Grand Rounds
Raafay Sophie, M.D.
10/16/2015
University of Louisville
Department of Ophthalmology and Visual Sciences
Patient Presentation

CC: Headache with seeing “specks and dancing spots”

HPI:
 15 yr old AAF, with hx of worsening headache
occurring daily for last 10 days – 3 ER visits
 Pain scale 6-8/10
 Constant, B/L, “pounding” and behind eye
 Nausea, photophobia, phonophobia- present
 No fever, nuchal rigidity
HPI continued

1st visit
 Treated as migraine with Excedrin

2nd visit
 CT head - possible Chiari 1 malformation
 Migraine cocktail in ED - started on Sumatriptan
prophylaxis and outpatient follow up with MRI

3rd visit
 Admitted for further workup- also noted to have
visual symptoms- ophthalmology consulted
HPI continued

Visual symptoms:

Since last 3 days she had been
 Seeing pink and purple spots intermittently
 Seeing blurry spots on her left and inferior side
 Going “cross-eyed” at times
History
•
PMHx: Migraines for 1 year, Amennhorea for 2 months
•
FAMHx: Unremarkable
•
ROS: Tinnitus with headache at times
•
MEDS: No ocular medication
•
ALLERGIES: NKDA
Exam
BMI: 41 kg/ m2
20/40
VACC
20/70
5→2
18
TP
P
19
Ishihara plates: 11/11 OU
Red Desaturation: mild reduction OS
no RAPD
5→2
Exam
EOM:
0
0
0
-1
0
-1
0
0
10 prism diopter ET in primary gaze
CVF:
OD: inferior defect
OS: temporal defect
Exam
LIDS/LASHES
OD
OS
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 Exam
OD
OS
MRI
MRI
MRI
MRV
Assessment
Neurology:
-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
Assessment
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 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
Hypertension (IIH)
•
Elevated intracranial pressure (ICP) with normal radiologic
studies, and normal CSF composition
Idiopathic Intracranial
Hypertension (IIH)
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.
Idiopathic Intracranial
Hypertension (IIH)
•
Signs: Almost all patients with IIH have papilledema.
Idiopathic Intracranial
Hypertension (IIH)
Idiopathic Intracranial
Hypertension (IIH)
Idiopathic Intracranial
Hypertension (IIH)
•
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
Idiopathic Intracranial
Hypertension (IIH)
• 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.
Idiopathic Intracranial
Hypertension (IIH)
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
Idiopathic Intracranial
Hypertension (IIH)
Lumbar puncture:
• Measure ICP
• Rule out infectious or inflammatory processes
Treatment
• 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?
.
Surgery
Indicated 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” 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.
Prognosis
• 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 CE
On 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
Figure 2. Histogram of Mean Deviation Values of Idiopathic Intracranial
Hypertension Treatment Trial Patients at Baseline
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 1. Symptoms
A, 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
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