Positional skull deformity (Pediatrics 2011;128:136; Clin Pediatr (Phila) 2007;46:292)
• Etiology: Limited or selective head rotation + supine position + flat resting surface + rapid skull growth + gravity = positional skull deformity
• Basic types
• Plagiocephaly: From Greek “plagios” for oblique or slanting and “kephalos” for head, asymmetric flattening of occiput w/ anterior displacement of ipsilateral ear, forehead, & cheek; resulting in shape similar to parallelogram; ∼70% right sided, not related to handedness
• Scaphocephaly/dolichocephaly: From Greek “skaphe” for boat or skiff, ↑’d anterior–posterior relative to biparietal diameter, often develops in premies
• Brachycephaly: From Greek “brakus” for short, ↑’d biparietal relative to AP diameter, can result from symmetric flattening of occiput; more common in Asia
• Epidemiology: Depends on criteria used, seems to have ↑’d significantly since AAP’s “Back to Sleep” campaign started, likely btw 13–48% of infants under 1 yo
• Risk factors
• Assoc w/ supine pos, male gender, firstborn status, motor delay or ↓ time on abd, ↑ use of car seats/carriers, unvaried feed position “head positional preference”
• Skull deformity may be present at birth, related to intrauterine constraint (multiples, oligohydramnios, breech), forceps or vacuum use at delivery, prematurity
• Can also result from or be exacerbated by torticollis or visual deficits
• Clinical presentation
• Vast majority of parents report no flattening of head at birth
• Flattening noted, worsens between 0–4 mo; typically stabilizes 4–6 mo; becomes less noticeable after 6 mo
• Flattening of occiput, anterior displacement of ipsilateral ear, forehead, and cheek
• Head takes on a parallelogram shape
• Differential: Lambdoid craniosynostosis presents similarly; rare: Incidence 3 in 100,000
• In contrast to DP, ear posteriorly displaced (“if the ear is near, steer clear”, and refer); head takes on a trapezoidal shape
• Palpable bony ridge at lambdoid suture, between occipital and parietal bones
• Diagnosis, prevention, and treatment
• Note risk factors, evaluate head shape at each well visit
• Imaging not needed; assess for torticollis or visual defects
• Prevention: “Tummy time” while awake and observed; alternate position of head (right vs. left occiput) while sleeping supine, as well as feeding position; minimize time in car seat and other supine seating
• Initial Rx: Infant head positioning w/ rounded/non-flattened side against mattress or car seat while sleeping or sitting supine (consider Δ position of infant orientation in crib so that items of visual interest are on non-flattened side), ↑ tummy time
• Physical therapy: Neck stretching if torticollis present (may be underdiagnosed), refer if not improved by 2–3 mo
• Helmet therapy: If deformation severe or no improvement by 4–6 mo with above interventions; may be expensive. Also useful for brachycephaly (symmetric decrease in AP diameter, increase in biparietal diameter) related to supine positioning
• Formal criteria for helmet involve differences in transcranial diagonal diameters
• Decision to refer: Assessment tool available at www.cranialtech.com
Craniosynostosis (Pediatr Clin North Am 2004;51:359; Pediatr Ann 2006;35:365)
• Etiology: Premature closure of sutures causing skull deformity
• Epidemiology: Uncommon, average incidence of 1 in 2,000
• Clinical presentation: Frequently has history of abnormal head shape since birth
• Head shape depends upon which sutures fuse prematurely (sagittal most common, then coronal, then metopic, then lambdoid; in 13% multiple sutures involved)
• Head growth restricted in direction perpendicular to prematurely fused suture while compensatory growth occurs parallel to affected suture (Virchow’s law)
• Resulting restriction can lead to ↑ ICP, cognitive, and neurologic deficits
• Lambdoid craniosynostosis presents similarly to deformational plagiocephaly
• Rare: Incidence 3 in 100,000
• In contrast to DP, ear posteriorly displaced (“If the ear is near, steer clear” and refer); head takes on a trapezoidal shape
• Sagittal craniosynostosis results in scaphalocephaly
• Most common single suture synostosis, 1 in 5,000, 80% males
• May cause frontal bossing and prominent occiput
• Coronal craniosynostosis leads to plagiocephaly if unilateral, brachycephaly if bilateral
• Most common synostosis assoc with syndrome (Alagille, Apert, Cornelia de Lange, Crouzon, Treacher Collins), typically bilateral
• Unilateral 60% 
; freq assoc w/ DDH, micrognathia torticollis; plagiocephaly is anterior, rather than posterior as in positional or lambdoid synostosis
• Diagnosis and treatment
• Immediate referral to neurosurgeon before imaging is appropriate if suspected
• Treatment is with helmet and/or surgery