Slide Dr Eko - 1.pdf

BURAM MENDADAK dr. Eko Hadi Waluyojati SpM RSUP Fatmawati Jakarta

KONSEP DASAR PENYAKIT MATA

ANATOMI BOLA MATA

RETINA NORMAL retina

papil n. optik

macula

BURAM MENDADAK • Perdarahan • Ablasio • Stroke

vitreous

retina

mata (Central Retinal Artery / Vein Occlusion)

• Retinopati • Neuropati

optik (Neuritis/ Iskemi)

• Gangguan

jalur penglihatan (tumor, infark otak)

• Intoksikasi

Ninety percent of clinical neuro-ophthalmology is in the taking of a history (after W.F. Hoyt). Attentive listening, specific questioning and careful evaluation of the information gained make up the foundation of what is primarily a diagnostic subspecialty. The effort invested in gathering this information saves time and avoids unnecessary, potentially dangerous and/or expensive diagnostic procedures.

ANAMNESIS • Anamnesa

sangat penting

History Taking

• Mulai

kapan visus turun?

When possible, the previous records of the patient’s care should be reviewed prior to beginning the interview. Usually, if the patient will allow, it helps to include in the conversation those other persons who have come to the visit, such as the patient’s spouse or close relatives. These people can often provide information that the patient does not know or cannot remember. Patients are often anxious or fearful, and the physician can put them more at ease by conversing in layperson’s terms rather than in the technical jargon used by clinicians. When caring for children, the history taken from one or both parents should not take too long, as the success of the ensuing examination may be hampered by the impatience of the child. When necessary, one should defer some of the more detailed questioning until after the examination has been completed. The proposed schema for historical questioning, given in ■ Table 1.1, provides a rough outline of the more common details to be discussed, and those that can be compressed or expanded, depending on the details of the case. When taking the current ophthalmic history, it is of particular importance to determine as precisely as possible

• Seberapa

cepat perburukan terjadi? Progresif atau berulang?

• Satu

atau dua mata? Nyeri/ tidak?

• Riwayat

penyakit mata sebelumnya?

• Keluhan

dan penyakit lain?

• Riwayat

keluarga dan sosial?

Fig. 1.1. Characteristic onsets and courses of neuro-ophthalmically relevant clinical syndromes

Neuro-Ophthalmic Emergencies From the very start of history taking, one should be alert for clues to the presence of potentially life-threatening or catastrophically blinding disorders. The disorders in this category are listed in ■ Table 1.2, which also gives corre-

ABLASIO RETINA • Unilateral • Tidak

nyeri

• Seperti

melihat tirai menutup lapang pandang penglihatan

• Faktor

risiko myopia tinggi

• Rujuk

segera ke spesialis mata sub retina

• Semakin

lama semakin buruk prognosis

PERDARAHAN VITREOUS • Unilateral • Tidak

nyeri

• Awalnya

melihat bintik-bintik (floaters) hitam kemerahan kemudian meluas menutup lapang pandang penglihatan

• Faktor

risiko kelainan vaskular (DM, hipertensi), ablasio retina, trauma, tumor

• Rujuk

retina

segera ke spesialis mata - sub

SUMBATAN ARTERI RETINA • Unilateral • Tidak

nyeri

• Faktor • Rujuk

risiko kelainan vaskular

segera ke spesialis mata sub retina

SUMBATAN VENA RETINA • Unilateral

Central Retinal Vein Occlusion

• Tidak

nyeri

• Faktor • Rujuk

risiko kelainan vaskular

segera ke spesialis mata sub retina

Branch Retinal Vein Occlusion

RETINOPATI DIABETIK • Unilateral • Tidak

nyeri

• Faktor • Rujuk

risiko DM

segera ke spesialis mata - sub retina

RETINOPATI HIPERTENSI

• Unilateral • Tidak

nyeri

• Faktor • Rujuk

risiko kelainan vaskular

segera ke spesialis mata - sub retina

egy in Schematic Form

f vision will have its source in one of egories: optical, macular, neural, chiasmal visual pathway. There can also ed developmental amblyopia, an open gering, a functional or psychological ple exaggeration of the problem in an mize a secondary gain (■ Fig. 2.1). For gories, there are specific guidelines to clarify the nature of the problem.

Penglihatan Pasien

cal Causes of Reduced Vision

m is the corrected visual acuity. Probm the start, however, beginning with best possible correction. Despite the ated refractometers, an experienced down the wrong path. What is more, blems that cannot be detected by conclinical refraction.

Fig. 2.1. How the patient characterizes his or her visual problem depends on the cause of the impairment. For refractive errors, the eye experiences blurring of images and double or ghosting of contrasting contours. The symptoms of macular disease are dominated by micropsia and metamorphopsia, whereas optic neuropathies more commonly are described as having darker images with poor color perception

NEUROPATI OPTIK ISKEMIK ANTERIOR • Kebanyakan • Tidak

nyeri

• Faktor • Bisa Chapter 8

H. Wilhelm, U. Schiefer

Fig. 8.19. NAION in the left eye (right image) with pronounced papillary swelling, hemorrhages at the disc margin, and exudates. Typical of one risk factor, the unaffected eye has a small optic disc with indistinct margins and no physiologic cup (the so-called disc at risk)

unilateral

risiko kelainan vaskular

disertai/ tanpa sakit kepala

• RAPD

(+)

• Optic

disc pucat atau edematous, bisa dengan bercak perdarahan/ eksudat

• Optic

disc mata sebelahnya cenderung kecil dgn risiko yang sama

physiologic cup (again, the so-called disc at risk; ■ Fig. 8.19), • Defek lapang pandang altitudinal and fluorescein angiographic studies have found the afTipe arteritic terdapat fected discs are usually located in a watershed zone between •Fig. 8.19. NAION inbisa the left eye (rightpada image)usia withlanjut pronounced denganswelling, kasushemorrhages Giant CellatArteritis the disc margin, and exudates. two adjacent choroidal regions fed by separate branches of papillary the short posterior Theanterior latter vessels areoptic end Typical of one risk factor, the unaffected eye has a small optic disc Fig. 8.18: The visual ciliary field in arteries. nonarteritic ischemic with indistinct margins and no physiologic cup (the so-called disc arteries, i.e.,(NAION). they have no collaterals. Withfiber impaired neuropathy A typical arcuate nerve deficit funcin the at risk)

particles, usually stop at vascular bifurcations, and seldom move into the peripheral arterioles.

•

Pearl

3.

The patient’s complaint often gives an important clue as to where one should look when searching for an embolus. If the area of visual loss is seen in the superotemporal quadrant of the visual field, for instance, the search should concentrate on the inferonasal fundus.

•

Fig. 14.2 Cholesterol embolus lodged in an arterial bifurcation in a 65-year-old patient with abrupt onset of a visual field defect

Perimetry will reveal whether any permanent damage has been done, or whether the occlusions were short lived and reversible, with only a temporary reduction in vision. Additional Tests Fig. 14.2 CholesterolDiagnostic embolus lodged in an arterial bifurcation in a 65-year-old patient with abrupt onset of a visual field defect Angiography

Fig. 14.3 Enlarged fundus photograph of a chain of fibrin/thrombocyte emboli in a retinal arteriole

Peri been reve

Carotid Doppler ultrasonography and B-scan echography of the major arteries supplying the brain (duplex scan, ■ Fig. 14.4) have largely replaced other diagnostic methCar ods, such as ophthalmodynamometry or ocular plethysof t mography and related techniques. These are nonetheless ■ Fi simple, reliable, and noninvasive. Cerebral angiographyods is mog seldom necessary in the workup of microembolic disease. sim Magnetic resonance angiography (MRA) is a suitable and noninvasive procedure that images the large arteries of seld the Mag neck and skull base in their entirety. It is particularly suited non to the diagnosis of dissecting carotid aneurysms. nec Fig. 14.3 Enlarged fundus photograph of a chain of fibrin/throm-

to th

NEURITIS OPTIK

Optic Disc Signs and Optic Neuropathies

• Bisa

unilateral/ bilateral

• Terdapat • Bisa Optic Disc Signs and Optic Neuropathies

Fig. 8.15. At the acute onset of optic neuritis the optic disc can appear completely normal (left) or swollen

nyeri pergerakan bola mata

disertai sakit kepala

• RAPD

(+)

• Autoimun

demyelinating disease

Fig. 8.16. The visual field in optic neuritis. In many cases, one finds

•a Idiopatik, infeksi,in this Multiple poorly definedpasca central scotoma, instance affecting a left

3. Age: in a majority of cases, 18 to 45 years eye the defect extends inferiorly into the peripheral visual field Sclerosis 4. Pain on eye movement 5. Central scotomas or nerve fiber bundle patterns of •IfOptic disc normal atau edematous visual field defects the patient gives no spontaneous history of pain on eye 6. Optic disc appearance normal or with mild edema movement, it is helpful to have the patient move through 7. Tendency to begin recovery as soon as 2 weeks after •the cardinal positions of gaze, and then to ask whether this Defek lapang pandang skotoma peak visual loss at onset. might have elicited discomfort. sentral yang 8. No evidence of systemic disease (other than MS) The visual field dapat loss at themeluas time of presentation of optic neuritis has classically been described as a central scotoma Fig. 8.16. The visual field in optic neuritis. In many cases, one finds aThis poorly centraltoscotoma, in this instance affecting a left list defined is not meant imply that cases of optic neuritis do (■ Fig. 8.16). While this is a common presentation, statisti-

Signs, Symptoms, and the Diagnoses of Optic Neuropathies

thies (■ Fig. 8.15). It is helpful to separate the cases into recognizably typical and atypical forms. The typical form meets the following conditions: 1. Usually unilateral 2. Acute

Neuropati optik

A patient with the recent onset of an optic neuropathy will most often complain of a sense of darkening and loss of Table 8.2. Optic neuropathies Optic neuritis

Nonarteritic anterior ischemic optic neuropathy (NAION)

Optic nerve or chiasmal compression

Onset

Acute ~1 day

Peracute ~1 h

Age group

<50 years

>50 years

Pain

On eye movements

Mostly diffuse

Optic disc, acutely

Normal or edematous

Optic disc, chronically

Normal or atrophic

Always edematous (exception: PION) Normal, edematous, or atrophy Note ! Sectoral or generalized atrophy Normal or atrophic, Optic disc edema is evident in about one third of rarelyoptic withneuritis. persistent edemasuggests patients with acute It merely

Visual field

Nerve fiber defects, central scotoma, generalized depression

Chapter 8 H. Wilhelm, U. Schiefer

Chronic

problem was not noticed by the patient. In the latter case, No age-related difference the physician should be concerned about the possibility of commonly headache a compressive opticMore neuropathy.

damagefiber is located in the anterior porArcuate nerve fiber defects,that the site of Nerve defects, tions of the opticcentral nerve. In the ONTT cases, however, it usually inferior altitudinal depression scotoma, was found that some of the cases with disc edema were generalized depression

General symptoms

Remitting and relapsing neurological deficits

MRI

Demyelination

Therapy

High-dose corticosteroids or no therapy

surprisingly associated with MRI evidence of inflamIschemic vascular disease mation located Possible as far backendocrinopathies as the intracanalicular portion of the nerve. The differentiation between papillitis and retrobulbarReveals neuritis mass is purely descriptive, as far as Nothing unusual the optic disc findings are concerned, and has no furReduce risk factors decompression, ther meaning orSurgical consequence with respect toradiation, treatment no intervention or prognosis foror recovery.

Prognosis, usual course

Substantial recovery

Little or no change

PION posterior optic neuropathy

114

Benign tumors, visual recovery

Additional Diagnostic Testing for Patients possible with Atypical Optic Neuritis

: Definition If the optic neuritis departs from one or more of the features described above, the episode should be recognized as atypical optic neuritis.

Fig. 8.17 of known signal in

GANGGUAN JALUR PENGLIHATAN • Bisa

unilateral/ bilateral

• Bisa

disertai sakit kepala

• RAPD

bisa (+) atau (-)

• Optic

disc bisa pucat atau edematous

• Defek

lapang pandang khas sesuai gangguan jalur penglihatan

Animation 3.2

Fig. 3.4. Schematic diagram of the course of the ganglion cell axons in the region of the chiasm and the corresponding visual field defects with their frequency of occurrence. The effect of the more common lesions within the various visual pathway segments in the chiasmal region on the central 30° of the visual field are diagrammed as examples. 1 Compressive optic neuropathies. 2 Partial prechiasmal lesion. 3 Anterior junctional syndrome with subtotal damage to anterior Wilbrand’s knee, which carries the afferent signals from the contralateral inferior nasal retinal fibers, which correspond to superior temporal visual field quadrant. This pattern is often found with advanced levels of damage or even complete loss of function in the ipsilateral optic nerve. 4 Disease of the central chiasmal region. 5 Posterior junctional syndrome with damage to the posterior knee of Wilbrand, which carries signals form the ipsilateral superior nasal retinal quadrant and represents the inferior temporal quadrant of the visual field. This ipsilateral defect is associated with a homonymous hemianopia to the contralateral side. 6 Lesion of the optic tract (from Schiefer et al. 2004; see “Further Reading”)

22

Visual field defects

Pathogenesis/differential diagnosis

4.1. Loss of peripheral visual field 4.1.1 Concentric constriction ●

● ● ● ● ● ●

●

Fatigue, poor concentration, cannot understand the task of divided attention Functional/malingering Tapetoretinal degeneration Vitamin A deficiency Retinoschisis Compressive optic neuropathy (see Chap. 8) Loss of nasal nerve fibers in glaucoma or optic disc drusen (see Chap. 8) Bilateral retrogeniculate damage to the posterior visual pathways (see Chaps. 3 and 12)

Additional testing, when indicated: ● ● ● ●

●

Family history Fundoscopy Electrophysiology Tests of functional disorders, exaggeration and/or malingering (see Chap. 15) When indicated: MRI, CT, lab testing

4.3 Central scotoma ●

●

Central retinopathy – Macular degeneration – Central areolar choroidal atrophy – Cone dystrophy – Pigment epitheliopathy Optic neuropathy (see Chap. 8) – Retrobulbar optic neuritis – Toxic or nutritional optic neuropathy – Hereditary – familial optic neuropathy – Compressive or infiltrative disease

Additional testing, when indicated: ● ● ● ●

●

Family history Fundoscopy Electrophysiology Tests of functional disorders, exaggeration, and/or malingering (see Chap. 15) MRI/CT, lab testing

4.4 Paracentral scotoma ● ● ● ●

Paramacular retinal/choroidal process A small nerve fiber bundle defect Atypical retrobulbar optic neuritis Artifactual displacement of a central scotoma by eccentric fixation at the scotoma’s margin (with corresponding displacement of the physiologic blind spot)

Chapter 4 U. Schiefer, J. Schiller, W. Hart

Pathogenesis/differential diagnosis

4.9 Hemianopsias

! Note

Every hemianopic visual field defect indicates chiasmal or postchiasmal disease until proven otherwise. When first discovered, such defects require immediate investigation, on a semiemergent basis, by means of CT or MRI imaging

4.9.1 Monocular hemianopsia (rare) ●

● ●

A prechiasmal process, e.g., optic nerve compression posterior to the optic canal Paraneoplastic retinopathy Functional disorder, exaggeration, malingering

Additional testing, when indicated: ● ●

●

● ● ● ● ●

4.9.2 Binocular hemianopsias 4.9.2.1 Bitemporal hemianopsia

Swinging flashlight test Color vision testing for hue discrimination and saturation sensitivity (pseudoisochromatic plates, such as the Ishihara series, and/or color sorting tests, such as the Farnsworth D15) Fundoscopy of the optic disc and the nerve fiber layer of the retina Ocular motility Trigeminal nerve function MRI/CT Electrophysiological testing (VEP) Endocrine testing, e.g., for hyperprolactinemia, panhypopituitarism

see also Animation 3.2

Visual field defects

● ● ● ●

Trigeminal nerve function MRI/CT Electrophysiological testing (VEP) Endocrine testing, e.g., for hyperprolactinemia, panhypopituitarism

4.9.2 Binocular hemianopsias 4.9.2.1 Bitemporal hemianopsia Chiasmal disorders – see Chap. 12 ● Space-occupying lesions, e.g.: – Pituitary adenoma – Optic nerve tumor – Meningioma – Aneurysm

A complete bitemporal hemianopsia, which is relatively uncommon, may cause the so-called hemifield slide phenomenon – see Chaps. 2, 15, and 22. (Complete bitemporal hemianopsias cause a loss of all binocular vision, with each eye seeing only its nasal hemifield. In the absence of binocular sensory input to fusional vergence movements, the eyes will adopt positions dictated by the mechanics of the ocular and orbital tissues)

Inflammatory disorders, e.g.: ● Multiple sclerosis, atypical retrobulbar optic neuritis – see Chap. 8 ● Wegener’s granulomatosis ● Abscess Vascular disorders, e.g.: ● Pitutiary apoplexy ● Vascular malformations ● Cavernous sinus disease – see Chapter 10 ● Radiation neuropathy ● Trauma Additional testing, when indicated: ●

48

Please refer to section 4.9.1 in this compendium

ed nerve fibers: ). Arch Ophthal-

Papiledema

ous atedphysiologic nerve fibers: e). Arch Ophthalseiner Adnexe.

atous physiologic vererbt. Z prakt

d seiner Adnexe.

n vererbt. Z prakt

ence of optic ted intracra-

esence of optic vated intracraof leakage of sc. It is actuaxonsof eonofcell leakage aminar disc. It isoptic actuwhy atrophic lion cell axons essure is optic eleelaminar only,atrophic it still sewhy of unilateral pressure is eleye only, et it still Baron al. es of unilateral thought that a Baron et al. ved eye from s thought that he perineural olved on of eye the from oplthe or perineural acquired tion of the opral finding is al or acquired I scanning of teral finding is causes. RI scanning by papillede-of

Fig. 8.11. Early papilledema in a patient with intracranial lymphoma. The signs are very subtle. They show only a mild loss of sharpness at the disc margin and early features of obscuration of the Fig. 8.11. Early papilledema in anear patient lymphovessels by swollen neural tissue thewith discintracranial margin. When seen ma. The signs are very subtle. They show only a mild loss of sharpstereoscopically, the elevation of the disc was readily apparent. The ness at the disc margin and early features of obscuration of the diagnosis of early papilledema was later confirmed by the patient’s vesselscourse by swollen neural tissue near the disc margin. When seen clinical stereoscopically, the elevation of the disc was readily apparent. The diagnosis of early papilledema was later confirmed by the patient’s clinical course

Visual fun Optic Discfrom Signs and Opticre N the function is Visual function in the first two stages is unaff complicate from the reversible enlargement of the blind s function is common in chronic papilledema a ropathy (se complicated by a maculopathy in addition to th the result o ropathy (see below). The macular changes are th the result of traction of the retina, caused by th and scarrin and scarring of the peripapillary retina. Atro edema, byad edema, by definition, is associated with severe ible damage to the nerve. Fig. 8.13. Chronic papilledema caused by a sagittal sinus thromible damag Fig. 8.13. Chronic papilledema caused by a sagittal sinus thrombosis. The X marks visible folds in the retina. Hyperemia and capillary ectasias subsided.visible The cup folds of the disc is remains very Hyperemia and capilbosis. Thehave X marks in the retina. small Causes of Papilledema

lary ectasias have subsided. The cup of the disc is remains very small Causes Any elevation of intracranial pressure can resu

Chapter 8 H. Wilhelm, U. Schiefer

phies. Physicians speak of simple optic atrophy, when they mean that aside from the change in color, no other stigmata of disease are present, including cupping, notching of the neuroretinal rim, vascular changes, or anomalies. In speaking of ascending atrophy, one is referring to damage originating in the retina, while descending atrophy is the result of damage to the retrobulbar portions of the ganglion cell axons, comparable to classical Wallerian degeneration (see Chap. 3).

edema whether by: ■ Intracranial mass lesions elevat ■ Obstruction of the path ofAny cerebrospinal flow by a mass, stenosis, or other cause edema whe ■ Limitation of intracranial space by deform ■ Intracr skull ■ Cerebral edema caused by tumors, inflam ■ Obstru with its high magnification and comparative shallow depth toxic disorders of field. If one at■first focuses rates on the of the macula flow by Increased ofcenter CSF production associat and then on the papillary surface, the difference can be exmors or inflammatory disorders ■to achieve Limitat pressed in the dioptric power shift necessary ■ Impaired outflow of CSF, e.g., caused by in focus. This is a rather accurate the relative skull cosity of thecomparison CSF, due to of protein formation focal distances to theormacula and the optic disc surface. The inflammation ■ Cerebra reverse approach the pressure risk of in error, for th ■ offers Elevated the especially venous sinuses younger observers with toxic di CSF their ample range of accommodation. It is all too easy to exaggerate the difference when dial■ macula. Increas ing in an increase minus power to focus on In in this connection, it should bethe clear that spinal Starting at the macula and adding positive dioptric mors power o be the primary cause of papilledema. The prominent to focus on the disc minimizes the chance for error.

Fig. 8.12. Fully developed, acute papilledema in a 62-year-old man with an intracranial tumor; the appearance is asymmetrical,Retrogeniculate damage in the human is not manifested Fig. 8.14. Disc atrophy in chronic papilledema. fundus appearance; the only exception to the moredeveloped, easily seen acute in the left image, which not uncom-by any change inblurring Fig.swelling 8.12. Fully papilledema in ais 62-year-old of the disc border and the vascular sheathing indicate that ■ Impaire Note this rule is in infants and very young children, in whom ! the problem is not one of primary optic atrophy mon acute papilledema. Striking the hyperemia and capilmanfor with an intracranial tumor; the are appearance is asymmetrical, Pearl damage to the retrogeniculate pathway can produce a Advanced stages of hypertensive cosity retinopatho lary theeasily surface of in the disc thewhich obscuration of even theectasias swellingon more seen the leftand image, is not uncomRule of thumb:duce For optic any eye that is emmetropic, or trans-synaptic degeneration with resultant disc pallor. disc edema. Marked narrowing the largest retinal vessels at the disc margin, again moreand apparent or infla mon for acute papilledema. Striking are the hyperemia capil- The capillary bed of the surface of the optic disc is espenearly so, 1 mm rial of elevation corresponds to +3 diopters vessels should be a clue that the disc sw inlary theectasias left image on the surface of the disc and the obscuration of evencially easy to seeStage change in red-free light, papilledema which causes(■the ■ levels Elevate 4. Atrophic Fig.tiny 8.14): Theofdisc has in focal belength. caused by severely elevated of bloo the largest retinal vessels at the disc margin, again more apparentvessels to contrast more sharply with the surrounding tis-

•

INTOKSIKASI

• Biasanya

salah satu jenis neuropati optik

• Intoksikasi

metanol

ing drugs. Toxic Optic Neuropathies

: Definition A toxic optic neuropathy is a bilateral optic neuropathy caused by the neurotoxic effects of medications (usually chronic) or environmental toxins (acute or chronic).

122

Other risk factors for malnutrition in- from data reported by Pokorny et al. 1979) Tableeasily 17.3. measured. Common drug-induced disturbances of color vision. (Expanded clude intestinal bypass or gastric stapling for weightType II Type I loss and the hepatic cirrhosisAcquired of alcoholism. protan TreatmentAcquired deutan red/green should include intramuscular injections of high dosesred/green dyschromatopsia dyschromatopsia of hydroxycobolamine.

Medication

Antidiabetics (oral)

+

Antipyretics Note

+

!

Type III Acquired tritan blue/yellow dyschromatopsia

Phenylbutazone Cyanocobolamine

should not be used, as it has much+ Nitrofurantoin derivatives trace+cyanide than hydroxyco-+ less effectand foritschelating bolamine Nalidixic acid does. – Phenothiazine

+?

+

Quinoline and its derivatives

+

+

Quinine

+

+

Sulfonamides

+

Tuberculostatics

+

Dihydrostreptomycin

+

Ethambutol

+

Isoniazid

+

PAS

+

Streptomycin

+

Arsenic

+

Chloramphenicol

+

Cyanide

+

Digitalis

++

+

Disulfiram

+

Ergotamine

+

Erythromycin Ethanol

+ +

Lead

+

MAO inhibitors

+

Indomethacin

+

Sildenafil

+ +

Trimethadon Vincristin PAS Para-aminosalicylic acid, MAO monoamine oxidase

+

+

Indomethacin

Thallium

+

+ +

composition of their mitochondrial and variations in tients withoperable chronicDNA and unremitting (which microangi erable or poorly meningiomas canarteritis sometimes be is unthe cytochrome oxidasemanaged enzymes in their mito- Medical (e.g., the use ofexpressed nonsteroidal immunosuppressants ministration ofsuccessfully a usual) can be fou with external radiation. Traumatic Optic Neuropathy cannot be corrected. The pathogen chondria. If the optic disc is not visibly atrophic at the time cyclosporine) be of benefit. This sort is the most comtherapymethotrexate is effective inorthe treatment ofcan prolactin-secreting inherited d understood, but ischemia is thoug of the diagnosis, there is a good prognosis for visual recovof steroid sparing requires the participation of an t doses of at least pituitary adenomas, usingstrategy dopamine agonists such as bro- Definition The dia : role. The time needed to develop t ery. Atrophic optic discs mean that any treatment is probinternist/rheumatologist fortumors propercarry case management. o uniform opathy can agreemocryptine (see Chap. 12). Such a high risk causative m Fig. 8.22. Panel D-15 test of hue discrimination in a patient with ably futile. In the case of suspected methanol intoxication, in ind e, but while doses ce depends of rapid growth during pregnancy. Therefore, such casesA traumatic optic neuropathy is one caused by trauma able and is not predictable tations, us autosomal dominant optic atrophy (ADOA). In typical cases oneof vision often th MRI scanning of the brain will often show degeneration in to the optic nerve, most frequently in the setting of a sient obscurations elieve the general mpression. require frequent perimetric study. Life-long ophthalmic Chapter 8 H. finds Wilhelm, U. Schiefer 14484, and often strong impairment of blue perception us, the basal ganglia. traffic accident with cranial and/or midface fractures. edema seem to be unrelated to the Optic Neuropathy otection of vision nerve fiber follow up Compressive is important for managing this group of disorspecific or se sual impairment can begin acutely trendbeen toward the eady ders. should Ethambutol andbeot ng ! Note Definition : Traumatic optic neuropathy results primarily from indirect arcuate visual field defects that aret tworeof treatdy or a full Optic nerve and chiasmal gliomasisinthat children require Autosomal Dominant Optic Neuropathies exposure agents, heavy metals An early symptom of toxic optic neuropathy of injury, rather than by direct crushing or tearing mecha- patients with chronic nevery optic 6disc especially attentive Hue care.discrimination Regardless of whether neurofibroCompressive optic neuropathy the impairment of h. In pabi all causeopen-angle asuch toxicasopti color vision impairment. tests is like ear the ormatosis is present or not, a very cautious approach to manDefinition optic D-15 nerveorfunction by space-occupying lesions an be given the nisms. that A direct blow to the:eye can cause an avulsion of the case with glaucoma, the and central-m mtheto Farnsworth panel the Farnsworth-Munsell first priority iswhite to ide Optic Disc Signs and Optic Neuropathies llen rectus agement is needed. Radiation therapy is indicated when the mechanically compress the optic nerve or optic tract. treatment to lowoptic nerve (more properly called an expulsion). The mechfield are initially spared. The proce en 100 hue can be useful when monitoring antitubercular block further expos An autosomal dominant optic neuropathy is a heredoby the prestumor shows a tendency toward rapid growth, which, howWith only a few exceptions, these disorders are largely When the ESR has anism appears to be one of afamilial sudden,optic explosive increaseassociated in impossible to determined stop, resulting in n etherapy. Ideally, a baseline record of color vision perby Pear the neuropathy with a mutation benign neoplasms or arterial aneurysms, which are disTreatment of Compressive Optic Neuropathies emosis, soever,should is not precede the usualthe case. under 8 years ofintraocular age, al formance startInofchildren treatment. pressure with rupture of the scleral coat in a as bilateral blindness. Patients with ofchronic syndrome toxic on chromosome 3, manifested optic atrophy Manyda cussed in detail in Chap. 12. A brief review of the funTreatment of compressive lesions is mostly surgical. Inop. Chiasmal irradiation is to be avoided, given the high probabilitycircumpapillary late ring where in thechildren sclera is very thin. Most monitored by an experienced oph of tobacco–alcohol and young adults. have ba damental principles is discussed here. erable or poorly operable meningiomas can sometimes be visual field ForMalnutrition small children, a chemotherapeuticoften, ap- this occurs in patients that have moderate to high Optic py Optic complications. Neuropathies of and Neuropathies maDisc thatSigns threatens in this manner ( inOptic question islecular cyanid successfully managed with external radiation. Medical nntincluding proach to slowing the tumor growth is preferable. In many in tobacco degrees of axial myopia and/or ainposterior staphyloma. The levels(ADOA) of papilledema thatsmoke. lastdou loIn Autosomal dominant optic neuropathy affects in Definition Signs and Symptoms of Compressive therapy is effective the treatment of prolactin-secreting : ng ntly affect- cases these tumors can behave as if they are dormant, showvitamins vitam eye has no light perception, pupil is fixedThe in agonists mid-dilamust be brought under (e.g., control, ei1 Leber’s Hereditary Optic Neuropathy boththesexes equally. functional impairment of vision Chap. Optic Neuropathy pituitary adenomas, using dopamine such as brocAn optic neuropathy of malnutrition is one caused by field. Intra- ing no signs of growth for many years. A surgical approach jections of hydroxy tion, and ophthalmoscopyand reveals disappearance of the op-a high dures the penetrance of the dominantly inherited trait are acetazolamide The optic discdeveloped in antumors eye parts withisaoflogical compressive neuropathy tumors carry risk or high-dose es a dietary In the the world, e optic cato deficiency. managing exophytic in those cases can mocryptine (see Chap. 12). Such Definition vitamin reve : Adragged ticdepends disc, with folds of retina that have been through punctures are not usually satisfac highly variable. few patients willsuch have Snellen acuities as B12)acan Other besigns normal, swollen, orofatrophic. appearance of rapid growth during pregnancy. Therefore, cases as this is showing most commonly deficiency vitamin BThe 12. Such haracterisofatumor expansion. Given the small numdisease. These vit the posterior require rupture.frequent Another mechanism appears be aophthalmic While a lumbar puncture poor perimetric as 20/200,Leber’s and aLife-long fewtowill have normal acuity.the The Other here hereditary optic neuropathy (LHON) isis a essent mitoon the timing, location, and the compression. study. be cases ber are of uncommon, and pediatric are most patients oftenduration caused bybe ccasionally, cases involved, shouldof managed els of cyanide and de small-vessel the intracanalicular portion of of it needs to be repe fieldfor suffers limited damage indiagnosis, the forminitially ofrarely relative mon. They Atrophy of the disc of pallor fiber infarction follow up isofvisual important managing this group disorchondrially inherited, severe, monocular but t- mistakmacrocytic anemia. Vitamin B12 (signs and folate levelsand areloss of nerve are at tertiary referral centers. Optic disc appearance in a patient with an infiltrating viduals may be mor easily measured. Other risk factors for malnutrition in-has already bundle striations) indicates that there been presumably ders.Fig. 8.21. cecocentral depressions. The pattern seen on testing ditional, the nerve, caused by shearing of the weeks perineural type of static threat to their vision can within binocular visual loss, most commonly inan athies. The optic neuropathy and chronic myeloic leukemia composition of their clude intestinal bypass or gastric stapling foroptic weight some permanent damage to the nerve, and a fullvessels. reOptic nerve and chiasmal gliomas incan children of the central visual field mimicrequire the appearance of a optic blood This commonly happens without a fracture, nerve sheath fenestration. Thisatrop typ young men. ssive damthe cytochrome oxid loss and the hepatic cirrhosis alcoholism. Treatment covery of function will not be possible. A swollenand optic discis initially especially care. Regardless of whether (pseudo)bitemporal visual field defect, which accompanb there noattentive ophthalmoscopic abnormality. Theneurofibrofective forsometimes control of headache, affected, or Uncommon OpticofNeuropathies chondria. If the optic should include intramuscular of high doses indicates that theinjections site of compression is in or near matosis is present or anormal very cautious approach toswelling manleads to not, aismisdiagnosis ofappeara chiasmal syndrome. mild, and eye the hasorno light perception, there aThe disc andIn protect theataxia, optic nerv n RAPD in visual acuity in LHON is reduced to 20/200 or worse. Infiltrating (Carcinomatous) Optic Neuropathies of the diagnosis, ther of hydroxycobolamine. bital apex. Orbital masses (including the swollen agement is needed. Radiation therapy is indicated the central earlyRAPD. cases, the visual field can havewhen almost normal apsively scoinhe ance,rectus and there is a profound When seeing acutely xaminer to Radiation Optic Neuropathy The visual field shows aanlarge, or cecocentral ery. Atrophic optic d amuscles of Graves’ disease) are usually marked by the prestumor shows a tendency toward rapid growth, which, howpearance. Reading ability is usually good. There is a limited type I dia injured patients, as in an emergency room the perception sinOptic Atrophy afterThe Damage Definition toma, setting, and color is badly damaged. acute ably futile. In the cas ns ! Note: Definition ence of local orbital signs, such as proptosis, chemosis, so- ever, is: not the usual case. In children under 8 years of age, course of progression with age. Typically, a blue/yellow hue DIDMOA gle most important bit of objective data is the presenceby to the Tract phase is marked peripapillary microangiopathy with or Cyanocobolamine should loss, not be used,mechanical as it has much A carcinomatous optic neuropathy isoraa visual loss withOptic MRI scanning of the matosensory and/or strabismus. Chiasmal irradiation is to be avoided, given the high probability late discrimination deficit is present. Red/green deficiencies are atrophy, an Radiation optic neuropathy is a consequence of highabsence of an RAPD.an The discneuritis-like will often be normal in irregular areas of apmicrovascular dilation, tortuosity, less effect forregion chelating trace cyanide than hydroxycooptic onset, caused by carcinomatous, the basal ganglia.and compression is marked by hemianopic visual field complications. For small children, a chemotherapeutic apDefinition ent should radiation therapy delivered to the region of optic less common. The Farnsworth panel D-15 and ophthalmo-as telangiectarophy also : pearance, only to develop manifest atrophy over the ensuvariations in caliber sometimes described bolamine dose does. lymphomatous, leukemic, or inflammatory disease defects that respect the vertical meridian, often including proach to slowing the tumor growth is preferable. In many unless denerve and/or chiasm, which often presents abruptly logical testing of The the relatives of the patient will lead !to amarked ous by forms Note ingin6affectweeks. Penetrating orbital injury with direct damage atrophy damage to sias. next stage (within aOptic few weeks) isafter in(e.g., sarcoidosis) with anthey initially normal appearing contralateral, “asymptomatic” eyes, and frequently cases these tumors can behave as if are dormant, showound. a manner that mimics acute retrobulbar optic neuritis. confirmation of thedisc diagnosis (■ acteristically Fig. 8.22). Many as to theIntraoptic nerve issigns much less common. in this bilateral, though creasing optic pallor and a disappearance theFriedrei initial opticquick disc or with discTreatment edema. An of early sympto ing the central 30° (of eccentricity) of the visual field. ing no of growth for many years. A surgical approach e diagnosis The loss of vision is usually profound and there is no affected individuals are unaware that their visual function setting Despite manymicroangiopathy. attemptsis tological study in this opticthat atrophy that develops color vision impi It those is notable the microangiopathy canalicular compression of the nerve (within the optic is ca-controversial. to managing exophytic tumors cases n intracaneffective therapy to reverse the process, once it has is subnormal. There is no effective therapy. therapy usually yields quick, temthe problem, it is showing not Corticosteroid known (1)expansion. surgical decompreslesions of theofoptic tract. can be found inathe asymptomatic carriers theFarnsworth maternallyp nal) can produce visual field defects that are uncharacterissignswhether of tumor Given smallthough numhoice is the begun. porary, improvement inremoval vision. This rapidbe response is often 100 hue can be u sion of the orbit canal, (2) ofshould orbital tic, variable in shape, and slowly progressive. Occasionally, ber and/or of cases optic involved, pediatric patients managed inherited deficit. n cases of cluereferral to the pathogenesis, which often a affecting re-by identification therapy. Ideally, lesions like meningiomas arising in this region are mistakat tertiary centers. bone fragments ina contact withunderlying the nerve, or diagnosis (3) conservaLesions this last portion The can beisconfirmed of the idian. One An interval of 9 to 12 months (or more) after radiation

Neuropati Optik lainnya

•

PEMERIKSAAN

• Tajam

penglihatan (visus)

• Refleks

pupil

• Lapang

pandang - tes konfrontasi

• Funduskopi

Chapter 2 H. Wilhelm, U. Schiefer, E. Zrenner

images that parallel clearly defined contours of high contrast within images. Occasionally, patients with this problem are referred to the strabismus surgeon when the complaint of diplopia is mistaken for a binocular problem. The ghosting of images caused by faults in lens clarity will invariably improve with the pinhole aperture disc. Swinging Flashlight Test

It helps to remember that the crux of the test lies in a comparison of the pupils’ consensual responses and their corresponding direct responses. If the consensual response is consistently and clearly better than the direct response, the ipsilateral optic nerve has a relative deficit, whereas if the direct response is consistently and clearly better than the consensual response, the contralateral optic nerve has a relative deficit. Relative Afferent Pupillary Defect

Video 2.1

If an optical defect has been ruled out, the swinging flashlight test is the next step in defining the nature of the problem. It is used specifically to detect evidence of an (asymmetric) optic neuropathy. The test is conducted as follows (■ Fig. 2.4). The patient is asked to fixate on a distant object in a dark room. An indirect ophthalmoscope or a halogen bulb flashlight can serve as the light source. One should illuminate the eyes with the light source held below the level of the line of sight, elevated at about a 45° angle (so that the patient can see over and beyond the light source). Initially both eyes are illuminated from two separate distances, during which one should note whether the two pupils are equal in size and Fig. 2.4. The sequence of events during the swinging flashthey respond well to the light (see Chap. 5). If no light test. Thewhether individual photos were taken at intervals of 0.25 s. They show that the right pupil constricts visibly at anisocoria is found and pupils respond well to the light about 1 s, after the onset of illumination. When the light the is transferred to the contralateral eye, both pupils transiently stimulus, test begin. dilate to a small degree, and thisthe dilation evencan continues after the light has arrived at the contralateral eye. A relative Using a somewhat light, one eye is illuminated, afferent pupillary defect is demonstrated in the left eye.dimmer This pattern of movement is considerably easier to see in real after photographic 2 to 3 s,sequences the light is shifted quickly to the contratime than it isand when studying like the one shown here

: Definition If the swinging flashlight test detects an abnormality, one can conclude that there is a relative afferent pupillary defect (RAPD). It is said to be relative, since the defect is always detected by comparison of one eye to the other. The examiner must observe the patient rather closely during this test, since the pupillary light reactions can vary considerably. When the pupils react sluggishly, a slower transfer will allow better dilation and greater constriction on arrival at the contralateral eye. For briskly reactive pupils, on the other hand, a quicker transfer of the stimulus is more helpful. In addition, the brightness of the light and its distance from the eye can affect the extent of constriction. With a too strong (or bright) stimulus a subtle RAPD might be overlooked because the pupillary sphincter will always

LAPANG PANDANG TES KONFRONTASI

FUNDUSKOPI • Perlu

alat oftalmoskop

• Lebih

mudah bila pupil lebar

• Perlu

jam terbang

• Dapat

mendeteksi peningkatan tekanan intrakranial dan kelainan lain

FUNDUSKOPI

Chapter 4

U. Schiefer, J. Schiller, W. Hart

Compendium of Visual Field Defects and Their Differential Diagnosis Perimetri Normal perimetric findings ●

●

Visual field defects

Normal peripheral isopters plotted with the III/4e test object of the Goldmann perimeter: temporal > 90°, nasal 60°, superior 50°, inferior 60° Physiologic blind spot: eccentricity 14°, horizontal diameter 6°, vertical diameter 10°; 2/5 above the horizontal meridian, 3/5 below the horizontal meridian

Pathogenesis/differential diagnosis

4.1. Loss of peripheral visual field 4.1.1 Concentric constriction ●

Fatigue, poor concentration, cannot understand the task

Chapter 4

U. Schiefer, J. Schiller, W. Hart

Fig. 4.2. Correlation between type of scotoma and location of disease in the visual pathway: If the defect lies in the superior half of the field, the causative lesion lies in the lower half of the retina or the lower half of the retrobulbar visual pathway, up to and including the primary visual cortex and vice versa. In addition, defects in the nasal half of the visual field correspond to the temporal hemiretina; the retinal ganglion cell axons originating there do not decussate at the chiasm, but remain in the ipsilateral half of the visual

pathway. A defect in the temporal hemifield maps to the nasal half of the retina, and the axons arising there decussate in the optic chiasm, projecting to the contralateral lateral geniculate body and cerebral hemisphere. All lesions of the afferent visual pathway lie in positions that are the opposite of their corresponding visual field defect, i.e., the mapping between field defect and lesion is both horizontal and vertically reversed

examined. Given such cooperation, one can determine

Fig. 4.1. The patient complaint, “My vision is getting worse,” is consistent with many potential causes and types of visual field loss. Particularly significant is the fact that this sort of complaint can be produced by either monocular or binocular types of visual dam-

cate.

age. This makes an initial examination of the visual fields of both eyes a necessary part of a complete neuro-ophthalmic examination

29

TAKE HOME MESSAGE

• Keluhan • Diplopia

buram mendadak

Terimakasih

The Initial Encounter: Taking a History and Recognition of Neuro-Ophthalmic Emergencies

KEGAWATDARURATAN NEURO-OFTALMOLOGIS

Table 1.2. Neuro-ophthalmic emergencies and their presenting symptoms Emergency Elevated intracranial pressure

Presenting signs and symptoms ● ● ● ● ● ●

Malignant hypertension

● ● ● ● ● ●

Carotid dissection

● ● ● ●

Pituitary apoplexy

● ● ● ● ● ●

Papilledema (see Chaps. 8 and 12) Bilateral sixth nerve palsies (see Chap. 10) Acuity initially unaffected – later stages marked by transient visual obscurations Parinaud’s syndrome (see Chap. 11) Headache (increasing in recumbency; see Chap. 16) Vomiting while in a fasting state Optic disc swelling consistent with papilledema, but accompanied by signs of systemic hypertension: “Copper wiring” of arterioles Arteriovenous crossing changes Branch vessel occlusions Hard and soft exudates Visual acuity and general health initially unaffected Acute Horner’s syndrome (see Chap. 5) Excruciating pain, radiating ipsilaterally into the neck, jaw, and/or ear Spontaneous onset (predisposed in Marfan’s or the Ehlers-Danlos syndromes) After trauma (sports injuries or chiropractic manipulations) Hemianopic visual field defects (see Chaps. 3, 4, and 12) Relative afferent pupillary defect (see Chap. 2) Restricted ocular motility Trigeminal nerve involvement (nerve V1+2) Optic atrophy in advanced stages of visual loss In extreme cases, decrease in or loss of consciousness leading to coma

Beware of: ● ● ●

Brainstem compression Cardiovascular or respiratory arrest Hemorrhagic (retinal) infarcts in venous sinus thrombosis

●

Cerebral infarct Myocardial infarct

●

Embolic brain infarction

●

● ●

Subarachnoid bleeding Elevated intracranial pressure that is life threatening or potentially blinding

● ●

Pituitary apoplexy

●

Spontaneous onset (predisposed in Marfan’s or the Ehlers-Danlos syndromes) After trauma (sports injuries or chiropractic manipulations) Hemianopic visual field defects (see Chaps. 3, 4, and 12) Relative afferent pupillary defect (see Chap. 2) Restricted ocular motility Trigeminal nerve involvement (nerve V1+2) Optic atrophy in advanced stages of visual loss In extreme cases, decrease in or loss of consciousness leading to coma

●

Subarachnoid bleeding Elevated intracranial pressure that is life threatening or potentially blinding

KEGAWATDARURATAN NEURO-OFTALMOLOGIS ● ● ● ● ●

Cerebral infarct

● ● ● ●

Aneurysms

● ● ● ●

Multiple vascular occlusions Wernicke’s encephalopathy (thiamine deficiency)

●

● ● ● ● ● ●

Orbital cellulitis

Acute oculomotor paralysis with pupillary involvement (see Chap. 10) Abrupt and excruciating headache Nuchal rigidity Clouding or loss of consciousness (see Chap. 21) Numerous retinal infarcts (cotton wool exudates) in the setting of a known or suspected endocarditis, paraneoplastic disorder, or vasculitis Nystagmus Oculomotor deficits Impaired consciousness Other cranial nerve deficits Alcoholic malnutrition Parenteral administration of thiamine (vitamin B1) produces a rapid recovery

●

Painful proptosis exophthalmos (see Chap. 9) Restricted ocular motility Inflammatory optic neuropathy (see Chaps. 8, 9 and 10) Regional and systemic signs of inflammatory disease

●

Severe anterior ischemic optic neuropathy ([AION], see Chap. 8)

● ● ●

Giant cell arteritis

Signs of elevated intracranial pressure (see Chaps. 8 and 12) Symptoms of hemiplegia or hemiparesis (see Chap. 21) Ocular motility disturbances Impairment or loss of consciousness

●

●

●

Elevated intracranial pressure that is life threatening with loss of vital brain centers for respiration, thermoregulation and/or circulation Subarachnoid hemorrhage

●

Cardiac arrest Malignancies Life-threatening cerebral infarcts

●

Death by multiorgan failure

● ●

●

●

Septic cavernous sinus thrombosis (particularly dangerous: mucormycosis) Blindness and/or life-threatening

Wernicke’s encephalopathy (thiamine deficiency)

● ● ● ●

Nystagmus Oculomotor deficits Impaired consciousness Other cranial nerve deficits Alcoholic malnutrition Parenteral administration of thiamine (vitamin B1) produces a rapid recovery

●

Death by multiorgan failure

KEGAWATDARURATAN NEURO-OFTALMOLOGIS ● ●

Orbital cellulitis

● ● ● ●

Giant cell arteritis

Painful proptosis exophthalmos (see Chap. 9) Restricted ocular motility Inflammatory optic neuropathy (see Chaps. 8, 9 and 10) Regional and systemic signs of inflammatory disease

Severe anterior ischemic optic neuropathy ([AION], see Chap. 8) ● Pain and tenderness in the temples or scalp, aggravated when combing or brushing hair ● Jaw claudication Chapter 1 U. Schiefer, H. Wilhelm ● Ocular motility deficits (rectus muscle ischemia) ● Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) markedly elevated Table 1.2. (Continued) ● Anorexia Emergency Presenting ● Malaise signs and symptoms

Whipple’s disease

●

●

● ●

Botulism

● ●

●

Rhythmic oculomasticatory movements are a pathognomonic disturbance of ocular motility: rhythmic convergence movements in synchrony with movements of the jaw and pharyngeal musculature Cause: bacterial enteritis (Tropheryma Whippelii) Clinical scenario: presents as a malabsorption syndrome Initially symptoms of a gastroenteritis (nausea, vomiting, constipation) starting 4 days after exposure (eating spoiled food) Subsequent bilateral pupillary paresis with reduced light responses and complete paralysis of accommodation (see Chap. 5), eventually developing a complete external ophthalmoplegia Further systemic paralysis, including the pharyngeal and respiratory muscles, and xerostomia

●

●

Septic cavernous sinus thrombosis (particularly dangerous: mucormycosis) Blindness and/or life-threatening myocardial or cerebral infarction

Beware of: ●

● ●

Disease leads to death, when untreated, but curable 3 with antifungal agent (clotrimazole)

Death by respiratory failure Also wound botulism (puncture wound with deep anaerobic sepsis). Note: The entry wound may have already healed or may have been forgotten, making it difficult to find

and ocethe hat the the nectrarbit out-

meore ues rect

•

•

Diagno

■ Table 9

used for A rat testing in raphy is Study of standoff bath tha cannot b tissue pe the soun bony orb ful when vascular commun the direc bital vess sive meth ment of o sies, and sues. The nature of tissues, fo include t quicker a advantag a limited

! No

.1

ing otilory the

bleeding. This is a common cause of bilateral fourth nerve Chapter 9 S. Pitz Exophthalmos palsies. The inferior orbital fissure is connected to the superior Table 9.2. The causes of lid retraction Pathogenesis Graves’space Disease confined within the bony orbit limits the expanorbital fissure. Through its cleft is a communication be- Theof Diplopia Disease Pathogenesis Graves’fossa. disease sion is anof autoimmune disorder. In 85% cases through its orbital contents, which thenofprolapse tween the orbital contents and those of the pterygoid there drains is initiallyanterior aGraves’ simultaneous hyperthyroid disease driven byof sympathetic opening.autoimmune This is theInitially signal feature many orbital It contains the inferior ophthalmic vein, which in due Müller’s muscle, Binocular diplopiablood can arise different state; the in 10% cases, a Pulsating hypothyroid state; hypertonus mostisofa the exophthalmos common accompanifromby thethree inferior orbitalmechanisms: structures, including in- ofdiseases. later by the inflammatory fibrosis by displacement of theportions globe, by of the theinferior motor vortex to Hashimoto’s thyroiditis. In thedefects remaining 5% ofmyositis cases, of structural inofthe sphenoid wings (Neurofiferior of damage the globeto(via veins). ment chronic cranial nerves, orIt by damage thethe extraocular early stages bromatosis) of Graves’ disease develop in disorders the absence ofarteriovenous or with vascular like then emptiestointo pterygoid muscles plexus. Thisthe soft tissue Dorsal midbrain disease Loss of inhibitory supranuclear any detectable dysfunction. (e.g., Graves’ disease, chronic progressive external fistulas. The pulsesign) synchronous of thenuclear globe is passage between the orbit and theophthalpterygopalatine fossa thyroid (so-called Collier’s inputmovement to the third nerve complex tonometry, when the moplegia, or myasthenia). provides a pathway of least resistance for the spread of most easily seen during applanation Diagnosis Graves’ Disease applanated circle surroundedMechanism by the tearthought film meniscus can pathologic processes, including bacterial infections and of Intracranial hypertension to be similar to thea effects of dorsal diagnosis disease, aside from history of midbrain be Graves’ seen pulsing. malignant cancers. The volume of venous flow The through the of disease inferior fissure is smaller, more variable, and less important thyroid problems, uses measures of visual acuity, pupillary Changes of Lid Position Note glaucoma drugsThe configuration Sympathomimetic !Topical than the flow through the superior ophthalmic vein. This light responses, and ocular motility. and effect (epinephrine, Dipivefrin, Pseudoexophthalmos a term tostudied. describe an apparent passage also contains the infraorbital nerve, which arisesof theclonidine/apraclonidine) movement lid margins should beiscarefully Fig.Ptosis 9.5. A patientfrom with the Graves’ disease. Striking proptosis, left forward protrusion of the caused maxillary division of the trigeminalThe nerve and examination slit-lamp should include theglobe measure of by primary ■more Table 9.1right, provides an overview of the potential causes than with bilateral retraction of both upper and lowerof Mechanical retraction Surgical effect, mass effect ocular disorders, suchtonometry as high axial myopia, passes through the foramen rotundum in the skull base and pressure intraocular applanation both in rather than of the by upper lid eyelids, and severe conjunctival hyperemia ptosis. byprimary pressureposition. on the Because globe from enlarged then through the inferior orbital fissure. It courses alongand in the downgaze of the fore- retrobulbar structures. the orbital floor in a groove on the dorsal surface of therectus muscle’s shortened traction on the globe, attempts to maxillary tissue,theand Table 9.1. The causes of ptosisroof, partly covered by connectiveforce eye into the primary position often result in a then through theand infraorbital canal to innervate the lower Lid Retraction adults, and it is usually a bilateral often very asymmetmarked, though transient, elevation of the intraocular presCongenital ptosis lid Can be isolated, associated and portions of the upper cheek. Diplopia ■ Table 9.2and lists the disordersdetermination that commonly cause lid ric disorder. lead misdiagnoses during sure. Visual field testing a sonographic (■ Fig. 9.4) This canwith antoipsilateral elevator palsy, the early stages of the disease.or Pearl of rectus muscleretraction. thickness by A-scan complete the workup. with the familial fibrosis syndrome, Binocular diplopia can muscle arisemust by thickening three mechanisms: withcommonly mandibulopalpebral synkinesis Pseudo–lid retraction be different ruled inout (unilateral Initial symptomsormost include foreign-body Echographic confirmation of rectus (The Marcus-Gunn jaw-winking Fractures of the orbital in additionthe to midportions causing by thewith globe, by damage to of theormotor because of compensation forno a contralateral bilatsensation, a feeling of retrobulbar pressure or floor, pain, tearing, ofdisplacement muscle belly,ofbut thickening atptosis, phenomenon) mechanical and paralytic disturbances of ocular motil- cranial or caused by damage to the extraocular muscles eral lid nerves, retraction by such as blurring, and photophobia. With later inflammatory in- the tendinous insertions, is characteristic of hypersympathotonia, Graves’ disease ity, also cause hypesthesia or complete somatosensory (e.g., Graves’ disease, chronic progressive external ophthalin thyroid orin panic attacks). Acquired ptosis Involutionalmuscles, (age-related dehiscence volvement of the extraocular diplopia begins to and differentiates it from storm, orbital anxiety, myositis, which the inloss the ipsilateral incisors, gingiva, cheek, and the moplegia, or myasthenia). of theinlevator tendon), innervational appear. All of these symptoms have a diurnal cycle, with the flammatory swelling extends all the way to the point of Pearl (third nerve palsy, of Horner’s syndrome), mucosal surface the upper lip. most symptomatic period at the time of awakening in the insertion. In cases that remain in doubt as to the correct myasthenic, or with chronic progressive LiftingCT the scan ptoticoflid allow normalization of the remorning. In addition, and virtually pathognomonic, is the diagnosis, a thin-section thewill orbit helps to rule external ophthalmoplegia, chronic use Changes of Lid Positioncontralateral to a monocular Theoforbital connective tissues are thin and delicate, and tracted upper contact lenses, topical preparations appearance of lid signs, including lidorswelling, lower lid re- out a mass lesion other than onelid or position more swollen muscles. they are difficult to demonstrate during surgical proceptosis. of corticosteroids traction, upper lid retraction with lid lag (not moving in High-resolution MRI scanning with determination of the Ptosis dures. Nonetheless, they provide significant barriers to the close synchrony spread with the globe during downward pursuit T2-relaxation time produces images can be used to 9.1 provides an that overview of the potential causes of of diseases between the various compartments that ■ Table movements), andthey infrequent blinking or aisstaring expresjudge thethe waterptosis. content in the rectus muscles, a correlate form: Tenon’s capsule a barrier that extends from Loss of Vision 129 for inflammatory edema. sion (■ Fig. 9.5). limbus These facial features are for many patients to the dural sheath of the optic nerve, separating the

structures. Chapter 9 S. Pitz

Video 9.1

the and wer

Patien scann cause heat o

Neuro-Ophthalmic Aspects of Orbital Disease

Table 9.3. Diagnostic testing for a suspected orbital mass

Massa intraorbita

Signs and symptoms

Methods

Remarks

“My glasses no longer work”

Manifest refraction

Lenticular myopia of uncontrolled diabetes? Axial hyperopia due to proptosis by orbital mass?

Axial proptosis

Hertel exophthalmometry

Mismatch between eyes of up to 2 mm is within normal limits – there is high statistical variance between examiners and examinations. Always compare to base line measures.

Axial proptosis

View eye position of patient from above and behind, looking down over the patient’s brow

Interocular differences >2 mm can be easily seen. Helpful when Hertel values show large interocular mismatch.

Horizontal or vertical globe displacement

Ruler Kestenbaum glasses

Compare with old photos of patient

Globe displacement

Palpation

Symmetrical resistance to retropulsion? Is there a bruit? Pulsatile exophthalmos? Crepitation?

Bruit

Auscultation

Murmur

Pupillary motility

See Chap. 5

Ocular motility

See Chaps. 10 and 11

Forced duction tests when indicated

Interpalpebral fissure

10–12 mm

Measure at primary position when patient is fully relaxed; For monocular ptosis or lid retraction, measure eyes individually

Compare upper lid positions in downgaze

Watch out for pseudowidening of lid fissure when contralateral to a ptotic lid; Vertical mismatch in downgaze (Graves’ congenital ptosis – postsurgical shortening of upper lid)

Levator function

12–18 mm

Maximum lid excursion (from full downgaze to full upgaze positions) while restraining frontalis movements (Fix the forehead position in place with your hand placed horizontally across the brow)

Intraocular pressure

Applanation tonometry

Interocular mismatch? Gaze direction effect on interocular pressure (IOP): Measure IOP in primary and upgaze positions. When elevation is markedly limited, measure IOP in partial downgaze versus partial upgaze attempt.

Prominent episceral vessels

Slit-lamp examination

Vascular distention by obstruction to orbital venous outflow?

Fundus

Ophthalmoscopy

Retinal/choroidal obstruction to venous outflow Optic disc edema, choroidal folds

Sakit kepala pada neuro-oftalmologi

Flow diagram. Diagnostic procedures for the workup of chronic headache

will immediately reveal the location and the nature of the disease process. CT and MRI scans, however, do not release the physician from his/her obligation to classify disorders according to their clinical presentations and to judge whether an imaging procedure is necessary. The neuroradiologist must determine the anatomic region within which

1. Fast eye movements (volitional refixation saccades) 2. Pursuit movements (and/or optokinetic nystagmus [OKN]) 3. Vestibulo-ocular reflex (VOR) 4. Near convergence

Kelainan pergerakan bola mata

Fig. 11.2. Flow diagram. From symptom to site to cause: a guide to the diagnosis of eye movement disorders.

The ophthalmologist should keep in mind that the differential diagnosis always includes the chance that a brain tumor might be at fault. It is not at all unusual for these potentially life-threatening diseases to present with purely ophthalmologic symptoms.

Massa intraorbita

Penglihatan buram berulang

Flow diagram. Sequence of diagnostic testing in the work up of suspected central nervous system causes of transient visual loss

ter 5

H. Wilhelm, B. Wilhelm

Prosedur diagnostik pada kelainan pupil

Diagnosis of Pupillary Disorder

57

6

Poster 4.2

recogniz typical p with the

Conclu

The phys metric d from thi a specifi the affer

Kelainan Perimetri karena Glaukoma

Furt

Fig. 4.9. Perimetric staging of glaucomatous nerve fiber bundle defects (modified from Aulhorn et al. 1977)

Aulhorn E matou Bajandas Slack, Gloor B (1 matis Harringto ical pe Kaiser HJ, Kölmel HW physi New Y Lachenma tions. Schiefer U Teil 1

■

Optic Disc Sig

Papil optik

The espe that sels

Narrowing of arterioles Fig. 8.1. Comparison of a large optic disc with a large physiologic cup■ (left) with a disc showing the pathological cupping ofi.e., glauco-the Regularity of caliber, with ma (right). The anomalous disc has a preserved rim of healthy tissue that is and widest atadjacent the inferior pole and most narrow in the temthe veins (normally a poral quadrant. In the glaucomatous optic disc this pattern is not seen, pathological cup extends all way to the margin ■and the Notes relative tothepathological andc of the disc disc of veins by arteries acu ■

•

Fig. 8.1. Comparison of a large optic disc with a large physiologic cup (left) with a disc showing the pathological cupping of glaucoma (right). The anomalous disc has a preserved rim of healthy tissue that is widest at the inferior pole and most narrow in the temporal quadrant. In the glaucomatous optic disc this pattern is not seen, and the pathological cup extends all the way to the margin of the disc

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pro vess of n spo whe is a sure puls abo Also eyes gen with puls

Pearl

A helpful mnemonic is the ISN’T rule: The breadth of These are common and important the neuroretinal rim is normally greatest at the Inferior disc margin, progressively narrower at the Superior especially hypertension and and diabet Nasal margins, and is narrowest at the Temporal disc margin.there If this rule met, the examiner should betw that is isanot close association consider that the possible presence of a pathological sels reduced form ofand cupping is more likely. rates of survival. A disc with no cup, frequently in combination with Very narrow, thready arteriole a smaller disc diameter, is thought of as a so-called disc at risk, as there is an association between this anatomiwith tapetoretinal degenerations, a cal anomaly and the likelihood of suffering damage from anterior ischemic optic neuropathy (AION; see the same prognostic significance. below). lary Capillary ectasias (prominent shu ano Optic Disc Size the disc and obscuration and near cal disc margin (early sign of edema) Similar to the color of the disc, the size cannot be quantita- The tively determined by conventional ophthalmoscopy. Blood men acutely developing V vessels at and close to the disc can bepapilledema. used to provide a the subjective comparison. (Vessels that appear to be too large the proximal retinal venous tree, usual for the disc are often a sign that the disc is unusually small, eyes andvessel vice versa.)as In addition, any ametropia shouldlamina be taken but it turns into the cr into consideration. Large eyes with axial myopia have discs sion por thatof appear larger during direct ophthalmoscopy, whereas (IC normal intracranial pressure

ISN’T rule!

Pearl A helpful mnemonic is the ISN’T rule: The breadth of

aphakic eyes have discs that appear smaller.

are

Fig. 8.18: The visual field in nonarteritic anterior ischemic optic neuropathy (NAION). A typical arcuate nerve fiber deficit in the lower half of the visual field in a right eye

AIONAnterior Ischemic Optic Neuropathy : Definition Anterior ischemic optic neuropathy (AION) is charac-

terized by the following triad: abrupt (usually monocular), painless loss of vision; optic disc swelling with or without surface hemorrhages and cotton-wool spots; and nerve fiber bundle defects in the visual field of the affected eye (■ Fig. 8.18). An important distinction must be made between the arteritic and nonarteritic forms of AION, in which systemic vascular diseases and local risk factors play a defining role. Signs and Symptoms of Nonarteritic Anterior Ischemic Optic Neuropathy

The average age of patients with acute nonarteritic anterior ischemic optic neuropathy (NAION) is about 61 years. The visual loss occurs abruptly and without a prodromal warning, usually while the patient is asleep or within a 12-h period, and there is no associated pain or headache. The initial swelling of the optic disc subsides within 2 months,

Fig. 8.19. NAION in the left eye papillary swelling, hemorrhages a Typical of one risk factor, the unaf with indistinct margins and no ph at risk)

physiologic cup (again, the so-c and fluorescein angiographic fected discs are usually located two adjacent choroidal region the short posterior ciliary arter arteries, i.e., they have no coll tion of the autoregulation of s sient periods of systemic hypo duce sufficient ischemia withi optic disc. The prelaminar port depends on the arterial supply Relative ischemia that lasts fo can result in an infarction of th to occur most commonly wh asleep, i.e., when mean arteria mum.

Treatment of Nonarteritic Optic Neuropathy

If there is no suspicion of an ar workup by the patient’s interni

CURRICULUM VITAE • Dr. Eko • Lahir

Hadi Waluyojati, SpM

di Semarang, 15 April 1979

• Dokter

Umum FKUI Lulus 2004

• Dokter

Spesialis Mata FKUI Lulus 2010

• Fellow

Fakoemulsifikasi FKUI 2010

• Anggota • Fellow

SMF Mata RSUP Fatmawati 2011

Neuro-Oftalmologi FKUI 2011