Managing Nystagmus Without An Eccentric Null Position 2016 AAPOS Meeting Workshop: Management of Childhood Nystagmus

Richard W. Hertle, M.D. The Childrens Vision Center, Akron Childrens Hospital Akron, OH

The Northeast Ohio Medical College, Rootstown, OH

I have personal financial relationships with:

OXFORD UNIVERSITY PRESS

Grant/Research Support THE NATIONAL INSTITUTES

OF HEALTH, THE NATIONAL EYE INSTITUTE,

BETHESDA MD, USA

Grant/Research Support - THE REBECCA S. CONSIDINE

RESEARCH INSTITUTE, AKRON, OHIO, USA

Shareholder and Patent Holder with RBG GROUP, LLC, USA

Acknowledgements Dongsheng Yang, PhD Shanghi, China

Louis F. DellOsso, PhD Cleveland, OH

Larry Abel, PhD Melbourne, AU

Jonathan Jacobs, PhD Cleveland, OH

Raymond Kraker, MPHS Tampa, FLA

Edmond F. Fitzgibbon , MD Bethesda, MD

Susan B. Mellow RN Bethesda, MD

Mitra Maybodi, MD Bethesda, MD

Robert Williams, PhD Memphis, TN

David B. Granet, MD La Jolla, CA

Deanna Stevens, MD Columbus, OH

William Anninger, MD Columbus, OH

Vannessa M Hill Columbus, OH

Joel S. Schuman, MD Pittsburgh, PA

Hiroshi Ishikawa, PhD Pittsburgh, PA

Leah Reznick, MD Pittsburgh, PA

Mingshia Zhu, PhD Pittsburgh, PA

Kenneth Adams, DO Albuquerque, NM

Matthew Kaufman, MS Pittsburgh, PA

Eric Hald Pittsburgh, PA

Tara Cronin, MD Pittsburgh, PA

Ellen Mitchell, MD Pittsburgh, PA

Jai Jeng Pittsburgh, PA

Ginger: Hudson, OH Scout: Wibeaux, MT

Kristen Carey, MD Pittsburgh, PA

Stephanie Knox Akron, OH

Robert Burnstine, MD Akron, OH

Erin Benjamin, MD Akron, OH

Michael Jandt Wibeaux, MT

Jennifer Eaton Akron, OH

Steven Schmidt, PhD Akron, OH

Jeffery Dunmire Akron, OH

Classification of Eye Movement Abnormalities and

Strabismus - Nystagmus Types

1. Peripheral Vestibular Imbalance Meniere, drug toxicity

2. Central Vestibular Imbalance Downbeat, Upbeat, drug toxicity

3. Instability of Vestibular Mechanisms PAN

4. Disorders of Visual Fixation Vision Loss, SSN, drug toxicity

5. Disorders of Gaze Holding GEN, ?APN, drug toxicity

6. Acquired Pendular Nystagmus central myelin, oculopalatal, Whipple,

drug toxicity

7. Saccadic Intrusions and Oscillations SWJ, MSO, opsoclonus

8. Miscellaneous Eye Movements SO Myokymia, OM neuromyotonia

9. Infantile Nystagmus Syndrome

congenital, motor, sensory, idiopathic, nystagmus blockage

10. Fusion Maldevelopment Nystagmus Syndrome

Latent, manifest latent, nystagmus blockage

11. Spasmus Nutans Syndrome

Without optic pathway glioma

With optic pathway glioma

Disease Name INFANTILE NYSTAGMUS SYNDROME (INS)

[Old Congenital Nystagmus and Motor and Sensory Nystagmus]

Criteria Infantile onset, ocular motor recordings show diagnostic (accelerating) slow phases

Common Associated

Findings

Conjugate, horizontal-torsional, increases with fixation attempt, progression from

pendular to jerk, family history often positive, constant, conjugate, with or without

associated sensory system deficits (e.g., albinism, achromatopsia), associated

strabismus or refractive error, decreases with convergence, null and neutral zones

present, associated head posture or head shaking, may exhibit a latent component,

reversal with OKN stimulus or (a)periodicity to the oscillation. Candidates on

Chromosome X and 6

May decrease with induced convergence, increased fusion, extraocular muscle surgery,

contact lenses and sedation.

General Comments Waveforms may change in early infancy, head posture usually evident by 4 years of

age. Vision prognosis dependent on integrity of sensory system.

CEMAS http://www.nei.nih.gov/news/statements/cemas.pdf

Evaluation Techniques: Afferent

System

Vision testing procedures

Behavioral Vision Testing

(acuity, color, stereo)

Visual Evoked Responses (flash,

pattern, sweep)

Electroretinography (flash,

pattern)

Contrast, Color and Visual Field

Testing

SD-OCT

Eye Movement Recordings

Methods

Contact

electrooculography

Infrared reflectance

Remote Video

Scleral contact

lens/magnetic search

coils.

INFRARED

SEARCH COIL

REMOTE VIDEO

CONTACT EOG

It has been found that such operation not only may greatly lessen torticollis,

but may also improve vision by lessening the nystagmus itself..

J. Ringland Anderson, Ocular Vertical Deviations and Treatment of Nystagmus, JB

Lippincott Co., Philadelphia, PA, second edition (1959), p 170

4,355 patients with INS, 2006-2014 Age 1.5 - 67 years (ave 14 years).

63% male.

Follow up after surgery 9-23 mos (ave 11.1 mos).

68% of the patients had other eye disease.

61% had an associated systemic diagnosis.

33% oculocutaneous albinism.

62% anomalous head posture.

21% had a periodic or aperiodic component.

71% had strabismus.

71% had a significant refractive error.

Hertle, RW, Yang, D, Carey K, Mitchell, EB. A Systematic Approach To Eye Muscle Surgery for Infantile Nystagmus

Syndrome: Results In 100 Patients. Binocular Vision and Strabismus Quarterly, 2010:25;72-93.

OPERATION TYPE (388 PATIENTS) PERCENT

Operation 1 Horizontal Head Posture Alone

Horizontal Rectus Recess and Resect or Recess and Tenotomy + Reattach 22

Operation 2 - Chin Down Head Posture (+/- Strabismus)

Superior Rectus Recess 5.0 mm + Inferior Oblique Myectomy 16

Operation 3 - Strabismus Alone

Primary Position Deviation Using at Least Two Recti Each Eye 15

Operation 4 Horizontal Head Posture + Strabismus

Fixing Eye Straightens Head + Non-fixing Eye Straightens Eyes 10

Operation 5 - Chin Up Head Posture (+/- Strabismus)

Inferior Rectus Recess 5.0 mm + Superior Oblique Tenectomy 5.0 mm 10

Operation 6 - No Head Posture, Strabismus or Vergence Damping

Horizontal Rectus Tenotomy + Reattach 9

Operation 7 - Multiplanar Head Posture (+/- Strabismus)

Transposition of Recti + Combinations of Oblique or Recti Recess 7

Operation 8 - Vergence Damping Alone (Artificial Divergence)

Medical Rectus Recess 3.0 mm + Lateral Rectus Tenotomy + Reattach 6

Operation 9 - Torsional Head Posture Alone

Horizontal Transposition of Vertical Recti 1 Tendon Width 5

BMR REC or RES or T

OPERATE ON ALL FOUR HORIZONTAL RECTI

SMALL DEVIATIONS BILATERAL RECESS + TENOTOMY

OR

LARGE DEVIATIONS BILATERAL RECESS + RESECT

OPERATION 3 STRABISMUS ALONE

BLR REC or RES or T

15%

I.E. - 30 ET + OD PREFERRED + FACE RIGHT

HEAD STRAIGHT WITH 50 BO OD

AND

EYES STRAIGHT WITH 20 BI OS

OD LATERAL RESECT + MEDIAL RECESS FOR 50 PRISM

AND

OS MEDIAL RESECT + LATERAL RECESS FOR 20 PRISM

OPERATION 4 HORIZONTAL HEAD POSTURE + STRABISMUS

10%

LR RESECT LR RECESS MR RESECT MR RECESS

TENOTOMY TENOTOMY

OPERATE ON ALL FOUR HORIZONTAL RECTI

TENOTOMY WITH REATTACHMENT

OPERATION 6 NO HEAD POSTURE-STRABISMUS-VERGENCE DAMPING

TENOTOMY TENOTOMY

9%

MR RECESS MR RECESS

OPERATE ON ALL FOUR HORIZONTAL RECTI

BILATERAL MEDIAL RECESS 3.0 mm

BILATERAL LATERAL RECTUS TENOTOMY AND REATTACH

OPERATION 8 VERGENCE DAMPING (ARTIFICAL DIVERGENCE)

LR TENOTOMY LR TENOTOMY

6%

Conclusions EOM Surgery and INS

Nine Operation Systematic Approach

Beneficial Effects of Surgery on: Binocular Optotype Visual Acuity

Head Posture

Strabismus

Nystagmus (ANAF)

Independent of Age

Operation Subtype

Associated

Ocular Diagnosis

Systemic Diagnosis

15

Axon

Myelin

2u

500u

Nerve

Ending

Hertle, RW, et. al. Neuroanatomy of The Extraocular

Muscle Tendon Enthesis In Macaque, Normal Human

and Patients with Congenital Nystagmus. JAAPOS. 2002;6:319-27

Dell'Osso LF, Wang ZI. Extraocular proprioception and new treatments

for infantile nystagmus syndrome.Prog Brain Res. 2008;171:67-75.

Fackelmann K, et. Al.,. Histochemical characterisation of

trigeminal neurons that innervate monkey extraocular

muscles.Prog Brain Res. 2008;171:17-20

Enthesial

Area

Brain Response to Proprioceptive

Disruption at Enthesis

WHY?

http://www.ncbi.nlm.nih.gov/pubmed/18718284http://www.ncbi.nlm.nih.gov/pubmed/18718284http://www.ncbi.nlm.nih.gov/pubmed/18718284http://www.ncbi.nlm.nih.gov/pubmed/18718284http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277http://www.ncbi.nlm.nih.gov/pubmed/18718277