Rapidly Developing Proptosis
Bernadette L. Koch, MD
DIFFERENTIAL DIAGNOSIS
Common
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Abscess, Subperiosteal, Orbit
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Infantile Hemangioma, Orbit
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Lymphatic Malformation, Orbit
Less Common
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Trauma, Orbit
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Idiopathic Orbital Inflammatory Disease (Pseudotumor)
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Rhabdomyosarcoma
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Metastatic Neuroblastoma
Rare but Important
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Langerhans Histiocytosis
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Lymphoproliferative Lesions, Orbit
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Traumatic Carotid-Cavernous Fistula
ESSENTIAL INFORMATION
Helpful Clues for Common Diagnoses
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Abscess, Subperiosteal, Orbit
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Key facts
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Pus between orbital wall and periosteum
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Almost always associated with sinusitis
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Anywhere in orbit, especially adjacent to ethmoid sinusitis
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Brain MR if intracranial complication of sinusitis suspected
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Imaging
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Lentiform, rim-enhancing extraconal fluid collection (phlegmon or abscess)
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± air-fluid level = drainable pus
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Usually with preseptal cellulitis and deviation of extraocular muscle (EOM)
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± edematous intraconal or extraconal fat, enlargement of EOMs (myositis)
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Infantile Hemangioma, Orbit
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Key facts
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Vascular neoplasm, NOT malformation
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Not well seen at birth; more apparent within 1st few weeks of life
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Proliferating phase: 1st year
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Involuting phase: 1-5 years
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Involuted phase: 5-7 years
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GLUT-1: Specific immunohistochemical marker expressed in all 3 phases of infantile hemangioma
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Congenital hemangioma: Rare variant, present at or before birth
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2 subtypes of congenital hemangioma = rapidly involuting (RICH) and noninvoluting (NICH)
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RICH shows involution by 8-14 months
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Imaging
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Proliferating phase: Solid, intensely enhancing mass with high-flow vessels
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Involuting phase: Fat infiltration, ↓ size
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May be part of PHACES syndrome: Posterior fossa abnormalities, hemangiomas, arterial anomalies, cardiovascular defects, eye abnormalities, sternal clefts
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Lymphatic Malformation, Orbit
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Key facts
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Congenital vascular malformation
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Lymphatic channels of varying sizes
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Present at birth, grows with child
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Hemorrhage or respiratory infection causes sudden ↑ in size with resultant rapidly developing proptosis
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Intracranial vascular anomalies present in ˜ 2/3 of patients with periorbital lymphatic malformation
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Imaging
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Uni- or multilocular; macrocystic or microcystic
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Intra- ± extraconal location
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Only septations enhance, unless mixed venolymphatic malformation
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Fluid-fluid levels best seen on MR
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Helpful Clues for Less Common Diagnoses
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Trauma, Orbit
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Key facts
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Metallic foreign bodies (FB) most dense
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Glass more dense than bone
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Wood: Very low density (˜ air density) → soft tissue density as it resorbs serum
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Living plant material ˜ soft tissue density
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Imaging
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± globe rupture, intraocular FB, intracranial injury
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Idiopathic Orbital Inflammatory Disease (Pseudotumor)
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Key facts
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Imaging
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Myositic: Most common subtype
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Diffuse enlargement and enhancement of EOMs, including tendinous insertions
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Lacrimal: Enlargement and enhancement
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Anterior: Thickening and enhancement of sclera ± choroid, ± involvement of retrobulbar fat, nerve, or sheath
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Diffuse: Intra- ± extraconal, may be mass-like, usually without globe deformity or bone erosion
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Apical: Enhancing orbital apex mass, extension through fissures
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Rhabdomyosarcoma
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Key facts
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H&N sites: Orbit, parameningeal (middle ear, paranasal sinus, nasopharynx), and all other H&N sites
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Imaging
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Soft tissue mass, variable enhancement
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± bone erosion
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± intracranial or sinus extension
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Metastatic Neuroblastoma
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Key facts
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Most common malignant tumor in children < 1 year of age
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Majority of primary tumors retroperitoneal adrenal origin
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Imaging
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Soft tissue mass + aggressive bone erosion and spiculated periosteal reaction
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When bilateral, frequently involves lateral orbital walls
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± intracranial extension
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Helpful Clues for Rare Diagnoses
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Langerhans Histiocytosis
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Key facts
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In H&N: Orbit, maxilla, mandible, temporal bone, cervical spine, skull
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Imaging
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Enhancing soft tissue mass with smooth osseous erosion
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Lymphoproliferative Lesions, Orbit
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Key facts
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Includes NHL and lymphoid hyperplasia (benign polyclonal reactive hyperplasia and indeterminate atypical hyperplasia)
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Imaging
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Diffuse or focal masses
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Homogeneous enhancing soft tissue mass anywhere in orbit
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Traumatic Carotid-Cavernous Fistula
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Key facts
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Direct fistula between cavernous ICA and cavernous sinus
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Secondary to trauma, venous thrombosis, or aneurysm
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Imaging
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Proptosis + large superior ophthalmic vein, cavernous sinus, and EOMs
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“Dirty” orbital fat related to edema
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