischemic necrosis of the deep orbit.

Treatment and Management

images  Subcutaneous debridement, resection of necrotic skin, and β-lactam antibiotics (penicillin, ceftriaxone, or clindamycin).


Elner VM, Demirci H, Nerad JA, et al. Periocular necrotizing fasciitis with visual loss. Ophthalmology 2006;113:2338–2345.

Thyroid Orbitopathy


Thyroid orbitopathy, the most common cause of unilateral or bilateral proptosis in adults, occurs in 50% of patients with Graves disease. Patients with thyroid orbitopathy may be hyperthyroid, hypothyroid, or even euthyroid. Ocular complications attributable to thyroid orbitopathy include ocular exposure, strabismus, and optic nerve compression.


images  Autoimmune disorder with antibodies to orbital fibroblasts demonstrated in some patients.

images  Fibroblasts can transform into adipocytes.

images  Stimulation of glycosaminoglycans or collagen production.

Signs and Symptoms

See Figure 7.5.

images  Lid retraction (early sign).

images  Lid lag on downgaze (early sign).

images  Periorbital edema.

images  Proptosis.

images  Conjunctival chemosis.

images  Enlarged vessels over rectus muscle insertions.

images  Corneal exposure, ulceration.

images  Extraocular motility restriction, with preferential extraocular muscle involvement (inferior > medial > superior > lateral rectus) sparing the tendons.

images  Elevated intraocular pressure with upgaze.

images  Compressive optic neuropathy with decreased visual acuity, decreased color vision, afferent pupillary defect, visual field defects.


images  Age: 25 to 50 years.

images  Ratio of women to men: 6 to 1.

Ophthalmic Findings

Werner’s “NOSPECS” (mnemonic) classification:

Class 0: No signs or symptoms.

Class 1: Only signs (eyelid retraction).

Class 2: Soft tissue periorbital swelling.

Class 3: Proptosis of eyes.

Class 4: Extraocular muscle involvement, diplopia.

Class 5: Corneal involvement, exposure.

Class 6: Sight loss by optic nerve compression.

Systemic Findings

images  Pretibial dermopathy.

images  Other features associated with a dysthyroid state: restlessness, tremor, weight loss with hyperthyroidism or lethargy, constipation, weight gain with hypothyroidism.

images  Associated with myasthenia gravis, smoking, family history.

Special Tests

images  Evaluate thyroid function: sensitive thyroid-stimulating hormone (TSH); T3 and T4 (thyrotropin receptor) antibodies; antithyroid antibodies; thyrotropin-releasing hormone (TRH) stimulation test; Werner or T3 suppression test.

images  Orbital imaging (Figure 7.6).

Extraocular muscle enlargement sparing tendons: Most commonly involved (in order): inferior rectus, medial rectus, and superior rectus muscles.

Least commonly involved: lateral rectus.

Increased fat volume.

Optic nerve compression by muscles in orbital apex.

Rule out other causes of proptosis.

Define anatomy of paranasal sinuses before orbital decompression with CT scan.


Inflammation and enlargement of orbital tissues, specifically the nontendinous portions of the extraocular muscles:

Early: lymphocyte and plasma cell infiltration of muscles, deposition of mucopolysaccharide by activated fibroblasts, and formation of collagen.

Late: fibrosis and fatty infiltration of muscles.

Disease Course

images  Acute inflammatory phase (6 to 18 months) evolves into chronic stable fibrotic phase.

images  Spontaneous remissions and exacerbations can occur.

images  Thyroid disease and orbital disease run independent courses; however, orbital disease may worsen after radioactive iodide thyroid gland treatment.

images  Optic neuropathy may occur in patients without significant proptosis, because of posterior compression of the nerve.

images  Fifty percent of patients with upper lid retraction may improve without intervention.

images  Motility fluctuates during the acute phase.

Treatment and Management

images  Local supportive measures: artificial teardrops, lubricating ointment, elevating head of bed.

images  Corticosteroids: given with H2 receptor antagonist, bisphosphonate, and calcium.

For acute inflammatory phase.

For compressive optic neuropathy.

Prednisone 60 to 100 mg for up to 1 week to assess steroid responsiveness.

Pulse IV methylprednisolone (10 mg/kg, max 1 g) repeat at 48-hour intervals × 3; may be repeated after 3 months if no response or recurs. Side effects of cardiac arrhythmia, liver toxicity, relapse.

Rebound effect may occur after cessation of steroids.

images  Other immunomodulators are being studied in refractory cases with active inflammation.

Cyclosporin A: inhibits T-cell and IL-2 activity; start 2 mg/kg BID.

Tacrolimus: inhibits T-cell and IL-2 activity.

images  Orbital radiation:

Traditionally recommended for acute inflammatory phase or compressive optic neuropathy; however, recent studies have failed to demonstrate significant benefits 12 months after radiotherapy, whether administered early or late in the course of Graves ophthalmopathy.

Risks include cataract formation and radiation retinopathy, especially in diabetics and patients receiving systemic chemotherapy.

For compressive optic neuropathy: dosage: 2,000 cGy in ten fractions over 2 weeks to posterior orbit.

images  Surgical management: a staged approach must be taken to the surgical rehabilitation of the patient with thyroid orbitopathy so as to avoid unnecessary procedures.

Orbital decompression.

Extraocular muscle surgery.

Adjustment of eyelid margin position.


images  Orbital decompression:

For optic neuropathy, corneal exposure, proptosis.

Remove bony walls of orbit to allow prolapse of orbital soft tissues and enlargement of orbital volume.

Medial wall, ethmoid sinus, orbital floor, maxillary sinus, lateral wall, temporalis fossa.

Good success rates in improving decreased vision attributable to optic neuropathy.

Complications: diplopia, hemorrhage, infection, cerebrospinal fluid leak, optic nerve injury.

images  Extraocular muscle surgery:

Perform only after motility disturbance is stable for 6 months.

Steroids and radiation therapy have minimal effect.

Inferior and medial rectus muscles most often affected: recess with adjustable sutures.

images  Lid margin position surgery.

Lid retraction due in part to fibrotic levator or Müller muscle, increase in sympathetic activity.

Recess either muscle to lower the lid, especially laterally, with or without spacer grafts such as sclera.

Raise the lower lid to help cover the cornea, with spacer grafts.

images  Blepharoplasty: Remove excess skin and fat to improve cosmesis.

Differential Diagnosis

Idiopathic orbital inflammation (pseudotumor).

Orbital amyloidosis.

Carotid cavernous fistula.

Orbital tumor.

Eyelid retraction: see p. 29 for differential.


Gorman CA, Garrity JA, Fatourechi V, et al. A prospective, randomized, double-blind, placebo-controlled study of orbital radiotherapy for Grave’s ophthalmopathy. Ophthalmology 2001;108:1523–1534.

Krassas GE, Boboridis K. Recent developments in the medical treatment of thyroid eye disease. Orbit 2006;25:117–122.

Meyer PAR. Avoiding surgery for thyroid eye disease. Eye 2006;20:1171–1177.

Sergott RC, Glaser JS. Graves’ ophthalmopathy: a clinical and immunologic review. Surv Ophthalmol 1981;26:1–21.

Shorr N, Seiff SR. The four stages of surgical rehabilitation of the patient with dysthyroid ophthalmopathy. Ophthalmology 1986;93:476–483.

Idiopathic Orbital Inflammation (Orbital Pseudotumor)


Acute idiopathic orbital inflammation typically presents with pain and may be diffuse or localized to a specific area of the orbit. Treatment is typically with oral steroids. Many systemic processes such as sarcoid, Wegener granulomatosis, or metastatic carcinomas may present in a similar fashion. These must be considered before the diagnosis of idiopathic orbital inflammation is made.



Signs and Symptoms

images  Anterior orbit inflammation/diffuse inflammation:


Erythema and edema of lids.


Decreased vision.

Decreased extraocular motility.


images  Apical inflammation:


Decreased vision.

Decreased extraocular motility.

images  Myositic inflammation:

Pain with eye movements.

Decreased extraocular motility.

Localized chemosis.

images  Lacrimal inflammation:


S-shaped eyelid deformity.

Localized chemosis.


All ages, men and women.

Ophthalmic Findings

Children may develop papillitis.

Special Tests

CT scan:

Anterior, diffuse, apical: enhancing infiltrative mass with irregular margins.

Myositic: fusiform enlargement of extraocular muscle involving the tendon.

Lacrimal: irregular swelling of lacrimal gland.


Polymorphous infiltrate of inflammatory cells including lymphocytes, neutrophils, plasma cells, and macrophages.

Disease Course

Generally resolves with treatment but may recur.

Treatment and Management

images  Obtain orbital scan.

images  NSAIDS for myositis.

images  Corticosteroids: first line in a typical case.

Start with 1.0 to 1.5 mg/kg/day × 1 to 2 weeks, with slow taper over 6 to 12 weeks.

Methylprednisolone 1 g/day × 1 to 3 days for vision loss

Rapid resolution usually occurs with steroids.

images  Biopsy if no improvement with steroids or if recurrence occurs, or atypical presentation.

images  Add immunosuppressants as steroid-sparing drugs.

images  Orbital irradiation or immunobiologic agents (infliximab) can be used in refractory cases.

Differential Diagnosis

images  Adults:

Orbital cellulitis.

Orbital inflammation secondary to the following:


Uveitis, dacryoadenitis, rare orbital lesions.

Tests: angiotensin-converting enzyme, lysozyme, chest x-ray (CXR): hilar adenopathy, positive gallium scan.

Pathology: noncaseating granulomas.

Wegener granulomatosis:

Upper and lower respiratory tract lesions, vasculitis, nephritis, orbital inflammation, uveitis.

Tests: antineutrophil cytoplasmic antibodies (ANCA), abnormal urinary sediment, abnormal CXR.

Pathology: necrotizing granulomatous inflammation and vasculitis

Sjögren syndrome:

Dry eyes, dry mouth, other associated autoimmune diseases, enlarged lacrimal glands.

Tests: Antinuclear antibodies (ANA, rheumatoid factor [RF], Sjögren antibodies).

Pathology: polymorphous nongranulomatous inflammatory infiltrate

Ruptured dermoid cyst.

Arteriovenous fistulas.

Orbital metastasis.

images  Children:

Orbital cellulitis.

Ruptured dermoid cyst.


Metastatic neuroblastoma.

Leukemic orbital infiltrate.


Harris GJ. Idiopathic orbital inflammation: a pathogenetic construct and treatment strategy. Ophthal Plast Reconstr Surg 2006;22:79–86.

Mombaerts I, Glodschmeding R, Schlingemann RO, et al. What is orbital pseudotumor? Surv Ophthalmol 1996;41:66–78.

Rootman J, Nugent R. The classification and management of acute orbital pseudotumors. Ophthalmology 1982;89:1040–1048.


Congenital Orbital Tumors

Dermoid and Epidermoid Cysts

Clinical Features

images  Onset: childhood.

images  Smooth cystic mass in lateral or superolateral orbit.

images  Differentiate superomedial lesion from encephalocele by CT scan.


images  Dermoid: cyst lined by keratinized epidermis with dermal appendages such as hair follicles and sebaceous glands.

images  Epidermoid: cyst lined by keratinized epidermis without dermal structures.


images  Complete excision.

images  Ruptured cyst results in severe inflammation.


Clinical Features

images  Solid choristoma located lateral to lateral limbus with orbital extension.

images  Associated with Goldenhar syndrome.


Solid tumor with adipose tissue, hair follicles.


images  Excise anterior component only if hairs cause irritation.

images  Extensive resection may result in motility problems and damage to lacrimal ductules.


Fry CL, Leone CR Jr. Safe management of dermolipomas. Arch Ophthalmol 1994;112:1114–1116.

Vascular Tumors

See Table 7.10.


Harris GJ. Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Am J Ophthalmol 1999;127:453–455.

Rootman J. Vascular malformations of the orbit: hemodynamic concepts. Orbit 2003;22:103–120.

Capillary Hemangioma

Clinical Features

images  Onset at few weeks of life, grows over first year, involutes over several years.

images  Superficial strawberry surface lesion or deep bluish orbital lesion.

images  Kasabach-Merritt syndrome: capillary hemangiomas associated with thrombocytopenia.


Fine network of endothelially lined vascular channels.


images  Amblyopia, strabismus, anisometropia: treat; otherwise, may observe, since involution will occur.

CT, computed tomography

images  Steroids:

Intralesional (betamethasone and triamcinolone): risk of emboli of steroid particles through vessels that anastomose with the orbital circulation and cause central retinal artery occlusion.

Topical (clobetasol [Temovate] steroid cream): risk of atrophy and hypopigmentation of skin and subcutaneous tissues.

Systemic oral steroids.

Watch for adrenal suppression and growth retardation with steroids.

images  Propranolol, systemic

Effective treatment for disfiguring infantile capillary hemangiomas.

Potential mechanisms of action: vasoconstriction, down-regulation of the RAF/mitogen-activated protein kinase pathway resulting in decreased expression of proangiogenic factors, and triggering apoptosis of capillary endothelial cells.

Risk of bradycardia, hypotension, hypoglycemia, bronchospasm.

images  Surgery: useful only in well-circumscribed deep lesions.


Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic Surg 1986;17:229–231.

O’Keefe M, Lanigan B, Byrne SA. Capillary haemangioma of the eyelids and orbit: a clinical review of the safety and efficacy of intralesional steroid. Acta Ophthalmol Scand 2003;81: 294–298.

Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358:2649–2651.


Clinical Features

images  Infiltrative nonencapsulated lesion of eyelid, orbit with onset in first decade

images  Abrupt enlargement with hemorrhage into lymphangioma (chocolate cyst)

images  May enlarge with upper respiratory tract infection.

images  Fluid levels on MRI, but no flow on MRA or MRV.


images  Large serum-filled spaces lined by endothelial cells.

images  Chocolate cysts: hemorrhage from interstitial capillaries.


images  Monitor and treat amblyopia, strabismus, and anisometropia, particularly after hemorrhage.

images  Drain hemorrhage.

images  Avoid surgical resection because of infiltrative nature.

images  Debulk lesion anteriorly as needed.

Differential Diagnosis

images  Orbital varices: weakened venous system that enlarges with increased venous pressure (e.g., Valsalva maneuver) and enhances on CT.


Harris GJ, Sakol PJ, Bonavolonta G, et al. An analysis of thirty cases of orbital lymphangioma: pathophysiologic considerations and management recommendations. Ophthalmology 1990;97:1583–1592.

Cavernous Hemangioma

Clinical Features

images  Most common benign primary orbital tumor of adults, particularly middle-aged women.

images  Gradual proptosis, decreased vision with optic nerve compression or induced hyperopia (Figure 7.7).

images  CT: well-circumscribed mass with homogeneous enhancement.


Encapsulated lesion consisting of large, dilated vascular spaces filled with red blood cells.


Surgical excision for lesions compromising ocular function.


Clinical Features

images  Uncommon tumor of middle-aged adults.

images  Similar presentation as cavernous hemangioma.

images  May cause conjunctival engorgement and prolapse, motility restriction.

images  CT: well-circumscribed lesion.


Large pericytes surround a capillary network.


images  Complete surgical excision.

images  May recur, undergo malignant transformation, metastasize.

Neural Tumors

Optic Nerve Glioma

See Figure 7.8.

Clinical Features

images  Optic nerve glioma develops during the first decade.

images  Twenty-five to fifty percent associated with neurofibromatosis type 1.

images  Painless proptosis, vision loss, afferent pupillary defect.

images  Chiasmal involvement may occur.

images  Imaging:

Plain film: enlargement of optic canal greater than 6.5 mm in diameter.

CT: S-shaped fusiform enlargement of optic nerve.

Magnetic resonance imaging (MRI): better than CT for evaluation of posterior extension of tumor (more common in NF). Isointense or hypointense on T1 and hyperintense on T2.


Pilocytic astrocytoma (hairlike elongated neoplastic glial cells) with Rosenthal fibers (eosinophilic cell processes).


images  Management of optic nerve gliomas is controversial because of the variability in natural history of these tumors; although most remain stable or progress slowly, others may grow rapidly.

images  Treatment includes observation with serial MRI, chemotherapy (first line for progressive lesions), radiation (if chemotherapy fails), or surgical excision.

images  Radiation therapy is given for nonresectable tumors with chiasmal invasion.

images  Surgical excision involves optic nerve resection and therefore results in blindness; thus, tumor should be excised for growth toward the chiasm or for a blind proptotic eye.


Lee AG. Neuroophthalmological management of optic pathway gliomas. Neurosurg Focus 2007;23(5):E1.

Liu GT. Optic gliomas of the anterior visual pathway. Curr Opin Ophthalmol 2006;17:427–431.


Clinical Features

images  Intracranial meningioma:

More common than primary optic nerve meningioma and can invade the orbit resulting in secondary optic nerve sheath meningioma.

Middle-aged adult women.

Sphenoid bone most commonly involved.

Proptosis, chemosis, lid edema, temporal fullness, late visual loss.

CT: bony change, hyperostosis.

images  Optic nerve meningioma (Figure 7.8):

Middle-aged adult women; less commonly, children with neurofibromatosis.

Proptosis, decreased vision, papilledema or optic atrophy, optociliary shunt vessels (collaterals from retinal to choroidal venous circulation; seen in 25% to 30%), choroidal folds.

CT/MRI: diffuse thickening or fusiform enlargement of optic nerve, nodular surface, “train track” sign (central lucent zone), intralesional calcification.


images  Meningothelial cells in various patterns.

images  Psammoma bodies (calcified whorls of degenerated cells).


images  Meningiomas are slow-growing tumors that may be observed while following the patient carefully for vision loss, motility disturbances, or ocular exposure that is due to proptosis.

images  Intracranial meningioma:

Excise for vision loss, motility disturbance, or ocular exposure.

Combined approach with neurosurgery for surgical resection or debulking.

images  Optic nerve meningioma:

More conservative treatment of optic nerve than intracranial meningioma, because of tumor location.

Tumor is resected with risk of intracranial tumor spread, progressive growth, or poor vision.

Complete surgical excision of optic nerve is performed with a blind eye and progressive tumor.

Stereotactic radiotherapy is considered an early treatment option to stabilize and even improve vision.


Dutton JJ. Optic nerve sheath meningioma. Surv Ophthalmol 1992;37:167–183.

Jeremic B, Pitz S. Primary optic nerve sheath meningioma. Cancer 2007;110:714–722.

Plexiform Neurofibromas

Clinical Features

images  Infiltrative, well-vascularized tumor within eyelid, orbit.

images  Upper lid lesion: “bag of worms,” S-shaped eyelid.

images  Associated with neurofibromatosis type 1.

images  Higher incidence of glaucoma in the ipsilateral eye.


Intertwining bundles of Schwann cells, axons, and endoneural fibroblasts


Debulk lesions with caution.


Clinical Features

images  Painless proptosis in middle-aged individuals.

images  Ten percent of schwannomas occur in patients with neurofibromatosis.

images  Usually benign with slow progression, rare malignant transformation.

images  CT: well-circumscribed ovoid tumor.


images  Encapsulated proliferation of Schwann cells.

images  Antoni A: tightly packed Schwann cells; Verocay body (palisading nuclei).

images  Antoni B: loosely arranged Schwann cells; degeneration within the tumor.





images  Most common metastatic orbital lesion in children.

images  Primary malignancy located in the adrenal medulla and less commonly in the cervical ganglia that can result in ipsilateral Horner syndrome.

images  Abrupt ecchymotic proptosis, bilateral in about 50%.

Mesenchymal Tumors



Rhabdomyosarcoma, the most common primary orbital malignancy of childhood, should be suspected in any child with the rapid onset of an orbital process. Biopsy of a suspected lesion should be performed immediately. The survival rate of patients with rhabdomyosarcoma has improved significantly with the use of chemotherapy and radiation therapy.

Signs and Symptoms

images  Sudden onset of unilateral proptosis.

images  Eyelid swelling, injection, ecchymosis.

images  Pain and decreased vision are uncommon.


Onset: age 7 to 8 years.

Ophthalmic Findings

images  Proptosis (80% to 100%), downward and lateral displacement of globe.

images  Palpable mass: superonasal quadrant in 70%.

images  Ptosis: may be the first sign.

images  Conjunctival and lid edema and erythema.

Systemic Findings

Regional lymphadenopathy with metastatic spread to lung and bone.

Special Tests

images  CT: Look for bony destruction; lesion may appear circumscribed or infiltrative.

images  Immediate biopsy.

images  Metastatic workup: CXR, bone marrow biopsy, lumbar puncture.


images  Embryonal:

Most common type.

Superonasal quadrant of the orbit.

Loose fascicles of undifferentiated spindle cells with rare cross striations.

images  Alveolar:

Most malignant form.

Inferior orbit.

Fibrovascular strands form alveolar-like compartments, which are lined with rhabdomyoblasts.

images  Pleomorphic:

Rarest form.

Occurs in adults.

Well differentiated with easily located cross striations.

images  Botyroid embryonal:

Variant of embryonal that occurs in grapelike clusters with invasion from the paranasal sinuses or conjunctiva.

Disease Course

images  Rapid progression of tumor with bony invasion and metastasis to lymph nodes, lung, bone marrow, and brain.

images  Prognosis is based on staging of disease and histopathologic features with range from best to worse prognosis: adult pleomorphic > embryonal (including botyroid) > alveolar.

Treatment and Management

images  When rhabdomyosarcoma is suspected, an immediate biopsy should be performed (complete or near-complete excisional biopsy), with an appropriate metastatic workup to stage the disease.

images  Radiation therapy and chemotherapy are the mainstays of treatment, resulting in a 95% 5-year survival rate.

Differential Diagnosis

Orbital cellulitis.

Idiopathic orbital inflammation.


Lymphangioma with hemorrhage.

Capillary hemangioma.

Ruptured dermoid cyst.


Shields JA, Shields CL. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol 2003;48:39–57.

Wharam M, Beltangady M, Hays D, et al. Localized orbital rhabdomyosarcoma: an interim report of the intergroup rhabdomyosarcoma study committee. Ophthalmology 1987;94:251–254.

Fibrous Histiocytoma

Clinical Features

Most common adult mesenchymal tumor of the orbit.

Middle-aged adults.

Proptosis, decreased vision.

Range of benign to malignant based on clinical behavior.

Local recurrence, rare metastasis.

CT: well-circumscribed homogeneous mass (similar to cavernous hemangioma or schwannoma).


Fibroblasts and histiocytes in cartwheel or storiform pattern; diagnosis of exclusion after immunohistochemistry rules out other spindle cell tumors such as solitary fibrous tumor (CD34), melanoma (S100, HMB-45), and leiomyoma (smooth muscle antigen, desmin).


Complete surgical resection or irradiation (malignant fibrous histiocytoma).


Shields JA, Shields CL. Orbital fibrous histiocytoma. In: Shields JA, ed. Eyelid, conjunctival, and orbital tumors, 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2008:626–631.

Solitary Fibrous Tumor

Clinical Features

images  Can involve any part of the orbit, following an indolent course.

images  Middle-aged adults.

images  Local recurrence with incomplete excision.


images  Spindled-shaped cells uniformly reactive to CD34, unlike other benign orbital tumors.


images  Complete surgical excision.


Bernardini FP, Conciliis C, Schneider S, et al. Solitary fibrous tumor of the orbit. Ophthalmology 2003;110:1442–1448.

Fibro-Osseous Tumors of the Orbit

See Table 7.11.


Selva D, White VA, O’Connell JX, et al. Primary bone tumors of the orbit. Surv Ophthalmol 2004;49:328–342.

Histiocytic Tumors

Langerhans Cell Histiocytosis (Histiocytosis X)

Clinical Features

images  Tender palpable superotemporal mass, rhinorrhea.

images  First decade of life.

images  Results from abnormal accumulations of Langerhans cells—histiocytes normally found in the epidermis.

images  Eosinophilic granuloma: solitary lytic bone lesion with soft tissue involvement.

images  Hand-Schüller-Christian syndrome: triad of diabetes insipidus, exophthalmos, and multifocal bony lesions.

images  Letterer-Siwe: fulminant fatal systemic disease with visceral involvement.


images  Granulomatous-histiocytic infiltrate.

images  Electron microscopy: tennis-racket–shaped Birbeck granules.


Surgical curettage, systemic or local corticosteroids, local irradiation, cytotoxic agents.


Margo CE, Goldman DR. Langerhans Cell Histiocytosis. Surv Ophthalmol 2008;53:332–358.

Ocular Adnexal Lymphoid Tumors


Lymphoproliferative lesions occur as a continuum from benign lymphoid hyperplasia (polyclonal) to malignant lymphoma (monoclonal) and should be regarded as a potentially systemic process. Orbital lymphoma is the most common malignant orbital tumor affecting older adults.

Signs and Symptoms

images  Progressive, painless gradual proptosis.

images  Motility disturbances.

images  Firm nodular anterior orbital mass.

images  Subconjunctival salmon patch.

images  Bilateral disease in 25% of cases.

images  Lacrimal gland involvement.


Sixth and seventh decades of life.

Rare in children.

Systemic Findings

Risk of developing systemic non-Hodgkin lymphoma based on anatomic site of origin (lowest to highest): conjunctival (10%), orbital (50%), and eyelid (66%).

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Oct 2, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on 7 Oculoplastics and Orbital Disease

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