Grand Rounds Raafay Sophie, M.D. 10/16/2015 University of Louisville Department of Ophthalmology and Visual Sciences

Grand RoundsGrand Rounds

Raafay Sophie, M.D.Raafay Sophie, M.D.

10/16/201510/16/2015

University of LouisvilleUniversity of Louisville

Department of Ophthalmology and Department of Ophthalmology and Visual SciencesVisual Sciences

Patient PresentationPatient Presentation CCCC: : Headache with seeing “specks and Headache with seeing “specks and

dancing spots”dancing spots”

HPIHPI: : 15 yr old AAF, with hx of worsening 15 yr old AAF, with hx of worsening

headache occurring daily for last 10 days headache occurring daily for last 10 days – 3 ER visits– 3 ER visits Pain scale 6-8/10Pain scale 6-8/10 Constant, B/L, “pounding” and behind Constant, B/L, “pounding” and behind

eye eye Nausea, photophobia, phonophobia- Nausea, photophobia, phonophobia-

presentpresent No fever, nuchal rigidity No fever, nuchal rigidity

HPI continuedHPI continued 11stst visit visit

Treated as migraine with ExcedrinTreated as migraine with Excedrin

22ndnd visit visit CT head - possible Chiari 1 malformationCT head - possible Chiari 1 malformation Migraine cocktail in ED - started on Migraine cocktail in ED - started on

Sumatriptan prophylaxis and outpatient Sumatriptan prophylaxis and outpatient follow up with MRIfollow up with MRI

33rdrd visit visit Admitted for further workup- also noted Admitted for further workup- also noted

to have visual symptoms- to have visual symptoms- ophthalmology consultedophthalmology consulted

HPI continuedHPI continued Visual symptoms:Visual symptoms:

Since last 3 days she had beenSince last 3 days she had been Seeing pink and purple spots Seeing pink and purple spots

intermittentlyintermittently Seeing blurry spots on her left and Seeing blurry spots on her left and

inferior sideinferior side Going “cross-eyed” at times Going “cross-eyed” at times

HistoryHistory• PMHx:PMHx: Migraines for 1 year, Amennhorea for 2 Migraines for 1 year, Amennhorea for 2

monthsmonths

• FAMHx:FAMHx: UnremarkableUnremarkable

• ROS: ROS: Tinnitus with headache at timesTinnitus with headache at times

• MEDS: MEDS: No ocular medicationNo ocular medication

• ALLERGIESALLERGIES: : NKDA NKDA

ExamExam

VACC TP P

20/40

20/70

18

19 no RAPD

5→2

5→2

BMI: 41 kg/ m2

Ishihara plates: 11/11 OU Red Desaturation: mild reduction OS

ExamExamEOM:

CVF: OD: inferior defectOS: temporal defect

00

-1-1

00

00

00

00

00

-1-1

10 prism diopter ET in primary gaze

Exam Exam OD OS LIDS/LASHES WNL WNL

CONJ white and quiet white and quiet

CORNEA clear clear

A/C deep and formed deep and formed

IRIS WNL WNL

LENS clear clear

Fundus ExamFundus ExamOD OS

MRIMRI

MRIMRI

MRIMRI

MRVMRV

AssessmentAssessmentNeurology:-Lab work up- CBC showed Hb 8.7, CMP unremarkable-Diamox 250 mg TID x 5days, then 500 mg TID

Neurosurgery:-Recommended medical management of papilledema-No LP needed at this time- Chiari 1 malformation-Possible outpatient decompression

Gynecology and Endocrine consulted for other medical problems

AssessmentAssessment

15 y/o obese girl with presumed benign intracranial hypertension and Chiari 1 malformation causing

-decreased visual acuity, -visual field defects, -early 6th nerve involvement

Follow UpFollow Up Hospital Course:•H/H of 6.0/20.6

• Red blood Cell transfusion with improvement of H/H to 8.0/26.3.

• Iron 325 mg BID • Progesterone only pill

•Discharged after 4 days on Acetozolamide 500 mg TID

Clinic follow up 3 days later:-Improvement in headache and visual symptoms-VA 20/20 OU-IOP 14/ 16 mmHg-Pupils 6->3 OU, no APD-Grade 3-4+ papilledema

-Continued with Acetozolamide 500 mg TID and will follow up in 2 weeks time

Idiopathic Intracranial Idiopathic Intracranial Hypertension (IIH)Hypertension (IIH)

• Elevated intracranial pressure (ICP) with normal radiologic studies, and normal CSF composition


Symptoms of elevated ICP

• Headache and nausea

• Transient visual obscurations

• Visual field loss (enlarged blind spots on perimetry testing. )

• Pulsatile tinnitus (pulse synchronous bruit).

• Early IIH shows normal visual acuity

• Diplopia (secondary to abducens nerve paresis)

• Other neurologic abnormalities other than abducens palsy are not associated with IIH.


• Signs: Almost all patients with IIH have papilledema.




• Incidence - 22.5/100,000 new cases/yr

• Peaks in the third decade of life

• Ninety percent of patients are women and 90% are obese

• Rare in prepubertal children and in lean adults


• Associated with• Vitamin A (>100,000 U/day)

• Tetracycline

• Minocycline

• Doxycycline

• Retinoic acid

• Lithium

• Use of or withdrawal of use from corticosteroids

• Sleep apnea

• Not been definitely associated with any specific endocrinologic dysfunction although hormonal abnormalities have been implicated.


MRI and MRV to rule out :

• Cerebral venous disorders such as cerebral venous obstruction

• Systemic or localized extracranial venous obstruction

• Dural arteriovenous malformation

• Systemic vasculitis

• Tumor

• Hydrocephalus

• Meningeal lesion


Lumbar puncture:

• Measure ICP

• Rule out infectious or inflammatory processes

TreatmentTreatment• Depends on symptomatology and vision status

• If headache is controlled with minor analgesics and optic nerve dysfunction is absent, no therapy may be required.

• For obese patients - weight loss

• Medical Therapy• Acetazolamide- first line• Topiramate -headache control, appetite

suppression, and carbonic anhydrase inhibition• Furosemide • Corticosteroids?

.

SurgerySurgeryIndicated for intractable headache or progressive vision loss despite maximally tolerated medical therapy.

•Optic nerve sheath fenestration (ONSF) • 1%–2% risk of vision loss from optic nerve injury,

central retinal artery occlusion (CRAO), or central retinal vein occlusion (CRVO).

•CSF diversion procedure (lumboperitoneal or ventriculoperitoneal shunt)

• Improvement of headache, abducens palsy

• May become occluded, infected, altered in position- reoperation

•Gastric bypass surgery • reduce both weight and ICP.

““Pediatric” IIHPediatric” IIH• Although pediatric typically refers to

children <18 years, pediatric IIH usually is used for prepubescent children

• Predilection for boys and nonobese children

• Several cranial neuropathies have been associated with pediatric IIH including cranial nerves (CNs) III, IV, VI, VII, IX, and XII

• The treatment for pediatric IIH is similar to that for adult IIH.

PrognosisPrognosis

• Up to 31-86% have some degree of permanent vision loss

• Up to 10% develop severe vision loss

• Implicated poor prognostic factors:• Male sex• African American race• Anemia

Chiari 1 Malformation (CM) : inferior tonsillar displacement (ITD) of 5 mm or more below the Foramen Magnum (FM)

Cerebellar Ectopia (CE): ITD more than 2 mm but less than 5 mm below the FM.

Retrospective review •68 patients with Psudotumor Cerebri and available brain MRI •MRIs were analyzed for cerebellar tonsillar position, and results were compared with original reports.

Results: By report: 8 (12%) had ITD - 4 had CM, 4 had CEOn review: 16 (24%) had ITD- 7 had CM, 9 had. All patients with ITD were female, most were overweight or obese, most had IIH.

Primary IIH causing ITD vs primary ITD causing IIH?

Multicenter, double-blind, placebo-controlled clinical trial, comparing acetazolamide vs placebo

Patients who meet modified Dandy criteria with mild to moderate disease defined as having “Perimetric mean deviation (PMD) between −2 and −7 dB on 24-2 SITA (Swedish interactive thresholding algorithm) Standard testing on automated perimetry”

Specific dietary plan and weight loss program along with a weight counsellor offered to all patients

Patient Characteristics:

•165 patients out of which 4 (2.4%) were men

•Mean (SD) age ---- 29.0 (7.4) years

•Mean (SD) BMI ---- 39.9 (8.3) kg/m2.

•65% white, 25% black, 10% other

•Mean (SD) CSF opening pressure 343.5 (86.9) mm H2O (range, 210–670 mm H2O).

Figure 5. Frisén Papilledema Grading

The average (SD) PMD • the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) • the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB).

Figure 2. Histogram of Mean Deviation Values of Idiopathic Intracranial Hypertension Treatment Trial Patients at Baseline

Figure 1. SymptomsA, Graph shows initial symptoms reported at study entry.B, Graph shows the frequency of all symptoms reported at study entry.

• Headache• Mean (SD) headache severity was 6.3

(1.9)• 51% reported as either constant or daily• 41% reported a premorbid history of

migraine (17% had migraine with aura).

• RAPD was found in 5.4% of eyes

• Binocular diplopia in 18%, 3% had an esotropia on examination

• A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding.

Figure 2. Adjusted Mean Change in Perimetric Mean Deviation (PMD) Over Time by Treatment Group

• Mean improvement in papilledema grade• acetazolamide: −1.31, from 2.76 to 1.45• placebo: −0.61, from 2.76 to 2.15• Rx effect: −0.70; 95% CI, −0.99 to −0.41; P

< .001)

• Vision-related quality of life (National Eye Institute VFQ-25)• acetazolamide: 8.33, from 82.97 to 91.30• placebo: 1.98, from 82.97 to 84.95• Rx effect: 6.35; 95% CI, 2.22 to 10.47; P = .003)

• Reduction in weight • acetazolamide: −7.50 kg, from 107.72 kg to

100.22 kg• placebo: −3.45 kg, from 107.72 kg to 104.27 kg• Rx effect: −4.05 kg, 95% CI, −6.27 to −1.83 kg;

P < .001).

THANK YOU

References1. Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13-18

2. http://webeye.ophth.uiowa.edu/eyeforum/cases/papilledema-grading.htm

3. Pediatric Ophthalmology and Strabismus- BCSC 2015-2016

4. Neuro-Ophthalmology- BCSC 2015-2016

5. Rowe FJ. Assessment of visual function in idiopathic intracranial hypertension: a prospective study. Eye (Lond). 1998;12 ( Pt 1):111-8.

6. Wall M. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain. 1991 Feb;114 ( Pt 1A):155-80.

7. Thurtell MJ, Bruce BB, Newman NJ, Biousse V. An Update on Idiopathic Intracranial Hypertension. Reviews in neurological diseases. 2010;7(0):e56-e68.

8. Banik R1, Lin D, Miller NR . Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci. 2006 Aug 15;247(1):71-5. Epub 2006 May 6.

9. Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, Katz DM, Keltner JL, Schron EB, McDermott MP; NORDIC Idiopathic Intracranial Hypertension Study Group. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014 Jun;71(6):693-701. doi: 10.1001/jamaneurol.2014.133.

10.Wall M, McDermott MP, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, Katz DM, Keltner JL, Schron EB, Kupersmith MJ Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee,. JAMA. 2014 Apr 23-30;311(16):1641-51. doi: 10.1001/jama.2014.3312.

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Grand Rounds Raafay Sophie, M.D. 10/16/2015 University of Louisville Department of Ophthalmology and Visual Sciences