Funny Shaped Heads

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Funny Shaped Heads
Ronald L. Young II, MD
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Funny shaped heads

• Etiologies
• Congenital deformities
• Positional molding
• Cephalohematomas
• Mass lesions

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Molded Heads

• Plagiocephaly
• Brachycephaly
• Scaphocephaly

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Positional Plagiocephaly

• Occipital flattening
• Anterior displacement ear
• Bulge forehead
• Bulge malar eminence
• Parallelogram
• Local bald spot

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Positional Plagiocephaly

• Associated with torticollis
• Short sternocleidomastoid on flat side
• Not stretched out
• Decreased rotation away from flat side
• Uterine constraint

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Positional brachycephaly

• Symmetric flat occiput
• Increased bi-parietal diameter
• No ridging
• Focal bald spot
• Severe cases have ears pointing down

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Positional brachycephaly

• Deep sleepers
• Dont move after falling asleep
• Dont sleep in bed
• Infant seat
• Bouncy seat
• Hypotonia
• Cause not effect

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Positional Scaphocephaly

• Premie head
• Common in severe premies
• Can evolve into craniostenosis
• VP Shunt

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Pathology

• Soft skull
• Weight of brain able to flatten skull
• Rapidly growing skull
• Self sustaining
• Flat tire

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Time Course

• Flat spot noticeable 1 m/o
• Deformity peaks 4 m/o (Corrected)
• Head control
• Primary deformity
• Flat spot
• Secondary deformity
• Compensatory bulge

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Time Course

• Improvement of the flat spot 6 m/o
• Posterior bulge
• Dent in inion 6 m/o
• Bulge takes 12-16 months 2 y/o
• Brain molds from inside

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Time Course

• Ear asymmetry 3 y/o
• Not cosmetic problem
• Rare residual deformity

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Why So Long? Head Growth
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Treatment Position Therapy

• Keep off the flat spot
• Sleep
• lt4 m/o Wedges and blankets
• Roll on side
• gt4 m/o Fetal position
• Let baby fall asleep in usual position
• Then rotate onto side in fetal position
• Start at naps

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Treatment Position Therapy

• Sit up in swing
• Get out of bouncy seat and infant seat
• Exer-saucer
• Walker
• Tummy Time

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TreatmentMolding Helmet

• Act like dental braces
• Slowly push in secondary deformity
• Keep off the primary deformity

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TreatmentMolding Helmet

• Patients with helmets look better faster
• Need to be worn to work
• Shoot for 23 hours / day
• Need to be custom fit
• Earlier the better
• Wont work as well after 12 mo

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TreatmentMolding Helmet

• Cost
• Cranialtech 3000
• Advanced Orthopro 1400
• Complications
• Compliance
• Skin breakdown

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TreatmentMolding Helmet

• My indications
• Borderline parents
• Cant get concept of keeping off flat spot
• Hypotonic children
• Wont have head control by 4 months
• Hydrocephalus post shunt
• Will not have brain growth to mold skull
• Significant facial bulge
• Just not getting better

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Craniosynostosis
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What is it?

• Premature fusion of the cranial sutures
• Sutures allow progressive enlargement of the
  skull with brain growth
• Normal sutural fusion is complete at 6 to 8 years
  of age
• Premature fusion produces progressive skull
  deformity

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Epidemiology

• Occurs in 1 in 2100 children
• Primary non-syndromic is most common
• Lambdoid synostosis is very rare
• Multiple sutures involved in 8 of non-syndromic
  cases

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Calvarial Development

• Intramembranous ossification
• Margins of calvarial bones form osteogenic front
• Sutures form at sites of near contact
• New bone is laid down by osteoblasts in spicules
  at the sutural margins
• Overall bone growth is driven by the expanding
  brain

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Calvarial Development
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Pathobiology
Known causes of craniosynostosis

• Teratogens
• Valproic acid, aminopterin, retinoic acid
• Malformation
• Microcephaly
• Encephalocele
• shunted hydrocephalus
• holoprosencephaly

• Metabolic disorders
• Rickets
• Hyerthyroidism
• Mucopolysaccharidoses
• Hurlers, Morquios
• Hematologic disorders
• Thalassemias, sickle cell anemia, polycythemia
  vera

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Pathobiology

• Syndromic
• Crouzon
• Aperts
• Pfeiffer
• Jackson-Weiss

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Pathobiology

• lt10 inherited or syndromic
• 90 spontaneous
• Uterine constraint
• Early drop into cervical canal
• Multiple births
• Severe back pain last month of pregnancy

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Pathobiology

• Premature closure of calvarial sutures also
  affects the skull base
• Coronal and metopic
• Effect extends to nasoethmoid complex, orbital
  roofs, and supraorbital ridges

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Diagnosis

• Characteristic skull shape
• Lack of movement at suture
• Palpable ridging of fused suture
• CT scan
• Fusion, sclerosis, skull base deformities

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Metopic
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Metopic
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Unicoronal
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Bicoronal
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Sagittal
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Lamdoid
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Cloverleaf deformity
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The case for surgery

• Aesthetic and functional
• Elevated ICP in 14 of single and 47 of children
  with multiple sutural synostosis
• Uncorrected children often socially isolated and
  stigmatized
• Low risk with modern craniofacial surgery

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Surgery
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Surgery
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Complications

• Blood loss
• Infection
• CSF leak
• Post surgical trauma
• Poor cosmetic result
• Persistent cranial defects

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Results
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Results
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Results
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?
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Thank You!