Droopy Eyelids

The Problem

“My child’s eyelid is droopy.”

Common Causes

Congenital ptosis

Other Causes

Myasthenia gravis Horner syndrome Third nerve palsy Eyelid or orbital mass Pseudoptosis

Eyelid retraction of opposite eye Eyebrow skin overhanging normal eyelid

KEY FINDINGS

History

Congenital ptosis Present from birth

Isolated, familial, or syndromic Often worse with fatigue Chin-up head posture

Myasthenia

Variable ptosis, worse with fatigue Often have strabismus/diplopia

Horner syndrome Congenital or acquired Unequal pupils

Decreased sweating on affected side Unequal iris colors (if congenital)

Third cranial nerve palsy Strabismus/diplopia Unequal pupils

Other symptoms depending on etiology Eyelid or orbital mass

Eyelid lesion or proptosis Possible limited eye movement

Other symptoms depending on etiology

Pseudoptosis

Mild appearance of ptosis due to excess skin overhanging eyelid

Squinting of eyelid due to other ocular disorder History of light sensitivity

Foreign body sensation or ocular discomfort Eyelid retraction on opposite side

Alternates between eyelid retraction of one eye and ptosis of the other

Proptosis of opposite eye

Other symptoms depending on etiology of proptosis

Examination

Congenital ptosis

Unilateral or bilateral drooping of eyelids Varies from mild to almost complete occlusion Decreased ability to elevate eyelid

Decreased eyelid crease

Brow lift and chin-up posture if marked ptosis

Myasthenia gravis Variable ptosis

Eyelid twitch (Cogan’s sign) Increased eyelid opening after rest,

ice test

Often have strabismus Horner syndrome

Usually mild-to-moderate ptosis Pupil smaller on affected side

Decreased sweating/facial flushing on affected side

Third nerve palsy

Usually moderate to marked ptosis Strabismus (eye out and down)

Unequal pupils (pupil larger on affected side, except may be smaller in congenital third nerve palsy)

Examination (continued)

Eyelid or orbital mass Visible lesion on eyelid Proptosis

Limited extraocular movements Pseudoptosis

Extra eyebrow skin

Eyelid height and function normal Strabismus

Appearance of ptosis due to strabismic eye being lower

Voluntary closure due to other ocular problems Corneal foreign body, abrasion

Other ocular inflammatory disorders Eyelid retraction on opposite side

If child fixes with retracted eye, opposite eye appears ptotic

If child fixes with normal eye, retraction worse in opposite eye

Possible proptosis on side with eyelid retraction

WHAT SHOULD YOU DO?

Children with congenital ptosis may develop amblyopia, particularly if the ptosis is unilateral and occludes the pupil. These children should be referred to a pediatric ophthalmologist to determine whether surgical treatment is indicated. Mild-to-moderate ptosis usually is not an immediate threat to vision, but evaluation is important due to its possible association with systemic diseases. Children with new onset of acquired ptosis, particularly if associated with signs of third nerve palsy or orbital mass, should be referred promptly for further evaluation.

Table 14–1.

Causes of Ptosis With Potential Serious

Systemic Implications

■Third nerve palsy

Intracranial tumor

Trauma

■Orbital tumor

Primary

Metatstatic

■Horner syndrome

Neuroblastoma

What Shouldn’t Be Missed

Acquired ptosis may be the initial sign of a serious underlying disorder, such as a third nerve palsy or an orbital tumor. Prompt diagnosis improves the outcome of most of these disorders (Table 14–1).

COMMON CAUSES

1.Congenital ptosis. Congenital ptosis is present at birth. It may be unilateral or bilateral, and varies in severity from mild to severe. Congenital ptosis may be familial or associated with an underlying syndrome, but is often an isolated finding in an otherwise healthy child. Severe congenital ptosis requires early repair due to the risk of amblyopia (Figure 14–1A and B).

2.Myasthenia gravis. Myasthenia gravis is rare, but ptosis is often the presenting complaint. It may be present at birth due to transplacental maternal antibodies, or may be acquired. The hallmark of myasthenia gravis is variability. It is worse when the child is fatigued. Variable strabismus is also commonly present.

A

B

FIGURE 14–1 ■ Severe congenital ptosis, left eye. (A) High risk of amblyopia because eyelid completely covers pupil. Note normal right upper eyelid crease (arrow) and absence of left upper eyelid crease.

(B) After surgery, patient can see normally.

90 ■ Section 2: Symptoms

FIGURE 14–2 ■ Horner syndrome, right eye. Note mild right ptosis and anisocoria (right pupil smaller than left).

Table 14–2.

Causes of Pseudoptosis

■Excess eyelid skin overhanging eyelid margin

■Eyelid retraction of opposite eye

■Voluntary lid closure

Eye irritation

Strabismus (especially exotropia) ■ Vertical strabismus

3.Horner syndrome. The ptosis in patients with Horner syndrome is usually mild to moderate. Patients have unequal pupils (smaller on the affected side), and may demonstrate decreased sweating of the brow on the affected side (Figure 14–2). Horner syndrome itself does not cause vision problems. Its importance lies in possible associations with systemic diseases, such as neuroblastoma.

4.Third nerve palsy. Patients with complete third nerve palsies usually have marked ptosis on the affected side, severe strabismus with the eye out and down, and a larger pupil on the affected side (although the pupil in some patients with congenital third nerve palsy may be smaller) (Figure 14–3). Severe ptosis from a third nerve palsy may cause amblyopia in young patients. The presence of an acquired third nerve palsy requires prompt evaluation.

5.Eyelid or orbital mass. A large number of eyelid and orbital lesions may cause secondary ptosis. In most eyelid lesions, this is a mechanical effect due to the increased weight of the eyelids, and the etiology is obvious on examination. Early orbital lesions may cause ptosis without marked

FIGURE 14–3 ■ Third nerve palsy, left eye. Marked ptosis of left eye. Note that the eye is also displaced downward and outward.

proptosis, and this possibility should be kept in mind in patients with acquired ptosis.

6.Pseudoptosis. This may occur for a variety of reasons (Table 14–2).

a.Excess brow skin on the affected side may produce mild apparent eyelid asymmetry. This is benign.

b.Eyelid retraction of the opposite eye. This may be an isolated finding, or a secondary effect of proptosis (usually due to an orbital mass).

c.Voluntary closure of the eye due to ocular irritation or light sensitivity.

d.Vertical strabismus, in which the eyelid on the side with the lower eye appears to have ptosis (Figure 14–4A and B).

A

B

FIGURE 14–4 ■ Pseudo-ptosis due to vertical strabismus. The left eye is hypotropic (lower than the right eye). (A) When the patient fixates with the right eye, the left eyelid appears to have ptosis because the left eye is lower than the right. (B) The left eyelid elevates when the patient fixates with the left eye. Note the visible sclera beneath the right iris due to associated upward movement of right eye.

Table 14–3.

Ptosis—Associations With Other Eye Findings

■Ptosis strabismus

Third nerve palsy (eye out and down)

Myasthenia gravis (variable strabismus)

Orbital mass

■ Ptosis unequal pupils (anisocoria)

Horner syndrome (pupil smaller on affected side) Third nerve palsy (pupil usually larger on affected side)

APPROACH TO THE PATIENT

The primary factors in the evaluation of a patient with ptosis are the age of onset and the presence of associated signs and symptoms, such as strabismus or unequal pupils (Table 14–3). Ptosis that is present at birth is usually due to isolated congenital ptosis, and the need for treatment is based on severity. Acquired ptosis at any age is usually not an immediate vision problem, but important primarily due to the possible presence of an underlying serious disorder.

History

The first important point in assessing patients with ptosis is identifying the age of onset. If present at birth, it is very likely isolated congenital ptosis. Horner syndrome may also be congenital, and is accompanied by unequal pupils (smaller on the affected side). Third nerve palsies and myasthenia rarely present at birth. Third nerve palsies are associated with unequal pupils and strabismus. Most congenital myasthenia is due to the transplacental transmission of maternal antibodies, with a maternal history of myasthenia gravis.

Congenital ptosis may be isolated, familial, or associated with numerous syndromes. Questions should be asked about other affected family members and systemic problems that could indicate a specific syndrome. Birth trauma may cause ptosis due to swelling of the eyelids. This is usually temporary and resolves as the edema improves, but more severe injury (e.g., due to forceps) may cause permanent ptosis. Congenital ptosis is typically somewhat variable, worse when the child is tired. If the ptosis is moderate or severe, parents may describe a chin-up head posture or excessive elevation of the brow, which the child uses to see beneath the drooping eyelid.

Isolated acquired ptosis in older children is rare. The presence of acquired ptosis raises concern for underlying systemic disorders. Most acquired ptosis

CHAPTER 14 Droopy Eyelids ■ 91

will be associated with other signs or symptoms that help identify an etiology. Myasthenia gravis patients have variable ptosis that is worse with fatigue. They often also have strabismus and diplopia. Patients who have eyelid retraction may present with a complaint of ptosis of the opposite eye, when it is actually the retracted eye that is abnormal. Other disorders in the differential diagnosis are discussed in greater detail elsewhere (Horner syndrome—Chapter 29; third cranial nerve palsy—Chapter 34; orbital mass—Chapter 26; eyelid lesions—Chapter 25).

Examination

Children who present with ptosis at birth should be examined for signs of birth trauma or other ocular disorders. In the absence of anioscoria or strabismus to suggest congenital Horner syndrome or third nerve palsy, most patients will have isolated congenital ptosis. This can vary from mild to severe. If the eyelid is obstructing the pupil, the patient may arch their brow to recruit the forehead muscles to help lift the eyelid (Figure 14–5), or use a chin-up head posture to look beneath the eyelid.

Eyelid function is measured by holding the patient’s head straight and moving a toy or other target from below the patient to above the patient. The normal eyelid will move 12 mm or more with this maneuver. Patients with severe ptosis typically have less than 5 mm of movement. In normal patients, small fibers of the levator muscle attach to the eyelid skin to form the upper eyelid crease. Because this muscle is underdeveloped in congenital ptosis, patients often have decreased upper eyelid creases (Figure 14–1A). The muscle may be stiff in patients with severe ptosis, which may cause incomplete eyelid closure.

The ptosis associated with myasthenia gravis is variable, and worse with fatigue. A characteristic sign of myasthenia gravis is Cogan’s lid twitch. When the patient looks rapidly from down to up, there is an initial

FIGURE 14–5 ■ Compensatory brow left due to congenital ptosis, left eye. The patient is elevating the brow to help lift the eyelid above the pupil.

overshoot of the lid, which then returns to its normal position. If the patient rests for a few minutes, the eyelid can usually be opened to a greater degree. Applying an ice pack to the eyelids can result in temporary normalization of the lid function. This often cannot be performed in young children due to their aversion to the cold temperature. Variable strabismus, which can mimic cranial nerve palsies, is also often present in patients with myasthenia gravis.

The findings associated with Horner syndrome, third nerve palsy, and orbital and eyelid masses are discussed in further detail elsewhere in this book.

Patients with a complaint of droopy eyelid may have pseudoptosis. In some patients this is because they have excess brow skin that hangs over the lid (Figure 14–6A and B). When this tissue is lifted by the examiner, the underlying eyelid height and function are found to be normal. Pseudoptosis may also occur due to ocular irritation, with the patient voluntarily squinting the eyelid closed. Examination of the cornea and other ocular structures should be performed in these patients. Rarely, patients may have the appearance of ptosis on one side that is actually due to eyelid retraction in the opposite eye. If the patient fixates visually with the eye

with the retracted eyelid, the contralateral eyelid will appear to droop.

PLAN

Infants with congenital ptosis should be referred to a pediatric ophthalmologist. Early referral is indicated if the ptosis is severe (i.e., blocking the pupil) due to the risk of amblyopia.

Patients with acquired ptosis need careful evaluation to look for associated ocular and systemic disorders. Urgent additional investigations are indicated if an acquired third nerve palsy or possible orbital mass is identified (Figure 14–7).

CHAPTER 14 Droopy Eyelids ■ 93

WHAT SHOULDN’T BE MISSED

The sudden onset of ptosis in children is unusual and often associated with other problems. The list of potential diagnoses is long, including orbital and central nervous system tumors.

When to Refer

■ Infants with ptosis

Early referral if severe

Routine referral if mild or moderate

■Older children with acquired ptosis

Urgent referral if signs of cranial nerve palsy or orbital abnormality