Thyroid Eye Disease

Ea RaksmeyPo Lindara

Outline

• Introduction to TED• Epidemiology• Pathogenesis • Clinical features

– Systemic – Ocular

– Diagnosis– Prognosis– Management:

– Supporting care– Medical– Surgical

Introduction• TED or Graves ophthalmopathy • Auto-immune • Excessive secretion of thyroid hormones • Mostly associated with:

– Graves hyperthyroidism – Hashimoto thyroiditis– Euthyroidism

• Characteristics – Lid retraction – Lid lag– Proptosis– Restcrictive extraocular myopathy – ON compressoin

Epidemiology

• Female 4x• Smokers 7x• 3rd-4th decade of life • Associated with:

– 90% Graves hyperthyroidism – 6% Euthyroidism – 3% Hashimoto thyroiditis – 1% Primary hypothyroidism

• Onset: – 20% of TED is diagnosed same time as hyperthyroidism – 60% of eye disease occur 1 year after thyroid disease – Only 30% of hyperthyroidism TED

Pathogenesis

Fibroblast

Adipogenesis Fat HypertrophySynthesis of GAG

Inflammatory reaction

IgGTSH-R mRNA synthesis

T-Cells Stimulation

Systemic Features

Ocular Features• Symptoms:

– Grittiness – Photophobia – Lacrimation – Retrobulbar discomfort

• Signs: – Lid retraction – Lid lag– Restrictive EOM movement– Proptosis– ON compression

• 2 Stages: – Congestive: remits within 3 years, 10% long term problems– Fibrotic: restrictive movement

Lid Retraction• 50%• Fibrotic contracture of levator:

– Worse in downgaze

• Secondary overreaction of levator-SR complex:– Secondary hypotrophia caused by fibrosis of IR– Worse in upgaze

• Humorally induced overreaction of Müller muscle:– Sympathetic stimulation by thyroid hormones

• 3 signs:

Dalrymple Sign Kocher Sign Von Graefe Sign

Proptosis

• Axial • Uni/bilateral • Symmetrical/asymmetrical • Inflammatory cells infiltration GAG fluid retention

increase orbital pressure proptosis

Restrictive EOM

• 30-50%• Inflammation of EOM

– cells infiltration retain fluid swelling compression– Muscle fibers degeneration fibrosis

• Elevation defect: IR fibrosis• Abduction defect: MR fibrosis• Depression defect: SR fibrosis• Adduction: LR fibrosis

ON Compression • Uncommon but serious • Inflammation of EOM cells infiltration GAG fluid

retention orbital pressure compression • +/- proptosis • Signs:

– Reduced VA +/- RAPD, color desaturation– VF defect: central or paracentral, increased IOP (confused with POAG)– OD is normal, might swollen and rarely atrophic

Enlargement of recti with tendon sparing

Diagnosis• 2 of 3 sign present

– Thyroid dysfunction:• Grave• Hashimoto• Thyroid Ab, TSH-R, TBII, TSI, antimicrosomal

– Orbital sign as above– Rx evidencen of uni/bilateral fusiform

enlargement of 1 or more• MR• IR• SR/ elevator complex

• If only 1 orbital sign; observe

Differential diagnosis

• VEIIN +Miscellous:– Vascular– Endocrine– Infection– Inflammation– Neoplastic– Miscellous

Prognosis– self-limiting disease average lasts 1 year– 2-3 years in smoker, 7 x develop the orbital– Reactivation 5-10%– Poor prognosis feature include:

• smoking• rapidly progressive orbitopathy• dermopathy

– Most patient require only support care– Intervention may be necessary if inflammation

is severe– long-term F/U:

• Visual loss from ON is uncommon• >50% thought that their looked abnormal• 38% were dissatisfied

Management

Treatement

• Supportive care:– Stop smoking– Lubricant (methylcellulose)– cool compress– head elevated– fluid diet– Prism lens– Botulinum toxin Mϋller muscle– 5% guanthidine (worse by side effect)

Medication• Corticosteroid:

– to suppress immune properties– Indication (active inflammation) :

• Erythema• Eyelid edema• Compressive Opticneuropathy

– starting:• 60-100mg orally• short-term pulse IV; 1g daily 1 week to 2

months• retrobulbar or peribulbar 20mg /0,5cc

triamcinolone weekly for 4 weeks(benificial on diplopia and EOM size without significan s.e.)

Medication• Cyclosporin :

– compare to steroid (21% vs 61%)– very limited role– s.e. nephrotoxicity, hypertension

• Methothrexate:– second-line treatment with recalcitrant TED

to control the clinical profile and delay surgery untill the disease stability

• Ticlonidine, IV immune globulin• Somatostatin analogue

Radiation therapy

– Mechanism effect is unclear– induce terminal differentiation of

fibroblast– kill tissue-bound monocyte– clinical trial design show no statistically

significant compared with the natural history of the diseas

– Should avoid with: diabetes, vasculitis, may exacerbate retinopathy.

Surgery

• Orbital decompression• Strabismus surgery• Eyelid retraction repair

Orbital decompression

• to create more space for the swollen tissue by expanding the wall of the orbit

• Indication:– CON– excessive proptosis :

• Globe subluxation• Cornea ulcer• Steroid depending• Intractable pain• Cosmetic

Strabismus surgery

• stable at least 6 months• to achieve binocular vision in primary

and downgaze position• most frequent is recession MR or IR

(6-7mm), no more than 3 muscle

Eyelid retractor retraction

• after orbital decompression done• indication:

– ocular discomfort– keratitis, including corneal ulceration, – globe subluxation,– cosmesis

Message home

– Eyelid retraction is the most common feature of TED

– TED is the most common uni/bilateral proptosis, markedly asymmetric

– 90% hyper, but 6% euthyroid– Severity is not parallel to serum level (TSH,

T3, T4..), but the smoking indeed 7x– Urgent care may be require for COM,

severe proptosis cornea decompensat– Surgery should be in order: Orbital

decompression Strabismus eyelid correction

Thank you!!