Arch Craniofac Surg Vol.21 No.

2, 80-86
Archives of Craniofacial Surgery https://doi.org/10.7181/acfs.2020.00059

Diagnosis and treatment of positional plagiocephaly

Review Article

Bok Ki Jung, Positional plagiocephaly is increasing in infants. Positional plagiocephaly is an asymmetric defor-
In Sik Yun mation of skull due to various reasons; first birth, assisted labor, multiple pregnancy, prematurity,
Department of Plastic and congenital muscular torticollis and position of head. Positional plagiocephaly can mostly be diag-
Reconstructive Surgery, Institute for nosed clinically and by physical examinations. The simplest way to assess the severity of plagio-
Human Tissue Restoration, Yonsei
cephaly is to use a diagonal caliper during physical examination, which measures the difference
University College of Medicine, Seoul,
Korea between the diagonal lengths on each side of the head. Plagiocephaly can be treated surgically or
conservatively. Positional plagiocephaly, which is not accompanied by craniosynostosis, is treated
conservatively. Conservative treatments involve a variety of treatments, such as change of posi-
tions, physiotherapy, massage therapy, and helmet therapy. Systematic approaches to clinical ex-
amination, diagnosis and treatment of positional plagiocephaly can be necessary and the age-ap-
propriate treatment is recommended for patients with positional plagiocephaly.

Keywords: Craniosynostosis / Helmet therapy / Plagiocephaly

INTRODUCTION claimed that sudden infant death syndrome (SIDS) is closely

related to the prone sleeping position [3,18]. In April 1992, the
Plagiocephaly can be classified into two types, plagiocephaly AAP recommended and enforced a campaign to encourage ev-
that is followed by craniosynostosis and positional plagioceph- ery parent to position the infants in the supine position when
aly in the absence of craniosynostosis [1]. In case of plagio- sleeping to prevent SIDS. Consequently, the incidence of SIDS
cephaly not accompanied by craniosynostosis, the sutures of decreased by more than 40%; however, the incidence of posi-
the skull are normal, and the growth of the skull is often not tional plagiocephaly increased by approximately 600% [18].
hindered [1-4]. However, the skull becomes asymmetrically The incidence of positional plagiocephaly is increasing every
distorted due to various reasons. The most common shape of year [19].
the head is flattened on one side on the posterior part of the Positional plagiocephaly can be defined as deformation and
head [5-8]. When plagiocephaly is accompanied by craniosyn- flattening of one side of the head by continuous application of
ostosis, deformation of the skull is caused by premature fusion an external force. Such deformation and flattening processes
of the sutures present in the skull. This condition is often surgi- mostly occur in the first few months after birth and are mainly
cally treated [9-13] and helmet therapy may also be performed affected by the position of the head [3,4]. The increasing num-
[14-17]. ber of patients with plagiocephaly and continued interest and
In the late 1980s, the American Academy of Pediatrics (AAP) demand of the parents have led to new treatments with the
range gradually increasing as well. Positional plagiocephaly can
Correspondence: In Sik Yun be prevented when the parents fully understand the causes of
Department of Plastic and Reconstructive Surgery, Gangnam Severance Hospital,
Yonsei University College of Medicine, 211 Eonju-ro, Gangnam-gu, Seoul 06273, the disease and show continued interest in infant welfare. Stud-
Korea
E-mail: eqatom@yuhs.ac
ies have reported that educating the parents on the posture and
Received February 20, 2020 / Revised March 2, 2020 / Accepted March 4, 2020 environment of the infant after birth is beneficial for the pre-

Copyright © 2020 Korean Cleft Palate-Craniofacial Association www.e-acfs.org

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/ pISSN 2287-1152
licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. eISSN 2287-5603

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https://doi.org/10.7181/acfs.2020.00059

vention of positional plagiocephaly [3,7,20]. If positional pla- infants, the deformed skull naturally corrects itself over time [1-
giocephaly is detected at a very early stage (before 2 months af- 4,7]. However, most infants sleep in the supine position and
ter birth) with congenital muscular torticollis, early physical turn their heads in response to certain stimuli. In conditions,
therapy for congenital muscular torticollis is very effective in such as congenital muscular torticollis, the tense neck muscle
correcting positional plagiocephaly. Furthermore, maintaining causes the infants to hold their neck and head in a preferred
a position that does not exert pressure on the conservatively flat position [21,22]. Maintaining the head in the same position for
part of the head or a placing the infant in the prone position a prolonged period will cause flattening of the part of the head
may be helpful [21,22]. Following the report of helmet therapy that is facing the floor, and the upper part of the face will begin
by Clarren et al. [23] in 1972, several studies have advocated to flatten thereafter. Both sides of the head will gradually de-
that helmet therapy is very effective as a supplementary treat- form, leading to an asymmetrical shape of the head [7]. When
ment for infants with severe positional plagiocephaly. a part of the skull becomes flat, the infant’s head naturally turns
towards the flat side and is pulled by gravity. The degree of flat-
CAUSES tening gradually worsens during the first 5–6 months after
birth. Compensatory asymmetry of the skull is observed as the
Risk factors for positional plagiocephaly include first birth, as- skull assumes the shape of a parallelogram. Consequently, the
sisted labor, multiple pregnancy, injuries from birth, congenital positions of the ear, lower jaw, and eye pit change, leading to fa-
malformation, premature infants, boys, and especially congeni- cial asymmetry [7,25]. If one side of the back of the head is se-
tal muscular torticollis and remaining in the prone position for verely flattened, the occipital bone is pushed towards the oppo-
a prolonged time [4,7,23,24]. site side on the back of the head, causing the opposite occipital
The most common cause of the disease is the deformation of bone to protrude prominently. Greater force of gravity is ap-
the head shape during birth that is not corrected due to infant plied to regions with a larger surface area. As the frontal bone
being placed in the same sleeping position. During birth, the on the opposite side of the occipital bone flattened by gravity
skull rapidly changes in shape due to the effect of gravity, fluidi- becomes flat, the ipsilateral frontal bone protrudes in a manner
ty of the cranial sutures, and plasticity of the brain. This allows similar to the occipital bone. Therefore, the head tilts towards
the relatively large head to exit the narrow birth canal. In most the flattened occipital bone and the position of the ears changes

Fig. 1. Mechanism underlying the development of positional plagiocephaly. When a portion of the skull becomes flat, the infant’s head is natu-
rally turned towards the flat part and is pulled back by gravity. The degree of flattening gradually increases, and compensatory asymmetry of
the skull is observed. Eventually, the skull is shaped like a parallelogram, and the positions of the ear, lower jaw, and eye pit change, thus, lead-
ing to facial asymmetry. Reprinted with permission from the Korean Pediatric Neurosurgical Society (the Korean Neurosurgical Society) [26].

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Jung BK et al. Positional plagiocephaly

(Fig. 1) [3,7,25,26]. In cases of multiple pregnancy, the limited TREATMENT

intrauterine space prevents fetal posture change. This can lead
to the application of constant force on the fetus’s head, leading Plagiocephaly can be treated surgically or conservatively. Pla-
to positional plagiocephaly [3,7,19]. Positional plagiocephaly is giocephaly, accompanied by craniosynostosis, is often surgically
often accompanied by congenital muscular torticollis, which treated [9-13]. Positional plagiocephaly, which is not accompa-
may contribute towards the development positional plagio- nied by craniosynostosis, is treated conservatively [14-17].
cephaly due to the neck position and tension in the neck mus- Conservative treatments involve a variety of treatments, such
cles and ligaments [21,22]. as change of positions [7], physiotherapy [30], massage therapy
Such progression of asymmetry terminates once the infant [7], and helmet therapy [30-33]. Promising outcomes can be
begins to move, change positions during sleeping, and stand. achieved from conservative treatments when the treatments are
The deformity of the skull can be naturally corrected over a pe- initiated at an early stage of the disease due to the plasticity of
riod of a few months. However, the degree of natural correction the cranium and cranial sutures of the infants [7,30-33]. Fur-
varies depending on the severity of asymmetry, and full correc- thermore, confirmation of other causes of positional plagio-
tion is rarely observed. The uncorrected asymmetrical shape of cephaly and treatment of possible congenital deformity are re-
the head shape at this point is mostly maintained throughout quired. Performing physiotherapy for the tense neck muscles
adulthood [3,7,25]. may be beneficial for congenital muscular torticollis [30].

DIAGNOSIS Principles of the helmet treatment

Many studies have reported the use of external braces for the
Plagiocephaly can mostly be diagnosed clinically and by physi- treatment of positional plagiocephaly. External braces include
cal examinations. Radiography of the skull is performed to de- helmet orthosis, cranial orthosis, cranial orthotic device, and or-
termine craniosynostosis, and ultrasound, magnetic resonance thotic headbands, which are known to be very effective for the
imaging, and computed tomography can be performed to correction of the asymmetrical shape of the head [30-34]. The
achieve a definitive diagnosis. Moreover, three-dimensional helmet generally covers the entire head like a bicycle helmet. The
(3D) reconstitution of the skull after imaging may also aid in headband covers the entire head, except for the top of the head.
detecting craniosynostosis [7,25]. Regardless of the design, customized head braces for patients are
The simplest way to assess the severity of plagiocephaly is to made from high-temperature thermoplastic material (Surlyn,
use a diagonal caliper during physical examination, which mea- Dupont, Wilmington, DE, USA) using high density hypoaller-
sures the difference between the diagonal lengths on each side genic medical grade foam (Fig. 2) [26,34-36]. First, the head of
of the head. A diagonal caliper is a cost-effective tool; however, the patient is scanned with a 3D laser to create a head model.
there may be errors between the measurements, which can re-
duce the accuracy of evaluating the severity of plagiocephaly.
Moreover, the difference between the diagonal lengths may be
reduced by the scalp or hair of the patient, and the error can be
large when the patient markedly when the measurements are
being recorded. A study reported that errors in the readings re-
corded by different examiners may be as high as 2.2 mm on an
average and that imaging data can aid in reducing such errors
when measuring the difference in the diagonal length [27,28].
Difference of 9–12 mm and more than 12 mm in the cranial
vault asymmetry are defined as mild to moderate and severe
asymmetry, respectively [27,28]. The cranial vault asymmetry
index is used to determine the severity of positional plagio-
cephaly and can be calculated as shown below. An index > 3.5
indicates severe asymmetry [27-29]. Fig. 2. Orthotic helmet. The helmet is shaped to cover the entire head,
Cranial vault asymmetry index= while the headband covers all parts of the head, except the top of the
head. High density hypoallergenic medical grade foam is used to make
Difference between cranial diagonal diameter the helmet. Reprinted with permission from the Korean Pediatric
× 100
Shorter cranial diagonal diameter Neurosurgical Society (the Korean Neurosurgical Society) [26].

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The helmet is so that the protruding portion of the head fits tight Table 1. Severity of positional plagiocephaly and recommendations
and there is empty space around the flat part to aid the head in for the time of initiating helmet treatment

assuming a symmetrical and typical shape. As the patient’s skull Age Treatments for positional plagiocephaly

grows, the growth of the protruding part of the head is limited, Before 4 months Posture change [34,41,42]
Physical therapy for congenital muscular torticollis [34]
while the extra space around the flat portion of the head allows
4–6 Months For mild to moderate positional plagiocephaly, continue
for more growth towards the relatively less flat part of the head posture change [34]
with less resistance [37]. Some studies suggest that faster correc- For severe positional plagiocephaly, initiate helmet therapy
tion can be achieved by applying pressure on the protruding [34,43]

portion of the head, which is actively growing [34-39]. Older than 6 months For mild to moderate positional plagiocephaly with no
improvements after posture change, initiate helmet
therapy [34,42]
Application of helmet treatment For severe positional plagiocephaly, initiate helmet therapy
There is controversy regarding the age at which helmet treat- [34,42]
ment should be initiated. If positional plagiocephaly is discov-
ered at an early stage, change of position is often the main when helmet therapy begins sooner. In particular, many studies
method of treatment with further observation. If mild posi- also report that the effect of helmet therapy is stronger when
tional plagiocephaly is observed before 4 months of age, chang- helmet therapy is performed in combination with posture
ing the sleeping position of the infant should be started early changing and physiotherapy for congenital torticollis. There-
[34]. The minimum age of patients eligible for helmet treat- fore, age-appropriate treatment is recommended for patients
ment is not defined, and helmet treatment should be initiated with positional plagiocephaly (Table 1) [34,41-43]. Once helmet
before the infants can control their head movements [38,40]. therapy is initiated, it is fundamental that the parents are in-
Helmet treatment is more effective if it is performed at an early formed that the patients must not wear the helmet for more
stage of development of the head, and the treatment should be- than 20 hours a day. The total duration of helmet treatment av-
gin when the skull is rapidly growing. Therefore, most doctors erages between 2 to 6 months, and the parents should note that
recommend initiating helmet treatment before 6 months of age the duration will vary depending on the degree of asymmetry
for rapid and effective correction of the head. When positional of the head shape and the time of commencement of treatment.
plagiocephaly is not corrected or improved on conservative Promising results can be obtained when the parents of the pa-
treatment, helmet treatment should be initiated for babies at the tient are interested in the head shape of the patient and actively
age of 6 months [34,41-43]. Approximately 85% of cranial participate in implementation of helmet therapy. The parents
growth is achieved in the first 12 months after birth, and the should be informed that correction of the shape of the head
cranial growth rate is significantly decreased from 12 months with active helmet treatment can also prevent facial asymmetry,
until 24 months of age. Therefore, helmet treatment is very ef- with the growth of the skull [7,30,39,45-47].
fective up until 12 months of age, and the effect is very weak if
the treatment is started after 12 months of age. The outcome of Complications and limitations of helmet therapy
asymmetry correction by skull growth is very poor after 18 The complications of helmet therapy, although very rare, in-
months of age [34,41-44]. clude (1) inadequate correction due to a poorly fitting helmet,
There are three reasons for the controversy regarding the age (2) damage to the skin at the site of application of pressure by
at which helmet treatment should be initiated. First, the degree the helmet, (3) damage to the scalp and temporary hair loss at
of asymmetry of the head determines the feasibility of helmet the site of application of pressure by the helmet, (4) contact al-
treatment. Therefore, variation in the results according to the lergic reaction of the skin depending on the material of the hel-
degree of asymmetry will be apparent, regardless of the timing met. Most complications, including skin and scalp damage,
of the helmet treatment. Second, most patients who receive hel- temporary hair loss, and allergic reactions, are naturally cured
met treatment also receive conservative treatment, such as without any special treatment and can be prevented if the par-
changing the posture, which limits the accurate determination ents pay attention. The complications arising from poorly fitting
of the effect of helmet treatment. Third, the duration of treat- helmets can also be prevented by modifying the helmet through
ment and pressure exerted by the helmet vary according to the parental attention and regular outpatient care [31,48-50]. How-
physicians and the type of helmet used. Thus, defining the ap- ever, helmet treatment has economic disadvantages compared
propriate age to start helmet therapy is challenging. to other conservative treatments [34,40,48,51]. The cost of the
However, most studies report that better results are obtained helmet varies with the type of the helmet; however, the helmet

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Jung BK et al. Positional plagiocephaly

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