Figure 18-10 Fetal alcohol syndrome. Asymmetric ptosis; telecanthus; strabismus; long, flat philtrum (arrow); anteverted nostrils. This child also had Peters anomaly, left eye, and myopia, right eye. (Reproduced with permission from Miller MT, Israel J,
Cuttone J. Fetal alcohol syndrome. J Pediatr Ophthalmol Strabismus. 1981;18(4):6–15.)
Nonsynostotic disorders of bone growth
These conditions involve overgrowth of bone, thereby creating a risk of stenosis of the optic canal and resultant compressive optic neuropathy.
Infantile osteopetrosis This rare and severe form of osteopetrosis is associated with optic canal stenosis. Bone marrow transplant has been reported to reverse the stenosis. The condition is autosomal recessive.
Craniometaphyseal dysplasia Craniometaphyseal dysplasia is a rare disorder of osteoclast resorption that causes hyperostosis of the cranial bones. The typical facial appearance includes frontal and paranasal bossing. Progressive sclerosis of cranial nerve foramina can result in compressive optic neuropathy.
Infectious and Inflammatory Conditions
Preseptal and orbital cellulitis are usually more rapidly progressive and more severe in children than in adults. See also BCSC Section 7, Orbit, Eyelids, and Lacrimal System.
Preseptal Cellulitis
Preseptal cellulitis, a common infection in children, is an inflammatory process involving the tissues anterior to the orbital septum. Eyelid edema may extend into the eyebrow and forehead. The periorbital skin becomes taut and inflamed, and edema of the contralateral eyelids may appear. Proptosis is not a feature of preseptal cellulitis, and the globe remains noninflamed. Full ocular motility and absence of pain on eye movement help distinguish preseptal from orbital cellulitis.
Preseptal cellulitis typically develops from 1 of 3 causes. Posttraumatic cellulitis may develop following puncture, insect bite, or laceration of the eyelid skin. In these cases, organisms found on the skin, such as Staphylococcus or Streptococcus species, are most commonly responsible for the infection.
The second cause of preseptal cellulitis is severe conjunctivitis such as epidemic keratoconjunctivitis or methicillin-resistant Staphylococcus aureus (MRSA) conjunctivitis, or skin infection such as impetigo or herpes zoster.
The third mechanism for preseptal cellulitis is secondary to upper respiratory tract or sinus infection. Streptococcus pneumoniae and other streptococcal species, and S aureus are the most common causative organisms.
Children with nonsevere preseptal infections who are not systemically ill can be treated with oral antibiotics as outpatients. Broad-spectrum drugs effective against the most common pathogens, such as cephalosporins or ampicillin-clavulanic acid combination, are usually effective. Particularly with eyelid abscesses, clindamycin may be an appropriate choice because of the increasing prevalence of MRSA, which should also be considered in patients who do not improve with treatment. Eyelid abscesses may require urgent incision and drainage.
If the child is younger than 1 year or has signs of systemic illness such as sepsis or meningeal involvement, hospitalization—for appropriate cultures, imaging of the sinuses and orbits, and
intravenous (IV) antibiotics—is appropriate. In newborns, dacryocystocele should be considered in the differential diagnosis (see Chapter 19).
Orbital Cellulitis
Orbital cellulitis is an infection of the orbit that involves the tissues posterior to the orbital septum. Orbital cellulitis is most commonly associated with ethmoid or frontal sinusitis. It can also occur following penetrating injuries of the orbit.
The etiologic agents most often responsible for orbital cellulitis vary with age. In general, children younger than 9 years have infections caused by a single aerobic pathogen. Children older than 9 years may have complex infections with multiple pathogens, both aerobic and anaerobic. S aureus and gram-negative bacilli are most common in the neonate. In older children and adults, S aureus, Streptococcus pyogenes, S pneumoniae, and various anaerobic species are common. Gram-negative organisms are found primarily in immuno-suppressed patients.
Early signs and symptoms of orbital cellulitis include lethargy, fever, eyelid edema, rhinorrhea, headache, orbital pain, and tenderness on palpation. The nasal mucosa becomes hyperemic, with a purulent nasal discharge. Increased venous congestion may cause elevated intraocular pressure. Proptosis, chemosis, and limited ocular movement suggest orbital involvement.
The differential diagnosis of orbital cellulitis includes nonspecific orbital inflammation, benign orbital tumors such as lymphatic malformation and hemangioma, and malignant tumors such as rhabdomyosarcoma, leukemia, and metastases.
Paranasal sinusitis is the most common cause of bacterial orbital cellulitis (Fig 18-11). In children younger than 10 years, the ethmoid sinuses are most frequently involved. If orbital cellulitis is suspected, orbital imaging is indicated to confirm orbital involvement, to document the presence and extent of sinusitis and subperiosteal abscess (Fig 18-12), and to rule out a foreign body in a patient with a history of trauma.
Figure 18-11 Bacterial orbital cellulitis with proptosis (A) secondary to sinusitis (B). (Courtesy of Jane Edmond, MD.)
Figure 18-12 Axial computed tomography (CT) image showing a medial subperiosteal abscess of the left orbit associated
with ethmoid sinusitis. (Courtesy of Jane Edmond, MD.)
It is crucial to distinguish orbital cellulitis from preseptal cellulitis because the former requires hospitalization and treatment with IV broad-spectrum antibiotics. Choice of IV antibiotic is based on the most likely pathogens until results from cultures are known. If associated sinusitis or subperiosteal abscess is present, pediatric otolaryngologists should be consulted. The patient should be observed closely for signs of visual compromise. Many subperiosteal abscesses in children younger than 9 years will resolve with medical management. Emergency drainage of a subperiosteal abscess is indicated for a patient of any age with either of the following:
evidence of optic nerve compromise (decreasing vision, relative afferent pupillary defect) and an enlarging subperiosteal abscess
an abscess that does not resolve within 48–72 hours of administration of antibiotics
Intraconal orbital abscesses are much less common than subperiosteal abscesses in children and require urgent surgical drainage.
Complications of orbital cellulitis include cavernous sinus thrombosis and intracranial extension (subdural or brain abscesses, meningitis, periosteal abscess), which may result in death. Cavernous sinus thrombosis can be difficult to distinguish from simple orbital cellulitis. Paralysis of eye movement in cavernous sinus thrombosis is often out of proportion to the degree of proptosis. Pain on motion and tenderness on palpation are absent. Decreased sensation along the maxillary division of cranial nerve V (trigeminal) supports the diagnosis. Bilateral involvement is virtually diagnostic of cavernous sinus thrombosis.
Other complications of orbital cellulitis include corneal exposure with secondary ulcerative keratitis, neurotrophic keratitis, secondary glaucoma, septic uveitis or retinitis, exudative retinal detachment, optic nerve edema, inflammatory neuritis, infectious neuritis, central retinal artery occlusion, and panophthalmitis.
Related conditions
Maxillary osteomyelitis is a rare condition of early infancy. Infection spreads from the nose into the tooth buds, with unilateral erythema and edema of the eyelids, cheek, and nose. Infection may spread and cause orbital cellulitis.
Fungal orbital cellulitis (mucormycosis) occurs most frequently in patients with ketoacidosis or severe immunosuppression. The infection causes thrombosing vasculitis with ischemic necrosis of involved tissue (Fig 18-13). Cranial nerves often are involved, and extension into the central nervous system is common. Smears and biopsy of the involved tissues reveal the fungal organisms. Treatment includes debridement and administration of amphotericin. Allergic fungal sinusitis is a less fulminant condition that frequently presents with orbital signs, including proptosis from remodeling of the bony orbit. See BCSC Section 7, Orbit, Eyelids, and Lacrimal System, for further discussion.
Figure 18-13 Mucormycosis, left orbit.
Childhood Orbital Inflammation
Several noninfectious, nontraumatic disorders cause orbital inflammation in children that may simulate infection or an orbital mass lesion; they deserve brief mention. Thyroid eye disease, the
most common cause of proptosis in adults, rarely occurs in prepubescent children but occasionally affects adolescents (Fig 18-14). Bilateral orbital inflammation may occur with sarcoidosis.
Figure 18-14 Thyroid eye disease with bilateral exophthalmos in a 15-year-old girl.
Nonspecific orbital inflammation
Nonspecific orbital inflammation (NSOI) (orbital pseudotumor; idiopathic orbital inflammatory syndrome) is an inflammatory cause of proptosis in childhood that differs significantly from the adult form. The typical pediatric presentation is acute and painful, more closely resembling orbital cellulitis than tumor or thyroid eye disease (Fig 18-15). NSOI is often bilateral and may be associated with systemic manifestations such as headache, nausea, vomiting, and lethargy. Uveitis is frequently present and occasionally constitutes the dominant manifestation. Imaging studies may show increased density of orbital fat, thickening of posterior sclera and the Tenon layer, or enlargement of extraocular muscles. The lacrimal gland is often involved. Sinusitis is typically not present. Treatment with a systemic corticosteroid usually provides prompt and dramatic relief, but recurrent disease is common. A slow tapering of corticosteroid dosage is usually required to prevent recurrence.
Belanger C, Zhang KS, Reddy AK, Yen MT, Yen KG. Inflammatory disorders of the orbit in childhood: a case series. Am J Ophthalmol. 2010;150(4):460–463.