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