Dec 2011

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SPECIAL EDITORIAL

Current Concepts in Deformational Plagiocephaly


Wojciech Dec, MD, and Stephen M. Warren, MD

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

FIGURE 3. Transcranial anthropometry compares diagonal


transverse cranial diameters. Note that anthropometric
measurements are being demonstrated on a volunteer; this
FIGURE 1. Frontal view of a child with right-sided posterior patient does not have posterior deformational plagiocephaly.
deformational plagiocephaly demonstrates ipsilateral
vertically elongated palpebral fissure and an inferiorly cephaly is another important variable in determining outcome.
positioned ipsilateral ear. Unilateral deformities can often be normalized with helmet ther-
apy; however, the deformity in patients with bilateral brachycephaly
improve with conservative therapy can be candidates for external can often be improved, but complete normalization is rare.9 Unlike
cranioplasty with a molding helmet.17 in synostotic craniofacial deformities, surgical correction does not
Helmet molding therapy takes advantage of the infant’s pli- play a role in the management of deformational plagiocephaly.
able skull and rapid brain growth.9,18 By restricting growth in one
direction, the helmet encourages the skull to preferentially expand
in the desired direction to improve symmetry. The challenge with CONCLUSIONS
helmet therapy is that it requires strict compliance with wearing the Supine positioning of infants during sleep has decreased the
helmet 23 hours per day, carries an additional financial cost, and incidence of sudden infant death syndrome, but it has also signifi-
requires frequent modifications of the helmet to accommodate for cantly increased the occurrence of deformational plagiocephaly. Pri-
head growth and correction of the deformity over a period of 4 to mary care providers must educate parents about supervised prone
5 months of therapy. positioning and adjusting head position during supine sleep to reduce
Successful treatment outcomes are associated with several the incidence and severity of skull deformities. Diagnosis is usually
variables. Correction of deformational plagiocephaly whether by based on history and physical examination by an expert clinician,
repositioning strategies or helmet therapy is linked to brain volume but computed tomography and ultrasound can be used selectively to
expansion. For example, the brain doubles in volume in the first confirm the diagnosis. Treatment includes altering the head position
9 months of life and achieves 80% of its final size by the age of during sleep and molding helmets for selected cases. Surgery does not
2 years.19 Therefore, the age at which positional or molding helmet play a role in the treatment of deformational plagiocephaly.
therapy is initiated is an important variable in determining how
much correction can be achieved; younger patients can achieve REFERENCES
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* 2011 Mutaz B. Habal, MD 7

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

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8 * 2011 Mutaz B. Habal, MD

Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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