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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.
doi:10.1016/j.coms.2004.08.006 oralmaxsurgery.theclinics.com
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. 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
undertaken with a combined pediatric neurosurgical [6] AAP Taskforce. Positioning and SIDS. Pediatrics
and pediatric craniofacial approach similar to that 1992;89:1120 – 6.
used in the management of craniosynostosis. [7] Posnick JC. Posterior plagiocephaly: unilateral lamb-
doid synostosis and skull molding. In: Posnick JC,
editor. Craniofacial and maxillofacial surgery in
children and young adults. Philadelphia7 WB Saun-
Summary ders; 2000. p. 231 – 48.
[8] Boere-Boonekamp MM, van der Linden-Kuiper C.
Positional preference/prevalence in infants and follow-
Plagiocephaly is the result of many causes.
up after two years. Pediatrics 2001;107:339 – 43.
Clinicians must be well versed in the differential [9] Clarren SK, Smith DW, Hanson JW. Helmet treatment
diagnosis of plagiocephaly to determine if treatment for plagiocephaly and congenital muscular torticollis.
should be surgical or nonsurgical. Craniosynostosis J Pediatr 1979;94:43 – 6.
is a fetal malformation and should be diagnosed [10] Clarren SK. Plagiocephaly and torticollis: etiology,
and treated relatively early in a child’s life with natural history, and helmet treatment. J Pediatr 1981;
cranio-orbital decompression and reshaping. Posi- 98:92 – 5.
tional plagiocephaly is a nonsynostotic condition in [11] Slate RK, Posnick JC, Armstrong DC, et al. Cervical
which there is a deformation of the cranial vault in spine subluxation associated with congenital muscular
torticollis and craniofacial anomaly. Plast Reconstr
the presence of otherwise open, normally function-
Surg 1993;91:7.
ing sutures. The reported incidence of positional
[12] Golden KA, Beals SP, Littlefield TR, Pomatto JK.
plagiocephaly has increased with the implemen- Sternocleidomastoid imbalance versus congenital mus-
tation of ‘‘Back to Sleep’’ guidelines for the pre- cular torticollis: their relationship to positional plagio-
vention of SIDS. Pediatricians also have maintained cephaly. Cleft Palate Craniofac J 1999;36:256 – 61.
an increased awareness of the importance of early [13] Huang MHS, Gruss JS, Clarren SK, Mouradain WE,
referral for evaluation of abnormal head shape during Cunningham ML, Roberts TS, et al. The differential
infancy. Positional plagiocephaly is a cranial vault diagnosis of posterior plagiocephaly: true lambdoid
deformation in the presence of open cranial vault su- synostosis versus positional molding. Plast Reconstr
tures with no negative neurologic consequences. A Surg 1996;98:765 – 76.
[14] Lo L, Marsh JL, Pilgram TK, Vannier MW. Plagioce-
distinct morphologic pattern and patent cranial su-
phaly: differential diagnosis based on endocranial
tures noted on confirmatory radiographic studies dis-
morphology. Plast Reconstr Surg 1996;97:282 – 91.
tinguish positional plagiocephaly from abnormal head [15] Pollack IF, Fasick P. Diagnosis and management of
shapes secondary to craniosynostosis. Management posterior plagiocephaly. Pediatrics 1997;99:180 – 5.
consists of conservative (nonsurgical) means, depend- [16] Hansen M, Mulliken JB. Frontal plagiocephaly. Clin
ing on the extent of the cranial asymmetry and any Plast Surg 1994;21:543 – 53.
contributing etiologic factors. [17] Bruneteau RJ, Mulliken JB. Frontal plagiocephaly:
synostotic, compensational, or deformational. Plast
Reconstr Surg 1992;89:21 – 31.
[18] Argenta LC, David LR, Wilson JA, Bell WO. An
References increase in infant cranial deformity with supine
sleeping position. J Craniofac Surg 1996;7:5 – 11.
[1] Kane AA, Mitchell LE, Craven KP, Marsh JL. [19] Turk AE, McCarthy JG, Thorne CHM, Wisoff JH. The
Observations on a recent increase in plagiocephaly ‘‘back to sleep campaign’’ and deformational plagio-
without synostosis. Pediatrics 1996;97:877 – 85. cephaly: is there cause for concern? J Craniofac Surg
[2] Cohen Jr MM. The etiology of craniosynostosis. In: 1996;7:12 – 8.
Cohen Jr MM, editor. Craniosynostosis: diagnosis, [20] Loveday BPT, de Chalain TB. Active counterposition-
evaluation, and management. New York7 Raven Press; ing or orthotic device to treat positional plagiocephaly?
1986. p. 65 – 6. J Craniofac Surg 2001;12:308 – 13.
[3] Dunn PM. Congenital postural deformities. Br Med [21] Vles JSH, Colla C, Weber JW, Beuls E, Wilmink J,
Bull 1976;32:71 – 6. Kingma H. Helmet versus nonhelmet treatment in
[4] Persing J, James H, Swanson J, Kattwinkel J. nonsynostotic positional plagiocephaly. J Craniofac
Prevention and management of positional skull defor- Surg 2000;11:572 – 4.
mities in infants. Pediatrics 2003;112:199 – 202. [22] Kelly KM, Littlefield TR, Pomatto JK, Ripley CE,
[5] Cohen Jr MM. Craniostenosis and syndromes with Beals SP, Joganic EF. Importance of early recognition
craniosynostosis: Incidence, genetics, penetrance, vari- and treatment of deformational plagiocephaly with
ability, and new syndrome updating. Birth Defects orthotic cranioplasty. Cleft Palate Craniofac J 1999;36:
1979;15:13 – 63. 127 – 30.