Case Report Anisocoria ~ Now 55 yr. Old caucasian female What? CC: droopy OD for 1 month, no other complaints or symptoms . Patient & Family Ocular & Medical Hx – Richard Mangan, OD, FAAO Negative The Eye Center of Richmond Adjunct Faculty, IU School of Medications: Evista, Calcium Sup. Optometry

Anisocoria Case Report Anisocoria Case Report

Clinical Findings: Clinical Findings Continued: BVA: 20/20 OD, OS Lid Eval : UL 2mm on OD; IPF 5mm ((--)) APD UL 1mm above cornea OS; IPF 8mm Size (light): 2.5mm OD, 3.0mm OS LL elevation OD Size (dim): 3.0mm OD, 6.5mm OS

Normal Near Response Color Equal EOM’s / CT: Normal

Anisocoria Case Report

Clinical Findings Continued: TA: 10/12 SLE: Normal DFE: C/D: 0.2 OD, 0.2 OS (good color) Macula & : Normal OU

1 Q2: Which of the following is Q1: Which of the following tests is least appropriate to confirm the diagnosis? the most likely diagnosis?

1. 44--10%10% test 1. Congenital Horner’s Syndrome 2. .125% test 2. Acquired Horner’s Syndrome 3. Paradrine 1% test 3. Adie’ ssTonicPupil Tonic 4. All of the above 4. CN III Palsy 5. None of the above 5. ArgyllArgyll--RobertsonRobertson Pupil

Q3: Which of the following is NOT considered Q4: Which of the following indicates the appropriate management for this condition? congenital / infantile form of this condition?

1. Chest XX--RayRay 1. Mild with excellent levator 2. Brain Imaging function 3. Referral to a Neurospecialist 2. Miotic Anisocoria , most apparent in darkness 4. All of the above are appropriate 3. Lower Lid Elevation 5. None of the above are appropriate 4. Ipsilateral Anhydrosis 5. Heterochromia

Q5: Which of the following requires the least aggressive workwork--up?up? The Pupil

1. Central / Pre-Pre-ganglionicganglionic Lesion Anatomical Considerations 2. PostPost--ganglionicganglionic Lesion Pathway of the Pupil Light Reflex Parasyypmpathetic Pathway y(p (Sphincter Muscle) – 4 Neuron Arc (afferent + Efferent) Sympathetic (Dilator Muscle of the Iris) ––33 Neuron Arc

2 Pupil Examination… …Be Systematic Pupil Examination What are the BCVA’s? …And are the acuities equal either corrected or with pinhole? Basics Are the patients pupils equal in size in bright and dim illumination? If not, is the anisocoria > in dim or bright illumination? Is the near accommodative reflex present and equal in both eyes? Are the accommodative amps = OU?

Pupil Examination (Cont.)

If the pupils are equal in size, is the direct light reflex equally strong in both eyes? Is the consensual light reflex equally strong in both eyes?

3 True or False?

A can cause and APD?

FALSE… If you are finding an APD , check your illumination source first. If you still have a + APD, need to find other cause.

YES or NO? True or False?

Is it possible to have can cause an disease and NOT have an APD? APD?

YES…if the disease is bilateral & EQUAL TRUE…If the Macular Degeneration is in BOTH eyes (I.e., toxic optic unilateral & severe enough (Va +/+/-- neuropathy) 20/400)

True or False? True or False?

Visual Acuity does not necessarily Only one functional pupil is needed to correlate with an RAPD? determine the presence of an APD?

TRUE…a person with endend--stagestage TRUE…because of the consensual light can have an APD with good response. central acuity. Good example at www.richmondeye.com

4 Identifying & Recording: Is PERRLA Enough? Exam Clinical Pearls

Ø PERRLA Iris Color Pupil Size D&C Reflex Accom Reflex Pupil Shape APD BIO > OD Green 4 => 7 3D / 3C 2+ R Neg Transilluminator > OS Green 4 => 7 3D / 3C 2+ R Neg Penlight Neutral Density Filters Fat Scan PER (3:6)RL (3+) A (++)

Normal Pupillary Phenomena Anisocoria: Case History

Pupillary Unrest vs. Hippus Near Synkinesis (The Near Triad) Direct & Consensual Responses APD / Marcus Gunn Defect www.richmondeye.com Physiological Anisocoria

Anisocoria: Case History Anisocoria: Exam Techniques

Temporal Aspects? ? Eye Pain? Hx of Trauma? Decreased VA? Drops or Ung’s? Arm, Chest, Head, Current Medications or Neck Pain? (including OTC’s)? Nuchal Rigidity? STD’s, Shingles, MS, Hx of Stroke, Thyroid Disease, Cancer, or Surgery Diabetes? Alcohol Usage?

5 Anisocoria: Exam Techniques Which is the Abnormal Pupil?

VA’s Pupillary The pupil that reacts sluggishly to light External Assessment: Ptosis Iris Color If Aniso > in Bright => Larger Pupil Anyhdrosis Pupil Size(s) & Shape Reactivity Parasympathetic Denervation EOM’s Dilation Lag Color VA, Red Near Response Desaturation If Aniso > in Dim => Smaller Pupil Vermiform Changes Abnormal Sympathetic Innervation SLE & Tonometry VF Testing & DFE

DDX of Anisocoria Anisocoria: Need to Rule-Rule-out!out!

P = Physiological P = Pharmacological Larger Pupil is ABN Smaller is ABN A = Adies Tonic A = AngleAngle--ClosureClosure Adie’s Tonic Pupil Horner’s Syndrome T = Third N. Palsy T = Trauma Compressive III N. ArgyllArgyll--RobertsonRobertson Pharmacological Pharmacological

H = Horner’s C = Congenital I = Iritis Malformation / Coloboma

ArgyllArgyll--RobertsonRobertson

Physiological Anisocoria Physiologic Anisocoria

Anisocoria of < 1mm (to 2mm) 20% of the US Population has Simple or Physiological Anisocoria. The degree of anisocoria can vary from day to day and even switch sides.

6 Adie’s Tonic Pupil Adie’s Tonic Pupil

Stats Key Findings Females 3:1 Dilated pupil with poor to absent direct Age: 20-20-4040 & consensual 80% Unilateral response. Initially Tonic near responses Becomes bilateral at Reduced Accom rate of 1-1-4%4% / year Amps Etiology Look for segmental

Idiopathic vs. Viral palsy of Iris sphincter muscle

Adie’s Tonic Pupil

Normal Room Illumination

Poor Direct Response Poor Consensual

(+) Near Response After Prolonged Effort

Adie’s Tonic Pupil: Isolated Third N. Palsy w/ Additional Testing Pupil Involvment

Dilute (0.125%) Pilocarpine Denervation Supersensitivity Parasympathetic defect occurs AFTER the fibers leave the . Exaggerated Pupillary Constriction Deep Tendon Reflexes Diminished Response => Holmes-Holmes-AdiesAdies Syndrome

7 Isolated Third N. Palsy w/ Pupil Involvement

Sudden Onset Unilateral Ptosis with Eye or Head Pain Acuity is Typically Unaffected unless damage in Superior Orbital Fissure Eye is in non-non-comitantcomitant exotropic & hypotropic position (“down & out”)

Isolated Third N. Palsy

Isolated Third N. Palsy w/ Isolated Third N. Palsy w/ Pupil Involvement Pupil Involvement

Posterior Communicating Artery Management (Most Common) Hospital Neurosurgical Consult ASAP Uncal Herniation Syndrome CT/MRI/MRA Space Occupying Lesion Lumbar Puncture Subdural Hematoma Cerebral Angiography Pituitary Apoplexy Ischemic Vascular Disease (Rare)

8 Anatomy of the Sympathetic Horner’s Syndrome Pathway to the Eye

Horner’s Syndrome: Horner’s Syndrome: Clinical Features Clinical Features

A. Moderate Ptosis (2(2--3mm)3mm) due to paralysis F. , more noticeable in dim illumination. of Muller’s muscle Note: Pupil rxns to light and near are B. “Upside Down Ptosis” - Mild elevation of the normal. lower lid due to paralysis of the smooth G. Anhydrosis on Ipsilateral side of face if muscle attached to the inferior tarsal plate. lesion is below the Superior Cervical rd C. Apparent due to A & B above Ganglion => Not a 3 order neuron. D. + Dilation Lag (classic finding) H. Increase in Amplitude of due to unopposed action of the E. Decreased IOP on affected side parasympathetic.

Horner’s Syndrome: Horner’s: Clinical Features…Lastly Localization of Lesion

4% Cocaine + Test => Anisocoria will increase Hydroxyamphetamine (Paradrine 1%) Preganglionic lesion => YES dilation Postganglionic lesion => No dilation If suspect pre-pre-ganglionicganglionic lesion => Chest CT or XX--ray.ray.

9 ArgyllArgyll--RobertsonRobertson Pupil: ArgyllArgyll--RobertsonRobertson Pupil Clinical Features

Pupils are small and frequently irregular Key Finding: LND Pupil (Remember ARPARP--PRA)PRA) Bilateral Asymmetric Pupil Involvement VA’s are typically NORMAL Poor dilation with Mydriatics

ArgyllArgyll--RobertsonRobertson Pupil: WorkWork--upup Anisocoria Case Report

As this is a Hallmark Sign for 55 yr. Old caucasian female , need to rule this out, as CC: droopy eyelid OD for 1 month, no well as HIV: other complaints or symptoms . Patient & Family Ocular & Medical Hx – FTAFTA--Abs,Abs, VDRL Negative Neurological workwork--upup Medications: Evista, Calcium Sup. Consider MRI, Lumbar Puncture

Anisocoria Case Report Anisocoria Case Report

Clinical Findings: Clinical Findings Continued: BVA: 20/20 OD, OS Lid Eval Pupils: UL 2mm on cornea OD; IPF 5mm ((--)) APD UL 1mm above cornea OS; IPF 8mm Size (light): 2.5mm OD, 3.0mm OS LL elevation OD Size (dim): 3.0mm OD, 6.5mm OS

((--)) LND Iris Color Equal EOM’s / CT: Normal

10 Q1: Which of the following tests is least Anisocoria Case Report appropriate to confirm the diagnosis?

Clinical Findings 1. 44--10%10% Cocaine test TA: 10/12 2. .125% Pilocarpine test SLE: Normal 3. Paradrine 1% test DFE: C/D: 0.2 OD, 0.2 OS (good color) 4. All of the above Macula & Retina: Normal OU 5. None of the above

Q2: Which of the following is Q1: Which of the following tests is least appropriate to confirm the diagnosis? the most likely diagnosis?

1. 44--10%10% Cocaine test 1. Congenital Horner’s Syndrome 2. .125% Pilocarpine test 2. Acquired Horner’s Syndrome 3. Paradrine 1% test 3. Adie’ ssTonicPupil Tonic Pupil 4. All of the above 4. CN III Palsy 5. None of the above 5. ArgyllArgyll--RobertsonRobertson Pupil

Q2: Which of the following is Q3: Which of the following is NOT considered the most likely diagnosis? appropriate management for this condition?

1. Congenital Horner’s Syndrome 1. Chest XX--RayRay 2. Acquired Horner’s Syndrome 2. Brain Imaging 3. Adie’ ssTonicPupil Tonic Pupil 3. Referral to a Neurospecialist 4. CN III Palsy 4. All of the above are appropriate 5. ArgyllArgyll--RobertsonRobertson Pupil 5. None of the above are appropriate

11 Q3: Which of the following is NOT considered Q4: Which of the following indicates the appropriate management for this condition? congenital / infantile form of this condition?

1. Chest XX--RayRay 1. Mild Ptosis with excellent levator 2. Brain Imaging function 3. Referral to a Neurospecialist 2. Miotic Anisocoria , most apparent in darkness 4. All of the above are appropriate 3. Lower Lid Elevation 5. None of the above are appropriate 4. Ipsilateral Anhydrosis 5. Heterochromia

Q4: Which of the following indicates the Q5: Which of the following requires the least congenital / infantile form of this condition? aggressive workwork--up?up?

1. Mild Ptosis with excellent levator 1. Central / Pre-Pre-ganglionicganglionic Lesion function 2. PostPost--ganglionicganglionic Lesion 2. Miotic Anisocoria , most apparent in darkness 3. Lower Lid Elevation 4. Ipsilateral Anhydrosis 5. Heterochromia

Q5: Which of the following requires the LEAST aggressive workwork--up?up? Thanks for your attention ☺

1. Central / Pre-Pre-ganglionicganglionic Lesion 2. PostPost--ganglionicganglionic Lesion

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