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You : Welcome to the Peds Clinic ! How can I help you? Mom : Destiny’s face is all screwed up. She was rejected from her fourth Gerber photo-shoot in the last 2 weeks! . Normal Infant Skull. Expansile. Flexible enough to get through vagina Molding

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You: Welcome to the Peds Clinic ! How can I help you?

Mom: Destiny’s face is all screwed up. She was rejected from her fourth Gerber photo-shoot in the last 2 weeks!


Normal infant skull
Normal Infant Skull

Expansile

  • Flexible enough to get through vagina

    • Molding

  • Expansile enough to accommodate rapid brain growth

Flexible



Suture growth
Suture Growth

  • Sutures allow growth perpendicular to them

    • Growth at suture lines related to brain growth


Suture closure

Ossification

By Age 8

Bone Union

By Age 20

Suture Closure



Craniosynostosis:

Early Fusion of a Suture


Sagittal Synostosis

“Boat-Head”

(Scaphocephaly)


Coronal Synostosis

“Bent-Head”

(Plagiocepahly)



Metopic Synostosis

“Triangle-Head”

(Trigonocephaly)



Lamboid Synostosis

MATTRESS

“Slant-Head”

(Occipital Plagiocephaly)



Clinical exam
Clinical Exam

  • OFC

  • Head shape (from above, side)

  • Ear and facial symmetry

  • Palpate suture lines & fontanelles

    • Look for ridging

  • Look for associated anomalies

  • Skull X-ray or CT


Craniosynostosis
Craniosynostosis

Secondary

Primary

Microcephaly

Prematurity

VP Shunting

Positioning

Isolated

Abnormal

Suture

Syndromic


Prematurity
Prematurity

  • Deformational Scaphocephaly

  • Impaired mobility & prolonged positioning

  • Persists until adulthood

  • Prevention:

    • Donut-shaped head supports

    • waterbed mattresses

  • Does not warrant intervention



Vp shunting
VP Shunting

  • Scaphocephaly

  • Chronic hydrocephalus thickens the skull

  • Once decompression with shunt, the suture fuses

  • Surgery Indications:

    • OFC > 50 cm (4-5+ STDs)

    • When VPS performed during when VLBW


Microcephaly
Microcephaly

  • Surgical correction not indicated

  • Abnormal OFC

    • in primary craniosynostosis, OFC remains normal yet oddly shaped

  • Rare cases of multisutural craniosynostosis restricting head growth, but manifests with increased ICP


Positional deformation
PositionalDeformation

  • Most common cause

  • Usually forehead asymmetry

  • Sometimes associated with torticollis

  • Usually acts on coronal or lamboidal suture

  • 40% of newborns


An epidemic of lamboidal plagiocephaly
An Epidemic of Lamboidal Plagiocephaly

  • 1992: Back to Sleep

  • Campaign

  • 1996: Tertiary Care

  • Centers report rise in

  • lamboidal plagiocephaly

  • from 3% to 20%


Sorting out the epidemic
Sorting out the “Epidemic”

  • 102 Patients with occipital plagiocephaly over 4 year period

  • Only 4 (3%) had true lamboidal synostosis

  • The rest were deformational

    • Only 3 were progressive (required surgery)

    • Other responded to positioning or helmets


Syndromic craniosynostosis
Syndromic Craniosynostosis

  • 10-20 % of cases

  • Autosomal Dominant

    • Linked to Chromosome 10q

  • Multi-sutural, complex cases

If a suture is fused,

check hands, feet, big toe and thumb



Crouzon s
Crouzon’s Syndromes

  • Normal intellect

  • Normal extremities

  • 5 % have acanthosis nigricans

  • 30 % have progressive hydrocephalus


Apert s crouzon s with hand involvement
Apert’s Syndromes “Crouzon’s with Hand Involvement”

  • Varying intellect (50 % with MR)

  • Mitten Glove Syndactyly

  • Cervical vertebral anomalies

  • Rare hydrocephalus



True craniosynostosis surgery
True Craniosynostosis & Surgery Syndromes

  • Single Suture Synostosis: Confirm by exam and skull x-rays

  • Complex cases: CT or 3D CT

  • X-Ray: Fused sutures have a broad ridge of overgrowth of solid bone along a previous suture, or suture is completely obliterated

  • Ridge is especially characteristic of fused sagittal suture


Management
Management Syndromes

  • Surgery vs. Conservative Management


The decision to operate
The Decision to Operate Syndromes

  • Raised ICP in 1/3 of cases, but no neuro impairment

  • Cosmetic considerations usually most important

    • affects peer acceptance, parent-child bonding, self-image and coping


Imaging
Imaging Syndromes

  • Skull X-ray

  • CT

  • 3-D CT


Surgery
Surgery Syndromes

  • If not part of syndrome, the earlier the operation the better

    • At the latest 6-12 months (by 12 months, skull is 85% of adult size)

    • For coronal suture, operate before 2 months because of facial symmetry and visual system development

    • Procedure depends on continuing skull growth

    • Hospitalization for 3-10 days


Surgery1
Surgery Syndromes

  • Syndromic cases may need special airway support

  • Blood loss significant due to scalp vascularity

    • transfusion rates 20-500 % of infant estimated blood volume

  • PICU stay (facial edema)

  • Results on xray within several days


Metopic Syndromes

Synostosis


Surgery2
Surgery Syndromes

  • Unilateral coronal suture: difficult. Orbital relocation as well.

  • Syndromic or multi-suture cases: staged repairs.


Apert post op
Apert: Post-Op Syndromes


Crouzon surgical pics
Crouzon Syndromes Surgical Pics


Conservative therapy for deformational plagiocephaly
Conservative Therapy for Deformational Plagiocephaly Syndromes

  • Re-positioning

  • If no improvement

  • by 6 months….

    • Helmet Molding


Custom Made for each head Syndromes

24/7 wear for 4 months


Ocular morbidity
Ocular Morbidity Syndromes

  • Pre- and post-op impairments seen with unilateral coronal and metopic synostoses

    • dossociated movements

    • amblyopia

    • refractive errors

  • Ophtho involvement in work-up and follow-up


Long term follow up
Long Term Follow-Up Syndromes

  • Speech

  • Genetic Counseling

  • Feeding / Swallowing

  • Ophtho


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