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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


  • Flexible enough to get through vagina

    • Molding

  • Expansile enough to accommodate rapid brain growth


Suture growth
Suture Growth

  • Sutures allow growth perpendicular to them

    • Growth at suture lines related to brain growth

Suture closure


By Age 8

Bone Union

By Age 20

Suture Closure


Early Fusion of a Suture

Sagittal Synostosis



Coronal Synostosis



Metopic Synostosis



Lamboid Synostosis



(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






VP Shunting







  • 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


  • 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

  • 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 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 Syndromes

  • Skull X-ray

  • CT

  • 3-D CT

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

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


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