A Parent's Guide to Plagiocephaly

Introduction

Plagiocephaly, sometimes referred to as a “flat head”, is the medical term used to describe a specific pattern of head shape asymmetry. The term “flat head” is misleading because, although the back of the head is flat, the entire head and sometimes the face itself become asymmetrical as well. Plagiocephaly is characterized by a flat area on the back of one side of the head with a bulging of the forehead and a forward repositioning of the ear on the same side. Although there are two different categories of plagiocephaly (positional and synostotic), it is usually assumed that the term plagiocephaly is referring to positional plagiocephaly, unless stated otherwise. Sometimes the term deformational plagiocephaly is used synonymously with positional plagiocephaly; both terms refer to the same form of plagiocephaly.

 

Illustration of Positional Plagiocephaly

Direction of Plagiocephaly Forces

The infant skull is made up of five bones loosely held together by fibrous structures called sutures.  These sutures serve as growth centers which allow the skull to grow quickly to accommodate the rapidly-growing infant brain during the first year of life. The sutures provide this vital function, but as a result, the skull is malleable during the first year of life because the bones are not fused in these areas. If, for some reason, there is an uneven amount of pressure placed on the infant skull, this malleability will result in the head becoming abnormally shaped by the pressure.  The most common abnormal head shape is plagiocephaly. Although this is associated with an abnormal head shape, it is not associated with any underlying brain abnormalities[1].

Child with Plagiocephaly

Plagiocephaly Head Shape

Almost 50% of newborns develop some form of positional plagiocephaly within the first few weeks of life. Although there is great awareness and anxiety associated with the helmet therapy that is sometimes necessary to correct this, with early diagnosis and proper treatment, only about 10% of children with positional plagiocephaly will require helmet therapy to correct their head shape. With early and proper treatment by a team of specialists, Plagiocephaly and facial asymmetry should correct to a level that is normal.

Brachycephaly

Image of child with brachycephaly

Brachycephaly is similar to plagiocephaly in that the cause and treatment are the same. The difference is that Brachycephaly is a different pattern of abnormal head shape. In a brachycephalic head, the back is symmetrically flat, not just on one side as in plagiocephaly. Similarly, the forehead will bulge symmetrically. Brachycephaly is caused by prolonged pressure in the middle of the back of the head as opposed to plagiocephaly which is caused by prolonged pressure on only one side of the back of the head.

Terminology

Technically, there are three separate terms that are related to the term plagiocephaly.

  • Positional plagiocephaly
  • Positional brachycephaly
  • Synostotic plagiocephaly

Because of the similarity of positional plagiocephaly and positional brachycephaly and because of the rare incidence of synostotic plagiocephaly, it is not uncommon for the term plagiocephaly to refer to positional plagiocephaly and positional brachycephaly as a group. Commonly doctors and medical personnel will use the term plagiocephaly when referring to any form of positional or deformational head shape abnormality.

Etymology

Plagio-cephaly
Plagio – “slanting, oblique,” from Greek plagios [1]
Cephalo- “head, skull, brain,” Modern Latin combining form of Greek kephalē “head” [2]

Brachy-cephaly
Brachy – “short,” from Latinized combining form of Greek brakhys “short” [3]
Cephalo- “head, skull, brain,” Modern Latin combining form of Greek kephalē “head” [2]

Craniosynostosis 
Cranion = skull – κρανίον (Ancient Greek)[4]
Syn = together – συν (Ancient Greek)[4]
Ostosos = bone – οστέον (Ancient Greek)[4]

“Back to Sleep”

The “back to sleep” campaign (now referred to as the “Safe to Sleep®” campaign) is an extremely important and successful campaign which has saved many infant lives by significantly reducing the incidence of SIDS (Sudden Infant Death Syndrome). However, it is felt to be a large factor contributing to the rise in the incidence of plagiocephaly.  Despite the increased incidence of plagiocephaly associated with “back to sleep”, it is of upmost importance to follow the “safe to sleep®” recommendations.

SIDS was identified as a medical identity as far back as 1969 when scientists coined the term. Beginning around 1988, doctors in the Netherlands noted a corelation between SIDS and back sleeping. Subsequent studies published in Australia, New Zealand, and the United Kingdom confirmed the significant link between SIDS and stomach sleeping. In 1992 the American Academy of Pediatrics Task Force officially recommended that U.S. babies be placed on their backs or sides to sleep to help reduce the risk of SIDS and in 1994 they officially launched the Back to Sleep® campaign as a way to bring public attention to SIDS and to educate caregivers on ways to reduce the risk of SIDS. The campaign has evolved to include such recommendations as types of bedding, positioning in crib, temperature regulation, avoidance of any head positioning devices, etc. As a result of this expansion in scope of the campaign, in 2012 the campaigns name was changed from the Back to Sleep campaign to the Safe to Sleep® campaign.

Safe To Sleep Brochure

Please click on the link below to view the official NIH Safe to Sleep® brochure.

Safe to Sleep® – Instructional Brochure 

Courtesy of the Eunice Kennedy Shriver National Institute of Child Health and Human Development.

The Plagiocephaly Epidemic

The “Safe to Sleep®” campaign has been tremendously successful and has saved a significant number of children’s lives by dramatically reducing the incidence of SIDS.  However, the increased back positioning associated with the campaign has led to a significant increase in the incidence of Plagiocephaly. In fact, as the Safe to Sleep® campaign continues to become even more effective by expanding to different cultures and languages, plagiocephaly is also becoming more common. This continued increase in its incidence has led some to refer to it as the Plagiocephaly Epidemic. Although plagiocephaly is a small price to pay for the life-saving effects this campaign has achieved, the increasing incidence does need to be addressed. Pediatricians, craniofacial surgeons, and neurosurgeons have been challenged to deal with this epidemic. Thankfully the American Academy of Pediatrics and the Congress of Neurologic Surgeons have provided evidence-based guidelines recommending proper treatment in an effort to prevent the overtreatment of plagiocephaly. The most recent guidelines are summarized in the Guidelines for Treatment section of this article. The most important element to remember is that it is of the utmost importance to follow the Safe to Sleep® guidelines. Plagiocephaly is just a side effect that can be treated.

Signs of Plagiocephaly

There are several signs of plagiocephaly that parents can look for:

  • A flattening on one side of the back of a child’s head.
  • A bulge of the forehead on the same side as the flattening on the back of the head.
  • The ear on the flattened side of the head may appear to be pushed forward when viewed from above.
  • The cheek and jaw may appear asymmetrical or protruding on the affected side
  • There may be an obvious head position preference to one side

Torticollis and Plagiocephaly

Often children are born with an abnormally tight or shortened neck muscle leading to an imbalance of neck functioning referred to as torticollis. When this occurs, the head will be rotated to the side of the affected muscle, resulting in a characteristically abnormal head position. This head position leads to asymmetrical pressure on the back of the infant’s skull when lying on their back in bed or when in strollers and car seats. The asymmetric pressure to one side of the head results in an abnormal amount of pressure on that side and typically results in some form of positional plagiocephaly. Almost 100% of children with plagiocephaly have some form of torticollis or neck instability leading to abnormal head positioning[6]. Early diagnosis and treatment of torticollis is essential in the treatment of plagiocephaly and helping to prevent the need for a cranial reshaping helmet.

At Plagiocare, our physical therapists are torticollis experts with expertise and years of experience in plagiocephaly. For more information please visit our torticollis page.

Facial Asymmetry

It is common for children with plagiocephaly to have facial asymmetry as well. Because the skull bones are connected to the facial bones, the pressure causing plagiocephaly can be transmitted to the facial bones as well. In addition, as described above, almost all children with plagiocephaly have some form of torticollis. Torticollis itself can cause facial asymmetry because of the pressure of the face against the neck or shoulder on the affected side. With proper diagnosis and treatment, the facial asymmetry associated with plagiocephaly and torticollis typically disappears over time. However, it is important for any facial asymmetry to be evaluated by an experienced doctor to assure that there are not more serious underlying conditions present.

If treated early and correctly by a team of specialists, plagiocephaly and facial asymmetry should correct to a level that is considered normal. Remember, nobody’s head and face are perfectly symmetrical.

Causes of Plagiocephaly

There are many factors which can cause positional plagiocephaly. It is believed that an asymmetrical functioning of the neck muscles referred to as torticollis is the underlying cause of most cases of plagiocephaly. When the neck muscles are imbalanced, the child’s head becomes turned to one side, and that side will experience an increased amount of pressure on the back of the head. This unbalanced pressure is felt to be the cause of the majority of cases of plagiocephaly. Although torticollis is a major factor in the development of plagiocephaly, below is a list of additional potential factors that may cause or exacerbate plagiocephaly.

  • Position of the fetus during pregnancy
  • Pregnancy with more than one child (twins, triplets, etc.)
  • Premature birth
  • NICU stay after birth
  • “Back to Sleep” positioning (NOTE: This is still essential for the baby’s safety)
  • Car seats and carriers

Plagiocephaly vs. Lambdoid Craniosynostosis

Lambdoid Synostosis vs Plagiocephaly

Plagiocephaly of an infant’s skull is a specific pattern of head deformation resulting from uneven pressure on the back of the skull.  This type of plagiocephaly is referred to as positional or deformational plagiocephaly. Plagiocephaly can also be the result of a premature fusion (synostosis) of the lambdoid bones in the back of the head referred to as synostotic plagiocephaly. The more technical term for this premature fusion is lambdoid craniosynostosis.  This premature fusion of the lambdoid bones prevents skull growth in the area, resulting in an asymmetrical head shape. Lambdoid craniosynostosis is much less common but significantly more serious than positional plagiocephaly. Since the brain is rapidly growing in an infant and the area of craniosynostosis does not allow for expansion of the skull, the brain begins to expand the skull in the areas where the skull bones have not fused. This asymmetrical expansion results in an asymmetrical head shape with a flatness in the area of synostosis. Although similar to positional plagiocephaly, the head shape associated with lambdoid craniosynostosis has distinct differences. In lambdoid craniosynostosis, the opposite side of the forehead will be bulging, and the ear is typically pulled down and back towards the flattened area on the back of the head (instead of being pushed forward as in plagiocephaly).  If a child has a flat head or abnormal head shape, it is important to have them evaluated by a doctor with expertise in plagiocephaly and craniosynostosis. Most of the time a diagnosis can be made with a complete examination as discussed in the evaluation section below. In some cases, extra radiology tests are required. In the case of craniosynostosis, treatment typically requires some form of surgical correction.

Evaluation

An initial evaluation should be performed as soon as there is any indication of plagiocephaly or torticollis, ideally at 7 weeks of age. A preliminary evaluation should begin with a pediatrician if they are experienced and up-to-date with the established guidelines and treatment protocols for the treatment of plagiocephaly and torticollis as described in the Guidelines for Treatment section. Otherwise, evaluation at a Plaigocare center or by a craniofacial surgeon or neurosurgeon is recommended. A proper evaluation should include a detailed history, physical exam, cranial measurements, torticollis evaluation, photos, and a 3-D light scan as detailed below.

HISTORY
The initial evaluation should begin by obtaining a thorough history, inquiring about potential causes of plagiocephaly, torticollis, and most importantly, craniosynostosis. The following elements should be included in a proper history:

  • Abnormal head shape present at birth
  • Developmental delays
  • Failure to meet milestones
  • Elements consistent with positional plagiocephaly
  • Multigestational pregnancies (twins, triplets, etc.)
  • Torticollis
  • Stay in the NICU
  • Normal head shape at birth
  • Compliant with “safe to sleep”

EXAMINATION
A thorough examination should include a comprehensive evaluation of the entire skull and face, evaluating for patterns of abnormal head shape and facial asymmetry. A proper exam should include the evaluation of the following:

  • Facial symmetry
  • Eye position
  • Ear position
  • Head shape
  • Palpation for any ridges indicating premature bone fusion
  • Palpation of both fontanels for size, shape, bulging, etc.
  • Evaluation from an aerial view to gain perspective of overall head shape and ear positioning

PHOTOS
Clinical photographs should be taken with either a stockinette in place or with wet combed hair. Photos should be taken using standard protocols. This involves photographing the face and skull from the same views and the same distance during each photo session. This allows for standardized comparison of photos. The standard views taken should include at least the following:

  • Frontal
  • Right and left ¾
  • Right and left profile
  • Back
  • Aerial

CRANIAL MEASUREMENTS
There are many measurements that will be calculated from the 3-D light scan. However, clinically, at least two measurements should be taken:

  • CVAI – Cranial Vault Asymmetry Index
  • CR – Cephalic Ratio

The measurements are discussed in detail in the section titled Understanding the measurements

3-D LIGHT SCAN
A 3-D light scan of the head should be performed during an initial visit. This gives the most accurate evaluation of the head shape and provides multiple informative measurements and calculations used in making a proper diagnosis. In addition, an initial light scan provides a baseline to be used for comparison at future examinations.

TORTICOLLIS EVALUATION
An evaluation for torticollis should be performed for any child when plagiocephaly is suspected. Torticollis is commonly the root cause of plagiocephaly and treatment of torticollis, if diagnosed, should be started at 7 weeks of age to help prevent the need for helmet therapy. Please see the Torticollis page for more information.

RADIOLOGY
The need for radiology studies is rare, but sometimes, this additional information is necessary. Additional studies may include a CT scan, MRI, Ultrasound, or standard X-Rays.

Understanding The Measurements

The two most common measurements used in the evaluation of plagiocephaly and brachycephaly are CVAI (Cranial Vault Asymmetry Index) and CR (Cephalic Ratio).  These two measurements are used as baseline guides to determine if plagiocephaly or brachycephaly exists, as well as the severity of each. These measurements are used as part of the decision process when developing a treatment plan, and they are used by most insurance companies when determining coverage.

CVAI – Cranial Vault Asymmetry Index

The CVAI measures the amount of asymmetry from one side of the head to the other. It is used to determine if plagiocephaly exists and if so how severe. A diagonal measurement from the left forehead to the back of the right side of the head (measurement A) is compared to a diagonal measurement from the right forehead to the back of the left side of the head (measurement B). The difference between measurement A and B is then calculated:

CVAI = ((A-B) x 100) / A or B (whichever is greater)

CR – Cephalic Ratio

The cephalic ratio is used to determine if brachycephaly is present and if so, how severe. The CR compares the width of the skull to the length of the skull.  

CR = (Width X 100) / length

Most individuals, institutions, and insurance companies refer to the CHOA (Children’s Hospital of Atlanta) scale when evaluating the values for CVAI and CR. The CHOA scale was published in the Journal of Craniofacial Surgery in 2006 and has been used since that time as the standard of care.

According to the CHOA scale:

  • Any CVAI of 6.5 or higher qualifies for helmet therapy.  
  • The cephalic ratio is a little more complicated, and it takes age and sex into account.  Using the scale below, most references use any CR greater than 2 standard deviations from normal to qualify for helmet therapy.

Diagnosis

The proper treatment of positional plagiocephaly starts with a correct diagnosis. The most important aspect of the diagnosis is to make sure that more serious issues such as craniosynostosis and hydrocephalus are not missed.

Using all of the elements of the evaluation described above, a diagnosis of plagiocephaly and/or torticollis can be made. Once the diagnosis is made, the severity of the head shape and the root cause must be determined. Only after the diagnosis, severity, and root cause are determined can a treatment plan be formulated.

Guidelines for Treatment

In November 2016 the Congress of Neurological Surgeons (CNS) and the AANS/CNS Joint Section on Pediatric Neurosurgery published an article in Neurosurgery titled:

“Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for the Management of Patients with Positional Plagiocephaly: Executive Summary”

The article outlines their official guidelines for the treatment of plagiocephaly and torticollis and was endorsed by the American Academy of Pediatrics (AAP) as their official guidelines.

Below are the summaries of their findings, a summary of the article can be found here:

POSITIONING RECOMMENDATIONS

  • Repositioning is an effective treatment for deformational plagiocephaly. However, there is Class I evidence from a single study and Class II evidence from several studies that repositioning is inferior to physical therapy and the use of a helmet.

PHYSICAL THERAPY RECOMMENDATIONS

  • Physical therapy is recommended over repositioning education alone for reducing prevalence of infantile positional plagiocephaly in infants beginning at 7 weeks of age.
  • Physical therapy is as effective for the treatment of positional plagiocephaly and recommended over the use of a positioning pillow to ensure a safe sleeping environment, in compliance with American Academy of Pediatrics recommendations.

HELMET THERAPY RECOMMENDATIONS

  • Helmet therapy is recommended for infants with persistent moderate to severe plagiocephaly after a course of conservative treatment (repositioning and/or physical therapy).

Helmet therapy is recommended for infants with moderate to severe plagiocephaly presenting at an advanced age

Treatment

General rules of treatment:

  • All treatment should begin with education regarding positioning and instructions for tummy time.
  • No crib bumpers, blankets, pillows or fluffy toys in the crib or bassinet, until cleared by a pediatrician. This is usually at least 6 to 12 months of age. This is part of the American Academy of Pediatrics’ official recommendations.
  • Children with any form of torticollis or neck instability should proceed with physical therapy
  • Cranial helmets are reserved for:
    • Children with severe head shape deformity
    • Children with mild or moderate head shape deformity that has not responded to education, tummy time, and physical therapy
    • Children who are older at the time of their initial evaluation may be more likely to require a helmet due to the decreased amount of time available for tummy time and physical therapy.

Elements of the Comprehensive Treatment Plan at Plagiocare

  • Positioning and tummy time instructions and classes – Visit our Tummy Time & Positioning Page
  • Physical Therapy – Visit our Torticollis page
  • Helmet Therapy – Visit our Helmet Therapy page
  • Continued monitoring of progress of all aspects of treatment performed by our pediatricians who are all cranial specialists.

When to see a doctor

If you see any of the signs above, we recommend that you have your child evaluated as soon as possible, ideally at 7 weeks of age. Early intervention is key in the treatment of plagiocephaly. Because your child is growing and developing so quickly at this age, corrective measures can be taken to work with their normal growth and solve the problem naturally during this small window of opportunity.  Please contact us anytime at Plagiocare if you have any questions or would like to schedule an evaluation.

Doctor Centers vs “Helmet Companies”

Currently there are two main types of treatment centers for plagiocephaly.  There are “helmet companies” and doctor run centers such as Plagiocare. A “helmet company” is a center typically run by orthotists, occupational therapists, or physical therapists. The only treatment  performed in such centers is helmet therapy. “Helmet companies” are not legally allowed to provide a helmet without a doctor’s prescription. On the other hand, there are a few centers in the country that have physicians, physical therapists, and orthotists all under one roof. These centers can provide comprehensive care for the treatment of plagiocephaly, including diagnosis, prescription, home instruction, physical therapy, group classes, and helmet therapy if necessary.  The goal of a doctor run comprehensive center is to reduce the need for helmet therapy with early intervention and physical therapy.

Preparing for your appointment

Your initial evaluation at a doctor run plagiocephaly center may take an hour or longer. Typically, this will include a physical examination by doctors, physical therapists, and sometimes an orthotist. Initial evaluation at a “helmet company” will be performed by an orthotist, occupational therapist, or sometimes a physical therapist. In addition to a clinical exam, clinical measurements, some form of a 3-D light scan, and photographs may be taken. Your doctor or “helmet company” representative will then discuss the results of their findings and make recommendations for treatment.

Questions to ask your doctor or "helmet company"

Below are a list of questions you may want to ask your doctor or “helmet company” representative:

  • Do you provide physical therapy treatment sessions to help prevent the need for a helmet?
  • What criteria do you use to determine if my child will need a helmet?
  • Do you follow the same guidelines endorsed by the American Academy of Pediatrics when determining if a helmet is necessary? If not, why?
  • Approximately what percentage of the children that you see require a helmet?
  • Do you charge different fees if the helmet is not covered by insurance?
  • Are you able to provide helmets without a prescription from a doctor?
  • What percentage of children in your center require a second helmet? Will I have to pay for a second helmet if one is necessary?
  • If not, why are you not able to provide helmets without a prescription?

Questions your doctor may ask you

Just to prevent being alarmed at a doctor’s visit, below are some of the more common questions a parent may be asked by the doctor when their child is being evaluated for plagiocephaly.

  • Was your child born at the expected time? Earlier? Later?
  • Was it a normal vaginal delivery or cesarean section?
  • Did your child spend any time in the NICU?
  • What was your child’s head shape at birth?
  • Do you notice any changes in the shape of the head?
  • Do you notice any preference for head positioning? Head to the left or right?
  • Has your child been diagnosed with torticollis?
  • Is your child meeting all milestones?
  • Any developmental delays?
  • Eating and sleeping OK?
  • Have you been practicing “safe to sleep”?
  • Have you been practicing tummy time?

Home Treatment

DO NOT STOP FOLLOWING THE SAFE TO SLEEP CAMPAIGN INSTRUCTIONS UNTIL YOU ARE INSTRUCTED BY YOUR PEDIATRICIAN.

Although one of the side effects of back sleeping is plagiocephaly, it is much more important to follow the “Back to Sleep” recommendations to prevent SIDS (sudden infant death syndrome). We can fix positional plagiocephaly. It is important that you are instructed on proper positioning for your child not only when sleeping but during the day while in carriages, car seats, etc.  It is also important that you are taught proper tummy time exercises and perform them on a regular basis. These practices alone may help to prevent the need for a helmet.

For more information please visit our Tummy Time & Positioning page.

Timeline of Treatment

The timeline will depend on when your child is referred to a specialist. However, there are a few key times to keep in mind.

  • 7 Weeks of age – The ideal time to start physical therapy for torticollis, when indicated.
  • 5-6 months of age – The ideal time for helmet therapy, when indicated.
  • After 6 months of age – The time at which helmet therapy may take longer and have less of an effect.

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