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T
he term plagiocephaly describes an asymmetric cranium. DIAGNOSIS
The word is derived from the Greek words Bplagios[ The key to the diagnosis of deformational plagiocephaly is
meaning slanted and Bkephale[ meaning head. A multitude to distinguish it from synostotic plagiocephaly because the 2 con-
of etiologic factors can result in plagiocephaly, including ditions are treated differently. Differentiating deformational and
premature cranial suture fusion and in utero or postnatal mechanical synostotic plagiocephaly can sometimes be challenging, but begins
deforming forces.1 The term deformational plagiocephaly specifi- with physical examination and an understanding of the natural his-
cally refers to a cranial asymmetry due to external compression. tory of the condition.10 Computed tomography scan can be used
These deformational changes are thought to originate in utero and to selectively to make the diagnosis in complex cases. In addition,
be exacerbated by sleeping in a supine position. Torticollis is a ultrasound is a relatively inexpensive and safe tool for distinguishing
common associated finding, and it is hypothesized to exacerbate deformational and synostotic plagiocephaly.11
posterior deformational plagiocephaly.2 Other factors associated Facial evaluation of a child with unilateral posterior defor-
with deformational plagiocephaly include multiple births, small mational plagiocephaly reveals an ipsilateral vertically elongated
maternal pelvis, breech position, oligohydramnios, male sex of the palpebral fissure and an inferiorly positioned ipsilateral ear (Fig. 1).
fetus, gestational diabetes, nulliparity of the mother, high birth Unlike in synostotic plagiocephaly, there is no deviation of the nasal
weight, large neonatal head size, vaginal delivery, prolonged length root. When evaluating from the superior (ie, vertex) view, the child’s
of postdelivery hospital stay (94 days), and prolonged duration of head has a parallelogram shape, with unilateral occipital flatten-
stage II labor.3 Collectively, these factors suggest that a larger fetal ing, contralateral occipital bossing, anterior displacement of the ip-
head (eg, male sex or gestational diabetes) in a smaller maternal silateral ear, and ipsilateral frontal bossing (Fig. 2).12,13 In contrast,
uterus (eg, oligohydramnios or nulliparity) tends to limit fetal move- positional brachycephaly is commonly associated with a normal
ment, increasing the likelihood that the fetus’s skull will be exposed facial appearance and bilateral occipital flattening with increased
to deforming forces. Interestingly, posterior deformational plagio- vertical cranial height.9
cephaly tends to affect the right occiput more commonly than the Transcranial anthropometry can be measured with calipers
left occiput, highlighting the importance of late gestational fetal po- to assess vault asymmetry (Fig. 3).14,15 To accomplish this, a sliding
sitioning in the uterus (ie, neonates are most commonly delivered left caliper is used to measure 2 oblique transverse cranial diameters,
occiput anterior). from the midpoint of the supraorbital rim to the midpoint of the
The incidence of deformational plagiocephaly increased, fol- contralateral parieto-occipital scalp. The difference between the
lowing the recommendations by the American Academy of Pediat- lengths of these 2 diagonals is calculated and recorded; a larger
rics in 1992 to place infants in a supine sleep position.4 Before the value indicates a greater degree of asymmetry. Calipers can also be
American Academy of Pediatrics recommendations, the incidence used to measure cephalic index for patients with positional brachy-
of posterior deformational plagiocephaly was estimated at 0.3%. cephaly. These numbers can be followed as an objective measure of
Today, the incidence has been reported to be as high as 48%, de- the results of treatment, which will in turn influence the decision to
pending on the diagnostic criteria.5Y8 Although the Back to Sleep implement additional therapies.
Campaign has been successful in reducing the rate of sudden infant
death, supine positioning promotes a persistent deforming force on
the malleable neonatal skull. If the infant preferentially lays on one TREATMENT
side, the weight of the head exacerbates a unilateral occipital flatten- Growth of the brain tends to restore symmetry of the cra-
ing and anterior displacement of the ipsilateral craniofacial skeleton niofacial skeleton when the compressive forces are eliminated.
(ie, unilateral posterior deformational plagiocephaly). In cases where Recognizing that supine positioning contributes to deformational
the infant lies evenly on the occiput, this results in bilateral efface- plagiocephaly has prompted primary care providers to educate
ment of the occipital bones and a compensatory increase in the verti- parents about the importance of supervised prone positioning and
cal height of the skull known as bilateral posterior deformational alternating head position from left to right during supine sleep.16
plagiocephaly or positional brachycephaly.9 Correct diagnosis and In cases where muscular torticollis is diagnosed, physical therapy
treatment are essential. to stretch the sternocleidomastoid and trapezius muscles is pre-
scribed. Infants are monitored on a monthly basis for signs of im-
provement. Those children who present early with mild or moderate
From the Institute of Reconstructive Plastic Surgery, New York University deformity and signs of improvement can be treated with positional
Medical Center, New York, New York. therapy. For example, when a child presents at 3 months of age with
Received November 2, 2010. a transcranial difference of 12 mm, we will often recommend posi-
Accepted for publication November 17, 2010. tional therapy and reevaluate the child in 6 to 8 weeks. At this age,
Address correspondence and reprint requests to Stephen M. Warren, MD,
the child’s head circumference will typically increase in size by 1 to
New York University, Institute of Reconstructive Plastic Surgery, 550
First Ave, New York, NY 10016; E-mail: stephen.warren.md@gmail.com 2 cm between visits, and if the occipital pressure is relieved, then
The authors report no conflicts of interest. the transcranial difference will typically decrease by 1 to 2 mm be-
Copyright * 2011 by Mutaz B. Habal, MD tween visits. Infants who present with severe deformity (transcra-
ISSN: 1049-2275 nial difference 920 mm), signs of significant anterior craniofacial
DOI: 10.1097/SCS.0b013e3182074e04 deformities, and developmental delay and those infants who do not
6 The Journal of Craniofacial Surgery & Volume 22, Number 1, January 2011
Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery & Volume 22, Number 1, January 2011 Special Editorial
Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Special Editorial The Journal of Craniofacial Surgery & Volume 22, Number 1, January 2011
10. Cunningham ML, Heike CL. Evaluation of the infant with an 16. Persing J, James H, Swanson J, et al. Prevention and management of
abnormal skull shape. Curr Opin Pediatr 2007;19:645Y651 positional skull deformities in infants. American Academy of Pediatrics
11. Regelsberger J, Delling G, Tsokos M, et al. High-frequency ultrasound Committee on Practice and Ambulatory Medicine, Section on Plastic
confirmation of positional plagiocephaly. J Neurosurg 2006;105: Surgery and Section on Neurological Surgery. Pediatrics 2003;112:
413Y417 199Y202
12. Hutchison BL, Thompson JM, Mitchell EA. Determinants of 17. Losee JE, Mason AC. Deformational plagiocephaly: diagnosis,
nonsynostotic plagiocephaly: a case-control study. Pediatrics 2003; prevention, and treatment. Clin Plast Surg 2005;32:53Y64
112:e316 18. Vles JS, Colla C, Weber JW, et al. Helmet versus nonhelmet treatment
13. Huang MH, Gruss JS, Clarren SK, et al. The differential diagnosis in nonsynostotic positional posterior plagiocephaly. J Craniofac Surg
of posterior plagiocephaly: true lambdoid synostosis versus positional 2000;11:572Y574
molding. Plast Reconstr Surg 1996;98:765Y774 19. Kamdar MR, Gomez RA, Ascherman JA. Intracranial volumes in a
14. Mulliken JB, Vander Woude DL, Hansen M, et al. Analysis of posterior large series of healthy children. Plast Reconstr Surg 2009;124:
plagiocephaly: deformational versus synostotic. Plast Reconstr Surg 2072Y2075
1999;103:371Y380 20. Kelly KM, Littlefield TR, Pomatto JK, et al. Importance of early
15. Farkas LG. Accuracy of anthropometric measurements: past, present, recognition and treatment of deformational plagiocephaly with orthotic
and future. Cleft Palate Craniofac J 1996;33:10Y18; discussion 19Y22 cranioplasty. Cleft Palate Craniofac J 1999;36:127Y130
Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.