Rapidly Developing Proptosis

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
      • FB, bone fragments, &/or adjacent hematoma image proptosis
      • ± globe rupture, intraocular FB, intracranial injury
  • Idiopathic Orbital Inflammatory Disease (Pseudotumor)
    • Key facts
      • Benign inflammatory process with fibrosis image painful proptosis
    • 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
Aug 10, 2016 | Posted by in PEDIATRICS | Comments Off on Rapidly Developing Proptosis

Full access? Get Clinical Tree

Get Clinical Tree app for offline access