Fall 2017 Newsletter

Follow our latest updates in our Fall 2017 Newsletter

Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: What’s Popping?

Ho Zhang-Nunes
Presenter: Tiffany Ho, MD Discussant: Sandy Zhang-Nunes, MD
 

History

  • 65-year-old Caucasian male with hyperthyroidism presents with:
    • Decreased vision of right eye x 2-3 weeks
    • Bilateral eye redness, swelling, and soreness x two months
  • Systemic medications: methimazole, atenolol, failed course of PO prednisone
  • Family history: Grave’s disease (mother and brother)
  • Social history: remote history of smoking

Exam Findings

  • BCVA (cc): 20/100 OD; 20/25 OS
  • IOP: 26, 16 mm Hg
  • Pupils: Round, reactive, no rAPD
  • Extraocular motility
    • Right: -1 adduction, -2 abduction, -2 supraduction, full infraduction
    • Left: full
  • Brightness sense: equal
  • Ishihara Color Plates: 1/14 OU
Figure 1: External photo showing bilateral upper and lower eyelid edema with erythema (right > left), bilateral lateral flare, superior and inferior scleral show, bilateral conjunctival injection/chemosis, right caruncle hyperemia.
Figure 2: Proptosis OD. Hertel (oculus): 25 mm < 123 mm > 21 mm
Figure 3: Lid lag on down gaze (Von Grafe’s sign).
Figure 4: Slit lamp examination of right eye. Diffuse injection and chemosis, caruncle edema, diffuse PEEs, +1 NSC (similar findings in left eye).
Figure 5: Fundus photo of right eye. Optic disc edema with obscuration of disc margin and fine vessels on disc. Superior disc hemorrhage. More tortuous vessels. Chorioretinal folds in macula.
Figure 6: Fundus photo of left eye. Disc sharp and pink, macula flat, vessels/periphery within normal limits.

 

Differential Diagnosis of Proptosis Causing Orbital Apex Syndrome

  • Vascular: Carotid-cavernous sinus fistula, cavernous sinus thrombosis, cavernous hemangioma
  • Inflammatory: Idiopathic orbital inflammatory syndrome, IgG4 related sclerosing disease
  • Tumors: optic gliomas, meningiomas, lymphoma, other malignancy
  • Endocrine: thyroid associated orbitopathy
  • Infections

Additional Investigations

Figure 7: CT orbits. (Left) Coronal view showing enlarged superior, medial, and inferior muscles with apical crowding. (Right) Axial view showing proptosis (R>L), enlarged posterior muscle belly with sparing of anterior muscle tendon.
Figure 8: Reliable HVF 30-2. Right eye shows generalized depression. Left eye shows inferior arcuate deficit.

 

Diagnosis

  • Thyroid Eye Disease with Compressive Optic Neuropathy

Pathophysiology of Thyroid Eye Disease

  • Immune-mediated disorder causing inflammation, enlargement, scaring of orbital fat and muscle
  • Associated with thyroid dysfunction (90% hyperthyroid, 3% hypothyroid, 6% euthyroid)
  • Abnormal lymphocytes produce thyroid stimulating receptor antibodies (TRAB)
  • Orbital fibroblasts (target cell) stimulated to secrete inflammatory cytokines, deposit hyaluronan, stimulate adipogenesis

Natural History

  • Self-limiting disease that follows a biphasic course in which patients move from active to quiescent phase
  • The disease will usually stabilize after an active period of 18 months that is followed by quiescent/inactive phase
  • Rundle’s curve plots clinical severity against time and reflects disease course
  • Treatment targeted to active phase

Clinical Assessment

  • Disease severity: Grading of spectrum of clinical features
    • Mild
      • Lid swelling, retraction, congestion
    • Moderate
      • Double Vision, eye muscle congestion, significant proptosis
    • Severe
      • Optic nerve compression, excessive exposure keratopathy
  • Disease activity: Where patient resides within disease course
  • Clinical Activity Score (CAS): developed based on patients with periocular inflammatory changes who will benefit from immune suppression (>4)
  • Erythema (2)
    • Eyelids, conjunctiva
  • Edema (3)
    • Conjunctiva (chemosis), caruncle, eyelids
  • Pain (2)
    • Fullness behind the globe, with extraocular movements
  • Orbital findings (3)
    • Proptosis (> or equal to 2mm between 1-3 months)
    • Visual Acuity (Decrease > or equal to 1 Snellen line)
    • Extraocular movements (Decrease 5 degrees in three months)

 

Prognosis

  • Clinical features of dysthyroid optic neuropathy. First three are most useful:
    • Impaired color vision
    • Optic disc swelling/atrophy
    • Apical crowding on imaging
    • Abnormal visual acuity
    • Relative afferent pupillary defect
    • Abnormal visual fields
  • Risk factors: increased age, rapid onset of symptoms (4-6 weeks), fluctuating thyroid levels, smoking, diabetes, life stressors (ie periocular surgery)

References

  • Douglas RS, McCoy A, Gupta, S. Thyroid Eye Disease. First Edition, New York: Springer, 2015.
  • Dolman, PJ. Medical and surgical options in thyroid eye disease. In: Levine MR, Allen RC. Manual of Oculoplastic Surgery. Fifth Edition, Springer, 2018.
  • Mourits MP, Prummel MF, Wiersinga WM, Koornneef L. Clinical activity score as a guide in the management of patients with Graves’ ophthalmopathy. Clin Endocrinol (Oxf) 1997;47(1):9-14.
  • Holds JB, Buchanan AG. Graves orbitopathy. Focal Points: Clinical Modules for Ophthalmologists. Module 11. San Francisco: American Academy of Ophthalmology; 2010.
  • Bahn RS. Graves’ Ophthalmopathy. English J Med. 2010;8(362):726-38.
  • McKeag D, Lane C, Lazarus JH, Baldeschi L, Boboridis K, Dickinson AJ, et al. Clinical features of dysthyroid optic neuropathy: A European Group on Graves’ Orbitopathy (EUGOGO) survey. Br J Ophthalmol. 2007;91(4):455-8.
  • Marcocci C, Kahaly GJ, Krassas GE, Bartalena L, Prummel M, Stahl M, et al. Selenium and the Course of Mild Graves’ Orbitopathy. N Engl J Med. 2011;364(20):1920-31.
  • Rootman DB. Orbital decompression for thyroid eye disease. Surv Ophthalmol. Elsevier Inc; 2018;63(1):86-104.
  • Neigel JM, Rootman J, Belkin RI, Nugent RA, Drance SM, Beattie CW, et al. Dysthyroid Optic Neuropathy: The Crowded Orbital Apex Syndrome. Ophthalmology. American Academy of Ophthalmology, Inc; 1988;95(11):1515-21.
  • Gold KG, Scofield S, Isaacson SR, Stewart MW, Kazim M. Orbital Radiotherapy Combined With Corticosteroid Treatment for Thyroid Eye Disease-Compressive Optic Neuropathy. Ophthal Plast Reconstr Surg 2018;34(2):172-7.
  • Chundury R V., Weber AC, Perry JD. Orbital radiation therapy in thyroid eye disease. Ophthal Plast Reconstr Surg. 2016;32(2):83-9.
  • Rootman J. Surgery for Thyroid Orbitopathy. Orbital Surgery: A Conceptual Approach. 2018.

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