Craniosynostosis may be associated with a syndrome or be isolated (nonsyndromic). Syndromic cases are accompanied by the involvement of several organ systems and additional malformations. Head shape is dependent on which sutures are fused. Differentiating craniosynostosis from deformational abnormalities requires taking a thorough history and physical of the infant. The following table describes characteristics that may help to differentiate the two. [Looman: 2012] [Nield: 2007] [Ursitti: 2011]
|Nonsynostonic cranial deformation||Craniosynostosis|
|Head shape at birth commonly
round and symmetrical
|Head shape at birth may be abnormal|
|History of constant supine
positioning with little prone
positioning or “tummy time”
|May have family history of craniosynostosis and/or genetic syndromes|
|Shows preference for one
head position and may
have bald patch on one
side of the back of the head
|Head shape may be
parallelogram or brachycephaly
|Head shape may be scaphalocephaly, trapezoidal, trigonocephaly, or brachycephaly|
|May present with torticollis|
|Ear, forehead, and/or
cheek may be anteriorly shifted
|Posterior displacement of the ear|
|Slight mobility of bones
next to sutures
|Bones next to the sutures are immobile|
|Usually bony ridges found
on palpation of sutures
|Bony ridges found near sutures on palpation|
- Deformational plagiocephaly is the flattening of one side of the posterior skull, creating an oblique or slanted head when viewed from the top. Ipsilateral (along the same side) frontal bossing and asymmetric facial features result, however, the degree of flattening of the occipital skull is more pronounced and results in a parallelogram shape when viewed from above the infant’s head. See Vertex views of lateral and posterior deformational plagiocephaly (Journal of Pediatric Health Care), Table 1 in [Looman: 2012].
- Deformational brachycephaly is the symmetric flattening of the occipital skull with compensatory bi-parietal widening, giving the appearance of a large head when viewed from the front. Some degree of asymmetry is commonly seen. See photo. These infants may also have a posterior protrusion at the top of the head. When looking at the infant skull from the side, the skull appears to slope downward towards the anterior portion of the head. This is called a “turricephaly” or “tall head.” Images of brachycephaly (Acta Paediatrica), Figure 3 (page 4) in [Ursitti: 2011].
- Deformational scaphocephaly (also known as dolichocephaly) is an uncommon variant seen in preterm infants who have been positioned on their sides, resulting in flattening of the sides of the head and compensatory increase in the anterior posterior dimension of the cranium. See photo. Preventive measures instituted in NICUs have decreased the incidence of this. Images of scaphocephaly (Acta Paediatrica), Figure 1 (page 3) in [Ursitti: 2011].
- Mechanical adjustments and exercises that can be done at home. These include positioning the infant’s rounded side of the head against the mattress and changing the crib’s position so the infant would need to look away from the flattened side to see people enter the room. [Laughlin: 2011] Repositioning only works prior to the infant acquiring independent head control, which typically occurs by 4 months of age, corrected. Within 2-3 months, most infants will show improvement. [Task: 2000]
- Physical therapy to address congenital muscular torticollis (CMT) and positional preferences. A physical therapist can teach parents to address congenital muscular torticollis (CMT) and positional preferences with 3 repetitions of stretches that are performed at each diaper change. [Laughlin: 2011] The stretches usually involve placing one hand on the child’s upper chest while the other gently rates the chin until it touches the shoulder. This is held for 10 seconds and then repeated for the other side. [Laughlin: 2011]
- Sleep orthotic or cradle: these devices position the infant’s occiput on a concave rather than a flat surface. This redistributes the surface pressure on the occiput but maintains the infant in the supine position. This approach only works prior to 3-4 months of age corrected, when infants develop sufficient head control to defeat its purpose.
- Helmet therapy: This orthotic is typically used for moderate to severe deformational flattening. [Rogers: 2011]
[Rogers: 2011] Several principles are important:
- Helmets are only effective when there is remaining skull growth
- Younger children correct quicker than older children, consistent with the rate of growth of the cranium
- Helmets do not apply pressure to the cranium but rather have foam selectively cut away from the area in which growth is desired thereby guiding growth in the path of least resistence. They do not mold or squeeze the cranium.
- A skilled orthotist is needed to monitor the growth of the infant’s head and the fit of the helmet.
- Preventative counseling by 4 weeks of age: Provide information about tummy time while the infant is awake and being observed (at least 30-60 minutes) and alternating head positon at night during sleep. Too much time in safety seats or swings should be discouraged. [Rogers: 2011]
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|Reviewing Author:||Faizi A. Siddiqi, MD - 6/2015|
|Content Last Updated:||6/2015|
Looman WS, Flannery AB.
Evidence-based care of the child with deformational plagiocephaly, Part I: assessment and diagnosis.
J Pediatr Health Care. 2012;26(4):242-50; quiz 251-3. PubMed abstract / Full Text
Nield LS, Brunner MD, Kamat D.
The infant with a misshapen head.
Clin Pediatr (Phila). 2007;46(4):292-8. PubMed abstract
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part I: terminology, diagnosis, and etiopathogenesis.
J Craniofac Surg. 2011;22(1):9-16. PubMed abstract
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part II: prevention and treatment.
J Craniofac Surg. 2011;22(1):17-23. PubMed abstract
Task Force on Infant Sleep Position and Sudden Infant Death Syndrome.
Changing concepts of sudden infant death syndrome: implications for infant sleeping environment and sleep position. American Academy of Pediatrics. Task Force on Infant Sleep Position and Sudden Infant Death Syndrome.
Pediatrics. 2000;105(3 Pt 1):650-6. PubMed abstract / Full Text