10 Congresso Nazionale Associazione Italiana della Tiroide Cagliari, dicembre 2016 Orbitopa)a basedowiana acuta

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1 10 Congresso Nazionale Associazione Italiana della Tiroide Cagliari, dicembre 2016 Orbitopa)a basedowiana acuta Luigi Bartalena Università dell Insubria a Varese

2 Prevalence of Graves orbitopathy in newly diagnosed Graves disease Tanda Bartalena, JCEM 2013

3 Degree of severity of GO of pa)ents newly referred to EUGOGO centers 2000 COHORT Percent of pa)ents p<0.001 p< COHORT p<0.001 Perros & EUGOGO, Br J Ophthalmol 2015

4 Incidence of moderate-to-severe Graves orbitopathy in Denmark Cases per million per year Laurberg, JCEM 2012

5 Natural history of Graves orbitopathy GO progressing with )me Percent Piantanida Bartalena, J Endocrinol Invest 2013

6 Risk factors for occurrence and progression of Graves orbitopathy Gene)c Smoking Age Endogenous Factors Exogenous Factors Hyper/Hypo Radioiodine Gender TRAb Graves Orbitopathy Oxida)ve Stress

7 Classifica)on of severity of GO recommended by EUGOGO Sight-threatening GO Dysthyroid op)c neuropathy (DON) and/ or corneal breakdown. Immediate interven)on is required Moderate to severe GO GO is not sight-threatening but has sufficient impact on QoL to jus)fy the risks of immunosuppressive or surgical interven)on Mild GO GO has only a minor impact on QoL insufficient to jus)fy the risks of immunosuppressive or surgical interven)on Bartalena & EUGOGO, Thyroid & EJE 2008

8 Possible reasons for urgent/emergent referral of a pa)ent with Graves orbitopathy Ø Dysthyroid Op<c Neuropathy (DON) Ø Corneal Breakdown Ø Subluxa<on of the Globe

9 Possible reasons for urgent/emergent referral of a pa)ent with Graves orbitopathy Ø Dysthyroid Op<c Neuropathy (DON) Ø Corneal Breakdown Ø Subluxa<on of the Globe

10 Pathogenesis of Dysthyroid Op)c Neuropathy (DON) Ø Direct compression of the op<c nerve by enlarged extraocular muscles Ø Impingement of its vascular supply by enlarged extraocular muscles (less likely) Ø Tight orbital septum preven<ng auto-decompression of orbital pressures by forward protrusion of the globe Ø Stretching of the op<c nerve in the sekng of extreme proptosis

11 Visual Acuity in DON Number of eyes Best Corrected Visual Acuity McKeag & EUGOGO, Br J Ophthalmol 2007

12 Clinical Ac)vity Score in DON McKeag & EUGOGO, Br J Ophthalmol 2007

13 Abnormal findings in DON Eyes with definite DON Eyes with equivocal DON Eyes with no DON Reduced color vision 77% 56% 7% Visual acuity 80% 59% 32% Apical crowding 95% 64% 43% Op)c disc swelling 56% 18% 5% Op<c nerve pallor 4% 12% 0% Visual field defect 71% 71% 13% Abnormal VEP latency 73% 86% 0% Proptosis 62% 81% 63% Op<c nerve stretch 33% 0% 43% RAPD 45% 40% NA McKeag & EUGOGO, Br J Ophthalmol 2007

14 Courtesy of Prof. Lelio Baldeschi

15 Glucocor)coids or orbital decompression for sight-threatening GO (DON)? Further treatments: Steroid group: 56% Surgery group: 83% Wakelkamp, Clin Endocrinol 2005

16 Response to intravenous glucocor)coids in 24 pa)ents (40 eyes) with DON Currò, Thyroid 2014

17 Clinical Ac)vity Score of pa)ents with DON following ivgc treatment Currò, Thyroid 2014

18 Orbits requiring orbital decompression due to poor response to ivgcs Currò, Thyroid 2014

19 Recommenda)on 17 Ø We recommend that DON be treated immediately with very high doses of intravenous GCs ( mg of MP for 3 consecu<ve days or on alternate days during the first week) and urgent orbital decompression be performed if response is absent or poor within 2 weeks If DON has resolved or improved a`er 2 weeks, pulses of weekly intravenous MP should be con<nued. Ø Strength of Recommenda)on:1; Evidence: ØØØO Bartalena & EUGOGO, Eur Thyroid J 2016

20 Possible reasons for urgent/emergent referral of a pa)ent with Graves orbitopathy Ø Dysthyroid Op<c Neuropathy (DON) Ø Corneal Breakdown Ø Subluxa<on of the Globe

21 Pathogenesis of Corneal Breakdown Ø Extreme proptosis Ø Extreme eyelid retrac<on Ø Ineffec<ve blinking with poor tear produc<on Ø Incomplete eyelid closure (lagophthalmos) Ø Cornea exposure when eyelids are approximated due to <ght inferior rectus muscle (absent Bell s phenomenon)

22 Ini)al treatment of corneal breakdown Ø Frequent use of lubricants Ø Intensive topical an<bio<cs Ø Eyelids may be taped closed Ø Globe coverage by botulinum toxin injec<ons into the levator muscle (3-4 days delay!) or by tarsorraphy Ø Orbital decompression, corneal transplant or amnio<c membrane gra`s may be required

23 Possible reasons for urgent/emergent referral of a pa)ent with Graves orbitopathy Ø Dysthyroid Op<c Neuropathy (DON) Ø Corneal Breakdown Ø Subluxa<on of the Globe

24 Subluxa)on of the globe Ø Anterior displacement of the globe equator beyond palpebral rim Ø Lid retrac<on behind the equator Ø Tethering of the op<c nerve Ø Extremely rare (0.01%) Ø Extraocular muscles are usually normal, orbital fat is generally marked increased Ø Tarsorraphy prior to decompression surgery

25 Courtesy of Prof. Lelio Baldeschi

26 Features of Sight-Threatening Graves Orbitopathy Sight-threatening condi)on Dysthyroid Op<c Neuropathy Corneal Ulcer Subluxa<on of the globe Risk factor Smoking Older pa<ents Severe inflamma<on Diabetes Extreme proptosis Lagophthalmos Excessive lid retrac<on Poor Bell s phenomenon Extreme proptosis with lid retrac<on Excessive fat enlargement with rela<vely normal EOM volume Sugges)ve signs/ symptoms Blurred vision not corrected by blinking Dulling of color vision Constant diplopia Op<c disc edema Rela<ve afferent pupillary defect Severe pain, especially if new in onset, sharp in quality and associated with blurred vision Pa<ent may describe an episode compa<ble with subluxa<on Referral to ophthalmologist Urgent Emergent Urgent

27 Referral pathways for pa)ents with GO Perros, Clin Med 2015; 2: 173-8

28 Take Home Messages Ø Urgent/emergent referral in the presence of: v severe ocular pain, of new onset and sharp in nature, associated with blurred vision v deteriora<on in vision that does not clear with blinking v history of subluxa<on of the globe

29 Grazie per l aqenzione