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A guide to the management of plagiocephaly

Alison Wall
MSc BSc HV SCM SRN FP Cert
Health Visitor/Child Protection Lead
West Hertfordshire Primary Care Trust

The incidence of plagiocephaly seems to be on the increase, and can be a real concern to parents. Therefore, it is important that primary care practitioners are well informed about this condition, and understand how it can be prevented or resolved by early intervention.

Plagiocephaly comes from the Greek word "plagio" meaning oblique, and "cephale" meaning head. The incidence is hard to estimate, as the condition is not routinely registered. There are also degrees of misalignment, ranging from mild to severe, depending upon the length of the cranial vault. The incidence is quoted to be as high as one in 50 infants.

Causes of plagiocephaly
The "Back to Sleep" campaign, launched in 1991, has made a significant difference in reducing the incidence of sudden infant death syndrome. However, babies can be left for long periods in the supine position, increasing the risk of plagiocephaly. Typically, parents carry their infants in car seats, subjecting the back of the head to prolonged pressure. Conditions in utero can also result in misshapen heads. Maternal fibroids, small maternal stature or low levels of amniotic fluid can cause pressing on the soft skull bones. Breech presentation can also cause a high degree of pressure on the head. Prematurity is another risk factor, with the baby having a more malleable skull. Multiple births result in confined space inside the womb, and may also cause unequal pressures on the skull.

Another contributory factor is congenital muscular torticollis, which has an incidence of one in 300 live births.1 Torticollis is the shortening of the sternocleidomastoid muscle on one side. Inexplicably, it affects males more than females, and is found more often on the right side. It causes the baby to flex its head and rotate to one side. The practitioner should be aware that torticollis is associated with congenital hip dislocation, so a thorough examination is required. About 90% of babies develop plagiocephaly when torticollis is also present.2 Treatment for torticollis involves stretching exercises under the supervision of a paediatric physiotherapist, and surgical intervention if the condition persists.

Types of plagiocephaly
Three types of misshapen skull have been recognised:

  • Plagiocephaly is typically defined as posterior flattening on one side. The eye on the flattened side can appear wider as a result, and the ears can be misaligned.
  • Brachycephaly occurs with bilateral occipital flattening. There is typically an increased width to length ratio head measurement. The posterior of the head appears higher than normal and bilateral frontal bulging generally pushes the ears forward.
  • Scaphocephaly or dolichocephaly is the least common variation. This occurs when the head is pushed into an elongated narrow shape. Here, the practitioner will find a decreased width to length ratio. Overall head height is increased, and the condition is marked with a noticeable curve to the occiput.

Differential diagnosis needs to be completed to exclude craniosyntosis. This is a condition where there is premature closure of one or more skull sutures.

The coronal and lamboid are the sutures most commonly affected. It is uncommon, with an incidence of about one in 2,500.3 Craniosyntosis is associated with congenital syndromes, such as Apert's and Crouzon's syndrome. Endocrinal problems, like an overactive thyroid, can also cause overproduction of bone tissue. The condition is resolved using surgical techniques, refashioning the bone to allow the brain to grow normally.

Intervention techniques
Repositioning
Following assessment, the health practitioner needs to advise the parents about relieving pressure on the flattened areas of the skull, to encourage alignment of the skull. Techniques may include changing the position of the bottle-fed baby, and turning the baby round in the cot. This advice is not as pertinent to breastfeeding mothers as they move their baby from one side to the other to do this. Toys can be placed on the opposite side to normal to encourage head turning. Since the launch of the "Back to Sleep" campaign, "tummy time" has become a commonly used phrase to ensure that the infant is turned from supine to prone often during the day. Lifestyle advice is helpful; for example, suggesting that less time is spent in a car seat, and encouraging more activities like swimming to promote muscle development.

Remoulding
If early repositioning does not resolve the asymmetry, a referral for an orthotic assessment may be advisable. A helmet may be recommended from about six months until possibly two years of age. A helmet cannot be worn until the baby has sufficient neck strength to tolerate the orthosis. A formal referral is required to an NHS centre, but private agencies will accept direct referral from parents. Private agencies, such as the London Orthotic Consultancy and Ossur, will ensure good communication of care by sending their assessment findings and scan reports to named health professionals. A vital requirement for the success of the intervention is for parents to be fully committed and compliant. Measurements are taken before helmet fitting, and head measurements are monitored over time. The helmet or band holds onto the more prominent areas, encouraging growth in the flattened areas.

Implications for practice
Although public health priorities can impact on each other (for example, encouraging dummy use to lower the risk of sudden infant death may compromise long-term sustainability of breastfeeding), there is no question that practitioners must endorse the "Back to Sleep" campaign. There is clear evidence that since this campaign began there has been a very significant drop in cot death.4 However, parents need to be informed that babies require changes to their position to prevent prolonged pressure on one side of the growing skull and brain tissue. Research has not shown any evidence of long-term sequelae, but cosmetic effects can persist, as can visual and dental misalignment. Repositioning and tummy time need to be stressed, and an insert in the child's personal health record may remind parents. Early repositioning techniques are preferable to costly remoulding services.

Professionals need to be able to measure growth measurements accurately and monitor progress. They also need to be confident in identifying when a problem is due to positional plagiocephaly or craniosyntosis. Plagiocephaly is five times more likely to occur than craniosyntosis, but both require early interventions.
The key difficulty is that opinions are so divided about the phenomenon. Many practitioners reassure parents that nothing needs to be done, and that the skull outline will improve over time. Although this may be true in some situations, it is not proven.

Another challenge is the fact that there is no universally accepted scale of severity. In July 2007, the Department of Health (DH) told the House of Commons that there were no figures regarding the incidence of plagiocephaly.5

The National Institute for Health and Clinical Excellence (NICE) discontinued looking into the evidence base for establishing guidelines, so practitioners have no practice protocols to follow.5 A literature review undertaken in 2004 showed that interventions were effective in other countries.6

Practitioners should take note of all parental issues and address them through the appropriate channels. It might then be possible to develop clear patient pathways in the future.

References
1. Scott FS, Coulter-O'Berry C. Identification and treatment of congenital muscular torticollis in infants. Journal of Prosthetics and Orthotics 2004;16(4S):18-23.
2. Cartwright CC. Assessing asymmetrical infant head shapes. Nurse Pract 2002;27(8):33-9.
3. Hylton-Plank L. The presentation of deformational plagiocephaly. Journal of Prosthetics and Orthotics 2004;16(4S):28-30.
4. Foundation for the Study of Infant Deaths. Cot death facts and figures. Available from: http://www.sids.org.uk/editpics/356-1.pdf
5. Otway C. Plagiocephaly: awareness, prevention and
treatment. Community Pract 2008;81(4):38-40.
6. Lima D. The management of deformational plagiocephaly: a review of the literature. Journal of Prosthetics and Orthotics 2004;16(4S):9-14.

Resources
Plagiocephaly Care UK
W: www.plagiocephalycare.org.uk

Yahoo Health Group: Plagiocephaly
W: http://health.groups.yahoo.com/group/Plagiocephaly

Craniosynostosis and Positional Plagiocephaly Support, Inc
W: www.cappskids.org

Yahoo Health Group: Untreated Positional Plagiocephaly
W: http://health.groups.yahoo.com/group/OlderPlag

CranioSupport Information
W: www.craniosupport.com

London Orthotic Consultancy
W: www.londonorthotics.co.uk

Ossur
W: www.ossur.co.uk