Nonsynostotic Deformational Plagiocephaly: Understand, Screen, and Intervene

Laura C. Steinmann, RN; Samantha Elaine Struthers, PhD, OTR/L

Disclosures

June 17, 2014

In This Article

Introduction to Deformational Plagiocephaly

Deformational plagiocephaly (DP) refers to asymmetry of the infant skull characterized by flattening or deformation. DP can include facial and neck asymmetry. Congenital muscular torticollis (CMT) is often involved[1] and should be ruled out as a causative factor for DP. In contrast to craniosynostosis, which occurs when 1 or more cranial sutures are prematurely fused (stenosed), nonsynostotic DP is not related to bone/skull fusion. Craniosynostosis warrants surgical correction and therefore must also be ruled out as a causative factor for DP.[2] Craniosynostosis may occur in conjunction with accompanying syndromes (eg, genetic or chromosomal disorders,[3] spinal disease, etc.), and these conditions should be recognized early and monitored closely by a multidisciplinary healthcare team. This report will refer to nonsynostotic DP as DP and will review it. Craniosynostosis is not discussed in this report.

DP has a short window of opportunity for intervention while the skull bones are malleable; after age 24 months, the cranial sutures are generally fused.[4] Parents of newborns should receive preventive counseling (anticipatory guidance) and information on positioning the infant as recommended by the American Academy of Pediatrics (AAP).[5] Ideally, information on a positioning program is provided at hospital discharge and no later than at the 1-month well-child visit.Infants should be screened for skull deformity at each well-child visit to detect deformities that occur after birth, as delineated in Bright Futures: Recommendations for Preventive Pediatric Health Care.[6] These guidelines recommend a skull deformity health risk assessment in babies up to 1 year of age; however, we have documented head shape changes in children up to age 24 months.

For infants with DP, parents should be instructed to follow a 2- to 3-month course of repositioning; if no improvement is seen, AAP recommends referral to a craniofacial specialist by age 4-6 months to diagnose and direct subsequent management, which may include molding helmets or surgery.[5]

Craniofacial asymmetry may be obvious or subtle. Knowing when to monitor and when to refer for management requires an ability to recognize early DP, distinguish DP from craniosynostosis, and recognize improvement or worsening of head asymmetry in an infant.[7]

The term "plagiocephaly" is commonly used to describe any head shape dysmorphology. An Internet search for the term finds many different names including facial scoliosis, flat head syndrome, parallelogram head, and others. European studies refer to frontal and occipital plagiocephalies. Parents and caregivers may refer to DP as a "flat spot." Researchers and health insurance policies commonly use the terms "positional"or "acquired" plagiocephaly.[8] These terms imply that DP can be avoided and may lack veracity given research suggesting multiple extrinsic and intrinsic contributing factors.

Head shape deformities, particularly DP, are not well understood by healthcare providers and payers, who classify this as low-priority, a minor cosmetic issue, or simply the result of positioning practices, and who do not recognize the need for intervention. Parents may assume guilt and feel blamed because their positioning practices allowed their child to acquire the condition, and may shun treatment because they are embarrassed. The terms "positional" or "acquired" plagiocephaly do not serve the best interests of families, and the term "deformational plagiocephaly" is preferred.[9]

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