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

Congenital Eyelid Anomalies 19

Figure 10: Clinical photograph of patient with bilateral congenital ptosis, with anti-mongoloid slant of palpebral fissures, with lower lid entropion. Left eye shows a failed corneal graft and lower lid external hordeolum

Differential Diagnosis

Congenital entropion must be distinguished from epiblepharon and distichiasis.

Management

Surgical correction is required if the patient develops keratopathy. The procedure of choice is reattachment of the lower lid retractors to the tarsus.

TARSAL KINK SYNDROME

Introduction and Signs & Symptoms

Tarsal kink syndrome is a rare entity with horizontal fold along the entire horizontal length of the tarsus. Tarsal kink results in entropion of the upper lid; in the severest cases, the child presents within a few weeks of age, and the lid margin is not visible. The patient may have absent lid crease, blepharospasm, lid edema and keratopathy. The patient commonly presents with corneal ulcer in early infancy

(Figures 11A to C).

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A

B

Figures 11A and B: Patient with bilateral tarsal kink and bilateral corneal ulcers. The upper lids show severe entropion, with obscuration of lid margin and absent lid crease

Differential Diagnosis

Tarsal kink syndrome with corneal infiltrate should be differentiated from patients presenting with other corneal ulcers.

Congenital Eyelid Anomalies 21

Figure 11C: The everted eyelid shows a crease in the tarsal plate along the entire horizontal length

Management

Surgical correction of the tarsal kink entails full-thickness blepharotomy the entire length of eyelid with marginal rotation. Due to the very early age of presentation and the associatedcornealulcerationandscarring,amblyopiatherapy is crucial after correction of the entropion (Figure 11D).

Figure 11D: The clinical photograph of the same patient after surgical correction of the entropion

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CONGENITAL ECTROPION

Introduction and Signs & Symptoms

Congenital ectropion of the lower lid is rarely isolated; it is more likely to be a part of a syndrome such as Kohn Romano syndrome (Figures 12A and B). Congenital ectropion of the lower lid often has vertical shortage of skin. Severe ectropion will cause epiphora and exposure, and the tarsal conjunctiva may become keratinized.

Lamellar ichthyosis is a systemic condition which may result in cicatricial ectropion of upper and lower lid at birth.

Occasionally there is an isolated eversion of the upper lid, for which the etiology is postulated as birth trauma causing vascular stasis and congestion, leading to lid eversion (Figure

13).

Differential Diagnosis

The ectropion due to shortage of skin should be differentiated from euryblepharon, which also causes lateral ectropion of the lower lid.

Management

Congenital ectropion requires full-thickness skin grafting; the preferred donor site is the retroauricular area.

Figure 12A: Clinical photograph of patient with bilateral congenital ptosis, blepharophimosis, telecanthus and lower lid ectropion

Congenital Eyelid Anomalies 23

Figure 12B: The lower lid shows severe ectropion, vertical shortening of the anterior lamella, keratinization and pigmentation of the conjunctiva.

The cornea shows exposure keratopathy

Figure 13: Left eye upper lid congenital ectropion, with complete eversion of eyelid

In lamellar ichthyosis, it may be difficult to identify any area of healthy skin as a potential donor site. Skin emollients should also be used liberally.

In the isolated upper lid eversion, one may escape surgery, and get by only with marginal traction suture to straighten the lid. If the ectropion persists, one may need to excise a part

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of the prolapsed conjunctiva to allow correction of the lid eversion.

EURYBLEPHARON

Introduction and Signs & Symptoms

Euryblepharon presents with bilateral symmetrical enlargement of the horizontal palpebral apertures, with vertical shortage of eyelid skin, elongated lid margins, and downward and lateral displacement of outer canthi. The horizontal palpebral fissure length is increased to approximately 35 mm from the average of 28-30 mm. There is lateral ectropion, lagophthalmos and reduced blink rate with exposure keratopathy. The exposure and reduced blink rate may cause epiphora (Figures 14A to C).

Differential Diagnosis

Euryblepharon should be distinguished from the congenital ectropion which has vertical shortage of skin, but not an elongated lid margin.

Other causes of epiphora in childhood should be differentiated from euryblepharon.

Figure 14A: Clinical photograph of patient with bilateral upper and lower lid lateral ectropion, with excess horizontal length

Congenital Eyelid Anomalies 25

Figure 14B: Photograph of the same patient after surgical correction of the euryblepharon. The corneas are scarred due to exposure

Figure 14C: Photograph of patient with mild degree of euryblepharon, with lateral ectropion of lower lid alone

Management

Mild degrees may be managed conservatively, with lubricants. Moderate degrees will require tarsorrhaphy to protect the cornea. A lateral canthoplasty with shortening of the upper and lower lids laterally may be beneficial. The severest forms may require staged correction with augmentation of the posterior lamella and anterior skin graft.

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CONGENITAL LID RETRACTION

Introduction and Signs & Symptoms

Congenital retraction of the upper lid may be unilateral or bilateral. A unilateral lid retraction may simulate contralateral ptosis. The condition is non-progressive. Other systemic features are usually normal.

Differential Diagnosis

Congenital lid retraction must be differentiated from other causes of lid retraction such as thyroid abnormalities, and Marcus Gunn phenomenon. Children developing eyelid retraction must be investigated for intracranial spaceoccupying lesions, hydrocephalus and postencephalitic sequelae (Figures 15A and B).

There has been a case report of accessory slip of levator palpebrae superioris causing lid retraction.

Investigation

Thyroid function tests may rule out endocrine-related lid retraction.

Management

Lid retraction may be treated by lid lengthening procedures.

CONGENITAL PTOSIS

Introduction and Signs & Symptoms

Congenital ptosis is the commonest of the congenital lid anomalies. The upper lid is at a lower level than normal, and the condition may be unilateral or bilateral. The affected eye may have limited elevation. A quick assessment of the severity of congenital ptosis is possible by looking at the red reflex of

Congenital Eyelid Anomalies 27

Figure 15A: Clinical photograph of patient with congenital retraction of left upper lid

Figure 15B: Photograph of the same patient after surgical correction of the lid retraction

the pupil through distant direct ophthalmoscopy, and recording how much of the pupil is obscured by the lid. Other indicators of the severity of the ptosis are over-action of the frontalis muscles and chin elevation on inspection of the face. A greater palpebral fissure height in down gaze is seen in the unilateral ptosis, since the defective levator palpebrae muscle is also deficient in ability to relax. A faint upper lid crease indicates weak levator action. Marcus Gunn phenomenon, i.e. synkinetic elevation of the ptotic lid with jaw movement should be looked for. Amblyopia may develop due to visual deprivation, refractive error or associated strabismus (Figures

16A and B).

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Figure 16A: Patient with right eye congenital ptosis

Figure 16B: Photograph of same patient after ptosis correction by frontalis suspension

Differential Diagnosis

Careful examination will rule out other causes of ptosis in the young.

Myasthenia gravis may rarely occur in a child. Variable ptosis, involvement of extraocular muscles and fatigability indicate myasthenic ptosis. The diagnosis may be confirmed by edrophonium test.

Chronic progressive external ophthalmoplegia may have an onset in the first decade of life, and show associated limitation of ocular movements.

Congenital ocular fibrosis presents with ptosis along with strabismus. The ocular movements are severely restricted.