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Children with new-onset proptosis should be referred promptly
to a pediatric ophthalmologist. The differential diagnosis includes
several life- and vision-threatening disorders.
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What Shouldn’t
Be Missed
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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.
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- 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 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 ...