S ECT I O N

21 Eyelids

234 BLEPHAROCHALASIS 374.34

John H. Sullivan, MD

San Jose, California

of elastin fibers in the quiescent stage. Fat atrophy eventually results in deep superior sulcus and a thin atrophic bronzecolored eyelid skin which resembles parchment. Multiple fine telangiectatic subcutaneous vessels are present and blepharoptosis results from thinning and atrophy of levator aponeurosis. Dehiscence of lateral canthal tendon causes horizontal phimosis and rounding of the lateral canthus.

ETIOLOGY

Blepharochalasis, a term coined by Fuchs a century ago, is a rare condition characterized by recurrent, episodic, painless periorbital edema, either unilateral or bilateral, which in its acute phase resembles angioedema. Evidence suggests an immunopathogenic mechanism, but the cause remains unknown. After repeated attacks, the eyelid skin loses elasticity and develops a characteristic thin, discolored appearance that resembles wrinkled cigarette paper or parchment. The upper eyelid fat pads may eventually atrophy, causing a typical skin concavity and psuedoepicanthal fold. The term blepharochalasis is frequently misused to indicate redundant skin of the aging eyelid, a common condition more accurately termed dermatochalasis.

●The pathogenesis is uncertain, but this condition may be immunogenic. Abundant IgA deposits are found around elastin fibers.

●Infrequently, occurrence is familial.

●There is an equal sex distribution.

●Sometimes blepharochalasis is associated with systemic illness, such as amyloidosis, dermatomyositis, or leukemia.

●There is an association with edema of the lips and thyroid enlargement in Laffer-Ascher syndrome.

DIAGNOSIS

The diagnosis is established by history and clinical features. There are no characteristic laboratory findings.

Differential diagnosis

●Angioedema: usually older age of onset; edema is seldom limited to the upper eyelids. There is an association with complement 1-esterase inhibitor deficiency and autoantibodies.

●Idiopathic lymphedema: unremitting brawny edema, usually in older patients.

●Dermatochalasis: a common aging process with redundant skin and fat prolapse.

●Tumor: progressively enlarging mass; the diagnosis is confirmed by biopsy results.

●Floppy lid syndrome: typically found in older, obese men; there is an association with papillary conjunctivitis.

TREATMENT

Active/acute phase: supportive

●Cold compresses can be used.

●Topical and systemic steroids are of limited value.

COURSE/PROGNOSIS

The initial onset is usually before the age of 20 with unilateral or bilateral swelling of the eyelids and conjunctiva which can last hours to several days.

Proptosis indicates orbital involvement and the frequency and severity of repeated episodes tend to lessen with age. Occasionally, attacks are aggravated by a triggering event, such as fever, upper respiratory infection, menstruation, or weeping. Lacrimal gland prolapse is common and in the early phase, atrophy of the skin and orbital septum exposes the fat pads. There are perivascular inflammatory infiltrates during the acute stage and loss

Quiescent/late stage: surgical

●Excise the redundant skin.

●Reposition the ectopic lacrimal gland.

●Reconstruct the lateral canthal tendon.

●The repair of blepharoptosis is often challenging.

COMMENTS

Little can be done to ameliorate the acute phase of blepharochalasis. The avoidance of triggering mechanisms, when present, may diminish the frequency of attacks. Immunopathologic studies are of interest, but as yet have not elucidated the

Eyelids • 21 SECTION

dence of this disease increases with age. Keratoconjunctivitis sicca, seborrheic dermatitis and acne rosacea are frequently observed along with meibomian dysfunction. Chalazion incidence is higher in these patients. Tear break-up time is usually reduced.

Various lid margin findings may be observed on examination, including erythema, architectural irregularity, thickening, injection and telangiectasia. Meibomian gland apertures may be displaced, irregular, decreased in numbers, pouting with secretions, or plugged with yellow concretions. In meibomian dysfunction, pressure applied to the lid margins may not expulse Meibomian secretions or it can produce large amounts of cloudy or foamy secretions.

Laboratory findings

Currently, specific diagnostic tests for blepharoconjunctivitis are not available.

Eyelid and conjunctival cultures may be performed in cases of suspected staphylococcal blepharitis, especially if the condition is chronic or worsening with treatment. Antibiotics should be discontinued before culture. Of eyelid cultures from patients with chronic blepharitis, 96% were positive for S. epidermidis, 93% were positive for Propionibacterium acnes, 77% were positive for Corynebacterium sp., 11% were positive for Acinetobacter sp., and 10.5% were positive for S. aureus. Results of aerobic and anaerobic cultures from lids and conjunctiva showed that the only group of patients with blepharitis who had a significant percentage of positive cultures for S. aureus were those with clinically defined staphylococcal and mixed staphylococcal/seborrheic blepharitis. More than 80% of patients in the mixed group have positive eyelid cultures for S. aureus, and 50% have positive conjunctival cultures.

TREATMENT

Systemic

●Systemic antibiotics (occasionally for severe cases), such as tetracycline.

Ocular

●Warm compresses to eyelids for 5 to 10 minutes b.i.d.

●Eyelid scrubs using baby shampoo or a commercial lid scrub b.i.d.

●Bacitracin (first choice) or erythromycin ointment to the lid margins after lid scrubs and to the cul-de-sac at bedtime.

●Rarely, short-term topical steroids for hypersensitivity corneal infiltrates.

●Preservative-free artificial tears and other dry eye treatments.

●Dermatologic consultation for seborrheic dermatitis.

PRECAUTIONS

Conjunctivitis medicamentosa may occur, especially in patients with associated keratoconjunctivitis sicca. Eyelid scrubs, soaps, and artificial tear preservatives may also cause hypersensitivity and allergic reactions. The use of preservative-free tears is recommended. Discontinuation of all potentially toxic medication

should result in significant improvement of keratitis medicamentosa. Alternative medical treatments must be considered in patients with specific known drug allergies or intolerance to drug-induced side effects. Use of tetracyclines during pregnancy is contraindicated.

Occasionally, more severe keratitis, phlyctenules, and corneal ulceration may require more intensive therapy.

COMMENTS

Blepharoconjunctivitis is commonly encountered by the ophthalmologist. Close observation of the specific eyelid, conjunctival, and corneal changes will help determine the type of blepharoconjunctivitis present and thus determine the type of treatment regimen best suited for the individual patient. Eyelid hygiene and topical antibiotics will cure or control staphylococcal or mixed seborrheic/staphylococcal blepharoconjunctivitis. When these therapies fail, attention should be given to other possible diagnoses or additional underlying problems. Discontinuation of all therapy and culturing or reculturing of the eyelids and conjunctiva may be indicated.

REFERENCES

Bowman RW, Dougherty JM, McCulley JP: Chronic blepharitis and dry eyes. Int Ophthalmol Clin 27:27–35, 1987.

Bowman RW, Miller K, McCulley JP: Diagnosis and treatment of chronic blepharitis. In: Focal points: clinical modules for ophthalmologists. San Francisco, American Academy of Ophthalmology, 1988.

Driver PJ, Lemp MA: Meibomian gland dysfunction. Surv Ophthalmol 40:343–367, 1996.

Groden LR, Murphy B, Rodnite J, et al: Lid flora in blepharitis. Cornea 10:50–53, 1991.

McCulley JP: Blepharoconjunctivitis. Int Ophthalmol Clin 24:65–77,

1984.

McCulley JP, Dougherty J, Deneau DG: Classification of chronic blepharitis. Ophthalmology 89:1173–1180, 1982.

Smolin G, Okumoto MA: Staphylococcal blepharitis. Arch Ophthalmol 95:812–816, 1977.

236 DISTICHIASIS 743.63

Phillip Hyunchul Choo, MD

Sacramento, California

Sumaira A. Arain, MD

Sacramento, California

Distichiasis is a condition in which lashes grow posterior to the normal row of lashes. It can be either congenital or acquired and may present as only a few isolated eyelashes, or as a complete accessory row of lashes. These lashes often rub directly on or become matted against the cornea, causing persistent eye pain and corneal damage.

236 CHAPTER Distichiasis •

Eyelids • 21 SECTION

ETIOLOGY/INCIDENCE

Congenital distichiasis

This is a rare condition, which may occur sporadically or as an autosomal dominant trait with variable expressivity and high penetrance. It is a developmental anomaly in which embryonic pilosebaceous units differentiate into hair follicles rather than into meibomian glands.

Congenital distichiasis can be isolated or occur in association with some forms of familial lymphedema, especially the 4th type of late-onset hereditary lymphedema that presents during adolescence. Other associated abnormalities include entropion, ptosis, congenital corneal hypesthesia, cleft lip and palate, vertebral anomalies, extradural cysts, webbed neck, yellow nails, peripheral vascular anomalies, trisomy 18, congenital heart defects, blepharocheilodontic syndrome and other systemic anomalies.

Acquired distichiasis

This is a more common form, and is often seen secondary to certain stimuli that provoke a metaplastic change within or near the meibomian gland. This metaplastic change can occur with diseases that affect both the eyelid margin and the conjunctiva, such as chronic blepharitis, staphylococcal hypersensitivity, meibomian gland dysfunction and meibomianitis, Stevens-Johnson syndrome, ocular cicatricial pemphigoid and chemical ocular injuries.

COURSE/PROGNOSIS

Distichiasis, if left untreated, may lead to corneal thinning, scarring, or even ulceration.

DIAGNOSIS

On slit-lamp examination, abnormal lashes are noted arising from or near the meibomian gland orifices. The cornea should be evaluated for any epithelial defects or signs of permanent scarring.

Clinical signs and symptoms

Symptoms may include decrease in vision, eye pain and irritation, foreign body sensation and tearing.

A penlight or slit lamp examination should reveal eyelashes growing posteriorly (along where one would expect the orifices of the meibomian glands to be) to the normal row of lashes. There may be secondary signs such as epithelial breakdown of the cornea, corneal scarring or pannus formation, and conjunctival injection and chemosis.

Differential diagnosis

Distichiasis should be differentiated from trichiasis and entropion. Trichiasis is a condition in which eyelashes grow from the normal anterior portion of the eyelid margin but are turned in or misdirected towards the eye. Entropion is a condition in which the entire eyelid margin turns inward.

PROPHYLAXIS

Proper and chronic treatment of diseases such as blepharitis and ocular cicatricial pemphigoid will decrease the stimuli to create the formation of acquired distichiatic eyelashes.

TREATMENT

Medical

No treatment may be necessary if the patient is asymptomatic and without keratopathy. For temporary symptomatic relief of epithelial breakdown of the cornea, lubricating drops and ointment are useful. Therapeutic soft contact lenses may also be helpful to protect the cornea in cases of corneal epithelial breakdown, as well as to provide symptomatic relief.

Surgical

Various surgical procedures have been described for the treatment of distichiasis. Epilation with electrolysis is useful in treating small numbers or a focal area of distichiatic lashes. This procedure is often done in the office with a local infiltrative block, while general anesthesia is required in treating infants and young children. With the aid of a slit-lamp or an operating microscope, the distichiatic eyelashes are identified and electrolysized. Various instruments such as the Ellman Surgitron (Ellman International Inc., Oceanside, NY) are commercially available for performing electrolysis of eyelashes. While performing electrolysis, it is important to treat the entire hair follicle. The eyelash should pull out effortlessly from the eyelid margin after treatment. If it does not, the follicle should be retreated. The success rate for trichiatic eyelashes is approximately 60–70%, and thus repeat treatment may be necessary, especially when treating multiple eyelashes.

Cryosurgery is an option for the treatment of a wider area of distichiasis. A double freeze-thaw treatment down to −20ºC is recommended for permanent destruction of the lash follicles. Since temperatures of −30ºC and lower may cause tissue necrosis and subsequent scarring, the use of a thermocouple to monitor actual tissue temperature is recommended. Furthermore, this approach is non-selective. The normal, more anterior eyelashes may also be permanently damaged by cryotherapy, leading to complete madarosis. For this reason, eyelid splitting approaches are more commonly used to treat wide areas of distichiatic eyelashes.

Lid splitting procedures involve an incision made in the eyelid margin immediately anterior to the row of distichiatic eyelashes. The use of a chalazion clamp and an ocular surgery blade are helpful. The incision is continued to about 3 mm in depth to expose the entire distichiatic eyelash follicle. Depending on the surgeon’s preference, the distichiatic lash follicles can then be treated either with direct application of electrolysis, direct excision, or cryotherapy applied to the conjunctival side of the eyelid margin. Later, the incision may be closed directly with sutures, or the anterior lamella can be recessed approximately 2 mm in relation to the posterior lamella and fixated to the tarsal plate.

In 1993, O’Donnell and Collin reviewed a series of 24 patients with distichiasis who underwent treatment with epilation, cryotherapy, or a lid splitting procedure followed by cryotherapy to the posterior lamella. They found that lid splitting with cryotherapy relieved symptoms without retreatment in 87% of patients. In 1997, Vaughn and others reported the results of an eyelid splitting procedure in 17 eyelids of 5 patients with distichiasis. After the lid was split, each aberrant eyelash follicle was individually excised or microhyphrecated and subsequently removed. This approach yielded excellent functional and cosmetic results. Furthermore, White in 1975 described a surgery in which the posterior portion of the eyelid margin containing the distichiatic eyelashes was directly excised. A mucous membrane graft was then sutured in place to cover the defect.

COMPLICATIONS

All of the above treatments could result in recurrence of distichiasis, formation of trichiasis, lid notching and cicatricial entropion.

Cryotherapy may result in significant post-operative swelling of the lid and the conjunctiva. Furthermore, cryotherapy applied to the conjunctiva may aggravate an underlying conjunctival disorder, as well as cause conjunctival scarring and shortening. In addition, lid necrosis can occur if the treatment temperature is −30ºC or less.

Eyelid splitting procedures may also lead to some potential complications. These include prolonged eyelid edema, keratinization of the posterior eyelid margin, and recurrence of the distichiatic eyelashes. Lastly, one should avoid damage to the normal eyelash follicles to avoid permanent madarosis.

Supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York.

REFERENCES

Anderson RL, Harvey JT: Lid splitting and posterior lamella cryosurgery for congenital and acquired distichiasis. Arch Ophthalmol 99:631–634, 1981.

Choo PH: Distichiasis, trichiasis, and entropion: advances in management. Int Opthalmol Clin 42(2):75–87, 2002.

Frueh BR: Treatment of distichiasis with cryotherapy. Surg Ophthalmol 12:100–103, 1981.

O’Donnell BA, Collin JR: Distichiasis: management with cryotherapy to the posterior lamella. Br J Ophthalmol 77:289–292, 1993.

White JH: Correction of distichiasis by tarsal resection and mucous membrane grafting. Am J Ophthalmol 80:507–508, 1975.

237 ECTROPION 374.10

Kostas G. Boboridis, MD, PhD

Thessaloniki, Greece

Ectropion is an eyelid malposition presenting from simple horizontal laxity to an outward turning of the lid margin away from its normal apposition to the globe. It may involve either eyelid but it mainly affects the lower.

ETIOLOGY/INCIDENCE

Ectropion is classified as both congenital and acquired. Acquired ectropion is further classified into four categories according to the underlying etiology, as involutional, paralytic, cictricial, or mechanical (Figure 237.1).

Congenital ectropion is caused by vertical shortage of the anterior lamella on the lateral third of the lower eyelid often combined with canthal tendon laxity. It is frequently associated with additional facial abnormalities as in Down syndrome, blepharophimosis and Möbius syndrome or lamellar ichthyosis. Isolated ectropion due to horizontal lid laxity is very rare congenital condition called euryblepharon.

Involutional, or senile, ectropion is the most common type encountered in the aging population. Senile stretching or weakening of the medial and more frequently the lateral canthal

tendon, dehiscence of the lower lid retractors, atrophy of the orbicularis muscle, and hypertrophy of the tarsus with fragmentation of its elastic and collagen fibers result in an outward turning of the lid. Involutional enophthalmos may exacerbate the condition. Although the upper eyelid can be affected in a condition called floppy eyelid syndrome, involutional ectropion usually is described for the lower eyelid.

Paralytic ectropion is caused by facial nerve palsy and is often unilateral. Bell’s palsy, trauma, cerebrovascular accidents, and damage to the peripheral seventh nerve during tumor removal are the most frequent causes. Loss of orbicularis muscle tone and decreased support to gravitational pull by stretching of the canthal tendons results in ectropion which becomes more pronounced with time.

Cicatricial ectropion is the result of scarring or contracture of the skin and/or orbicularis muscle causing vertical shortening of the eyelid due to trauma, inflammatory processes or skin disorders. Overzealous blepharoplasty of the lower lid can result in a cicatricial ectropion or lid retraction.

Mechanical ectropion is caused by the weight of a mass on the anterior lamella or mid-face ptosis, pulling the lid down. A tumor of the lower eyelid, fluid accumulation secondary to sinusitis, thyroid disease, lupus, or other medical problems can result in mechanical ectropion. Patients with mid-face ptosis can have sagging of the cheek and face, resulting in a mechanical ectropion.

COURSE/PROGNOSIS

All forms of ectropion, especially involutional and paralytic, worsen with time if untreated, resulting in conjunctival hypertrophy and keratinization. The increased exposure of the ocular surface may result in poor lubrication of the corneal epithelium with an increased risk of exposure keratopathy and infection. Punctum eversion, sagging of the lid and lacrimal pump failure usually result in chronic epiphora.

DIAGNOSIS

Clinical signs and symptoms

The main symptoms are chronic epiphora and recurrent conjunctivitis. Examination of the eyelid position and contour, punctum position and size, orbicularis function, Bell’s phenomenon, tear film stability and patency of the lacrimal system as well as recognition of cicatricial components are all necessary in evaluating ectropion. The degree of horizontal laxity is assessed with the distraction test, by drawing the center of the eyelid away from the globe and measuring the distance. More than 8–10 mm is considered abnormal. The eyelid elasticity and orbicularis tone are assessed with the snap back test by pulling the lid from the globe and releasing it. An eyelid that snaps back only after the patient has blinked is considered abnormal. A lateral pull on the lid reveals medial canthal tendon laxity when the punctum is drawn to or beyond the medial limbus. Medial pull reveals lateral canthal tendon laxity when the palpebral angle is pulled for more than 10 mm from the lateral orbital rim.

TREATMENT

There is no systemic treatment.

237 CHAPTER Ectropion •

Ocular

Asymptomatic or mild cases require only topical artificial tear drops. Intense lubrication and antibiotic ointment are indicated for symptomatic patients with chronic conjunctivitis. The eyelids may need to be taped closed at bedtime, especially in paralytic cases. Softening of the skin and lid margin with topical steroid ointment is beneficial in advanced cases. Intralesional steroid injection may soften the scar in cicatricial ectropion. Surgical intervention is reserved for chronic symptomatic cases that require permanent solution.

Surgical

Congenital

Severe and symptomatic cases require a full-thickness skin graft for vertical lengthening of anterior lamella combined with canthal tendon tightening.

Involutional

For mild cases, the inverting suture technique can shorten the posterior lamella and provide temporary relief. Three doublearmed sutures are passed in mattress fashion through the lid,

from the palpebral conjunctiva at the inferior border of the tarsus out cutaneously at the level of the orbital rim.

For generalized horizontal laxity, tightening of the lid can be performed by resecting a full-thickness wedge of the eyelid at the lateral canthal angle, as described by Bick. However, this does not address and rather exacerbates canthal tendon laxity resulting in rounding of the fissure.

For punctum eversion a medial spindle procedure is performed, in which a diamond-shaped excision of tarsoconjunctiva is performed in the posterior lamella bellow the punctum. A double armed absorbable suture engaging the retractors closes the defect and exits through the skin below the level of incision, effectively acting as an inverting and posterior lamella shortening suture.

When there is punctum eversion with horizontal laxity, the ‘lazy T’ procedure is indicated in which the medial spindle is combined with a medial full thickness wedge excision. If medial tendon laxity is present a canthal ligament (anterior or posterior limp) plication is also performed.

The method of choice to correct involutional ectropion and any form of horizontal laxity is the lateral tarsal strip or canthal

238 CHAPTER Entropion •