Case Analysis I- Lecture 9

1. Case Analysis I- Lecture 9 Liana Al-Labadi, O.D.

2. If you hear hoof beats, thinkhorses—not zebras

3. CC: Dr. I see a black curtain over my eyes

4. “Dr. I See All Black” • Frequency: Constantly (all the time, everyday) @ D&N • Onset: Suddenly10 days ago, but has noticed it more over the past 2 days • Location: Both eyes • Duration: Lasts for a short time but I feel it’s there all the time • Associated Factors: • Blurry vision • A lot of headaches • Relief: • Headaches get better with parcetamol but I still see a black curtain • Severity: • The blacking out is pretty bad, I just can’t stand it anymore. The headaches are there all the time.

5. DIFFERENTIAL DIAGNOSIS????

6. “Dr. I See All Black” • Migraine • DES / Keratitis/ Blepharitis/ Iritis /AACG • Vitreous detachment /Retinal break • Angiospasm/ vasospasm • Optic disc drusen/ Orbital tumor • Papilledema • ON / MS/ SLE • Embolic/Carotid emboli / Cardiac emboli • IV drug use • Hypoperfusion • Coagulation disorders /Inflammatory arteritis • Carotid stenosis/Ophthalmic artery stenosis • Cardiac failure or arrhythmia • Increased blood viscosity • Intraocular hemorrhage • Intracranial tumor • Psychogenic

7. “Dr. I See All Black” • POH: • (+) Near sightedness • Negative for asthenopia, surgery, pain, & flashes • Negative for AMD, DR, Cats & Glc • (+) DIPL-?????? • (+) Trauma- 14 years ago????? • LEE: 6 months ago (unknown doctor)- Status normal • FOH: Negative for AMD, DR, Glc, Cat • LPE: Does not remember • PMH: • (+) stress (+) ENT (+) Respiratory • Negative for HTN/DM/Cancer/Neuro • FMH: (+) HTN- Father; (+) DM- Parents; Migraines- Parents • MED: None • Allg: NKDA; No seasonal allergies • SH: Reading • Occupation: Student • No alcohol consumption ; (+) smoking- Argeeleh

8. “Dr. I See All Black” • Entrance Testing: • DVA (c): • OD: 20/50 PH: ??????????? • OS: 20/30 PH: ??????????? • Motility: S&F OD, OS • Pupils: 4mm/4mm RRL OD, OS; No APD • Confrontations: • OD: Slight inferior constriction • OS: Full

9. “Dr. I See All Black” • Additional Tests: • Lensometry: • OD: -3.75 -0.50x 153 • OS: -3.75 -0.50x 153 • Manifest Refraction: • OD: -4.00 -0.50x 165 VA: “All black” • OS: -3.75 -0.50x 153 VA: “All black”

10. “Dr. I See All Black” • SLE: • L/L: trace MGD OD, OS • Conj: No injection OD, OS • K: Clear OD, OS • Iris: Flat & brown OD, OS • AC: No cell & no flare/ D&Q OD, OS • Lens: Clear OD, OS • IOP (TA): • ??????????

11. “Dr. I See All Black”

12. “Dr. I See All Black” • DFE: • CDR: 0.15 round OD, OS • NRR: 360˚ optic nerve swelling/elevation OD, OS • Blurry disc margins OD, OS • Superior flame-shaped heme OD • Inferior flame-shaped heme OS • Macula: Flat OD, OS • Posterior Pole: Flat OD, OS • Vessels: • Dilated & tortuous OD, OS • Periphery: ????

13. “Dr. I See All Black” • Differential Diagnosis: • Papilledema • Retinal Vein Occlusion • Optic Neuritis/Papillitis • Ischemic Optic Neuropathy • Compressive Optic Neuropathy • Infiltrative Optic Neuropathy • Systemic etiology: • Sarcoid • Leukemia

14. “Dr. I See All Black” • Assessment: • Bilateral optic never disc edema • Unknown etiology • Sx of TVO + Headaches + DIPL • Plan: • Pt education on condition • Refer for CT scan • Refer patient for neurological work-up • Pt education on importance of follow-up with neurology appointment

15. “Dr. I See All Black” • F/U Visit: • Patient was told it was “benign” • Began oral medications

16. “Dr. I See All Black” • Assessment: • Bilateral Papilledema • Plan: • RTC in 2 months for DFE + Visual Field

17. Papilledema-Definition • Optic disc swelling produced by increased intracranial pressure

18. Papilledema-Symptoms • Symptoms: • Episodes of transient, often bilateral visual loss • Lasting seconds • Symptoms precipitated after rising from a lying or sitting position (altering intracranial pressure) • Headache • Double vision • Nausea • Vomiting • Tinnitis • Decrease in visual acuity (rare) • Mild decrease in VA can occur in an acute setting if there is macular disturbance • Visual field defects & severe loss of central vision • Occur only with chronic papilledema

19. Papilledema-Signs • Critical Signs: • Bilaterally swollen, hyperemic discs • Early papilledema- disc swelling may be asymmetric • NFL edema causing blurring of the disc margin and often obscuring the blood vessels • Other Signs: • Papillary pr peripapillary retinal hemorrhages • Loss of venous pulsation • 20% of the normal population do not have venous pulsation • Dilated, tortuous retinal veins • Normal pupillary response • Normal color vision • Enlarged blind spot

20. Papilledema-Signs • Signs of chronic papilledema: • Any hemorrhages or cotton-wool spots resolve • Disc hyperemia disappears • The disc becomes gray in color • Narrowing of the peripapillary retinal vessels • Optociliary shunt vessels may develop on the disc • Loss of color vision, VA, VF defects (especially inferionasally) may also occur

21. “Dr. I See All Black”

22. Papilledema Differential Diagnosis • Other Differential Diagnosis: • Pseudopapilledema • Not true disc swelling • Vessels overlying the disc are not obscured • The disc is not hyperemic • The surrounding NFL is normal • Spontaneous venous pulsation is often present • Secondary to optic disc drusen or congenitally anomalous disc • Hypertensive optic neuropathy • Optic nerve disc swelling caused by extremely high blood pressure • Narrowed arterioles • A/V crossing changes • Heme with or with out CWS in the peripheral retina and posterior pole

23. Papilledema Differential Diagnosis • Other Differential Diagnosis: • Orbital optic nerve tumors: • Unilateral disc swelling • May have proptosis • Amiodarone toxicity • Patient present with acute visual loss and disc edema • Diabetic papillopathy: • Benign disc edema in one or both eyes of a diabetic patient • Telagectasia of BVs and NV of the disc may occur • Thyroid-related optic neuropathy:

24. Papilledema Etiology • Primary & metastatic intracranial tumora • Hydrocephalus • Pseudotumor cerebri (PTC) • Subdural & epidural hematomas • Subarachnoid hemorrhage • These patients have severe headaches • Arteriovenous malformation • Brain abscess- often produces high fever • Meningitis • Fever, stiff neck, headache • Encephalitis • Cerebral venous sinus thrombosis

25. Papilledema Work-Up • History & physical examination • Including blood pressure measurement • Ocular examination • Pupils, color vision, DFE, VF • Emergency MRI with MRV (magnetic resonance venography) of the head • CT scan may be done if MRI is not available emergently • Lumbar puncture with CSF analysis and opening pressure measurement • Done if MRI/MRV or CT scan results are normal

26. Papilledema Treament • Treatment • Directed at the underlying cause of the increased intracranial pressure

27. Pseudotumor Cerebri (PTC) • AKA Idiopathic Intracranial Hypertension (IIH) • A syndrome in which patients present with symptoms and signs of elevated intracranial pressure • The nature of which may be idiopathic or due to various causative factors • Diagnosis of exclusion • Need to eliminate all other possible etiologies

28. Pseudotumor Cerebri (PTC) • Symptoms: • Headaches • Between 90% and 98% of patients with IIH present with headache • TVO • Transient episodes of visual loss- typically lasting seconds • Percipitated by changes in posture • Double vision • Tinnitis • Nausea • Vomitting • Occurs predominantly in obese women of childbearing age (20 to 44 years) • “Fat 40 disease” • Some studies suggest that excess weight in the abdominal area causes a chain reaction from increased intra-abdominal pressure, eventually leading to increased intra-cranial venous pressure

29. Pseudotumor Cerebri (PTC) • Critical Signs: • Papilledema due to increases intracranial pressure • Bilateral papilledema is a hallmark sign of the disease • Negative MRI/MRV of the brain • MRV added to evaluate the venous system of the brain and to rule out a sinus thrombosis that is usually missed with an MRI. • Without this information, the patient with a sinus thrombosis could be misdiagnosed with the more benign PTC • Increased opening pressure on lumbar puncture with normal CSF composition • Opening pressure exceeds 250mm of water • Other Signs: • Unilateral or bilateral 6th nerve palsy with no other neurological signs

30. Pseudotumor Cerebri (PTC) • Differential Diagnosis: • Same as Papilledema- refer to previous slides

31. Pseudotumor Cerebri (PTC) • Associated Factors: • Obesity “Fat 40” • Significant weight gain • Pregnancy • Medications: • Oral contraceptives • Tetracyclines • Nalidixic acid • Cyclosporine • Vitmain A (>100,000 U/day) • Systemic steroid withdrawl http://www.revoptom.com/content/d/cornea/c/15325/

32. Pseudotumor Cerebri (PTC) • Work-Up: • History: • Specifically inquire about weight gain & medications • Ocular Exam: • Pupils, EOMs, color vision & optic nerve evaluation • Visual field test- very important for following up patients • Systemic Exam: • Measure blood pressure & temperature • Thorough blood work with CBC to R/O infectious etiologies • MRI/MRV of orbit and brain • Must be done immediately • Need to R/O a space-occupying lesion • If normal refer for neuro-ophthalmic evaluation, including a lumbar puncture, to rule out other causes of papilledema & to determine the CSF opening pressure

33. Pseudotumor Cerebri (PTC) • Treatment: • PTC may be a self-limited process. • Treatment is indicated in the following situations: • Severe, intractable headaches • Evidence of progressive decrease in visual acuity or visual field loss • Some ophthalmologists suggest treating all patients with papilledema • Method of treatment: • Wight loss- if overweight or recent increase in weight – 1st line tx • Acetazolomide (Diamox) • Use with caution in sulfa-allergic patients • Can decrease CSF production by 50% • Discontinuation of any causative medication • If method of treatment unsuccessful • Consider systemic steroids • Optic nerve sheathing surgery if reduced VA • Neurosurgical shunt if headaches are a prominent symptom

34. Pseudotumor Cerebri (PTC) • Prognosis: • Typically good if the underlying condition is addressed promptly • Follow-Up: • If acute: • Monitor every 3 months in the absence of visual field loss • If chronic: • Initially follow-up every 3-4 weeks to monitor visual acuity and visual field loss then every 3 months • Patient education: • Educate patients that papilledema can lead to optic atrophy & irreversible vision damage if left untreated