• Orbital cellulitis from adjacent ethmoiditis: Most common cause of proptosis in children. It is of paramount importance to quickly rule out this etiology. See 7.3.1, ORBITAL CELLULITIS.
• Dermoid and epidermoid cysts: Manifest clinically from birth to young adulthood and enlarge slowly. Preseptal dermoid cysts may become symptomatic in childhood and are most commonly found in the temporal upper eyelid or brow, and less often in the medial upper eyelid. The palpable, smooth mass may be mobile or fixed to the periosteum. Posterior dermoids typically become symptomatic in adulthood and may cause proptosis or globe displacement. Dermoid cyst rupture may mimic orbital cellulitis. The B-scan US, when used, reveals a cystic lesion with good transmission of echoes. Because of the cystic configuration and specific signal properties, CT and MRI are usually diagnostic. See 14.3, MAGNETIC RESONANCE IMAGING.
• Hemangioma of infancy (capillary hemangioma): Seen from birth to 2 years, generally show slow progressive growth over the first 6 to 9 months with slow involution thereafter. May be observed through the eyelid as a bluish mass or be accompanied by a red hemangioma of the skin (strawberry nevus, stork bite), which blanches with pressure (see Figure 7.4.1.1). Proptosis may be exacerbated by crying. It can enlarge over 6 to 12 months, but spontaneously regresses over the following several years. Not to be confused with the unrelated cavernous venous malformation (cavernous hemangioma) of the orbit, typically seen in adults.
• Rhabdomyosarcoma: Average age of presentation is 8 to 10 years, but may occur from infancy to adulthood. May present with explosive proptosis, edema of the eyelids, a palpable eyelid lesion or subconjunctival mass, new-onset ptosis or strabismus, or a history of nosebleeds. Hallmarks are rapid onset and progression. Pain may occur in a minority of cases. Urgent biopsy and referral to a pediatric oncologist is warranted if suspected.
• Metastatic neuroblastoma: Seen during the first few years of life (usually by the age of 5 years). Abrupt presentation with unilateral or bilateral proptosis, eyelid ecchymosis, and globe displacement. The child is usually systemically ill, and 80% to 90% of patients presenting with orbital involvement already have a known history of neuroblastoma. Note that metastatic neuroblastoma may also present as an isolated Horner syndrome in a child due to metastasis to the lung apex. Prognosis decreases with age.
• Lymphangioma: Usually seen in the first 2 decades of life with a slowly progressive course but may abruptly worsen if the tumor spontaneously bleeds. Proptosis may be intermittent and exacerbated by upper respiratory tract infections. Lymphangioma may present as an atraumatic eyelid ecchymosis. Concomitant conjunctival, eyelid, or oropharyngeal lymphangiomas may be noted (a conjunctival lesion appears as a multicystic mass). MRI is often diagnostic. The B-scan US, when used, often reveals cystic spaces. See Figure 7.4.1.2.
• Optic nerve glioma (juvenile pilocytic astrocytoma): Usually first seen at the age of 2 to 6 years and is slowly progressive. The presentation includes painless axial proptosis with decreased visual acuity and a relative afferent pupillary defect. Optic nerve atrophy or swelling may be present. May be associated with neurofibromatosis (types I and II), in which case it may be bilateral. Prognosis decreases with chiasmal or hypothalamic involvement. See 13.13, PHAKOMATOSES.
• Plexiform neurofibroma: Seen in the first decade of life and is pathognomonic for neurofibromatosis type I. Ptosis, eyelid hypertrophy, S-shaped deformity of the upper eyelid, or pulsating proptosis (from the absence of the greater sphenoid wing) may be present. Facial asymmetry and a palpable anterior orbital mass may also be evident. See 13.13, PHAKOMATOSES.
• Leukemia (granulocytic sarcoma): Seen in the first decade of life with rapidly evolving unilateral or bilateral proptosis and, occasionally, swelling of the temporal fossa area due to a mass. Typically, granulocytic sarcoma precedes blood or bone marrow signs of leukemia (usually acute myelogenous leukemia) by several months. Any patient with a biopsy-proven granulocytic sarcoma of the orbit must be closely followed by an oncologist for leukemia development. Acute lymphoblastic leukemia can also produce unilateral or bilateral proptosis.
• Langerhans cell histiocytosis (LCH): May present in the orbit as a rapidly progressive mass with bony erosion on imaging. Three variants are encountered in the orbit: (1) multifocal, multisystem LCH (Letterer–Siwe disease) occurs in children <2 years old with an aggressive multisystem course and poor prognosis; (2) multifocal unisystem LCH (Hand–Schüller–Christian disease) occurs in children 2 to 10 years of age. The classic triad includes exophthalmos, lytic bone lesions, and diabetes insipidus from pituitary stalk infiltration; (3) unifocal LCH (eosinophilic granuloma) typically causes bony erosion in the superolateral orbit suggestive of malignancy. Occurs in older children and adults. Systemic progression occurs in only a minority of cases.

7-4.1.2 T-2-weighted MRI of orbital lymphangioma with subacute blood cyst.

7-4.1.1 Hemangioma of infancy.

Workup

1. History: Determine the age of onset and the rate of progression. Does the proptosis vary (e.g., with crying or position)? Nosebleeds? Systemic illness? Fever? Weight loss? Recent URI? Purulent nasal discharge?
2. External examination: Look for an anterior orbital mass, a skin hemangioma, or a temporal fossa lesion. Measure any proptosis (Hertel exophthalmometer) or globe displacement. Refer to a pediatrician for abdominal examination to rule out mass or organomegaly.
3. Complete ocular examination, including visual acuity, pupillary assessment, color vision, IOP, refraction, and optic nerve evaluation. Check the conjunctival cul-de-sacs carefully.
4. Urgent imaging with either CT (axial, coronal, and parasagittal views) or MRI (with gadolinium-DTPA and fat suppression) of brain and orbits to rule out infection or neoplasia.
5. If paranasal sinus opacification is noted in the clinical setting of orbital inflammation, consider immediate systemic antibiotic therapy (see 7.3.1, ORBITAL CELLULITIS).
6. In cases of acute onset and rapid progression with evidence of mass on imaging, an emergency incisional biopsy for frozen and permanent microscopic evaluation is indicated to rule out an aggressive malignancy (e.g., rhabdomyosarcoma).
7. Other tests as determined by the working diagnosis (usually performed in conjunction with a pediatric oncologist):
   • Rhabdomyosarcoma: Physical examination (look especially for enlarged lymph nodes), chest and bone radiographs, bone marrow aspiration, lumbar puncture, and liver function studies.
   • Leukemia: CBC with differential, bone marrow studies, etc.
   • Neuroblastoma: Abdominal imaging (e.g., CT or MRI), urine for vanillylmandelic acid, radioiodinated metaiodobenzylguanidine scintigraphy.
   • LCH: CBC with differential, comprehensive metabolic panel, serum osmolarity, and skeletal survey.

1. Dermoid and epidermoid cysts: Complete surgical excision with the capsule intact. If the cyst ruptures, the contents can incite an acute inflammatory response.
2. Hemangioma of infancy: Observe if not causing visual obstruction, astigmatism, and amblyopia. All hemangiomas of infancy will eventually involute. In the presence of visual compromise (e.g., amblyopia and optic neuropathy), several treatment options exist:
   1. Systemic ß-blockers: While the exact mechanisms remain unclear, propranolol has become the preferred option in the treatment of refractory and rapidly proliferating infantile hemangiomas. Side effects of propranolol include hypoglycemia, hypotension, and bradycardia. Asthmatics and those with reactive airway disease are at risk for bronchospasm. Therefore, patients should be evaluated by a pediatrician pretreatment and monitored throughout the course of treatment. The initial dose of ß-blocker is typically given in conjunction with cardiopulmonary monitoring. Note that not all lesions respond to this therapy.
   2. Oral corticosteroids: Used less frequently since the introduction of ß-blockers. The dose is 2 to 3 mg/kg, tapered over 6 weeks. IOP must be monitored, and patients should be placed on GI prophylaxis.
   3. A local corticosteroid injection (e.g., betamethasone 6 mg/mL and triamcinolone 40 mg/mL) is seldom used. Care should be taken to avoid orbital hemorrhage and central retinal artery occlusion during injection. Skin atrophy and depigmentation are other potential complications. Note that periocular injection of triamcinolone is contraindicated by the manufacturer because of the potential risk of embolic infarction. See note in 6.2, CHALAZION/HORDEOLUM.
   4. Surgical excision: If the hemangioma is circumscribed and accessible, excision can be performed effectively and is often curative.
   5. Interferon therapy: Usually reserved for large or systemic lesions that may be associated with a consumptive coagulopathy or high-output congestive heart failure (Kasabach–Merritt syndrome). There is a risk of spastic diplegia with this therapy. No longer used except in rare cases because of other viable alternatives, including propranolol.
3. Rhabdomyosarcoma: Managed by urgent biopsy and referral to a pediatric oncologist in most cases. Local radiation therapy and systemic chemotherapy are given once the diagnosis is confirmed by biopsy and the patient has been appropriately staged. Significant orbital and ocular complications can occur even with prompt and aggressive management. Overall, the long-term prognosis for orbital rhabdomyosarcoma has greatly improved over the past 50 years due to advances in chemotherapy and radiotherapy, and exenteration is no longer the standard of care. Prognosis depends on the subtype of rhabdomyosarcoma, location of the lesion (orbital lesions have the best prognosis), and stage of the disease. Note that prognosis for orbital lesions decreases with spread to adjacent anatomy (paranasal sinuses or intracranial vault).
4. Lymphangioma: Most are managed by observation. Surgical debulking is performed for a significant cosmetic deformity, ocular dysfunction (e.g., strabismus and amblyopia), or compressive optic neuropathy from acute orbital hemorrhage, but may be difficult because of the infiltrative nature of the tumor. Incidence of hemorrhage into the lesion is increased after surgery. May recur after excision. Aspiration drainage of hemorrhagic cysts (“chocolate cysts”) may temporarily improve symptoms. Sclerosing therapy has become the most frequent management option for large, cystic lesions.
5. Optic nerve glioma: Controversial. Observation, surgery, radiation, and/or chemotherapy are used variably on a case-by-case basis.
6. Leukemia: Managed by a pediatric oncologist. Systemic chemotherapy for leukemia. Some physicians administer orbital radiation therapy alone in isolated orbital lesions (chloromas, granulocytic sarcomas) when systemic leukemia cannot be confirmed on bone marrow studies. However, patients need to be monitored closely for eventual systemic involvement.
7. Metastatic neuroblastoma: Managed by a pediatric oncologist in most cases. Local radiation and systemic chemotherapy.
8. Plexiform neurofibroma: Surgical excision is reserved for patients with significant symptoms or disfigurement. The lesions tend to be vascular, infiltrative, and recurrent.
9. LCH: Therapy depends on the extent of the disease. Multisystem involvement requires chemotherapy. With unifocal involvement (eosinophilic granuloma in adults), debulking and curettage are usually curative.

1. Tumors with rapid onset and progression require urgent attention, with appropriate and timely referral to a pediatric oncologist when necessary.
2. Tumors that progress more slowly may be managed less urgently.