This child presents with an apparently non-traumatic cranial deformity. Once non-accidental trauma has been evaluated, the differential diagnosis includes craniosynostosis and positional plagiocephaly. The newborn skull is comprised of bony plates separated by sutures – this allows for some transient deformation of the skull during birth and growth of the infant’s brain, which reaches 75% of its adult size within the first two years of life. Premature closure of the sutures – craniosynostosis – can cause growth and development retardation and hydrocephalus.
Craniosynostosis may be sporadic or associated with other craniofacial syndrome such as Apert syndrome (bicoronal synostosis, maxillary hypoplasia “flat face”, exorrbitism, arched palate), Crouzon syndrome (clinically resembles a milder form of Apert, cervical spine abnormalities), and Pfeiffer syndrome (craniosynostosis, midface hypoplasia, syndactyly). Children with craniosynostosis (especially in the syndromic context) need multiple stages of surgical reconstruction to allow for growth of the brain. These children will need non-emergent CT of the head for assessment and surgical planning (A). Although craniosynostosis is associated with many congenital syndromes, this well appearing child without acute deficit would not benefit from emergent MRI (B), especially not as a screening test.
Positional plagiocephaly is a subacute deformation of the skull from mechanical pressure. The child prefers a certain position, or is predisposed to a particular position due to congenital torticollis. Interestingly, since the beginning of the “Back to Sleep” campaign to prevent sudden infant death syndrome (SIDS), the incidence of positional plagiocephaly has increased dramatically. The child characteristically has anterior displacement of the ear on the affected side (D). This is not seen in craniosynostosis, where the ears keep their relative positions.
To prevent and treat positional plagiocephaly, the parents should continue to put the infant on his back to sleep, but can vary the child’s position in the crib (some infants tend to position their heads to face the entrance of the room). Parents should maximize time the child is not on his back – increase “tummy time” by a minute per day, place a towel roll or caregiver’s arm under the child’s upper chest when he is prone to support him, and when supine, give him something interesting to look at so that he faces the opposite direction of the deformity.
Positioning techniques are most helpful in the first six months of age; cases that are severe or do not respond to basic techniques could be treated with a customized (and expensive) head-molding helmet; after one year, these techniques are less effective.
Although non-accidental trauma (NAT) should always be in the differential of any newborn complaint, the lack of red flags after a thorough history and physical and the presence of this common finding make NAT much less likely (C). In this child’s case, follow up with his pediatrician and a short discussion of the above techniques are warranted.