Transcript Plagiocephaly

Skull Deformities
in Pediatrics
Case 1

7wk female with parental concern over
head shape

PMHx:
 35wk
twin A; NICU x 6days (FEN, ID, bili)
 Normal feeding/wets/stools
 Progressive “flattening” of head since birth
 No hx IVH; no neurological symptoms
 No flattening in twin
Case 1

Physical Exam:
Ht, Wt, HC tracking
 Alert, NAD
 AFOSF, RR present
 Flattened R occiput,
with protrusion of R
forehead and R ear
farther forward
 Mild flattening of L
face
 O/W unremarkable

What’s normal?

Skull growth mainly
secondary to brain
growth
40% adult size at term
 90% adult size by 1yo
 95% adult size by 6yo


Suture closure



PF 3-6mo
AF 9-18mo
C,S,L sutures 40years
What’s most likely?

Deformational Plagiocephaly:
 Asymmetric
head
 Up to 48% healthy infants
 Most common referral to Peds NSGY
 Risk
Factors:
 Intrauterine
crowding/positioning
 Decreased mobility (dev.delay, MR, prematurity)
 Posturing (torticollis, C-spine defect)
 “Back to Sleep” campaign
 Increased physician awareness
What’s the worry?

Craniosynostosis:
 Premature
closure of sutures
 1 in 2,100-3,000 live births
 Isolated (80%) vs. Syndromic (20%)
 Risk
Factors:
 Single-gene
mutations (FGFR, TWIST, MSX2)
 Rickets, hyperthyroidism
 Phenytoin, retinoids, VA, MTX, fluconazole
What’s a Pediatrician to do?
The H&P, of course!

Molding
 Pressure
in AP dimension (labor)
 Resolves in hours-weeks

Deformation
 Pressure
 No

in local region (occipital)
sutural ridging; bones mobile
Synostosis
 Premature
 Palpable
closure of sutures
ridges over suture; bones immobile
What’s normal?
PARALLELOGRAM
TRAPEZOID
Deformational Plagiocephaly
Lambdoid Synostosis
What’s not?
Metopic Synostosis
Bicoronal Synostosis
What’s not?
Sagittal Synostosis
Deformational Plagiocephaly

AKA: Positional, Posterior, Occipital,
Plagiocephaly without synostosis

Diagnosis:
 Usually
clear based on H&P
 Head shape (parallelogram)
 Xray if unsure – sutures patent
Deformational Plagiocephaly

Treatment:
 Repositioning
 Effective
in 85% mild cases
 Alternate sleeping sides
 Encourage “tummy time”
 Discourage carseats
 Helmet
 23
hrs/day
 Frequent adjustments

Typically does not require NSGY referral
When to refer?

True craniosynostosis
 1/5-1/6
syndromic
 Increased risk ICP, hydrocephalus, Chiari

Multi-D Team:
 NSGY,
Plastics, Ophtho, Neuro, ENT,
Orthodontics, Psych, Genetics, Social Work

Neurosurgery: 3-9 months old
Case 2
Newborn female, term SVD
 No maternal hx; no family hx
 Physical Exam:

 Large,
low AF
 ?Fused coronal/lambdoid sutures?
 Significant caput
 Hypertelorism
 Syndactyly on 3 extremities; clubfoot
 Broad, flat hallux
Case 2

Craniofrontonasal Dysostosis
 X-linked
 Variable
expression (F>M)
Genetics eval
 Orthopaedics eval
 Ophthalmology eval
 Neurosurgery/OMFS eval
