Craniosynostosis vs Positional Plagiocephaly

What’s Wrong With My Baby’s Head?
Cathy Cartwright, RN, MSN, PCNS
Pediatric Clinical Nurse Specialist
Plagiocephaly – asymmetry and twisted condition of the head
Greek (plagios –oblique + kephalē – head)
History of Deforming the Head
 Artificial deformation of the neonatal skull
 A way to differentiate from others (like tattoos, body piercing)
 Known since 4000 BC in ancient Phoenicia
 Compressing front and back of head with board and pads
 Cradle-board
 Tightly wrapping the head with a binding
Most craniosynostosis is recognizable at birth
 Some molding of the skull during birth process, but usually
normalizes by 3 weeks of age
Craniosynostosis – premature closure of one or more cranial sutures
 Nonsyndromic – most common
 Over 90 syndromes
 Partial closure of one suture can cause a deformity
 Important to recognize craniosynostosis or positional molding early so
appropriate intervention can take place
 Genetic conditions (mutations in fibroblast growth factor receptors)
 Metabolic disorders - hyperthyroidism
 Hematologic disorders
 Overshunting hydrocephalus (secondary craniosynostosis)
 In utero head restraint – multiple births, maternal smoking, valproate
 Brain is contained in neurocranium which is comprised of skull base
and cranial vault
 Sutures allow infants head to reshape during and after birth process
and to accommodate rapidly expanding brain
 Removing skull in neonate with intact dura results in the dura
regenerating the skull with suture placed as dictated by the dura
 Bone growth occurs from the expanding brain
 Skull is 35% adult size at birth and 90% adult size by 7 yrs
Nonsyndromic Craniosynostosis
 1 out of 2100 children
 Multiple suture synostoses involving 2 or more cranial sutures occur
in 4-8% of nonsyndromic craniosynostosis
 Simple craniosynostosis is usually random in occurrence but 2-6% of
isolated sagittal synostosis and 8-14% of coronal synostosis were
found to be familial
 In utero head restraint also thought to be a cause but is most
commonly seen as positional plagiocephaly
Sagittal Synostosis – most common
 Called scaphocephaly (boatlike shape to skull)
 40-60% of all craniosynostosis
 Bitemporal narrowing, frontal bossing, occipital cupping, palpable
sagittal ridge
CT - Closed sagittal suture with ridge
Lateral skull x-ray
Coronal Synostosis
 Anterior plagiocephaly
 20-30% of all craniosynostosis
 Vertical dystopia, nasional deviation, flattening of frontal bone on
affected side
 Strabismus from ipsilateral superior oblique paresis and compensatory
contralateral head tilt present in 50-65% of unilateral coronal
 See ophthalmologist familiar with craniosynostosis
 AP skull film shows harlequin appearance to orbit as superior orbital
rim is elongated
CT - closed L coronal suture
AP skull x-ray - Harlequin sign (L)
Metopic Synostosis
 Trigonocephaly
 Involves metopic suture
 10% of all craniosynostosis
 Triangular shape
 Bitemporal narrowing
 Parietal bossing
 Hypotelorism
 Metopic ridge
 Can have normal skull shape and just a ridge (fussy?)
Lambdoid Synostosis
 Occipital (or posterior) plagiocephaly
 Involves lambdoid suture
 1-2% of all craniosynostosis
 Trapezoid shape to skull when viewed from above
 Tilted skull base (affected side displaced inferiorly
 Affected ear displaced inferiorly and posteriorly
 Palpable ridge along suture line
 Occipitomastoid bulge
 Towne’s View skull x-ray
Classifications of Craniosynostosis
Type of Craniosynostosis
Suture Involved
Bitemporal narrowing
Frontal bossing
Occipital cupping
Palpable sagittal ridge
Anterior plagiocephaly
Vertical dystopia
Nasional deviation
Flattening of frontal
bone on affected side
Triangular shape
Bitemporal narrowing
Parietal bossing
Metopic ridge
Posterior plagiocephaly
Trapezoid shape
Tilted skull base
Important to differentiate between lambdoid synostosis (treatment is
surgery) and positional plagiocephaly (usually no surgery).
Lambdoid Synostosis
Positional Plagiocephaly
Usually present at birth
Usually not present at birth
Trapezoid shape when viewed
from above
Parallelogram shape when viewed
from above
Ipsilateral ear displaced posteriorly
and inferiorly
Ipsilateral ear displaced anteriorly
Bony ridge palpable over closed
lambdoid suture
No bony ridge over lambdoid suture
Unilateral occipito-parietal
flattening posteriorly
Usually unilateral occipito-parietal
flattening but can be bilateral
When viewed posteriorly there is
an ipsilateral occipitomastoid bulge
and the skull base appears tilted
When viewed posteriorly the skull
base is horizontal and no occipitomastoid bulge
Radiographic evidence of closed suture
(Towne’s view, CT with bone windows,
CT with 3D recon)
Radiographic evidence of open
May have torticollis
Positional Plagiocephaly
 Deformational forces such as the birth process, can shape the skull
 Infant brain grows rapidly during first several months after birth
 This growth expands the skull into its normal shape
 H.C. is 35 cm at birth and increases 9 cm by 6 mo and 12 total by 1
year – 2 ¼ cm from age 1 -2 yrs and ¾ cm from age 2-3 yrs
 Deformational forces can have a significant impact during the period
of rapid skull growth (infant seat, car seat, mattress, swing, stroller)
 2 mo – 700 hours sleeping - Frequently noticed by pediatrician at 2
mo exam (normal at birth)
 Head must be rotated to redistribute the forces of gravity
 Further aggrevated by torticollis - tightening of sternocleidomastoid or
cervical muscles that prevent the infant from turning his head 180
 Significant increase in positional plagiocephaly since 1992 when the
American Academy of Pediatrics initiated the back to sleep campaign
and recommended that infants sleep on their backs or sides to
decrease the incidence of SIDS
 Parents relate that baby preferred to sleep on back with head turned to
one side
 Can have flattening of one side or the entire occipital bone
 Head shaped like parallelogram
Treatment for Positional Plagiocephaly
 Can be prevented – reposition the infant’s head when lying supine,
starting from birth - doesn’t cause brain damage
 Toys or objects of interest placed on nonpreferential side
 Alternate arms when feeding
 Put sibling on nonpreferred side
 Tummy time when awake
 Cranial orthotic device to correct moderate to severe cases if parents
have tried to reposition without success
 Most effective between 4 – 12 months
 Refer to orthotist experienced in cranial orthotic devices for positional
 Reimbursement issues (careful with documentation – indicate that
parents tried repositioning without success if true)
 Correct torticollis if present
 Static stretching exercises
 Confirm no C-spine defect first
 Slowly turn head 90 degrees toward non-preferential side, hold it for
10 sec – someone may need to hold shoulders
 Correct head tilt
 Do 5-6 times per day or with every diaper change
 Sternocleidomastoid tumor of infancy
Syndromic Craniosynostosis
 Present with characteristic group of findings
 Multiple cranial suture synostoses including sutures of the cranial
base, resulting in complex face and skull deformities
 Frequently associated with medical problems including
hydrocephalus, papilledema, respiratory distress and failure to thrive
 Most common are Crouzon, Apert, Pfeiffer
Crouzon Syndrome
 First described by French neurologist in 1912
 Autosomal dominant
 Incidence – 1/25,000 births
 Caused by multiple mutations in fibroblast growth factor receptor 2
 Bicoronal synostosis (short cranium, broad, flat forehead, may have
sagittal or lambdoid synostosis or even a cloverleaf deformity –
 Various degrees of exorbitism (exophthalmos, proptosis),
hypertelorism and maxillary/midface hypoplasia
 High risk for serious ocular abnormalities (papilledema, optic atrophy,
corneal exposure and proptosis)
 Tarsorraphy
 Conductive hearing loss
 Serious airway compromise, challenges with oral feeding (trach, GT)
 At risk for developing hydrocephalus and/or Chiari
Apert Syndrome
 Acrocephalosyndactaly Type I (named after French neurologist who
described the syndrome in 1906)
 Most complex of craniofacial syndromes
 Autosomal dominant
 Incidence 1/50,000 to 1/160,000
 Mutation of FGFR2 gene
 Multiple suture synostosis
 Skulls are turricephalic (tower-like)
 Flat and elongated forehead, bitemporal widening and bilateral
flattening of the occiput
 Beaked nose
 May have hydrocephalus and agenesis of the corpus callosum
 Syndactaly (fusion of digits in hands and feet)
 Dental abnormalities (cleft palates), conductive hearing loss, cardiac
anomalies and chronic acne
 Mental retardation and learning disabilities are higher in this group
than in Crouzon, although many can have normal intelligence
Pfeiffer Syndrome
 Autosomal dominant
 Incidence of approx. 1 in 200,000
 Caused by mutations in FGFR1 or FGFR2
 Multiple suture synostosis, varying degrees of mental retardation,
midface hypoplasia and upper airway anomalies
 Proptosis
 Broad thumbs and great toes
Important for these children to be evaluated by an experienced craniofacial
Craniofacial Team
 Craniofacial Surgeon
 Neurosurgeon
 Orthodontist
 Geneticist
 Speech Pathologist
 Social Worker
 Audiologist
Advanced Practice Nurse
Pediatric Dentist
Treatment for Craniosynostosis
 The American Medical Association defines cosmetic surgery as
“surgery performed to reshape normal structures of the body in order to
improve the patient’s appearance and self-esteem. Reconstructive surgery
is performed on abnormal structures of the body, caused by congenital
defects, developmental abnormalities, trauma, infection, tumors or
disease. It is generally performed to improve function, but may also be
done to approximate a normal appearance”
 Also done for cosmetic and psychological benefits
Can’t wear ball caps, helmets, hard to pull shirts on over head
Vertical dystopia
Possible increased ICP – can lower IQ
1888 – L.C. Lane performed first craniectomy to remove a stenosed
suture on a 9 mo old infant
 High mortality rates, anesthesia high risk, abandoned until 1927 to
prevent blindness
Usually not before 3 mo of age (3-12 mo is usually time to operate)
Many techniques:
 Fronto-orbital – metopic, unicoronal, bicoronal, syndromic (usually
staged procedures) with calvarial vault remodeling
 Pi – extended strip craniectomy
 Hung span – correct severe sagittal synostosis
 Strip craniectomy – treats sagittal synostosis, best before 6 mo of age
 Endoscopic strip craniectomy – all sutures, best results before 4
months of age
Important to identify craniosynostosis early so pt. has more options and
may undergo less invasive treatments
Endoscopic strip craniectomy
 Developed by Jimenez and Barone in 1996
 Calvarial vault remodeling – blood losses from 25% - 500%
 267 patients over 8 years
 Decrease in blood loss/transfusions
 Overnight stay
 Molding helmet for approximately 1 year
Postoperative Nursing Care
 frequent v.s. with neuro assessments
 watch for early signs of blood loss, electrolyte imbalance, neurologic
deterioration or CSF leak
 swelling, airway problems
 pain control
 parent participation
 discharge and follow up care
 Babies with sagittal synostosis should lie with the back of the head on
the mattress to decrease the AP length
Long Term Outcome
 286 patients from 1996-2004
 Questionnaires mailed to 141 known addresses
 Asked parents to evaluate cosmetic, psychosocial, neurocognitive and
overall satisfaction
 Mean follow up – 6.1 years (range 3-11 years)
 100% would recommend procedure to family/friends and do it again
 92% excellent school performance
 <5% had revision of any kind
 10% teased sometimes
 No child reported being teased often
American Medical Association Policy of House of Delegates, June 1989, p.A-89.
Definitions of “cosmetic” and “reconstructive” surgery, H-435.992. Council of Medical
Services Annual Meeting.
Cartwright, C.C., Jimenez, D.F., Barone, C.M., & Baker, L. (2003). Endoscopic strip
craniectomy: a minimally invasive treatment for early correction of craniosynostosis.
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