망막 컨퍼런스 R3 박율리/ AP.박영훈

CASE 61/F 최O집 C/C: Ocular pain c proptosis(OD) Past Hx DM/HBP (+/+) for 30/30 yrs, po medi HD d/t DM ESRD 6년 전 심장 판막 이상으로 심장 수술함(순천향대학병원) Ocular op/trauma(-/-) Gls (-) Eye drop (-) 최혜집 25168352

Ocular exam VA: OD 0.16 (0.2) OS 0.5 (1.0) IOP: OD 19 OS 16 mmHg by AP Lid OD mod. swellling OS No swellling Conj. OD mod. injected c corkscrew vs. c mod.chemosis OS not injected Cornea OD rl.clear OS clear AC OD 3CT ar center, <1/4CT at pph & cell(-) OS Deep& cell(-) Pupil OD round & nl sized, LR(+) OS round & nl sized, LR(+), RAPD(+-/-), NVI(-/-) Lens OD mild cortical opacity c mild nucleosclerosis OS mild cortical opacity c mild nucleosclerosis Ocular bruit(+-)

proptosis, hyperemic conjunctiva, and chemosis proptosis, hyperemic conjunctiva, and chemosis. Slit lamp examination revealed tortuous conjunctival and episcleral vessels tortuous corkscrew-type blood vessels in the conjunctiva that come up to the limbus and appear different from those seen with other causes of red eye. In contrast to generalized conjunctivitis, these vessels are localized to certain areas, such as the interpalpebral conjunctiva.

Fds OD nl. optic disc c multiple retinal Hm. at 4Q c ME(+) OS nl. optic disc c thin ERM

MRI On CT, there is dilation of the superior ophthalmic vein and mild right-sided prop-tosis (2). The MRA demonstrates the abnormal signal in the left and posterior right cavernous sinus (3,4). enlargement of extraocular muscles 1. Distension of Rt. superior ophthalmic vein 2. Soft tissue swelling in right orbit, either by inflammation or congestion. 3. Fluid collection with diffusion restriction in the Lt. maxillary sinus with dark SI involving Lt. maxillary sinu, maxillary infundibulum, and hiatus seminlumaris, accompanying destruction of Lt. med. orbital wall : RO fungal sinusitis. 4. A small cystic change in left frontal periventricular region, suggesting old lacunar infarction. 5. Probable reversed venous flow at left internal jugular vein and sigmoid sinus (left).

Diagnosis & plan Diagnosis Plan R/O CCF, Rt. R/O CRVO c ME NS consult FAG O-MOXx4, O-LONx4, O-DCFx4 TAGF IOP 28/10 NS consult -> 의뢰하신 환자 진료 잘 보았습니다.dural AVF, cavenrous type으로 입원 검사후 치료 예정입니다 신용삼 드림 severe conj engorgeAC cell (-)R/O CRVO c ME-->massive preR H--> VH ODR/O CCF --> dural AVF 입원합시다.Fd-photo 360 도투석NS, nephro consultorbit MRI

1wk later VA HM/0.8 IOP 28/10 Severe conj engorge AC cell (-) VH /OD R/O CCF --> dural AVF <Plan> Adm. O-MOXx4, O-LONx4, O-1ATx2 /OD O-COSTx2, O-BMDPx2, O-TVTxhs /OD NS consult : dural AVF, cavernous type으로 입원 검사후 치료 예정.

FAG

HD #2 VA HM/ 1.0 IOP 19/ 16 mmHg (O-COST x2, O-BMDP x2, O-TVT x hs / OD) Lid OD mod. Swellling OS No swellling Conj. OD mod. injected c corkscrew vs. c mod.chemosis OS not injected Cornea OD rl.clear OS clear AC OD 3CT ar center, <1/4CT at pph & cell(trace) OS Deep& cell(-) Fds OD blurry invisible d/t vit. hem HE 30 HD O-MOX x4, O-LON x4, O-1AT x2, O-TMO O-COST x2, O-BMDP x2, O-TVT x hs / OD ENT consult : 비강 내 특이소견 없으나 MRI 이상소견으로 추후 F/U예정

HD #5 VA HM IOP 18 mmHg (O-COST x2, O-BMDP x2, O-TVT x hs / OD) Lid OD severe swellling Conj. OD mod. injected c corkscrew vs. c mod.chemosis -inc. Cornea OD rl.clear AC OD 3CT ar center, <1/4CT at pph & cell(rare) Fds OD blurry invisible d/t vit. Hm Cavernous type dural arteriovenous fistula with inflow from both ICA/ECA and outflow to right superior ophthalmic vein (thrombosed) Performed embolization using 5 detachable coils and 21 fibered pushable coils Intravit. Avastin inj.(OD) HE 30 HD O-MOX x4, O-LON x4, O-1AT x2, O-TMO O-COST x2, O-BMDP x2, O-TVT x hs / OD -> coil embolization위해 NS transfer

After embolization (HD #9) “ 많이 나아졌어요’ VA HM IOP 19 mmHg (O-COST x2, O-BMDP x2, O-TVT x hs / OD) Lid OD mild swellling Conj. OD mod. Injected Cornea OD rl.clear AC OD 3CT ar center, <1/4CT at pph & cell(rare) Fds OD blurry invisible d/t vit. Hm conjunctival hyperemia and proptosis were improved HE 30 O-MOX x4, O-LON q2hr, O-1AT x2, O-TMO O-COST x2, O-BMDP x2, O-TVT x hs / OD ppV 예정

Operation 2012.04.17 ppV+ppL+Endolaser+FAE+C3F8 gas inj.(OD) by AP.박영훈

POD #1 VA FC20cm(N-C) IOP 12 mmHg (O-COST x2, O-BMDP x2, O-TVT x hs / OD) Lid OD mod swelling c erythema Conj. OD severe injected c chemosis, well-approximated wx., leakage(-) Cornea OD mild edematous c DM folding, total epidefect(+) AC OD 3CT ar center, <1/4CT at pph & cell(+) Lens OD Aphakia, iris pigment (+) on ant. surface Fds OD seems to be nl optic disc c multiple retinal hm. c well applied endolaser scar at 360 degree, gas 90 % sitting 취침시 HE 45 도 이상 O-MOX x4, O-LON x4, O-1AT x2, O-TMO O-COST x2, O-BMDP x2, O-TVT x hs / OD H-LON 30mg

POD #12 VA (0.1) IOP 8 mmHg (O-COST x2, O-BMDP x2 / OD) Fds OD flat

REVIEW

Risk factors Systemic vascular diseases DM, Hypertension, carotid insufficiency Ocular diseases Open angle glaucoma, ischemic optic neuropathy, pseudotumor cerebri, tilted optic nerve heads Hematologic alterations Hyperviscosity syndromes, dysproteinemias, blood dyscrasias, anemia, elevated plasma homocysteine, factor XII deficiency, anti-phospholipid antibody syndrome, activated protein C resistance, protein C or S deficiency Inflammatory/autoimmune vasculitis : SLE Medications : oral contraceptives, diuretics, Hepatitis B vaccine Infective vasculitis : HIV, syphilis, herpes zoster, sarcoidosis

CCF 발생기전 유발원인 아직 명확히 밝혀져 있지는 않음 선천질환 ? 해면정맥동내에 정맥혈전이 형성된 후 경막동맥의 얇은 벽이 파열되어 동정맥루가 형성 - 폭넓은 지지. 유발원인 고혈압, 동맥경화 등의 혈관질환 Ehlers-Danlos 증후군과 같은 교원질환 드물게 수술이나 경한 외상 후

During early phase visual impairment is initiated by stagnant-flow anoxia -> ischemia of optic nerve, mechanical, axonal conduction block of optic nerve caused by elevated IOP or compression of optic nerve or chiasm by distended cavernous sinus retina appears normal visual impairment is reversible

if high pr. generated by fistula continues irreversible optic neuropathies, optic nerve atrophy, infarction,trauma to nerve CCF with visual impairment, fistula obliteration is urgently required to prevent irreversible change when vision worsen to blindness, recovery is not possible despite successful CCF embolization

증상 결막충혈, 안구돌출, 복시, 시력저하, 안구운동마비 등 안과적 증상이 가장 흔함 주로 중년 이후의 여성 드물게 남성, 소아, 영아 합병증 폐쇄각녹내장,망막중심정맥폐쇄, 증식성 망막병증, 허혈성 시신경병증 뇌피질정맥 으로 배출로가 형성되어 뇌내출혈 증상 결막충혈, 안구돌출, 복시, 시력저하, 안구운동마비 등 안과적 증상이 가장 흔함 두통, 이명, 청진상 잡음

Orbital Sxs: retrograde venous flow or pressure of CS -> elevation of venous pr. in orbit chemosis, exophthalmos, bruit, eyelid swelling, decreased vision, increased IOP, ocular pain, retinal hemorrhage ٭ Visual loss mech. 1. retinal ischemia blood away from ophthalmic a. in high flow 2. marked elevation of intraorbital venous pr. -> reduce AV gradient in retina -> compromise retinal perfusion

Cavernous Sx: steal phenomenon of blood supply to CN bulging or elevated pressure of CS ptosis, diplopia, anisocoria, ophthalmoplegia 6th palsy (m/c in Type A, location) ٭ diplopia mech 1. cranial n. dysfx. resulting from arterialized pr. within CS and ischemia of the vasa nervosum 2. restriction of extraocular m. (vascular engorgement of m.) Cerebral Sx: reflux into petrosal vein into perimesencephalic, cerebellar vein hemorrhage, infarction

이러한 증상을 결정하는 요소로 특히 해면정맥동에서의 배출로의 방향이 중요. 이러한 증상을 결정하는 요소로 특히 해면정맥동에서의 배출로의 방향이 중요. 후방의 상추체정맥동(superior petrosal sinus)이나 하추체정맥동 (inferior petrosal sinus) 안구운동신경의 마비나 외안근의 이상으로 복시가 나타나기도 하나 대부분 무증상 전방의 상안정맥 대부분 결막충혈, 안구돌출, 이명과 같은 증상과 안압상승, 망막혈관 확장 .

Diagnosis CT MRI MRA Cerebral arteriography relatively low sensitivity for detection of AVMs calcification and hypodensity may be noted MRI more sensitive in detection of small hemorrhagic foci ass. with AVM cavernous sinus tumors that may mimic CCF MRA demonstrate feeding a. and draining v. Cerebral arteriography ‘‘gold standard’’

Barrow et al, 1985 (based on angiographic criteria(a. supply pattern) Type A (Direct) direct shunts btw the ICA and CS mainly due to trauma (including iatrogenic) intracavernous ICA aneurysm abnormalities in collagen deposition and the media of a. (Ehlers-Danlos syndrome, fibromuscular dysplasia, pseudoxanthoma elasticum) high-flow lesion not to resolve spontaneously Type B,C,D (Indirect); dural AVF based on source of the feeding arteries cause:unclear high or low flow likely to spon. thrombosis therapy; Sxs, drainage pattern

Type B feeding vessels from the intracavernous ICA Type C from only the external carotid artery Type D from ICA and external carotid artery

Treatment cause symptoms and clinical course angiographic features Decision: cause symptoms and clinical course angiographic features patient’s medical condition manual carotid compression microsurgery endovascular treatment radiosurgery combine