Case Presentation 12/19Presenting: clerk 陳豪宏

Instructor: 張丞賢老師

Patient Profile

Name: 謝 X 莉 Age: 46 years old Gender: female Chart number: 24097375 Date of admission: 2011/12/12

Chief complaint

Right eye pain for 1 week.

Present Illness 1

This 46 years old female suffered from insidious right eye pain for 1 week. The accompanying symptoms are red eye, tearing (od). She had hyperthyroidism s/p subtotal thyroidectomy on 2011-8-18.

Thyroid orbitopathy with compressive optic neuropathy was diagnosed this September. She was admitted to our hospital and received steroid pulse therapy on September 5th this year.

Present Illness 2

After discharge on September 9th. She came for OPD follow-up on September 14th. Diplopia was still complained. OCT showed loss of nerve fibrous layer.

She was admitted for operation on September 20th, during which orbital decompression (ou) of temporal sides and muscle recession of inferior rectus were done.

On December 12th, she was admitted for steroid pulse therapy and orbital decompression of nasal sides.

Present Illness Summary

Thyroidectomy

08/18

First Steroid Therapy 09/05~09/09

First Decompression

(Temporal Sides)

09/19~09/23

Second decompression

12/12~ now(op:12/16)

Past History

1. hyperthyroidism, s/p operation2. hypertension(+)3. asthma(+)4. heart disease(-)5. DM(-)6. depressive disorder under medical treatment Operation history: 1. L-spine s/p operation 1.5 year ago2. hyperthyroidism s/p subtotal thyroidectomy

on 2011-8-18 at 謝外科3. Orbital Decompression on 09/20 Admission history:

As listed in the Present Illness

Personal History

1. Cigarette Smoking : +, 1/2 pack per day

2. Alcohol : denied3. Occupation history : denied4. Contact history : denied5. Travel history : denied6. Family History: denied family

systemic disease7. Allergy History: denied

Nutritional and Mental status

Nutritional Status: Weight: 81.6kg; Height:

151.5cm; BMI: 35.55 Mental Status: Consciousness: alert. Mentality:

normal.

Current Medicine

2011-9-13 PSY OPD Dr. 葉怡君 EFEXOR 速悅 XR(Venlafaxine) 1#AM

1#HS */PC*28 Estazolam #(Eurodin) 管四 #2

QD/HS* 28 Inderal 10mg (Propranolol) #1

BID/PC* 28 Xanax 0.25mg (Alprazolam) 管四 #1

BID/PC* 28 Zyprexa 5mg (Olanzapine)**** #1

QD/HS* 28

Physical Examination 1

Vital sign: BP: 131 / 105 mmHg, PR: 80 bpm, RR: 18 cpm, BT:

37 ℃ <Ophthalmic examination> 2011/12/12 OD OS

Eyeball 25>-----------105------------<24 Conjunctiva injection injection Cornea clear clear AC moderate, clear moderate-shallow,

clear Iris np np Pupil 4mm 4mm Light reflex +/+ +/+ Lens clear clear

Physical Examination 2

Fundus C/D=0.4 C/D=0.7 disc: temporal pale EOM 20 20 | | 35 ---|---45 45---|---30 | | 30 10

Tonopen: straight: R: 21,22 L: 22,23 Upward: R: 27,28 L: 27,29 Downward: R: 22 L:22 Vod: 0.1x 1/5 Vos: ND/10cm

Orbital CT

Hypertrophy of extraocular muscles (all of ou)

Abnormal Lab finding

12/12: Hb: 12.0 Hct: 35.3 MCV: 79.8 Plt: 502k

Diagnosis

Thyroid orbitopathy with compressive optic neuropathy

Plan:

Pulse therapy of Solumedrol 500mg q12h IV

Arrange Auto-P, VEP, OCT of disc. Orbital decompression under

general anesthesia.

Progress 12/13

S: eye pain(-) Red eye ↓ O: EOM: 20 20

40 45 35 10

35 (od) 10 (os) Cornea: clear Conjunctival congestion:

↓ Chemosis: ↓ AC: deep, clear A+P: arrange VEP, OCT of disk today.

Prepare endoscopic decompression

Progress 12/14

S: eye pain(-) O: EOM: 20 20

35 45 35 35

35 (od) 10 (os) Cornea: clear Conjunctiva: mild congested Chemosis: ↓ AC: deep, clear VEP: delayed potency and amplitude

(ou) A+P: Keep pulse therapy day 2

Progress 12/15

S: Still blurred vision (ou) O: EOM: 20 20

35 45 40 35

35 (od) 20 (os) Cornea: clear Conjunctiva: Congestion ↓ ↓ Chemosis: (-) few SPK in the left lower eye IOP: 24.7 (os), 20.5 (od). A+P: Arrange endoscopic orbital

decompression (ou) tomorrow

Operation on 12/16

Endoscopic orbital decompression done in this morning. (8.00 am)

Procedures taken:1. Preparation for anesthesia Endoscope usage:2. Use periosteal elevator to destruct cribriform pyparacea

temporally and posteriorly.3. Destruct the ethmoid bone.4. Rigid orbital soft tissue with fibrotic membran was noted.5. Incision of the fibrotic membrane and let the orbital fat

protrude out from orbital medial wall.6. Hemostasis adequately. The operation was done successfully and the patient

returned to the ward for recovery.

Progress 12/16

S: no obvious pain of wound post-op. Still blurred vision

O: Endoscopic wound of the surgery. Condition fine.

A+P:

DiscussionDiscussionA Brief Introduction of Thyroid A Brief Introduction of Thyroid

OrbitopathyOrbitopathy

Thyroid Eye DiseaseThyroid Eye Disease

Thyroid eye disease has many Thyroid eye disease has many names:names: Thyroid-associated orbitopathy (TAO)Thyroid-associated orbitopathy (TAO) Thyroid orbitopathyThyroid orbitopathy Grave's orbitopathyGrave's orbitopathy Among othersAmong others

All these terms mean the same thing:All these terms mean the same thing: inflammation of the tissues around inflammation of the tissues around

and behind the eye producing varying and behind the eye producing varying amounts of swelling and scarring.amounts of swelling and scarring.

IntroductionIntroduction

It is an autoimmune disease.It is an autoimmune disease. There are at least various theories to There are at least various theories to

explain its cause and development. explain its cause and development. Thyroid eye disease usually occurs Thyroid eye disease usually occurs

in people with a history of thyroid in people with a history of thyroid problems. problems. However, thyroid eye disease can occur However, thyroid eye disease can occur

decades before, or decades after, the decades before, or decades after, the development of thyroid gland disease. development of thyroid gland disease.

PathogenesisPathogenesis

The volume of The volume of 1.1. The extraocularThe extraocular2.2. retroorbital connective and adipose tissue retroorbital connective and adipose tissue are increased, due to inflammation and the are increased, due to inflammation and the

accumulation of hydrophilic glycosaminoglycans (GAG), accumulation of hydrophilic glycosaminoglycans (GAG), principally hyaluronic acid, in these tissues. principally hyaluronic acid, in these tissues.

GAG secretion by fibroblasts is increased by GAG secretion by fibroblasts is increased by activated T-cell cytokines such as tumor necrosis activated T-cell cytokines such as tumor necrosis factor (TNF) alpha and interferon gamma.factor (TNF) alpha and interferon gamma. It implies that T-cell activation is an important part of It implies that T-cell activation is an important part of

this immunopathology. this immunopathology. The accumulation of GAG causes a change in The accumulation of GAG causes a change in

osmotic pressure, which in turn leads to a fluid osmotic pressure, which in turn leads to a fluid accumulation and an increase in pressure within the accumulation and an increase in pressure within the orbit. orbit.

These changes displace the eyeball forward and can These changes displace the eyeball forward and can also interfere with the function of the extraocular also interfere with the function of the extraocular muscles and the venous drainage of the orbits. muscles and the venous drainage of the orbits.

Pathogenesis (Summary)Pathogenesis (Summary)

Autoimmune caused Inflammation Autoimmune caused Inflammation

T cell: TNF-a and interferon r T cell: TNF-a and interferon r

Fibroblasts: GAGFibroblasts: GAG

Osmotic pressureOsmotic pressure

Fluid accumulation and IOPFluid accumulation and IOP

Eyeball forwardEyeball forward Venous drainage malfunctionVenous drainage malfunction EOM dysfunction EOM dysfunction

↑

↑

↑

↑

Risk FactorsRisk Factors

1.1. Treatment: Ra-I therapy may be more Treatment: Ra-I therapy may be more likely to lead to the development or likely to lead to the development or worsening of TO than medication or worsening of TO than medication or subtotal thyroidectomy. subtotal thyroidectomy.

2.2. Sex: TO: female > maleSex: TO: female > male3.3. According to studies, smoking According to studies, smoking

definitely makes thyroid eye disease definitely makes thyroid eye disease worse. worse.

increase in the connective tissue volume increase in the connective tissue volume of the orbit, but not the extraocular of the orbit, but not the extraocular muscle volumes.muscle volumes.

4.4. Thyrotropin receptor autoantibodiesThyrotropin receptor autoantibodies HHigher titer -> Higher prevalence and igher titer -> Higher prevalence and

longer course of TO.longer course of TO.

Epidemiology Epidemiology

Approximately 20 to 25 percent of Approximately 20 to 25 percent of patients with Graves' patients with Graves' hyperthyroidism have clinically hyperthyroidism have clinically obvious TO. obvious TO. Along with the eye signs of thyroid Along with the eye signs of thyroid

hormone excess (lid retraction and hormone excess (lid retraction and stare), at the time of diagnosis of the stare), at the time of diagnosis of the hyperthyroidism. hyperthyroidism.

However, many more patients However, many more patients with Graves' hyperthyroidism have with Graves' hyperthyroidism have evidence of TO in imaging studies.evidence of TO in imaging studies.

Clinical PresentationsClinical Presentations

proptosis and periorbital edema proptosis and periorbital edema

Clinical Presentations 2Clinical Presentations 2

The patient may be distressed by the The patient may be distressed by the appearance of his or her eyes.appearance of his or her eyes.

Possible major symptoms:Possible major symptoms:1.1. sense of irritationsense of irritation2.2. excessive tearing that is often made excessive tearing that is often made

worse by exposure to cold air, wind, worse by exposure to cold air, wind, or bright lightsor bright lights

3.3. eye or retroorbital discomfort or paineye or retroorbital discomfort or pain4.4. blurred visionblurred vision5.5. DDiplopiaiplopia6.6. and occasionally loss of vision. and occasionally loss of vision.

ProptosisProptosis

The degree of proptosis The degree of proptosis (exophthalmos) is dependent on the (exophthalmos) is dependent on the depth of the orbit and the degree of depth of the orbit and the degree of enlargement of the retroocular muscles enlargement of the retroocular muscles and retroorbital fibrous and fatty and retroorbital fibrous and fatty tissue. tissue.

The proptosis may be usually The proptosis may be usually symmetric, but is often asymmetric, symmetric, but is often asymmetric, and may be accompanied by a and may be accompanied by a sensation of pressure behind the sensation of pressure behind the eyeballs. eyeballs.

The proptosis may be masked by The proptosis may be masked by periorbital edema, which is a common periorbital edema, which is a common accompaniment.accompaniment.

Apparent proptosisApparent proptosis

Many patients with hyperthyroidism Many patients with hyperthyroidism have lid retraction secondary to have lid retraction secondary to thyroid hormone excess.thyroid hormone excess.

It leads to stare and lid lag, resulting It leads to stare and lid lag, resulting from contraction of the levator from contraction of the levator palpebrae muscles of the eyelids. palpebrae muscles of the eyelids.

The stare may give the appearance of The stare may give the appearance of proptosis, when none in fact exists proptosis, when none in fact exists ("apparent proptosis"). ("apparent proptosis").

These signs alone do not indicate the These signs alone do not indicate the presence of ophthalmopathy, and presence of ophthalmopathy, and subside when the hyperthyroidism is subside when the hyperthyroidism is treated. treated.

Physical Examinations 1Physical Examinations 1

Inspection of the conjunctivae and Inspection of the conjunctivae and periorbital tissue, looking for chemosis periorbital tissue, looking for chemosis and periorbital edema.and periorbital edema.

Determination of the extent to which Determination of the extent to which the upper and lower lids can be closed. the upper and lower lids can be closed. Because failure of apposition promotes Because failure of apposition promotes

drying and ulceration of the cornea.drying and ulceration of the cornea. Assessment of EOM. Assessment of EOM.

inability to achieve or maintain inability to achieve or maintain convergence. convergence.

Limitation of upward gaze. It leads to a Limitation of upward gaze. It leads to a characteristic head-back position in order to characteristic head-back position in order to see ahead. see ahead.

double vision.double vision.

Physical Examinations 2Physical Examinations 2

exophthalmometer. exophthalmometer. measurement of the distance between measurement of the distance between

the lateral angle of the bony orbit and an the lateral angle of the bony orbit and an imaginary line tangent to the most imaginary line tangent to the most anterior part of the cornea. anterior part of the cornea.

The upper limit of normal is 20~22 mm. The upper limit of normal is 20~22 mm. as high as 30 mm in patients with severe as high as 30 mm in patients with severe

proptosis.proptosis. Visual acuity and color vision should Visual acuity and color vision should

be assessed by simple reading tests be assessed by simple reading tests and color charts, and visual fields and color charts, and visual fields should be evaluated by confrontation. should be evaluated by confrontation.

Assessment of SeverityAssessment of Severity

NO SPECS by American Thyroid NO SPECS by American Thyroid Association Association

Class 0 — No symptoms or signsClass 0 — No symptoms or signs Class I — Only signs, no symptoms (eg, lid Class I — Only signs, no symptoms (eg, lid

retraction, stare, lid lag)retraction, stare, lid lag) Class II — Soft tissue involvementClass II — Soft tissue involvement Class III — ProptosisClass III — Proptosis Class IV — Extraocular muscle involvementClass IV — Extraocular muscle involvement Class V — Corneal involvementClass V — Corneal involvement Class VI — Sight loss (optic nerve Class VI — Sight loss (optic nerve

involvement)involvement)

Differential DiagnosisDifferential Diagnosis

eye signs of thyroid hormone excess eye signs of thyroid hormone excess Bilateral eye signs simulating TO: Bilateral eye signs simulating TO:

1.1. severe obesitysevere obesity

2.2. Cushing's syndromeCushing's syndrome

3.3. orbital myositisorbital myositis

4.4. histiocytosishistiocytosis

5.5. myasthenia gravismyasthenia gravis

6.6. very rarely: orbital tumorsvery rarely: orbital tumors

7.7. statin-induced EOM myopathy. statin-induced EOM myopathy.

Differential Diagnosis 2Differential Diagnosis 2

For possible unilateral TO, space-For possible unilateral TO, space-occupying lesions of the orbit must be occupying lesions of the orbit must be ruled out first.ruled out first.

When necessary, the diagnosis can be When necessary, the diagnosis can be confirmed by ultrasonography and CT.confirmed by ultrasonography and CT. It is important not to inject iodinated It is important not to inject iodinated

contrast material in patients with Graves' contrast material in patients with Graves' disease especially if radioiodine therapy is disease especially if radioiodine therapy is contemplated.contemplated.

If diagnos is not in doubt, only tests If diagnos is not in doubt, only tests necessary are: necessary are: serum TSHserum TSH FFree T4ree T4 TSHR antibodies.TSHR antibodies.

TreatmentTreatment

treat according to the severity. treat according to the severity. Most patients have mild disease Most patients have mild disease

and do not have progression and do not have progression during follow-up. during follow-up.

The treatment of TO includes The treatment of TO includes 1.1. reversal of hyperthyroidismreversal of hyperthyroidism2.2. relief of symptomsrelief of symptoms3.3. reduction of inflammation in the reduction of inflammation in the

periorbital tissues.periorbital tissues.

Effects of anti-Effects of anti-hyperthyroidism therapieshyperthyroidism therapies

Subtotal thyroidectomy and Subtotal thyroidectomy and antithyroid drugs do not appear to antithyroid drugs do not appear to have a negative influence on the have a negative influence on the course of orbitopathy. course of orbitopathy.

However, there is increasing However, there is increasing evidence that radioiodine therapy evidence that radioiodine therapy can cause the development or can cause the development or worsening of Graves' orbitopathy worsening of Graves' orbitopathy more often than antithyroid drug more often than antithyroid drug therapy or surgery. therapy or surgery.

Symptomatic treatmentSymptomatic treatment

Eye shadesEye shades Artificial tears (saline eye drops)Artificial tears (saline eye drops) Raising the head of the bed when sleep. Raising the head of the bed when sleep. Photophobia and sensitivity to wind or Photophobia and sensitivity to wind or

cold air can be relieved by use of dark cold air can be relieved by use of dark glasses. glasses.

Glucocorticoids (oral or IV), are the Glucocorticoids (oral or IV), are the primary treatment for severe Grave‘s primary treatment for severe Grave‘s orbitopathy. orbitopathy.

Radiation and surgical decompression Radiation and surgical decompression can also be used in selected patients. can also be used in selected patients.

Therapies for severe TOTherapies for severe TO

Oral Prednisone:Oral Prednisone: effective treatment for TO. More side effective treatment for TO. More side

effects. It has been seen to induce effects. It has been seen to induce liver failure.liver failure.

Intravenous glucocorticoid pulse Intravenous glucocorticoid pulse therapy: therapy: Fewer side effects and better clinical Fewer side effects and better clinical

outcome inoutcome in Radiotherapy: benefits Radiotherapy: benefits

controversialcontroversial

Orbital decompression Orbital decompression surgerysurgery

Three major indications:Three major indications:1.1. If glucocorticoid therapy or orbital If glucocorticoid therapy or orbital

irradiation fails to halt progression of irradiation fails to halt progression of TOTO

2.2. If loss of vision is threatened either by If loss of vision is threatened either by 1.1. ulceration or infection of the corneaulceration or infection of the cornea2.2. changes in the retina or optic nervechanges in the retina or optic nerve

3.3. For cosmetic correction of severe For cosmetic correction of severe proptosis proptosis

surgery should be avoided for as long as surgery should be avoided for as long as possible until the disease stabilizes under possible until the disease stabilizes under corticosteroid suppressioncorticosteroid suppression

Orbital decompression Orbital decompression surgerysurgery

The orbit may be decompressed by The orbit may be decompressed by removing the lateral wall, the roof, or removing the lateral wall, the roof, or the medial wall and the floor. the medial wall and the floor.

Uptodate suggests the last procedure, Uptodate suggests the last procedure, also known as transantral also known as transantral decompression.decompression. the surgeon removes the floor and medial the surgeon removes the floor and medial

wall of the orbit to allow decompression. wall of the orbit to allow decompression. It does not leave a scar on the face, and It does not leave a scar on the face, and

avoids craniotomy.avoids craniotomy.

Result of decompression Result of decompression surgerysurgery

An excellent result can usually be An excellent result can usually be achieved, with substantial reduction in achieved, with substantial reduction in proptosis and edema. proptosis and edema.

However, diplopia usually does not However, diplopia usually does not improve and may worsen, so that eye improve and may worsen, so that eye muscle surgery is almost always muscle surgery is almost always needed later.needed later.

Timing of Surgery: Clinical outcome Timing of Surgery: Clinical outcome appears to be better if decompression appears to be better if decompression surgery is performed after rather than surgery is performed after rather than before glucocorticoid therapy. before glucocorticoid therapy.

Other operationsOther operations

1.1. Fat decompression surgery:Fat decompression surgery: RRemoval of the retroorbital adipose tissueemoval of the retroorbital adipose tissue

2.2. Bilateral lateral tarsorrhaphy may be Bilateral lateral tarsorrhaphy may be performed to minimize or prevent performed to minimize or prevent corneal damage.corneal damage.

3.3. Surgical recession of Muller's muscle Surgical recession of Muller's muscle and the levator will correct upper lid and the levator will correct upper lid retraction. retraction.

However, decompression surgery is However, decompression surgery is preferable for both of these problems preferable for both of these problems because it is more effective both because it is more effective both functionally and cosmetically.functionally and cosmetically.

ReferenceReference

Uptodate articles:Uptodate articles:1.1. Pathogenesis and clinical features Pathogenesis and clinical features

of Graves' ophthalmopathy of Graves' ophthalmopathy (orbitopathy)(orbitopathy)

2.2. Treatment of Graves' orbitopathy Treatment of Graves' orbitopathy (ophthalmopathy) (ophthalmopathy)

The End The End

Thank you.Thank you.