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Scalp, Face, Skull, and Jaw Signs
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Because the scalp is extremely vascular, scalp wounds bleed profusely. Wounds do not gape unless the galea aponeurotica is lacerated. If gaping is noted, an open skill fracture should be suspected.
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Fluctuant scalp mass—hematoma, abscess, fracture
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When blood or pus accumulates in the skin or subcutaneous layer of the scalp, it is sharply localized and the mass readily slides over the skull. A boggy, fluctuant mass in the entire adult scalp results from blood or pus in the loose connective tissue under the aponeurosis; the same finding in a young child may also be an evidence of parietal bone fracture. A fluctuant mass bounded by the skull suture lines indicates subperiosteal blood or pus or a depressed fracture. A hematoma under the periosteum usually has a soft center that is plastic, whereas the edges are firm and feel much like a depressed fracture.
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The scalp is tender, soft, and boggy. The infection extends rapidly, causing edema of the eyelids and pinnae; regional lymph nodes are tender and swollen.
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A common lesion, it is either single or multiple. Arising from the skin, it slides easily over the skull. The mass is firm, nontender, and often hemispheric. If infected, it bleeds easily and may be mistaken for a squamous cell carcinoma.
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In the subcutaneous layer it feels smooth and soft; the finger slides around its edges. When it occurs beneath the pericranium, its movement is strictly limited, but the finger can detect a smooth, rounded border.
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Suppuration of the preauricular lymph node produces an abscess that may ulcerate. The swelling is localized, tender, and sometimes warm. The source of infection is in the side of the face, pinna, anterior wall of the external acoustic meatus, anterior third of the scalp, eyebrows, or eyelids.
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Masseter muscle hypertrophy
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One or both masseter muscles undergo spontaneous hypertrophy producing facial swelling mimicking parotid gland swelling. If the entire mass hardens when the patient clench his teeth, it is muscular.
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Redness of the cheeks
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Erythema, scaling, pustules, and tenderness in a malar distribution may be because of sunburn, cellulitis, rosacea, seborrheic dermatitis, discoid or systemic lupus erythematosus (SLE), or acne vulgaris.
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Absence of normal transverse furrowing with upward gaze is a sign of hyperthyroidism. Deep wrinkling, with longitudinal furrowing and prominence of intervening tissue is the bulldog skin in pachydermatosis. Unilateral loss of wrinkling results from facial nerve (CN VII) paralysis.
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Enlarged adult skull—Paget disease
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See Paget disease. In addition to bone pain, the patient may complain that his hats have become too small. The calvarium is large compared with the facial bones. A bruit is sometimes heard in the skull.
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Mastoid pain and tenderness—mastoiditis
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Skull masses—neoplasms
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An osteoma frequently occurs in the outer table of the skull, producing a hard, sessile bony eminence. Hard or soft masses in the cranial bones may be a pericranial sarcoma, carcinomatous metastasis, lymphomas, leukemia, or multiple myeloma.
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Trismus is the forceful closing of the jaws from spasm of the muscles of mastication. Trismus is common with tetanus, but it has many other causes. Failure of the mouth to open from other causes is far more common. CLINICAL- OCCURRENCE: Local Disorders: impacted third molar, TMJ arthritis, malignant external otitis, lymphadenitis, trigeminal neuralgia, scleroderma, dermatomyositis; Disorders With Widespread Muscle Spasm: trichinosis, rabies, tetany, tetanus, strychnine poisoning, typhoid fever, cholera, septicemia; Cerebral Disorders: encephalitis, epilepsy (transient), catalepsy, hysteria, malingering.
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Inability to close the jaw—TMJ dislocation
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Because the TMJ is a shallow biconcave surface, it can easily partially sublux or completely dislocate. After a yawn or an upward blow on the chin with the mouth wide open, the jaw cannot be closed. The mandible protrudes and the lower teeth override the upper. There is a depression or pit anterior to the tragus that is more obvious when bilateral; in unilateral dislocation, the pretragal depression occurs only on the affected side. No movement of the mandibular head is felt when palpating through the external acoustic meatus on the affected side.
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A visible crease extending at least one-third of the distance from tragus to posterior pinna has been associated with a higher rate of cardiac events in the patients admitted to the hospital with suspected coronary heart disease [Elliott WJ, Powell LH. Diagonal earlobe creases and prognosis in patients with suspected coronary artery disease. Am J Med. 1996;100:205–211].
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Earlobe nodule: gouty tophus
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In long-standing gout, accumulations of sodium urate crystals may occur in the helix and antihelix; they also occur in the olecranon bursa, the tendon sheaths, and the aponeuroses of the extremities. The nodules are painless, hard, and irregular. They may open discharging chalky contents.
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A developmental eminence in the upper third of the posterior helix, this condition is harmless. It must be distinguished from acquired nodules, such as tophi.
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Nontender nodules may be basal cell carcinomas, rheumatoid nodules, or leprosy. Cartilage calcification is a rare complication of Addison disease.
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Trauma or a hemostatic defect results in blood accumulating between the cartilage and the perichondrium. There is a tender, blue, doughy mass, usually without spontaneous pain. Prompt incision and drainage avoids suppuration or cauliflower ear.
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Recurring inflammation—relapsing polychondritis
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There is inflammation and degeneration of cartilage especially of the pinna, nasal septum, laryngeal cartilages, tracheal and bronchial rings; joint cartilages may be affected. The ear is painful, swollen, and reddened, except over the lobule. Hoarseness indicates laryngeal involvement, blindness may result from involvement of the sclerae, and tinnitus and deafness from middle ear involvement. Rarely, degeneration of the aortic or mitral valve ring produces valvular regurgitation or aortic aneurysm.
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A favorite site is just behind the pinna. It is soft and slightly fluctuant.
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External Acoustic Meatus Signs
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The wax of Native Americans and East Asians is often dry and flakey, and more yellow than amber. Excessive wax production or a narrow meatus leads to impacted cerumen and partial or complete obstruction of the canal. When obstruction is complete, partial deafness results. Tinnitus or dizziness may occur. A partial obstruction may suddenly become complete when water enters the meatus during bathing or swimming. The obstructing wax is easily seen in the external meatus.
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Discharge from the ear—otorrhea
++
Discharge from the ear has many causes. The type of discharge suggests the diagnosis: Yellow Discharge: melting cerumen; Serous Discharge: eczema, early ruptured acute otitis media; Bloody Discharge: trauma of the external canal or longitudinal temporal bone fracture causing TM and external canal laceration; Purulent Discharge: chronic external otitis, perforation of acute suppurative otitis media, chronic suppurative or tuberculous otitis media with or without cholesteatoma.
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Seborrheic dermatitis commonly causes scaling and pruritus of the choana and meatus. Medicated eardrops can cause contact dermatitis.
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Either squamous cell or basal cell carcinoma can involve the meatal epithelium. Pain and discharge are the presenting symptoms. In advanced stages, deafness and facial paralysis may occur.
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Children often place objects in their ears. A purulent discharge from the canal or an earache may be the first indication.
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A bulbous, reddened, pedunculated mass arises from the wall or the middle ear. Moving it with forceps may reveal the origin. They cause a foul purulent discharge.
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Exostoses and chondromas
++
Exostoses form nodules in the osseous canal near the TM. They rarely produce obstruction, although the TM may be partially obscured. A single bony osteoma may occur. Rarely, chondromas arise from the cartilaginous canal, usually without obstruction.
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A red, tender prominence with or without a pustule forms in the cartilaginous canal producing extreme pain.
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Pain in the ear with vesicles in the canal and facial weakness is because of herpes zoster of CN VII (Ramsey–Hunt Syndrome), Chapter 14, Facial Weakness and Paralysis.
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Infection with Mycoplasma pneumoniae causes severe ear pain and an inflamed TM with often hemorrhagic vesicles (bullous myringitis).
++
A healed suppurative middle ear infection has eroded through the TM leaving an oval hole through which the middle ear cavity is seen. Other than some decrease in auditory acuity, chronic perforations are asymptomatic.
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Lateralizing Weber test—ipsilateral conductive hearing loss or contralateral neurosensory loss
++
When neurosensory hearing is intact bilaterally, the sound will lateralize to the side of conductive loss, which loses the masking effect of background noises. Neurosensory loss on one side results in a louder sound on the opposite side. Therefore, lateralization of sound to the right ear means conductive loss on the right or perceptive loss on the left.
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Bone conduction greater than air conduction (Rinne-negative test)—conductive hearing loss
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When amplification of sound by the TM and ossicles is impaired, direct transmission of vibrations through bone to the cochlea is louder than sound transmitted through air. Conductive hearing loss results from obstruction of the auditory canal, damage to the TM, middle ear fluid, and destruction or ankylosis of the ossicles.
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Balance and position Sense signs
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Lacrimation usually refers to any condition resulting in tears, although strict usage indicates an overproduction. Epiphora means an overflow of tears from any cause. CLINICAL OCCURRENCE: Increased Secretion: weeping from emotion, irritation from foreign body, corneal ulcer, conjunctivitis, coryza, measles, hay fever, poisoning (iodide, bromide, arsenic); Lacrimal Duct Obstruction: congenital, cicatrix, eyelid edema, lacrimal calculus, dacryocystitis; Separation of the Puncta from the Globe: facial paralysis, aging, chronic marginal blepharitis, ectropion, proptosis.
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Widened palpebral fissures
++
The fissures are widened by lid retraction (contraction of Mueller muscle) or protrusion of the globe. With eyes in the primary position the upper lid covers the limbus and a white scleral strip usually shows between limbus and lower lid. Widening of the palpebral fissure uncovers the upper border of the limbus to expose white sclera superiorly. When there is no actual proptosis, widened fissures produce the optical illusion of global protrusion. A few normal persons have widened palpebral fissures.
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Exophthalmos (ocular proptosis)
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Proptosis is proven by measurement. Unilateral proptosis is recognized by comparing the two eyes. If both eyes seem equally prominent, inspect them in profile (Fig. 7-28). Unilateral proptosis suggests orbital tumor or inflammation. When the globe is displaced medially, disease of the lacrimal gland should be suspected; upward displacement suggests disease in the maxillary sinus; lateral displacement can occur from a lesion in the ethmoid or sphenoid sinus. Graves disease is the most common cause of bilateral proptosis. CLINICAL OCCURRENCE: Unilateral Exophthalmos: Graves disease, mucocele, orbital cellulitis and abscess, cavernous sinus thrombosis, orbital periostitis, myxedema, orbital fracture, hemangioma, orbital neoplasm, arteriovenous aneurysm, fungal infection, histiocytosis; Bilateral Exophthalmos: Graves disease, myxedema, acromegaly, cavernous sinus thrombosis, empyema of the nasal accessory sinuses, lymphoma, leukemia, histiocytosis.
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Thyrotoxicosis increases sympathetic stimulation producing contraction of Mueller muscle in the upper lid. Lid lag indicates increased tone even without widened fissures in the primary position. Usually the finding is bilateral; occasionally one fissure is much wider than the other. Other Lid Signs in Hyperthyroidism—Stellwag Sign: infrequent blinking; Rosenbach Sign: tremor of the closed eyelids; Mean Sign: global lag during elevation; Griffith Sign: lag of the lower lids during elevation of the globes; Boston Sign: jerking of the lagging lid; Joffroy Sign: absence of forehead wrinkling with upward gaze, the head being tilted down.
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Narrowed palpebral fissures—enophthalmos
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The globe is recessed in the orbit. When bilateral, it is usually caused by decreased orbital fat or congenital microphthalmos. Unilateral enophthalmos is caused by trauma or inflammation. Enophthalmos has been described as a sign of Horner syndrome; actually, the accompanying droop of the eyelid merely produces an optical illusion of globe recession.
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Failure of lid closure—paralysis of orbicularis muscle
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The facial nerve (CN VII) supplies the orbicularis oculi muscle. Damage to CN VII, as in Bell palsy, causes partial or complete paralysis of the orbicularis. When complete, both upper and lower lids remain retracted so the eye is unprotected and tears drain onto the face. Bell phenomenon occurs when the globes elevate during attempted closure of the lids. Failure of lid closure is also present in severe grades of exophthalmos.
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Failure of lid opening—ptosis of the lid
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The congenital form is usually bilateral from paralysis of or failure to develop the levator palpebrae superioris. The acute acquired condition usually results from disease of the oculomotor nerve (CN III). In the congenital form, the eyelid will show lid lag as the child looks down. With CN III lesion paralysis of other eye muscles may be present.CLINICAL OCCURRENCE: supranuclear lesions (e.g., encephalitis), Horner syndrome, paralysis of the levator muscle, levator dehiscence, thinning of levator tendon (the lid droops but has normal excursion, 15–18 mm).
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Frequent spasmodic lid closure, either unilateral or bilateral, may progress to the point that it interferes with function. It is a form of focal dystonia. Unilateral disease may follow Bell palsy.
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Epicanthal fold—Down syndrome
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Shortened palpebral fissures—fetal alcohol syndrome
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The combination of shortened palpebral fissures, epicanthic folds, shortened nose with anteverted nostrils, hypoplastic upper lip with thinned vermilion and flattened or absent philtrum, together with mental retardation, is stigmata of fetal alcohol syndrome.
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Lid inversion—entropion
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Structural changes or muscular contraction turns the eyelashes inward to impinge upon the globe. Spastic entropion occurs only in the lower lid and is caused by increased tone of the orbicularis oculi; the lid turns in only when forcibly closed (Fig. 7-29A). Cicatricial entropion occurs in either lid from contracture of scar tissue, as in trachoma. Irritation from the inverted eyelashes may produce blepharospasm.
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Lid eversion—ectropion
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The lid turns outward (Fig. 7-29B). Both lids may be affected by spastic or cicatricial ectropion, but paralytic ectropion involves only the lower lid. Senile atrophy of tissues sometimes results in ectropion rather than entropion.
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Heliotrope or violaceous discoloration of the periorbital skin occurs in dermatomyositis and after chronic quinacrine ingestion.
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Generalized reddening of the lids is nonspecific. Erythema of the nasal half of the upper lid suggests inflammation of the frontal sinus. Disease of the lacrimal sac can cause erythema of the medial lower lid. Hyperemia of the temporal upper lid should suggest dacryoadenitis. The lid is frequently red over a sty.
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Blueness of the eyelid occurs from thrombosis of the orbital veins, orbital tumors, and arteriovenous malformations in the orbit.
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Trauma to the lids may result in extravasation of blood into the surrounding tissue, colloquially known as a “black eye.” Palpebral hematoma can occur from nasal fracture. The appearance of hematoma many hours after head trauma suggests a skull fracture; the greater the time interval, the more remote the fracture site. Fractures of the basilar skull may produce hematoma of the lid several days later. Involvement of both eyes is called raccoon sign.
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Noninflammatory edema is frequent in acute nephritis, but uncommon in chronic nephritis and cardiac failure (Fig. 7-30A). Lid edema occurs early in the course of both myxedema and Graves ophthalmopathy. Palpebral edema is frequent in angioedema and trichinosis. Contact dermatitis frequently involves the lids; the patient may not react to an allergen on the hands, but when transferred to the lids swelling occurs. Local infections cause inflammatory edema of the eyelids, which is readily identified by finding redness, warmth, and pain. More serious causes are thrombosis of the intracranial sagittal sinus or cavernous sinus.
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Xanthelasma are raised yellow, painless, and nonpruritic plaques on the upper and lower lids near the inner canthi (Fig. 7-30B). They grow slowly and may disappear spontaneously. They are frequently associated with elevated cholesterol.
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Seborrheic blepharitis is an oily inflammation of the lid margins that produces greasy flakes of dried secretion on the eyelashes and reddening of the lid margins. When ulceration of the lid margin occurs, it is usually staphylococcal blepharitis. Angular blepharitis is a specific disease caused by the diplococcus of Morax–Axenfeld, in which the margins near the temporal canthi are inflamed.
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External hordeolum (sty)
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When a sebaceous gland of an eyelash hair follicle becomes inflamed, a pustule forms on the lid margin (Fig. 7-30C). It may be surrounded by hyperemia and swelling. Many rupture and heal spontaneously.
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Internal hordeolum and meibomian cyst (chalazion)
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Acute inflammation of a meibomian (tarsal) gland is termed an internal hordeolum or internal sty. A granuloma of the gland is known as a chalazion or meibomian cyst (Fig. 7-30D). These internal sebaceous gland lesions produce localized swelling that frequently causes a protrusion on the lid. Lid eversion shows hyperemia, a localized cyst or enlarged gland.
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Obstruction of the lacrimal duct produces acute inflammation of the lacrimal gland with pain and tenderness within the temporal edge of the orbit. It must be distinguished from orbital cellulitis and upper lid hordeolum (Fig. 7-30E).
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Nasolacrimal duct obstruction leads to inflammation and infection. Patients present with pain and an overflow of tears onto the cheek (epiphora). Symptoms are increased by irritants such as wind, dust, or smoke. Tenderness, swelling, and redness are present beside the nose, near the medial canthus (Fig. 7-30F). The swelling is anterior to the eyelid, distinguishing it from hordeolum. Fluid can be expressed with pressure on the duct. Conjunctivitis, blepharitis, and lid edema may be present.
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See Chapter 14, Eye Movement Signs. Abnormalities of eye movements may be caused by either primary disease of the extraocular muscles or disease of the central nervous system and CNs. For the nonspecialist the ability to distinguish neurologic from primary muscle disease is most critical. Therefore, these signs are discussed with the neurologic examination.
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Restriction of motion
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Movement of the globe may be restricted in all directions by orbital tumor or the increase in orbital contents with Graves disease.
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Subconjunctival vessels
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The scleral vessels may be prominent in normal individuals. They run in from the sides (Fig. 7-31A).
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Subconjunctival hemorrhage
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Bleeding under the conjunctiva is obvious and harmless (Fig. 7-31B). It may be induced by coughing, sneezing, weight lifting, or defecation; frequently, the cause is not apparent.
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Conjunctival injection
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Mild diffuse capillary hyperemia of the scleral and palpebral conjunctivae, without hemorrhage, is common in the coryza phase of respiratory infections or from exposure to direct sunlight or environmental irritants. It may be mildly uncomfortable. Significant pruritus, pain, or discharge should suggest another disorder such as allergic conjunctivitis.
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Conjunctival edema—chemosis
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The conjunctiva is swollen and transparent; it is usually associated with edema of the lids. The edema may be demonstrated by looking at the globe in profile while pressing the lower lid against the bulbar conjunctiva; the edge of the lid pushes up a wave of edematous bulbar conjunctiva (Fig. 7-31C). It is frequent in Graves ophthalmopathy.
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Hyperemia of the globe and ciliary flush
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Bulbar conjunctival injection is the dilation of the radial conjunctival vessels and their branches running from the fornices toward the center of the cornea (Fig. 7-32A). When the deeper, net-like episcleral vessels dilate they produce a more violaceous injection, particularly noted as ciliary flush at the corneal limbus (Fig. 7-32B). Suffusion in the conjunctiva blanches with pressure; the ciliary flush does not blanch. Ciliary flush indicates inflammation of the uveal tract (see Uveal tract inflammation—uveitis (iritis, iridocyclitis, and choroiditis)).
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Inflammation of the conjunctiva, with or without infection, is conjunctivitis. The patient may awaken with eyelids stuck shut and a gritty or burning sensation with excessive lacrimation. There is marked redness of the eye(s) from hyperemia of the palpebral and peripheral global conjunctival vessels. There are many causes; consider viral and bacterial infections, foreign–body reaction, allergies, and blepharitis. DDX: Awakening with the lids stuck together by purulent discharge increases the probability for bacterial infection; itching suggests a nonbacterial etiology.
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Hyperemic conjunctiva with calcification
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These lesions occur when the serum calcium–phosphorus product exceeds 70 in renal failure and sarcoidosis. Conjunctiva Lesions: The segments from limbus to canthus at 7 to 10 o’clock and at 2 to 5 o’clock show hyperemic reddening, calcified plaques, and pingueculae. The eyes are painful or feel gritty. The affected areas contain calcium deposits, visible to the unaided eye or through the slit lamp. Cornea Lesions: This is called band keratopathy occurring in hypercalcemia and in renal disease with conjunctival calcification. White material is visible in the limbal arcs at 2 to 5 o’clock and 7 to 10 o’clock. The slit lamp reveals calcium deposits.
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Chronic irritation from wind and dust is thought to stimulate the growth of the pinguecula, resulting in the extension of a vascular membrane over the limbus toward the center of the cornea, called a pterygium. This is a raised, subconjunctival fatty structure, growing in a horizontal band toward and over the pupil (Fig. 7-33A); vision may be obstructed. It has a firm attachment to the bulbar surface and is strictly horizontal; it is usually bilateral. A pseudopterygium is a band of scar tissue that may extend in any direction and adhere only partially to the bulbar conjunctiva, so a probe may be passed beneath it.
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Pigmented pingueculae
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Brownish pigmentation of the pingueculae is one of the few signs of Gaucher disease; others are hepatosplenomegaly, thrombocytopenic purpura, and patchy brown pigmentation on the face and the legs.
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Inflammation in the iris or anterior chamber produces a purulent discharge in the anterior chamber. The opaque fluid settles in the dependent portion of the chamber where it is seen as a fluid level behind the cornea (Fig. 7-33B). Iritis is a common cause.
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Lusterless cornea—superficial keratitis
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Corneal inflammation or drying causes epithelial loss. There is loss of the normal corneal luster with grayness of the anterior stroma. A ciliary flush is often present. Fluorescein staining demonstrates ulceration or denuded epithelium. A corneal ulcer is extremely painful and causes miosis and photophobia. Disruption of the epithelium demands urgent expert therapy, because visual loss can occur rapidly. CLINICAL OCCURRENCE: There are many causes of superficial keratitis including contact lens-related ulcers, infected abrasions, herpes simplex and zoster, corneal exposure, trigeminal nerve (CN V) injury, amiodarone deposits, and spread of infection from the conjunctiva.
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Cloudy cornea—interstitial keratitis
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The prototype is congenital syphilis, in which interstitial keratitis, deafness, and notched teeth constitute the Hutchinson triad. During the early stages of inflammation, between ages 5 and 15 years, a faint opacity begins in the central zone of the cornea together with a faint ciliary flush. Usually, pain and lacrimation occur as well. Later, the cornea becomes diffusely clouded, so the iris may be obscured. Blood vessels grow into the cornea. After the acute stage, there is permanent corneal opacity. Acquired syphilis and tuberculosis are occasional causes.
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A gray opaque band in the cornea, 1.0- to 1.5-mm wide, is separated from the limbus by a narrow clear zone (Fig. 7-33C). Early on only a segment of the circumference is involved; later the circle is completed. It is present to some degree in most persons older than 60 years of age; it is bilateral. If seen before age 40, hyperlipidemia should be suspected.
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Keratoconjunctivitis sicca—Sjögren syndrome
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Lymphocytic infiltration of exocrine glands (lacrimal and salivary) reduces tear flow producing dry inflamed eyes. Sjögren syndrome (Sjögren syndrome—keratoconjunctivitis sicca) is likely if persistent dry eyes, dry mouth, and a positive Schirmer test (Schirmer test of tears and Fig. 7-33D) are present without obvious cause. Similar findings can be seen with HIV infection and sarcoidosis.
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Kayser–Fleischer ring—Wilson disease
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A 2-mm-wide golden-brown circular band is seen in the peripheral cornea near the limbus. The ring is formed by the copper deposited in Descemet membrane, the basement membrane of the cornea endothelium. This deposit begins superiorly, spreads inferiorly; it accompanies the neurologic manifestations of Wilson disease. A slit lamp is often required to see the ring.
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Central corneal opacity
++
This results from trauma or infection and is seen in 75% of patients with Hurler syndrome.
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Dots in the cornea—Fanconi syndrome
++
Cystine crystals are deposited throughout the stroma with no accompanying inflammatory reaction.
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Yellow sclera—icterus and fat
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In obstructive jaundice (Jaundice) conjugated bilirubin infiltrates all body tissues and fluids; it colors the sclera evenly. The conjunctiva of the fornices is usually deeper yellow because it is thicker. Deposits of fat beneath the conjunctiva commonly impart a yellow color to the periphery, leaving the perilimbal area relatively white. It is more obvious with advancing age and in the patients with anemia.
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Red sclera—scleritis and episcleritis
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Inflammation of the sclera and/or Tenon capsule can produce loss of scleral integrity. Scleritis may be diffuse or nodular and frequently associated with autoimmune diseases. The patient will have severe, deep, boring pain. In sunlight, the lesion will have a violaceous, red–purple appearance. Suppurative scleritis is rare and usually metastatic. Tuberculosis, sarcoidosis, and syphilis cause a granulomatous scleritis with localized scleral elevation and nodule formation. Scleral thinning may be non-necrotizing (scleromalacia perforans) or necrotizing with acute inflammation surrounding an area of ischemia which may ulcerate. Episcleritis is a much milder form of inflammation involving the fascial sheath of the globe (Tenon capsule) and appears clinically as a diffuse or nodular violaceous injection (Plate 14).
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Blue sclera—osteogenesis imperfecta
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Light reflecting off the pigmented choroid appears blue through the thinned sclera. This finding is classic for osteogenesis imperfecta; it may be mimicked by minocycline deposits, scleral thinning after scleritis, or age-related calcification of the horizontal rectus muscle insertions.
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Brown sclera—melanin or homogentisic acid
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Patches of melanin are commonly seen on the conjunctiva of dark-complexioned people, especially blacks. In alkaptonuria with ochronosis wedge-shaped areas of brown homogentisic acid color the sclera near the attachments of the ocular muscles with their apices toward the limbus.
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Scleral protrusion—staphyloma
++
Scleral injury or increased intraocular pressure leads to a protrusion from the surface of the globe. An anterior staphyloma forms near the cornea creating a characteristic profile (Fig. 7-33E).
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Discoloration or disruption of the layers of the lens produce focal or diffuse opacities that can obstruct and/or scatter light before it reaches the retina. Because nearly all adults have some lens opacity, a clinical definition of cataract implies interference with vision [Asbell PA, Dualan I, Mindel J, et al. Age-related cataract. Lancet. 2005;365:599–609]. Some cataracts are seen by shining a light beam obliquely through the lens (focal illumination), by ophthalmoscopic inspection against the red retinal reflex with 0 diopter magnification from approximately 40 cm (15.7 inch), or by using + 10 diopter magnification with close inspection (direct illumination). Many can only be identified with the slit lamp. Centrally placed cataracts can be seen without pupillary dilatation; those in the periphery are only visualized with dilation. This discussion is limited to cataracts detectable without mydriatics or the slit lamp.
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Anterior and posterior polar cataract
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A small congenital white plaque can be seen in the center of the pupil. It results from a congenital defect in the anterior or posterior capsule.
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Diffuse yellow to brown discoloration occurs first in the central portion of the lens then gradually becomes diffuse discoloration throughout the lens. There is a black spot centrally against the red retinal reflex.
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Wedge-shaped anterior or posterior cortical opacities, arranged radially and extending in from the periphery, appear gray with the penlight and black against the red retinal reflex (Plate 16).
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Fibrosis of the posterior capsule is a common sequela of cataract surgery; the peripheral lens epithelial cells migrate across the capsular bag left to support the intraocular lens implant. This is more correctly an opacified posterior capsule. It appears as dense folds of tissue and clusters of clear vesicles.
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Older diabetic patients have an increased tendency to develop nuclear or cortical cataracts with no distinctive character. Juvenile diabetic patients acquire a distinctive snowflake cataract containing chalky white deposits; the entire lens subsequently becomes milky.
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Posterior subcapsular cataract
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This lesion is commonly seen after long-term use of corticosteroids, with diabetes, and after trauma or uveitis (Plate 17).
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Lens subluxation and dislocation
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Rupture of the zonula ciliaris (zonule of Zinn) permits the lens to move from its fixed position behind the pupil. Slight displacement, with the lens still backing the pupillary aperture, is termed subluxation (Fig. 7-34A); it is manifested by tremulousness of the iris (iridodonesis), when the eye moves horizontally. Viewed through the ophthalmoscope, the equator of the lens may show as a dark, curved line crossing the pupil; a double image of the retina with different magnifications may be seen, one through the lens, the other without the lens. When the lens is displaced completely it is a dislocation; when it enters the anterior chamber (Fig. 7-34B), it is easily seen. Lenticular displacement is usually caused by trauma. Nontraumatic dislocation occurs in several hereditary conditions including Marfan disease, homocystinuria, and hereditary spherophakia.
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Intraocular pressure changes
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Increased tension occurs in glaucoma; decreased tension is seen with myotonic dystrophy, globe rupture, and extreme dehydration. Accurate assessment is made by measurement of pressure with the tonometer.
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The following article provides a good review of retinal diseases with pathophysiology and photographs of many retinal signs [D’Amico DJ. Diseases of the retina. N Engl J Med. 1994;331:95–106].
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Increased cup-to-disk ratio—glaucoma
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Myelinated nerve fibers
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Myelination of the optic nerve fibers usually ends at the lamina cribrosa; infrequently myelin sheaths are maintained in the retinal nerve fiber layer (Fig. 7-35B). Semi-opaque white patches emerge from the optic disk spreading into one or two retinal quadrants. The disk margin appears frayed and the underlying vessels are partially or completely obscured. It is a normal variation of no clinical significance. Patches of myelinated nerves may occur remote from the disc.
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Disk pallor—optic atrophy
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Damage to the optic nerve (compression, ischemia, inflammation, or increased intracranial pressure) leads to atrophy of the nerve fibers and loss of normal vascularity (Fig. 7-35C and Plate 18). The disk is pale pink, yellow, or white; the margins may be less distinct and the physiologic cup and lamina cribrosa are variably seen. The emerging vessels may be surrounded by perivascular glial sheathing, seen as white lines. DDX: Pigmented high water marks around the nerve or residual exudate in the peripapillary retina may indicate previous disc edema, suggesting increased intracranial pressure producing the optic atrophy. It is important to recognize that an atrophic nerve can no longer swell, so cannot be used to monitor the presence of papilledema. A common cause of incidentally found optic atrophy is brain tumor, thus all optic atrophy should be evaluated promptly by an ophthalmologist. In optic atrophy from chorioretinitis, the disk may have a yellow cast, and the surrounding retina may contain hemorrhages, areas of atrophy, and pigment. The distinction between optic atrophy resulting from intrinsic optic nerve lesions versus increased intracranial pressure cannot be made reliably by the physical findings; interestingly, disc pallor does not occur in glaucoma until very late in its course. CLINICAL OCCURRENCE: Intrinsic Optic Nerve Lesions: multiple sclerosis, syphilis; Increased Intracranial Pressure: idiopathic intracranial hypertension, brain tumors; optic nerve compression without increased intracranial pressure.
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Disk edema—papillitis, optic neuritis
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When optic neuritis involves the portion of the optic nerve within the globe, papillitis, with loss of vision, is produced (Fig. 7-35D) with disk edema indistinguishable from papilledema. Visual loss occurs early in optic neuritis and usually later with papilledema. The disk is hyperemic and its margins may be indistinct from edema in the peripapillary nerve fiber layer. The disk surface may be elevated above the surrounding retina (a + 1 or + 2 lens correction is required to focus on the disk) [Balcer LJ. Optic neuritis. N Engl J Med. 2006;354:1273–1280]. CLINICAL OCCURRENCE: ocular inflammation (e.g., uveitis, retinitis, sympathetic ophthalmia), intrinsic inflammation of the optic nerve (e.g., demyelinating optic neuritis in multiple sclerosis, neuromyelitis optica—Devic syndrome), intracranial inflammation (e.g., meningitis, venous sinus thrombosis), infections (e.g., syphilis, tuberculosis, influenza, measles, malaria, mumps), and intoxications (e.g., methyl alcohol).
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Anterior ischemic optic neuropathy (AION)
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Infarction of the optic nerve head results from inadequate perfusion of the posterior ciliary arteries. AION occurs in two forms, the arteritic, related to giant cell arteritis, and the nonarteritic, which occurs in patients with vasculopathies such as hypertension or diabetes mellitus and intercurrent hypotension. The onset is usually sudden and painless, with profound visual loss, typically altitudinal, involving the upper and lower field. The optic nerve appears edematous, with scant hemorrhage and more pallor than typical for papilledema. DDX: In a patient of age >55 years, it is imperative to search for giant cell arteritis. The nonarteritic form is commonly accompanied by a small to absent optic cup in the uninvolved eye and often a period of systemic hypotension.
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Increased intracranial pressure causes the cerebrospinal fluid (CSF) to compress the optic nerve within its sheath resulting in axoplasmic flow stasis and ischemia (Fig. 7-36A). Early papilledema causes a C-shaped halo of nerve fiber layer edema that surrounds the disc with a gap temporally (Plate 19). With more advanced papilledema, the halo becomes circumferential. Next there is obscuration of major vessels as they leave the disc; and later there is obscuration of vessels on the optic disc. The emerging vessels bend sharply in passing over the elevated disk edge (Plate 20). Macular retinal edema creates traction folds (choroidal folds), seen as white lines radiating from the macula (Fig. 7-36B). Patients with papilledema will have an enlarged physiologic blind spot documented with formal visual field testing. DDX: In contrast to papillitis, central vision is unimpaired, but, like glaucoma, there is usually peripheral visual loss. CLINICAL OCCURRENCE: The principal causes are brain tumor and idiopathic intracranial hypertension. Less common causes are hydrocephalus, malignant hypertension, subarachnoid hemorrhage, meningitis, and salicylate poisoning.
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Pseudopapilledema—drusen bodies
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These are granular deposits in the optic disk that cause pseudopapilledema. The distinctions between early papilledema and drusen bodies are reported as follows: drusen cause obvious irregular, lumpy, bumpy elevation of the disk; drusen are pink or yellow, the surface of papilledema is hyperemic; drusen cause the nerve fiber layer to glisten and often show a halo of feathery reflections, whereas the layer in papilledema is dull; drusen are in the disk center; in papilledema, the vessels show absence of venous pulsation and the light reflexes are dulled; drusen make the disk outline irregular and are more frequent in small, hypermetropic eyes.
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Distention of retinal veins suggests retinal vein occlusion, polycythemia vera, cyanotic congenital heart disease, leukemia, and macroglobulinemia.
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Hemorrhage may occur in any layer of the retina. The shape of the hemorrhage frequently reveals its depth. A large, deep hemorrhage in the choriocapillaris produces a dark, elevated area that looks like a melanotic tumor (Fig. 7-37A); you should suspect a subretinal vascular membrane seen in association with macular degeneration. A smaller, more superficial hemorrhage appears as a round red spot, with blurred margins, called a blot hemorrhage (Fig. 7-37B). Microaneurysms are also round red spots, but their borders are sharp, they are not reabsorbed like hemorrhages, and they may occur in clusters about vascular sprigs (Fig. 7-37C). Flame-shaped hemorrhages occur in the nerve fiber layer of the retina; they are red and striated (Fig. 7-37D and Plate 23). In the subhyaloid or preretinal hemorrhage, a pool of blood accumulates between the retina and the hyaloid membrane forming a boat-shaped hemorrhage appearing as a turned-up half-moon; the straight upper border is a fluid level (Fig. 7-37E). A small hemorrhagic spot with a central white area is called a Roth spot, (Fig. 7-37F) classically seen in subacute bacterial endocarditis and leukemia. CLINICAL OCCURRENCE: Many conditions produce retinal hemorrhages, examples are hypertension, diabetes mellitus, papilledema, retinal vein occlusion, SBE, HIV, SLE, Takayasu arteritis, macroglobulinemia, thiamine deficiency, leukemia, polycythemia, sickle cell disease, and sarcoidosis.
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Diabetic retinopathy often leads to blindness from damage to the macular region. Microaneurysms occurring around the macula need to be distinguished from blot hemorrhages. With advanced diabetic retinopathy there are white or yellow waxy exudates having distinct, often serrated, borders (Plate 24). The exudates gradually coalesce to form a broken circle around the macula. Neovascularization of the disk or neovascularization elsewhere in the retina is an ominous sign requiring laser phototherapy (Plates 25 and 26). The signs of atherosclerosis and hypertension are sometimes superimposed [Frank RN. Diabetic retinopathy. N Engl J Med. 2004;350:48–58]. DDX: Although microaneurysms around the macula are characteristic of diabetes, retinal microvasculopathy with cotton–wool spots, intraretinal hemorrhages, and microaneurysms occurs in radiation retinopathy and in some patients with HIV-AIDS.
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Sudden loss of vision occurs when the central retinal artery is occluded, usually from thrombosis or embolism. Early the retina is very pale from ischemic edema; the arteries are extremely narrowed, and the smaller ones are invisible (Fig. 7-38A and Pate 21). Although the veins are full, they are pulseless. The lack of circulation is demonstrated by the absence of induced pulsation in either artery or vein with pressure on the eyeball. Retinal edema initially causes macular pallor that is less dense over the fovea because of an anatomical lack of the nerve fiber layer at this location. The fovea appears as a cherry red spot because of the visualization of the choroidal blood flow within the macular edema; this finding will disappear as the edema resolves over weeks. Occlusion of a branch artery causes findings limited to its distribution area. CLINICAL OCCURRENCE: Common causes are vascular disease, cardiac valve disease or vegetations, rheumatic fever, and vasculitis, most commonly temporal arteritis. Rarely, it is a complication of SLE, sickle cell disease, cryoglobulinemia, syphilis, or thromboangiitis obliterans.
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Central retinal vein thrombosis is followed by engorgement and tortuosity of all visible veins (Fig. 7-38B). Significant nerve fiber layer and blot hemorrhages appear throughout the retina. Edema of the macula and disk is commonly present. Occlusion of a branch of the central vein produces findings limited to its drainage area. CLINICAL OCCURRENCE: Venous occlusive disease is most associated with hypertension, as the stiffened arterioles will compress the more compliant retinal veins as they cross in their common sheath. Hypercoagulable states can also cause venous occlusion from sluggish blood flow and can be seen in polycythemia, multiple myeloma, macroglobulinemia, and leukemia. In sickle cell disease, multiple venous thromboses may be accompanied by neovascularization.
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The degree of involvement of the retinal vessels by the arteriolar sclerotic process has been variously scored. Table 7-2 represents the Kirkendall and Armstrong modification of the Scheie classification. The retinal changes do not necessarily parallel atherosclerotic disease elsewhere in the body.
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Normal retinal arteries show a central bright stripe from light reflecting off the curved blood column. Increases in wall thickness cause widening of the stripe and brightening of the reflex. In moderate disease, the walls look like burnished copper (copper wire reflex); in advanced disease, the entire width of the artery reflects as a white stripe (silver wire reflex).
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Normal vessel walls are invisible. Wall thickening with lipid infiltration produces a milky white streak on either side of the blood column called pipestem sheathing.
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Arteriovenous crossings
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As the arterial and arteriolar walls become denser and less compliant, arteriovenous crossing signs are produced (Fig. 7-39A). Arteriovenous nicking (Fig. 7-39B) occurs when the thickened arterial sheath obscures a short segment of the more compliant vein, seen as a notch on either side of the artery at their crossing. Deviation of the vein is produced when the stiffened artery causes the vein to assume a 90 degrees crossing angle (Fig. 7-39C); the normal angle is acute. When an overlying vein is elevated by the thickened artery, it is called humping (Fig. 7-48D). Tapering of the veins results if the artery compresses the vein (Fig. 7-39E). When venous flow is partially obstructed, the vein dilates upstream from the artery, called banking (Fig. 7-39F), which may induce a retinal vein occlusion.
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Hypertensive retinopathy
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Arterial hypertension produces distinctive retinal signs that often coexist with the signs of arteriolar sclerosis (Plates 22 and 23). For example, the appearance of a retina may be classified as “grade 3 arteriolosclerosis, grade 4 hypertension.” The signs attributed to hypertension may also be graded by using the classification of Kirkendall and Armstrong (Table 7-3) [Wong, T, Mitchell P. The eye in hypertension. Lancet. 2007;369:425–435]. Most ophthalmologists will simply describe the retinal and vascular finding without the use of these grading scales.
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Many diseases and pathogenic process leave their marks in the retina as scars, deposits, pigmentation, etc. In addition, active retinal disease and systemic diseases with retinal manifestations can appear as unifocal or multifocal spots against the normal retina. Careful retinal examination of patients with confusing systemic disease may assist in diagnosis. The clinician’s first task is to distinguish active disease from the residuals of past events.
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Ulcerated atherosclerotic plaque in the aorta or carotid artery sheds cholesterol crystals that become lodged at the bifurcations of retinal vessels. Patients may be asymptomatic or present with transient monocular visual loss, amaurosis fugax, or transient ischemic neurologic attacks (TIA) in a carotid distribution. Finding cholesterol emboli proves plaque rupture with embolization. It is difficult, if not impossible, to differentiate cholesterol emboli from calcific emboli in the diseased heart valves.
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Thickening and swelling of the terminal retinal nerve fibers results from ischemic infarcts. Gray to white areas with ill-defined fluffy borders occur within the posterior pole of the retina (Plate 22). They are often accompanied by microaneurysms that may rupture producing small striate hemorrhages known as flame hemorrhages. CLINICAL OCCURRENCE: Hypertension, diabetes, SLE, HIV, central retinal vein occlusion, papilledema.
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This is lipid deposition from leaking capillaries left behind as a high water mark after the retinal pigment epithelium resorbs the associated serous fluid. These are small white spots with sharply defined edges. They are deeper than the superficial retinal vessels and the superficial cotton wool patches.
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Old inflammation or scarring may appear as a pigmented region of the retina.
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White or yellow spots are seen in the retinae of intravenous drug users who have injected ground-up tablets containing talc.
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Cytomegalovirus (CMV) retinitis
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Advanced immunosuppression from HIV infection is frequently accompanied by cytomegalovirus infection of the retina. Patients describe visual loss, blurring, floaters, and flashes of light. Look for whitening of the retina, cotton-wool spots, and intraretinal hemorrhages. Although less common, consider varicella zoster infection, toxoplasmosis, and syphilis [Cunningham ET, Margolis TP. Ocular manifestations of HIV infection. N Engl J Med. 1998;339:236–244]. CMV retinitis has become less common with the advent of highly active antiviral therapy.
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Candida endophthalmitis
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Systemic Candida infection associated with immunosuppression and indwelling venous catheters can be difficult to diagnose. Patients have fever, but blood cultures are often negative. Small white patches on the retina may be the only sign of disease. With advancing disease, there is pain, visual disturbance, and large white globular lesions are seen invading the vitreous.
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Although vision is much reduced, the only visible sign may be a few spots of pigment near the macula and blurring of the macular borders. In other cases, subretinal hemorrhages, patches of retinal atrophy, yellow drusen, and pigmented areas may be seen.
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Inherited singly or as a component of several syndromes, retinitis pigmentosa is manifested by arteriolar narrowing, waxy pallor of the optic disc, and perivascular retinal pigmentation. Night blindness is the earliest symptom; later, all types of vision are greatly impaired as the retinal degeneration progresses from the periphery to the posterior retina. Spidery strands of pigmented spots form a girdle about the global equator (Fig. 7-40A).
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These probably represent elastic tissue degeneration. Broad lines of pigment radiate from the optic disk, branching like blood vessels (Fig. 7-40B). They occur in Paget disease and in pseudoxanthoma elasticum.
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Retinal detachments may be symptomatic or asymptomatic. Patients complain of flashing lights followed by floaters and then a curtain crossing their vision. The earliest sign is elevation of a retinal area so that it is out of focus with the surrounding structures. The arteries and veins in the separated membrane appear elevated (Fig. 7-41). When widely separated, the retinal sheet is gray and frequently folded. Underlying inflammation may produce areas of choroiditis and vitreous opacities. A torn edge may be encountered, often shaped like a horseshoe. The cause of detachment is often undetermined.
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Epistaxis (nosebleed)
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In the anterior nose, the most common site of bleeding is Kiesselbach plexus, a vascular network in the anterior nasal septum. Posteriorly, hemorrhage occurs frequently at the back third of the inferior meatus; vessels in the region are large and belong to the external carotid artery system. In some cases, there are multiple oozing points in the mucosa. Nosebleed can be a spontaneous and trivial occurrence or a sign of serious local or generalized disease. Hemorrhage from the external nares is obvious, but bleeding from the choana must be distinguished from hemoptysis and even hematemesis. The first problem is to ascertain the bleeding site and judge whether trauma or some predisposing disease is present. Inquire about trauma, predisposing local or systemic disease, and the amount of blood lost. To examine the patient observe universal precautions: use gloves, gown, and face protection to avoid blood contamination. Remove the clots by suction or have the patient clear the nose by blowing. Inspect the anterior nasal chambers, especially the septum. If hemorrhage is so profuse as to obscure the site, advance the sucker tip backward in small increments, clearing the blood at each step, until a point is reached where the passage immediately fills after clearing; this is the bleeding site. Blood-tinged fluid suggests a CSF leak. Consult textbooks for methods of arresting hemorrhage. CLINICAL OCCURRENCE: Local Causes: coughing, sneezing, nose picking, fractures, lacerations, foreign bodies, adenoid growth, nasopharyngeal fibroma, angioma, rhinitis sicca. Generalized Causes: Congenital: hereditary telangiectasia; Inflammatory/Immune: Granulomatosis with polyangiitis (Wegener), lethal midline granuloma; Infectious: viral rhinitis, typhoid fever, scarlet fever, influenza, measles, infectious mononucleosis, diphtheria, pertussis, psittacosis, Rocky Mountain spotted fever, erysipelas, mucosal leishmaniasis; Metabolic/Toxic: pernicious anemia, aspirin, scurvy; Mechanical/Trauma: (see local causes) changes in atmospheric pressure (mountain climbing, caisson disease, flying) exertion; Neoplastic: nasopharyngeal carcinoma, squamous cell carcinomas, leukemia; Vascular: coagulopathy, cirrhosis, uremia, hemophilia, von Willebrand disease, thrombocytopenia; Elevated Arterial Pressure: hypertension, aortic coarctation; Elevated Venous Pressure: cor pulmonale, congestive heart failure, superior vena cava syndrome.
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Nasal and maxilla fracture
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Nasal fractures are simple or comminuted; seldom are they compound. A blow from the side displaces both nasal bones to the opposite side, producing an S-shaped curve in the dorsum nasi. The septum may be fractured with or without nasal bone fracture. Frontal blows depress the nasal bones. Palpation along the inferior border of the orbit may disclose an irregularity indicating fracture of the maxilla; a fragment may displace downward into the sinus. Malocclusion of the teeth indicates displacement of the maxilla. Fracture of the zygoma produces flattening of the cheek.
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Lesions of CN I, often shearing of the nerve ending passing through the cribriform plate, or nasal obstruction produce loss of smell. Anosmia is invariably accompanied by a perceived change in the taste of food, which seems bland and unpalatable. The most common identified cause is closed head trauma.
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Congenital nasal deformities
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Disturbances in development of the nose are myriad, but they pose little problem in diagnosis. Perhaps the most common is cleft nose from incomplete fusion at the tip and dorsum (Fig. 7-42B).
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Acquired nose deformities
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Acquired deformities are commonly the result of trauma, infection, or neoplasms. Rhinophyma is an erythematous bulbous enlargement of the distal two-thirds of the nose from multiple sebaceous adenomas (Fig. 7-42A). It may follow longstanding rosacea. Saddle nose is distinguished by the sunken bridge (Fig. 7-42C) that results from loss of cartilage; common causes are septal hematoma or abscess. Rarely, it can follow relapsing polychondritis, granulomatosis and polyangiitis (Wegener), or congenital or acquired syphilis. A crooked nose results from fracture.
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Vestibule folliculitis
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Mild inflammation around the hair follicles is evident on inspection.
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Vestibule furunculosis
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A small superficial abscess forms in the skin or mucous membrane. The area is extremely tender, swollen, and reddened. Swelling may involve the nasal tip, alae nasi, and upper lip (Fig. 7-43A). Avoid instrumentation or other trauma to pyogenic lesions within the triangle anterior to a line from the corners of the mouth to the glabella; this may cause spread of the infection directly to the cavernous sinus.
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Fissures often develop at the mucocutaneous junction. They become overlaid with crusts that cover tender surfaces.
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In the adult the nasal septum is seldom precisely a midline structure. The cartilaginous and bony septum may deviate as a hump, spur, or shelf to encroach on one nasal chamber, occasionally causing obstruction. Columnar dislocation of the septum may occur.
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The cartilaginous septum is usually involved. Perforation is commonly caused by chronic infection, repeated trauma in picking off crusts, as a result of nasal or transphenoidal pituitary surgery, or cocaine abuse. Perforation is readily demonstrated by looking in one naris while a light shines in the other (Fig. 7-43B).
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Even slight trauma to the nose may produce bleeding under the mucoperichondrium, usually causing bilateral hematomas. Nasal obstruction necessitates breathing through the mouth. The hematoma is seen as a violaceous, compressible, obstructive mass. The columella may be widened; the nasal tip pales from stretching of the skin. Pressure by the hematoma on the anterior ethmoidal nerve may cause anesthesia of the tip. Long-standing hematomas interfere with the blood supply of the septum, causing slow necrosis of the cartilage and a saddle nose deformity.
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The septum swells into both nasal chambers, and the mucosa is edematous. Infection of a septal hematoma invariably results in loss of cartilage; it must be immediately incised, drained, and treated with appropriate antibiotics. There is risk of progression through the angular veins to produce cavernous sinus thrombosis.
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Children frequently put objects into the nose that remain for long periods and produce foul, purulent unilateral discharge.
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Sinus carcinomas cause obstruction, bloodstained discharge, and constant, boring pain. They invade bone: in the orbit, they may cause ocular disturbances; in the maxillary antral floor, upper teeth may become loose, a denture may no longer fit properly, or bulging and softness of the hard palate is seen.
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Cerebrospinal rhinorrhea
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A traumatic fistula occurs between the subarachnoid space and the nasal cavity. A unilateral discharge of clear spinal fluid occurs after head injury or surgery. The fluid may be blood tinged but is easily distinguished from nosebleed. Jugular vein compression increases the flow. If spinal fluid is suspected, the specimen should be tested for b2-transferrin. There is substantial risk for meningitis; recurrent meningitis should lead to a search for CSF leak.
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Nasal discharge—acute suppurative sinusitis
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Nasal discharge—chronic suppurative sinusitis
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Sinusitis and periorbital edema—periorbital abscess
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Sinusitis and periorbital edema—orbital cellulitis
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Nasal polyps are sessile or pedunculated mucosal overgrowths developing after recurrent episodes of mucosal edema. They are frequently seen in long-standing allergic rhinitis, aspirin-sensitive asthma, and cystic fibrosis. Polyps are commonly multiple, most frequently protruding from the middle meatus as smooth, pale, spheric mucosal masses (Fig. 7-44B). Polyps may enlarge to obstruct the air passages; they frequently recur after removal. DDX: they are mobile and insensitive, distinguishing them from swollen turbinates.
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Periorbital masses—mucocele and pyocele
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Permanent obstruction of the frontal or ethmoid sinus orifices causes mucus secreted by their mucosae to accumulate. The resulting sac, or mucocele, slowly enlarges; the pent-up mucus exerts pressure on the surrounding structures and erodes bone, behaving like a neoplasm. The sac may eventually erode through the floor of the frontal sinus or the lateral ethmoid wall producing a painless swelling beneath the supraorbital ridge, medial to the ocular globe (Fig. 7-45). The painless mass feels rubbery and slightly compressible. The globe is pushed downward and laterally, causing diplopia; proptosis may also occur. Upward and medial motions of the eye are restricted. Intranasal examination may be negative. An infected mucocele is termed a pyocele. DDX: Swelling from the mucocele occurs above the inner canthus; dacryocystitis forms a swelling below the canthus.
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Benign papillomas are often found in the vestibule. Slow-growing, benign neoplasms of the sinuses are usually osteomas or chondromas. They grow slowly and cause no symptoms until air passages or a sinus orifice is obstructed. Inverted papillomas grow downward into the underlying tissues so are difficult to resect.
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The cause is unknown, but some classify it as one of the angiocentric immunoproliferative lesions. The inflammation is attended by granuloma formation. It is most common in fifth and sixth decades, with a slight preference for women. Symptoms include sneezing, nasal stiffness, obstruction, and pain. Signs are rhinorrhea, nasal congestion, and paranasal sinusitis progressing to inflammation and ulcerations of the nasal septum, palate, and nasal ali. Advanced disease is indicated by destruction of midfacial structures including pharynx, mouth, sinuses, and eyes with death from cachexia, pneumonia, meningitis, or hemorrhage. Indolent ulceration and mutilation suggest the diagnosis. DDX: Unlike Wegener granulomatosis, here there is no systemic involvement or primary vasculitis.
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Granulomatosis and polyangiitis (Wegener)
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Breath odor is an important diagnostic sign. There is great variation in olfactory acuteness, and description of odors is meaningless; experience is necessary. A foul breath odor, fetor oris, is common in infection (dental, tonsillar) atrophic rhinitis, putrefaction of food (achalasia, esophageal diverticula, pyloric obstruction), and infected sputum (bronchiectasis, lung abscess). Acetone on the breath indicates ketonemia in diabetic or starvation acidosis. In some patients with uremia, ammonia is detected. A curious musty odor occasionally is smelled in patients with severe liver disease. When a person has inhaled or swallowed volatile hydrocarbons, the odor is detectable in the exhaled air. Alcohol on the breath indicates recent ingestion, but medical illness, trauma, or the ingestion of other drugs must be excluded as comorbid conditions. A few patients in coma have no alcohol odor on the breath, whereas the aspirated gastric contents smell strongly of alcohol. The chronic alcoholic may smell of acetaldehyde instead of alcohol. When garlic is eaten, the methyl mercaptan causing its odor excreted from the lungs for more than 24 hours.
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In the embryo, incomplete fusion of the frontonasal process with the two maxillary processes leaves a persistent cleft in one or both sides of the upper lip. Cleft lip is sometimes accompanied by cleft palate.
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The lips may appear large in cretinism, myxedema, acromegaly, and collagen injections.
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Labial vesicles—herpes simplex (cold sores, fever blisters)
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Latent herpes simplex virus is reactivated producing local inflammation when the carrier develops another infectious disease, has local trauma, or is exposed to sunlight. Groups of vesicles containing clear fluid are surrounded by areas of erythema. Frequently, they occur on the lips. The lesions may burn or smart.
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Cheilosis (angular stomatitis)
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Maculopapular and vesicular lesions are grouped on the skin at the corners of the mouth and the mucocutaneous junction (Fig. 7-46A). Irritation of the skin leads to crusting and fissuring. Often accompanying profuse salivation from any cause, it is specifically associated with riboflavin deficiency or ill-fitting dentures; secondary Candida infection is common (perlèche). The entire labial surface may be inflamed from overexposure to sunlight, actinic cheilosis.
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Painful localized swelling with erythema and increased skin warmth suggests early cellulitis or carbuncle. When it occurs on the upper lip, it may be exceedingly dangerous, because the veins drain into the cavernous sinus.
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An early lesions is indurated and discoid; later, it becomes warty and crusted, forming a shallow ulcer that slowly extends. The ulcerated border is elevated, sometimes pearly (Fig. 7-46B). Regional lymph nodes are involved late. It is more frequent in men and 95% of the cases are on the lower lip. Biopsy all the ulcers that are more than 2 weeks old.
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The initial lesion of syphilis occurs at the inoculation site. The lip is the most common extragenital site of primary syphilitic chancre; usually the upper lip is involved. The lesion is discoid, without sharply defined borders and can be moved over the underlying tissues. It soon ulcerates to exude a clear fluid teeming with Treponema pallidum. The regional lymph nodes are involved early and feel larger and softer than carcinomatous nodes. Serologic tests for syphilis are frequently negative while the chancre is present.
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Molluscum contagiosum
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A nodular growth in the lip may ulcerate to discharge caseous material. The ulcer border may be elevated. The lesion is caused by Molluscipoxvirus. The resemblance to carcinoma may be striking, so biopsy may be required.
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The white radial scars about the angles of the mouth are stigmata of previous syphilitic lesions (Fig. 7-46C).
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A dry, flat, light-colored precancerous growth occurs on the lip and produces scaling; it bleeds easily.
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Lip pigmentation—Peutz–Jeghers syndrome
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Multiple pigmented brown to black spots on the lips resemble freckles (Fig. 7-46D); freckles are uncommon on the mucosa. This autosomal dominant syndrome is associated with intestinal polyposis and an increased risk for gastrointestinal cancer.
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Lip telangiectasias—hereditary hemorrhagic telangiectasia
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The most obvious lesions may occur on the buccal mucosa, tongue, and lips. See Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease).
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Oral mucosa and palate signs
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Xerostomia, Sjögren syndrome
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Buccal pigmentation—Addison disease
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Small patches of pigment in the buccal mucosa are common in blacks and other darkly pigmented races. In whites, however, dappled brown pigment in the lining of the cheek strongly suggests Addison disease or Peutz–Jeghers syndrome.
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An obstructed mucous gland produces a blue-domed translucent cyst anywhere on the buccal surface.
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Mucosal sebaceous cysts (Fordyce spots)
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The lip, cheek, and tongue mucosa show isolated white or yellow, sometimes slightly raised, spots <1 mm in diameter. Often a bit of white sebum may be expressed from the lesion. They are painless and harmless.
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Koplik spots (measles)
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Koplik spots are the earliest diagnostic sign of the measles; they are pathognomonic. One or two days before the exanthem appears, small white spots appear opposite the molars, and sometimes elsewhere, on the buccal mucosa (Fig. 7-47B). Each is surrounded by a narrow red areola.
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The lesions are thin, bluish-white, spiderweb lines resembling leukoplakia. Circumscribed areas of flattened papules on the flexor surfaces of the wrists and the middle of the shins strengthen the diagnosis of lichen planus (Lichen planus).
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This often occurs at the site of chronic irritation from ill-fitting dentures or smokeless tobacco; it is precancerous. Tobacco and alcohol are cocarcinogens. The first lesion is a whitened hyperkeratotic plaque. On the tongue, one or more areas on the dorsal surface show obliteration of the papillae with thin white lesions that are wrinkled and sometimes pearly. Early lesions coalesce; as they persist and enlarge, they become chalk white and thick and are palpably more firm than the adjacent mucosa. Biopsy is indicated.
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Infection of the oral mucosa with Candida spp. occurs in patients who are diabetic, immunosuppressed (e.g., HIV, immunosuppressant drugs), or have received broad spectrum antibiotics. The lesions may be painless or cause mouth soreness. Examination reveals white plaques that are easily removed with a tongue blade. Less commonly, the mucosa is erythematous and thin, without the white plaques. Pain with swallowing suggests concomitant Candida esophagitis.
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Telangiectasias—hereditary hemorrhagic telangiectasia
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Early and obvious lesions occur on the buccal mucosa, tongue, and lips. See Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease).
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Oral vesicles, blisters, and ulcers
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Several diseases result in oral vesicles or bullae, often with multiple ulcerations. The major mechanisms of disease are infection- and immune-mediated processes. Larger ulcers result from tissue destruction because of infectious, neoplastic, or metabolic causes.
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Primary infection with herpes simplex causes severe stomatitis with painful vesicles that rupture to form shallow ulcers, which heal slowly.
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Infection with coxsackievirus 16 results in fever and sore throat. On examination, there are small vesicles or whitish papules on the soft palate.
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Painful chronic ulcers may be surrounded by the characteristic lacy white mucosal lines.
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Disseminated histoplasmosis
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A persistent oral ulcer can be the presenting sign of disseminated histoplasmosis. Diagnosis is made by biopsy.
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Cicatricial pemphigoid
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Autoantibodies directed against hemidesmosomes in the basal layer of the mucosa and skin lead to the separation of the epithelial layers with blister formation. Pain is mild to moderate. The incidence increases with age. Oral lesions may be accompanied by skin lesions (Fungal Infections). The course is chronic and recurrent. It must be distinguished from pemphigus vulgaris (Fungal Infections).
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Stevens–Johnson syndrome. This is a severe allergic reaction with generalized involvement of the skin and mucous membranes. The most common cause is medication exposure. Early recognition, withdrawal of the offending agent, and supportive therapy may be lifesaving.
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Aphthous ulcer (canker sore)
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A few small vesicles appear in crops on the tip and sides of the tongue and on the labial and buccal mucosa. After vesicle has ruptured, the lesion is a small, round, painful ulcer with a white floor, yellow margins, and surrounding narrow erythematous areola. The cause is unknown. Recurrent or persistent aphthous ulcers are seen in Crohn disease and Behçet syndrome [Scully C. Aphthous ulceration. N Engl J Med. 2006;355:165–172].
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Mucous patches (condyloma latum)
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This is the common lesion of secondary syphilis, occurring on the tongue and the buccal and labial mucosa regardless of the site of the primary lesion. The patches are round or oval, 5 to 10 mm in diameter, slightly raised, and covered by gray membrane. They may ulcerate slightly. They feel indurated and are painless. Regional lymphadenopathy is common.
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Ionizing irradiation, especially of the mandible, leads to suppression of bone turnover and acute or delayed necrosis of bone with ulceration of the overlying mucosa. Bisphosphonate therapy (especially intravenous bisphosphonates for malignant hypercalcemia and myeloma) also suppresses bone turnover and is associated with bone necrosis and ulceration of the overlying mucosa. Patients present with one or more slowly progressive often painful ulcerations exposing underlying bone. A history of irradiation or bisphosphonate use is essential for making the diagnosis. Formerly, exposures to white phosphorus in the munitions industry caused a similar syndrome known as phossy jaw [Woo SB, Hellstein JW, Kalmar JR. Bisphosphonates and osteonecrosis of the jaws. Ann Intern Med. 2006;144:753–761].
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The bone marrow and oral and intestinal mucosa are the most rapidly proliferating tissues in the body. Cytotoxic chemotherapy transiently stops proliferation and leads to impaired mucosal repair. Mouth ulcers commonly occur 5 to 10 days after intermittent bolus cytotoxic chemotherapy. They can occur anytime in the course of chronic oral therapy with alkylating agents or antimetabolites.
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Reddened parotid duct orifice—mumps
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The parotid (Stensen) duct orifice, opposite the upper second molar, may become reddened in mumps or other acute parotitis.
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Bony palate protuberance—torus
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This is a common anatomic variation; a bony knob or ridge occurs in the midline of the hard palate. It is harmless.
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There are many causes for high-arched palate. It is common in Marfan and Turner syndromes.
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A midline opening in the hard palate results from congenital failure of fusion of the maxillary processes. It is usually associated with cleft lip but also occurs in isolation. Its severity varies from a complete cleft of the entire soft and hard palate, including the alveolar ridge, to a partial cleft of the soft palate alone.
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This results from incomplete cleft of the soft palate and may be accompanied by disoriented palatal muscles. Test for elevation of the uvula; deviation of the uvula and asymmetry of the soft palate suggest muscular abnormality.
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Wide interdental spaces
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This may occur congenitally or be acquired as the jaw enlarges in acromegaly.
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Usually the presence of cavities in the teeth is obvious. Decreased saliva production following irradiation or with sicca syndrome is associated with an increased risk for caries.
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Loss of dental enamel results from erosion by regurgitated acidic stomach contents in patients with bulimia nervosa and by acid water in swimming pools with excessive chlorination.
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Opaque chalk-white spots, 1 to 2 mm in diameter, are seen scattered on the surface of multiple teeth. This is a harmless condition found exposure to large amounts of fluoride in the water during childhood.
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Notched teeth—Hutchinson teeth
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This results from congenital syphilis. The permanent upper central incisors are misshapen (Fig. 7-48A). They are smaller than normal and peg topped, resembling the frustum of a cone; the tips are notched. Notching is one component of the Hutchinson triad, together with interstitial keratitis and labyrinthine deafness.
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An abscess forms at the root tip within the bone. The inflammation produces a very painful increase in intraosseous pressure. An abscess suspected when toothache pain is accentuated by tapping the tooth with a tongue blade or probe. Tender swelling occurs in the adjacent gum and a draining sinus tract may form.
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Gum bleeding signals local gum lesions or generalized disorders of the blood vessels or hemostasis. The patient may complain of bleeding accompanying brushing or may notice blood in expectorated phlegm. Other signs of a systemic vascular or hemostatic process should prompt direct questioning about bleeding from the gums because many patients do not consider this abnormal.