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Infant Head Shape EvaluationSavannah Brown MPAS, PA-CJessica Grant MPAS, PA-CLaura Hanna MPAS, PA-CElizabeth Wetz MS, MPAS, PA-CKaylee Williams MPAS, PA-C

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February 25, 2017

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Objectives

• Recognize patterns of single suture, non-syndromic craniosynostosis

• Understand the common physical findings differentiating craniosynostosis and positional plagiocephaly

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Skull Growth

• The skull grows in response to increasing brain and CSF volume

• The brain size triples by 1 year and approximates 85% adult growth by 3 years of age

• Normal growth occurs perpendicular and parallel to sutures.


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Skull Growth


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Parietal Bone

Frontal Bone

Frontal Bone

Coronal Suture

Frontal (Metopic)

Suture

Anterior Fontanelle

Coronal Suture

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Parietal BoneFrontal Bone

Coronal Suture

Anterior Fontanelle

LambdoidSutureSquamosal

Suture

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Parietal BoneFrontal Bone

Coronal Suture

Anterior Fontanelle

Parietal Bone

Frontal Bone

Coronal Suture

Frontal (Metopic)

Suture

Posterior Fontanelle

Lambdoid Sutures

Sagittal Suture


Posterior Fontanelle

Lambdoid Sutures

Sagittal Suture

Parietal Bone

Parietal Bone

Occipital Bone

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Skull Maturation

• Normal suture fusion

– Metopic: 6 to 8 months

– Sagittal: 22 years

– Coronal: 24 years

– Lambdoidal: 26 years

• Normal fontanelle closure

– Posterior fontanelle: 3-6 months

– Anterior fontanelle: 9-12 months


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Abnormal Head Shape Evaluation


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Infant Head Shape Changes

• Macrocephaly Neurosurgery

• Microcephaly Neurology

• Scaphocephaly/Brachycephaly/Plagiocephaly Plastic Surgery


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Plagiocephaly

Plagio = “twisted” or “oblique”

+

Cephaly = head

• Is a descriptor of head shape, not necessarily a diagnosis


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Head shapes

• Scaphocephaly: “boat”

– sagittal synostosis versus long term hospitalization positional changes

• Anterior plagiocephaly

– unicoronal synostosis versus positional plagiocephaly

• Posterior plagiocephaly

– lambdoid synostosis versus positional plagiocephaly

• Trigonocephaly: “triangle”

– metopic synostosis versus benign metopic ridge

• Brachycephaly: “short”

– bicoronal synostosis versus positional brachycephaly


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Basic Exam (NOT anthropometry)

• Head size

• Overall shape

• Cephalic index

• Suture ridging or mobility

• Areas of flattening and prominence

• Ear placement

• Forehead shape and symmetry

• Intercanthal distance

• Orbit symmetry


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Craniosynostosis


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Craniosynostosis

Definition

• Premature fusion of one or more cranial sutures

• Incidence is 1:2500 live births


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Craniosynostosis: Virchow

• Inhibition of growth perpendicular to stenosed suture

• Compensatory growth parallel to closed suture

• Essentially, compensatory growth occurs at all patent sutures, especially the adjacent ones



NORMAL CRANIOSYNOSTOSIS

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Is this purely an aesthetic problem?

Elevated Intracranial Pressure

• Elevated intracranial pressure (ICP)

• 13% incidence of increased ICP with isolated synostosis

• 42% incidence of increased ICP with multiple suture synostosis

Complications of Elevated Intracranial Pressure

• Blindness: optic nerve atrophy, corneal exposure

• Developmental delay

• Attention issues

• Learning and language deficits

• Decreased IQ in non-operated patients when compared to patients who have surgery before 1 year of age.


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Intracranial Pressure

Symptoms

• Headache

• Nausea/vomiting

• Visual problems

• Motor, behavioral, and intellectual problems

• Vault too small for growing brain

• CSF production > egress


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Sagittal Synostosis

• Incidence: 40-50% of craniosynostosis

• Morphology: Scaphocephaly (“boat shaped”)

• Ridging in the line of sagittal suture

• Increased AP diameter and decreased biparietal width

• Bitemporal to biparietal ratio is abnormal

• Frontal bossing and occipital “coning”

• Male/female ratio: 4:1



NORMALSAGITTAL

CRANIOSYNOSTOSIS


NORMAL SAGITTAL CRANIOSYNOSTOSIS

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Treatment: Extended Strip Craniectomy


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Metopic Synostosis

• 23-28% of craniosynostosis

• Trigonocephaly

• Most obvious with midline vertical forehead ridge and keel shaped forehead

• A spectrum of severity



FRONT

BACK

NORMAL METOPIC CRANIOSYNOSTOSIS

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Metopic Spectrum


Benign metopic ridge versus metopic craniosynostosis


Metopic Synostosis

• Bitemporal narrowing

• Hypotelorism

• Epicanthal folds

• Omega sign (CT)

• Anterior cranial base increased in the AP dimension

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Unilateral Coronal Synostosis

• Incidence: 20% of craniosynostosis

• Morphology: Anterior plagiocephaly (Twisted Skull)

• Ipsilateral frontal flattening and contralateral frontoparietal bossing

• Ridging along the closed coronal suture (flat side of forehead)

• Ipsilateral occipitoparietal flattening and contralateral occipitomastoid bossing (Trapezoid)

• Ipsilateral temporal fossa bulge/convexity


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Unilateral Coronal Synostosis

• Ipsilateral ear and zygoma anteriorly displaced

• Anteriorly displaced ipsilateral glenoid fossa causes the chin to point to contralateral side

• Recessed supraorbital and lateral rim; shallow orbit

• Recessed zygoma and inferior rim

• Root of nose constricted and deviated to ipsilateral side


NORMAL LEFT CORONAL CRANIOSYNOSTOSIS


NORMAL LEFT CORONAL CRANIOSYNOSTOSIS

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Bilateral Coronal Synostosis

• Incidence: 10% of craniosynostosis

• Morphology: Brachycephaly

• Recession of supraorbital ridges with tall and broad forehead

• Shallow orbits and hypertelorism

• Increased vertical and transverse dimension of skull, but short in AP direction




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Bicoronal CraniosynostosisTreatment: Cranial Vault Distraction

• 4-6months or older

• Less blood loss

• Shorter anesthesia time (3-4 hours)

• Longer treatment time (2-3 months)

• Same Hospitalization (4-7 days)

• Same Scaring (Ear-to-ear coronal scar)

• Much greater intracranial volume gains

• Delay FOA to an older age


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Lambdoid Synostosis

• Incidence: Less than 3% of craniosynostosis

• Morphology: Posterior plagiocephaly or occipital plagiocephaly

• Ipsilateral occipital flattening and mastoid air cell prominence, downward cant of the posterior skull base to the affected side

• Larger petrous ridge angle and middle cranial fossa on the unaffected side



NORMALRIGHT LAMBDOID

CRANIOSYNOSTOSIS

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Positional plagiocephaly



Deformational Plagiocephaly

• Incidence: 1:300 in the general population (Probably more now)

• No functional sequelae- normal development, no risk of increased intracranial pressure due to head shape

Types & Occasional Associations

• Benign metopic ridge

• Scaphocephaly: NICU stay, breech presentation

• Plagiocephaly: Torticollis

• Brachycephaly: low tone

Positional Plagiocephaly


Deformational Scaphocephaly

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Positional Plagiocephaly

• Head assumes parallelogram configuration

• Ipsilateral occipital flattening and frontal bossing

• Ear displaced anteriorly

• Not craniosynostosis, but deformation secondary to external forces

• Supine positioning: American Academy of Pediatrics recommends infants sleep supine to lessen risk of sudden infant death syndrome (SIDS); this has led to an increasing incidence of deformities.

• Rotational forces: Torticollis, vertebral abnormalities, and visual field deficits may all cause preferential rotation and unequal pressure on the occiput.


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Plagiocephaly versus Craniosynostosis


Positional PlagiocephalyCraniosynostosisCraniosynostosis

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Positional Plagiocephaly CraniosynostosisUnicoronal Craniosynostosis

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Positional PlagiocephalyCraniosynostosisUnicoronal Craniosynostosis

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Positional Plagiocephaly CraniosynostosisLambdoid Craniosynostosis


POSITIONAL PLAGIOCEPHALY

RIGHT LAMBDOID CRANIOSYNOSTOSIS

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Positional Plagiocephaly CraniosynostosisLambdoid Craniosynostosis

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Treatment: Plagiocephaly

• Increased tummy time, core strengthening

• Repositioning when asleep and awake

• Physical therapy for torticollis

• Helmet as needed


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In Summary… History and Prognosis

Plagiocephaly

• Abnormal head shape not present at birth, worse at 3-4 months of age, begins to improve as child gains core strength and neck control

• History of prolonged positioning

• No functional implications

Craniosynostosis

• Abnormal head shape present at birth, worsening over time

• Concern for increased intracranial pressure, surgery indicated

• Needs timely referral (so as not to miss out on strip if applicable)


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In Summary… Diagnosis

• Scaphocephaly: “boat”

– sagittal synostosis versus deformational plagiocephaly

• Anterior plagiocephaly

– unicoronal synostosis versus positional plagiocephaly

• Posterior plagiocephaly

– lambdoid synostosis versus positional plagiocephaly

• Trigonocephaly: “triangle”

– metopic synostosis versus benign metopic ridge

• Brachycephaly: “short”

– bicoronal synostosis versus positional brachycephaly


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In Summary… Diagnosis

• If history and exam hasn’t convinced you it isn’t craniosynostosis FLASH protocol CT for diagnosis

• Skull XR has low sensitivity and specificity

• No FLASH (low dose radiation protocol) CT available?

– Refer to nearest craniofacial center


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In Summary… Treatment

Sagittal synostosis• <5 months: strip craniectomy + helmet

• >5 months: open remodeling at 9-12 months of age ideally

Coronal synostosis• Open remodeling + fronto-orbital

advancement at 9-12 months of age ideally

Metopic synostosis• <5 months: strip craniectomy + helmet

• Open remodeling + fronto-orbital advancement at 9-12 months of age ideally

Lambdoid synostosis• Open remodeling at 9-12 months of age

Multi-suture and/or late diagnosis synostosis• Open remodeling

• Cranial distraction

Plagiocephaly and Benign Metopic Ridge• Will continue to improve on own until skull

growth is complete

• Helmet may be indicated or desired by family

• No surgery indicated at any time


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References

Not otherwise mentioned:

• Huang, et al. The Differential Diagnosis of Abnormal Head Shapes: Separating Craniosynostosis from Positional Deformities and Normal Variants. Cleft Palate-Craniofacial Journal, May 1998, Vol. 35 No. 3.


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Special Thanks

www.drderderian.com


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Thank You!

www.childrens.com

Department of Plastic and Craniofacial Surgery


http://www.childrens.com/

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