eric a. lichtenstein, md faap · incomitant strabismus a/v patterns duane syndrome cranial nerve...

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A b n o r m a l H e a d P o s i t i o n A b n o r m a l H e a d P o s i t i o n Eric A. Lichtenstein, MD FAAP Eric A. Lichtenstein, MD FAAP

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Abnormal Head PositionAbnormal Head Position

Eric A. Lichtenstein, MD FAAPEric A. Lichtenstein, MD FAAP

tortustortus twistedtwistedcollumcollum neckneck

TorticollisTorticollis

TiltTilt

Head tiltHead tilt

TurnTurn

Face turnFace turn

Chin upChin up

Chin downChin down

Abnormal Head Position (AHP)Abnormal Head Position (AHP)

TiltTilt headhead

TurnTurn faceface

ChinChin up/downup/down

Ocular AHPOcular AHP

adaptive mechanismadaptive mechanism

neck control required neck control required

Ocular AHP: AdaptationOcular AHP: Adaptation

Strabismus Strabismus (avoid double)(avoid double)

Ptosis Ptosis (seek stereo)(seek stereo)

Nystagmus Nystagmus (improve acuity)(improve acuity)

Ocular AHPOcular AHP

Double vision Double vision (strabismus)(strabismus)

Stereo acuity Stereo acuity (ptosis)(ptosis)

Visual acuity Visual acuity (nystagmus)(nystagmus)

Not issues during sleep Not issues during sleep

AHP HxAHP Hx

Does AHP persist with sleep?Does AHP persist with sleep?Ocular: noOcular: no

How old when AHP began?How old when AHP began?Ocular: 3 to 4 monthsOcular: 3 to 4 months

AHP d/dxAHP d/dx

•• Muscular/skeletalMuscular/skeletal

•• AuditoryAuditory

•• Ocular Ocular

Ocular AHPOcular AHP

Straighten the headStraighten the headStrabismus?Strabismus?

Ptosis?Ptosis?Nystagmus?Nystagmus?

Ocular AHPOcular AHP

Strabismus Strabismus

PtosisPtosis

NystagmusNystagmus

AHP: StrabismusAHP: Strabismus

Avoid double visionAvoid double vision

Enhance depth perceptionEnhance depth perception

StrabismusStrabismus

ComitantComitantAngle Angle samesame in every gazein every gaze

AHP: no benefitAHP: no benefit

IncomitantIncomitantAngle Angle variesvaries with gazewith gaze

AHP: may benefitAHP: may benefit

Incomitant strabismusIncomitant strabismus

A/V PatternsA/V PatternsDuane SyndromeDuane Syndrome

Cranial Nerve PalsiesCranial Nerve PalsiesMiscellaneousMiscellaneous

AA--pattern: Chin Uppattern: Chin Up

AA--patternpattern

VV--pattern: Chin Downpattern: Chin Down

VV--patternpattern

Duane syndromeDuane syndrome

Synkinesis 3Synkinesis 3rdrd/6/6thth nervenerveCoCo--contraction contraction (medial/lateral recti)(medial/lateral recti)

CongenitalCongenitalHearing deficitsHearing deficits

Duane syndromeDuane syndrome

Duane syndromeDuane syndrome

AADD--duction (looking in) duction (looking in) –– lids closelids close

Duane syndromeDuane syndrome

AABB--duction (looking out) duction (looking out) –– lids openlids open

Duane syndromeDuane syndrome

Lid height changes with gazeLid height changes with gaze

Duane syndromeDuane syndrome

Duane syndromeDuane syndrome

Duane syndromeDuane syndrome

Duane syndromeDuane syndrome

Duane SyndromeDuane SyndromeBefore surgeryBefore surgery After surgeryAfter surgery

Duane Syndrome?Duane Syndrome?

8 mo healthy 8 mo healthy ♂♂

Acute onset AHPAcute onset AHP

URI 1 week priorURI 1 week prior

Acute onsetAcute onset

Poor APoor ABB--duction duction

Lid height constantLid height constant

Duane SyndromeDuane Syndrome

4 months later4 months later 11 months later11 months later

PostPost--viral 6th Nerve Palsy: Observeviral 6th Nerve Palsy: Observe

66thth Nerve Palsy: When to ScanNerve Palsy: When to Scan

Not postNot post--viralviral

Not isolatedNot isolatedany other cranial nerveany other cranial nerve

any descending tract paresis/palsyany descending tract paresis/palsy

Motor or other developmental delayMotor or other developmental delay

44thth Nerve Nerve ““PalsyPalsy””Most common CN AHPMost common CN AHP

Abnormal rotation: Abnormal rotation: Lax SO tendonLax SO tendon

Not actually a palsyNot actually a palsy

Congenital SOP = Tendon LaxityCongenital SOP = Tendon Laxity

2/3 of adults with 2/3 of adults with SOP are congenital SOP are congenital casescases

Neck and face Neck and face changeschanges

MidMid--facial facial hemihemi--hypoplasiahypoplasia

Ocular AHPOcular AHP

Strabismus Strabismus

PtosisPtosis

NystagmusNystagmus

PtosisPtosis

PtosisPtosis

PtosisPtosis

Ocular AHPOcular AHP

Strabismus Strabismus

PtosisPtosis

NystagmusNystagmus

NystagmusNystagmus

Involuntary rhythmic oscillationInvoluntary rhythmic oscillation

Conjugate : same direction Conjugate : same direction

Dysconjugate: different directionsDysconjugate: different directions

NystagmusNystagmus

AmplitudeAmplitudeFrequencyFrequencyMagnitude = Amplitude x FrequencyMagnitude = Amplitude x Frequency

Null zone: where magnitude is Null zone: where magnitude is leastleast

Horizontal, conjugate nystagmus.Horizontal, conjugate nystagmus.

Horizontal, dysconjugate nystagmus.Horizontal, dysconjugate nystagmus.

Dysconjugate nystagmus.Dysconjugate nystagmus.

Vertical, conjugate Vertical, conjugate

Vertical, Dysconjugate Vertical, Dysconjugate (see(see--saw)saw)

NystagmusNystagmusCan be benignCan be benign

Can herald CNS massCan herald CNS mass

““SafeSafe”” -- horizontal in all directions of gazehorizontal in all directions of gazepresent present ““since birthsince birth””decreasesdecreases with near viewingwith near viewing** albinism** albinism

Horizontal, conjugate nystagmus.Horizontal, conjugate nystagmus.

Congenital Motor NystagmusCongenital Motor Nystagmus

BenignBenign

““SafeSafe”” ---- horizontal in all directions of gazehorizontal in all directions of gazepresent present ““since birthsince birth””decreasesdecreases with near viewingwith near viewing

Null zone evident (AHP present)Null zone evident (AHP present)

Congenital Motor NystagmusCongenital Motor Nystagmus

BenignBenign

““SafeSafe”” ---- horizontal in all directions of gazehorizontal in all directions of gazepresent present ““since birthsince birth””decreasesdecreases with near viewingwith near viewing

Null zone evident (AHP present)Null zone evident (AHP present)

Null zoneNull zone

Nystagmus dampened by gazeNystagmus dampened by gaze

AHP utilized to look into null zoneAHP utilized to look into null zone

Can change early in lifeCan change early in life

Can be shifted (surgery)Can be shifted (surgery)

NystagmusNystagmus

AHPAHP PostPost--opop

NystagmusNystagmus

AHPAHP PostPost--opop

NystagmusNystagmus

AHPAHP PostPost--opop

NystagmusNystagmus

AHPAHP PostPost--opop

NystagmusNystagmus

AHPAHP PostPost--opop

Ocular AHPOcular AHP

Straighten the headStraighten the headStrabismus?Strabismus?

Ptosis?Ptosis?Nystagmus?Nystagmus?