Craniosynostosis

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You Welcome to the Peds Clinic ! How can I
help you? Mom Destinys face is all screwed
up. She was rejected from her fourth Gerber
photo-shoot in the last 2 weeks!
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Normal Infant Skull
Expansile
Flexible

• Flexible enough to get through vagina
• Molding
• Expansile enough to accommodate rapid brain growth

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Infant Skull Anatomy
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Suture Growth

• Sutures allow growth perpendicular to them
• Growth at suture lines related to brain growth

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Suture Closure
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Early Closure Causes Growth Parallel to the Suture
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Craniosynostosis Early Fusion of a Suture
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Sagittal Synostosis
Boat-Head (Scaphocephaly)
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Coronal Synostosis
Bent-Head (Plagiocepahly)
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Right Coronal Synostosis
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Metopic Synostosis
Triangle-Head (Trigonocephaly)
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Metopic Synostosis
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Lamboid Synostosis
MATTRESS
Slant-Head (Occipital Plagiocephaly)
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Isolated Primary Craniosynostosis Sutures Involved
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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

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Craniosynostosis
Secondary
Primary

• Microcephaly
• Prematurity
• VP Shunting
• Positioning

Isolated Abnormal Suture Syndromic
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Prematurity

• Deformational Scaphocephaly
• Impaired mobility prolonged positioning
• Persists until adulthood
• Prevention
• Donut-shaped head supports
• waterbed mattresses
• Does not warrant intervention

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Former Preemies Head
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VP Shunting

• Scaphocephaly
• Chronic hydrocephalus thickens the skull
• Once decompression with shunt, the suture fuses
• Surgery Indications
• OFC gt 50 cm (4-5 STDs)
• When VPS performed during when VLBW

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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

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PositionalDeformation

• Most common cause
• Usually forehead asymmetry
• Sometimes associated with torticollis
• Usually acts on coronal or lamboidal suture
• 40 of newborns

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An Epidemic of Lamboidal Plagiocephaly

• 1992 Back to Sleep
• Campaign
• 1996 Tertiary Care
• Centers report rise in
• lamboidal plagiocephaly
• from 3 to 20

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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

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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
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Distinguishing Clinical Features in the
Craniosynostosis Syndromes
Muenke Crouzon Jackson-Weiss Apert Pfeiffer Bear-Stevenson
Thumbs Normal Normal Fused to fingers Broad, deviated Normal
Hands  Carpal fusion Normal Variable Bone syndactyly Variable brachydactyly Normal
Great Toes  Broad Normal Broad, deviated Fused to toes Broad, deviated Normal
Feet  Tarsal fusion Normal Abnormal tarsals Bone syndactyly Variable brachydactyly Normal
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Crouzons

• Normal intellect
• Normal extremities
• 5 have acanthosis nigricans
• 30 have progressive hydrocephalus

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ApertsCrouzons with Hand Involvement

• Varying intellect (50 with MR)
• Mitten Glove Syndactyly
• Cervical vertebral anomalies
• Rare hydrocephalus

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Apert Extremity Findings
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True Craniosynostosis Surgery

• 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

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Management

• Surgery vs. Conservative Management

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The Decision to Operate

• 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

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Imaging

• Skull X-ray
• CT
• 3-D CT

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Surgery

• 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

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Surgery

• 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

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Metopic Synostosis
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Surgery

• Unilateral coronal suture difficult. Orbital
  relocation as well.
• Syndromic or multi-suture cases staged repairs.

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Apert Post-Op
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CrouzonSurgical Pics
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Conservative Therapy for Deformational
Plagiocephaly

• Re-positioning
• If no improvement
• by 6 months.
• Helmet Molding

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Custom Made for each head 24/7 wear for 4 months
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Ocular Morbidity

• 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

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Long Term Follow-Up

• Speech
• Genetic Counseling
• Feeding / Swallowing
• Ophtho

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