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Diagnosis and Management of Posterior Plagiocephaly

Ian F. Pollack, MD*; H. Wolfgang Losken, MBChB, FCS(SA), FRCS(Ed)‡; and Patricia Fasick, OTR/L§

ABSTRACT. Objective. The management of infants be effectively managed by nonsurgical means. The im-
with posterior plagiocephaly has been controversial both pact of positional preference on the development of this
because of widely differing estimates in the literature of process is discussed. Pediatrics 1997;99:180 –185; children,
the relative frequencies of true lambdoidal synostosis vs deformation, lambdoid, plagiocephaly, synostosis, treat-
positional molding and because of divergent approaches ment.
to treating this problem in different institutions. Based
on our experience, we hypothesized that the vast major-
ity of children with posterior plagiocephaly did not have ABBREVIATION. CT, computed tomography.
true synostosis and that the cosmetic impairment in such
patients could be effectively treated with nonsurgical
modalities.
The management of patients with posterior plagio-
Methods. Between 1992 and 1995, we prospectively cephaly has long presented a challenge both for the
applied in 71 infants a consistent management philoso- primary care physician involved in making the initial
phy for these malformations that has incorporated a de- diagnosis and the craniofacial team involved in pre-
tailed evaluation of sutural anatomy as the basis for a scribing a treatment plan. This deformity may result
physiologic approach to treatment. This approach has from or be exacerbated by a variety of factors, such as
been directed at distinguishing true synostosis from de- a restrictive intrauterine environment, torticollis, a
formational plagiocephaly and at avoiding surgery for strong preference for sleeping position in early in-
patients with deformational abnormalities by using a
fancy, and neurologic impairment.1– 6 The ap-
combination of nonsurgical modalities to restore normal
cranial growth dynamics. All children first underwent proaches that have been used for managing this de-
skull radiographs to determine whether the lambdoidal formity have varied widely in different centers,
sutures were patent. In equivocal cases, computed to- which has complicated efforts to formulate reliable
mography was also performed. Patients without true syn- guidelines for diagnostic evaluation and treatment.
ostosis were enrolled on a course of positional therapy. In particular, the issue of which children require
In patients that did not improve after 2 to 3 months, a operation has often rested as much on institutional
custom-fitted orthoplastic molding helmet was applied bias as on objective factors. For example, in some
to facilitate passive skull recontouring. centers, moderate to severe posterior plagiocephaly
Results. Forty children had patent sutures based on
skull radiographs, and 29 others, in whom the radio- has been treated with limited craniectomy tech-
graphs were equivocal, had open sutures based on com- niques7 or posterior calvarial reconstruction8 –11 per-
puted tomography, thus establishing the diagnosis of formed early in infancy in the hope of optimizing
deformational plagiocephaly in 69. Predisposing factors cranial appearance. However, pathologic studies
for this deformity included a strong positioning prefer- have indicated that the majority of patients who
ence during early infancy (n 5 67), torticollis (n 5 10), undergo operation for this deformity have narrow-
prematurity (n 5 6), and developmental delay (n 5 2). ing rather than true synostosis of the affected lamb-
Only two patients had true lambdoidal synostosis; in doidal suture. This observation has encouraged sev-
each case, this was associated with synostosis of the
eral groups to attempt to treat the deformity using
posterior sagittal suture and was managed effectively
with cranial reconstructive surgery. Thirty-five patients nonsurgical means.1,4 – 6,12,13
with deformational plagiocephaly had a dramatic im- Advances in imaging technology coupled with re-
provement in their cranial contour with positional ther- finements in surgical as well as nonsurgical modali-
apy alone; 34 patients failed to improve and were treated ties have facilitated efforts at our institution to stan-
with molding helmets. All but five children, each of dardize the management of young children with
whom was more than 6 months old at initial intervention synostosis and posterior plagiocephaly. We prospec-
(P < .025), developed a normal or nearly normal head tively applied a consistent management philosophy
shape with these measures. in a series of 71 children who were treated between
Conclusion. The vast majority of children with pos-
terior plagiocephaly do not have true synostosis and can
1992 and 1995. The overall goal of this approach was
to avoid subjecting the child to operation for defor-
mational (nonsynostotic) deformities and, instead, to
From the *Departments of Neurosurgery, ‡Plastic Surgery, and §Occupa-
tional Therapy, Children’s Hospital of Pittsburgh, Cleft Palate-Craniofacial
rely heavily on nonsurgical means to restore normal
Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsyl- cranial growth dynamics. Patients were initially
vania. treated with physical therapy and positional therapy
Received for publication Jan 11, 1996; accepted Mar 18, 1996. to encourage normal neck mobility and to discourage
Reprint requests to (I.F.P.) Department of Neurosurgery, Children’s Hos-
pital of Pittsburgh, 3705 Fifth Ave., Pittsburgh, PA 15213.
continued dependency on the flattened area. Patients
PEDIATRICS (ISSN 0031 4005). Copyright © 1997 by the American Acad- who failed to improve with these approaches were
emy of Pediatrics. then treated with a molding orthoplastic helmet to

180 PEDIATRICS Vol. 99 No. 2 February 1997


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encourage the growing skull to adopt a more normal
contour. The rationale for using such an approach is
based on several factors. First, because 85% of post-
natal skull growth occurs during the first year of life,
the helmet, if fitted appropriately, can harness this
property to gradually mold the developing skull into
a more normal configuration. In many ways, this
technique resembles the practice of cranial recon-
touring that was used in a variety of cultures to
achieve a distinctive cranial shape.4,13–17 The power of
such an approach to gradually remodel the calvarial
appearance is illustrated strikingly in anthropologic
and paleontologic studies. Second, the use of such a
conservative strategy does not preclude the perfor-
mance of surgery later in infancy or early childhood,
but if successful clearly diminishes the potential
morbidity involved in treating this problem. The re-
sults of this study validate the efficacy of this ap-
proach and provide insights into possible ways of
minimizing the incidence of posterior plagiocephaly.

METHODS
Diagnostic Evaluation
From July 1992 to March 1995, 71 children younger than 1 year
were referred to our institution for evaluation and treatment of
posterior plagiocephaly, presumed to be secondary to lambdoidal
synostosis. Thirty-seven were younger than 6 months at initial
referral; 34 were between 6 months and 1 year. All children
underwent plain radiographs of the skull to define the sutural
anatomy. In patients with sutures that all were clearly patent, no
further evaluation was performed. However, in those children
with sutures that were visualized poorly or apparently closed,
computed tomography (CT) scans of the brain, calvarial vault, and
cranial base, supplemented by three-dimensional reconstructions,
were routinely performed. For children with plagiocephaly in the
absence of synostosis (deformational plagiocephaly), a careful as-
sessment was made of the following factors: 1) the timing at which
the deformity was detected (eg, at birth or postnatally) and at
which therapy was initiated; 2) the association of various predis-
posing factors, such as prematurity, torticollis, and developmental
delay; and 3) the relation between positioning preferences of the
parent and/or infant and the cranial deformity.

Treatment
Our overall management philosophy was based on the premise
that children with deformational molding (eg, those with signifi-
cant skull deformity but without synostosis), if detected early in
infancy, should generally be managed without surgery. Thus,
children with posterior plagiocephaly in the absence of true syn-
ostosis were initially managed with positional therapy. Parents
were instructed to keep the child from lying on the flattened
occipital region by using a combination of sandbags or pillows to
keep the child from rolling onto the deformed side. In patients
with associated torticollis, physical therapy was also initiated to
encourage gradual stretching of the neck musculature and in-
creased range of neck movements. Particular attention was fo-
cused on keeping the child from continuing to lie on the flattened
occipital region. These therapies were continued for a 2- to
3-month period.
For patients in whom the above measures were ineffective in
correcting the cranial deformity, a molding orthoplastic helmet
was fashioned to facilitate skull growth in the areas of flattening Fig 1. Frontal (A) and side (B) views of a molding orthoplastic
(Fig 1, A and B). The cost of the helmet was approximately $400.00. helmet used for passive cranial remodeling. The helmet has been
This was constructed by first making a plaster mold of the pa- fitted with inserts that apply gentle pressure to the areas of
tient’s skull. The mold was used to create a plastic model slightly maximal prominence, but leave the flattened areas open to grow.
larger than the patient’s calvarium, which was custom-fitted with
inserts to provide gentle pressure to the areas of maximal protu-
berance. Areas of skull flattening were maintained largely free of
compression to encourage passive expansion of the calvarium in several times daily for evidence of irritation or abrasions. The
these areas during the course of subsequent cranial growth. The patient was reexamined every 4 to 6 weeks after beginning helmet
helmet was worn for approximately 22 hours per day to provide therapy to determine whether the helmet needed to be refitted or
a consistent stimulus to calvarial growth. The scalp was checked recontoured internally to adjust to the child’s continued skull

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growth. Helmet therapy was discontinued after a symmetrical
calvarial contour had been established.
Surgical intervention was recommended for patients with ob-
jective evidence of lambdoidal suture synostosis or for those with
severe deformational plagiocephaly that did not respond to con-
servative measures. This consisted of resection of the involved
suture and perisutural bone with reconstruction of the surround-
ing calvarium to achieve an appealing cranial contour.

Outcome Assessment
To evaluate the results of our approach and to identify patients
who had unacceptable outcomes, we assessed outcome in two
ways. First, the parents of each of the affected children were
questioned closely regarding their satisfaction with the child’s
appearance, particularly whether they believed the child’s cranio-
facial features were 1) normal or nearly normal, 2) abnormal but
acceptable, or 3) unacceptable and in need of further intervention.
Second, members of the craniofacial team (I.F.P. or H.W.L.) as-
sessed outcome using a similar scale. In cases involving a discrep-
ancy between the opinions of the craniofacial team and the pa-
tient’s family regarding the cosmetic outcome, the least favorable
response was recorded as the final outcome.

RESULTS
In 40 of the 71 patients referred for evaluation,
skull radiographs clearly demonstrated patency of
all the cranial sutures. In 31 patients, the skull radio-
graphs were inconclusive and CT was performed;
this demonstrated patent lambdoidal sutures in 29
children, 4 of whom had sclerosis and thickening of
the bone adjacent to the suture without obvious clo-
sure of the suture. Only two children had radio-
graphic evidence of true lambdoidal synostosis. In
both cases, this was bilateral, although asymmetrical
in extent, and associated with clear-cut posterior sag-
ittal synostosis.
The 69 patients with nonsynostotic (deforma-
tional) plagiocephaly all exhibited a characteristic
craniofacial appearance. The ear on the affected side
was typically displaced away from the site of maxi-
mal occipital flattening and thus was positioned an-
teriorly, inferiorly, and laterally in comparison to the
contralateral side. The contralateral occipital region
was characteristically protuberant, and in most cases,
the ipsilateral frontal and temporal regions were also
prominent and the contralateral frontal region was
somewhat recessed, which gave the head a parallel-
ogram18 shape (Fig 2, A and B). In some cases, the
face was also affected, with prominence ipsilateral to
the side of occipital flattening and canting of the nose
Fig 2. A, Characteristic cranial features of moderate right occip-
toward the contralateral side. ital deformational plagiocephaly as seen from a vertex view. The
In 24 of the 69 infants with deformational plagio- right occiput is flattened and there is compensatory prominence of
cephaly, the calvarial asymmetry was apparent at the left occiput and right frontal and posterior temporal areas. The
birth. Thirty-five children were noted to develop left frontal region is recessed relative to the right side. The right
asymmetrical occipital flattening during the first 2 ear is also displaced anteriorly, inferiorly, and laterally, away from
the flattened area. B, Axial computed tomography image (bone
months of life. In 10 patients, the parents had not windows) in a patient with right occipital plagiocephaly demon-
been aware of the deformity until it was noted by a strates marked right occipital and left frontal flattening with com-
health care provider or other nonfamily member, pensatory bulging of the contralateral occipital and ipsilateral
and thus were not certain of the time of onset of the frontal regions, which produces a parallelogram shape of the
calvarium. The lambdoid sutures are clearly visible (as are the
deformity. In 35 children, the deformity was re- coronal and sagittal sutures anteriorly) and are open.
ported to clearly have worsened between the time it
was noted initially and the time of referral. Thirty-
five children were younger than 6 months at the time developmental delay as a result of prematurity (n 5
of referral and initiation of therapy; 34 were between 6) or Down syndrome (n 5 2). Sixty-seven of the
6 months and 1 year. families reported that the child had predominantly
Ten patients had associated torticollis that had been positioned supine since birth; two children had
been noted shortly after birth. Eight patients had been positioned prone. In each of the children, a

182 TREATMENT OF POSTERIOR PLAGIOCEPHALY


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strong side-to-side positional preference was re-
ported, such that the child was noted to lie virtually
always on the flattened region of the skull (right 5
49; left 5 20).
Thirty-five children were managed strictly with
positional therapy and physical therapy and devel-
oped a normal or nearly normal appearance after 2 to
3 months of treatment, as assessed by both the family
and the surgeon. Thirty-four patients with significant
residual deformity after the aforementioned ap-
proach were placed in a molding orthoplastic hel-
met. All but five achieved a normal or nearly normal
appearance. Each of these five children was older
than 6 months at initial referral and initiation of
therapy (P , .025, Fisher’s exact test). In addition,
two of the five children with significant residual
deformity had Down syndrome. In both cases, the
head shape was improved by the helmet, but was
clearly abnormal as judged by both the family and
the surgeon, and might potentially have benefitted
from operative intervention; however, the surgeon
(and families) did not favor cranial reconstruction
because these children each had a severe associated
cardiac anomaly. Two other patients with subopti-
mal responses had severe torticollis in conjunction
with profound flattening of the left posterolateral
hemicranium. In both patients, the appearance was
improved but not normalized by physical therapy Fig 3. After treatment with a molding orthoplastic helmet, the
patient in Fig 2A has had normalization of her head shape with
and helmet therapy. Finally, one patient with perisu- little if any detectable frontal or occipital asymmetry.
tural sclerosis showed only a modest improvement
with helmet therapy. In each of the above three pa-
tients, the family was satisfied with the degree of the bilateral lambdoid and sagittal craniectomies, the
correction achieved with the helmet, and viewed the occipital bone plate was freely mobile. A series of
ultimate cranial shape as abnormal but acceptable. relaxing cuts was made in the remaining occipital
Accordingly, they did not wish to pursue surgical bone, which allowed in situ remodeling of the pos-
therapy despite the fact that in two children, the terior calvarial vault. Postoperatively, both patients
surgeon believed that the result was suboptimal and have had excellent cosmetic results with no residual
might have been improved by operative interven- deformity and normal cranial growth.
tion.
In the other 29 patients who underwent helmet DISCUSSION
therapy, the cosmetic results with respect to calvarial The generally favorable results achieved in the
symmetry and contour were uniformly normal or aforementioned unselected series of infants with
nearly normal with no cosmetically significant defor- posterior plagiocephaly largely validate the efficacy
mity (Fig 3). The only residual deformity that was of the management schema proposed. This approach
commonly apparent on close inspection was subtle achieved normal or nearly normal results in all but 5
ear position asymmetry, which was visible when of 69 patients with deformational plagiocephaly and
looking down at the top of the patient’s head, but not a gratifying outcome in the 2 patients with true lamb-
apparent on other projections. doidal synostosis.
As noted previously, only two patients in this A particularly important observation of this report
series were found to have radiographic evidence of was that the vast majority of patients with posterior
true lambdoidal synostosis. The cranial appearance plagiocephaly have deformational changes of the cal-
in these children differed from the characteristic fea- varial vault in the absence of underlying synostosis.
tures of children with deformational plagiocephaly The routine use of CT, supplemented if necessary by
in that the skull was somewhat brachycephalic with three-dimensional reconstructions, for cases in
prominent ridging over the lambdoidal sutures. In whom the patency of the lambdoidal suture was in
addition, in contrast to the situation with deforma- question on plain radiographs facilitated a more re-
tional plagiocephaly, the ear position in these two alistic assessment of the true frequency of lambdoi-
patients was displaced toward rather than away dal synostosis in this population of patients. The
from the site of maximal occipital flattening. Because rarity of actual synostosis among patients with pos-
both patients had synostosis of not only the lamb- terior plagiocephaly contrasts with the situation for
doidal but also the sagittal sutures, these children anterior plagiocephaly, in which true coronal synos-
were treated by posterior calvarial reconstruction. tosis is present in a substantial percentage of pa-
First, strip craniectomies were performed that en- tients.19 A review of our recent records indicates that
compassed the involved sutures. After completion of fewer than 20% of children with anterior plagioceph-

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aly have a deformational rather than synostotic basis ously noted by several groups.1,4 – 6,12,13 An important
for their calvarial deformity. The implication of this caveat in the use of these helmets for children with
observation is that the occipital region may be inher- severe plagiocephaly is that the efficacy of the helmet
ently more susceptible to pressure-induced defor- depends heavily on the intrinsic growth capacity of
mity than the fronto-orbital region. the infant skull, which enlarges substantially during
In this context, it is interesting that virtually all of the first year of life and can be recontoured relatively
the children who developed posterior plagiocephaly easily. As the calvarium increases in size and thick-
after birth spent the bulk of their time lying on their ness and the bones of the cranial base become se-
occiput with the head angled onto the flattened side. verely deformed by the chronic effects of positional
Parents consistently reported positioning their chil- molding, the chances for achieving an adequate cor-
dren supine, but in most cases the child tended to rection of the deformity without the need for exten-
rest with the head turned slightly to one side or the sive surgery become small. In this regard, each of the
other. In some children, who exhibited clear-cut pla- five infants in this series with suboptimal results
giocephaly at birth, the directional preference re- after conservative therapy were older than 6 months
sulted from the tendency of the head to roll onto the when intervention was initiated. Moreover, we have
flattened area. A similar situation was observed in noted almost no benefit from either positional ther-
children with torticollis. In general, parents did not apy or helmet therapy in four other patients (not
attempt to counteract this positioning tendency (un- included in this series), who because of delayed re-
til they were specifically instructed to do so), which ferral began treatment after 1 year of age. Thus, the
often exacerbated the initial deformity. In other chil- earlier that intervention is initiated, the more easily
dren who did not exhibit flattening at birth or torti- an acceptable cosmetic result can be achieved. With
collis, the basis for the directional preference was expeditious treatment, the need for surgery can gen-
often idiosyncratic. Many parents reported that they erally be avoided.4 – 6,12,13 In some ways, these nonop-
consistently positioned the baby on one side without erative results parallel the outcome obtained after
realizing that this might lead to gradual deformation surgical correction of sagittal synostosis. In children
of the skull. Once the flattening developed, the child younger than 4 months of age, any one of a number
tended always to lie on the flattened area unless of procedures that remove the offending suture will
measures were taken to prevent this. In view of the allow relatively normal cranial growth and ensure an
recent trend toward supine positioning for infants to acceptable cosmetic result.22,23 In contrast, in older
minimize the risk of sudden infant death syn- children, much more extensive and potentially dan-
drome,20,21 it is apparent that parents must be care- gerous operations are required to achieve a similarly
fully instructed to alternate positioning from one acceptable cosmetic correction.24,25 These results
side to the other to avoid progressive calvarial de- highlight the importance of early diagnosis and
formity. This applies particularly strongly to chil- prompt intervention for the aforementioned defor-
dren with congenital torticollis, in whom the neck mities to ensure a favorable outcome.
position preference can rapidly translate into a se- Although, in the past, early surgery was com-
vere calvarial deformity. monly recommended for children with posterior pla-
Because most children with posterior plagioceph- giocephaly, the rationale for this approach was gen-
aly have open cranial sutures and, in theory, preser- erally based on the mistaken assumption that
vation of relatively normal cranial growth potential, affected children had true synostosis or at least in-
there is a strong rationale to apply nonsurgical cipient closure of the lambdoid suture. Such a phe-
means as an initial step to treat the calvarial defor- nomenon certainly occurs in a small percentage of
mity. Moreover, because of the compensatory con- infants with posterior plagiocephaly,26 but in our
tralateral occipital and ipsilateral frontal growth that experience the frequency of this process was much
occurs with deformational occipital plagiocephaly, less common than predicted from previous studies.
this process does not produce cranial volume restric- Our results demonstrate that the presence of perisu-
tion to inhibit brain growth, and thus the major tural sclerosis does not indicate incipient lambdoid
reason for intervention is to optimize the calvarial synostosis, because three of four patients with this
contour to achieve an acceptable cosmetic result. Our finding had normalization of their cranial shape with
results indicate that the majority of infants with de- positional and/or helmet therapy. In contrast, the
formational plagiocephaly will have a favorable out- two patients with true lambdoid synostosis had un-
come with positional therapy and, if needed, physi- mistakable obliteration and ridging of the lambdoid
cal therapy to combat any underlying torticollis and suture, which was confirmed both by direct inspec-
reestablish a full range of neck mobility. Patients tion intraoperatively and by pathologic examination.
who do not respond to these measures will generally In summary, our results indicate that the vast ma-
show a gratifying response to a course of therapy jority of infants with posterior plagiocephaly have
with a molding orthoplastic helmet. Although five of deformational molding rather than true synostosis,
our patients did have significant residual deformity and that these two entities exhibit distinct pheno-
after a course of helmet therapy, they each nonethe- typic features. Good cosmetic results can be achieved
less showed a noticeable improvement such that the in the vast majority of patients with nonsynostotic
families did not wish to pursue additional interven- plagiocephaly using nonoperative modalities. Oper-
tion. ative intervention is appropriate for the small per-
The value of molding helmets for selected children centage of patients with true synostosis and those
with calvarial vault abnormalities has been previ- with deformational plagiocephaly that fails to re-

184 TREATMENT OF POSTERIOR PLAGIOCEPHALY


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spond to conservative measures. Because the pheno- approach to lambdoid synostosis. Proceedings of the 23rd Annual Meet-
ing of the Joint Section of Pediatric Neurosurgery, St Louis, MO. 1994:
typic characteristics of deformational plagiocephaly
113. Abstract
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OTR/L, Pamela Judy, OTR/L, and other members of the Occupa- 16. Nichter L, Persing J, Horowitz J, Edgerton M. External cranioplasty:
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Diagnosis and Management of Posterior Plagiocephaly
Ian F. Pollack, H. Wolfgang Losken and Patricia Fasick
Pediatrics 1997;99;180
DOI: 10.1542/peds.99.2.180
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly


publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and
trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove
Village, Illinois, 60007. Copyright © 1997 by the American Academy of Pediatrics. All rights
reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

Downloaded from pediatrics.aappublications.org at Florida Atlantic University on July 16, 2015


Diagnosis and Management of Posterior Plagiocephaly
Ian F. Pollack, H. Wolfgang Losken and Patricia Fasick
Pediatrics 1997;99;180
DOI: 10.1542/peds.99.2.180

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pediatrics.aappublications.org/content/99/2/180.full.html

PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly


publication, it has been published continuously since 1948. PEDIATRICS is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 1997 by the American Academy
of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

Downloaded from pediatrics.aappublications.org at Florida Atlantic University on July 16, 2015

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