Ординатура / Офтальмология / Английские материалы / Clinical Ocular Pharmacology 5th edition_Bartlett, Jaanus_2008

claw-like appendages, which allow it to easily grasp the widely spaced hair located in those regions; for this reason it is also referred to as the “crab” louse. Body or head lice are much less likely to inhabit these regions because their bodies are much narrower, which prevents them from easily grasping the hair. The parasites survive by sucking the blood of their host, which has not been shown to transfer disease. However, the fecal material and saliva excreted by the parasites can be both toxic and antigenic, resulting in an inflammatory response manifested by conjunctivitis, marginal keratitis, and preauricular lymphadenopathy.

Diagnosis

Diagnosis is made based on careful slit-lamp examination, which readily detects the eggs (nits) attached to the eyelashes or eyebrows (Figure 23-20).The adult lice vary in size from 1.0 to 1.5 mm and have a translucent body, which makes them more difficult to visualize. After they have fed, reddish brown fecal material in the lower abdomen can be easily seen (Figure 23-21), and occasionally they may be seen moving on the eyelash margin.

Severe itching and irritation characterize phthiriasis palpebrarum. Blepharoconjunctivitis, blood-stained thickened discharge from fecal matter on the lid margins, nits, and adult parasites on the eyelashes may all be visible.

Faint bluish-gray spots, known as maculae caeruleae, may be seen which are caused by a salivary enzyme conversion of bilirubin to biliverdin. A preauricular lymphadenopathy may also be present.

Management

The scalp and body, including the pubic areas, should be treated as well as the eyelids. In addition, for treatment to

Figure 23-20 Heavy infestation of nits seen on the upper eyelashes. (Courtesy Dr. Laura Kenyon, University of Houston, College of Optometry.)

Figure 23-21 Phthiriasis palpebrarum attached to lid. Head (large black arrow); claws grasping eyelashes (small white arrows). Fecal matter may be viewed as a dark spot located in the lower end of the louse abdomen (large white arrow). (Courtesy Dr. Laura Kenyon, University of Houston, College of Optometry.)

be effective thorough investigation and treatment of all contacts should be performed, including family members and sexual partners.

In cooperative patients the nits, which are strongly attached to the eyelashes, can be mechanically removed. This procedure is most easily performed using threeprong forceps and attempting to slide the egg case toward the tip of the eyelash.When removal is not possible,lashes bearing eggs should be epilated.Typically,there are so many nits that not all the lashes can be epilated; consequently, this may be accomplished over several visits. The practitioner can remove the adult parasites with forceps using the slit lamp, but again this procedure is somewhat uncomfortable,especially for children. Other reported treatments include cutting the eyelashes at their base, cryotherapy, and pharmacologic eradication. Most notably, bland petrolatum ointment can be thickly applied twice daily for 2 weeks to smother the parasites. However, this particular treatment has little effect on the nits, and therapy should be continued twice daily for 10 to 14 days to ensure that all the eggs have hatched and that the emerging parasites have been adequately treated. Care must be taken to examine the lids for live organisms, and treatment must continue until no lice or viable nits are present.

Anticholinesterase agents, such as 0.25% physostigmine ointment, are also a viable treatment option and may be applied to the lid margins. Side effects, such as miosis and browache, may limit their use. Gamma benzene hexachloride should be avoided on treating the lid condition because of potential ocular irritation and

chemical conjunctivitis. Similarly, pyrethrin gel and other pediculicides should not be used near the eye. One reported study cited the efficacy of a one-time application of sodium fluorescin (NaFl) 10% to 20% swabbed onto the lid margins as an in-office procedure. It was reported to eradicate all live louse and nits; however, no further studies have been done to support this claim. An oral antihelmintic agent, Ivermectin, given in two doses of 200 mcg/kg one week apart, has also surfaced as a louse eradicator. It has been reported that within 2 days all lice were killed with this method; however, it cannot be used in those weighing less than 15 kg and only with caution in pregnant or breast-feeding women.

Scalp, body, and pubic hair must be treated with an appropriate pediculicidal agent in combination with careful nit removal via fine-tooth comb.Although lindane (gamma benzene hexachloride), an insecticide, is generally considered the drug of choice for the treatment of head and pubic lice, a pyrethrin-based pediculicide (RID) is equally effective and is available over the counter. A single application to the affected body areas is usually adequate to eradicate the lice.The application should be repeated in 1 week if viable nits persist or if new nits appear. A few all natural products containing essence of fruit oils have been developed.These agents are reported to eradicate infestation in a two-step process that uses a one-time shampoo application and a follow-up rinse for prevention of reinfestation. There is no comb-out necessary, and lice are reported to be killed within 40 minutes of a single application.Translucent empty nits are signs of inactive infestation and require no further treatment. Because lindane may lead to central nervous system toxicity, it must be used cautiously in infants, children, and pregnant women, and excessive application or exposure should be avoided. None of the above-mentioned treatment options is approved for use around the eye or on the eyelids.

It is necessary to examine and treat family members or sexual contacts due to the high risk of reinfestation. Clothing, linens, and grooming instruments should be laundered or sterilized by exposing to dry heat at 140°F (50°C) for 20 to 30 minutes. This heat sterilization can usually be accomplished at the highest temperature settings of most household dryers. Contaminated cosmetics should be discarded.

BENIGN TUMORS OR PAPILLOMAS

Verrucae

Verrucae, commonly known as warts, are benign skin tumors that can affect any part of the body, including the eyelids. The morphology of these benign lesions is quite characteristic. A verruca vulgaris is a raised,multilobulated, grape-like mass of tissue that is attached to the body by a stalk (pedunculated) of varying thickness (Figure 23-22A). A verruca plana is a round,slightly raised,flat wart (sessile)

varying in size from a few millimeters to several centimeters in diameter. It is cauliflower-like and pitted in appearance and may be darkly pigmented (Figure 23-22B). A cutaneous horn is a cornified verruca vulgaris (Figure 23-22C). Because cutaneous horns are keratinized and firm,they do not have the fleshy-soft consistency of verruca vulgaris.

Etiology

Verruca vulgaris, verruca plana, and cutaneous horn are forms of viral warts produced by the human papilloma virus (HPV).They are caused by an overgrowth of normal epithelium that may become keratinized and pigmented. Because they occur only in the superficial tissue layer, the body does not recognize them as foreign and therefore does not mount an immune reaction. They are most commonly found in children and young adults and may

be spread by direct contact; thus the eyelid or face is usually a secondary site of infection. Because these lesions are viral in nature, they tend to shed viral toxins and desquamated epithelium onto the conjunctiva, which sometimes results in a secondary mild chronic conjunctivitis.Verrucae on the eyelids or in close proximity to the globe are most symptomatic. Of particular importance are warts that occur on the lid margin among the lashes.

Diagnosis

The most common type of wart to occur on the face and lid area is the flat wart (verruca plana).These are round, slightly raised, 2 to 6 mm in diameter, tan to yellow-pink, and with a granular surface.They may be quite numerous and even confluent. A small black center is not uncommon and represents thrombosed blood vessels.

Management

Verrucae are self-limiting but can be very serious in the immunosuppressed. Treatment is primarily cosmetic but also prevents further dissemination. Most verrucae lesions resolve spontaneously after several months to years; therefore therapy should be conservative. Because the lesions are localized to the epidermis, most treatments are limited to this level and should not result in scarring. Benign treatments include topical applications of irritants; salicylic acid and lactic acid,applied under an occlusive barrier,can be purchased over the counter. More advanced treatment modalities include cryotherapy, surgical removal, or electroor chemical cautery. Neither of these cautery methods is suitable for lesions on the lid margin because of the risk to the ocular surface.

Sudoriferous (Mucoid/Moll) Cysts

Etiology/Diagnosis

Sudoriferous cysts are small, round, translucent, elevated masses caused by blockage of the ducts of Moll’s glands. One or more lesions, ranging from 2 to 4 mm in diameter, may be observed on the anterior eyelid margin.The cysts are painless but can occasionally cause irritation or interfere with successful contact lens wear. They are filled with clear watery fluid (Figure 23-23).

Management

Most cysts do not require treatment; however, excessively large lesions or ones that cause ocular irritation can be managed surgically.The most common treatment involves puncturing the center of the lesion to allow for drainage; however, they tend to reform after this type of treatment. The lesions rarely reappear if the dome of the cyst is excised.

Figure 23-24 Milia located on the medial aspect of the right upper eyelid. (From Kanski JJ. Eyelids. In: Clinical ophthalmology: a systematic approach. Philadelphia: Butterworth-Heinemann, 2003:15.)

Sebaceous Cysts

Etiology/Diagnosis

Sebaceous cysts are benign retention cysts of sebum. They often appear in the geriatric population due to aging. Milia are small (0.5 mm), round, sebaceous cysts that tend to remain intracutaneous (Figure 23-24). They are common on the eyelids, are whitish in color, are found away from the lid margin, and cause little irritation.They are important only from a cosmetic standpoint.

Subcutaneous sebaceous cysts are yellowish in color, may be larger than milia (up to 10 to 12 mm), are asymptomatic, and are firm to the touch (Figure 23-25). The capsule and its contents are moveable under the overlying skin. Often the plugged orifice of the gland duct is visible.

402 CHAPTER 23 Diseases of the Eyelids

These cysts can occur singly or in groups and are bothersome only from a cosmetic perspective.

Management

Milia are easily removed without the use of anesthesia. A small stab incision is carefully made through the surface of the lesion using the point of a no. 11 disposable scalpel or of a 25or 27-gauge hypodermic needle. The sebum contained in the cyst is expressed with cotton-tipped applicators or smooth forceps.The interior of the cyst is then cauterized with dichloroacetic acid applied with a sharpened wooden applicator.The removal site is usually invisible in 2 weeks.

Subcutaneous sebaceous cysts must be removed by total excision, because simple incision usually results in recurrence.

Xanthoma Palpebrarum (Xanthelasma)

Xanthoma palpebrarum is an elevated yellowish discoloration that occurs most commonly in women during the fourth and fifth decades of life.The lesions usually occur bilaterally on the medial aspect of the upper eyelids (Figure 23-26).There is no race predilection.

Etiology

Xanthelasma is caused by an infiltration of the dermis by xanthoma cells, which are benign histiocytes that imbibe lipids. The condition may occur independently, without associated systemic disease, or may be a manifestation of hypercholesterolemia or other associated disturbance of lipid metabolism.

Patients with xanthelasma, particularly younger individuals, should be evaluated for elevated serum lipid levels, because 30% to 50% will have hyperlipoproteinemia. Among the remaining 50% to 70%, some have subtle changes in lipid composition that may indicate a tendency toward atherosclerotic changes. Thus, in addition to

Figure 23-26 Xanthelasma of the medial right upper eyelid.

cosmesis, the major concern is the potential for atherosclerotic cardiovascular disease, as well as possible systemic disorders such as diabetes mellitus and cirrhosis.

Diagnosis

The diagnosis of xanthelasma is made based on the clinical presentation of the cutaneous lesions.They are oval or elongated yellowish plaques occurring just beneath the skin. There is no concomitant inflammation or pain, but they may be of cosmetic concern.

Management

Removal is only considered when cosmesis is of primary concern. Treatment modalities include chemical cautery, electrodesiccation, cryotherapy, laser ablation, or surgical excision. Complications of laser and cryotherapy include scar formation and pigmentary changes. Chemical cautery and surgical excision tend to produce better results with less scarring. Recurrence is extremely high and must be considered before initiating any of the aforementioned treatment options.

MALIGNANT PERIOCULAR LESIONS

The periocular area is a common site for malignant cutaneous lesions. Five percent to 10% of all skin cancer affects the lid and surrounding areas. Basal cell carcinomas (BCCs) are by far the most common lesions, followed by squamous cell carcinoma (SCC) and sebaceous cell carcinoma. Any of these malignancies can be fatal if there is orbital invasion with intracranial spread; for this reason it is prudent to discuss the etiology, diagnosis, and management options. Risk factors for development include, most notably, ultraviolet radiation exposure. Another potential etiologic factor implicated in BCC, SCC, and actinic keratosis (AK) is HPV. It has been shown that HPV-DNA can be detected in up to 50% of patients with BCC, in up to 60% of patients with SCC, and in over 90% of AK patients. Careful observation and documentation of all suspicious lid lesions are paramount to accurate and timely diagnosis. The clinician is urged to use a simple list of notable characteristics, termed the “ABCs” (Box 23-3).

Box 23-3 ABCs of Suspicious Skin Lesions

A––Asymmetric shape

B––Border irregularity

C––Color mottling of variability

D––Diameter > 6 mm

E––Elevation

Data from Myers M, Gurwood AS. Periocular malignancies and primary eye care. Optometry 2001;72:706.

Basal Cell Carcinoma

BCCs represent the most common form of human malignancy. Roughly 80% to 90% of all BCCs occur on the head and neck and 20% of those occur on the lid or lid margin. BCCs account for 90% of all eyelid tumors; thus extreme care must be taken when evaluating any suspicious eyelid lesion.The incidence is 500/100,000 people in the United States, with 60 years the average age at diagnosis.

There are three forms of BCC: nodular, sclerosing, and ulcerative. Not all malignancies exhibit the typical pearly rounded boarders that have come to denote the diagnosis of a BCC. BCCs tend to occur on the lower lid and in the medial canthus, are slow growing, locally invasive, and are only rarely metastatic. Because of their destructive nature, it is imperative that they be diagnosed early to prevent the mutilation that is inherent with some of the more invasive treatment options.

Etiology

BCCs arise in the basal cell layer of the epidermis and are insidious in nature.They then invade the tarsus and, if left unchecked, break through the orbital septum into the orbit. Most are thought to be caused by an overexposure to sunlight, which is why they are often found on exposed areas of the body such as the face, ears, neck, scalp, shoulders, and back. There have been reports of BCC on unexposed areas of the skin, but this is atypical. Other reported risks include exposure to arsenic, radiation, or after tattooing. These factors have not been adequately proven and are anecdotal. Patients most at risk are fair-skinned individuals with blond or red hair and blue or green eyes; BCC is seen much less often in darkskinned people. Other risks include sun exposure early in childhood, sun exposure due to job or leisure activity, or living in extremely sunny climates. In at least one study cigarette smoking was implicated as being a risk factor for development of BCC in women.

Diagnosis

The most common warning signs of a BCC are a lesion that is present for months to years, is changing in size or shape, tends to bleed, or has remained open for at least 3 weeks. Other hallmark signs are the pearly borders and telangiectatic vessels present across the lesion surface (Figure 23-27), just under the epithelium. Most BCCs are painless, unless secondarily infected, and are firm to the touch.

Nodular BCCs are typically shiny or translucent elevated lesions, of any color, that resemble a mole. They may occur anywhere on the lid or lid margin and might be confused with a papilloma. It is important to remember that papillomas do not grow over time, do not bleed, and have a “normal” overlying skin appearance. The nodule may ulcerate, forming the most easily recognized BCC,

Figure 23-27 Basal cell carcinoma of the right upper eyelid. Note the telangiectatic vessels right below the skin surface and the loss of cilia over much of the lesion’s surface. (Courtesy Dr. Justina Taube, University of Houston, College of Optometry.)

with the typical shiny rolled border and ulcerated center (Figure 23-28).

Sclerosing, infiltrating, or morpheaform BCCs are flat indurated plaques that have very ill-defined margins, which make the skin appear shiny and taut.These lesions may easily be confused with chronic blepharitis, which may present with tylosis ciliaris and irregular lid margins (Figure 23-29) but does not cause a significant alteration in the position or the destruction of the marginal tissue and cilia.

Any suspicious lesion that appears to alter the surrounding skin, causing loss of eyelashes or irregular lid margins, which cannot be determined as benign, must be referred for biopsy; this is the only true method to diagnosis a malignancy. It is not uncommon to confuse a BCC

Figure 23-28 Basal cell carcinoma with typical shiny rolled borders and an ulcerated center. (From Kanski JJ. Eyelids. In:Clinical ophthalmology:a systematic approach. Philadelphia: Butterworth-Heinemann, 2003:21.)

404 CHAPTER 23 Diseases of the Eyelids

Figure 23-29 Sclerosing basal cell carcinoma mimicking chronic blepharitis. (From Kanski JJ. Eyelids. In: Clinical ophthalmology: a systematic approach. Philadelphia: Butterworth-Heinemann, 2003:21.)

for sebaceous cell carcinoma or SCC because they share common cutaneous characteristics. One distinguishing factor is that BCCs have a much slower growth rate than most other lid malignancies.

Management

Management always includes referral to a dermatologist or oculoplastic surgeon for confirmatory biopsy and treatment. Treatment options include immune system modulators, 5-fluorouracil, chemotherapy, curettage and electrodesiccation, surgical excision, radiation, Mohs’ micrographic surgery, cryoor laser surgery, and photodynamic therapy.The cure rate is 95% posttreatment, which depends on the size, location, and histopathology of the malignancy.

Sebaceous and Squamous Cell Carcinomas

Sebaceous cell carcinomas are relatively rare, but when they do occur it is usually in the sebaceous glands of the eyelids, namely the meibomian glands. They may resemble benign conditions, such as chalazion or unilateral blepharoconjunctivitis, which may cause a delay in the diagnosis and lead to local invasion and the potential for metastasis to lymph nodes and other organs. It is for this reason that they have a higher morbidity and mortality rate than a BCC. The mean age at diagnosis is similar to BCC, being more common in older (>60 years) adults, but a greater frequency is seen in females. Lesions are more common in the upper lid followed by the lower. Sebaceous cell carcinomas are difficult to discern (Figure 23-30) unless they have well-defined borders; a high degree of suspicion should exist when a patient presents with a recurrent chalazion in the same area. Hallmark signs of any malignant lid lesion are destruction of the overlying skin features and eyelash loss. When these lesions occur on the upper lid, they tend to have a yellow appearance. Management includes prompt referral to an oculoplastics specialist for biopsy and treatment (see Management, above, for BCC).

SCCs are epithelial malignancies that are extremely invasive, fast growing, with the potential to be fatal.They are rare, accounting for only 5% to 10% of all eyelid tumors with an incidence of 0.09 to 2.42/100,000. SCCs are often associated with actinic (solar) keratosis (see below) and are found most often on the lower eyelid or lid margin of elderly men but may be found on the upper eyelid as well (Figure 23-31). Because of their suspected actinic origin, they often occur after radiation treatment for other lesions. SCCs can metastasize but do so more often if they are large, if they deeply penetrate underlying tissue, if they have an undifferentiated histologic subtype,

Figure 23-31 Squamous cell carcinoma of the left upper eyelid margin. (From Kanski JJ. Eyelids. In: Clinical ophthalmology: a systematic approach. Philadelphia: ButterworthHeinemann, 2003:22.)

or if the patient is immunocompromised. Diagnosis and treatment options are similar to BCC and sebaceous cell carcinoma (see above).

PREMALIGNANT OR KERATINOCYTIC INTRAEPIDERMAL NEOPLASIA

Actinic Keratosis

Etiology

AKs are common, sun-induced, inflammatory skin lesions traditionally defined as being “premalignant.” Recently, it was suggested they be reclassified as a malignancy “in situ”because they do possess the capability of converting to a neoplasm, usually SCC. The incidence of an AK converting to an SCC is 0.075% to 0.096% per lesion per year. AKs that are large or found on the lips are more likely to convert to an SCC. Pathogenesis begins with ultraviolet exposure, causing a morphologic change in keratinocytes and leading to the classic AK lesions seen clinically.As stated previously, HPV is also a possible etiologic factor in development.

Diagnosis

Clinically, AKs present as a broad, rough, pink or red lesion with an overlying thick yellow scale (Figure 23-32). Management is controversial because many lesions have been reported to spontaneously resolve. Proponents of treatment point out that because there is a possibility of conversion to an SCC and because many treatment options are noninvasive and well tolerated, most lesions, even if they are small, should be treated.

Management

Treatment options include destructive therapy (i.e., cryotherapy, curettage, or shave excision), field destruction (i.e., ablative laser resurfacing, dermabrasion, and

Figure 23-32 Actinic keratosis of the upper eyelid showing roughened surface with overlying scale. (From Kanski JJ. Eyelids. In: Clinical ophthalmology: a systematic approach. Philadelphia: Butterworth-Heinemann, 2003:16.)

chemical peel), or topical chemotherapy (i.e., 5-fluorouracil, diclofenac, topical amino levulinic acid and blue light exposure, tretinoin, or Imiquimod). The treatment is selected based on the size, number, and location of the lesions.

LID AND LASH ANOMALIES

Trichiasis and Distichiasis

Etiology

Trichiasis is an acquired condition in which some or all of the eyelashes are directed inward toward the globe. It is most often the result of aging; however, it may also be caused by an inflammatory process or trauma that causes scarring and fibrosis around the eyelash follicles at the lid margin. Potential etiologies include cicatricial conjunctivitis, trachoma, herpes simplex and herpes zoster, chronic blepharitis,lacerations,burns,and postsurgical procedures.

Distichiasis can be an acquired or, rarely, a congenital condition in which there is an accessory row of eyelashes emanating from the meibomian gland orifices. When congenital, it may occur sporadically or may be autosomal dominant.

Both conditions can cause a wide range of symptoms, the most common a foreign body sensation and a red irritated eye. Severe or debilitating symptomatology is a result of corneal surface damage, including corneal abrasion and superficial punctate keratitis. Corneal hypoesthesia with subsequent neurotrophic ulceration is also possible.

Diagnosis

Occasionally, the involved lashes are without pigment, making them very difficult to see; therefore diagnosis is

406 CHAPTER 23 Diseases of the Eyelids

based on a careful slit-lamp examination. Whenever the lid margin is altered or if entropion is present, meticulous external assessment is necessary to rule out the possibility of a malignancy.

Management

Treatment depends on the severity of the condition and patient symptoms. In mild cases, with only a few lashes involved, epilation is easiest to perform and has no side effects except for mild discomfort during the procedure. Electrolysis may also be preformed using the Pro-Lectro ophthalmic epilator (Figure 23-33). With this handheld instrument, a mild current is generated and directed into the lash follicle. Once destruction has occurred, the lash may be easily removed with forceps. Cryoablation and diathermy may also be used for mild cases.When severe, surgical correction is needed. Surgery poses more serious

A

B

Figure 23-33 (A) Pro-Lectro ophthalmic epilator. (B) Stylus being inserted into empty eyelash follicle for electrolysis.

postoperative complications and is therefore reserved for the most severe cases.

Lagophthalmos

Lagophthalmos is a common condition in which the eyelids do not fully close, either with a blink or during sleep.

Etiology

The etiology depends on the type of lagophthalmos present. Physiologic or nocturnal lagophthalmos occurs when the eyelids do not fully close during sleep. Orbital lagophthalmos is due to severe proptosis, as in Graves’ thyroidopathy (see Chapter 32). Mechanical lagophthalmos is secondary to scarring of the lid muscle or other lid tissue. Paralytic, the most common form of lagophthalmos, occurs secondary to Bell’s palsy (see Chapter 22) and incomplete blink of unknown etiology.

Diagnosis

The diagnosis of lagophthalmos or incomplete blink is usually made based on the patient’s symptoms, slit-lamp examination, and gross observation during a blink. Patients usually complain of ocular irritation, which is worse upon awakening. Biomicroscopy reveals SPK over the inferior portion of the cornea or over the area of exposure. The patient should be asked to blink while at the slit lamp; they should be closely examined outside of the slit lamp, which will often reveal the exposure; or occasionally a family member will confirm that the patient sleeps with his or her eyes open.

Management

Management in most cases is aimed at relieving symptoms unless the lagophthalmos is orbital or paralytic; in these cases the underlying cause must be addressed. If the exposure is mild and nocturnal, lubricating ointment at bedtime is indicated. If this does not resolve the signs and symptoms, taping the lids at bedtime or having the patient wear a sleep mask may be helpful. In more severe cases a moisture chamber, made from a pair of swim goggles, may be used at night. If the lagophthalmos is mechanical, artificial tears used every few hours during the day may be necessary. If there is evidence of a secondary bacterial infection, appropriate antibiotic ointment and/or drops should be initiated. Bandage contact lens wear may also be indicated. In very severe or long-stand- ing cases, oculoplastic surgery may be necessary.

EYELID HYPERLAXITY

Floppy Eyelid Syndrome and Lax Eyelid

Syndrome

Eyelid hyperlaxity has been reported to include two separate syndromes, floppy eyelid syndrome (FES) and lax eyelid syndrome (LES). FES was first reported in 1981 and

was described by the clinical triad of obesity, easily everted “floppy” upper eyelids, and an associated chronic papillary conjunctivitis. Patients are usually middle-aged obese men who have nonspecific symptoms, either monocular or binocular, which include a thick mucoid discharge and a nonspecific ocular irritation that is worse upon awakening. There have been reported cases in women, children, and the nonobese; however, this is the exception, not the rule.The condition is usually bilateral but tends to be worse on the side on which the patient sleeps.

LES was reported in 1994 and is thought to be much more prevalent. It is described as having similar characteristics as FES, such as lid hyperlaxity and ocular irritation, but it does not include the other classic findings of obesity, easily inverted tarsus, and/or papillary conjunctivitis.

Both syndromes are thought to be associated with obstructive sleep apnea (OSA) and normal tension glaucoma; however, this correlation remains questionable.

Etiology

FES has been associated with keratoconus, hyperglycinemia, obesity, and OSA, which suggests mechanical abnormalities, metabolic dysfunction, degenerative processes, and connective tissue disorders as possible etiologies.The exact mechanism for both the eyelid hyperlaxity syndromes remains unknown. FES maybe related to a decrease in the elastin fiber content of the tarsus and thus a loss of integrity, causing eyelid eversion during sleep, which creates mechanical irritation of the lids, cornea, and conjunctiva. In addition, poor contact between the loose upper eyelid and the globe, found in both syndromes, may interfere with distribution of the tear film over the cornea and conjunctiva, creating ocular surface drying and irritation.

In FES it is thought that sleeping with an everted eyelid causes the pretarsal orbicularis oculi and skin to override the lid margins, which causes the eyelashes to point downward (eyelash ptosis). Because some patients with FES also have keratoconus,an underlying connective tissue disorder may be implicated.

Although the reason remains unclear, patients with FES and LES are frequently found to have some degree of sleep disorder of breathing.The most likely relationship is an elastic tissue abnormality.

Diagnosis

A “rubbery,” hyperlax, easily everted upper tarsus that “rolls” outward when the lid is mechanically elevated (Figure 23-34) and eyelashes that point downward and curl either toward the cornea or in various directions are reliable indicators of FES. Upper eyelid vertical hyperlaxity is determined by mechanically elevating the upper lid to its maximum position and measuring the distance between the lid margin and the center of the pupil; hyperlaxity of the lid is considered to be a measurement

Figure 23-34 Floppy eyelid syndrome. (From Kanski JJ. Eyelids. In: Clinical ophthalmology: a systematic approach. Philadelphia: Butterworth-Heinemann, 2003:39.)

equal to 15 mm.The upper eyelid may also hyperextend when the lid is pulled downward. A mucoid discharge with papillary conjunctivitis is also seen.The conjunctival inflammation is thought to be caused by rubbing of the palpebral tarsal conjunctiva on the bedding during sleep. Another factor is the poor contact between the lax upper eyelid and the globe (seen in both FES and LES). In addition, the patient may be observed by a family member sleeping with one or both of the upper eyelids everted.

LES is diagnosed in much the same way; however, the patient may not be obese or may not have an easily everted upper tarsus.A careful history, including sleeping patterns, is very useful information.

Management

Treatment consists of ocular lubrication for symptoms or signs of dry eye and treating any secondary bacterial infection with an appropriate topical antibiotic. Topical lubricants alone usually cannot control the symptoms of FES or LES. Preventing lid eversion generally requires lid taping or use of nocturnal eye shields.The definitive treatment, however, is surgical tightening of the eyelid and therefore requires an oculoplastics consult.

Because OSA is a cause of considerable morbidity and mortality, the clinician is advised to recommend sleep studies if the patient reports heavy snoring or other symptoms of OSA. A few cases of resolved hyperlaxity eyelid syndromes with treatment for OSA have been reported.

EYELID MYOKYMIA OR BENIGN EYELID TWITCHING

Eyelid myokymia or benign eyelid twitching is a common localized form of facial myokymia. It is a transient condition in which mild to moderate fine undulating contractions of the orbicularis muscle occurs,causing an annoying