Plagiocephaly and Craniosynostosis

Introduction

Variation in head shape is a common reason for referral to paediatric outpatient clinics.

Positional Plagiocephaly is very common and results from moulding forces (such as position in utero, deformational forces during delivery, lying in the same position) on the developing skull. The typical features are an asymmetrical head shape with flattening on one side of the occiput. The ear, cheek and forehead may be pushed forward on the flattened side. Head shape tends to be a parallelogram when viewed from above. (The nose is straight, and the ears are aligned in the transverse plane). Positional plagiocephaly is a cosmetic condition. It does not affect brain growth or development and will generally resolve with conservative management.

Craniosynostosis (or Synostotic Plagiocephaly) is a rare condition that may cause a variety of abnormal head shapes. It arises from premature fusion of one or more of the cranial sutures. Craniosynostosis tends to cause a progressive deformity with more severe skull and facial asymmetry. There may also be premature closure of fontanelles and ridging along sutures.

Brachycephaly is the term used when the head is shortened in length and increased in both width and height with flattening of the occiput. This is usually due to deformational forces on the developing infant skull. Rarely it may be the result of craniosynostosis or syndromes such as Trisomy 21.

Pre-referral investigations

There are no investigations required for these referrals, noting that:

• Positional plagiocephaly is a clinical diagnosis and no imaging is required
• Plain film skull x-rays will not change management and should not be performed.

Pre-referral management

• Exclude the possibility of craniosynostosis
• Reassure parents that most cases of positional plagiocephaly will resolve with conservative measures
• Encourage parents to sleep baby on back as per SIDS guidelines (see Useful Resources Below)
• Reposition baby’s head to their non-preferred side while sleeping
• Practice supervised tummy time and side lying every day while the baby is awake (see Tummy Time link below)
• Encourage baby to look in both directions by
  • changing cot positions
  • sleeping baby at opposite ends of the cot
  • repositioning toys and mobiles
  • alternating sides on which baby is held.
• If positional plagiocephaly or torticollis, consider a referral to Child Development Services or a private physiotherapist.
• Monitor head circumference with serial measurements
• Helmet therapy is no longer used or recommended by Perth Children’s Hospital.

When to refer

Refer to Perth Children’s Hospital Plastics Department if:

• Concern of craniosynostosis
• Severe head shape anomaly
• Skull shape not typical of positional plagiocephaly
• Head shape worsening with time, not responding to conservative measures
• Ridging of sutures

Refer to General Paediatric Medicine if:

• Small or closed anterior fontanelle
• Bulging fontanelle
• Head circumference not tracking appropriately along growth percentiles
• Concerns regarding development or muscle tone
• Dysmorphic features
• Abnormal eye movement
• Excessive vomiting, sleepiness, irritability.

How to refer

• Routine non-urgent referrals from a GP or a Consultant should go to the Central Referral Service
• Routine non-urgent referrals from private hospitals go to the PCH Referral Office
• Urgent referrals (less than seven days) should be discussed with the on call general paediatric registrar.

Essential information to include in your referral

• Birth history - Positional Plagiocephaly is associated with prematurity, restricted intrauterine environment, assisted delivery, prolonged labour, unusual birth position
• Growth parameters and percentile charts, especially head circumference. For children born before 37 weeks gestation, please use Fenton’s growth chart.
• Physical examination findings
  • Skull shape, sutures and fontanelles
  • Ear and eye position
  • Eye movement
  • Neck mobility or masses
  • Spine and hip examination
  • Dysmorphic features
• Developmental history

Useful resources

• Plagiocephaly - Raising Children
• What is plagiocephaly (flat head)? - Red Nose
• Plagiocephaly - Clinician Assist WA
• Safe Infant Sleeping - Healthy WA
• Safe sleeping - Red Nose
• Fenton Growth Chart - University of Calgary
• Tummy time for your baby - Royal Children’s Hospital

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