PEDIATRIC CERVICAL SPINE DEFORMITY Joseph Donnelly, M.D. SFORP1/3/05.

PEDIATRIC CERVICAL PEDIATRIC CERVICAL SPINE DEFORMITYSPINE DEFORMITY

Joseph Donnelly, M.D.Joseph Donnelly, M.D.

SFORPSFORP

1/3/051/3/05

DEVELOPMENTDEVELOPMENT

• EACH VERTEBRAE DEVELOP FROM THE EACH VERTEBRAE DEVELOP FROM THE CAUDAL AND CRANIAL ½ OF 2 CAUDAL AND CRANIAL ½ OF 2 SCLEROTOMES SCLEROTOMES – C1 and C2 primitive centrum fuse to form C1 and C2 primitive centrum fuse to form

odontoid processodontoid process

• ATLASATLAS– Body ossifies at 6-24 mosBody ossifies at 6-24 mos– Arch closure (final canal diameter) at 6-7 yoArch closure (final canal diameter) at 6-7 yo

• Further growth by periosteal appositional growth only Further growth by periosteal appositional growth only (external, no canal change)(external, no canal change)


• AXISAXIS– ODONTOIDODONTOID

• 2 PRIMARY OSSIFICATION CENTERS2 PRIMARY OSSIFICATION CENTERS– Coalesce by 3 mosCoalesce by 3 mos– Separated from C2 by dentocentral synchondrosisSeparated from C2 by dentocentral synchondrosis

Gradually closes btwn 3-6yoGradually closes btwn 3-6yo

• TIP BECOMES AN APOPHYSIS TIP BECOMES AN APOPHYSIS – Chondrum terminaleChondrum terminale– Begins ossifying 5-8yoBegins ossifying 5-8yo– Fuses at 10-13yo Fuses at 10-13yo

– NEURAL ARCHES CLOSED BY 6-7 YRSNEURAL ARCHES CLOSED BY 6-7 YRS• Similar to atlas, no increase in canal size after this pointSimilar to atlas, no increase in canal size after this point


• C 3-7C 3-7– 3 OSSIF. CTRS @ BIRTH3 OSSIF. CTRS @ BIRTH

• 1 BODY, 2 ARCHES1 BODY, 2 ARCHES

• POST SYNCHONDROSIS POST SYNCHONDROSIS CLOSES @ 2 YRSCLOSES @ 2 YRS

– FACETS START FACETS START HORIZONTAL , BECOME HORIZONTAL , BECOME VERTICAL WITH GROWTHVERTICAL WITH GROWTH


• C 3-7C 3-7– BODY GROWTHBODY GROWTH

•VERTICAL – ENCHONDRAL OSSIFICATIONVERTICAL – ENCHONDRAL OSSIFICATION

•CIRCUMFERENTIAL – PERIOSTEAL CIRCUMFERENTIAL – PERIOSTEAL APPOSITIONAPPOSITION

RADIOGRAPHIC RADIOGRAPHIC PARAMETERSPARAMETERS• WHAT IS PATHOLOGIC IN AN ADULT WHAT IS PATHOLOGIC IN AN ADULT

CAN BE NORMAL IN A GROWING CHILDCAN BE NORMAL IN A GROWING CHILD– ADIADI– SACSAC– PSEUDOSUBLUXATION C 1-2PSEUDOSUBLUXATION C 1-2– OS-ODONTOIDIUMOS-ODONTOIDIUM– GROWTH CENTERS ARE NOT FRACTURESGROWTH CENTERS ARE NOT FRACTURES

RADIOGRAPHIC RADIOGRAPHIC PARAMETERSPARAMETERS• ADI (ATLANTO-DENS ADI (ATLANTO-DENS

INTERVAL)INTERVAL)– Measure on lateral Measure on lateral

flex/ext films, flex/ext films, voluntary motion in voluntary motion in awake patientawake patient• ANT ASPECT OF DENS ANT ASPECT OF DENS

TO THE POST ASPECT OF TO THE POST ASPECT OF THE ANT RING OF THE THE ANT RING OF THE ATLAS ON BOTH FILMSATLAS ON BOTH FILMS

– NL: < 5mm kids, NL: < 5mm kids, <3mm adults<3mm adults

RADIOGRAPHIC RADIOGRAPHIC PARAMETERSPARAMETERS

• Anterior arch of atlas can override Anterior arch of atlas can override odontoid on extension in 20% of kidsodontoid on extension in 20% of kids

• Why ADI increase in kids?Why ADI increase in kids?– ↑ ↑ ligamentous laxityligamentous laxity– ↑ ↑ cartilage component of dens and atlascartilage component of dens and atlas

RADIOGRAPHIC RADIOGRAPHIC PARAMETERSPARAMETERS• SAC (SPACE AVAILABLE FOR THE CORD)SAC (SPACE AVAILABLE FOR THE CORD)

– POST ASPECT OF DENS TO ANT ASPECT OF POST POST ASPECT OF DENS TO ANT ASPECT OF POST RING OF ATLASRING OF ATLAS

– >13 mm in adults and teens>13 mm in adults and teens– Need at least the diameter of the odontoid availableNeed at least the diameter of the odontoid available

RADIOGRAPHIC RADIOGRAPHIC PARAMETERSPARAMETERS• SACSAC

– Steel’s rule of thirdsSteel’s rule of thirds•1/3 cord1/3 cord•1/3 odontoid1/3 odontoid•1/3 space available (“safe zone”)1/3 space available (“safe zone”)

– ATTENUATION OF TRANSVERSE ATTENUATION OF TRANSVERSE ATLANTAL LIG LEAVES ONLY THE ALAR ATLANTAL LIG LEAVES ONLY THE ALAR LIG (i.e. TRISOMY)LIG (i.e. TRISOMY)•ALAR LIG ALONE CANNOT PROTECT FROM ALAR LIG ALONE CANNOT PROTECT FROM

SCI WITH EVEN MILD TRAUMASCI WITH EVEN MILD TRAUMA

COMMON NORMAL COMMON NORMAL VARIANTSVARIANTS• Absence of cervical lordosisAbsence of cervical lordosis

– Mimics splinting of injuryMimics splinting of injury

• PseudosubluxationPseudosubluxation• C1 multiple ossification centers/ spina bifidaC1 multiple ossification centers/ spina bifida

– Can mimic fxCan mimic fx– Look for smoot cortical margins Look for smoot cortical margins – Lack hematoma on CTLack hematoma on CT

• Spina bifida occultaSpina bifida occulta• C2 dentocentral synchondrosisC2 dentocentral synchondrosis

– CLOSES BY 11 YEARSCLOSES BY 11 YEARS

• Anterior wedging of C3 seen in 7%Anterior wedging of C3 seen in 7%

PSEUDOSUBLUXATIONPSEUDOSUBLUXATION

• ANT DISPLACE OF C2 ON C3ANT DISPLACE OF C2 ON C3– C 3-4 less commonC 3-4 less common– 9% of kids 1-7yo9% of kids 1-7yo– Posterior line of SwischukPosterior line of Swischuk

• Line from ant aspect of C1 posterior Line from ant aspect of C1 posterior arch to same on C3arch to same on C3

• Should be within 1mm of same of C2Should be within 1mm of same of C2

• >2mm= pathologic>2mm= pathologic

– CAUSESCAUSES• Horizantal facetsHorizantal facets

– Esp. in upper Esp. in upper

• ↑ ↑ RELATIVE HEAD SIZERELATIVE HEAD SIZE

• ↑ ↑ LIG LAXITYLIG LAXITY


• ANT DISPLACE OF C2 ON ANT DISPLACE OF C2 ON C3C3– CAUSESCAUSES

• Horizontal facetsHorizontal facets– Esp. in upper C spineEsp. in upper C spine– Change from 30deg to 70deg Change from 30deg to 70deg

during growthduring growth

• Large relative head sizeLarge relative head size

• General ligamentous laxityGeneral ligamentous laxity

– Treatment Treatment • Do nothingDo nothing

OS ODONTOIDIUMOS ODONTOIDIUM

• TIP OF ODONTOID IS DIVIDEDTIP OF ODONTOID IS DIVIDED– Apical segment lacks basilar supportApical segment lacks basilar support

• VERY RAREVERY RARE

• X-RAY – oval ossicle, smooth marginsX-RAY – oval ossicle, smooth margins

• CAUSES ?CAUSES ?– Old fx non-unionOld fx non-union

•MRI’s have shown cord changes c/w traumaMRI’s have shown cord changes c/w trauma

– AVNAVN– Congenital anomalyCongenital anomaly


• SYMPTOMSSYMPTOMS– NECK PAINNECK PAIN– VERT ART OCLUSION (C1-2 MOTION)VERT ART OCLUSION (C1-2 MOTION)

• SYNCOPE, VERTIGO, N/V, VISUAL DEFECITSSYNCOPE, VERTIGO, N/V, VISUAL DEFECITS

– NEURO SXS (RARE)NEURO SXS (RARE)•Posterior translation of os into cordPosterior translation of os into cord

•Transient paresis, myelopathy, paralysisTransient paresis, myelopathy, paralysis

•SUDDEN DEATHSUDDEN DEATH


• TREATMENTTREATMENT– SURGERY (C1-2 PSA, INST, HALO)SURGERY (C1-2 PSA, INST, HALO)

•ADI > 10 mmADI > 10 mm

•SAC SAC << 13mm 13mm

•NEUROLOGIC SXNEUROLOGIC SX

•PERSISTANT PAINPERSISTANT PAIN

•PROGRESSIVE INSTABILITYPROGRESSIVE INSTABILITY

TORTICOLLISTORTICOLLIS

• Combined head tilt and rotatory Combined head tilt and rotatory deformitydeformity

• Indicates C1-2 problemIndicates C1-2 problem– 50% rotation in C-spine at this joint50% rotation in C-spine at this joint

• Large differential diagnosisLarge differential diagnosis– Osseous vs. nonosseousOsseous vs. nonosseous

TORTICOLLISTORTICOLLIS

• DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES– MUSCULAR (82%)MUSCULAR (82%)– ATLANTO-AXIAL ROTATORY SUBLUXATIONATLANTO-AXIAL ROTATORY SUBLUXATION– CNS LESIONCNS LESION– BIRTH TRAUMABIRTH TRAUMA– CONGENITAL SPINE DEFORMITYCONGENITAL SPINE DEFORMITY

• KLIPPEL-FEILKLIPPEL-FEIL• OCCIPITO-CERVICAL SYNOSTOSISOCCIPITO-CERVICAL SYNOSTOSIS• GOLDENHAR SYNDGOLDENHAR SYND• HEMIATLASHEMIATLAS

– BASILAR IMPRESSIONBASILAR IMPRESSION– ODONTOID ANOMALY (OS ODONTOIDIUM)ODONTOID ANOMALY (OS ODONTOIDIUM)

CONGENITAL MUSCULAR CONGENITAL MUSCULAR TORTICOLLISTORTICOLLIS

• 82% OF ALL TORTICOLIS82% OF ALL TORTICOLIS– 75% right sided75% right sided– 8-20% also have DDH8-20% also have DDH

• CONGENITAL CONSTRICTION OF SCMCONGENITAL CONSTRICTION OF SCM

• HEAD TILT WITH ROTATION HEAD TILT WITH ROTATION OPPOSITE TILTOPPOSITE TILT

• FAMILIAL COMPONENTFAMILIAL COMPONENT

CONGENITAL MUSCULAR CONGENITAL MUSCULAR TORTICOLLISTORTICOLLIS• CAUSE UNKNOWNCAUSE UNKNOWN

– INTRAUTERINE SCM INTRAUTERINE SCM COMPARTMENT SYNDROME COMPARTMENT SYNDROME FROM NECK COMPRESSIONFROM NECK COMPRESSION• SCM VENUS OCLUSION ON SCM VENUS OCLUSION ON

HISTOPATHOLOGYHISTOPATHOLOGY

• MYOFIBROSIS MYOFIBROSIS → CONTRACTION→ CONTRACTION

– NEUROLOGICNEUROLOGIC• Spinal accessory N. injurySpinal accessory N. injury

– FETAL POSITIONFETAL POSITION– EMBRYOLOGICEMBRYOLOGIC– BIRTH TRAUMABIRTH TRAUMA


• PLAGYCEPHALYPLAGYCEPHALY– Flattening of head on side of contractureFlattening of head on side of contracture– Due to sleeping position (prone in U.S.)Due to sleeping position (prone in U.S.)– Untreated: eye/ear levels become unequalUntreated: eye/ear levels become unequal

• X- RAYSX- RAYS– Always normal in congenital muscular torticollisAlways normal in congenital muscular torticollis– Check hipsCheck hips

• RARELY A TREATABLE NEUROLOGIC CAUSERARELY A TREATABLE NEUROLOGIC CAUSE• SYRINX, SPINAL CORD TUMOR, CHIARI, POST FOSSA SYRINX, SPINAL CORD TUMOR, CHIARI, POST FOSSA

TUMOR, OCULAR PATHOLOGY (involuntary head tilt)TUMOR, OCULAR PATHOLOGY (involuntary head tilt)


• TREATMENTTREATMENT– 90% RESOLVE WITHOUT SURGERY90% RESOLVE WITHOUT SURGERY

•STRETCHING, PTSTRETCHING, PT•Crib toy modificationCrib toy modification

– After 1yo, stretching usually unsuccessfulAfter 1yo, stretching usually unsuccessful– SURGERY: GOOD RESULTS UP TO 12 YO SURGERY: GOOD RESULTS UP TO 12 YO

•UNIPOLAR RELEASEUNIPOLAR RELEASE•BI-POLAR RELEASEBI-POLAR RELEASE

– Z-LENGTHENING OF STERNAL INSERTION MAINTAINS NECK Z-LENGTHENING OF STERNAL INSERTION MAINTAINS NECK CONTOURCONTOUR

•MID-SCM TRANSECTIONMID-SCM TRANSECTION

– MINERVA BRACE FOR 6-12 WEEKSMINERVA BRACE FOR 6-12 WEEKS


• TREATMENTTREATMENT– FACIAL ASSYMETRY CORRECTION FACIAL ASSYMETRY CORRECTION

RELATIVE TO GROWTH REMAINING AT RELATIVE TO GROWTH REMAINING AT TIME OF CORRECTION (REMODELING)TIME OF CORRECTION (REMODELING)

– BEST SURGERY TIME BTWN 1 AND 4YOBEST SURGERY TIME BTWN 1 AND 4YO

ATLANTO-AXIAL ROTARY ATLANTO-AXIAL ROTARY SUBLUXATIONSUBLUXATION

• COMMON PROBLEMCOMMON PROBLEM• RANGE- MILD SUBLUX TO COMPLETE RANGE- MILD SUBLUX TO COMPLETE

DISLOCATIONDISLOCATION• X-RAY: difficult to assessX-RAY: difficult to assess

• LAT MASS OF C1 SHIFTS ANTERIORLY, APPEARS WIDER THAN LAT MASS OF C1 SHIFTS ANTERIORLY, APPEARS WIDER THAN THE NARROWER, MORE POST DISPLACED OPPOSITE LAT THE NARROWER, MORE POST DISPLACED OPPOSITE LAT MASSMASS

• POSTERIOR ARCHES DON’T SUPERIMPOSE DUE TO HEAD TILTPOSTERIOR ARCHES DON’T SUPERIMPOSE DUE TO HEAD TILT• CAN BE NL CHILD WITH ROTATED HEADCAN BE NL CHILD WITH ROTATED HEAD• CT WITH LEFT AND RIGHT ROTATION WILL DEMONSTRATE ITCT WITH LEFT AND RIGHT ROTATION WILL DEMONSTRATE IT

– C1/ODONTOID RELATIONSHIP IS CONSTANT IN A FIXED C1/ODONTOID RELATIONSHIP IS CONSTANT IN A FIXED DEFORMITY DEFORMITY

ATLANTO-AXIAL ROTARY ATLANTO-AXIAL ROTARY SUBLUXATIONSUBLUXATION• 4 TYPES:4 TYPES:

– I) ROTATORY DISPLACE, NO ANT I) ROTATORY DISPLACE, NO ANT SHIFT (SHIFT (most commonmost common))• RESOLVES SPONTANEOUSLYRESOLVES SPONTANEOUSLY

– II) ROT DISPLACE, WITH < 5mm II) ROT DISPLACE, WITH < 5mm ANT SHIFTANT SHIFT• NOTED ON FLEX/EXT X-RAYS NOTED ON FLEX/EXT X-RAYS

(ADI)(ADI)

– III) ROT DISPLACE, > 5mm ANT III) ROT DISPLACE, > 5mm ANT SHIFT (SHIFT (very rarevery rare))• HIGH RISK OF PARALYSIS/DEATHHIGH RISK OF PARALYSIS/DEATH

– IV) ROT DISPLACE, POST SHIFT IV) ROT DISPLACE, POST SHIFT ((very rarevery rare))• HIGH RISK OF PARALYSIS/DEATHHIGH RISK OF PARALYSIS/DEATH


• ETIOLOGYETIOLOGY– MINOR TRAUMAMINOR TRAUMA

•MOST COMMONMOST COMMON

– FRACTUREFRACTURE– FOLLOWING ENT SURGERYFOLLOWING ENT SURGERY– FOLLOWING URIFOLLOWING URI


• PRESENTATIONPRESENTATION– ACUTE TORTICOLLISACUTE TORTICOLLIS– PAIN WITH NECK ROMPAIN WITH NECK ROM

•Long SCM painful due to resisting deformityLong SCM painful due to resisting deformity

– PLAGYCEPHALY SEEN IN LONG-PLAGYCEPHALY SEEN IN LONG-STANDING CASESSTANDING CASES

ATLANTO-AXIAL ROTARY ATLANTO-AXIAL ROTARY SUBLUXATIONSUBLUXATION• GRISEL SYNDROMEGRISEL SYNDROME

– SPONTANIOUS ATLANTO-AXIAL SUBLUX FOLLOWING URI, SPONTANIOUS ATLANTO-AXIAL SUBLUX FOLLOWING URI, OR ENT SURG (T/A)OR ENT SURG (T/A)

– DIRECT CONNECTION BTW PERIDONTAL VENUS PLEXUS DIRECT CONNECTION BTW PERIDONTAL VENUS PLEXUS AND SUBOCCIPITAL EPIDURAL SINUSES AND SUBOCCIPITAL EPIDURAL SINUSES • TRANSPORTS SEPTIC EXUDATES CAUSING ATLANTO-AXIAL TRANSPORTS SEPTIC EXUDATES CAUSING ATLANTO-AXIAL

HYPEREMIAHYPEREMIA– TEMPORARY LIGAMENTOUS LAXITYTEMPORARY LIGAMENTOUS LAXITY– SUBLUXATIONSUBLUXATION

– CHILDREN’S FACETS MORE HORIZONTALCHILDREN’S FACETS MORE HORIZONTAL• FACETS HAVE MENISCUS-LIKE SYNOVIAL POCKETS FACETS HAVE MENISCUS-LIKE SYNOVIAL POCKETS → CAN → CAN

BE TRAPPEDBE TRAPPED

– USUALLY RESOLVES SPONTANEOUSLYUSUALLY RESOLVES SPONTANEOUSLY


• TREATMENTTREATMENT– < 1 WEEK OF SYMPTOMS< 1 WEEK OF SYMPTOMS

•SOFT COLLAR FOR COMFORT, RESTSOFT COLLAR FOR COMFORT, REST• IF NO REDUCTION: HALTER TRACTION, VALIUMIF NO REDUCTION: HALTER TRACTION, VALIUM

– 1 – 4 WEEKS OF SYMPTOMS1 – 4 WEEKS OF SYMPTOMS•ADMIT, HALTER TX, MAY NEED HALO TXADMIT, HALTER TX, MAY NEED HALO TX•CONFIRM REDUCTION ON DYNAMIC CTCONFIRM REDUCTION ON DYNAMIC CT• IF NO ANT DISPLACEMENT, SOFT COLLAR 1-2 WKIF NO ANT DISPLACEMENT, SOFT COLLAR 1-2 WK• IF ANT DISPLACEMENT, BRACE TO IMMOBILIZE IF ANT DISPLACEMENT, BRACE TO IMMOBILIZE

FOR 6 WEEKS (allow ligaments to heal)FOR 6 WEEKS (allow ligaments to heal)


– > 1 MTH> 1 MTH•HALO TRACTION FOR THREE WEEKSHALO TRACTION FOR THREE WEEKS

•MANY WILL REDUCE, THEN LOSE REDUCTION MANY WILL REDUCE, THEN LOSE REDUCTION AFTER TX RELEASEDAFTER TX RELEASED

•SOME WILL NOT REDUCE AT ALLSOME WILL NOT REDUCE AT ALL

•SURGERY INDICATIONS (C1-2 PSF)SURGERY INDICATIONS (C1-2 PSF)– FIXED DEFORMITYFIXED DEFORMITY– LOSS OF REDUCTION AFTER TREATMENTLOSS OF REDUCTION AFTER TREATMENT– ANT DISPLACEMENT > 5mmANT DISPLACEMENT > 5mm– NEURO SXNEURO SX– >3 MONTH DURATION OF SYMPTOMS>3 MONTH DURATION OF SYMPTOMS

KLIPPEL-FEIL SYNDROMEKLIPPEL-FEIL SYNDROME

• Congenital fusions of Congenital fusions of cervical vertebraecervical vertebrae

• Clinical triad:Clinical triad:– Low posterior hairlineLow posterior hairline– Short neckShort neck– Limited neck motionLimited neck motion

• Abnormal embryologic Abnormal embryologic development of development of vertebraevertebrae

• Incidence= 0.7%Incidence= 0.7%


• SPRENGEL DEFORMITY (33%)SPRENGEL DEFORMITY (33%)

• CARDIAC ANOMALIESCARDIAC ANOMALIES

• RENAL ANOMALIESRENAL ANOMALIES– Get renal U/SGet renal U/S

• PULMONARY ANOMALIESPULMONARY ANOMALIES

• DEAFNESSDEAFNESS

• SCOLIOSISSCOLIOSIS

• X-RAYS VARIABLE:X-RAYS VARIABLE:– Simple block vertebrae to bizzare anomaliesSimple block vertebrae to bizzare anomalies


• INSTABILITY COMMON ADJACENT TO INSTABILITY COMMON ADJACENT TO FUSED LEVELSFUSED LEVELS– Get flex/ext views prior to anesthesiaGet flex/ext views prior to anesthesia– Commonly see C1-2 and C3-4 fusion Commonly see C1-2 and C3-4 fusion

with instability risk at unfused C2-3with instability risk at unfused C2-3


• NO CLEAR TX GUIDELINESNO CLEAR TX GUIDELINES– Treat similar to congenital scoliosisTreat similar to congenital scoliosis– High risk of developing instabilityHigh risk of developing instability

•Avoid contact sportsAvoid contact sports

•Cervical traction, collars, analgesics for Cervical traction, collars, analgesics for mechanical sxmechanical sx

– Surgery Surgery •Fuse for neuro signs due to instabilityFuse for neuro signs due to instability

•Surgery for cosmesis alone= unwarranted and Surgery for cosmesis alone= unwarranted and riskyrisky

ATLANTO-OCCIPITAL ATLANTO-OCCIPITAL SYNOSTOSISSYNOSTOSIS

• Anterior arch C1 fused to occiputAnterior arch C1 fused to occiput– Posterior arch typically hypoplasticPosterior arch typically hypoplastic

• C1 height variably decreasedC1 height variably decreased– Allows odontoid to project into foramen magnumAllows odontoid to project into foramen magnum

• ““primary basilar impressionprimary basilar impression

• Often dysplastic odontoidOften dysplastic odontoid

• Congenital fusion C2-3 in 70%Congenital fusion C2-3 in 70%

• Lower C-spine deformities commonLower C-spine deformities common


• FINDINGS: (LIKE KLIPPEL-FEIL)FINDINGS: (LIKE KLIPPEL-FEIL)– SHORT BROAD NECKSHORT BROAD NECK– DECREASED NECK ROMDECREASED NECK ROM– LOW HAIR LINELOW HAIR LINE– SPRENGEL’S DEFORMITYSPRENGEL’S DEFORMITY– SHORT STATURESHORT STATURE– HYPOSPADIASHYPOSPADIAS– G-U ANOMALIES G-U ANOMALIES – EAR DEFORMITYEAR DEFORMITY– CLEFT PALATECLEFT PALATE– JAW ANOMALIESJAW ANOMALIES

ATLANTO-OCCIPITAL ATLANTO-OCCIPITAL SYNOSTOSISSYNOSTOSIS• OFTEN ASYMPTOMATIC UNTIL 40’SOFTEN ASYMPTOMATIC UNTIL 40’S• X-RAYS DIFFICULT TO EVALUATEX-RAYS DIFFICULT TO EVALUATE

– Aim beam 90deg to skull, not C-spineAim beam 90deg to skull, not C-spine• CT USUALLY NECESSARYCT USUALLY NECESSARY

– ““head wag” technique in young childrenhead wag” technique in young children• Skull blurs, C1-2 articulation visibleSkull blurs, C1-2 articulation visible

• 50% DEVELOP C1-2 INSTABILITY50% DEVELOP C1-2 INSTABILITY– ADI CAN BE > 12 mmADI CAN BE > 12 mm

• NEURO SX’S CAN DEVELOP SLOWLYNEURO SX’S CAN DEVELOP SLOWLY– C1-2 instability progresses with ageC1-2 instability progresses with age


• Neuro symptomsNeuro symptoms– Due to compression of brainstem or Due to compression of brainstem or

anterior upper cord by posteriorly anterior upper cord by posteriorly projecting densprojecting dens

– Pyramidal signs most common (spastic, Pyramidal signs most common (spastic, hyperreflex, weak, poor gait)hyperreflex, weak, poor gait)

– Can also get cranial N. involvement, Can also get cranial N. involvement, posterior column involvment from posterior column involvment from foramen lipforamen lip


• TREATMENTTREATMENT– C-COLLARS, BRACES, TRACTIONC-COLLARS, BRACES, TRACTION– TRAUMA AVOIDANCETRAUMA AVOIDANCE– SURGERYSURGERY

•HIGH RATE OF PARALYSISHIGH RATE OF PARALYSIS

•WITH C1 – C2 INSTABILITY: C1-2, OR WITH C1 – C2 INSTABILITY: C1-2, OR OCCIPUT – C2 FUSIONOCCIPUT – C2 FUSION

– DECOMPRESSION IF NECESSARYDECOMPRESSION IF NECESSARY

BASILAR IMPRESSIONBASILAR IMPRESSION

• Indentation of skull Indentation of skull floor by upper C-spinefloor by upper C-spine

• Cephalad tip of dens Cephalad tip of dens can protrude into can protrude into foramen magnumforamen magnum– Brainstem Brainstem

compressioncompression– Vascular compromiseVascular compromise– CSF flow alterationsCSF flow alterations

• Primary vs. secondaryPrimary vs. secondary


• Primary basilar impressionPrimary basilar impression– More commonMore common– Congenital abnormality Congenital abnormality – Often assoc with other vertebral defectsOften assoc with other vertebral defects

•Klippel FeilKlippel Feil•Abnormal odontoidAbnormal odontoid•Atlas hypoplasiaAtlas hypoplasia•AO fusionAO fusion

– 1% incidence1% incidence


• Secondary basilar impressionSecondary basilar impression– DevelopmentalDevelopmental– Less commonLess common– Due to softening of the occiputDue to softening of the occiput

– PAGETSPAGETS– OSTEOMALACIAOSTEOMALACIA– RICKETSRICKETS– RENAL OSTEODYSTROPHYRENAL OSTEODYSTROPHY– OSTEOGENESIS IMPERFECTAOSTEOGENESIS IMPERFECTA– ACHONDROPLASIA, HYPOCHONDROPLASIAACHONDROPLASIA, HYPOCHONDROPLASIA– JRAJRA– ANKYLOSING SPONDYLITISANKYLOSING SPONDYLITIS– NEUROFIBROMATOSISNEUROFIBROMATOSIS


• FINDINGSFINDINGS– SHORT NECK (78%)SHORT NECK (78%)– FACIAL ASSYMETRYFACIAL ASSYMETRY– PAINFUL/DECREASED CERVICAL MOTION (53%)PAINFUL/DECREASED CERVICAL MOTION (53%)– NEUROLOGIC SX’SNEUROLOGIC SX’S

• MOTOR WEAKNESS, LIMB PARESTHESIASMOTOR WEAKNESS, LIMB PARESTHESIAS• OFTEN ELICITED WITH MINOR TRAUMAOFTEN ELICITED WITH MINOR TRAUMA• WEAKNESS/PARAESTHESIAWEAKNESS/PARAESTHESIA• OFTEN ASSOCIATED WITH CHIARI OFTEN ASSOCIATED WITH CHIARI • CRANIAL N INVOLVEMENT (V, IX, X, XII)CRANIAL N INVOLVEMENT (V, IX, X, XII)• HRYDROCEPHALUS FROM CSF BLOCKAGEHRYDROCEPHALUS FROM CSF BLOCKAGE


• RADIOLOGYRADIOLOGY– PLAIN FILMS DIFFICULT TO PLAIN FILMS DIFFICULT TO

ASSESSASSESS• McGREGORS BEST FOR McGREGORS BEST FOR

SCREENINGSCREENING– LANDMARKS EASY TO SEE AT ALL LANDMARKS EASY TO SEE AT ALL

AGES ON LATERAL FILMAGES ON LATERAL FILM– HARD PALATE HARD PALATE → OCCIPUT→ OCCIPUT

• McRAE BEST FOR MEASURING McRAE BEST FOR MEASURING CLINICAL SIGNIFICANCECLINICAL SIGNIFICANCE

– DEFINES FORAMEN OPENINGDEFINES FORAMEN OPENING– DENS ABOVE LINE= SXDENS ABOVE LINE= SX

– CT- BONY ANATOMY, DEGREE OF CT- BONY ANATOMY, DEGREE OF INVAGINATIONINVAGINATION

– MRI- NEURAL MRI- NEURAL ANATOMY/COMPRESSIONANATOMY/COMPRESSION

Hard

Hard palate


• TREATMENTTREATMENT– Difficult, requires multidiscipline Difficult, requires multidiscipline

approachapproach•Ortho, neurosurgery, neuroradiologyOrtho, neurosurgery, neuroradiology

– Treatment is surgicalTreatment is surgical•PSF in extension at OC junctionPSF in extension at OC junction

•Anterior excision of odontoid if can’t be Anterior excision of odontoid if can’t be reducedreduced

•May need suboccipital decompressionMay need suboccipital decompression

FAMILIAL CERVICAL FAMILIAL CERVICAL DYSPLASIADYSPLASIA• Atlas deformityAtlas deformity

• Epidemiology unknownEpidemiology unknown

• Partial absence of C1Partial absence of C1– Usually posterior elementsUsually posterior elements

• PresentationPresentation– Torticollis, cervical “clunk”, suboccipital pain, Torticollis, cervical “clunk”, suboccipital pain,

decreased ROMdecreased ROM

• Radiographs difficult to readRadiographs difficult to read

• Instablility= Occiput-C1Instablility= Occiput-C1– Get flex/ext MRIGet flex/ext MRI

FAMILIAL CERVICAL FAMILIAL CERVICAL DYSPLASIADYSPLASIA• TREATMENTTREATMENT

– SERIAL FOLLOW-UP SERIAL FOLLOW-UP •Q6-12mosQ6-12mos

– SURGERY FOR:SURGERY FOR:•PAINPAIN• INSTABILITYINSTABILITY•PROGRESSIVE DEFORMITYPROGRESSIVE DEFORMITY•NEUROLOGIC SYMPTOMSNEUROLOGIC SYMPTOMS•PSF OCCIPUT TO C2 WITH HALOPSF OCCIPUT TO C2 WITH HALO

HEMI-ATLASHEMI-ATLAS

• 3 TYPES3 TYPES– I. ISOLATED HEMI-ATLAS I. ISOLATED HEMI-ATLAS – II. HEMI-ATLAS WITH LOWER SPINAL II. HEMI-ATLAS WITH LOWER SPINAL

ANOMALYANOMALY– III. HEMI-ATLAS WITH ATLANTO-III. HEMI-ATLAS WITH ATLANTO-

OCCIPITOL ARTHRODESISOCCIPITOL ARTHRODESIS•OFTEN ASSOC. W :OFTEN ASSOC. W :

– FORAMEN MAGNUM STENOSISFORAMEN MAGNUM STENOSIS– CHIARICHIARI– VERTEBRAL ARTERY ANOMALYVERTEBRAL ARTERY ANOMALY

SANDIFER SYNDROMESANDIFER SYNDROME

• GERD with torticollisGERD with torticollis• Neck tilt likely attempt to ease reflux Neck tilt likely attempt to ease reflux

discomfortdiscomfort• Can be diagnosis of exclusionCan be diagnosis of exclusion

– No tight SCMNo tight SCM– X-rays of c-spine normalX-rays of c-spine normal– Upper GI shows hiatal hernia, GERDUpper GI shows hiatal hernia, GERD

• Treating GERD usually resolves Treating GERD usually resolves torticollistorticollis

THE ENDTHE END

PEDIATRIC CERVICAL SPINE DEFORMITY Joseph Donnelly, M.D. SFORP1/3/05.

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Transcript of PEDIATRIC CERVICAL SPINE DEFORMITY Joseph Donnelly, M.D. SFORP1/3/05.