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

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