1. Case presentation 서울성모병원 안과 R2 성윤미
Let me talk about the case on “Inflammatory disease of orbit”
사은옥

2. 2004 at 전남대학교 사0옥 F/54 c/c proptosis(OS) LET 25 D&V 0 -3
Orbit MRI : LIR, LMR hypertrophy-> orbital myositis(OS)
TFT: WNL
r/o inflammatory pseudotumor(OS)
Plan) po steroid 80 mg
A 54 year-old Female patient visited on 2004.
The chief complaint was proptosis of Lt. eye
Esotropia was observed on her Lt. eye
EOM was restricted when she gazed upper & lateral on her Lt. eye
Hypertrophy of LIR and LMR was observed on MRI, so she was diagnosed orbital myositis on her left eye.
It was within normal range, her thyroid function test.
so she was prescribes steroid 80 mg
There were positive respo nse to steroid the rapy
relapse and aggrevation

3. CT : RMR, RIR, RLR hypertrophy
at 삼성병원
Exo 13/7 mm
Exo 13/10
Dsc XT 4, LhoT 25
D&V
CT : LMR, LIR hypertrophy
TFT f/u : T3, T4 ▼, TSH 정상, anti TPO Ab ▲
-> 스테로이드 중단
RT -> 2mo later
complete ptosis(OS) -> steroid 복용
1 yr later
CT : RMR, RIR, RLR hypertrophy
After 3yrs, She visited another hospital
Enophthalmos was observed on her Lt. eye
There was restriction when she look upper side on her left eye.
CT showed hypertrophy of LMR and LIR
TFT was in normal range.
Radiotherypy was tried and 2 mo later comple te pto u sis of an Lt. eye was developed. So steroid was re-used
1yr later, Enophthalmos on her left eye was aggravated.
and CT showed hypertrophy of RMR, RIR, RLR

4. 2008.10.14 c/c 눈이 돌출되요(OD) o/s) 1 mo ago
Associated Sx: Periocular pain
DM/HBP(+/+) for 3yrs, steroid induced
Ocular trauma/op(-/-)
And she visited our hospital on Octo u ber 14th, The chief complaint was proptosis of an Rt. Eye, which occu rred 1 months ago. She claimed the Sy mtom, which was also accompanied with pain
She had diabe tes & hyperte nsion d/t use of steroid for 3yrs
She had no other ophthalmic hi story.

5. Ocular Examinations VA 0.8/0.8 MRD OD 2.5/OS -0.5
Exo 11/8 mm( at base 115mm)
EOM
Her visual acuity was 0.8.
MRD1 was 2.5 on her right eye, -0.5 on her left eye.
EOM was restricted all directions.

6. Cue list Proptosis, Rt. Enothalmos, Lt. EOM limitation c pain, Lt.
Orbit CT : EOM hypertrophy, Rt.
TFT : WNL
Steroid response(+)
r/o inflammatory pseudotumor
r/o thyroid associated ophthalmopathy, less likely
r/o sclerosing pseudotumor
Plan) keep po steroid (80mg)
Thisi is the patient’s problem list
Proptosis on her Rt. eye
Limitation of EOMs and accompanied with ocular pain
Hypertrophy of Rt. EOMs in orbit CT
Normal thyroid function
Positive steroid response
Therefore, a guess could be r/o inflammatory pseudotumor, r/o thyroid associated ophthalmopathy.
We planned keep her po steroid

7. 2009.2.16 c/c 눈이 조금 떠져요 MRD1 0/0 -4 -4 EOM -4 ----- -2 -4 ----- -2
EOM
Plan) po steroid 2.5mg
4 mos later,
MRD1 was 0 on both eyes
Limitaion of EOMs was a ggravated(애그러배이티드) all directions on both eyes

8. 2010.2.17 S: RLL 부은 것 많이 호전됨 intermittent diplopia
1yr later, she complaint I ntermittent diplo u pia.
And still remained limitation of EOMs on both eyes. Especially when she gaze upper side.

9. In 9 cardinal pho u to u s,
Left exotropia was presented in the primary position.
Limitation of EOM in the both eyes were noticeable, particularly when staring at upper side, as well as
while staring at the right or the lower side.

10. Plan 1) Orbit CT c enhancement f/u 2) TFT f/u
We planned to follow up orbit CT and TFT

11. 2010.8.11 T3↓ fT4 nl TSH ↑ TPO Ab ↑ Orbit CT(enhance) showed
diffuse enlargement of the EOM at the Rt. medial,
inferior, and lateral rectus and left medial and inferior
rectus muscles
We could suspect thyroid ophthalmopathy. Because of central enlargement of muscle with a sparing of tendons
But hypothyroidism was slightly presented, in the examination of 갑상선 function.
The level of TPO a ntibody was elevated.
T3↓ fT4 nl
TSH ↑
TPO Ab ↑

12. 2010.12.20 VA 1.0/0.4 D&V -4 -4 -1 ---- 0 -4 ----- 0 0 -2
Dsc 45LXT /14LhoT
Endocrinology consultation for nl TFT but elevated TPO ab level
After 2 months,
Limitation of EOMs was shown. Especially when she gazed upper direction.
And exotropia and hypotropia were shown in her lt. eye.
We planned to consult for elevated level of TPO antibody

13. Endocrinology reply TFT T3 0.68 ng/ml ▼, fT4 0.97 ng/dl
TSH 5.80 mIU/L ▲
TSH R AB +16.0
TSI 63
Asymptomatic hypothyroidism -> synthyroxine 복용
We received a repl y from e ndo u crinology.
The decrease of T3 level and the increase of TSH were shown in the TFT follow up, which was conducted by 내분비내과.
According to the doctors of 내분비내과, they diagnosed it as asymptomatic hypothyroidism, and then, they let the patient
start to take synthyroxine.
while the positive response of TSI level should be shown in the 69% of population(probability) as a sensitive marker
in Euthyroid grave’s dz, the patient was presented the normal level.

14. 2012.02.06 Orbit CT(enhance) S: binocular diplopia(+)
levaotr resection(OS) by Pf 양석우
VA 0.8/0.4
D&V
Dsc 45 LXT, 14LhoT
TFT
T ng/ml ▼
fT ng/dl
TSH 7.61 mIU/L ▲
TSH R AB 0.08
TPO Ab ▲
Orbit CT(enhance)
She still complaint diplopia and limitation of EOMs when upper and Rt. Gaze
Orbit CT showed Asymmetric thickening of Rt medial rectus muscle compared with Lt medial rectus m.

15. 외사시 and 하사시 were observed in the left eye on the primary position
외사시 and 하사시 were observed in the left eye on the primary position. Also, 외안근 제한 was shown in cases of Upper gaze and Rt gaze.

16. 2012.10.22 VA 1.0/0.4+2 Iop 19/18 Ach R Ab 음성 Dsc 45LXT -3 -3
D&V
Plan) BLR rec + LMR res
The verification of conducting a surgery (이 의미 맞니?)
In the last v isit, because of the 외사시 in the left eye was stable condition,
we were planning to conduct a surgery. .

17. Problem list 좌안의 안검하수: 방사선치료의 합병증 좌안의 안구함몰: sclerosing pseudotumor??
좌안의 내전장애
2004년 당시에는 LMR 비후로 인한 좌안의 외전장애
2007년 기록부터 좌안의 외전장애는 없고 오히려 내전장애 기술
원인???
Ocular MG가 병발? : Ach R Ab (-), 일중변동(-)
Myositis의 급성기에는 침범된 근육의 마비가 관찰됨
2004년 보였던 LMR 비후가 현재는 많이 호전됨

18. TFT의 이상 2004년- 2007년 전남대병원 : 정상이라고 기술 : 그러나 Auto Ab 검사 유무 알 수 없음
2007년 삼성병원 : T3,4 감소,TSH 정상, TPO Ab 증가
현재 T3,4 감소 TSH 증가 TPO Ab 증가로 갑상샘기능저하증으 로 약 복용 중

19. TAO usually occurs in Grave’s diseas with hyperthryoidism, and some times with euthyroid and hypothyroidism.
but severe orbitopathy is rare in patients with hypothyroidism such as Hashimoto’s thyroiditis
according to this journal, 2 cases were reported.
In these cases MR was involved instead of IR which is usually involved in TAO.
Patients in this case report had radiation therapy and steroid therapy.
We can consider

20. Inflammatory Diseases Orbital myositis

21. Clinical Features most common nonspecific inflammatory syndrome
three profiles: isolated, recurrent, and atypical
isolated and recurrent disease
typically present with periorbital inflammation and swelling, retrobulbar pain, and pain on movement
diplopia, conjunctival injection
(often focal over muscle insertions),
and proptosis

22. Clinical Features Atypical (7%)
lack pain, lack restriction of movement
unusual or abnormal CT patterns
Progressive
associated with psychophysical changes such as optic neuropathy
require biopsy

23. Clinical Features Sudden presentation of periorbital pain
exacerbated by movement
associated with clinical inflammation
All of the vertical and horizontal muscles
involved in almost equal numbers
Underlying immunologic disorder(50%)
Antecedent flu-like illness

24. Clinical Features Isolated cases involve one muscle MR : m/c
Recurrent disease
multiple muscle involvement
often bilateral
Recurrent episodes
involvement of new muscles that differ from the primary pr esentation
Imaging
muscle enlargement associated with tendon swelling
slightly ragged appearance to the borders

25. Differential Diagnosis
Major differential diagnosis is Graves' orbitopathy
Painless in onset (unless severe and infiltrative)
Slowly progressive
Lid retraction
Limitation of gaze in the direction opposite to the muscle involved
Deterioration of visual function (color vision, visual fields, and visual acuity)
Extraocular muscle enlargement
usually fusiform and tapers toward the muscle insertion on the globe
IR>MR>SR >LR

30. Treatment Unilateral single-muscle disease
NSAIDs or relatively low-dose corticosteroids
improve rapidly and low recurrent rate
bilateral or multiple muscle disease
Recurrent↑
pulsed intravenous corticosteroids or high-dose oral cortic osteroids tapered over a 4- to 6-week period
fail to respond or persist → biopsy
persistent, recalcitrant, and recurrent disease : immunosup pressive drugs

31. 1 muscle: either with nonsteroidal anti-inflammatory drugs or moderate doses of corticosteroids (up to mg prednisone tapered rapidly on a symptomatic basis)
with bilateral or multiple muscle disease or with a significant apical component
: high-dose ( mg prednisone) or pulsed steroids (1 g methylprednisolone IV under supervision)
3. Progressive or recurrent episodes
: addition of biopsy and immunosuppressives & Other authors have recommended the use of radiotherapy