Thyroid associated orbitopathy

Presentation is made by

Dr. Sohel MahmudMBBS, DO.

Eye specialist & surgeonDhaka, Bangladesh.

A stone made statue of a

man with unilateral

proptosis at the time of

ancient Greece.

Introduction Thyroid

associate

d

orbitopat

hy

Graves’

ophthalmop

athy

Thyroid

orbitopat

hy

Dysthyroid

ophthalmopa

thy

Thyroid

eye

disease

Thyrotoxic

exophthal

mos

Introduction cont...

Thyroid-associated orbitopathy (TAO) is

an autoimmune inflammatory disorder that

can affect the orbital and periorbital tissue,

the thyroid gland and rarely the pretibial

skin or digits.

Epidemiology TAO • Commonly presents during fourth and fifth decades• Median age at the time of diagnosis 43years• Range 8-88years

Epidemiology of TAO cont...

86%Women

14% Men TAO affects women approximately 6 times

more frequently than men.

Smokers are up to 7 times more likely

than nonsmokers to develop TED. 89%Smokers

11%Nonsmokers

Etiology

• TAO typically associated with Graves’ hyperthyroidism but may also occur with Hashimoto’s thyroiditis or in absence of thyroid dysfunction

Pathogenesis

CD1

54

Orbital

fibrobla

st

T

cell

Up-regulation –

1. IL-6

2. IL-8

3. PGE2

Synthesis of-

Hyaluronan

GAG is

increased

Derived from

neural crest and

possess

developmental

plasticity

1

A subpopulation

undergoing adipocyte

differentiation causing fatty

hypertrophy particularly in

those younger than 40

years

2

Up-regulation of

TSH-R mRNA

synthesis

Adipogenesis-

Expansion of

orbital fat

compartment

3

Circulating IgG

activates insulin like

growth factorFound

in a

majority

with

Graves

disease

4

HistopathologyFindings on histological examination-

• Fibrosis with degenerative changes in the eye

muscles

• Lymphocytic cell infiltration

• Enlargement of fibroblasts

• Accumulation of mucopolysaccharides

• Interstitial edema

• Increased collagen production

Grossly

Enlarge extraocular muscles in TAO

Clinical features of TAO

Eye signs of TAO

Dalrymple

sign

Von Graefe

sign

Kocher

sign

Goldzieher’s

sign

Courtesy by oculoplasty dept. of

NIO&H

Mechanism of lid retraction

•Fibrotic contracture of the levator

•Secondary overaction of the levator superior

rectus complex

•Humorally induced overaction of muller muscles

Courtesy by oculoplasty dept. of

NIO&H

Proptosis• Axial• Uni/bilateral• Symmetrical/ asymmetrical

Severe proptosis leads

to -

•Exposure keratopathy

•Corneal ulcer

•Infection

Courtesy by oculoplasty dept. of

NIO&H

Restrictive myopathy

• Initially by inflammatory edema later for fibrosis

•Elevation defect

•Abduction defect

•Depression defect

•Adduction defect

Courtesy by oculoplasty dept. of

NIO&H

Optic neuropathy

• Uncommon but serious complication• Caused by compression of the optic nerve or its

blood supply at the orbital apex

Stages TAO

•Congestive

•Static

•Fibrotic/quiescent

Courtesy by oculoplasty dept. of

NIO&H

NOSPECS classification of TAO

Systemic features

Hypothyroid Hyperthyroid

Goitre

Pretibial

myxoedema

Acropachy

Vitiligo

Systemic features cont…

InvestigationThyroid function test-

OthersUSG of thyroid glandThyroid scanningFNACThyroid scintigraphyECGEchocardiography

•Serum free T3,T4,TSH

•Thyroid stimulating immunoglobulin

(TSI)

•Thyroid binding inhibitory Ig

•CT scan of orbit and brain

Investigation cont…

Courtesy by oculoplasty dept. of

NIO&H

•Fusiform enlargement of muscle

•Tendon spearing

•Muscle border smooth

On CT scan of orbit and brain-

Investigation cont…

Diagnostic criteria

The diagnosis of TAO is made when 2 of the following 3 signs ofthe disease are present:

1. Concurrent or recently treated immune-related thyroid

dysfunction (l or more of the following):

a. Graves hyperthyroidism

b. Hashimoto thyroiditis

c. Presence of circulating thyroid antibodies without a

coexisting dysthyroid state (partial consideration given):

TSH-receptor (TSH-R) antibodies, thyroid-binding

inhibitory immunoglobulins (TBll), thyroid-stimulating

immuno-globulins (TSI), antimicrosomal antibody.

The diagnosis of TAO is made when 2 of the following 3 signs ofthe disease are present:

2. Typical orbital signs (l or more of the following):

a. Unilateral or bilateral eyelid retraction with typical

temporal flare (with or without lagophthalmos)

b. Unilateral or bilateral proptosis (as evidenced by

comparison with

patient's old photos)

c. Restrictive strabismus in a typical pattern

d. Compressive optic neuropathy

e. Fluctuating eyelid edema/erythema

f. Chemosis/caruncular edema

Diagnostic criteria cont…

The diagnosis of TAO is made when 2 of the following 3 signs ofthe disease are present :

If only orbital signs are present the patient should continue tobe observed for other orbital diseases and for the futuredevelopment of a dysthyroid state

3. Radiographic evidence of TAO- unilateral/bilateral

fusiform enlargement

of 1 or more of the following :

a. Inferior rectus muscle

b. Medial rectus muscle

c. Superior rectus/levator complex

d. Lateral rectus muscle

Diagnostic criteria cont…

Treatment of TAO

Supportive measures

Medical management

Orbital radiation therapy

Surgical management

Treatment of TAO cont…

1. Smoking

cessation

2. Lubricating eye

drops

3. Cool compression

4. Salt restriction

5. Elevation of head

6. Wearing sunglass

Treatment of TAO cont…

1. Systemic corticosteroids

•Starting dose 60-100 mg orally

•Short-term pulse intravenous dose 1gm daily

several

times a weak for up to 2 months

2. Others

• Cyclosporine

• Ticlopidine

• Intravenous immune globulin

• Somatostatin analogues

Treatment of TAO cont…

• Orbital irradiation is prescribed for moderate to severe

inflammatory

symptoms, diplopia and visual loss in patients with TAO

• Typical dose 2000 rad to each orbit, delivered as 200

rad/day for

10 days

Treatment of TAO cont…

1. Orbital

decompression

2. Strabismus surgery

3. Eyelid surgery

Treatment of TAO cont…

• Observation

• Patient education/lifestyle

changes

• Smoking cessation

• Salt restriction

• Elevation of head of bed

• Wearing sunglasses

• Ocular surface lubrication

• Topical cyclosporine

• Eyelid taping at night

• Moisture goggles/chambers

• Prism glasses or selective ocular

patching

• Moderate-dose oral steroid therapy

• High-dose oral steroid therapy

• Intravenous steroid therapy

• Surgical orbital decompression

• Strabismus surgery

• Eyelid surgery

• Periocular radiotherapy

Refractory disease

• Steroid-sparing immunomodulators

(rituximab)

Mild

diseas

e

Moder

ate

diseaseSevere

disease

Prognosis• TAO is a self-limiting disease • On average lasts 1 year in nonsmokers and between 2 and 3

years in smokers• After the active disease plateaus, a quiescent burnt-out phase

ensues • Reactivation of inflammation occurs in approximately 5%-

10% of patients over their lifetime

Poor prognostic features-

• Smoking

• Rapidly progressive (typically

congestive) TAO

• Presence of myxoedema

THANK YOU & THANK TO

THEM