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Chapter 1 – Eyelids

INTRODUCTION 2 Anatomy 2 Terminology 3 General considerations 3

BENIGN NODULES AND CYSTS 4 Chalazion 4 Other cysts 4

BENIGN EPIDERMAL TUMOURS 4 Squamous cell papilloma 4 Basal cell papilloma 7 Actinic keratosis 7

BENIGN PIGMENTED LESIONS 8 Freckle (ephelis) 8 Congenital melanocytic naevus 8 Acquired melanocytic naevus 9

BENIGN ADNEXAL TUMOURS 9 Syringoma 9 Pilomatricoma 10

MISCELLANEOUS BENIGN TUMOURS 10 Capillary haemangioma 10 Port-wine stain 13 Pyogenic granuloma 14 Xanthelasma 14 Neurofibroma 15

MALIGNANT TUMOURS 15 Rare predisposing conditions 15 Basal cell carcinoma 16 Squamous cell carcinoma 18 Keratoacanthoma 19 Sebaceous gland carcinoma 20 Lentigo maligna and melanoma 21 Merkel cell carcinoma 22 Kaposi sarcoma 22 Treatment of malignant tumours 23

DISORDERS OF LASHES 27 Anatomy 27 Trichiasis 27 Congenital distichiasis 27 Acquired distichiasis 28 Eyelash ptosis 28 Trichomegaly 28 Madarosis 29 Poliosis 29

ALLERGIC DISORDERS 29 Acute allergic oedema 29 Contact dermatitis 29 Atopic dermatitis 31

BACTERIAL INFECTIONS 31 External hordeolum 31 Impetigo 31 Erysipelas 31 Necrotizing fasciitis 32

VIRAL INFECTIONS 32 Molluscum contagiosum 32 Herpes zoster ophthalmicus 33 Herpes simplex 33

BLEPHARITIS 34 Chronic anterior blepharitis 34 Chronic posterior blepharitis 36 Associations of chronic blepharitis 37 Phthiriasis palpebrarum 37 Angular blepharitis 38 Childhood blepharokeratoconjunctivitis 38

PTOSIS 39 Classification 39 Clinical evaluation 39 Simple congenital ptosis 41 Marcus Gunn jaw-winking syndrome 41 3rd nerve misdirection syndromes 41 Involutional ptosis 41 Mechanical ptosis 42 Surgery 42

ECTROPION 46 Involutional ectropion 46 Cicatricial ectropion 46 Paralytic ectropion 46 Mechanical ectropion 49

ENTROPION 49 Involutional entropion 49 Cicatricial entropion 50

MISCELLANEOUS ACQUIRED DISORDERS 50 Varices 50 Blepharochalasis 50 Floppy eyelid syndrome 50 Eyelid imbrication syndrome 55 Eyelid retraction 55

COSMETIC EYELID AND PERIOCULAR SURGERY 55 Involutional changes 55 Non-surgical techniques 56 Surgical techniques 57

CONGENITAL MALFORMATIONS 58 Epicanthic folds 58 Telecanthus 58 Blepharophimosis ptosis and epicanthus inversus syndrome 58 Epiblepharon 58 Congenital entropion 59 Coloboma 59 Euryblepharon 61 Microblepharon 61 Ablepharon 62 Cryptophthalmos 62 Congenital upper lid eversion 63 Ankyloblepharon filiforme adnatum 63

Introduction

Anatomy

The skin consists of the epidermis, dermis and skin appendages (Fig. 1.1A), comprising a wide range of cell types capable of proliferation and neoplastic transformation. The range of cutaneous tumours is thus very extensive, ranging from common papillomas and basal cell carcinomas to much rarer skin appendage and soft tissue tumours in the dermis. Both benign and malignant tumours are classified according to their cell of origin as well as to their location in the epidermis, dermis or one of the skin appendages. This chapter considers only those of interest to ophthalmologists.

Fig. 1.1 Eyelid skin (A) Normal skin is composed of keratinized stratified epithelium that covers the surface; pilosebaceous elements are conspicuous in the dermis and a few blood vessels and sweat glands are also seen; (B) dysplasia with loss of cell polarity; (C) dyskeratosis – a non-surface epithelial cell producing keratin; (D) parakeratosis – retention of cell nuclei into the surface keratin layer

Epidermis

The epidermis consists of four layers of keratin-producing cells (keratinocytes). It also contains melanocytes, Langerhans cells and Merkel cells. From superficial to deep, the layers of the epidermis are: 1   Keratin (horny) layer is very thin and consists of flat cells devoid of nuclei. 2   Granular cell layer consists of one or two layers of diamond-shaped or flattened cells containing keratohyaline granules. 3   Stratum spinosum layer is approximately five cells in thickness. The cells are polygonal and have abundant eosinophilic cytoplasm. Their free borders are united by intercellular bridges (desmosomes), hence the alternative term ‘prickle’ cell layer. 4   Basal cell layer comprises a single row of columnar-shaped cells that give rise to more superficial cells. Basal cells contain melanin derived from adjacent melanocytes.

Dermis

The dermis is much thicker than the epidermis. It is composed of connective tissue and contains blood vessels, lymphatics and nerve fibres in addition to fibroblasts, macrophages and mast cells. Upward dermal projections (papillae) interdigitate with downward epidermal projections (rete ridges). In the eyelid the dermis lies on the orbicularis muscle. Skin appendages (adnexae) lie deep in the dermis or within the tarsal plates. 1   Sebaceous glands are located in the caruncle and within eyebrow hairs. Tiny sebaceous glands are associated with the thin (vellus) hairs covering periocular skin. 2   Meibomian glands are modified sebaceous glands located in the tarsal plates. They empty through a single row of about 30 openings on each lid. A gland consists of a central duct with multiple acini, the cells of which synthesize lipids (meibum) that pass into the duct and form the outer layer of the precorneal tear film. 3   Glands of Zeis are modified sebaceous glands that are associated with lash follicles. 4   Glands of Moll are modified apocrine sweat glands which open either into a lash follicle or directly onto the anterior lid margin between the lashes. They are more numerous in the lower lid. 5   Eccrine sweat glands are distributed throughout the eyelid skin and are not confined to the lid margin, unlike glands of Moll. 6   Pilosebaceous units comprise hair follicles together with their sebaceous glands.

Terminology

Clinical 1	Macule: a localized area of colour change without infiltration or elevation. A macule may be pigmented (freckle), hypopigmented (vitiligo) or erythematous (capillary haemangioma). 2    Papule: a small solid elevation of skin which may be flat-topped or dome-shaped. 3    Vesicle: a small circumscribed lesion containing fluid. 4    Pustule: a collection of pus. 5    Crust: a dried skin exudate. 6    Nodule: a solid area of raised skin. 7    Cyst: a nodule consisting of an epithelial-lined cavity filled with fluid or semi-solid material. 8    Plaque: a palpable, shallow elevation of the skin, usually more than 2 cm in diameter. 9    Scale: thickening of the horny layer keratin in the form of readily detached fragments. 10  Papilloma: a tag-like projection from the skin surface. 11  Ulcer: a circumscribed area of skin loss that extends through the epidermis into the dermis.

Histological

1   Hyperkeratosis is an increase in thickness of the keratin layer and appears clinically as scaly skin. Hyperkeratosis may be a feature of both benign and malignant epithelial tumours. 2   Acanthosis is thickening of the squamous cell layer. 3   Dysplasia is an alteration of the size, morphology and organization of cellular components of a tissue. There is disturbance of normally structured and recognized layers of tissue (e.g. loss of cell polarity; Fig. 1.1B).

4   Dyskeratosis is keratinization other than on the surface (Fig. 1.1C). 5   Parakeratosis is the retention of nuclei into the keratin layer (Fig. 1.1D). 6   Carcinoma in situ (intraepidermal carcinoma, Bowen disease) exhibits dysplastic changes throughout the thickness of the epidermis and marked hyperkeratosis; see Fig.1.22A).

General considerations

Benign skin lesions are much more common and varied than malignancies. 1   Classification is based on the structure of origin: epidermal, adnexal or dermal. 2   Diagnosis. The clinical characteristics of benign lesions are lack of induration and ulceration, uniform colour, limited growth, regular outline and preservation of normal lid margin structures. In the vast majority of cases diagnosis is straightforward although occasionally biopsy may be required if the appearance is unusual. •   An incisional (shave) biopsy using a knife removes a portion of the lesion for histology and is usually employed for large superficial lesions such as seborrhoeic keratosis. In some cases the bulk of the lesion is also removed and no further treatment is required, provided histology confirms a benign lesion. •   An excision biopsy is performed on small tumours and fulfils both diagnostic and treatment objectives.

3   Treatment options include: •   Excision of the entire lesion and a small surrounding portion of normal tissue. •   Marsupialization involves the removal of the top of a cyst allowing drainage of its contents and subsequent epithelialization. •   Other options include ablation with laser or cryotherapy.

Benign nodules and cysts

Chalazion

Pathogenesis

A chalazion (meibomian cyst) is a chronic, sterile, granulomatous inflammatory lesion caused by retained sebaceous secretion leaking from the meibomian or other sebaceous glands into adjacent stroma. A chalazion secondarily infected is referred to as an internal hordeolum.

Diagnosis

1   Histology shows a lipogranulomatous inflammatory reaction containing epithelioid histiocytes, multinucleated giant cells and plasma cells (Fig. 1.2A). 2   Presentation is at any age with a gradually enlarging painless nodule. Very occasionally a large upper lid chalazion may press on the cornea, induce astigmatism and cause blurred vision. 3   Signs •   A nodule within the tarsal plate (Fig. 1.2B) that may be tender if inflamed. •   Eversion of the lid may show an associated polypoidal granuloma if the lesion has ruptured through the tarsal conjunctiva (Fig. 1.2C). •   A ‘marginal’ chalazion is similar except that it involves a gland of Zeis and is therefore located not in the tarsal plate but on the anterior lid margin. •   Patients with meibomian gland disease or rosacea are at increased risk of chalazion formation which may be multiple (Fig. 1.2D) and/or recurrent. •   It is very important not to mistake a sebaceous gland carcinoma for a ‘recurrent chalazion’. In doubtful cases, the lesion should be biopsied and examined histologically.

Fig. 1.2 Chalazion. (A) Histology shows a lipogranuloma; the large pale cells are epithelioid cells and the well-demarcated empty space contained fat which has been dissolved out during processing; (B) chalazion involving the lower lid; (C) conjuctival granuloma; (D) multiple chalazia in a patient with acne rosacea; (E) chalazion clamp

Treatment

Treatment may not be required because at least a third of chalazia resolve spontaneously and an internal hordeolum may discharge and disappear. Persistent lesions may be treated as follows: 1   Surgery. The eyelid is everted with a special clamp (Fig. 1.2E), the cyst is incised vertically and its contents curetted through the tarsal plate. 2   Steroid injection into the lesion is preferable if close to the lacrimal punctum because of the risk of surgical damage. •   Between 0.2 and 2 mL of 5 mg/mL triamcinolone diacetate aqueous suspension diluted with lidocaine (or equivalent) to a concentration of 5 mg/mL is injected through the conjunctiva into the tissue around the lesion with a 30-gauge needle. •   The success rate following one injection is about 80%. In unresponsive cases a second injection can be given 2 weeks later. Subsequent local skin depigmentation is uncommon.

3   Systemic tetracycline may be required as prophylaxis in patients with recurrent chalazia, particularly if associated with acne rosacea.

Other cysts

1   Epidermal inclusion cyst is usually caused by implantation of epidermis into the dermis following trauma or surgery. It is a slow-growing, round, firm, superficial or subcutaneous lesion containing keratin (Fig. 1.3A). 2   Epidermoid cyst is uncommon and usually developmental, occurring along embryonic lines of closure. It is similar in appearance to an epidermal inclusion cyst. 3   Dermoid cyst is usually subcutaneous or deeper and is typically attached to the periosteum at the lateral end of the brow (Fig. 1.3B). It is caused by skin sequestered during embryonic development. 4   Sebaceous (pilar) cyst is caused by a blocked pilosebaceous follicle and contains sebaceous secretions. It is only rarely found on the eyelid although it may occasionally occur at the inner canthus (Fig. 1.3C). 5   Cyst of Zeis is a small, non-translucent cyst on the anterior lid margin arising from obstructed sebaceous glands associated with the eyelash follicle (Fig. 1.3D). 6   Cyst of Moll (apocrine hidrocystoma) is a small retention cyst of the lid margin apocrine glands. It appears as a round, non-tender, translucent fluid-filled lesion on the anterior lid margin that may have a bluish tinge (Fig. 1.4A). 7   Eccrine hidrocystoma is less common but similar in appearance to a cyst of Moll except that it is usually located along the medial or lateral aspects of the lid, and is close to but does not involve the lid margin itself (Fig. 1.4B). 8   Milia are caused by occlusion of pilosebaceous units resulting in retention of keratin and represent tiny epidermal cysts. They are tiny, white, round, superficial papules which tend to occur in crops (Fig. 1.4C). 9   Comedones are plugs of keratin and sebum within the dilated orifice of hair follicles that often occur in patients with acne vulgaris. They may be either open (blackheads) containing a darkened plug of oxidized material (Fig. 1.4D), or closed (whiteheads).

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Fig. 1.3 (A) Epidermal inclusion cyst. (B) dermoid cyst; (C) sebaceous cysts; (D) cyst of Zeis (Courtesy of A Pearson – fig. A)

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Fig. 1.4 (A) Cyst of Moll; (B) eccrine hidrocystomas; (C) milia; (D) comedones (Courtesy of A Pearson – figs B and D)

Benign epidermal tumours

Squamous cell papilloma

A squamous cell papilloma (fibroepithelial polyp) is a very common condition that has a variable clinical appearance but common histological features. 1   Histology shows finger-like projections of fibrovascular connective tissue covered by irregular acanthotic and hyperkeratotic squamous epithelium (Fig. 1.5A). 2   Signs •   A flesh-coloured, narrow-based, pedunculated lesion (skin tag; Fig. 1.5B). •   A broad-based (sessile) lesion which may exhibit a raspberry-like surface (Fig. 1.5C). •   A hyperkeratotic filiform lesion similar to a cutaneous horn (Fig. 1.5D).

3   Treatment involves simple excision. 4   Differential diagnosis includes viral wart, seborrhoeic keratosis and intradermal naevus.

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Fig. 1.5 Squamous cell papilloma. (A) Histology shows finger-like projections of fibrovascular connective tissue covered by irregular acanthotic and hyperkeratotic squamous epithelium; (B) skin tag; (C) sessile lesion with a raspberry-like surface; (D) hyperkeratotic filiform lesion (Courtesy of J Harry – fig. A; A Pearson – fig. D)

Basal cell papilloma

Basal cell papilloma (seborrhoeic keratosis, seborrhoeic wart, senile verruca) is a common, slow-growing condition found on the face, trunk and extremities of elderly individuals. 1   Histology shows expansion of the squamous epithelium of the epidermis by a proliferation of basal cells. The acanthotic epidermis may show keratin-filled cystic inclusions: either horn cysts within the mass or invaginations of surface keratin forming ‘pseudohorn’ cysts (Fig. 1.6A). 2   Signs. A discrete, greasy, brown plaque with a friable verrucous surface and a ‘stuck-on’ appearance (Fig. 1.6B). 3   Treatment involves shave excision of flat lesions and excision of pedunculated lesions. 4   Differential diagnosis includes pigmented basal cell carcinoma, naevus and melanoma.

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Fig. 1.6 Basal cell papilloma. (A) Histology shows an elevated expansion of the epidermis with proliferation from basal cells; horn cysts and pseudohorn cysts are evident; (B) typical ‘stuck-on’ appearance (Courtesy of J Harry – fig. A; A Pearson – fig. B)

Actinic keratosis

Actinic (solar, senile) keratosis is a common slow-growing lesion that rarely develops on the eyelids. It typically affects elderly, fair-skinned individuals who have been exposed to excessive sunlight and most frequently occurs on the forehead and backs of the hands. It has a low potential for transformation into squamous cell carcinoma. 1   Histology shows irregular dysplastic epidermis with hyperkeratosis, parakeratosis and cutaneous horn formation (Fig. 1.7A). 2   Signs •   Hyperkeratotic plaque with distinct borders and a scaly surface that may become fissured (Fig. 1.7B). •   Occasionally the lesion is nodular or wart-like and may give rise to a cutaneous horn.

3   Treatment involves biopsy followed by either excision or cryotherapy, especially for multiple lesions.

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Fig. 1.7 Actinic keratosis. (A) Histology shows irregular dysplastic epidermis with hyperkeratosis, parakeratosis and cutaneous horn formation; (B) clinical appearance (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth- Heinemann 2001 – fig. A; M Jager – fig. B)

Benign pigmented lesions

Freckle (ephelis)

A brown macule due to increased melanin in the epidermal basal layer, generally in sunlight- exposed areas (Fig. 1.8).

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Fig. 1.8 Freckle (ephilis)

Congenital melanocytic naevus

Congenital naevi are uncommon and histologically resemble their acquired counterparts. Large lesions have potential for malignant transformation of up to 15%. 1   Signs •   Usually small with uniform colour. •   A kissing or split naevus is a rare type of congenital naevus that involves the upper and lower eyelid (Fig. 1.9A) and may occasionally contain numerous hairs (Fig. 1.9B). •   The lesion may rarely cover a large area of the body (‘giant hairy naevus’ – Fig. 1.9C).

2   Treatment, if necessary, involves complete surgical excision.

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Fig. 1.9 Congenital melanocytic naevus. (A) Split naevus; (B) split naevus containing hair; (C) extensive cutaneous involvement (Courtesy of A Pearson – fig. B; U Raina – fig. C)

Acquired melanocytic naevus

1   Classification, clinical appearance and potential for malignant transformation of naevi are determined by their histological location within the skin as follows: a   Junctional naevus occurs in young individuals as a uniformly brown macule or plaque (Fig. 1.10A). The naevus cells are located at the junction of the epidermis and dermis and have a low potential for malignant transformation (Fig. 1.10B). b Compound naevus occurs in middle-age as a raised papular lesion. The shade of pigment varies from light tan to dark brown but tends to be relatively uniform throughout (Fig. 1.10C). The naevus cells extend from the epidermis into the dermis (Fig. 1.10D). It has a low malignant potential which is related to the

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junctional component. c   Intradermal naevus, the most common, typically occurs in old age. It is a papillomatous lesion, with little if any pigmentation, that may show dilated vessels and protruding lashes (Fig. 1.10E). Histologically the naevus cells are confined to the dermis and have no malignant potential (Fig. 1.10F). d  Histological variants of naevi include balloon cell naevi, halo naevi, Spitz naevi (juvenile melanomas) and dysplastic naevi (atypical moles). Multiple dysplastic naevi constitute the dysplastic naevus syndrome (atypical mole syndrome – AMS). Individuals with AMS are at increased risk of developing conjunctival and uveal naevi and cutaneous, conjunctival and uveal melanomas.

2   Treatment is indicated for cosmetic reasons or concern about malignancy. Excision must be complete because following incomplete removal it may be difficult to differentiate recurrence from melanoma both clinically and histologically.

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Fig. 1.10 Acquired melanocytic naevus. (A) Junctional naevus; (B) histology shows heavily pigmented naevus cells at the epidermal/dermal junction. (C) Compound naevus; (D) histology shows naevus cells both at the epidermal/dermal junction and within the dermis. (E) Intradermal naevus; (F) histology shows naevus cells within the dermis separated by a clear zone from the epidermis (Courtesy of J Harry – figs B, D and F)

Benign adnexal tumours

Syringoma

Syringomas are benign proliferations arising from eccrine sweat glands. They are characterized by small papules that are often multiple and bilateral (Fig. 1.11).

Fig. 1.11 Syringomas (Courtesy of A Pearson)

Pilomatricoma

Pilomatricoma (pilomatrixoma, calcifying epithelioma of Malherbe) is derived from the germinal matrix cells of the hair bulb. It affects children and young adults and is more common in females. It is the commonest hair follicle proliferation seen by ophthalmologists. Malignant change is rare. 1   Histology shows irregular epithelial islands exhibiting viable basophilic cells at the periphery and degenerate shadow cells more centrally (Fig. 1.12A). Calcification is frequently present and there is often a foreign body giant cell reaction. 2   Signs. Deep, dermal, purplish, mobile nodule that may be hard due to calcification (Fig. 1.12B). 3   Treatment involves excision.

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Fig. 1.12 Pilomatricoma. (A) Histology shows viable basophilic cells to the right and degenerate shadow cells to the left; (B) clinical appearance (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth- Heinemann 2001 – fig. A; J Krachmer, M Mannis and E Holland, from Cornea, Elsevier 2005 – fig. B)

Other less common hair follicle proliferations include trichofolliculoma, trichoepithelioma and trichilemmoma.

Miscellaneous benign tumours

Capillary haemangioma

Capillary haemangioma (strawberry naevus), although rare, is one of the most common tumours of infancy and presents shortly after birth. The female to male ratio is 3:1. Eyelid

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haemangiomas have a predilection for the upper lid and may have orbital extensions. Occasionally the lesion may also involve the skin of the face and some patients have strawberry naevi on other parts of the body. It is important to be aware of the association between multiple cutaneous lesions and visceral haemangiomas. 1   Histology shows proliferation of varying-sized vascular channels in the dermis and subcutaneous tissue (Fig. 1.13A). 2   Signs. Unilateral, raised bright red lesion (Fig. 1.13B) which blanches on pressure and may swell on crying. A large lesion on the upper lid may cause mechanical ptosis (Fig. 1.13C). 3   Treatment of periocular and orbital haemangiomas is described in Chapter 3. Figure 1.14 shows the results of treatment with steroid injections.

Fig. 1.13 Capillary haemangioma. (A) Histology shows vascular channels of varying size within the dermis and subcutaneous tissue; (B) small haemangioma; (C) mechanical ptosis due to a large lesion (Courtesy of J Harry – fig. A)

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Fig. 1.14 (A) Large capillary haemangioma; (B) appearance several weeks following steroid injection (Courtesy of U Raina)

Port-wine stain

A port-wine stain (naevus flammeus, cavernous haemangioma) is a rare congenital, subcutaneous lesion consisting of large ectatic vessels of varying calibre. It most frequently occurs on the face and is usually unilateral and segmental but occasionally may be bilateral. Some patients have associated Sturge–Weber syndrome (see below).

Cutaneous features

1   Histology shows dilated blood-filled spaces separated by thin fibrous septa (Fig. 1.15A). 2   Signs •   A sharply demarcated soft pink patch which does not blanch with pressure (Fig. 1.15B). •   With age, the lesion does not grow but darkens to red or purple (Fig. 1.16A and B). • The overlying skin may become hypertrophied, coarse, nodular and friable and

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may bleed or become infected (Fig. 1.16C).

3   Treatment with laser, if undertaken during early life, is effective in decreasing the amount of skin discoloration in relatively flat or mildly hypertrophic lesions. Photodynamic therapy may be useful for resistant lesions.

Fig. 1.15 Port-wine stain. (A) Histology shows widely dilated blood-filled spaces separated by fibrous septa; (B) clinical appearance (Courtesy of L Horton – fig. A)

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Fig. 1.16 (A–C) Progression of port-wine stain with time

Sturge–Weber syndrome

Sturge–Weber syndrome (encephalotrigeminal angiomatosis) is a congenital, sporadic phacomatosis. 1   Classification •   Trisystem disease involves the face, leptomeninges and eyes. •   Bisystem disease involves the face and eyes or the face and leptomeninges.

2   Signs •   Port-wine stain, extending over the area corresponding to the distribution of one or more (Fig. 1.16) branches of the trigeminal nerve. •   Ipsilateral parietal or occipital leptomeningeal haemangioma may cause

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contralateral focal or generalized seizures, hemiparesis or hemianopia.

3   Ocular features are ipsilateral glaucoma, episcleral haemangioma, iris heterochromia, and diffuse choroidal haemangioma (see Ch. 12).

Pyogenic granuloma

A pyogenic granuloma is a fast-growing vascularized proliferation of granulation tissue which is usually antedated by surgery, trauma or infection, although some cases are idiopathic. 1   Histology shows granulation tissue with wide, thin-walled vascular channels and inflammatory cells infiltrating a loose stroma (Fig. 1.17A). 2   Signs. A painful, rapidly growing, vascular polypoidal lesion (Fig. 1.17B) that may bleed following relatively trivial trauma. 3   Treatment involves excision.

Fig. 1.17 Pyogenic granuloma. (A) Histology shows inflamed vascularized connective tissue; (B) clinical appearance (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-

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Heinemann 2001 – fig. A)

Xanthelasma

Xanthelasma is a common, frequently bilateral condition which is usually found in middle-aged and elderly individuals. Xanthelasma (and corneal arcus) (see Fig. 1.18B) can be associated with increased levels of serum cholesterol, especially in young males. 1   Histology shows lipid-laden histiocytes in the dermis (Fig. 1.18A). 2   Signs. Multiple yellowish subcutaneous plaques usually located at the medial aspects of the eyelids (Fig. 1.18B). 3   Treatment for cosmetic reasons is either by excision or preferably destruction with a carbon dioxide or argon laser. Patients with the highest recurrence rate are those with persistently elevated cholesterol levels.

Fig. 1.18 Xanthelasma. (A) Histology shows foamy histiocytes within the dermis; (B) clinical appearance – note corneal arcus (Courtesy of J Harry – fig. A; M Zatouroff, from Physical Signs in General Medicine, Mosby- Wolfe, 1996 – fig. B)

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Neurofibroma

Plexiform neurofibromas typically affect children who also have neurofibromatosis type I (NF1). Solitary neurofibromas typically occur in adults, 25% of whom have NF1. 1   Histology shows proliferation of Schwann cells, fibroblasts and nerve axons (Fig. 1.19A). 2   Signs. The tumour typically affects the upper lid and gives rise to a characteristic S- shaped deformity (Fig. 1.19B). 3   Treatment of solitary lesions involves simple excision but removal of plexiform lesions may be difficult especially if they are diffuse.

Fig. 1.19 Neurofibroma. (A) Histology shows proliferation of Schwann cells, fibroblasts and nerve axons, and wavy collagen fibres; (B) characteristic S-shaped eyelid (Courtesy of J Harry – fig. A)

Malignant tumours

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Rare predisposing conditions

Young patients who suffer from one of the following conditions may develop eyelid malignancies: 1   Xeroderma pigmentosum is an AR disease characterized by skin damage on exposure to natural sunlight, which gives rise to progressive cutaneous pigmentation abnormalities (Fig. 1.20A). Affected patients have a bird-like facies and a great propensity to the development of basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and melanoma, which may be multiple. Conjunctival malignancies have also been reported. 2   Gorlin–Goltz syndrome (naevoid basal cell carcinoma syndrome) is a rare AD disorder characterized by extensive congenital deformities of the eye, face, bone and CNS. Many patients develop multiple small BCC during the 2nd decade of life (Fig. 1.20B) and are also predisposed to medulloblastoma, breast carcinoma and Hodgkin lymphoma. 3   Muir–Torre syndrome is a rare AD condition that predisposes to cutaneous and internal malignancies. Cutaneous tumours include BCC, sebaceous gland carcinoma (SGC) and keratoacanthoma. Colorectal and genitourinary carcinoma is the most common systemic tumour. 4   Bazex syndrome is an XLD condition characterized by eczematous and psoriasiform lesions associated with carcinomas of the upper respiratory and digestive tracts. Eyelid BCC may also occur. 5   Other predispositions include immunosuppression, prior retinoblastoma and albinism.

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Fig. 1.20 Predispositions to eyelid malignancies. (A) Xeroderma pigmentosum; (B) Gorlin–Goltz syndrome (Courtesy of J Krachmer, M Mannis and E Holland, from Cornea, Mosby 2005 – fig. B)

Basal cell carcinoma

General features

BCC is the most common human malignancy and most frequently affects elderly patients. The most important risk factors are fair skin, inability to tan and chronic exposure to sunlight. Ninety per cent of cases occur in the head and neck and about 10% of these involve the eyelid. BCC is by far the most common malignant eyelid tumour, accounting for 90% of all cases. It most frequently arises from the lower eyelid, followed in relative frequency by the medial canthus, upper eyelid and lateral canthus. The tumour is slow-growing and locally invasive but non- metastasizing. Tumours located near the medial canthus are more prone to invade the orbit and sinuses, are more difficult to manage than those arising elsewhere and carry the greatest

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risk of recurrence. Tumours that recur following incomplete treatment tend to be more aggressive.

Histology

The tumour arises from the cells that form the basal layer of the epidermis. The cells proliferate downwards (Fig. 1.21A) and characteristically exhibit palisading at the periphery of a tumour lobule of cells (Fig. 1.21B). Squamous differentiation with the production of keratin results in a hyperkeratotic type of BCC. There can also be sebaceous and adenoid differentiation while the growth of elongated strands and islands of cells embedded in a dense fibrous stroma results in a sclerosing (morphoeic) type of tumour.

Fig. 1.21 Basal cell carcinoma. (A) Histology shows downward proliferation of lobules of basal (purple) cells; (B) histology shows palisading of cells at the periphery of a tumour lobule; (C) nodular tumour; (D) rodent ulcer; (E) large rodent ulcer; (F) sclerosing tumour (Courtesy of J Harry – figs A and B)

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Clinical types

The main clinical features of epidermal cell malignancy are ulceration, lack of tenderness, induration, irregular borders and destruction of lid margin architecture. 1   Nodular BCC is a shiny, firm, pearly nodule with small dilated blood vessels on its surface. Initially, growth is slow and it may take the tumour 1–2 years to reach a diameter of 0.5 cm (Fig. 1.21C). 2   Noduloulcerative BCC (rodent ulcer) has central ulceration, pearly raised rolled edges and dilated and irregular blood vessels (telangiectasis) over its lateral margins (Fig. 1.21D); with time it may erode a large portion of the eyelid (Fig. 1.21E). 3   Sclerosing BCC (morphoeic) is less common and may be difficult to diagnose because it infiltrates laterally beneath the epidermis as an indurated plaque (Fig. 1.21F). The margins of the tumour may be impossible to delineate clinically and the lesion tends to be much more extensive on palpation than inspection. On cursory examination a sclerosing BCC may simulate a localized area of chronic blepharitis. 4   Other types not usually found on the lid are cystic, adenoid, pigmented and multiple superficial.

Squamous cell carcinoma

General features

SCC is a much less common, but typically more aggressive tumour than BCC with metastasis to regional lymph nodes in about 20% of cases. Careful surveillance of regional lymph nodes is therefore an important aspect of initial management. The tumour may also exhibit perineural spread to the intracranial cavity via the orbit. SCC accounts for 5–10% of eyelid malignancies and may arise de novo or from pre-existing actinic keratosis or carcinoma in situ (Bowen disease, intraepidermal carcinoma; Fig. 1.22). Immunocompromised patients such as those with AIDS or following renal transplants are at increased risk. The tumour has a predilection for the lower eyelid and the lid margin. It occurs most commonly in elderly individuals with a fair complexion and a history of chronic sun exposure. The diagnosis of SCC may be difficult because certain ostensibly benign lesions such as keratoacanthoma and cutaneous horn may reveal histological evidence of invasive SCC at deeper levels of sectioning.

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Fig. 1.22 Carcinoma in situ. (A) Histology shows dysplastic changes throughout the thickness of the epidermis, together with hyperkeratosis; (B) red scaling plaque (Courtesy of L Horton – fig. A; H Frank – fig. B)

Histology

The tumour arises from the squamous cell layer of the epidermis. It is composed of variable- sized groups of atypical epithelial cells with prominent nuclei and abundant eosinophilic cytoplasm within the dermis (Fig. 1.23A). Well-differentiated tumours may show characteristic keratin ‘pearls’ and intercellular bridges (desmosomes).

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Fig. 1.23 Squamous cell carcinoma. (A) Histology shows acanthotic squamous epithelium and eosinophilic (pink) islands of dysplastic squamous epithelium within the dermis; (B) nodular tumour with surface keratosis; (C) ulcerating tumour; (D) cutaneous horn (Courtesy of L Horton – fig. A; A Singh, from Clinical Ophthalmic Oncology, Saunders 2007 – fig. B; H Frank – fig. C; S Farley, T Cole and L Rimmer – fig. D)

Clinical types

The clinical types are variable and there are no pathognomonic characteristics. The tumour may be indistinguishable clinically from a BCC but surface vascularization is usually absent, growth is more rapid and hyperkeratosis is more often present. 1   Nodular SCC is characterized by a hyperkeratotic nodule which may develop crusting erosions and fissures (Fig. 1.23B). 2   Ulcerating SCC has a red base and sharply defined, indurated and everted borders but pearly margins and telangiectasia are not usually present (Fig. 1.23C). 3   Cutaneous horn with underlying invasive SCC (Fig. 1.23D).

Keratoacanthoma

Keratoacanthoma is a rare tumour that usually occurs in fair-skinned individuals with a history of chronic sun exposure. It is found more frequently than would be expected by chance in

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patients on immunosuppressive therapy. Histopathologically, keratoacanthoma is regarded as part of the spectrum of SCC. 1   Histology shows irregular thickened epidermis surrounded by acanthotic squamous epithelium. The sharp transition from the thickened to normal adjacent epidermis is referred to as shoulder formation (Fig. 1.24A); a keratin-filled crater may be seen. 2   Signs in chronological order: •   A pink, rapidly growing, hyperkeratotic lesion, often on the lower lid (Fig. 1.24B), which may double or treble in size within weeks (Fig. 1.24C). •   Growth ceases for 2–3 months, after which spontaneous involution occurs. •   During the period of regression a keratin-filled crater may develop (Fig. 1.24D). •   Complete involution may take up to a year and usually leaves an unsightly scar.

3   Treatment involves complete surgical excision. Other options include radiotherapy, cryotherapy and topical or intralesional 5-fluorouracil.

Fig. 1.24 Keratoacanthoma. (A) Histology shows irregularly thickened eosinophilic epidermis with a keratin-containing cup and well-marked shoulder formation; (B) hyperkeratotic nodule; (C) large tumour; (D) keratin-filled crater during involution

Sebaceous gland carcinoma

General features

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Sebaceous gland carcinoma (SGC) is a very rare slowly-growing tumour which most frequently affects the elderly, with a predisposition for females. It usually arises from the meibomian glands, although on occasion it may arise from the glands of Zeis or from sebaceous glands in the caruncle. In contrast to BCC and SCC, the tumour occurs more commonly on the upper eyelid where meibomian glands are more numerous. In about 5% of cases there is simultaneous involvement of both lids on one side, probably due to intraepithelial spread or to spontaneous development of multiple primaries. The clinical diagnosis of SGC is frequently difficult because, in its early stages, external signs of malignancy may be subtle so that the tumour may resemble a chalazion or blepharitis. However, the presence of yellowish material within the tumour is highly suggestive of SGC. Because of frequent difficulties in diagnosis and delay in treatment, the overall mortality rate is about 5–10%. Adverse prognostic features include upper lid involvement, tumour size of 10 mm or more and duration of symptoms of over 6 months. SGC that arises from the glands of Zeis is thought to have a more favourable prognosis.

Histology

The tumour consists of lobules of cells with pale foamy vacuolated lipid-containing cytoplasm and large hyperchromatic nuclei (Fig. 1.25A).

Fig. 1.25 Sebaceous gland carcinoma. (A) Histology shows cells with large hyperchromatic nuclei and vacuolated cytoplasm; (B) nodular tumour; (C) spreading tumour; (D) pagetoid spread

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(Courtesy of A Garner – fig. A; A Singh, from Clinical Ophthalmic Oncology, Saunders 2007 – fig. B; S Tuft – fig. C; H Frank fig. D)

Clinical types

Although SGC does not have a characteristic clinical appearance it may present with the following: 1   Nodular SGC presents as a discrete, hard nodule, most commonly within the upper tarsal plate, that may exhibit yellow discoloration due to the presence of lipid (Fig. 1.25B). Because the lesion may masquerade as a chalazion, it is recommended that any ‘chalazion’ of an unusual consistency should undergo full-thickness resection and histological examination. 2   Spreading SGC infiltrates into the dermis and causes a diffuse thickening of the lid margin (Fig. 1.25C) that may result in loss of lashes and be mistaken for ‘chronic blepharitis’. Occasionally the tumour may exhibit multifocal non-contiguous origins. 3   Pagetoid spread refers to extension of the tumour within epithelium including the palpebral (Fig. 1.25D), forniceal or bulbar conjunctiva. This may lead to the mistaken diagnosis of an inflammatory condition.

Lentigo maligna and melanoma

Melanoma rarely develops on the eyelids but is potentially lethal. Although pigmentation is a hallmark of skin melanomas, half of lid melanomas are non-pigmented and this may give rise to diagnostic difficulties. Features suggestive of melanoma include recent onset of a pigmented lesion, change in an existing pigmented lesion, irregular margins, asymmetrical shape, colour change or presence of multiple colours, and diameter greater than 6 mm in diameter.

Lentigo maligna

Lentigo maligna (melanoma in situ, intraepidermal melanoma and Hutchinson freckle) is an uncommon condition that develops in sun-damaged skin in elderly individuals. Malignant change may occur, with infiltration of the dermis. 1   Histology shows intraepidermal proliferation of spindle-shaped atypical melanocytes that replace the basal layer of the epidermis (Fig. 1.26A). 2   Signs •   A slowly expanding pigmented macule with an irregular border (Fig. 1.26B). •   Nodular thickening and areas of irregular pigmentation are highly suggestive of malignant transformation (Fig. 1.26C).

3   Treatment is usually by excision.

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Fig. 1.26 Lentigo maligna. (A) Histology shows melanoma cells proliferating within the basal layers of the epidermis; (B) lentigo maligna; (C) melanoma arising from lentigo maligna (Courtesy of L Horton – fig. A; S Delva – figs B and C)

Melanoma

1   Histology shows large atypical melanocytes within the dermis (Fig. 1.27A). 2   Signs a   Superficial spreading melanoma is characterized by a plaque with an irregular outline and variable pigmentation (Fig. 1.27B). b  Nodular melanoma is typically a blue-black nodule surrounded by normal skin (Fig. 1.27C).

3   Treatment is usually by wide excision and may include local lymph node removal.

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Fig. 1.27 Melanoma. (A) Histology shows melanoma cells within the dermis; (B) superficial spreading melanoma; (C) nodular melanoma (Courtesy of J Harry – fig. A)

Merkel cell carcinoma

Merkel cell carcinoma is a fast-growing tumour which typically affects the elderly. Although Merkel cells lie within the epidermis, the tumour appears to arise from the dermis. Its rarity may lead to difficulty in diagnosis and delay in treatment. The tumour is highly malignant and 50% of patients have metastatic spread at presentation. 1   Histology shows sheets of cells with scanty cytoplasm, round or oval nuclei and numerous mitotic figures (Fig. 1.28A). 2   Signs. A violaceous, well-demarcated nodule with intact overlying skin, most frequently involving the upper eyelid (Fig. 1.28B). 3   Treatment is by excision, frequently combined with chemotherapy.

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Fig. 1.28 Merkel cell carcinoma. (A) Histology shows a sheet of Merkel cells; (B) clinical appearance (Courtesy of J Harry and L Misson, from Ocular Ophthalmic Pathology, Butterworth- Heinemann 2001 – fig. A)

Kaposi sarcoma

Kaposi sarcoma is a vascular tumour which typically affects patients with the acquired immune deficiency syndrome (AIDS). Many patients have advanced systemic disease although in some instances the tumour may be the only clinical manifestation of HIV infection. 1   Histology shows proliferating spindle cells, vascular channels and inflammatory cells within the dermis (Fig. 1.29A). 2   Signs. A pink, red-violet to brown lesion (Fig. 1.29B) which may be mistaken for a haematoma or a naevus. 3   Treatment is by radiotherapy or excision.

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Fig. 1.29 Kaposi sarcoma. (A) Histology shows a proliferation of predominantly spindle-shaped cells; vascular channels are evident; (B) clinical appearance (Courtesy of J Harry – fig. A)

Treatment of malignant tumours

Biopsy

The two types of biopsy are (a) incisional, using a blade or a biopsy punch, in which only part of the lesion is removed to allow histological diagnosis, and (b) excisional, in which the entire lesion is removed and a histological diagnosis made; the latter may be: 1   Shave excision using a blade to remove shallow epithelial tumours, such as papillomas and seborrhoeic keratosis. 2   Full-thickness skin excision for tumours that are not confined to the epidermis.

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Surgical excision

Surgical excision aims to remove the entire tumour with preservation of as much normal tissue as possible. Smaller tumours can be removed via an excision biopsy and the defect closed directly, whilst awaiting histological confirmation of complete clearance. Most small BCCs can be cured by excision of the tumour together with a 2–4 mm margin of clinically normal tissue. More radical surgical excision is required for large BCC and aggressive tumours such as SCC, SGC and melanoma. It may not be possible to close the defect at the time of initial removal, but it is necessary to ensure complete clearance of tumour prior to undertaking any reconstruction. Rapid processing of paraffin-embedded specimens can reduce the interval to confirmation of histological clearance but still requires closure as a separate procedure. Faster confirmation can be achieved using either frozen-section control or micrographic surgery, and reconstruction can then take place on the same day. 1   Standard frozen section involves histological examination of the margins of the excised specimen at the time of surgery to ensure that they are tumour-free. If no tumour cells are detected, the eyelid is reconstructed; if some are present in a particular area, further excision is performed until the specimen is tumour-free. 2   Mohs micrographic surgery involves layered excision of the tumour. Specimens around the eye are usually examined frozen as the fixing paste used in the technique as initially described produces ocular irritation. Processing of each layer enables a map of the edges of the tumour to be developed. Further tissue is taken in any area where tumour is still present until clearance is achieved. Although time-consuming, this technique maximizes the chances of total tumour excision whilst minimizing sacrifice of normal tissue. This is a particularly useful technique for tumours that grow diffusely and have indefinite margins with finger-like extensions, such as sclerosing BCC, SCC, recurrent tumours and those involving the medial or lateral canthi. However, the irregular contours around the eyelids and extension of tumours into orbital fat can make interpretation difficult, and specialist training is required in this technique.

Reconstruction

The technique of reconstruction depends on the extent of tissue removed and whether this is full- or partial-thickness. It is important to reconstruct both anterior and posterior lamellae. If one of the lamellae has been sacrificed during excision of the tumour, it must be reconstructed with similar tissue. Anterior lamellar defects may be closed directly or with a local flap or skin graft. Full-thickness defects may be repaired as follows: 1   Small defects involving less than one-third of the eyelid can usually be closed directly, provided the surrounding tissue is sufficiently elastic to allow approximation of the cut edges (Fig. 1.30). If necessary, a lateral cantholysis can be fashioned to mobilize additional tissue if the defect cannot be reapproximated. 2   Moderate size defects involving up to half of the eyelid may require a flap (e.g. Tenzel semicircular) for closure (Fig. 1.31). 3   Large defects involving over half of the eyelid may be closed by one of the following techniques: a   Posterior lamellar reconstruction may involve an upper lid free tarsal graft, buccal mucous membrane or hard palate graft, or a Hughes flap from the upper lid (Fig. 1.32). b  Anterior lamellar reconstruction may involve skin advancement, a local skin flap or a free skin graft (Fig. 1.33). At least one reconstructed lamella requires its own blood supply to maximize the viability of any free graft.

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Fig. 1.30 Direct closure. (A) Pre-operative appearance of a basal cell carcinoma; (B) appearance following excision; (C) direct closure of defect (Courtesy of A Pearson)

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Fig. 1.31 Tenzel flap. (A) Pre-operative appearance; (B) appearance following excision; (C) appearance following closure of the flap (Courtesy of A Pearson)

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Fig. 1.32 Posterior lamellar reconstruction with a Hughes upper lid flap. (A) Preoperative appearance; (B) appearance following excision; (C) postoperative appearance with the flap yet to be divided (Courtesy of A Pearson)

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Fig. 1.33 Anterior lamellar reconstruction with a free skin graft. (A) Pre-operative appearance; (B) appearance following excision; (C) skin graft in place (Courtesy of A Pearson)

Laissez-faire

Full reconstruction of the defect created by tumour removal may not always be required. In the laissez-faire approach the wound edges are approximated as far as possible and the defect is allowed to granulate and heal by secondary intention. Even large defects can often achieve a satisfactory outcome with time.

Radiotherapy

The recurrence rate following irradiation is higher than after surgery, and radiotherapy does not allow histological confirmation of tumour eradication. Recurrences following radiotherapy are

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difficult to treat surgically because of the poor healing properties of irradiated tissue.

1   Indications •   Small BCC which does not involve the medial canthal area in patients who are either unsuitable for or refuse surgery. •   Highly radiosensitive tumours such as Kaposi sarcoma.

2   Contraindications •   Medial canthal BCC because radiotherapy would damage the canaliculi and result in epiphora. •   Upper eyelid tumours because subsequent keratinization results in a chronically uncomfortable eye. •   Aggressive tumours such as sclerosing BCC, SCC and SGC.

3   Complications •   Skin damage and madarosis. •   Nasolacrimal duct stenosis following irradiation to the medial canthal area. •   Conjunctival keratinization, dry eye, keratopathy and cataract. •   Retinopathy and optic neuropathy.

Many of these complications can be avoided if the globe is protected by a special shield during irradiation.

Cryotherapy

1   Indications. May be considered for small superficial BCC. 2   Contraindications are similar to those of radiotherapy, although cryotherapy may be a useful adjunct to surgery in patients with epibulbar pagetoid extensions of SGC, sparing the patient an extenteration. 3   Complications include skin depigmentation, madarosis and conjunctival overgrowth.

Disorders of lashes

Anatomy

The lashes (cilia) are slightly more numerous in the upper (approximately 100) than in the lower lid. The lash roots lie against the anterior surface of the tarsus. The cilia pass between the orbicularis oculi and the muscle of Riolan, exiting the skin at the anterior lid margin and curve away from the globe. Scarring of the tarsal plate and conjunctiva can alter their position and direction. Following intense inflammation lashes may grow abnormally from meibomian gland openings (distichiasis).

Trichiasis

Trichiasis is a very common acquired condition which may occur in isolation or as a result of scarring of the lid margin secondary to chronic blepharitis and herpes zoster ophthalmicus. Trichiasis should not be mistaken for pseudotrichiasis secondary to entropion when in some cases the in-turning of the eyelid may be intermittent and the condition may be mistaken for

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true trichiasis and inappropriately treated.

Signs

Trichiasis is characterized by posterior misdirection of lashes arising from normal sites of origin (Fig. 1.34A and B). Trauma to the corneal epithelium may cause punctate epithelial erosions, with ocular irritation worsened by blinking. Corneal ulceration and pannus formation may occur in severe long-standing cases.

Fig. 1.34 Trichiasis. (A) Mild; (B) severe; (C) cryotherapy; (D) appearance following laser ablation (Courtesy of A Pearson – figs A and C)

Treatment

1   Epilation with forceps is simple and effective but recurrences within 4–6 weeks are almost inevitable. 2   Electrolysis is useful for a few isolated lashes but is tedious and may cause scarring. Frequently multiple treatments are required to obtain a satisfactory result. An electrocautery needle is inserted down the shaft of the lash root and current applied until coagulated tissue bubbles to the surface. The lash is then removed. Retreatment for recurrences is required in about 40% of cases. 3   Cryotherapy is very effective in eliminating profuse lashes (Fig. 1.34C). With a special cryoprobe a double freeze–thaw cycle at −20°C is applied. Complications include necrosis, depigmentation (especially in dark-skinned individuals), damage to meibomian glands which may adversely affect the precorneal tear film, and notching of the lid margin. 4   Argon laser ablation is useful for a few scattered lashes and is performed as follows:

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a   The initial settings are 50 µm, 0.2 second and 1000 mW. b  The laser is fired at the root of the lash and a small crater formed. c   The spot size is increased to 200 µm and the crater deepened to reach the follicle (Fig. 1.34D). d  Most patients are cured by one or two sessions.

5   Surgery involving full-thickness wedge resection or anterior lamellar rotation excision may be useful for a localized crop of lashes resistant to other methods of treatment.

Congenital distichiasis

Congenital distichiasis is a rare condition that occurs when a primary epithelial germ cell destined to differentiate into a specialized sebaceous gland (meibomian gland) of the tarsus develops into a complete pilosebaceous unit. The condition is frequently inherited in an AD manner with high penetrance but variable expressivity. The majority of patients also manifest primary lymphoedema of the legs (lymphoedema–distichiasis syndrome). 1   Signs •   A partial or complete second row of lashes emerging at or slightly behind the meibomian gland orifices. •   The aberrant lashes tend to be thinner and shorter than normal cilia and are often directed posteriorly. They are usually well tolerated during infancy and may not become symptomatic until the age of 5 years.

2   Treatment of the lower lid is with cryotherapy. Distichiasis of the upper lid involves lamellar eyelid division and cryotherapy, which is performed as follows: a   An incision is made along the grey line dividing the lid into anterior and posterior lamellae (Fig. 1.35A). b  The posterior lamella and lash follicles are frozen to −20°C with a double freeze– thaw cycle (Fig. 1.35B). c   The lamellae are surgically re-apposed.

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Fig. 1.35 (A) Lamellar lid separation; (B) cryotherapy to the posterior lamella (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Acquired distichiasis

Acquired distichiasis (metaplastic lashes) is caused by metaplasia and dedifferentiation of the meibomian glands to become hair follicles. The most important cause is late stage cicatrizing conjunctivitis associated with chemical injury, Stevens–Johnson syndrome and ocular cicatricial pemphigoid. 1   Signs •   Variable number of lashes which originate from meibomian gland orifices. •   Unlike congenital distichiasis, the cilia tend to be non-pigmented and stunted (Fig. 1.36), and are usually symptomatic.

2   Treatment of mild cases is as for trichiasis. Severe cases require lamellar eyelid division and cryotherapy to the posterior lamella.

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Fig. 1.36 Acquired distichiasis (Courtesy of R Bates)

Eyelash ptosis

Eyelash ptosis is a downward sagging of upper eyelid lashes (Fig. 1.37A). The condition may be idiopathic or associated with floppy eyelid syndrome, dermatochalasis with anterior lamellar slip or long-standing facial palsy.

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Fig. 1.37 Miscellaneous eyelash disorders. (A) Eyelash ptosis; (B) trichomegaly; (C) madarosis; (D) poliosis (Courtesy of A Pearson – fig. A; L Merin – fig. B; S Tuft – fig. C)

Trichomegaly

Trichomegaly is excessive eyelash growth (Fig. 1.37B); the main causes are listed in Table 1.1.

Table 1.1 -- Causes of trichomegaly 1   Acquired •   Drug-induced – topical prostaglandin analogues phenytoin and ciclosporin •   Malnutrition •   AIDS •   Porphyria •   Hypothyroidism •   Familial

2   Congenital • Oliver–McFarlane syndrome – pigmentary retinopathy, dwarfism and mental

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handicap •   Cornelia de Lange syndrome – mental and physical developmental abnormalities. •   Goldstein–Hutt syndrome – cataract and hereditary spherocytosis. •   Hermansky–Pudlak syndrome – albinism and bleeding diathesis.

Madarosis

Madarosis is a decrease in the number of lashes (Fig. 1.37C); the main causes are shown in Table 1.2

Table 1.2 -- Cause of madarosis 1  Local •  Chronic anterior lid margin disease •  Infiltrating lid tumours •  Burns •  Radiotherapy or cryotherapy of lid tumours

2  Skin disorders •  Generalized alopecia •  Psoriasis

3  Systemic diseases •  Myxoedema •  Systemic lupus erythematosus •  Acquired syphilis •  Lepromatous leprosy

4  Following removal •  Iatrogenic for trichiasis •  Trichotillomania – psychiatric disorder of hair removal

Poliosis

Poliosis is a premature localized whitening of hair, which may involve the lashes and eyebrows (Fig. 1.37D); the main causes are shown in Table 1.3.

Table 1.3 -- Causes of poliosis 1 Ocular •  Chronic anterior blepharitis •  Sympathetic ophthalmitis •  Idiopathic uveitis

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2  Systemic •  Vogt–Koyanagi–Harada syndrome •  Waardenburg syndrome •  Vitiligo •  Marfan syndrome •  Tuberous sclerosis

Allergic disorders

Acute allergic oedema

Acute allergic oedema is usually caused by pollen or by insect bites. 1   Signs. Sudden onset of bilateral pitting periorbital oedema (Fig. 1.38A), often accompanied by conjunctival swelling (chemosis). 2   Treatment with systemic antihistamines may be helpful.

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Fig. 1.38 Allergic disorders. (A) Acute allergic oedema; (B) contact dermatitis; (C) atopic dermatitis

Contact dermatitis

Contact dermatitis is an inflammatory response that usually follows exposure to a medication or preservative, cosmetics or metals. An irritant can also cause a non-allergic toxic dermatitis. The individual is sensitized on first exposure and develops an immune reaction on further exposure. Reaction is mediated by a delayed type IV hypersensitivity response. 1  History of exposure and re-exposure to a potential allergen. 2  Symptoms include itching and tearing following exposure. 3  Signs •  Lid oedema, scaling, angular fissuring and tightness (Fig. 1.38B). •  Chemosis, redness and papillary conjunctivitis. •  Punctate corneal epithelial erosions.

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4  Treatment •  Stopping exposure to the allergen, if it can be identified. •  Use of non-preserved drops, if sensitivity to preservatives suspected. •  Cold compresses for symptomatic relief. •  Topical steroids may be helpful but are rarely required. •  Oral antihistamine for severe cases. •  Care to avoid re-exposure (record in notes).

Atopic dermatitis

Atopic dermatitis (eczema) is a very common idiopathic condition, typically associated with asthma and hay fever. Eyelid involvement is relatively infrequent but when present is invariably associated with generalized dermatitis. 1   Signs. Thickening, crusting and vertical fissuring of the lids associated with staphylococcal blepharitis and madarosis (Fig. 1.38C). 2   Treatment is with emollients to hydrate the skin and the judicious use of mild topical steroids such as hydrocortisone 1%. It is also important to treat associated infection. 3   Ocular associations: a   Common include vernal disease in children and chronic keratoconjunctivitis in adults. b  Uncommon include keratoconus, presenile cataract and retinal detachment.

Bacterial infections

External hordeolum

An external hordeolum (stye) is an acute staphylococcal abscess of a lash follicle and its associated gland of Zeis. It is more common in children and young adults. 1   Signs •   A tender swelling in the lid margin pointing anteriorly through the skin, usually with a lash at the apex (Fig. 1.39A). •   Multiple lesions may be present and occasionally abscesses may involve the entire lid margin.

2   Treatment involves topical antibiotics, hot compresses and epilation of the associated lash.

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Fig. 1.39 Bacterial infections. (A) External hordeolum (stye); (B) impetigo; (C) erysipelas; (D) necrotizing fasciitis

Impetigo

Impetigo is an uncommon superficial skin infection caused by S. aureus or S. pyogenes which most frequently affects children. Involvement of the eyelids is usually associated with painful infection of the face. 1   Signs. Erythematous macules rapidly developing into thin-walled blisters which produce golden-yellow crusts on rupturing (Fig. 1.39B). 2   Treatment is with topical antibiotics and oral flucloxacillin or erythromycin.

Erysipelas

Erysipelas (St Anthony's fire) is an uncommon, acute subcutaneous spreading cellulitis, usually caused by S. pyogenes through a site of minor skin trauma. 1   Signs •   An expanding, well-defined, indurated, erythematous subcutaneous plaque (Fig. 1.39C). •   Primary lid involvement, when it occurs, is usually severe and may result in secondary contracture.

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2   Treatment is with oral antibiotics.

Necrotizing fasciitis

Necrotizing fasciitis is an extremely rare rapidly progressive necrosis initially involving subcutaneous soft tissues and later the skin. It is usually caused by S. pyogenes and occasionally S. aureus. The most frequent sites of involvement are the extremities, trunk and perineum, as well as postoperative wound sites. Unless treatment is early and appropriate death may result. Periocular infection is rare and may be secondary to trauma or surgery. 1   Signs. Periorbital redness and oedema leading to formation of large bullae and black discoloration of skin due to gangrene secondary to underlying thrombosis (Fig. 1.39D). 2   Complications include ophthalmic artery occlusion, lagophthalmos and disfigurement. 3   Treatment involves intravenous benzylpenicillin, debridement of necrotic tissue and reconstructive surgery.

Viral infections

Molluscum contagiosum

Molluscum contagiosum is a skin infection caused by a human specific double-stranded DNA poxvirus which typically affects otherwise healthy children, with a peak incidence between 2 and 4 years. Transmission is by contact and subsequently by autoinoculation. Multiple, and occasionally confluent, lesions may develop in immunocompromised patients. A distribution in the chin-strap region is common in HIV-positive individuals. 1   Histology shows a central pit and lobules of hyperplastic epidermis with intracytoplasmic (Henderson-Patterson) inclusion bodies that displace the nuclear remnant to the edge of the cell. The bodies are small and eosinophilic near the surface and large and basophilic deeper down (Fig. 1.40A). 2   Signs •   Single or multiple pale, waxy, umbilicated nodules (Fig. 1.40B). •   Lesions on the lid margin (Fig. 1.40C) may shed virus into the tear film and give rise to a secondary ipsilateral chronic follicular conjunctivitis. Unless the lid margin is examined carefully the causative molluscum lesion may be overlooked. •   White cheesy material consisting of infected degenerate cells can be expressed from the lesion.

3   Treatment may not be necessary unless the lesion is very close to the lid margin. Options include shave excision, cauterization, cryotherapy or laser.

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Fig. 1.40 Molluscum contagiosum. (A) Histology shows lobules of hyperplastic epidermis and a pit containing intracytoplasmic inclusion bodies which are small and eosinophilic near the surface and larger and basophilic more deeply; (B) multiple molluscum nodules; (C) lid margin nodule (Courtesy of A Garner – fig. A; N Rogers – fig. B)

Herpes zoster ophthalmicus

Herpes zoster ophthalmicus (HZO) is a common, unilateral infection caused by varicella-zoster virus. It typically affects the elderly but may occur at an earlier age. It tends to be more severe in immunocompromised individuals. 1   Presentation is with pain in the distribution of the first division of the trigeminal nerve. 2   Signs •   A maculopapular rash on the forehead (Fig. 1.41A). •   Progression through vesicles and pustules to crusting. •   Periorbital oedema may spread to the other side (Fig. 1.41B), giving the erroneous

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impression that the condition is bilateral.

3   Ocular complications (see Ch. 6). 4   Treatment •   Oral aciclovir 800 mg five times daily for 7–10 days; alternatives include valaciclovir 1 g t.i.d., famciclovir 500 mg t.i.d. and brivudine 125 mg once daily. •   Topical aciclovir or penciclovir cream, and a steroid-antibiotic combination such as Fucidin-H (hydrocortisone 1%, fusidic acid 2%) can be used t.i.d. until the crusts have separated.

Fig. 1.41 Herpes zoster ophthalmicus (A) Maculopapular rash; (B) vesicles and crusts, and periorbital oedema

Herpes simplex

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1   Pathogenesis. Primary infection or rarely reactivation of herpes simplex virus previously dormant in the trigeminal ganglion. 2   Diagnosis •   Prodromal facial and lid tingling, lasting about 24 hours. •   Eyelid and periorbital vesicles on the lid margin (Fig. 1.42A) that break down over 48 hours. •   Associated papillary conjunctivitis, discharge and lid swelling. •   Dendritic corneal ulcers can develop, especially in atopic patients. •   Gradually settles over 6–8 days. •   Involvement can be very severe in atopic patients (eczema herpeticum – Fig. 1.42B).

3   Treatment •   Topical antiviral (aciclovir cream) 5 times daily for 5 days. •   Oral aciclovir 400–800 mg 5 times daily for 3–5 days; famciclovir and valaciclovir are alternatives. •   Add co-amoxiclav or erythromycin for secondary staphylococcal infection in patients with eczema herpeticum.

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Fig. 1.42 Herpes simplex. (A) Vesicular rash; (B) eczema herpeticum

Blepharitis

Chronic anterior blepharitis

Chronic marginal blepharitis is a very common cause of ocular discomfort and irritation. Involvement is usually bilateral and symmetrical. Blepharitis may be subdivided into anterior and posterior although there is considerable overlap and both are often present. The poor correlation between symptoms and signs, the uncertain aetiology and mechanisms of the disease process all conspire to make management difficult.

Pathogenesis

Anterior blepharitis affects the area surrounding the bases of the eyelashes and may be staphylococcal or seborrhoeic. The former is thought to be the result of an abnormal cell

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mediated response to components of the cell wall of S. aureus which may also be responsible for the red eyes and the peripheral corneal infiltrates seen in some patients. Seborrhoeic blepharitis is often associated with generalized seborrhoeic dermatitis that may involve the scalp, nasolabial folds, behind the ears, and the sternum. Because of the intimate relationship between the lids and ocular surface, chronic blepharitis may cause secondary inflammatory and mechanical changes in the conjunctiva and cornea.

Diagnosis

1   Symptoms do not provide a reliable clue to the type of blepharitis and are caused by disruption of normal ocular surface function and reduction in tear stability. Because of poor correlation between the severity of symptoms and clinical signs it can be difficult to objectively assess the benefit of treatment. •   Burning, grittiness, mild photophobia, and crusting and redness of the lid margins with remissions and exacerbations are characteristic. •   Symptoms are usually worse in the mornings although in patients with associated dry eye they may increase during the day.

2   Signs a   Staphylococcal blepharitis •   Hard scales and crusting mainly located around the bases of the lashes (collarettes; Fig. 1.43A). •   Mild papillary conjunctivitis and chronic conjunctival hyperaemia are common. •   Long-standing cases may develop scarring and notching (tylosis) of the lid margin (Fig. 1.43B), madarosis, trichiasis and poliosis. •   Secondary changes include stye formation, marginal keratitis and occasionally phlyctenulosis. •   Associated tear film instability and dry eye syndrome are common.

b  Seborrhoeic blepharitis •   Hyperaemic and greasy anterior lid margins with sticking together of lashes (Fig. 1.43C). •   The scales are soft and located anywhere on the lid margin and lashes.

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Fig. 1.43 Chronic anterior blepharitis. (A) Collarettes; (B) scarring of the lid margin; (C) greasy lid margin with sticky lashes

Treatment

There is little evidence to support any particular treatment protocol for anterior blepharitis. Patients should be advised that a permanent cure is unlikely, but control of symptoms is usually possible. 1   Lid hygiene •   A warm compress applied for several minutes to soften crusts at the bases of the lashes. •   Lid cleaning to mechanically remove crusts involves scrubbing the lid margins once or twice daily with a cotton bud dipped in a dilute solution of baby shampoo or sodium bicarbonate. • Commercially produced soap/alcohol impregnated pads for lid scrubs are available

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but care should be taken not to induce mechanical irritation. •   The eyelids can also be cleaned with diluted shampoo when washing the hair. •   Gradually, lid hygiene can be performed less frequently as the condition is brought under control but blepharitis often recurs if it is stopped completely.

2   Antibiotic a   Topical sodium fusidic acid, bacitracin or chloramphenicol is used to treat acute folliculitis but is of limited value in long-standing cases. Following lid hygiene the ointment should be rubbed onto the anterior lid margin with a cotton bud or clean finger. b  Oral azithromycin (500 mg daily for three days) may be helpful to control ulcerative lid margin disease.

3   Weak topical steroid such as fluorometholone 0.1% q.i.d. for one week is useful in patients with severe papillary conjunctivitis, marginal keratitis and phlyctenulosis although repeated courses may be required. 4   Tear substitutes are required for associated tear film instability and dry eye.

Chronic posterior blepharitis

Pathogenesis

Posterior blepharitis is caused by meibomian gland dysfunction and alterations in meibomian gland secretions. Bacterial lipases may result in the formation of free fatty acids. This increases the melting point of the meibum preventing its expression from the glands, contributing to ocular surface irritation and possibly enabling growth of S. aureus. Loss of the tear film phospholipids that act as surfactants results in increased tear evaporation and osmolarity, and an unstable tear film.

Diagnosis

There is poor correlation between the severity of symptoms and the clinical signs. 1   Symptoms are similar to anterior blepharitis. 2   Signs •   Excessive and abnormal meibomian gland secretion manifest as capping of meibomian gland orifices with oil globules (Fig. 1.44A). •   Pouting, recession, or plugging of the meibomian gland orifices (Fig. 1.44B). •   Hyperaemia and telangiectasis of the posterior lid margin. •   Pressure on the lid margin results in expression of meibomian fluid that may be turbid or toothpaste-like (Fig. 1.44C); in severe cases the secretions become so inspissated that expression is impossible. •   Lid transillumination may show gland loss and cystic dilatation of meibomian ducts. •   The tear film is oily and foamy, and froth may accumulate on the lid margins or inner canthi. •   Secondary changes include papillary conjunctivitis and inferior corneal punctate epithelial erosions.

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Fig. 1.44 Chronic posterior blepharitis. (A) Capping of meibomian gland orifices by oil globules; (B) plugged meibomian gland orifices; (C) expressed toothpaste-like material (Courtesy of J Silbert, from Anterior Segment Complications of Contact Lens Wear, Butterworth-Heinemann 1999 – fig. C)

Treatment

It is very important to inform the patient that a cure is unlikely. Although remission may be achieved recurrence is usual, particularly when treatment is stopped prematurely. 1   Lid hygiene •   Warm compresses and hygiene are performed as for anterior blepharitis except the emphasis is on massaging the lid to express accumulated meibum. •   Massaging toward the lid margin edge to ‘milk’ meibum and physical expression of the glands by the physician (see Fig. 1.44C).

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2   Systemic tetracyclines are the mainstay of treatment but should not be used in children under the age of 12 years or in pregnant or breast-feeding women because they are deposited in growing bone and teeth, and may cause staining of teeth and dental hypoplasia (erythromycin is an alternative). •   The rationale for the use of tetracyclines is their ability to block staphylococcal lipase production at well below the minimum inhibitory antibacterial concentration. •   Tetracyclines are particularly indicated in patients with recurrent phlyctenulosis and marginal keratitis, although repeated courses of treatment may be needed. a   Oxytetracycline 250 mg b.d. for 6–12 weeks. b  Doxycycline 100 mg b.d. for one week and then daily for 6–12 weeks. c   Minocycline 100 mg daily for 6–12 weeks; skin pigmentation may develop after prolonged use (Fig. 1.26). d  Erythromycin 250 mg daily or b.d. may be used in children.

3   Topical therapy involves antibiotics, steroids and tear substitutes for evaporative dry eye.

Associations of chronic blepharitis

1   Tear film instability is found in 30–50% of patients, probably as a result of imbalance between the aqueous and lipid components of the tear film allowing increased evaporation. Tear film break-up time is typically reduced. 2   Chalazion formation, which may be multiple and recurrent, is common, particularly in patients with posterior blepharitis. 3   Epithelial basement membrane disease and recurrent epithelial erosion may be exacerbated by posterior blepharitis. 4   Cutaneous a   Acne rosacea is often associated with meibomian gland dysfunction. b  Seborrhoeic dermatitis is present in >90% of patients with seborrhoeic blepharitis. c   Acne vulgaris treatment with isotretinoin is associated with the development of blepharitis in about 25% of patients; it subsides when the treatment is stopped.

5   Bacterial keratitis is associated with ocular surface disease secondary to chronic blepharitis. 6   Atopic keratoconjunctivitis is often associated with staphylococcal blepharitis. Treatment of the blepharitis often helps the symptoms of allergic conjunctivitis and vice versa. 7   Contact lens intolerance. Long-term contact lens wear is associated with posterior lid margin disease. Inhibition of lid movement and the normal expression of meibomian oil may be the cause. There may also be associated giant papillary conjunctivitis making lens wear uncomfortable. Blepharitis is also a risk factor for contact lens-associated bacterial keratitis.

Table 1.4 summarizes the features of chronic blephoritis.

Table 1.4 -- Summary of characteristics of chronic blepharitis

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Feature	Anterior blepharitis Posterior blepharitis Staphylococcal	Seborrhoeic Lashes	Deposit	Hard	Soft Loss	++	+ Distorted or trichiasis ++	+	Lid margin	Ulceration	+ Notching	+		++ Cyst	Hordeolum	++ Meibomian			++ Conjunctiva	Phlyctenule	+ Tear film	Foaming			++ Dry eye	+	+	++ Cornea	Punctate erosions	+	+	++ Vascularization	+	+	++ Infiltrates	+	+	++ Associated disease Atopic dermatitis	Seborrhoeic dermatitis Acne rosacea

Phthiriasis palpebrarum

1   Pathogenesis. The crab louse Phthirus pubis is adapted to living in pubic hair (Fig. 1.45A). An infested person may transfer the lice to another hairy area such as the chest, axillae or eyelids. Phthiriasis palpebrarum is an infestation of lashes which typically affects children living in poor hygienic conditions. 2   Symptoms consist of chronic irritation and itching of the lids. 3   Signs •   The lice are anchored to the lashes by their claws (Fig. 1.45B). •   The ova and their empty shells appear as oval, brownish, opalescent pearls adherent to the base of the cilia (Fig. 1.45C). •   Conjunctivitis is uncommon.

4   Treatment a   Mechanical removal of the lice and their attached lashes with fine forceps. b  Topical yellow mercuric oxide 1% or petroleum jelly applied to the lashes and lids twice a day for 10 days. c   Delousing of the patient, family members, clothing and bedding is important to prevent recurrences.

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Fig. 1.45 Phthiriasis palpebrarum. (A) Phthirus pubis; (B) louse anchored to lashes; (C) louse, ova and shells (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth- Heinemann, 2001 – fig. A; D Smit – fig. C)

Angular blepharitis

1   Pathogenesis. The infection is usually caused by Moraxella lacunata or S. aureus although other bacteria, and rarely herpes simplex, have also been implicated. 2   Signs •   Often unilateral red, scaly, macerated and fissured skin at the lateral and medial canthus (Fig. 1.46). •   Associated papillary and follicular conjunctivitis may occur.

3   Treatment involves topical chloramphenicol, bacitracin or erythromycin.

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Fig. 1.46 Angular blepharitis

Childhood blepharokeratoconjunctivitis

Childhood blepharokeratoconjunctivitis is a poorly defined condition which tends to be more severe in Asian and Middle Eastern populations. 1   Presentation is usually at about 6 years of age with recurrent episodes of chronic redness (Fig. 1.47A) and irritation that results in constant eye rubbing and photophobia which may be misdiagnosed as allergic eye disease. 2   Signs •   Chronic anterior or posterior blepharitis which may be associated with recurrent styes or meibomian cysts. •   Conjunctival changes include diffuse hyperaemia, bulbar phlyctens and follicular or papillary hyperplasia. •   Corneal changes include superficial punctate keratopathy, marginal keratitis, peripheral vascularization (Fig. 1.47B) and axial subepithelial haze.

3   Treatment •   Lid hygiene and topical antibiotic ointment at bedtime. •   Topical low dose steroids (prednisolone 0.1% or fluorometholone 0.1%). •   Erythromycin syrup 125 mg daily for 4–6 weeks.

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Fig. 1.47 (A) Right childhood blepharokeratoconjunctivitis; (B) peripheral vascularization (Courtesy of S Tuft)

Ptosis

Classification

Ptosis is an abnormally low position of the upper lid which may be congenital or acquired. 1   Neurogenic ptosis is caused by an innervational defect such as 3rd nerve paresis and Horner syndrome (see Ch. 19). 2   Myogenic ptosis is caused by a myopathy of the levator muscle itself, or by impairment of transmission of impulses at the neuromuscular junction (neuromyopathic). Acquired myogenic ptosis occurs in myasthenia gravis, myotonic dystrophy and progressive external ophthalmoplegia (see Ch. 19). 3   Aponeurotic ptosis is caused by a defect in the levator aponeurosis. 4   Mechanical ptosis is caused by the gravitational effect of a mass or by scarring.

Clinical evaluation

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History

The age at onset of ptosis and its duration will usually distinguish congenital from acquired cases. If the history is ambiguous, old photographs may be helpful. It is also important to enquire about symptoms of possible underlying systemic disease, such as associated diplopia, variability of ptosis during the day and excessive fatigue.

Pseudoptosis

A false impression of ptosis may be caused by the following: 1   Lack of support of the lids by the globe may be due to an orbital volume deficit associated with an artificial eye, microphthalmos, phthisis bulbi (Fig. 1.48A), or enophthalmos. 2   Contralateral lid retraction, which is detected by comparing the levels of the upper lids, remembering that the margin of the upper lid normally covers the superior 2 mm of the cornea (Fig. 1.48B). 3   Ipsilateral hypotropia causes pseudoptosis because the upper lid follows the globe downwards (Fig. 1.48C). It disappears when the hypotropic eye assumes fixation on covering the normal eye. 4   Brow ptosis due to excessive skin on the brow, or 7th nerve palsy, which is diagnosed by manually elevating the eyebrow (Fig. 1.48D). 5   Dermatochalasis in which there is excessive skin on the upper lids (see Fig. 1.77A); this may also cause mechanical ptosis.

Fig. 1.48 Causes of pseudoptosis. (A) Right phthisis bulbi; (B) contralateral lid retraction; (C) ipsilateral hypotropia; (D) bilateral brow ptosis (Courtesy of S Webber – figs C and D)

Measurements

1 Margin–reflex distance is the distance between the upper lid margin and the corneal

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reflection of a pen torch held by the examiner, at which the patient is directly looking (Fig. 1.49); normal is 4–4.5 mm. 2   Palpebral fissure height is the distance between the upper and lower lid margins, measured in the pupillary plane (Fig. 1.50). The upper lid margin normally rests about 2 mm below the upper limbus and the lower 1 mm above the lower limbus. This measurement is shorter in males (7–10 mm) than in females (8–12 mm). Unilateral ptosis can be quantified by comparison with the contralateral side. Ptosis may be graded as mild (up to 2 mm), moderate (3 mm) and severe (4 mm or more). 3   Levator function (upper lid excursion) is measured by placing a thumb firmly against the patient's brow to negate the action of the frontalis muscle, with the eyes in downgaze (Fig. 1.51A). The patient then looks up as far as possible and the amount of excursion is measured with a rule (Fig. 1.51B). Levator function is graded as normal (15 mm or more), good (12–14 mm), fair (5–11 mm) and poor (4 mm or less). 4   Upper lid crease is the vertical distance between the lid margin and the lid crease in downgaze. In females it measures about 10 mm and in males 8 mm. Absence of the crease in a patient with congenital ptosis is indirect evidence of poor levator function, whereas a high crease suggests an aponeurotic defect. The skin crease is also used as a guide to the initial incision in some surgical procedures. 5   Pretarsal show is the distance between the lid margin and the skin fold with the eyes in the primary position.

Fig. 1.49 Margin–reflex distance. (A) Normal; (B) mild ptosis; (C) moderate ptosis; (D) severe ptosis

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Fig. 1.50 Measurement of palpebral fissure height

Fig. 1.51 Measurement of levator function

Associated signs

1   Increased innervation may flow to the levator muscle of a unilateral ptosis, particularly in upgaze. Associated increased innervation to the contralateral normal levator will result in lid retraction (see Fig. 1.76A). The examiner should therefore manually elevate the ptotic lid and look for a droop of the opposite lid. If this occurs, the patient should be warned that surgical correction may induce a drop in the opposite lid. 2   Fatigability is tested by asking the patient to look up without blinking for 30 seconds. Progressive drooping of one or both lids, or an inability to maintain upgaze, is suggestive of myasthenia (see Fig. 19.95A and B). Myasthenic ptosis may show an overshoot of the upper lid on saccade from downgaze to the primary position (Cogan twitch sign) and

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also a ‘hop’ on side-gaze. 3   Ocular motility defects, particularly of the superior rectus, must be evaluated in patients with congenital ptosis. Correction of an ipsilateral hypotropia may improve the degree of ptosis. 4   Jaw-winking phenomenon can be detected by asking the patient to chew and move the jaws from side to side (see below). 5   Bell phenomenon is tested by manually holding the lids open, asking the patient to try to shut his eyes and observing upward and outward rotation of the globe. A weak Bell phenomenon carries a risk of postoperative exposure keratopathy, particularly following large levator resections or suspension procedures.

Simple congenital ptosis

1   Pathogenesis. Probably failure of neuronal migration or development with muscular sequelae; a minority are hereditary. 2   Signs (Fig. 1.52): •   Unilateral or bilateral ptosis of variable severity. •   Absent upper lid crease and poor levator function. •   In downgaze the ptotic lid is higher than the normal because of poor relaxation of the levator muscle. This is in contrast to acquired ptosis in which the affected lid is either level with or lower than the normal lid on downgaze. •   Following surgical correction the lid lag in downgaze may worsen.

3   Associations: •   Superior rectus weakness may be present because of its close embryological association with the levator. •   Compensatory chin elevation in severe bilateral cases. •   Refractive errors are common and more frequently responsible for amblyopia than the ptosis itself.

4   Treatment should be carried out during the preschool years once accurate measurements can be obtained, although it may be considered earlier in severe cases to prevent amblyopia. Most cases require levator resection (see below).

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Fig. 1.52 Congenital ptosis; (A) Mild right ptosis; (B) good levator function; (C) severe left ptosis with absent lid crease; (D) very poor levator function; (E) severe bilateral ptosis; (F) very poor levator function

Marcus Gunn jaw-winking syndrome

About 5% of all cases of congenital ptosis manifest the Marcus Gunn jaw-winking phenomenon. The vast majority are unilateral. Although the exact aetiology is unclear, it has been postulated that a branch of the mandibular division of the 5th cranial nerve is misdirected to the levator muscle. 1   Signs •   Retraction of the ptotic lid in conjunction with stimulation of the ipsilateral pterygoid muscles by chewing, sucking, opening the mouth (Fig. 1.53) or contralateral jaw movement. •   Less common stimuli to winking include jaw protrusion, smiling, swallowing and clenching of teeth.

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•   Jaw-winking does not improve with age, although patients may learn to mask it.

2   Surgery should be considered if jaw-winking or ptosis represents a significant functional or cosmetic problem. Although no surgical treatment is entirely satisfactory possible approaches include: a   Unilateral levator resection for mild cases with levator function 5 mm or better. b  Unilateral levator disinsertion and part resection with ipsilateral brow (frontalis) suspension for more severe cases. c   Bilateral levator disinsertion and part resection with bilateral brow suspension to produce a symmetrical result.

Fig. 1.53 Marcus Gunn jaw-winking syndrome. (A) Moderate left ptosis; (B) retraction of the lid on opening the mouth

3rd nerve misdirection syndromes

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3rd nerve misdirection syndromes may be congenital, or more frequently may follow acquired 3rd nerve palsies. Ptosis may also occur following aberrant facial nerve regeneration. 1   Signs. Bizarre movements of the upper lid which accompany various eye movements (Fig. 1.54). 2   Treatment is by levator disinsertion and brow suspension.

Fig. 1.54 3rd nerve redirection. (A) Moderate right ptosis; (B) retraction of the lid on right gaze (Courtesy of A Pearson)

Involutional ptosis

Involutional ptosis is an age-related condition caused by dehiscence, disinsertion or stretching of the levator aponeurosis, restricting transmission of force from a normal levator muscle to the upper lid. Due to fatigue of the Müller muscle it frequently worsens towards the end of the day, so that it can sometimes be confused with myasthenic ptosis. 1   Signs

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•   Variable, usually bilateral, ptosis with a high upper lid crease and good levator function (Fig. 1.55). •   In severe cases the upper lid crease may be absent, the eyelid above the tarsal plate very thin and the upper sulcus deep.

2   Treatment options include levator resection, advancement with reinsertion or anterior levator repair.

Fig. 1.55 (A) Severe bilateral involutional ptosis with high upper lid creases and deep sulci; (B) reasonable levator function, particularly on the left

Mechanical ptosis

Mechanical ptosis is the result of impaired mobility of the upper lid. It may be caused by dermatochalasis, large tumours such as neurofibromas (Fig. 1.56), heavy scar tissue, severe oedema and anterior orbital lesions.

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Fig. 1.56 Mechanical ptosis due to a neurofibroma

Surgery

Anatomy

1   The levator aponeurosis fuses with the orbital septum about 4 mm above the superior border of the tarsus (Fig. 1.57). Its posterior fibres insert into the lower third of the anterior surface of the tarsus. The medial and lateral horns are expansions that act as check ligaments. Surgically, the aponeurosis can be approached through the skin or conjunctiva. 2   Müller muscle is inserted into the upper border of the tarsus and can be approached transconjunctivally. 3   The inferior tarsal aponeurosis consists of the capsulopalpebral expansion of the inferior rectus muscle and is analogous to the levator aponeurosis. 4   The inferior tarsal muscle is analogous to Müller muscle.

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Fig. 1.57 Anatomy of the eyelid

Conjunctiva–Müller resection

1   Indications include mild ptosis with levator function of at least 10 mm. This includes most cases of Horner syndrome and very mild congenital ptosis. The maximal lift is 2– 3 mm. 2   Technique. Müller muscle and overlying conjunctiva are excised (Fig. 1.58A) and the resected edges reattached (Fig. 1.58B).

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Fig. 1.58 Conjunctiva–Müller resection. (A) Clamping of conjunctiva and Müller muscle; (B) appearance after excision and suturing

Levator resection

1   Indications are ptosis of any cause provided levator function is at least 5 mm. The extent of resection is determined by the amount of levator function and the severity of the ptosis. 2   Technique involves shortening of the levator complex through either an anterior (skin – Fig. 1.59) or posterior (conjunctival) approach.

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Fig. 1.59 Anterior levator resection. (A) Skin incision; (B) dissection and resection of levator aponeurosis; (C) levator reattachment to the tarsal plate (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Brow suspension

1 Indications •  Severe ptosis (>4 mm) with very poor levator function (<4 mm). •  Marcus Gunn jaw-winking syndrome. •  Ptosis associated with aberrant regeneration of the 3rd nerve. •  Blepharophimosis syndrome. •  Ptosis associated with 3rd nerve palsy. •  Unsatisfactory result from previous levator resection.

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2   Technique involves suspension of the tarsus from the frontalis muscle with a sling consisting of autologous fascia lata (Fig. 1.60) or non-absorbable material such as prolene or silicone.

Fig. 1.60 Brow suspension. (A) Site of incisions marked; (B) threading of fascia lata strips; (C) tightening and tying of strips (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Ectropion

Involutional ectropion

Involutional (age-related) ectropion affects the lower lid of elderly patients. It results in epiphora and in long-standing cases the tarsal conjunctiva may become chronically inflamed, thickened and keratinized (Fig. 1.61).

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Fig. 1.61 (A) Severe long-standing involutional ectropion; (B) keratinization of the tarsal conjunctiva (Courtesy of R Bates– fig. B)

Pathogenesis

The following age-related changes are contributory: 1   Horizontal lid laxity, which is demonstrated by pulling the central part of the lid 8 mm or more from the globe, with its failure to snap back to its normal position on release without the patient first blinking (Fig. 1.62A). 2   Medial canthal tendon laxity, which is demonstrated by pulling the lower lid laterally and observing the position of the inferior punctum. If the lid is normal the punctum should not be displaced more than 1–2 mm. If laxity is mild the punctum reaches the limbus, and if severe it reaches the pupil (Fig. 1.62B). 3   Lateral canthal tendon laxity, which is characterized by a rounded appearance of the lateral canthus and the ability to pull the lower lid medially more than 2 mm. 4    Disinsertion of lower lid retractors is occasionally relevant.

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Fig. 1.62 Pathogenesis of involutional ectropion. (A) Horizontal lid laxity; (B) medial canthal tendon laxity

Treatment

The methods of repair depend on the underlying aetiology and the predominant location of the ectropion as follows: 1   Generalized ectropion is treated with horizontal lid shortening. This is achieved either by excision of a tarso-conjunctival wedge (Fig. 1.63) or increasingly with a lateral canthal sling procedure (see Fig. 1.66B). 2   Medial ectropion may be treated with a medial tarsoconjunctival diamond excision, usually combined with a lateral canthal sling as horizontal laxity often co-exists. 3   Medial canthal tendon laxity, if marked, requires stabilization prior to horizontal shortening to avoid excessive dragging of the punctum laterally.

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Fig. 1.63 Horizontal lid shortening to correct ectropion. (A) Vertical cut; (B) excision of a pentagon; (C) closure (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Cicatricial ectropion

Cicatricial ectropion is caused by scarring or contracture of the skin and underlying tissues which pulls the eyelid away from the globe (Fig. 1.64A). If the skin is pushed up over the orbital margin with a finger the ectropion will be relieved and the lids will close. Opening the mouth tends to accentuate the ectropion. Depending on the cause, both lids may be involved and the defect may be local (e.g. trauma) or general (e.g. burns, dermatitis and ichthyosis). 1   Mild localized cases are treated by excision of the offending scar tissue combined with a procedure that lengthens vertical skin deficiency, such as Z-plasty. 2 Severe generalized cases require transposition flaps or free skin grafts (Fig. 1.64B and

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C). Sources of skin include the upper lids, as well as posterior auricular, preauricular and supraclavicular areas.

Fig. 1.64 Correction of cicatricial ectropion. (A) Preoperative appearance; (B) free-skin graft in place; (C) postoperative appearance (Courtesy of A Pearson)

Paralytic ectropion

Paralytic ectropion is caused by ipsilateral facial nerve palsy (Fig. 1.65A) and is associated with retraction of the upper and lower lids and brow ptosis; the latter may mimic narrowing of the palpebral aperture.

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Fig. 1.65 Paralytic ectropion. (A) Left facial palsy and severe ectropion; (B) lagophthalmos (Courtesy of A Pearson)

Complications

1   Exposure keratopathy which is caused by lagophthalmos (Fig. 1.65B). 2   Epiphora is caused by malposition of the inferior lacrimal punctum, failure of the lacrimal pump mechanism and an increase in tear production resulting from corneal exposure.

Temporary treatment

Temporary treatment is aimed at protecting the cornea in anticipation of spontaneous recovery of facial nerve function. 1 Lubrication with tear substitutes during the day, and instillation of ointment and taping

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shut of the lids during sleep are usually adequate in mild cases. 2   Botulinum toxin injection into the levator to induce temporary ptosis. 3   Temporary tarsorrhaphy, a procedure in which the lateral aspect of the upper and lower lids are sutured together, may be necessary, particularly in patients with a poor Bell phenomenon in which the cornea remains exposed when the patient attempts to blink.

Permanent treatment

Permanent treatment should be considered when there is irreversible damage to the facial nerve as may occur following removal of an acoustic neuroma, or when no further improvement is likely after Bell palsy. Treatment is aimed at reducing the horizontal and vertical dimensions of the palpebral aperture by one of the following procedures: 1   Medial canthoplasty may be performed if the medial canthal tendon is intact. The eyelids are sutured together medial to the lacrimal puncta (Fig. 1.66A) so that the puncta become inverted and the fissure between the inner canthus and puncta is shortened. 2   Lateral canthal sling may be used to correct residual ectropion and raise the lateral canthus (Fig. 1.66B). 3   Upper eyelid lowering can reduce the risk of exposure. 4   Gold weight implantation in the upper lid can assist closure.

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Fig. 1.66 Permanent treatment of paralytic ectropion. (A) Medial canthoplasty; (B) lateral canthal sling – refashioned canthal tendon from the lower lid is passed through a button hole in the tendon from the upper lid (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Mechanical ectropion

Mechanical ectropion is caused by tumours on or near the lid margin (Fig. 1.67) which mechanically evert the lid. Treatment involves removal of the cause if possible, and correction of significant horizontal lid laxity.

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Fig. 1.67 Mechanical ectropion

Entropion

Involutional entropion

Involutional (age-related) entropion affects mainly the lower lid because the upper has a broader tarsus and is more stable. The constant rubbing of the lashes on the cornea in long- standing entropion (pseudotrichiasis – Fig. 1.68A) may cause irritation, corneal punctate epithelial erosions and, in severe cases, ulceration and pannus formation.

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Fig. 1.68 (A) Involutional entropion and pseudotrichiasis; (B) preseptal orbicularis over-riding the pretarsal orbicularis

Pathogenesis

The pathogenesis involves age-related degeneration of elastic and fibrous tissues within the eyelid resulting in the following: 1   Horizontal lid laxity caused by stretching of the canthal tendons and tarsal plate. 2   Vertical lid instability caused by attenuation, dehiscence or disinsertion of the lower lid retractors. Weakness of the latter is recognized by decreased excursion of the lower lid in downgaze. 3   Over-riding of the pretarsal by the preseptal orbicularis during lid closure tends to move the lower border of the tarsus anteriorly, away from the globe, and the upper border towards the globe, thus tipping the lid inwards (Fig. 1.68B). 4   Orbital septum laxity with prolapse of orbital fat into the lower lid.

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Treatment

Temporary treatment is with lubricants, taping, soft bandage contact lenses or orbicularis chemodenervation with botulinum toxin injection. Surgical treatment aims to correct the underlying problems as follows: 1   Horizontal lid laxity is usually present and is corrected with a lateral canthal sling or a full-thickness wedge excision. 2   Over-riding and disinsertion a   Transverse everting sutures prevent over-riding of the preseptal orbicularis and provide temporary correction lasting several months (Fig. 1.69). b  Wies procedure gives a longer-lasting correction. It consists of full-thickness horizontal lid-splitting and insertion of everting sutures (Fig. 1.70). The scar creates a barrier between the preseptal and pretarsal orbicularis, and the everting suture transfers the pull of the lower lid retractors from the tarsus to the skin and orbicularis. c   Jones procedure tightens the lower lid retractors, thus increasing their pull and creates a barrier between the preseptal and pretarsal orbicularis (Fig. 1.71). It can be performed as primary treatment but is frequently reserved for recurrences.

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Fig. 1.69 Lid everting suture for entropion. (A) Three double armed sutures are passed blow the tarsal plate; (B) sutures are tied; (C) schematic (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

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Fig. 1.70 Wies procedure for entropion. (A) Full-thickness incision; (B) sutures are passed through the conjunctiva and lower lid retractors; (C) sutures are passed anterior to the tarsal plate to exit inferior to the lashes; (D) schematic (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001 – figs A–C).

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Fig. 1.71 Jones procedure for entropion. (A) Incision exposes the lower border of the tarsal plate; (B) reflection of the orbital septum and fat pad to expose the lower lid retractors; (C) tightening of retractors by plication; (D) schematic (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Cicatricial entropion

1   Pathogenesis. Severe scarring of the palpebral conjunctiva, which pulls the upper or lower lid margin towards the globe. Causes include cicatrizing conjunctivitis, trachoma, trauma and chemical injuries. 2   Treatment a   Medical treatment is aimed at protecting the cornea from abrasion by the lashes with a contact lens. b  Surgical treatment of mild cases is by transverse tarsotomy (tarsal fracture) with anterior rotation of the lid margin. Treatment of severe cases is difficult and is directed at replacing deficient or keratinized conjunctiva and replacing the scarred

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and contracted tarsus with composite grafts.

Miscellaneous acquired disorders

Varices

Eyelid varices are usually associated with orbital involvement (see Ch. 3). They appear as unilateral dark red or purple lesions subcutaneous lesions which in some cases become apparent only with a Valsalva manoeuvre (Fig. 1.72).

Fig. 1.72 Eyelid varices. (A) Before Valsalva; (B) during Valsalva (Courtesy of G Rose)

Blepharochalasis

Blepharochalasis is an uncommon condition characterized by recurrent episodes of painless, non-pitting oedema of both upper lids which usually resolves spontaneously after a few days. 1   Presentation is usually around puberty and with time, the episodes become less frequent. 2   Signs •   A hypertrophic form with orbital fat herniation and an atrophic form with absorption of orbital fat have been described. •   Redundant and atrophic lid skin resembling wrinkled cigarette paper. •   Severe cases may give rise to stretching of the canthal tendons and levator aponeurosis resulting in ptosis (Fig. 1.73). •   Lacrimal gland prolapse may occur.

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3   Differential diagnosis includes drug-induced urticaria and angioedema. 4   Treatment involves blepharoplasty for redundant upper lid skin, and correction of ptosis.

Fig. 1.73 Left aponeurotic ptosis and very thin upper lid skin resulting from blepharochalasis.

Floppy eyelid syndrome

Floppy eyelid syndrome is an uncommon unilateral or bilateral condition, which is often misdiagnosed. It typically affects obese middle-aged men who sleep face down with their lids everted by the pillow. Nocturnal exposure and poor contact of the lax lid with the globe in combination with tear film abnormalities result in keratoconjunctivitis. 1   Signs •   Redundant upper lid skin (Fig. 1.74A). •   Loose and rubbery tarsal plates (Fig. 1.74B) that are early to evert with gentle pressure on the skin below the brow (Fig. 1.74C). •   Chronic, intense micropapillary conjunctivitis of the superior tarsal conjunctiva (Fig. 1.74D). •   Punctate keratopathy, filamentary keratitis and superior superficial vascularization may be present. •   Other findings include eyelash ptosis, lacrimal gland prolapse, ectropion and aponeurotic ptosis.

2   Associations include keratoconus, skin hyperelasticity and joint hypermobility, obstructive sleep apnoea, diabetes and mental retardation. 3   Treatment of mild cases involves lubrication and nocturnal eye shields or taping of the lids. Severe cases require horizontal lid shortening to stabilize both upper eyelids and ocular surface and prevent nocturnal lagophthalmos.

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Fig. 1.74 Floppy eyelid syndrome. (A) Redundant upper lid skin; (B) loose and rubbery tarsal plates; (C) easily everted eyelid; (D) papillary conjunctivitis of the upper tarsal conjunctiva (Courtesy of J Nerad, K Carter and M Alford, from Oculoplastic and Reconstructive Surgery, in Rapid Diagnosis in Ophthalmology, Mosby 2008 – fig. B; S Tuft – fig. C)

Eyelid imbrication syndrome

1   Signs •   Eyelid imbrication syndrome is an uncommon disorder in which the upper lid overlaps the lower. •   It may be unilateral or bilateral and leads to ocular irritation. •   Associated signs include chronic papillary conjunctivitis of the upper tarsal conjunctiva and staining of the upper lid margins with rose bengal.

2   Associations include floppy eyelid syndrome, eyelid tumours, mucous membrane disease, surgical trauma and self-harming behaviour. 3   Treatment may involve full-thickness upper lid wedge resection, lateral canthal tendon plication, and lower lid horizontal shortening using a tarsal strip procedure.

Eyelid retraction

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Lid retraction is suspected when the upper lid margin is either level with or above the superior limbus (Fig. 1.75A); the causes are listed in Table 1.5.

Fig. 1.75 (A) Left lid retraction in thyroid eye disease; (B) following eyelid lowering procedure (Courtesy of A Pearson)

Table 1.5 -- Causes of lid retraction 1  Thyroid eye disease 2  Neurogenic •  Contralateral unilateral ptosis (Fig. 1.76A) •  Unopposed levator action due to facial palsy •  3rd nerve misdirection •  Marcus Gunn jaw-winking syndrome •  Collier sign of the dorsal midbrain (Parinaud syndrome – Fig. 1.76B) •  Infantile hydrocephalus (setting sun sign – Fig. 1.76C) •  Parkinsonism (Fig. 1.76D) •  Sympathomimetic drops

3  Mechanical

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•  Surgical over-correction of ptosis •  Scarring of upper lid skin

4  Congenital •  Isolated •  Duane retraction syndrome •  Down syndrome •  Transient ‘eye popping’ reflex in normal infants

5  Miscellaneous •  Prominent globe (pseudo-lid retraction) •  Uraemia (Summerskill sign) •  Idiopathic

Fig. 1.76 Causes of lid retraction. (A) Unilateral myasthenic ptosis with contralateral lid retraction; (B) Collier sign; (C) ‘setting sun’ sign in infantile hydrocephalus; (D) Parkinsonism (Courtesy of R Bates– fig. C)

Treatment

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Where there is no loss or tightness of upper eyelid skin, retraction is corrected by surgical release of the eyelid retractors, usually via a transconjunctival posterior approach. Mild retraction may be treated with Müller muscle recession (see Fig. 1.75B). Moderate to severe retraction may require levator aponeurosis recession.

Cosmetic eyelid and periocular surgery

Involutional changes

The following involutional (age-related) changes around the eyes can lead to both functional and cosmetic concerns that may require treatment: •    Reduction in cutaneous elasticity and thickness leads to loose, wrinkled skin. •    Weakening of the orbital septum may lead to orbital fat prolapse. •    Thinning and stretching of the canthal tendons, levator aponeurosis and lower lid retractors may lead to eyelid laxity and ptosis. •    Atrophy of orbital and eyebrow fat pads may lead to enophthalmos and eyebrow sagging. •    Weakening of the frontalis muscle and epicranial aponeurosis may cause descent of the eyebrows and increasing looseness of upper eyelid skin. •    Thinning and stretching of the mid-face support leads to descent with formation of a tear trough depression and exacerbation of lower eyelid changes. •    Thinning and resorption of periorbital bone increases the appearance of surplus overlying tissues.

Non-surgical techniques

Botulinum toxin injections to periocular muscles

1   Indications include reduction in wrinkling particularly for ‘crows-feet’ at the lateral canthus and for glabellar frown lines, and ‘brow lift’ by reduction in action of brow depressors. 2   Complications include temporary lagophthalmos, ptosis, ectropion and diplopia.

Tissue fillers

1   Hyaluronic acid fillers can be used to temporarily fill in hollows and replace lost volume. They are injected deep to orbicularis and the effects generally last 6–12 months. 2   Transfer of fat gives a more permanent replacement. Although most transplanted fat previously resorbed, newer techniques, such as Coleman fat transfer, have led to much smaller individual fat grafts with survival of approximately 50–60% of the transferred tissue.

Skin resurfacing

Removal of the superficial layers of the skin, by chemical peels or laser, can lead to reduction in wrinkling, increased evenness of pigmentation, removal of blemishes and improved texture by generating new epidermis and increasing collagen production in the dermis.

Surgical techniques

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Upper eyelid blepharoplasty

1   Upper eyelid involutional changes are characterized by surplus upper eyelid skin (dermatochalasis) that leads to baggy lids with indistinct creases and pseudo-ptosis. It may cause a heavy sensation around the eyes, brow ache and, in more advanced cases, obstruction of the superior visual field (Fig. 1.77A). 2   Upper eyelid blepharoplasty (Fig. 1.77B) is effective for removal of surplus upper eyelid skin and can be combined with reduction of the superior orbital fat pads. Care must be taken prior to surgery to look for ptosis of the eyelid or eyebrow and ocular surface dryness. 3   Complications include removal of excess skin leading to lagophthalmos and corneal drying, and removal of excess orbital fat leading to an unattractive hollowed out upper eyelid sulcus.

Fig. 1.77 (A) Severe dermatochalasis causing reduction of upper visual field; (B) appearance following surgery (Courtesy of A Pearson)

Lower eyelid blepharoplasty

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1   Lower lid involutional changes are characterized by excess skin and/or prolapsed orbital fat (Fig. 1.78A). 2   Lower lid blepharoplasty can address these problems (Fig. 1.78B). Where there is excess skin an anterior approach is used to raise a skin/muscle flap which can be lifted and re-draped on the lid with the surplus removed. At the same time the inferior orbital fat pads can be reduced by a small incision through the septum. Bulging of the lower eyelid fat pads without eyelid laxity or surplus skin is best reduced by a posterior, transconjunctival approach. 3   Complications include lower eyelid retraction, contour abnormalities (particularly lateral drooping), or frank ectropion.

Fig. 1.78 (A) Mild dermatochalasis and excess lower lid skin; (B) appearance following upper and lower lid blepharoplasty (Courtesy of A Pearson)

Brow ptosis correction

1   Brow ptosis frequently accompanies dermatochalasis (Fig. 1.79A) and may also follow facial nerve palsy or localized trauma. Lifting of the brow needs to precede or occasionally be combined with upper eyelid blepharoplasty.

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2   Techniques of brow-lift include: •   Direct brow-lift in which an incision is made above the eyebrow hairs and an ellipse of skin removed (Fig. 1.79B). •   Endoscopic brow-lift in which small incisions within the hair-line enable endoscopic elevation of the whole forehead tissues and release at the eyebrow periosteum to allow lifting of the eyebrows through sutures supported on frontal bone anchors within the hair-line.

Fig. 1.79 (A) Right brow ptosis and dermatochalasis; (B) following a direct brow-lift procedure (Courtesy of A Pearson)

Congenital malformations

Epicanthic folds

Epicanthic folds are bilateral vertical folds of skin that extend from the upper or lower lids towards the medial canthi. They may give rise to a pseudoexotropia. 1   Signs

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a   Palpebralis. The folds are symmetrically distributed between the upper and lower lids (Fig. 1.80A); this is the most common type in Caucasians. b  Tarsalis. The folds originate in the medial aspects of the upper lids and extend medially before dissipating (Fig. 1.80B); this is the most common type in Orientals. c   Inversus is associated with the blepharophimosis syndrome. The folds start in the lower lids and extend upwards to the medial canthal areas (Fig. 1.80C). d  Superciliaris. The folds arise above the brow and extend downwards to the lateral aspect of the nose.

2   Treatment of small folds is by Y–V plasty (Fig. 1.80D), whilst large folds require a Mustardé Z-plasty.

Fig. 1.80 Epicanthic folds. (A) Palpebralis; (B) tarsalis; (C) inversus; (D) V-Y plasty (Courtesy of R Bates – fig. D)

Telecanthus

Telecanthus is an uncommon condition that may occur in isolation or in association with blepharophimosis syndrome. 1   Signs. Increased distance between the medial canthi as a result of abnormally long medial canthal tendons (Fig. 1.81). It should not be confused with hypertelorism in which there is wide separation of the orbits. 2   Treatment involves shortening and refixation of the medial canthal tendons to the anterior lacrimal crest, or insertion of a transnasal suture. 3   Associated systemic syndromes include Waardenburg, Möbius, Treacher Collins, Rubinstein-Taybi and Turner.

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Fig. 1.81 Telecanthus

Blepharophimosis ptosis and epicanthus inversus syndrome

1   Inheritance is usually AD. BPES 1 (with premature ovarian failure) and BPES 2 (without premature ovarian failure) are caused by type 1 mutations in FOXL2 gene on chromosome 3. 2   Signs (Fig. 1.82) •   Moderate to severe symmetrical ptosis with poor levator function. •   Telecanthus and epicanthus inversus (see Fig. 1.80C). •   Poorly developed nasal bridge and hypoplasia of the superior orbital rims.

3   Treatment initially involves correction of epicanthus and telecanthus, followed a few months later by bilateral frontalis suspension. It is also important to treat amblyopia, which is present in about 50% of cases.

Fig. 1.82 Blepharophimosis ptosis and epicanthus inversus syndrome

Epiblepharon

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Epiblepharon is very common in Orientals and should not be confused with the much less common congenital entropion. 1   Signs •   An extra horizontal fold of skin stretches across the anterior lid margin and the lashes are directed vertically, especially in the medial part of the lid (Fig. 1.83A and B). •   When the fold of skin is pulled down the lashes turn out and the normal location of the lid becomes apparent (Fig. 1.83C).

2   Treatment is not required in the majority of Caucasians because spontaneous resolution with age is the rule. Persistent cases are treated by excising a strip of skin and muscle, and fixation of the skin crease to the tarsal plate (Hotz procedure – Fig. 1.84A–C).

Fig. 1.83 (A) Epiblepharon; (B) lashes pointing upwards; (C) normal position of lashes following manual correction

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Fig. 1.84 Hotz procedure. (A) Incision marks on the medial two-thirds of the eyelid; (B) skin sutures passed deeply through the inferior tarsal border; (C) skin crease created by the sutures (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001)

Congenital entropion

Upper lid entropion

Upper lid entropion is usually secondary to mechanical effects of microphthalmos, which cause variable degrees of upper lid inversion.

Lower lid entropion

Lower lid entropion is caused by improper development of the inferior retractor aponeurosis.

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1   Signs •   Inturning of the entire lower eyelid and lashes with absence of the lower lid crease (Fig. 1.85). •   When downward pressure is applied to the lid, the entire length pulls away from the globe.

2   Treatment involves the excision of a strip of skin and muscle, and fixation of the skin crease to the tarsal plate (Hotz procedure).

Fig. 1.85 Congenital entropion

Coloboma

A coloboma is an uncommon, unilateral or bilateral, partial or full-thickness eyelid defect. It occurs when eyelid development is incomplete, due to either failure of migration of lid ectoderm to fuse the lid folds, or to mechanical forces such as amniotic bands. 1   Upper lid colobomas occur at the junction of the middle and inner thirds (Fig. 1.86A) and may occasionally be associated with Goldenhar syndrome. 2   Lower lid colobomas occur at the junction of the middle and outer thirds and are frequently associated with systemic conditions, most notably Treacher Collins syndrome (Figs 1.86B and 1.87) and amniotic band syndrome. 3   Treatment of small defects involves primary closure, while large defects require skin grafts and rotation flaps.

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Fig. 1.86 (A) Upper lid colobomas; (B) lower lid colobomas in Treacher Collins syndrome (Courtesy of U Raina – fig. A)

Treacher Collins syndrome

Treacher Collins syndrome (mandibulofacial dysostosis)is characterized by malformation of derivatives of the first and second branchial arches. 1   Inheritance is AD with high penetrance and variable expressivity, although 60% of cases occur with no family history and are thought to arise by de novo mutation. The gene involved is the ‘treacle’ gene TCOF1 on chromosome 5q. 2   Systemic features •   Bilateral hypoplasia of the mandible and zygoma and beaked nose (Fig. 1.87) •   Micrognathia and malformed ears •   Conductive deafness.

3   Ocular features •  Extorted slanting of the palpebral apertures •  Colobomas of the lateral lower eyelids •  Cataract •  Microphthalmos •  Atresia of the lacrimal passages.

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Fig. 1.87 Treacher Collins syndrome

Euryblepharon

1   Signs •   Horizontal enlargement of the palpebral fissure with associated lateral canthal malposition and lateral ectropion (Fig. 1.88). •   In severe cases it may result in lagophthalmos and exposure keratopathy.

2   Associations include lateral displacement of the proximal lacrimal drainage system, a double row of meibomian gland orifices, telecanthus and strabismus. 3   Treatment involves lateral canthal tightening or tarsorrhaphy.

Fig. 1.88 Euryblepharon (Courtesy of D Taylor and C Hoyt, from Pediatric Ophthalmology and Strabismus, Elsevier 2005)

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Microblepharon

Microblepharon is characterized by small eyelids, often associated with anophthalmos (Fig. 1.89).

Fig. 1.89 Microblepharon associated with anophthalmos

Ablepharon

1   Signs. Deficiency of the anterior lamellae of the eyelids (Fig. 1.90A). 2   Treatment involves reconstructive skin grafting. 3   Systemic anomalies. Ablepharon-macrostomia syndrome is characterized by an enlarged fish-like mouth (Fig. 1.90B), ear and genital anomalies, and redundant skin.

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Fig. 1.90 (A) Ablepharon; (B) following reconstruction – note enlarged fish-like mouth (Courtesy of D Taylor and C Hoyt, from Pediatric Ophthalmology and Strabismus, Elsevier 2005 – fig. A; H Mroczkowska – fig. B)

Cryptophthalmos

1   Signs •   In complete cryptophthalmos the lids are replaced by a layer of skin which is fused with a microphthalmic eye (Fig. 1.91A). •   Incomplete cryptophthalmos is characterized by microphthalmos, rudimentary lids and a small conjunctival sac (Fig. 1.91B).

2   Systemic association. Fraser syndrome is an AD condition characterized by syndactyly, urogenital anomalies, malformations of the upper airway and craniofacial structures and mental handicap.

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Fig. 1.91 Cryptophthalmos. (A) Complete; (B) incomplete (Courtesy of D Meyer – fig. A)

Congenital upper lid eversion

Congenital upper lid eversion is a rare condition more frequently seen in infants of Afro- Caribbean origin, in Down syndrome and in congenital ichthyosis (collodion skin disease – Fig. 1.92). It is typically bilateral and symmetrical. It may either resolve spontaneously with conservative treatment or may require surgery.

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Fig. 1.92 Congenital upper lid eversion in a patient with collodion skin disease (Courtesy of D Meyer)

Ankyloblepharon filiforme adnatum

1  Inheritance. Most cases are sporadic although some are AD. 2  Signs. The upper and lower eyelids are joined by thin tags (Fig. 1.93). 3  Treatment involves transection with scissors; anaesthesia is not required.

Fig. 1.93 Ankyloblepharon filiforme adnatum (Courtesy of D Taylor and C Hoyt, from Pediatric Ophthalmology and Strabismus, Elsevier 2005)

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