Ronald L. Young II, MD

Funny shaped heads
Etiologies

Congenital deformities

Positional molding

Cephalohematomas

Mass lesions

Molded Heads
Plagiocephaly
Brachycephaly
Scaphocephaly

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

Positional Plagiocephaly
Associated with “ torticollis ”
Short sternocleidomastoid on flat side

Not stretched out
Decreased rotation away from flat side
Uterine constraint

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

Positional brachycephaly
Deep sleepers

Don ’ t move after falling asleep
Don ’ t sleep in bed

Infant seat

Bouncy seat
Hypotonia

Cause not effect

Positional Scaphocephaly
“ Premie head ”
Common in severe premies
Can evolve into craniostenosis

VP Shunt

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

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

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

Time Course
Ear asymmetry 3 y/o

Not cosmetic problem
Rare residual deformity

Why So Long? Head Growth

Treatment
Position Therapy
Keep off the flat spot
Sleep



<4 m/o Wedges and blankets
Roll on side
>4 m/o Fetal position
Let baby fall asleep in usual position

Then rotate onto side in fetal position
Start at naps

Treatment
Position Therapy
Sit up in swing
Get out of bouncy seat and infant seat
Exer-saucer
Walker
“ Tummy Time ”

Treatment
Molding Helmet
Act like dental braces
Slowly push in secondary deformity
Keep off the primary deformity

Treatment
Molding 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
Won ’ t work as well after 12 mo

Treatment
Molding Helmet
Cost

Cranialtech $3000

Advanced Orthopro
Complications

Compliance

Skin breakdown
$1400

Treatment
Molding Helmet
My indications

Borderline parents
Can ’ t get concept of keeping off flat spot

Hypotonic children
Won ’ t have head control by 4 months

Hydrocephalus post shunt
Will not have brain growth to mold skull

Significant facial bulge

Just not getting better

Craniosynostosis

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

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

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

Calvarial Development

Pathobiology
Known causes of craniosynostosis
Teratogens
Metabolic disorders


Rickets
Valproic acid, aminopterin, retinoic acid

Hyerthyroidism
Malformation Mucopolysaccharido ses

Hurler ’ s, Morquio ’ s
Hematologic disorders

Thalassemias, sickle cell anemia, polycythemia vera




Microcephaly
Encephalocele shunted hydrocephalus holoprosencephaly

Pathobiology
Syndromic


Crouzon
Apert ’ s

Pfeiffer

Jackson-Weiss

Pathobiology
<10% inherited or syndromic
90% spontaneous

Uterine constraint
Early drop into cervical canal
Multiple births
Severe back pain last month of pregnancy

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

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

Metopic

Metopic

Unicoronal

Bicoronal

Sagittal

Lamdoid

Cloverleaf deformity

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

Surgery

Surgery

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

Results

Results

Results

Thank You!