INFLAMMATORY AND IMMUNE DISEASE

Volkmann’s arm fracture) may be emergency, and patients may have oculocardiac reflex or nausea and vomiting, especially with upgaze.

Surgical repair needed for entrapment causing diplopia within 20 degrees of primary gaze or fracture that is occupationally limiting, or for >2 mm enophthalmos, >50% of floor defect (volume enlargement best seen on coronal CT), large rim defect, telecanthus, or aesthetic considerations. Usually do surgery within 7 to 14 days to limit scarring.

Le Fort I: high transverse maxillary fracture (all Le Fort fractures involve maxillary and pterygoid plate), no orbital involvement.

Le Fort II: pyramidal, nasal, lacrimal, maxillary bones, and medial wall and floor. May involve the NLD.

Le Fort III: craniofacial dysjunction with floor, medial, and lateral wall fractures. May involve the optic canal.

Naso-orbital-ethmoidal (NOE): frontal, maxillary, lacrimal, ethmoidal fractures with depressed nasal bridge, epistaxis, tearing, cerebrospinal fluid rhinorrhea, and telecanthus (>30 mm). Treat with transnasal wiring with mini-plate fixation of medial canthal tendons.

Zygomatico-complex (ZMC): also called ‘‘tripod’’ fracture, which is a misnomer, as the fracture actually has four components (fractures around the frontozygomatic suture, zygomatic-maxillary suture, zygomatic-arch fracture, and floor fracture; may have an orbital rim fracture). Patients have canthal dystopia (lower lateral canthus is usually 2 mm higher than medial canthus), temporal subconjunctival hemorrhage, and features of floor fracture. Look for associated globe injuries.

Roof: usually ‘‘blow-in’’ fracture with potential for cerebrospinal fluid leak or CNS herniation. May need neurosurgical evaluation.

Inflammatory and Immune Disease

IDIOPATHIC CHRONIC ORBITAL INFLAMMATION, ORBITAL PSEUDOTUMOR

Chronic, lymphoproliferative inflammation that is usually unilateral. Sixty percent of patients are age 20 to 60 (peak age 40 to 50), and present with pain, rapid onset (weeks to months) proptosis, ophthalmoplegia, and chemosis.

Clinical presentations depend on which orbital tissue is primarily involved:

Anterior diffuse inflammation (28%): most common presentation; mimics cellulitis with pink ‘‘boggy’’ edema in anterior two thirds of orbit. Biopsy if recurrent.

Dacryoadenitis (17%): ‘‘S’’-shaped lid. Biopsy; oral steroids are not as effective, and may debulk early. Often treated with antibiotics to first rule out infectious cause.

Myositis: third most common presentation. Usually affects SR and MR (opposite of Graves’ disease) with tendons involved

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78 ORBIT, EYELIDS, AND OCULAR ADNEXA

TABLE 2–2

Comparison of Thyroid-Related Immune Orbitopathy (TRIO) and Myositis

IR, inferior rectus muscle; MR, medial rectus muscle; SR, superior rectus muscle; LR, lateral rectus muscle.

(usually spared in Graves’). (See Table 2–2 for a comparison of Graves’s disease and myositis.) May be bilateral; better radiation response (less steroids needed); associated with greater rate of recurrence and systemic symptoms.

Sclerosing: rare form; chronic course, with tissue destruction and poor prognosis.

Tolosa-Hunt syndrome (7.6%): a type of parasellar or orbital apex

syndrome, with pain (CN V1) or corneal anesthesia, diplopia or ptosis (CN III, IV, VI), chemosis, venous congestion, Horner’s syndrome, ON swelling, and optic atrophy. Usually affects males; unilateral but may be bilateral. Differential diagnosis: anything that involves the cavernous sinus (meningioma, pituitary adenoma, glioma, chordoma, mucocele, etc.). Treat with long-term steroids.

Pediatric patients are more likely to have bilateral inflammation and have constitutional symptoms (nausea, vomiting, headache, and photophobia), poorer prognosis; more likely associated with uveitis, ON involvement, scleral thinning, muscle infiltration, Tenon’s space edema, increased erythrocyte sedimentation rate (ESR), and eosinophilia. Rule out rhabdomyosarcoma and cellulitis. Good response to steroids; avoid radiation therapy.

Differential diagnosis: lymphoma is the most common diagnosis; this is often confused with pseudotumor and may be steroid responsive. Lymphangioma with bleeding, Wegener’s granulomatosis and vasculitis may be painful. Also consider metastatic carcinoma, TRIO, and lacrimal gland tumors.

CT shows ‘‘dirty fat’’ appearance in the orbit; may have bony remodeling. On MRI the lesion is often isointense to fat on T2 (lymphoma is hyperintense).

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Pathology shows polyclonal hypocellular infiltrate in which T cells

predominate (Thelper > Tsuppressor). May be a spectrum with lymphoma, which is usually monomorphic with more B cell predominance, and higher proliferating cell nuclear antigen (PCNA). If benign, then more likely to have Dutcher bodies, macrophages, and vascular pattern. Pathologic subtypes:

Vasculitis: more aggressive treatment needed, rule out Wegener’s granulomatosis.

Eosinophilic: uncommon, usually in children; associated with vasculitis.

Sclerosing: more fibrosis.

Granulomatous: uncommon, Tolosa-Hunt type; rule out foreign body, tuberculosis, syphilis, or fungal causes.

Treat initially with oral steroids 60–80 mg daily with 4–6 week taper. Seventy-eight percent of patients have good response, often immediate (92% improve if ON involved), and 37% are cured; however, 52% of cases recur. Radiation 1500–2000 cGy, but many fail radiation therapy, especially myositis variant. May try cyclophosphamide, methotrexate, cyclosporine, and azathioprine. Consider surgery for atypical or recurrent disease or to debulk dacryoadenitis. A delay in treatment leads to decreased response, increased recurrence, and more biopsies. Surgical risks include: inflammation exacerbation (60%), residual proptosis (60%), muscle restriction (50%), ptosis (7%), and loss of the eye (3%).

THYROID-RELATED IMMUNE ORBITOPATHY (TRIO), GRAVES’ DISEASE

Autoimmune disease of retrobulbar tissue. Trio of involvement: muscle enlargement, optic neuropathy, and keratopathy.

Graves’ thyroid disease: idiopathic hyperthyroidism, 2% incidence; female to male ratio is up to 7:1, onset age 20 to 30 (affected males are slightly older). Symptoms include weight loss, increased appetite, sweating, heat intolerance, tremor, fatigue, palpitations, and irritability. Graves’ original triad: thyroid enlargement, palpitations, and exophthalmos. Classic clinical triad: hyperthyroidism, orbitopathy, and pretibial edema.

Thyroid orbitopathy: affects 30 to 70% of patients with Graves’ disease; male to female ratio ¼ 1:4, onset age 50; more severe in smokers. Typically presents within 18 months of thyroid dysfunction but may occur anytime. Twenty-five percent of orbitopathy patients develop thyroid dysfunction within 1 year, 50% within 5 years; overall 70% of euthyroid orbitopathy patients eventually develop thyroid dysfunction.

Pathogenesis: antigen against thyroid follicular cell circulates and cross-reacts with orbital tissue. Antithyroid antibodies mainly target orbital fibroblasts, which increase in number and activate local immune

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80 ORBIT, EYELIDS, AND OCULAR ADNEXA

reaction. Cytokine production is especially upregulated, which further stimulates fibroblast proliferation (is thus self-propagating).

Increased incidence with family history, humoral immunity (Ab to thyroid-stimulating hormone [TSH] receptor, long-acting thyroid stimulator [LATS], thyroid-stimulating immunoglobulin [TSI]), and cell-mediated immunity, human leukocyte antigen (HLA) DW-3 and B-8, and associated with other autoimmune diseases (up to 10% of patients may have myasthenia gravis).

Orbital volume is increased due to increased glycosaminoglycans from fibroblasts and thus increased orbital fluid volume (TRIO worse after sleeping on back), increased muscle size (younger patients tend to have less muscle enlargement than older Graves’ patients), fibroblasts, and fat proliferation. Chemosis may also be from hypertrophy of smooth muscle in infraorbital groove (Mu¨ller’s muscle of the orbit) compressing venous outflow.

Signs and symptoms: gradual and insidious course; 80 to 90% are bilateral. Werner classification of presentation (does not represent the sequence of the disease in all patients; mnemonic: NO SPECS):

No signs and symptoms

Only signs, no symptoms: eyelid retraction (Dalrymple’s sign; most common finding), eyelid lag on downgaze (Graefe’s sign), lagophthalmos, temporal flaring of lids (lid peak normally medial to pupil), edema, festoons, vascular injection.

Superior limbal keratoconjunctivitis (SLK): velvety injection of the superior conjunctiva and peripheral cornea that stains with Rose Bengal; treat with silver nitrate solution (not sticks).

Soft tissue signs and symptoms (mnemonic: RELIEF):

Resistance to retropulsion

Edema of conjunctiva

Lacrimal gland enlargement

Injection over the horizontal rectus muscles insertions

Edema of eyelids, lymphedema

Fullness of lids

Proptosis: minimal 21–23 mm, moderate 24–27 mm, severe >28 mm

EOM involved: IR affected more than MR > SR > LR, differential IOP (>6 mmHg) with upgaze. Muscle involvement is considered minimal if there is restriction only in extremes of gaze, or moderate if obviously limited ductions, or severe if frozen globe is present. Spares tendon (unlike pseudotumor). Diplopia typically worse with vertical or lateral gaze.

Cornea involved: considered minimal if only superficial punctate keratitis is present, moderate if ulcerated, or severe if perforated. Exposure keratopathy may be from proptosis (inadequate closure),

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lid retraction (poor blink), or IR restriction (poor Bell’s phenomenon).

Sight loss: decreased vision, disk edema, pallor, VF defects, dyschromatopsia, or APD. Considered minimal if VA is 20/ 20–60 or better, moderate if 20/70–200, or severe if 20/200 to no light perception (NLP). Optic neuropathy (present in 2.5% of cases) may be secondary to inflammation, apical compression by enlarged muscle, apex fat prolapse, or proptosis stretch. May also have gaze-evoked amaurosis. Treat actively with steroids until definitive treatment.

Clinical presentations:

Type I: symmetric proptosis and lid retraction, minimal congestion and inflammation, less myopathy with fatty infiltrate only.

Type II: more congestion and chemosis, increased myositis, restrictive myopathy, inflammation, and ON compression (smoking increases risk and severity of type II presentation).

Lipogenic variant: little EOM involvement, mainly just increased fat with increased risk of globe subluxation.

Medical management: treat early with tears, lubrication, taping at night, elevated head of bed, prisms for diplopia, and prednisone 40– 100 mg every day for 4 weeks, then taper (best for inflammatory features; no effect on proptosis, retraction, or myopathy). Radiation therapy 2000–3500 rads in 10 fractions for 2 weeks to the posterior orbit, targeting lymphocytes and fibroblasts; has similar effect as steroids but effect is delayed 3 weeks, so continue steroids during treatment. Prednisone and radiation therapy are contraindicated in vasculitis. Also treat coexistent hyperthyroidism usually with thyroid ablative therapies. And 10% of patients have orbitopathy flare with I-131 treatment; thus, consider prednisone 40 mg every day for prophylaxis during radioactive iodine treatment.

Surgical treatment: treat early for malignant proptosis or optic neuro-

pathy; otherwise, wait 1 year (or at least until disease is stable for 6 months). Order of surgeries: (1) orbital decompression, (2) strabismus surgery, (3) eyelid retraction repair, and (4) dermatochalasis or skin resurfacing.

Decompression: fat decompression if this is the predominant component of orbitopathy. Often only 2–6 mm of decreased proptosis with fat removal only. If bony decompression is indicated, determine how many walls to decompress by imaging (CT scan). Types: two wall (orbital floor and medial wall), ‘‘balanced’’ two wall (medial and lateral wall), three wall (medial wall, floor, and lateral wall), and four wall (add roof removal; often performed with neurosurgeon). Apical decompression is best obtained with medial wall removal, either external or transnasal endoscopic (higher incidence of diplopia).

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