Differential Diagnosis of Optic Disc Swelling Hyun Ah Kim

(1)

Differential Diagnosis of Optic Disc Swelling

Hyun Ah Kim

Department of Neurology, Keimyung University Dongsan Hospital, Daegu, Korea

Differential diagnosis of optic disc swelling

Keimyung University Dongsan Hospital Department of Neurology Hyun Ah Kim

Normal optic disc

Pseudodisc edema

Optic nerve drusen : disc elevation Medullated nerve fibers : blurred margins Morning glory syndrome : elevated disc Tilted disc : blurred margins

Bergmeister’s papilla

(2)

Clin Neuroophthalmol 9(Suppl 2):S115-122, October 2019 S116 http://neuro-ophthalmology.co.kr

Optic drusen

Papilledema Blurred edges Disc elevation

Lack of a physiologic cup Altered contour

The hemorrhages generally follow the nerve fiber bundles Flame like hemorrhage

Buried drusen Calcified deposits In the second and third decades

Deep or buried drusen are difficult to distinguish from true disc edema

Cause an anomalous branching pattern and peripapillary atrophy.

Circumferential hemorrhage

Optic drusen

Superficial drusen

• Superficial drusen

Buried drusen

Drusen Fluorescein angiography

Papilledema Drusen

Medullated nerve fibers Morning glory syndrome

(3)

Tilted optic disc

Congenital anatomic variant The optic nerve enters the eye at an oblique angle and is rotated on the anterior-posterior axis Frequently bilateral M/C : elevated superotemporal disc and flattened inferonasal disc Peripapillary atrophy and situs inversus of the retinal vessels (vessels that exit nasally before turning temporally)

Myopia, astigmatism, and visual field defects (particularly superotemporal visual field loss)

Bergmeister’s papilla

Differential diagnosis of optic disc edema

Unilateral

1. Neoplastic (optic nerve glioma, optic nerve sheath meningioma) 2. Inflammatory (demyelination, sarcoidosis, systemic lupus erythematosus) 3. Infectious (cat-scratch disease, syphilis)

4. Metabolic/toxic

5. Hereditary (Leber hereditary optic neuropathy) 6. Ocular (uveitis, hypotony)

7. Vascular (NAION, arteritic anterior ischemic optic neuropathy, perioperative anterior ischemic optic neuropathy)

Bilateral

1. Malignant hypertension 2. Mass (intracranial)

3. Mucked-up drainage (venous sinus thrombosis, hydrocephalus) 4. Medications (tetracycline, steroid withdrawal)

5. Meningitis (cryptococcal) 6. Morbid obesity (IIH)

(4)

Arteritic vs. nonarteritic AION

Arteritic vs. nonarteritic AION Arteritic vs. nonarteritic AION

International Ophthalmology Clinics 2009

(5)

International Ophthalmology Clinics 2009 International Ophthalmology Clinics 2009

International Ophthalmology Clinics 2009

(6)

Papilledema

A specific form of disc edema resulting from

elevated intracranial pressure

Generally exhibits a minimal acuity deficit, but may demonstrate transient visual

obscurations associated with postural changes.

Headache, intermittent diplopia, vomiting

and/or nausea, and pulsatile tinnitus Visual field defects

Case

27/F with IUP 15 wk

C/C headaches and diplopia for 1 month P/I 이전에 mild한 headache 있던 환자로 내원1달전부터 뒷목에 누르는 듯한 통증과 두통 전과 다르게 심하다, 욱신거리고 누르는 듯한 두통 한번씩 심해지고 저녁에 더 심하다

몸을 숙이면 심해지고 두통때문에 한번씩 잠에서 깬다 수시간씩 지속, 1달동안 매일 아프다

두통약을 먹으면 일시적으로 호전 최근 수평복시가 발생

한쪽눈을 가리면 괜찮아진다 Photophobia(+) N/V(+/+)

두통이 있을 때 번쩍거리는 빛과 점들이 보일때가 있다 시력저하(-)

한번씩 귀가 멍멍하고 맥박뛰는 듯한tinnitus(+)

N/ExVisual acuity : 20/20 RAPD (-/-)

Lt. lateral 6^thCN palsy

Brain MRI : dilation of the perioptic nerve sheath, flattening of the posterior sclerae

brain MRA, MRV : UR

lumbar puncture : 360 mm of CSF with normal CSF contents.

-> 직후 두통 사라지고, 복시 호전보이는 양상 Treatment

: 500 mg of acetazolamide 2 times a day

Automated perimetry

Fundoscopy Pseudotumor cerebri

Primary pseudotumor cerebri

Idiopathic intracranial hypertension Includes patients with obesity, recent weight gain, polycystic ovarian syndrome, and thin children

Secondary pseudotumor cerebri Cerebral venous abnormalities Cerebral venous sinus thrombosis Bilateral jugular vein thrombosis or surgical ligation Middle ear or mastoid infection

Increased right heart pressure Superior vena cava syndrome Arteriovenous fistulas

Decreased CSF absorption from previous intracranial infection or subarachnoid hemorrhage Hypercoagulable states Medications and exposures Antibiotics

Tetracycline, minocycline, doxycycline, nalidixic acid, sulfa drugs

Vitamin A and retinoids

Hypervitaminosis A, isotretinoin, all-trans retinoic acid for promyelocytic leukemia, excessive liver ingestion

Hormones

Human growth hormone, thyroxine (in children), leuprorelin acetate, levonorgestrel (Norplant system), anabolic steroids

Withdrawal from chronic corticosteroids Lithium

Chlordecone Medical conditions Endocrine disorders Addison disease Hypoparathyroidism Hypercapnia Sleep apnea Pickwickian syndrome Anemia Renal failure Turner syndrome Down syndrome

(7)

(Neurology 2013)

1. Required for diagnosis of pseudotumor cerebri syndrome A. Papilledema

B. Normal neurologic examination except for cranial nerve abnormalities

C. Neuroimaging: Normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion and no abnormal meningeal enhancement on MRI, with and without gadolinium, for typical patients (female and obese), and MRI, with and without gadolinium, and magnetic resonance venography for others; if MRI is unavailable or contraindicated, contrast-enhanced CT may be used

D. Normal CSF composition

E. Elevated lumbar puncture opening pressure (>250 mm CSF in adults and >280 mm CSF in children [250 mm CSF if the child is not sedated and not obese]) in a properly performed lumbar puncture

2. Diagnosis of pseudotumor cerebri syndrome without papilledema

In the absence of papilledema, a diagnosis of pseudotumor cerebri syndrome can be made if B–E from above are satisfied, and in addition the patient has a unilateral or bilateral abducens nerve palsy

In the absence of papilledema or sixth nerve palsy, a diagnosis of pseudotumor cerebri syndrome can be suggested but not made if B–E from above are satisfied, and in addition at least 3 of the following neuroimaging criteria are satisfied:

i. Empty sella

ii. Flattening of the posterior aspect of the globe

iii. Distention of the perioptic subarachnoid space with or without a tortuous optic nerve iv. Transverse venous sinus stenosis

Grade I

Grade of papilledema

Grade IV Grade II

Grade III Grade I

Grade V

Clinical features of

Pseudotumor cerebri syndrome Visual field defect

Visual loss in at least one eye : over 90% of patients M/C : enlargement of the physiologic blind spot

loss of inferonasal portions of the visual field Central defects: uncommon

Blindness in about 5% of cases

With treatment there is significant perimetric improvement about 50% of patients.

Recent weight gain -> worsening of vision

Visual field defect in papilledema Grades of visual loss

(8)

Differential Diagnosis of Optic Disc Swelling Hyun Ah Kim

Differential Diagnosis of Optic Disc Swelling

Differential diagnosis of optic disc swelling

Normal optic disc

Pseudodisc edema

Optic drusen

Optic drusen

Drusen Fluorescein angiography

Medullated nerve fibers Morning glory syndrome

Tilted optic disc

Bergmeister’s papilla

Differential diagnosis of optic disc edema

Arteritic vs. nonarteritic AION

Arteritic vs. nonarteritic AION Arteritic vs. nonarteritic AION

Papilledema

A specific form of disc edema resulting from

Generally exhibits a minimal acuity deficit, but may demonstrate transient visual

obscurations associated with postural changes.

and/or nausea, and pulsatile tinnitus Visual field defects

Case

Automated perimetry

Fundoscopy Pseudotumor cerebri

Grade of papilledema

Clinical features of

Pseudotumor cerebri syndrome Visual field defect

Visual field defect in papilledema Grades of visual loss

Thank you for your attention