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The Management of Deformational Plagiocephaly: A Review of the Literature

Dulcey Lima, CO, OTR/L


The purpose of this article is to objectively review the past and more recent literature on deformational plagiocephaly to provide a context for the accompanying articles in this dedicated issue. For this review, the term deformational plagiocephaly is used to describe head shape deformities whose etiology is mechanical in nature and is not caused by synostosis or premature fusion of one or more cranial sutures. This review discusses the literature as it relates to specific treatment options and protocols for infants with deformational plagiocephaly. Articles presenting data on potential long-term effects of deformational plagiocephaly are also reviewed and Table 1 grades studies based on the strength of the scientific rigor. Table 1 also contains a synopsis of the articles related to orthotic, surgical, and repositioning management regimes.

TERMINOLOGY

No consistent terminology exists for this head shape deformity caused by mechanical forces encountered in utero or after birth. Clarren 1 used the term "deformational plagiocephaly," Bruneteau and Mulliken 2 proposed the term "deformational frontal plagiocephaly," and Hansen and Mulliken 3 used the term "deformational occipital plagiocephaly" in their article published in 1994 to describe flattening in the occipital area. In Pollack et al.'s article 4 in 1997, the term "posterior plagiocephaly" was used generally to describe posterior flattening that was either synostotic or nonsynostotic. Later in the same article, the terms "deformational molding" and "deformational plagiocephaly" were also used to describe infants whose head shape deformities were nonsynostotic. Panchal et al. 5 use "plagiocephaly without synostosis" and "occipital nonsynostotic plagiocephaly" in an article published in 2001. News publications frequently use the lay term "flat head syndrome," which, like many of the other terms, does not adequately describe the triplanar characteristics of this deformity. Clearly, there is a need for consistent terminology to describe this condition, which is caused by prolonged mechanical forces on the infant's rapidly growing skull.

INCIDENCE

The lack of consistent terminology continues to thwart efforts to establish the incidence of deformational plagiocephaly. In addition, there has been no research that establishes the point at which normal asymmetry leaves off and deformational plagiocephaly begins. A study by Boere-Boonekamp and van der Linden-Kuiper 6 of The Netherlands sought to establish the incidence of positional head preference in a 2001 study involving 7609 infants below the age of 6 months. Neck asymmetry range of motion and the shape of the child's head were noted and compared with matched samples. Children were followed 6 months later, at 1 year, and 2 years after the initial contact. The prevalence of positional preference in infants below the age of 6 months was 8.2%. In children younger than 8 weeks, the prevalence was 10%, decreasing to 3% by 26 weeks. The prevalence of flattening of the occiput was calculated at 9.9% of the total number of children and was much higher than the authors expected. At the 2- to 3-year follow up, the skull asymmetry was still present in 45% of the children who exhibited asymmetry in the first 6 months of life, which seems to discount the widely held belief that these head shape deformities "shape up" on their own.

Peitsch et al. 7 published their findings of the incidence of cranial asymmetry in healthy newborns in 2002. In their sample of 201 healthy newborns, the researchers found a 13% incidence of localized cranial flattening and other "anomalous" head shapes in 11% of the infants who were singletons. In twins, there was a much higher incidence of localized flattening of 56%. Neonates with areas of occipital flattening had a mean of 4.74 mm of difference in their transcranial measurements, which was visually obvious in the small newborn heads measured. Infants classified as normal had a mean transcranial difference of only 0.88 mm. No follow-up measurements were included in this study because the intent was to document the incidence of deformation present at birth.

ETIOLOGY

There are several risk factors identified with the development of deformational plagiocephaly with high agreement among researchers. These include multiple births, 2,6–11 unusual birth position, 2,6–10 first-born, 2,6,8,11 and male gender. 6–15 Additional factors found to be significant are neck involvement (particularly torticollis), 1,4,6,10,11,15–23 premature birth, 4,8,10,11,24 supine positioning, 6,8,11–13,25,26 developmental delay, 1,4,5,11,27 and prolonged labor, 6–8 among other less common causes.

LONG-TERM CONSEQUENCES OF UNTREATED DEFORMATIONAL PLAGIOCEPHALY

Studies discussing the long-term consequences of deformational plagiocephaly are limited. The increase in this deformity has largely been in the last decade because parents have been instructed to place children on their backs to sleep, and the widespread use of carriers as daytime positioning devices has become a standard parenting practice. Long-term studies with large sample sizes are just now becoming possible as this first group of children introduced to these child care practices reaches middle school and younger children reach school age. It is only now that suspected problems like higher-than-normal numbers of children requiring orthodontic procedures, treatment for scoliosis, visual disturbances, 19 and auditory problems can be identified and studied because there are large numbers of children to assess. School services should be another area of investigation as researchers determine if children with deformational plagiocephaly that were untreated have a higher incidence of learning problems or social issues than norm-matched groups of children. 27

DEVELOPMENTAL ISSUES

Several authors identified developmental delay as a factor contributing to deformational plagiocephaly, but it is not known whether the developmental delay is the cause or the effect of deformational plagiocephaly. 1,4,5,11,27 Miller and Clarren 27 published a paper in 2000 researching whether there was an increased rate of developmental delay in schoolaged children diagnosed with deformational plagiocephaly as infants. They compared the number of school services needed by these children with the number of services needed by their siblings. They then compared those figures with the statistics from the state of Washington that documented the percentage of children in each grade requiring special services in 1997. A significant difference was found between the need for special education services in the group with deformational plagiocephaly compared with their siblings. Services needed by the children with deformational plagiocephaly included speech therapy, occupational therapy, and physical therapy. The researchers did not determine whether deformational plagiocephaly was an early sign of subtle brain dysfunction or whether early brain molding can lead to subtle brain dysfunction, but their research is ongoing.

Panchal et al. 5 studied neurodevelopment in 21 infants diagnosed with "plagiocephaly without synostosis" through the use of a mental developmental index (MDA) and psychomotor developmental index (PDI), which they compared with a standardized Bayley Scale of Infant Development-II (BSID II). 28 The infants were not treated for their deformational plagiocephaly and were found to score differently than the standardized distribution scores. Most (82.6%) of the children scored within normal limits, but 8.7% demonstrated delays that were significantly higher than expected (normal = 2.7%). No children were identified in the accelerated range of functioning. The authors suggested that further study is needed to determine if these results persist over time and to determine if children will score differently if they are treated for their plagiocephaly with a cranial remolding orthosis.

A presentation at the American Cleft Palate and Craniofacial Association Meeting in 2004 by Patel et al. 29 reported additional problems with development related to visual field problems. Further research needs to identify whether these areas of developmental dysfunction are significant enough to affect the normal development of a child or whether the dysfunction represents a benign problem.

STUDIES WITH RANDOM CONTROL GROUPS OR COHORT STUDIES

There is no study published with a random control group. In the United States, the use of cranial remolding orthoses and repositioning are prescribed frequently for deformational plagiocephaly, and it is questionable whether it is ethical to withhold treatment of either modality to study nontreatment with a random control group. Both types of treatment are mentioned in the majority of studies published about deformational plagiocephaly, 1–5,8,10–14,16–18,24 which could indicate that repositioning and treatment with a cranial remolding orthosis have become the standard of care for this deformity. It is possible that a randomized study with a control group can be done as an international study. A facility using cranial remolding orthoses and/or repositioning in the United States can be compared with infants in another country where treatment is either not available or is not the standard of care for deformational plagiocephaly.

Among several cohort studies, 4,10,13,16,18,30–33 are four reports comparing the results of two groups of patients: one group treated with positional therapy and the other group treated with a cranial molding orthosis. Another cohort study by Teichgraeber et al. 33 compared the orthotic treatment results of infants with deformational plagiocephaly with those with deformational brachycephaly. Teichgraeber found that infants with both skull shape deformities improved with orthotic management, but the head shape deformities of infants with deformational plagiocephaly improved more than those with deformational brachycephaly.

SURGICAL MANAGEMENT OF DEFORMATIONAL PLAGIOCEPHALY

Several studies avoid advocating the use of surgery to treat deformational plagiocephaly unless the head shape deformity is severe and the infants fail to respond to repositioning or orthotic management. 2–4,9,13,16–18,25,26,30,33–35 However, in a study of 204 patients over a 16-year period in Australia, David et al. 36 reported that 19 patients had surgery to correct their severe plagiocephaly after trying repositioning as the only treatment modality. No orthotic management was used in this study.

STUDIES USING SUBJECTIVE MEASURES

Researchers of deformational plagiocephaly sometimes use parent satisfaction or other subjective measures to assess improvement after treatment. Vles et al. 31 used a rating scale pre- and posttreatment. Pollack's group 4 questioned parents about whether they considered their child's craniofacial features: normal or nearly normal, abnormal but acceptable, or unacceptable and in need of further intervention. Members of the craniofacial team used the same scale, and at the end of treatment, the scaled assessment was repeated to assess change. David and Menard 36 asked parents if they considered their child to have an acceptable improvement in their head shape.

REFERRAL CRITERIA

There is widespread agreement that it is important to identify head shape deformities early. This is best described in the article by the American Academy of Pediatrics (AAP) clinical report entitled "Prevention and Management of Positional Skull Deformities in Infants." 35 This article advises the pediatrician and other allied health providers involved in newborn care practices to educate parents on ways to decrease the risk of deformational plagiocephaly. Tummy time while the child is supervised and awake is encouraged to prevent flattening and to facilitate development of the shoulders and trunk. The article suggests that healthcare providers discuss alternate activities with parents, including changing the position of the baby in the crib and changing the infant's orientation to the surroundings. Evaluation of cranial symmetry is recommended at each visit for healthcare services. Authors suggest examining the baby from the top of the head because from this view the head shape, ear position, and cheekbone symmetry can be assessed and documented. The healthcare provider should also examine the face and the neck for signs of asymmetry, and have an understanding of how to differentiate deformational plagiocephaly from craniosynostosis.

This report goes on to suggest that x-rays and other diagnostic tests are not necessary for ruling out craniosynostosis for most children; however, if craniosynostosis is suspected, the child should be referred to a specialist for further evaluation. If therapeutic positioning and exercise as advised by the healthcare provider does not improve the skull shape after 2 to 3 months, the AAP report suggests that the physician refer the child to a pediatric craniofacial specialist. The specialist can provide further evaluation and make recommendations that could include skull molding helmets or surgery. The specialist could also recommend physical therapy if the neck range of motion does not improve with exercises and repositioning within 2 to 3 months. 35

The AAP report notes that the best response to helmets occurs in the age range of 4 to 12 months as a result of the malleability of the infant's skull and rapid growth during this period. 35 These recommendations are substantiated by studies involving repositioning and/or the use of cranial molding orthoses as treatment modalities for this population. Researchers overwhelmingly recommend that orthotic treatment be initiated by 6 months of age to receive optimal results, 1– 4,10,12,14,17–19,24,26,30 and continue to study the variable of age and its impact on treatment results.

CONCLUSIONS

This article provides a brief overview of the literature related to deformational plagiocephaly. Table 1 gives more definitive information about selected studies, and more details are provided in the articles contained in this issue. There is a great deal of agreement about many elements of deformational plagiocephaly: there is an increased incidence of this condition across the world, the etiology is well documented, and both pre- and postnatal factors contribute to the deformity. Researchers agree that excessive time spent in the supine position has escalated the incidence of deformational plagiocephaly. They also agree that early identification and parent education about tummy time and alternate positions while the infant is awake and supervised can decrease the deformity if treatment is started early. In cases in which the deformity is still significant after a repositioning trial, the deformity will often resolve or be reduced with the use of a cranial molding orthosis, especially if treatment is completed by 12 months of age. Children older than 12 months could still benefit from orthotic management, but treatment often takes longer and outcomes could be less successful than when treatment is initiated earlier. 37

ACKNOWLEDGMENTS

Practitioners across the world have freely shared their clinical skills and expertise with the author, and this communication has enhanced understanding of this increasing deformity. Thanks are extended to the American Academy of Orthotists and Prosthetics and the Department of Education Grant to "Project Quantum Leap" for providing the context and opportunity to study these children. Hopefully, this collaborative effort will also point the way toward creative solutions for the prevention and treatment of deformational plagiocephaly.

Correspondence to: Dulcey Lima, CO, OTR/L, 21 W. 272 Coronet Rd., Lombard, IL 60148; e-mail: dulceylima@comcast.net .


DULCEY LIMA, CO, OTR/L, is affiliated with Orthomerica Products, Inc., Lombard, IL.

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