POSITIONAL
PLAGIOCEPHALY
INTRODUCTION
Positional skull deformation is a condition in which the shape of an infant’s skull deforms as a result of prolonged external forces (Van Wijk et al., 2014). The two most common forms of skull deformation include Plagiocephaly and Brachycephaly (Van Wijk et al., 2014).
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Plagiocephaly is characterised by unilateral parieto-occipital flattening with ipsilateral frontal bossing of the forehead and anterior shift of the ipsilateral ear and cheek (Baird et al., 2016). As such, plagiocephaly often results in a parallelogram deformity of the head (Baird et al., 2016). In contrast, Brachycephaly is characterised by symmetrical bi-occipital flattening, with anterior-posterior foreshortened head and temporal bossing (Kelly, Joganic, & Beals, 2018).
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As skull deformation does not affect the development of an infant’s brain it is often considered a purely cosmetic condition due to the facial asymmetry and misalignment of the eyes and/or ears that often accompanies this condition (Van Wijk et al., 2014). In more severe cases however, both Plagiocephaly and Brachycephaly have been associated with auditory processing disorders, mandibular asymmetry, impaired occlusal function, and visual field defects (Kelly et al., 2018).
A rise in the prevalence of skull deformation occurred after widespread implementation of the American Academy of Paediatrics’ “Back to Sleep” recommendation that infants be positioned in a supine sleeping position to prevent Sudden Infant Death Syndrome (SIDS; Baird et al., 2016). Skull deformation is most prevalent between two (16% - 22%) and four months (20%) after birth, with the prevalence reducing as infants become older due to the achievement of gross motor milestones and varying positions of play (Van Wijk et al., 2014).
For the purpose of this educational resource, positional Plagiocephaly will remain the focus of the information below pertaining to the physiotherapist’s role in identifying head shape deformities and determining the best possible course of treatment / management.
Figure 43. Plagiocephaly and Brachycephaly
(Flat Head Syndrome, 2019)
CLINICALLY RELEVANT ANATOMY
At birth, the newborn’s skull is soft and malleable. The newborn skull consists of five major bones (two
frontal, two parietal, and one occipital) that are separated by connective tissue junctions known as cranial sutures. The sutures are highly necessary to facilitate movement and moulding of the cranium through the birth canal during labour. They also allow for rapid postnatal growth and development of the brain (Lipsett & Steanson, 2019).
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The bones that shape the cranium are initially unfused, leaving several gaps between the individual bones of the infant’s skull. These gaps are composed of membranous connective tissue and are known as fontanelles. Fontanelles are often referred to as “soft spots” and are one of the most prominent anatomical features of the newborn’s skull. The most notable sutures include the anterior and posterior fontanelles (Lipsett & Steanson, 2019).
Figure 44. Cranial Sutures
(Children’s Health Queensland Hospital
and Health Service, 2019)
The anterior fontanelle is the largest fontanelle and resembles a diamond shape ranging in size from 0.6cm to 3.6cm. The average closure time for the anterior fontanelle ranges from 13 to 24 months (Lipsett & Steanson, 2019). Unlike the anterior fontanelle, the posterior fontanelle is triangular and completely closes within approximately 6 to 8 weeks after birth. On average, the posterior fontanelle is 0.5cm in diameter (Lipsett & Steanson, 2019).
As the growth and development of the newborn continue, each fontanelle will close within their respective timeframes by a process known as intramembranous ossification (Lipsett & Steanson, 2019). As such, it is essential the growth of a newborn’s skull is monitored to ensure it is tracking as expected. The infant’s maternal child health nurse should measure the circumference of the infant’s head at each visit and plot their growth on an appropriate chart. If the head circumference fails to track as expected or abnormalities of sutures and/or the fontanelles are noted, this is a red flag for further assessment (Victorian Government, 2009).
AETIOLOGY
Positional Plagiocephaly occurs as a result of prolonged, unevenly distributed mechanical forces on the malleable bones of the neonatal cranium that direct the growth of the cranium towards an asymmetrical shape (Baird et al., 2016). Plagiocephaly may occur antenatally or during birth due to in utero constraint or assisted delivery methods. More commonly however, Plagiocephaly occurs during the postnatal period due to the infant exhibiting a positional head preference and/or spending an extended period of time with their head in a constant resting position (Baird et al., 2016).
RISK FACTORS
According to Renz-Polster and De Bock (2018) a variety of intrinsic and extrinsic risk factors are associated with the development of Plagiocephaly including:
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Male gender
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First born child
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Multiple birth (ie. twins and above)
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Breech or transverse position in utero
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Use of forceps or vacuum extraction during delivery
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Prematurity
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Macrocephaly (ie. more than 2 standard deviations above expected head size for corresponding age)
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Oligohydramnios (ie. decreased amniotic fluid inside the uterus)
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Limited cervical spine range of motion due to Torticollis
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Positional head preference position
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Poor tolerance of tummy time
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High cumulative exposure to supine positioning
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Slow achievement of gross motor milestones
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Non-varying nursing habits
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Multifunction infant carriers
CLINICAL PRESENTATION
It is quite common for a newborn baby to have an unusually shaped head. This may be related to their position in the uterus during pregnancy or caused by moulding during labour. Depending on the cause, most babies' heads should return to a normal shape within approximately six weeks after birth (Lennartsson & Nordin, 2019).
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Occasionally a baby's head does not return to a normal shape. When viewed from above, infants with Plagiocephaly will have a parallelogram head shape. In its most classical form, Plagiocephaly will present as unilateral parieto-occipital flattening with ipsilateral frontal bossing of the forehead and anterior shifting of the ipsilateral ear and cheek (Aarnivala et al., 2015).
Figure 45. Plagiocephaly
(Children’s Hospital of Philadelphia, 2019)
OBJECTIVE ASSESSMENT
PALPATION
Cranial Sutures: The four cranial sutures, including the Metopic Suture, Coronal Suture, Sagittal Suture and Lambdoid Suture should be carefully palpated noting for any ridges or overlapping (Lewis, 2014). A prematurely fused suture is indicative of Craniosynostosis and occurs in approximately 1 in 1,000 newborns (Lewis, 2014). Craniosynostosis is a congenital defect, that results in impaired brain development and an abnormally shaped skull. If left untreated, Craniosynostosis may cause serious complications such as developmental delay, sensory, respiratory and neurological dysfunction, anomalies affecting the eye and psychological disturbances (Kajdic, Spazzapan, & Velnar, 2018).
Anterior Fontanelle: The anterior fontanelle should also be carefully palpated, noting whether it is open, closed, soft, patent or bulging (Lewis, 2014). Usually the anterior fontanelle is 0.6cm to 3.6cm. A large anterior fontanelle may be indicative of increased intracranial pressure, Down Syndrome, Hypophosphatemia, Trisomy, or Congenital Hypothyroidism (Lewis, 2014).
Sternocleidomastoid (SCM): Both SCM muscles should be palpated, noting for any masses or lumps (Lewis, 2014). Palpable lumps or masses usually located within the middle to lower third of the SCM muscle are often indicative of a benign tumour (Nilesh & Mukherji, 2013). The presence of a SCM tumour is indicative of Congenital Muscular Torticollis (CMT) and may be the potential cause for Plagiocephaly developing (Nilesh & Mukherji, 2013).
Figure 46. Palpating an Infant’s Skull
(Vargo, 2016)
CERVICAL RANGE OF MOTION (ROM)
A strong association between CMT, positional head preference, and the onset of the Plagiocephaly has been suggested (Murgia et al., 2016). CMT is characterised by unilateral shortening of the SCM muscle. The presence of muscular imbalances of the neck may cause the infant to adopt a position of comfort that results in persistent rotation, thus increasing the risk of Plagiocephaly (Murgia et al., 2016).
Measuring active and passive cervical rotation and lateral flexion ROM in infants with Plagiocephaly forms an essential component of the physical examination (Lewis, 2014). The assessment allows the practitioner to determine if a head rotation preference, decreased active cervical ROM and/or CMT are contributing factors to the development of Plagiocephaly. It is also essential to assess the severity of Plagiocephaly to guide the provision of therapeutic interventions and monitor their effectiveness (Murgia et al., 2016).
Test Administration: Cervical Rotation A/PROM
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To assess an infant’s cervical rotation ROM (Murgia et al., 2016):
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Place the infant supine on a firm flat surface
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Place the practitioner’s right hand on the infant’s right shoulder to provide stabilisation
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Starting in midline, shake a rattle / toy to left side of the infant’s head to encourage active cervical rotation to the left
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Once the infant has reached their maximum degree of active ROM, using the practitioner’s left hand, gently apply pressure on the right side of their face to obtain full passive ROM
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Repeat steps 2 to 4 on the opposite side
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Test Administration: Cervical Lateral Flexion A/PROM
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To assess an infant’s cervical lateral flexion AROM (Seager, French, & Meldrum, 2019):
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Hold the infant at their mid-trunk level and suspend in the vertical position
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Slowly bring the infant into a horizontal position
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Observe the infant’s cervical lateral flexion active ROM during lateral head righting
To assess an infant’s cervical lateral flexion PROM (Murgia et al., 2016):
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Place the infant supine on a firm flat surface
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Place one hand on the infant’s left shoulder to provide stabilisation
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Place the other hand across the infant’s occipital bone
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Gently bring the infant’s right ear toward their right shoulder
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Repeat steps 2-4 on the opposite side
Figure 47. Cervical Rotation PROM
(Paediatric Orthopaedic Specialists 2018)
Figure 48. Lateral Flexion ROM
(Paediatric Orthopaedic Specialists 2018)
Test Interpretation
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Cervical rotation passive ROM is considered to be normal at 110°, whilst cervical lateral flexion passive ROM is considered to be normal at ~70° (Murgia et al., 2016). Decreased cervical rotation and/or lateral flexion ROM may be contributing factors to the development of an infant’s Plagiocephaly (Murgia et al., 2016).
Figure 49. Cervical ROM
(Murgia et al., 2016)
Figure 50. Lateral Flexion ROM
(Seager, French, & Meldrum, 2019)
SEVERITY ASSESSMENT FOR PLAGIOCEPHALY
The Severity Assessment for Plagiocephaly is a commonly used assessment tool that examines the severity of Plagiocephaly, and monitors progress made during treatment (Öhman, 2011).
The scale consists of five subcategories: 1) posterior flattening; 2) ear misalignment; 3) forehead asymmetry; 4) neck involvement; and 5) facial asymmetry (Öhman, 2011). Each subcategory consists of 4 images that represent a continuum of asymmetry and may be used as a visual guide when assigning scores (Lennartsson, Wennergren, & Nordin, 2015).
Each item is given a score from 0 to 3 that is indicative of the degree of asymmetry noted for the particular feature. Each item is to be scored as follows: 0 represents no asymmetry, 1 represents mild asymmetry, 2 represents moderate asymmetry and 3 represents severe asymmetry (Lennartsson et al., 2015). As an infant’s skull asymmetry often falls between 2 levels, half scores may be used.
Test Administration
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To perform the Severity Assessment for Plagiocephaly (British Columbia Children’s Hospital, 2008):
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Sit the infant on the floor if able to sit independently or alternatively supported in their caregiver’s / practitioner’s lap if unable to sit independently
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Observe the side and degree of posterior flattening by looking down from above the infant
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Place fingers in both of the infant’s ear canals and identify which ear is anteriorly positioned whilst looking down from above the infant
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Observe the infant’s forehead whilst looking down from above the infant and determine if the positioning corresponds with the infant’s ear position
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Determine whether there is any neck involvement by observing the infant’s head position in the coronal plane
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Observe the infant’s eye level in the coronal plane to determine whether the infant’s face is symmetrical
Figure 51. Severity Assessment for Plagiocephaly (Öhman, 2011)
Test Interpretation
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The total sum of scores is calculated with a minimum score of 0 representing no asymmetry, and a maximum score of 15 representing severe asymmetry in all 5 categories (Öhman, 2011). According to Öhman (2011), the final score assigned to the severity of an infant’s Plagiocephaly may be influenced by the practitioner’s experience.
DEVELOPMENTAL MILESTONE SCREENING
For more information on developmental milestone screening
SPINE AND TRUNK SCREENING
For more information on spine and trunk screening
HIP SCREENING
For more information on hip screening
FOOT SCREENING
For more information on foot screening
TREATMENT OPTIONS
For the majority of infants, Positional Plagiocephaly is a largely preventable condition. According to Flannery, Looman and Kemper (2012), Plagiocephaly may be effectively prevented or treated with simple repositioning techniques that minimise mechanical pressure on the infant’s head. The following treatment techniques are most effective if implemented from birth. For most infants, regular repositioning of the infant’s head prior to 4 months of age will result in optimal outcomes (Red Nose, 2018).
SLEEPING POSITIONS
In accordance with the Sudden Infant Death Syndrome guidelines, infants should always be placed in a supine sleeping position (Red Nose, 2018). It is important that infants are not placed in a side-lying position to prevent Plagiocephaly as the risk of infants rolling into prone and suffocating is greatly increased (Red Nose, 2018).
A young infant will generally stay in the same position they are placed to sleep until they develop the skills to move themselves (Cummings, 2011). To reduce prolonged pressure on one side of the skull, parents are encouraged to alternate their infant’s head position (left and right) each time the infant is put down to sleep (Cummings, 2011).
As infants become more alert and interested in their environment, they tend to turn to the direction in which they receive increased stimuli (Eg. direction of light from window / direction that parents enter the room). Positioning the infant with his/her head to the foot or to the head of the bed on alternating days is recommended to ensure the infant is lying equally on both sides of the occiput (Cummings, 2011). Alternatively, changing the position of the cot in the room may have the same effect (Royal Children’s Hospital, 2018e).
Figure 52. Recommended Sleeping Positions (Red Nose, 2018)
FEEDING POSITIONS
Alternating the holding position used when feeding the infant is recommended (Eg. hold in left arm for one feed and the right arm for the next feed; Red Nose, 2018).
Figure 53. Tummy Time
(Royal Children’s Hospital, 2017b)
TUMMY TIME
‘Tummy time’ refers the period in which an awake infant is positioned in prone whilst being supervised by an adult (Cummings, 2011). Tummy time facilitates offloading of the skull and provides the infant with an opportunity to progress developmental milestones that require the prone position (Cummings, 2011). Evidence suggests that infants should initially partake in several minutes of tummy time at least 3 times per day and gradually increase to 10 to 15 minutes of tummy time 3 times per day, to reduce the development of Plagiocephaly (Cummings, 2011).
PLAY TIME
When the infant is awake and alert, playing in a supine position with rattles, toys, or faces placed in different positions may be appropriate to encourage the infant to turn their head equally in both directions (Royal Children’s Hospital, 2018e). Moreover, time spent in side-lying with parental assistance and supervision may effectively offload the skull (Royal Children’s Hospital, 2018e).
Figure 54. Play Positions
(Royal Children’s Hospital, 2017b)
CARRYING POSITIONS
When the infant is awake, avoid prolonged periods of supine positioning in car seats, carriers, and bouncers as these positions place additional pressure on the skull. Instead, vary an infant’s position regularly by cuddling the infant in an upright position, using a sling, or carrying the infant over the parent’s shoulder, in prone or on their side (Royal Children’s Hospital, 2018e).
For more information on carrying positions
STRETCHES
For more information on stretches
HELMET THERAPY
Infants with severe, persistent skull deformity that has been unresponsive to counter-positioning may need to be treated with helmet therapy (Royal Children’s Hospital, 2018e). A lightweight helmet is made by an orthotist using 3 dimensional images of the infant’s skull. The helmet helps to reshape the infant’s skull by taking pressure of the affected area and allowing the skull to grow into the space provided (Royal Children’s Hospital, 2018e).
Helmet therapy is most effective when started between 6 and 8 months of age, and completed before 12 months of age, as this is the time of rapid growth of the skull (Royal Children’s Hospital, 2018e). The helmet is usually worn 23 hours per day and is only removed for bathing purposes. Helmet therapy usually takes between two and six months to complete, and the orthotist will review the infant every 3-4 weeks (Royal Children’s Hospital, 2018e).
RECOMMENDED REFERRALS
Referral to the Royal Children’s Deformational Plagiocephaly Clinic is recommended when infants score greater than 8/15 on the Plagiocephaly Severity Assessment Scale, or if the infant scores 3 for any of the first 3 items, or if the combined score of the first 3 items is greater than 6. The referral must be made by the infant’s General Practitioner, and the referral form is available on the Royal Children’s Hospital website (Henty, 2018).
For infants with Craniosynostosis, referral to an infant’s General Practitioner and Paediatrician is essential to facilitate appropriate radiographic investigations and initiate referral to the Plastic and Maxillofacial Department at the Royal Children’s Hospital (Royal Children’s Hospital, 2019b).