Orbital Neoplasms

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Orbital Neoplasms

  1. 1. Thyroid Ophthalmopathy Ellen Davis April 27, 2007
  2. 2. Thyroid Ophthalmopathy <ul><li>Inflammatory disorder of the eye </li></ul><ul><li>Commonly complicates Graves’ disease but can also be associated with Hashimoto’s thyroiditis, or in euthyroid pts </li></ul><ul><li>50% of pts with Graves’ have clinical ophthalmopathy, but up to 70% of the remaining half have ophthalmopathy by imaging </li></ul>
  3. 3. Epidemiology <ul><li>Prevalence of thyroid ophthalmopathy = 0.4% </li></ul><ul><li>Women > Men </li></ul><ul><ul><li>But severity greater in men </li></ul></ul><ul><li>Mean age of appearance = 41 yrs </li></ul><ul><li>May of exacerbated by stress, smoking, and infection with certain gram-negs </li></ul><ul><li>Most common cause of exophthalmos </li></ul><ul><ul><li>>50% of cases </li></ul></ul><ul><ul><li>Of note, other causes of exophthalmos include primary hyperadrenalism, longstanding steroid use or acromegaly. </li></ul></ul>
  4. 4. Pathogenesis <ul><li>Autoimmune process manifesting as: </li></ul><ul><ul><li>Extraocular m. myositis </li></ul></ul><ul><ul><ul><li>T-cell inflammatory infiltrate </li></ul></ul></ul><ul><ul><li>Fibroblast proliferation </li></ul></ul><ul><ul><li>Glycosaminoglycan overproduction </li></ul></ul><ul><ul><li>Orbital congestion </li></ul></ul><ul><li>Increase in soft tissue mass within bony orbit due to extraocular muscle enlargement, increased orbital fat and connective tissue </li></ul><ul><li>Later in disease, inflammatory infiltrate replaced by widespread fibrosis </li></ul><ul><ul><li>“ Inactive” phase </li></ul></ul><ul><ul><li>Occurs about 8mo to 3yrs after onset </li></ul></ul>
  5. 5. Pathogenesis <ul><li>Inflammatory cells activated by TSH receptor antigen </li></ul><ul><ul><li>TSH receptor mRNA and protein found in orbital fibroblasts and adipocytes </li></ul></ul><ul><ul><li>TSHR expression greater in retro-orbital tissues of Graves’ pt compared to other tissues. </li></ul></ul><ul><ul><li>Correlation between severity of ophthalmopathy and serum TSHR Ab concentrations </li></ul></ul>
  6. 6. Initial Signs/Symptoms <ul><li>Foreign body sensation </li></ul><ul><li>Epiphora (tearing) </li></ul><ul><li>Photophobia </li></ul><ul><li>Lid retraction (normally, should not see sclera above iris) </li></ul><ul><li>Lid lag </li></ul><ul><li>Lid, conjunctival and periorbital edema </li></ul><ul><li>Injection over horizontal muscle insertions </li></ul>
  7. 7. Exophthalmos <ul><li>Usually bilateral and symmetric </li></ul><ul><li>Pathological changes displace eye forward and can interfere with muscle actions and venous drainage. </li></ul>Note enlarged extraocular mm.
  8. 8. Histology <ul><li>Fluid and inflammatory cells separate the muscle bundles of the extraocular muscles. </li></ul>
  9. 9. Complications <ul><li>Limitation of ocular motility --> diplopia </li></ul><ul><ul><li>Inferior rectus > medial r. > superior r. </li></ul></ul><ul><li>Exposure keratopathy --> dry eye, tearing, corneal ulceration or infection </li></ul>
  10. 10. Complications <ul><li>Optic nerve compression at orbital apex by enlarged muscles </li></ul><ul><ul><li>May present with blurry vision, color loss, afferent pupillary defect, or visual field loss </li></ul></ul><ul><ul><li>More likely when superior rectus is enlarged or if no exophthalmos (form of self-decompression) </li></ul></ul>
  11. 11. Optic neuropathy <ul><li>Optic neuropathy as result of optic nerve compression from enlargement of extraocular muscles </li></ul>
  12. 12. DDx of Thyroid Ophthalmopathy <ul><li>Orbital tumors (primary or metastatic) </li></ul><ul><li>Orbital pseudotumor </li></ul><ul><li>Wegener’s granulomatosis </li></ul><ul><li>Orbital infection </li></ul><ul><li>Carotid-cavernous sinus fistula </li></ul>
  13. 13. Radiologic Evaluation <ul><li>Usually employed if cause of exophthalmos is unclear (ie. normal thyroid lab studies, or hx/PE inconsistent with thyroid disease) </li></ul><ul><li>Also to determine optic nerve involvement if not obvious by fundoscopic exam </li></ul><ul><li>Distinct sparing of muscle tendons in thyroid ophthalmopathy </li></ul>
  14. 14. Radiologic Evaluation <ul><li>Non-contrast enhanced coronal orbital CT scan most helpful to assess size of extraocular mm. </li></ul><ul><li>Symmetric bilateral exophthalmos and hypertrophy of fat. Axial muscles are mildly involved. </li></ul><ul><li>Enlargement of levator palpebrae superioris and superior rectus complex. </li></ul>
  15. 15. <ul><li>Axial CT of orbits demonstrating medial rectus enlargement </li></ul>
  16. 16. <ul><li>Medial and lateral rectus muscle enlargement with orbital apex crowding, leading to optic neuropathy </li></ul>
  17. 17. <ul><li>Axial, coronal and oblique sagittal T1-weighted orbital sections: </li></ul><ul><li>Hypertrophy of retrobulbar fat and enlargement of all four rectus muscles. Belly of inferior rectus m. moderately hyperintense due to fatty infiltration. </li></ul>
  18. 18. Treatment <ul><li>Symptomatic treatment: </li></ul><ul><ul><li>Artificial tears </li></ul></ul><ul><ul><li>Eye shades </li></ul></ul><ul><ul><li>Raise head of bed at night </li></ul></ul><ul><li>Diplopia can be managed with prism glasses </li></ul><ul><ul><li>Eventually may require strabismus surgery, most often with recession of inferior rectus to compensate for restriction. </li></ul></ul><ul><li>Conserve useful vision </li></ul><ul><ul><li>Minimize amount of exposed cornea </li></ul></ul><ul><ul><ul><li>May require lid surgery </li></ul></ul></ul><ul><ul><li>Treat optic neuropathy </li></ul></ul>
  19. 19. Antithyroid Treatment <ul><li>Randomized trial of 443 Graves’ pts with slight or no ophthalmopathy </li></ul><ul><ul><li>Radioiodine alone (150 pts) </li></ul></ul><ul><ul><ul><ul><li>15% developed or had worsening ophthalmopathy </li></ul></ul></ul></ul><ul><ul><li>Radioiodine + prednisone (135 pts) </li></ul></ul><ul><ul><ul><ul><li>None developed or had worsening of eye disease </li></ul></ul></ul></ul><ul><ul><li>Radioiodine + Methimazole (148 pts) </li></ul></ul><ul><ul><ul><ul><li>2% of pts with ophthalmopathy improved </li></ul></ul></ul></ul><ul><ul><ul><ul><li>3% worsened </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Remaining had no change </li></ul></ul></ul></ul>
  20. 20. Antithyroid Treatment <ul><li>Radioiodine therapy can increase risk of development or worsening of Graves’ ophthalmopathy </li></ul><ul><ul><li>Worsening may be prevented by steroids </li></ul></ul><ul><ul><li>No change with concurrent methimazole </li></ul></ul>
  21. 21. Radioiodine Therapy <ul><li>Probably best to avoid radioiodine in pts with moderate/severe ophthalmopathy, or delay until ophthalmopathy stable </li></ul><ul><li>Use in caution in pts with other risk factors for ophthalmopathy </li></ul>
  22. 22. Thyroidectomy <ul><li>Near total thyroidectomy may be associated with less progression of proptosis compared to subtotal surgery </li></ul><ul><li>Radioiodine therapy after near total thyroidectomy --> less active ophthalmopathy compared to pts with thyroidectomy alone. </li></ul>
  23. 23. Treatment: Immunosuppression <ul><li>Steroids controversial </li></ul><ul><ul><ul><li>Immediate benefit decays with time </li></ul></ul></ul><ul><ul><ul><li>Usually reserved for pts with optic neuropathy </li></ul></ul></ul><ul><li>Azathioprine or cyclophosphamide </li></ul><ul><ul><li>May have role in combo with steroids, but are less beneficial alone </li></ul></ul><ul><ul><li>Cyclosporine esp when steroids are tapered or stopped. </li></ul></ul>
  24. 24. Orbital Radiation <ul><li>Kills retroorbital inflammatory cells </li></ul><ul><li>Effectivity controversial </li></ul><ul><ul><li>May have role in pts who cannot tolerate steroids </li></ul></ul><ul><ul><li>May be more effective if combined with steroids </li></ul></ul><ul><ul><li>One randomized trial showed improved eye motility and diplopia, but no prevention of worsening of ophthalmopathy. </li></ul></ul>
  25. 25. Orbital Decompression Surgery <ul><li>Restore normal orbital anatomy </li></ul><ul><li>Indications: </li></ul><ul><ul><li>Progression despite steroids or radiation </li></ul></ul><ul><ul><li>Vision threatened by corneal or optic nerve compromise </li></ul></ul><ul><ul><li>Severe proptosis --> cosmetic improvement </li></ul></ul>
  26. 26. Orbital Decompression <ul><li>Transantral decompression </li></ul><ul><ul><li>Involves removal of floor and medial wall of orbit </li></ul></ul><ul><ul><li>No facial scarring, no craniotomy </li></ul></ul><ul><li>Usually improvement in proptosis and edema </li></ul><ul><li>May not improve diplopia </li></ul><ul><ul><li>Strabismus surgery usually needed </li></ul></ul>
  27. 27. <ul><li>CT guided orbital decompression shows inferomedial periorbita incision with extensive fat decompression into sinus cavity </li></ul>
  28. 28. <ul><li>Transantral decompression. </li></ul>
  29. 29. The End
  30. 30. References <ul><li>Yanoff: Ophthalmology, 2nd ed., Copyright 2004 Mosby, Inc. Chapter 201: Ocular myopathies. </li></ul><ul><li>UptoDate: Graves’ orbitopathy: Diagnosis and Treatment. </li></ul><ul><li>Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed., Copyright 2001. Chapter 106: Inflammatory disease of the orbit. </li></ul><ul><li>Relation between therapy for hyperthyroidism and the course of Graves' ophthalmopathy. Bartalena L; Marcocci C; et al. NEJM 1998 Jan 8;338(2):73-8. </li></ul><ul><li>Total thyroidectomy for the treatment of hyperthyroidism in patients with ophthalmopathy. Kurihara H. Thyroid 2002 Mar;12(3):265-7. </li></ul><ul><li>El-Kaissi S. Frauman AG. Wall JR. Thyroid-associated ophthalmopathy: a practical guide to classification, natural history and management. Internal Medicine Journal. 34(8):482-91, 2004 Aug. </li></ul><ul><li>Selected images from: </li></ul><ul><ul><li>Cummings: Otolaryngology: Head & Neck Surgery, 4th ed., Copyright ゥ 2005 Mosby, Inc. </li></ul></ul><ul><ul><li>Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed., Copyright ゥ 2005 </li></ul></ul>

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