Ординатура / Офтальмология / Английские материалы / Oculoplasty and Reconstructive Surgery Made Easy_Garg,Touky, Nasralla_2009

Congenital Eyelid Anomalies 9

MICROBLEPHARON AND ABLEPHARON

Introduction and Signs & Symptoms

A microblepharon is a rare vertical foreshortening of the eyelid. The upper lid is more commonly affected. Apparent ablepharon may be a severe microblepharon, and one should look carefully for rudimentary eyelids. It may be associated with clinical anophthalmos. It is associated with lagophthalmos and exposure keratopathy. There may be additional ocular and systemic abnormalities (Figure 4).

Investigation

Evaluation should be performed for addditonal systemic and ocular abnormalities. In a microphthalmic eye, assessment for visual potential is required.

Figure 4: Patient with right eye euryblepharon, left eye clinical anophthalmos with upper and lower lid microblepharon. The eyelids are short vertically as well as horizontally

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Differential Diagnosis

Microblepharon and ablepharon should be differentiated from lid colobomas.

Management

The surgical management plan depends on the visual potential and the specific features. Correction may require rotation flaps from the cheek, lid sharing procedures, pedicle flaps or skin grafts.

ANKYLOBLEPHARON

Ankyloblepharon Filiforme Adnatum

Signs and Symptoms

The condition shows fine extensile cords attaching upper and lower lids, which may be single or multiple. The cords decrease the palpebral fissure height and reduce lid excursion. It has been reported n association with trisomy 18.

This may be associated with ectodermal dysplasia and cleft lip or cleft palate – this is the autosomal dominant HayWell’s syndrome.

Treatment

The cord is simply severed, and the epithelial tags involute.

Congenital Ankyloblepharon

Introduction

Congenital ankyloblepharon is caused by developmental arrest leading to growth aberration at either canthus, inner or outer.

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Signs and Symptoms

It is caused by partial fusion of the upper and lower lid, and results in horizontal foreshortening of the palpebral fissure. The commoner external variety is at the lateral canthus, causing an appearance of pseudoexotropia. The internal ankyloblepharon at the inner canthus causes pseudoesotropia.

Differential Diagnosis

Pseudo-eso- or exotropia must be differentiated from true strabismus.

Treatment

Ankyloblepharon requires surgical separation of the eyelids.

MEDIAL CANTHAL ECTOPIA

Introduction

Medial canthal ectopia is caused by developmental arrest in the second month of intrauterine life.

Signs and Symptoms

The medial canthal tendon is displaced inferiorly along with surrounding canthal structures. It attaches to the junction of the medial wall of the orbit and the inferior orbital rim. There may be associated nasal cleft deformities and nasolacrimal duct obstruction (Figure 5).

Differential Diagnosis

The condition is to be differentiated from medial canthal dystopia acquired after trauma.

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Figure 5: Clinical photograph of patient with right eye corneal dermoid, medial canthal ectopia both eyes and small colobomatous lid notching in left eye. The medial canthus is displaced downwards, and is associated with congenital nasolacrimal duct obstruction. The patient has undergone surgical correction of cleft lip and palate previously

Investigation

The child should be assessed for nas-lacrimal duct obstruction.

Treatment

The condition requires surgical supraplacement of the medial canthal tendon: a Z-plasty or a Y-V plasty may be used. The associated nasolacrimal duct obstruction should also be corrected.

EPICANTHUS

Introduction and Signs & Symptoms

Epicanthus is a semi-lunar fold of skin at the medial canthus, with its concavity directed outward. The epicanthal fold obscures the view of the medial canthal angle, the caruncle and plica. An epicanthus may be isolated or be an associated feature in a patient with congenital ptosis or Blepharophimosis syndrome. There are several varieties of epicanthus.

• Epicanthus tarsalis extends from the upper lid

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•Epicanthus inversus extends from the lower lid (associated with Blepharophimosis-ptosis-epicanthus inversus syndrome—BPES)

•Epicanthus palpebralis extends from the upper lid to the lower lid

•Epicanthus superciliaris extends from below the eye-brow. Epicanthus may give an appearance of pseudoesotropia

(Figures 6A to C).

Differential Diagnosis

Any epicanthus should be examined to rule out associated BPES.

Figure 6A: Patient with right eye congenital ptosis, with epicanthus tarsalis both eyes. A fold of skin extends from the upper lid to the lower lid at the medial canthus

Figure 6B: Patient with bilateral congenital ptosis and bilateral epicanthus inversus. A semilunar fold of skin extends from the upper lid to the lower lid

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Figure 6C: Epicanthus palpebralis, with a semilunar fold of skin extending on both upper and lower lids. The epicanthal fold obscures the medial canthal structures and gives an appearance of pseudostrabismus

Treatment

Epicanthus is corrected by Mustarde double Z-plasty, modified Y-V plasty or C-U plasty.

CENTURION SYNDROME

Introduction and Signs & Symptoms

The patient of centurion syndrome has a history of epiphora since childhood, which worsens at puberty. Syringing of the nasolacrimal duct is patent. The syndrome derives its name from the resemblance of the patients to the Roman centurions in their helmets. The medial canthal tendon is taut and attached anteriorly, causing medial stand-off of the lower lid and punctum. The lacrimal punctum is displaced forward out of the lacrimal lake, causing epiphora. The facial appearance is typical, with a sharp medial canthus, long horizontal palpebral fissure and square eyebrows (Figure 7).

Differential Diagnosis

The condition is to be differentiated from other causes of epiphora.

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Figure 7: Patient with centurion syndrome, with sharp medial canthal angles, square eyebrows, and elongated horizontal palpebral aperture.

The medial canthal tendon is prominent and attached anteriorly, not allowing proper apposition of the lacrimal punctum to the tear meniscus

Investigation

Lacrimal syringing will be patent, but dye disappearance will be delayed.

Treatment

The treatment is surgical, anterior medial canthal tendon release, with medial canthoplasty or punctoplasty.

DISTICHIASIS

Introduction and Signs & Symptoms

Distichiasis is an extra row of eyelashes behind the normal ciliary margin at the sites of meibomian glands. It may involve the whole length of the lid or only a segment. The distichiatic lashes rubbing on the ocular surface can induce epiphora or even keratopathy. Congenital distichiasis is associated with lower limb lymphedema; the gene responsible is FOXC 2

(Figure 8).

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Figure 8: Incomplete row of eyelashes growing on the medial aspect of the upper lid; the lashes are at the sites of meibomian gland orifices

Differential Diagnosis

Distichiasis is to be differentiated from other conditions with abnormal positions or abnormal directions of eyelashes, such as trichiasis, epiblepharon and entropion.

Investigation

Instillation of fluorescein in the eye will help to detect early keratopathy.

Treatment

If the patient is asymptomatic, observation alone suffices. A small number of lashes may be treated by electrolysis. In extensive distichiasis, the lid can be split along the grey line and cryo applied to the posterior lamella at the roots of the distichiatic eyelashes.

Congenital Eyelid Anomalies 17

EPIBLEPHARON

Introduction and Signs & Symptoms

Epiblepharon comprises a horizontal redundant lid fold of skin and orbicularis in the lower lid, causing vertical orientation of the eyelashes. There may be coexistent entropion. The eyelashes rubbing on the cornea cause epiphora, and may cause keratopathy. It is commoner in Mongolian features. It may be associated with inferior oblique weakness. Epiblepharon often resolves spontaneously by 3 years of age (Figures 9A and B).

Differential Diagnosis

The condition is to be differentiated from congenital entropion and distichiasis.

Investigation

Instillation of fluorescein dye will help to detect any keratopathy. Careful examination will rule out associated inferior oblique weakness.

Treatment

Epiblepharon may be treated with lubricants in the expectation of spontaneous resolution. If the cornea shows

Figure 9A: Patient with bilateral epiblepharon with high tear meniscus

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Figure 9B: Clinical photograph showing redundant horizontal fold of lower lid skin and orbicularis, with vertical orientation of eyelashes

keratopathy, surgical intervention is required. The redundant skin and orbicularis oculi muscle is excised in the form of a spindle; usually 1-3 mm of excision would suffice. In severe cases everting sutures may be used in addition.

CONGENITAL ENTROPION

Introduction

Congenital entropion affects the lower lid, and results from inadequate development of the lower lid retractors. This causes lower lid malpositions similar to that in senile entropion. The entropion is often accompanied by epiblepharon and epicanthus (Figure 10).

Signs and Symptoms

In congenital entropion the lower lid rolls inwards, and the eyelashes rub on the ocular surface. The patient may be symptomatic, with epiphora and keratopathy.