Bulging Eyeball

The Problem

“My child’s eye is bulging.”

Common Causes

Orbital tumors

Orbital cellulitis

Orbital lymphangioma

Orbital pseudotumor

Infantile glaucoma

Other Causes

High myopia

Craniofacial malformations

Thyroid opthalmopathy

Capillary hemangioma

KEY FINDINGS

History

Diplopia may occur with any of these lesions Decreased vision more common with rapidly

growing lesions Orbital tumors

Rhabdomyosarcoma—rapid painless proptosis Optic nerve glioma—may present rapidly

More common in patients with neurofibromatosis Other tumors—gradual proptosis

Orbital cellulitis

Pain, fever, systemic illness Rapid onset

History of sinus disease

Lymphangioma

Rapid-onset proptosis if acute bleeding

Orbital pseudotumor

Pain, worse with eye movement

Often systemic symptoms (fever, malaise)

Infantile glaucoma

Excess tearing

Light sensitivity (photophobia)

Examination

All lesions with proptosis may have limited eye movements, decreased vision, and conjunctival swelling

Orbital tumor

Often nontender proptosis Orbital cellulitis

Periocular erythema and edema Abnormal pupil reactions

Lymphangioma

Usually subtle proptosis unless acute hemorrhage Acute hemorrhage may produce marked proptosis

and swelling Orbital pseudotumor

Pain with eye movement Inflammation over extraocular muscles

Infantile glaucoma

Cornea enlarged, may be cloudy Overflow tearing

WHAT SHOULD YOU DO?

Children with new-onset proptosis should be referred promptly to a pediatric ophthalmologist. The differential diagnosis includes several lifeand vision-threatening disorders.

What Shouldn’t Be Missed

Proptosis is a serious condition that requires prompt evaluation and treatment. In particular, patients with decreased vision or signs of orbital cellulitis should be referred immediately.

FIGURE 15–1 ■ Rhabdomyosarcoma, left orbit. The left eye is bulging forward. Note stretched appearance of upper and lower eyelids, and decreased left lower eyelid skin crease.

COMMON CAUSES

1.Orbital tumors. The most common primary orbital tumor in children is rhabdomyosarcoma, which classically presents with rapid onset of painless proptosis (Figure 15–1). Optic pathway gliomas affecting the optic nerve sometimes present with rapid onset of proptosis due to mucinous degeneration. Metastatic lesions, including neuroblastoma, leukemia, and lymphoma, are less common.

2.Orbital cellulitis. Orbital cellulitis is a serious infection that most commonly results from contiguous spread of sinus disease (Figure 15–2). Prompt treatment with intravenous antibiotics is indicated. Orbital cellulitis is frequently associated with subperiosteal orbital abscesses, which may improve with antibiotics and not require surgical drainage.

3.Lymphangioma. Lymphangiomas are congenital lesions that may not be noticed initially. These lesions are prone to internal hemorrhage, which presents with the rapid onset of proptosis (Figure 15–3). This may be difficult to distinguish from an orbital tumor without a biopsy.

4.Orbital pseudotumor. Orbital pseudotumor is an idiopathic condition characterized by inflammation of the orbital tissue. It is often

FIGURE 15–2 ■ Left orbital cellulitis. Note edema and erythema of left periocular skin.

FIGURE 15–3 ■ Proptosis secondary to left orbital lymphangioma with acute hemorrhage. The bulging eye is often more easily noted when viewed from above.

preceded by a systemic febrile illness, and presents with marked periocular pain. It may be localized to the extraocular muscles (myositis). It characteristically responds very promptly to systemic corticosteroid treatment.

5.Infantile glaucoma. Although not an orbital disorder, glaucoma that presents in infancy or early childhood may cause enlargement of the eyeball, with a clinical appearance similar to proptosis (Figure 15–4). Affected children often have cloudy corneas and excess tearing due to corneal irritation.

6.Other causes. Apparent proptosis may result from underlying abnormalities of the orbit or eyeball itself. Patients with craniofacial abnormalities or craniosynostosis may have shallow orbits, and patients who are markedly nearsighted (myopic) may have elongated eyes. Orbital hemorrhage due to trauma or bleeding disorders may also cause proptosis (Figure 15–5A and B).

APPROACH TO THE PATIENT

Proptosis in children is an unusual problem that requires urgent evaluation. The signs and symptoms are usually readily apparent (Table 15–1). The differential diagnosis

FIGURE 15–4 ■ Infantile glaucoma, right eye. The eyeball and cornea are much larger on the right, which gives an appearance similar to proptosis.

96 ■ Section 2: Symptoms

A

B

FIGURE 15–5 ■ Orbital hemorrhage, left eye. (A) Left eye is bulging forward and displaced down. (B) Magnetic resonance image of left superior orbital hemorrhage. (Figure A and B are reprinted with permission from SLACK Incorporated: Bart DJ, Lueder GT. Orbital hemorrhage following extracorporeal membrane oxygenation in a newborn. J Pediatr Ophthalmol Strabismus. 1997;34(1):65–67.)

Table 15–2.

Systemic Diseases Associated With Proptosis

■Orbital or optic nerve tumors

Primary orbital tumors

–Rhabdomyosarcoma

Metastatic orbital tumors

–Leukemia

–Neuroblastoma

Optic nerve tumors

–Optic glioma (neurofibromatosis)

■Thyroid dysfunction (Graves disease)

■Craniofacial malformations

history of sinus disease. Trauma, particularly if associated with an orbital foreign body, may also cause orbital cellulitis. Children with orbital pseudotumor often have a history of preceding systemic illness, including fever and malaise. Bulging eyeballs due to glaucoma usually occur in infancy, and are accompanied by symptoms of excess tearing and light sensitivity.

for orbital lesions is large, with many rare disorders (Table 15–2). However, a few entities account for the majority of cases.

History

The primary historical considerations are the rapidity of onset and the association of inflammatory signs. Larger lesions may obstruct eye movements and produce diplopia. Lesions that compress the optic nerve may cause decreased vision and abnormal pupil reactions. Although uncommon in children, thyroid ophthalmopathy may occur, and a review of systems for symptoms of hypoor hyperthyroidism should be included. The orbit may be a site of metastatic disease, which would be a likely etiology for proptosis in patients with a known history of leukemia, lymphoma, or neuroblastoma. Most patients with orbital cellulitis have a

Table 15–1.

Symptoms of Orbital Mass

■Proptosis

■Limited eye movements (diplopia)

■Ocular irritation (due to corneal exposure)

■Decreased vision

Examination

The initial examination of patients with proptosis should focus on the vision and whether there are signs of infection. The visual acuity and pupil reactions should be assessed. Examination of extraocular movements may reveal limitation. The presence of periocular erythema and edema should be noted. Patients with rapid proptosis may have conjunctival swelling or corneal irritation due to exposure. Myositis may produce localized inflammation over an extraocular muscle. Infantile glaucoma that causes enlargement of the eye usually also causes clouding of the cornea.

PLAN

All patients with new-onset proptosis require referral for further evaluation(Figure 15–6). The main initial decision is how soon this needs to be performed. Patients with signs and symptoms of orbital cellulitis and patients whose vision is decreased should be referred immediately, either directly to the referring physician’s office or through an emergency room. Other patients with proptosis should be seen promptly.

WHAT SHOULDN’T BE MISSED

Rapid treatment of orbital cellulitis is indicated in all patients, but particularly in those who are immunocompromised, due to the risk of cavernous sinus

Craniofacial

abnormality

Treat underlying disorder

thrombosis or spread of infection to the central nervous system. Patients with proptosis and decreased vision may have optic nerve compression, which requires immediate treatment.

When to Refer

■Patients with acquired proptosis should be referred promptly for evaluation