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Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446

Positional plagiocephaly: evaluation and management


John Caccamese, DMD, MDa, Bernard J. Costello, DMD, MDb,
Ramon L. Ruiz, DMD, MDc,*, Ann M. Ritter, MDd
a
Department of Oral and Maxillofacial Surgery, University of Maryland Medical System, 419 West Redwood Street,
Suite 410, Baltimore, MD 21043, USA
b
Department of Oral and Maxillofacial Surgery, University of Pittsburgh Medical Center, Magee-Women’s Hospital,
Children’s Hospital of Pittsburgh, 3471 Fifth Avenue, Suite 1112, Pittsburgh, PA 15213, USA
c
Oral and Maxillofacial Surgery/Pediatric Craniofacial Surgery, Southwest Florida Oral and Facial Surgery,
5285 Summerlin Road, Suite 101, Fort Meyers, FL 33919, USA
d
Department of Pediatric Neurosurgery, Children’s Hospital of North Carolina, University of North Carolina at Chapel Hill,
148 Burnett-Womack Building, Chapel Hill, NC 27599, USA

Plagiocephaly is a clinically descriptive term for chanical forces (eg, positional plagiocephaly), and (3)
an oblique or asymmetric head shape that does not disruptions, which are morphologic defects of an
imply a definitive diagnosis. Throughout the scien- organ or part of an organ that result from the
tific literature, however, plagiocephaly frequently has breakdown or interference with an originally normal
been used broadly to describe asymmetries of the structure (eg, amniotic band disruptions) [2].
anterior or posterior cranial vault, often without During the first 2 years of life, brain growth oc-
differentiating between synostotic (ie, fused sutures) curs at a rapid rate. In the newborn, the presence of
and nonsynostotic (ie, patent suture) deformities [1]. open cranial vault sutures (eg, metopic, coronal,
Understanding the heterogenous nature of plagio- sagittal, and lambdoid) creates a flexible complex of
cephaly is critical because treatments vary dramati- cranial bones that allows the brain to expand. This
cally depending on the specific underlying diagnosis. flexibility of the cranium, which allows unrestricted
Depending on the specific cause of the cranial vault brain development, also may permit the cranial vault
deformity, the required management may range from to be deformed. ‘‘Positional plagiocephaly’’ is
observation to more extensive intervention, including defined as a cranial vault asymmetry in the presence
major surgery for reconstruction of the cranial vault. of patent cranial vault sutures. This type of skull
Congenital anomalies may be classified into sev- molding or deformational plagiocephaly is commonly
eral basic categories: (1) malformations, which result seen in neonates and infants, and previous intra-
during embryogenesis (eg, craniosynostosis) and are uterine constraint, the birthing process, or repetitive
morphologic defects of an organ, part of an organ, or a sleep positioning all can contribute to the severity of
larger area of the body that result from an intrinsically the cranial vault asymmetry. In contrast, craniosynos-
abnormal developmental process, (2) deformations, tosis is a malformation that occurs almost universally
which occur later in fetal life or postnatally and are during fetal development and is defined as the pre-
alterations in the form or position of a previously nor- mature fusion of a cranial vault suture or sutures that
mally formed structure secondary to nondisruptive me- results in arrested skeletal growth and a head shape
abnormality. The different forms of craniosynostosis
each produce unique craniofacial dysmorphologies.
* Corresponding author. Clinicians must be able to differentiate between
E-mail address: pedmaxillofacial@aol.com (R.L. Ruiz). the dysmorphic craniofacial patterns produced by

1042-3699/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved.
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440 J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446

these different causes. This article presents the current ence may be associated with torticollis, ocular con-
diagnostic approach, differential diagnosis, and treat- ditions, scoliosis of infancy, limited abduction of the
ment options for positional plagiocephaly. contralateral hip, and foot abnormalities [8].
In a recent study performed in the Netherlands that
involved a large cohort of patients, the prevalence of
Incidence and etiology of positional plagiocephaly positional preference was noted to be 8.2% and was
seen most commonly in children younger than 16 weeks
During the first few days of life, cranial molding of age. First-born children, premature children, and
that resulted from the passage of the child’s head breech positioned infants were at the highest risk for
through the birth canal or early decent into the positional preference. Supine sleeping positioning
mother’s pelvis is frequently noted. This asymmetry and repetitive positioning during feeding were posi-
usually resolves spontaneously within the first sev- tively correlated with positional preference [8].
eral weeks of life in most infants. No treatment is Another potential contributing factor to positional
required other than routine observation during well- plagiocephaly is congenital muscular torticollis
baby check-ups. (CMT). CMT is an abnormal head tilt that results
Another group of children demonstrates normo- from congenital tethering of one of the sternocleido-
cephalic morphology after birth but subsequently mastoid muscles. The result of this unilateral muscu-
develops cranial vault asymmetry during early post- lar problem is a persistent aberrant head position that
natal development. This positional plagiocephaly is causes skull molding and asymmetry, which affect
the result of postnatal external forces, usually from the anterior or posterior cranial vault. One theory
repetitive sleep positioning, that act on a flexible regarding the cause of CMT has proposed that late
cranium. Previous reports have estimated the inci- intrauterine constraint leads to venous occlusion and
dence of positional plagiocephaly as low as 1:300 and damage of the sternocleidomastoid muscle [9,10].
as high as 1:2 infants under the age of 1 year [3,4]. In This insult generally results in unilateral fibrosis and
contrast, the incidence of lambdoid suture cranio- shortening of the sternocleidomastoid muscle. Other
synostosis, which also produces posterior plagio- suggested causes include intrinsic malformations or
cephaly, is approximately 1:150,000 live births [2]. ‘‘pseudo-tumors’’ of the sternocleidomastoid muscu-
Unilateral coronal craniosynostosis is another rele- lature, neurologic disorders, and hemiatrophy/hyper-
vant condition because it produces anterior cranial trophy [9,10]. Whatever the cause, the fetal head is
vault asymmetry that may be confused with deforma- molded while obliquely compressed by the muscle
tional changes. It has a relative incidence of approxi- tethering. Consequently, contralateral deviation of the
mately 1:10,000 live births [5]. chin, lateral neck flexion, and decreased range of
In 1992, the American Academy of Pediatrics motion are frequently noted on examination. In
started the ‘‘Back to Sleep’’ campaign, which infants in whom CMT is severe, the cranial asym-
recommended that infants be placed on their sides metry becomes more pronounced in the first few
or backs during sleep to reduce the risk of sudden months of life as the infant’s head is maintained in a
infant death syndrome (SIDS) [6]. Since instituting relatively fixed position.
the campaign in 1992, the incidence of SIDS has On physical examination, head tilt, limited neck
decreased by 40%. At the same time, however, a range of motion, and contralateral facial flattening
significant increase in the number of children referred should be noted. Persing et al [4] recommend the
for management of posterior plagiocephaly without rotating chair test to aid in making the diagnosis of
synostosis was observed [7]. Early on, several centers CMT associated with positional plagiocephaly. To
reported a sudden increase in the number of unilateral perform this test, the examiner holds the infant while
lambdoid ‘‘synostosis’’ cases being seen. Later, it was the parent keeps the infant’s interest. The examiner
realized that the increased number of referrals was rotates in the chair and observes the infant’s range of
actually repetitive sleep positioning that caused non- neck motion. The difference in neck mobility toward
synostotic posterior plagiocephaly [1,7]. and away from the affected side may aid in making
Positional preference also has been recognized as the diagnosis of CMT [1].
a significant contributor to cranial asymmetry since Slate et al [11] noted asymptomatic cervical spine
the early 1990s. Positional preference merely refers to subluxations in half of their study population with
an infant or child’s preference in maintaining the head CMT. This finding coincided with a 62% rate of
turned to one side most of the time. Active move- difficult or breech deliveries. Whether these findings
ments of the head to the opposite side are few, and were the result of birth trauma or unfavorable
passive mobility may be limited. Positional prefer- intrauterine positioning was not clear.
J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446 441

Other researchers have suggested a less severe during the first 3 to 6 months of life. By contrast,
variant of CMT called sternocleidomastoid imbal- patients with craniosynostosis-related plagiocephaly
ance, in which the position of the neck is not fixed have a history of cranial deformity noticed immedi-
but only intermittently tilted with a normal range of ately or shortly after birth. This significant difference
motion attributable to a weakened sternocleidomas- is consistent with the fact that craniosynostosis is an
toid. This condition may be more common than intrauterine event, with the deformity starting during
CMT and more amenable to physical therapy [12]. fetal development, and plagiocephaly secondary to
If left untreated, however, the resultant head defor- repetitive positioning occurs only after external forces
mity remains the same. When positional plagio- have had ample time to mold the cranium during
cephaly is associated with CMT, it is imperative early postnatal growth.
that it be recognized and addressed early. Cervical Infants with positional posterior plagiocephaly
spine anomalies and extraocular muscle disorders frequently present with a characteristic, recognizable
must be ruled out, and appropriate physical therapy cranial vault morphology that is highly suggestive, if
is undertaken at an early age. not diagnostic, for the condition. Examiners who are
familiar with the specific asymmetry typically en-
countered in positional plagiocephaly are able to dif-
Diagnosis ferentiate it from the dysmorphic pattern associated
with lambdoid suture craniosynostosis (Figs. 1 and 2).
Routine surveillance of an infant’s head shape On clinical examination, patients with benign posi-
should be incorporated into periodic well-child visits tional skull molding (ie, positional plagiocephaly)
during the first year of life [1]. The presence of a exhibit occipitoparietal flattening on the side of the
cranial vault asymmetry during the early postnatal posterior cranial vault affected by external forces
period should alert the examiner to the possibility of (eg, repetitive sleep positioning). At the same time,
craniosynostosis. Early referral to a craniofacial or ipsilateral frontal bossing and anterior displacement
neurosurgical specialist is indicated when there is a of the ear on the affected side are found. The mor-
persistent cranial asymmetry or a skeletal pattern phologic pattern is consistent with a flexible cranial
consistent with synostosis. Understanding the differ-
ences between craniosynostosis and positional pla-
giocephaly is important, because craniosynostosis
usually requires surgical reshaping of the cranial
vault to increase intracranial volume and correct the
dysmorphic shape. Conversely, positional plagio-
cephaly is a disorder that does not require surgery
in most cases. A diagnostic dilemma for pediatricians
frequently occurs because the two conditions may
seem to have similar morphologic features. As a
result, an important initial step in the management
of abnormal head shape is the determination of an
accurate diagnosis separating synostotic from non-
synostotic plagiocephaly.
Diagnosis of positional plagiocephaly primarily is
based on a thorough history and physical examination
and is confirmed radiographically. History taking
should include details of the parental medical history
and family history, the antenatal and birth histories of
the child, giving consideration to any exposures or
complications of the pregnancy, and a current medical Fig. 1. Illustration of cranial vault dysmorphology character-
history for the infant. A detailed history often istic of posterior positional plagiocephaly. Postnatal external
forces, such as repetitive sleep positioning, cause unilateral
provides information that strongly suggests the
occipitoparietal flattening. Because the sutures are open and
diagnosis even before the actual clinical evaluation. the cranial vault complex is flexible, all of the structures on
For example, when an infant presents with positional the affected side are displaced anteriorly. Additional findings
plagiocephaly, parents frequently report that the include ipsilateral frontal bossing and forward displacement
child’s head shape was well rounded at the time of of the ear. The outline of the cranial vault, as viewed from
birth and the asymmetry subsequently developed above, develops a form that resembles a parallelogram.
442 J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446

Fig. 2. Bird’s-eye view of a child with benign positional


skull molding (ie, positional plagiocephaly) secondary to Fig. 3. Illustration of cranial vault dysmorphology character-
repetitive sleep positioning. Three key findings are present istic of posterior plagiocephaly secondary to unilateral
on clinical examination: unilateral occipitoparietal flatten- lambdoid suture craniosynostosis. In this example, arrested
ing, ipsilateral frontal bossing, and ipsilateral forward development across the right lambdoid suture causes flat-
displacement of the ear. tening of the occipital and parietal bones. The lack of cranial
growth on the affected side also produces retraction of the
vault that allows forward movement of all structures forehead on the right (ipsilateral) side, and the right ear is
on the side subjected to external deformational forces. displaced posteriorly and inferiorly when compared with the
The head shape associated with positional plagio- left. Compensatory overgrowth also occurs at the sutures
that remain open, which causes contralateral frontal bossing.
cephaly has been described as having a parallelo-
From a bird’s-eye view, the cranial vault may take on the
gram deformity when examined from above (bird’s-
shape of a trapezoid.
eye view) [13]. By comparison, the cranial vault de-
formity seen with lambdoid suture craniosynostosis is
characterized by distinctly different features (Fig. 3,
ture areas to establish that the cranial vault sutures are
Table 1). When a lambdoid suture is absent, there is
patent. In cases in which plain radiographic images
arrested skeletal growth across the affected occipital
do not permit conclusive evaluation of the sutures,
and parietal bones, which also results in significant
the use of a complete craniofacial CT scan is indicated
flattening or posterior plagiocephaly, but that is where
(Fig. 5). CT evaluation allows a more detailed view of
the similarities to positional plagiocephaly end. In
the suture regions and provides additional informa-
lambdoid synostosis, the arrested growth causes
tion about the three-dimensional skeletal morphology.
retraction of the ipsilateral forehead. The cranial base
Such detailed studies are used more commonly for
involvement seen in synostosis also causes posterior-
surgical planning in patients with surgical problems
inferior displacement of the ipsilateral ear. Because
(ie, synostosis) but may also be of value in cases of
of compensatory growth that occurs at the sutures
that remain open, contralateral frontal bossing is seen.
From bird’s-eye view, the deformity is often de-
scribed as having a trapezoidal shape (Fig. 3).
Although the diagnosis of positional plagio- Table 1
cephaly primarily is made on the clinical evaluation Nonsynostotic versus synostotic plagiocephaly
of the presenting dysmorphology, confirmatory radio- Positional plagiocephaly Lambdoid synostosis
graphic studies are required. After careful clinical Occipitoparietal flattening Occipitoparietal flattening
examination is performed, a complete skull series of Ipsilateral frontal bossing Ipsilateral frontal flattening
plain radiographs is usually sufficient to establish Forward displacement of Posterior displacement of
definitively the diagnosis and exclude the possibility the ipsilateral ear the ipsilateral ear
of craniosynostosis (Fig. 4). A diagnostic, high- No bony ridging Bony ridging along involved
quality study permits adequate visualization of all su- suture region
J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446 443

Fig. 4. (A – D) A complete skull series of plain radiographs is frequently adequate imaging for confirmation of the diagnosis of
positional plagiocephaly. Diagnostic films provide a clear view of all open major cranial vault suture regions.

suspected positional plagiocephaly, in which the plain or ‘‘impending synostosis,’’ to describe cranial vault
film study results are equivocal. sutures that otherwise appear patent on a radiograph
In addition to confirming the status of the cranial or CT scan. The idea is that the suture appears
vault sutures, radiographic imaging studies also radiographically normal but somehow will demon-
provide insights regarding the morphology of the strate abnormal physiology. We know that the
cranial base. Lo et al [14] detailed changes in the premature fusion of cranial vault sutures (ie, cranio-
endocranial base seen in unilateral coronal and uni- synostosis) is an intrauterine event that is present at
lateral lambdoid synostosis and compared them to birth when it occurs. Unfortunately, some centers still
those of skull molding. They made the observation use this type of inappropriate terminology, which has
that the endocranial base of infants with craniosyn- an impact on the clinical decision making related to
ostosis was likely to deviate from the 0°- to 180°- patients with abnormal head shape. The result may be
anteroposterior axis by more than 7° toward the confusion among health care providers and even
affected suture, whereas this was not the case in po- surgical intervention for a patient with a nonsurgical
sitional plagiocephaly. problem (ie, positional plagiocephaly). As recently as
Although current radiographic imaging techniques the 1990s, posterior positional plagiocephaly was
have become increasingly refined, allowing for misdiagnosed frequently and treated inappropriately
detailed visualization of the cranial anatomy (includ- as unilateral lambdoid synostosis. This was realized
ing the sutures), wide variation remains regarding the at many centers with increasing numbers of lambdoid
radiographic descriptions used for positional plagio- synostosis cases being treated and what some sur-
cephaly and craniosynostosis. At some medical geons termed ‘‘functional lambdoid synostosis’’ when
centers, radiologists continue to use outdated and the histopathologic diagnosis did not support true
confusing terminology, such as ‘‘fibrous synostosis’’ synostosis [11,13]. From a chronologic standpoint,
444 J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446

Fig. 5. (A – D) Complete craniofacial CT scan with three-dimensional reconstructions for evaluation of a cranial vault asymmetry.
The study reveals that all of the cranial vault sutures are open, which rules out craniosynostosis. CT imaging provides the
most detailed view of the sutures and quantitative data about the cranial vault morphology.

this activity coincided with the initiation of the there are no associated negative functional neurologic
American Academy of Pediatrics’ ‘‘Back to Sleep’’ consequences, so operative intervention is not indi-
campaign. We know that the cranial asymmetry of cated. Despite the lack of neurologic insult, there
unilateral lambdoid synostosis is distinct from that of may be a significant skeletal deformity that must
posterior positional plagiocephaly; perhaps more im- be addressed.
portantly, we recognize positional plagiocephaly as a When the degree of skeletal asymmetry is mild
nonsurgical problem in most patients [1,6,7,13 – 19]. and is detected early in life, providing parental in-
struction about alternating head movements and
avoiding repetitive positioning is usually the most
Treatment appropriate initial measure. Parents are instructed to
monitor and encourage frequent changes in their
The treatment of positional plagiocephaly is children’s head position throughout the course of the
generally nonsurgical. If craniosynostosis is con- day and avoid repetitive sleep positioning. Reposi-
firmed, then reconstruction of the cranial vault to tioning of a baby’s head while in carriers and car seats
increase intracranial volume and establish a more is an important component of this regimen. Parents
normal morphology is completed using a team ap- are also taught to lay infants down to sleep in the
proach with a pediatric neurosurgeon and pediatric supine position and alternate the right and left
craniofacial surgeon. Positional plagiocephaly does occipital regions. When awake, children should be
not result in restriction of brain growth. As a result, fed from the right and left sides to discourage the
J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446 445

development of a voluntary position preference. maximal improvement in cranial shape. Progress is


Supervised ‘‘tummy time’’ should be encouraged followed weekly by an orthotist or physical therapist,
once infants are capable of lifting their head and have at which time skull growth and incremental correc-
acceptable arm strength. Generally, these guidelines tion of the deformity are evaluated. The inner lining
should be followed until the physician is confident is modified accordingly or, in situations that involve
that the deformity is improving or until infants are rapid growth or correction, a new band is fabricated.
sitting and crawling consistently and spending less The greatest benefit from the use of custom-made
time on their back. cranial orthotic devices is achieved when they are
Infants who present with positional skull molding used before 1 year of life and ideally when they are
and moderate to severe plagiocephalic skull defor- initiated before 6 months of age [22]. This benefit
mity benefit from treatment with a custom-made cra- arises because of the propulsive rate of brain growth
nial orthotic device (Fig. 6). Orthotics are of two basic during this period, which drives outward expansion
varieties: (1) custom-fitted molding helmets that and remodeling of the cranial vault. Usually, a cranial
facilitate passive cranial reshaping and (2) dynamic orthotic is used for a 4-month period before the child
or ‘‘active’’ orthotic cranioplasty devices that use outgrows it and the band loses its ability to effect
the growth potential of the brain and skull and mild cranial morphology. Because the speed of brain
constrictive forces to mold the cranial skeleton (eg, expansion slows down after 1 year of life, treatment
DOC Band, Cranial Technologies, Tempe, Arizona; performed between 12 and 18 months of age usually
STAR Band, Orthomerica, Newport Beach, Califor- is associated with diminished reshaping. By the time
nia). Clarren et al [9] first reported the use of custom a child is 18 to 24 months old, the benefit of cranial
helmet therapy for infants with CMT and positional orthotic treatment is limited, and after 24 months,
plagiocephaly. Others have since described the use of minimal to no changes are likely.
‘‘dynamic’’ and passive devices with similar efficacy Cranial orthotic devices produce the most signifi-
[15,19 – 21]. The idea is to apply direct pressure or cant improvements in the position of the cranial vault
limit further growth and deformity in the areas of segments to round out the shape of the head. Changes
frontal and occipital prominence, while the helmet is in auricular position are more limited, and even
relieved over adjacent flattened areas to allow for patients who achieve excellent correction of the cra-
ongoing brain growth to drive cranial vault expansion nial shape often demonstrate persistent asymmetry in
and remodeling. Physicians recommend that the their ear alignment and position. The same is true
orthotic be worn for 22 to 23 hours a day to obtain when an underlying skull base asymmetry is noted.
Successful management of positional plagio-
cephaly requires that any contributing factors be
addressed at the same time that the cranial asymmetry
is treated. In patients with CMT, simultaneous
physical therapy and parent-directed exercises are
prescribed. If a patient demonstrates an abnormal
head tilt that is related to extraocular muscle im-
balance or cervical spine anomalies, then pediatric
ophthalmologic consultation and pediatric neurosur-
gical evaluation are undertaken, respectively.
Surgical treatment of infants with positional
plagiocephaly is not undertaken. In rare instances, a
child with an untreated skull deformity demonstrates
severe residual asymmetry after cranial growth is
finished. Fortunately, this is a rare occurrence and
usually is associated with a previous history that
involves repetitive positioning for prolonged periods
of time. For example, a child with a complicated
medical situation and history of prolonged bed rest
during early life may have developed a severe skull
asymmetry that went untreated. Although these
Fig. 6. A child with positional plagiocephaly undergoing residual deformities do not affect brain function,
nonsurgical treatment with the use of a custom-made cranial they may be severe and cause significant negative
orthotic device. psychosocial consequences. Surgical intervention is
446 J. Caccamese et al / Oral Maxillofacial Surg Clin N Am 16 (2004) 439 – 446

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