Management of Positional Skull Deformities: Who Needs a Helmet?
Martin W. Stallings, MD, FAAPKings Mountain Pediatrics; Kings Mountain, NC 28086, USA
To the Editor.—
The recent clinical report in the July 2003 issue of Pediatrics "Position and Management of Skull Deformities in Infants" is interesting and informative. It is intriguing that the simple policy of babies sleeping on their backs can have such a profound effect on decreasing sudden infant death syndrome (SIDS). One cannot help but wonder what other factors in our lives may be causing a significant illness or morbidity. We have learned that changing the sleeping position to decrease SIDS causes another abnormality—positional skull deformity. This is an easy trade-off that we gladly accept to prevent such a horrendous illness as SIDS.
I propose that there is another pediatric abnormality that is affected by the "Back to Sleep" policy but in a favorable manner. This is metatarsus adductus, a once-common finding on routine physical examinations but now, in my experience, very uncommon. With children now sleeping on their backs, the feet are free to assume the normal position, dictated by normal growth after being cramped and possibly adducted in utero. When babies sleep on their abdomen, the feet are often tucked inward due to the normal flexing of the hips, knees, and ankles. This puts inward torsion on the forefoot, which promotes it to become adducted or to maintain the previous in utero-acquired adduction. Colleagues whom I have informally queried agree with the decreased incidence of metatarsus adductus. Studies could and should be done to confirm and quantitate this decrease.
Once again, if confirmed, this makes us aware that changes we may recommend in one’s life may affect other aspects that are unanticipated. As guardians of children’s health, we should always be alert to this possibility.
John Arthur Persing, MD
Yale Plastic Surgery; New Haven, CT 06510-3206, USA
In Reply.—
We have little comment on the letter by Dr Martin Stallings, because we did not study this condition.
However, a number of colleagues have noted a similar reduction in incidence of metatarsus adductus with the supine sleep-positioning recommendations. It might be worth additional, separate study to clarify the issue further.
We would caution Dr Stallings, however, that one of the main points of the paper was not that we should accept a skull deformity for anti-sudden infant death syndrome (SIDS) development. (Dr Stallings notes that we would readily accept some deformed skulls in order to reduce the likelihood of SIDS.) We would agree if this were the only alternate option. However, we can largely prevent skull deformity by simple measures such as alternating the head position on a nightly basis from the first day of life and still get the benefit of supine positioning-related SIDS reduction.
Peter D. Rappo, MD, FAAP
Department of Pediatrics; Harvard University School of Medicine; Brockton, MA 02301, USA
To the Editor.—
I read the report entitled "Prevention and Management of Positional Skull Deformities in Infants"1 in the July 2003 issue of Pediatrics with interest. The American Academy of Pediatrics has provided a useful summary to primary care clinicians regarding the diagnosis, evaluation, and management of a clinical problem with an apparent sixfold increase in frequency since the implementation of the "Back to Sleep" campaign. I feel compelled, however, to comment on some practical issues that are likely to be confronted by pediatric generalists as they implement management strategies for patients who have positional plagiocephaly. Although parents in suburban practice are knowledgeable about the current sleep-position recommendations, it is apparent that these recommendations are not universally followed among providers of day-care services and within certain ethnic communities, thus demonstrating differential rates of plagiocephaly depending on the acceptance of the initial positioning guidelines. Although preventive counseling, mechanical adjustments, and exercise are logical interventions, there is a surprising reluctance for parents to position their children in a supine fashion, even when the children are awake. If clinicians accept that most children do not have a true restrictive torticollis, there may still be some resistance to motion as the caretaker attempts to move the infant’s head through a 180° arc. This statement would have been additionally helpful if it had recommended an increased frequency of visit follow-up to assure compliance with and improvement from an exercise program. A particularly distressing personal experience has been the seeming unwillingness of pediatric physical therapists to train or encourage parents in an appropriate exercise-management program.
If we postulate that children with craniosynostosis are diagnosed and managed correctly, then the need for surgery and orthotic devices should be comparatively low. The literature for orthotic-device outcomes is surprisingly thin, shows only modest improvement effects over nonmechanical interventions, and is largely generated by individuals directly involved in the manufacture of cranial remodeling devices. Review of the approval of such devices on the Food and Drug Administration website (www.fda.gov) reveals a similar dearth of references as to the effectiveness, safety, and usefulness of these orthotic devices. There is an evolving literature that suggests that children with occipital plagiocephaly have large heads and increased Sudbury fluid between the skull and meninges. There is also speculation that this fluid may lead to a greater risk of plagiocephaly due to the nondeformability of this fluid relative to other cranial structures.2 Although there is limited anecdotal information from pediatricians about side effects from such cranial orthotic devices (pressure sores, local irritation, increased irritability, and odor issues), visits to parent support web sites for "Plagio babies" reveal the range of difficulties that parents have leaving the helmets on for 23 hours a day over several months. Finally, there is some speculation in the neurosurgical community that children with external hydrocephalus and consequent positional plagiocephaly may be at greater risk for serious head trauma from seemingly minor injuries. Although there are no data that suggest that the use of cranial-remodeling devices leads to direct intracranial injury, it is difficult not to speculate that the application of steady pressure, for an extended period of time, to a cranial axis with some deformable and nondeformable structures would not have some potential effect beyond simple reshaping of the infant head. Such speculation would seem to suggest a potential research alliance between pediatric generalists and specialists and mechanical engineers with an understanding of fluid dynamics. As the number of children with this condition continues to increase, the recommendations of the American Academy of Pediatrics and its potential guidance for future research endeavors will assume a level of paramount importance.
REFERENCES
1. Persing J, James H, Swanson J, et al. Prevention and management of positional skull deformities in infants.
Pediatrics.2003; 112
:199
–202
2. Sawin PD, Muhonen MG, Menezes AH. Quantitative analysis of cerebrospinal fluid spaces in children with occipital plagiocephaly. J Neurosurg.1996; 85 :428 –434[Web of Science][Medline]
John Arthur Persing, MD
Yale Plastic Surgery; New Haven, CT 06510-3206, USA
In Reply.—
Dr Rappo describes a number of points regarding the management of infants with plagiocephaly. First, he states that there is a variable degree of compliance with supine positioning for sleep by different groups, day-care providers, and ethnic groups. We agree that this is the case, and we have outreach programs that describe both the importance of supine positioning for sudden infant death syndrome prevention and avoidance of skull deformity. We have not encountered the same degree of resistance among physical therapists who teach the exercises. Rather, there are a number of competing exercise programs that individual therapists seem to advocate. Regardless of the type of technique, the basic components relate to stretching muscles that are abnormally shortened to include the lateral neck musculature and the sternocleidomastoid. Monitoring of this clearly needs to be done either through the pediatrician’s office or a combination of the physical therapist’s office and a craniofacial-team program.
It is true also, as Dr Rappo has noted, that literature related to orthotic devices is scant, and the outcomes are relatively difficult to discern. This largely relates to the inaccuracies of the measuring tools currently available for measuring the skull shape. As has been mentioned earlier, the skin surface "cephalometric" measurements are not terribly reliable, related to the lack of an absolute cephalometric point from which to measure and the fact that soft-tissue components overlie the bony landmarks. This could be obviated by doing computed tomography scans. However, the risk of repeated radiation exposure, anesthesia and sedation, and cost for the child outweigh the potential benefits of this analysis (at least on a widespread basis). Laser measurements that have been developed for 3-dimensional imaging do show the prominence of these measurements without the added risk of radiation. However, the tools are very expensive, but they may be used primarily for developing standards and efficacy in a research setting.
Macrocrania with or without external hydrocephalus has been associated with a higher incidence of plagiocephaly in clinical practice. Presumably this is related to the fact that it is harder to lift a heavier head with immature neck muscles than it is a smaller head. The concern related to intracranial effects of a helmet on brain development is untested as yet. Although it is remotely possible that there could be some internal effects related to selective pressure on high pressure points on the skull, one has to remember that in addition, there is selectively less pressure in areas of flattening. The amount of pressure delivered by helmets, generally, is also extraordinarily low. It is not significantly different from that which the child receives when lying on the back of the head going to sleep. We think the major point is that this is unknown and could be investigated further. In any study design one would also have to sort out preconditions such as prematurity and primary neurologic disease (which may predispose a child to plagiocephaly) among other conditions before conclusions could be drawn.
Shelley Lanzkowsky, MD
Morristown, NJ 07960, USA
To the Editor.—
I read with interest an article published in the July 2003 issue entitled "Prevention and Management of Positional Skull Deformities in Infants." In my experience, positional plagiocephaly improves within the second year of life. The authors state, however, that those requiring helmet therapy should receive it between 4 and 12 months. I would like to ask the authors how they determine at 4 months or so which kids would require helmet therapy without spontaneous resolution. Where I practice, helmet therapy is not always covered by insurance and costs thousands of dollars. I would hate to refer patients for a costly therapy if a condition is benign and transient.
John Arthur Persing, MD
Yale Plastic Surgery; New Haven, CT 06510-3206, USA
In Reply.—
Dr Lanzkowsky asked an appropriate question: What severity and at what age should helmet therapy be considered?
It is a difficult question to answer because, although there is debate about this issue, we believe there are no reliable, accurate standards to determine who should get a helmet and who should not. It is true that there is improvement in skull shape for some that occurs with time, and no additional treatment is necessary. Therefore, this approach is appropriate for this subgroup. However, it is also true that skull deformity may persist for many, and after 1 year of age, the degree of improvement is relatively slight. The bone of the skull at this time becomes more dense and mineralized and does not remodel readily. We have not seen many patients develop significant improvement in skull shape beyond 15 to 18 months of age with conservative measures (observation alone). Quite the reverse, we have seen many patients who have persistent deformity into late childhood once the deformity has persisted to 
2 years of age. The deformity in the occiput, however, is oftentimes masked by additional hair growth, and to the casual review, the deformity "has gotten better." The mainstay of treatment for improvement in skull shape relates to physical therapy so that the infant does not prefer to lie on areas in the occiput that are flattened already. If full range of motion of the neck is achieved, there is a lesser deforming influence for the skull but also, and particularly so, in the face. In truth, most if not all of us have some asymmetry in the face, and we should anticipate that a small degree of asymmetry is "normal"; what we are addressing here is minimizing the asymmetry to the level that it is not recognized as a "deformity" as the child grows older.
To determine which patients improve with helmet therapy, we attempted to use measurements of the long and short axis of the skull by calipers, but these measurements in many have been irregularly reproducible; the exact measuring points are not defined readily, and soft tissue overlying the bone (ie, the scalp) is compressible. A much more accurate method for measure would be serial computed tomography scans, but clearly this would be a significant health risk to the patient related to the repeated radiation and anesthesia/sedation exposure, as well as the significant expenditure. Laser-based measurement systems are an excellent alternative, but they are costly. Therefore, we are left with a more subjective, judgment-based analysis that includes not only physical features such as the apparent degree of skull deformity but also social factors to determine treatment. We use a crude scale of mild, moderate, and severe deformity based on the visible deformity. We also factor in how old the child is. (We know that after 1 year of age the skull is much more difficult to remodel using a helmet.) Social factors such as how much time the child is actually receiving care from the parent are also important. This may be influenced by the parents return to work and leaving the child with nonfamily caregivers. The attention paid to exercises typically is not as good as when both parents are actively involved in the care of the child in the home. Persistence of deformity also may be influenced by multiparity and willingness or ability to comply with physical therapy exercises. It may be difficult for a family member to spend sufficient time to treat the affected child (or children) due to the time requirements for care of the other children.
In summary, our treatment plan at this time is admittedly subjective because of the lack of inexpensive, reproducible, and accurate measurements. Recognition that there are varying states of maturity of bone, despite the same chronologic age (meaning that some children will be resistant to change at 1 year of age, and others will not be as resistant) and social factors such as the amount of time the caregivers are actually working on (and are motivated to work on) the exercises to improve range of motion, is important for understanding the need for flexibility in recommending different therapies.
The cost is, or can be, high for helmet treatment; hopefully, the cost for this treatment will reduce as time passes. However, we do not think the condition is as benign from a skull deformity and social/psychological standpoint as assumed in the past.
PEDIATRICS (ISSN 1098-4275). ©2004 by the American Academy of Pediatrics
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