Pediatric Annals

Feature Article 

Safe Sleep, Plagiocephaly, and Brachycephaly: Assessment, Risks, Treatment, and When to Refer

Judith M. Marshall, MS, APRN; Farooq Shahzad, MBBS

Abstract

The Safe to Sleep campaign started in 1994, reducing the risk of sudden infant death syndrome (SIDS) by 40% to 60%. However, an undesirable consequence has been a 400% to 600% increase in positional head deformities. We review the risks for positional plagiocephaly or brachycephaly, treatment modalities, and when to refer. Differential diagnoses for non-positional deformities are discussed. Risks for positional head deformities include prenatal, perinatal and postnatal factors. These include torticollis, inadequate tummy time, abnormal intrauterine positioning, premature or postmature birth, prolonged labor, complex medical conditions, prolonged hospitalizations, developmental delay, and use of supportive or convenience devices. Recommended treatment involves repositioning techniques or physical therapy with or without helmet use. Early referral to physical therapy or a head shape program insures better outcomes for full correction of the deformity. The severity of residual deformities is directly related to the age at which the child is referred. [Pediatr Ann. 2020;49(10):e440–e447.]

Abstract

The Safe to Sleep campaign started in 1994, reducing the risk of sudden infant death syndrome (SIDS) by 40% to 60%. However, an undesirable consequence has been a 400% to 600% increase in positional head deformities. We review the risks for positional plagiocephaly or brachycephaly, treatment modalities, and when to refer. Differential diagnoses for non-positional deformities are discussed. Risks for positional head deformities include prenatal, perinatal and postnatal factors. These include torticollis, inadequate tummy time, abnormal intrauterine positioning, premature or postmature birth, prolonged labor, complex medical conditions, prolonged hospitalizations, developmental delay, and use of supportive or convenience devices. Recommended treatment involves repositioning techniques or physical therapy with or without helmet use. Early referral to physical therapy or a head shape program insures better outcomes for full correction of the deformity. The severity of residual deformities is directly related to the age at which the child is referred. [Pediatr Ann. 2020;49(10):e440–e447.]

The Safe to Sleep campaign (formerly called Back to Sleep program) was launched in 1994 based on research and a consensus by the American Academy of Pediatrics Task Force on Infant Positioning and SIDS (based on research published in 19921) to reduce the risk of sudden infant death syndrome (SIDS). This program promotes placing infants on their backs during sleep.1 Since the program's inception, the incidence of SIDS has been reduced by 40% to 60%.2 An undesirable consequence of increased supine positioning has been a 400% to 600% increase in positional plagiocephaly and brachycephaly. Plagiocephaly is flatness on either side of the occiput, and brachycephaly is flatness across the entire occiput (Figure 1). Because of parental concerns for placing their child in the prone position while sleeping, a lack of “tummy time,” (ie, time spent in the prone position) is the most likely cause of abnormal head shape.1 This article presents an illustrative case and guide for providers in assessment, treatment, and referral criteria for an infant with nonsynostotic plagiocephaly and brachycephaly.

Positional cranial deformities. AP, anterior/posterior. Reprinted from Marshall15 with permission.

Figure 1.

Positional cranial deformities. AP, anterior/posterior. Reprinted from Marshall15 with permission.

The most important part of the initial assessment for an abnormal head shape is to evaluate for craniosynostosis. Craniosynostosis is the premature fusion of the cranial sutures. The diagnosis can be made on clinical examination in most cases. If the diagnosis is still in question, an ultrasound of the suture in question or a craniofacial computed tomography scan can be performed. Children with craniosynostosis should promptly be referred to a craniofacial plastic surgeon.

The parents' greatest concern is that the abnormal head shape portends a brain abnormality. Frequently, parents blame themselves for the head flattening. In nonsynostotic plagiocephaly or brachycephaly, it is important to reassure the parents that the brain is completely normal. In addition, identifying risk factors for plagiocephaly puts the parent at ease that their child was at a higher risk for developing a head shape abnormality. Once craniosynostosis is ruled out and positional plagiocephaly is diagnosed, the provider should promptly refer the child to a treatment program.

Illustrative Case

A 5.5-month-old male infant presents with parental concern for flatness at the back of his head. The parents have been laying him down to sleep on his back as recommended and have planned “tummy time” as well. The patient was born at 39 weeks gestation by spontaneous vaginal delivery to a 30-year primigravida mother after an uncomplicated pregnancy. His birth weight was 5 lbs. 15 ounces and his Apgar scores were 9 and 9 at 1 and 5 minutes of age, respectively. Since birth, his mother has been breast-feeding and supplementing feeds with formula to enhance weight gain and overall growth. His weight gain and growth have been on target and he has achieved his developmental milestones appropriately. He has a grade II systolic heart murmur secondary to a small muscular ventricular septal defect diagnosed by a pediatric cardiologist and echocardiography was performed. He was also seen by a pediatric neurosurgeon at age 5 months because of concern about head shape. Skull radiographs demonstrated open sutures and he was diagnosed with positional plagiocephaly. His grandfather is a pediatrician who is familiar with plagiocephaly. Upon examination, the grandfather noted mild torticollis in addition to the abnormal head shape and recommended physical therapy and consideration for helmet therapy. The infant underwent a comprehensive program of physical therapy that included tummy time exercises in the management of his torticollis. His parents and grandparents were actively involved and were satisfied with the results of this program.

Natural Progression of Cranial Vault Development

The cranial vault expands due to the outward force exerted by the growing brain. The tensile force pushing the cranial bones apart stimulates bone formation at the cranial sutures. Bone growth occurs continuously.3

The muscles attached to the posterior part of the skull also exert force on the cranial vault. These muscles (suboccipital, splenius capitis, trapezius, and the sternocleidomastoid muscle) (Figure 2) get stronger as the baby spends more time lifting up the head. They all act to round out the occipital bone. In congenital muscular torticollis, these muscles are tighter or contracted on one side. This leads to an imbalance of muscular forces on the cranial vault, resulting in skull and facial skeletal abnormalities.

Paraspinal muscles.

Figure 2.

Paraspinal muscles.

Development of Plagiocephaly and Brachycephaly

If a child spends a greater amount of time in the supine rather than the prone position, the growing brain is unable to exert force against the external pressure on the back of the head. Another effect of decreased tummy time is that the muscles of the neck and back are unable to strengthen and thus do not exert the force needed to round out the occiput (Figure 2). There are a number of risk factors that cause weakened muscles and encourage supine positioning. Some of these risk factors are modifiable, and some are not.

Risk Factors

Risk factors are identified through a careful and thorough prenatal and birth history and physical examination of the child. Many of the risk factors influence each other and, therefore, they should all be considered together (Figure 3).

Risk factors. Reprinted from Marshall15 with permission.

Figure 3.

Risk factors. Reprinted from Marshall15 with permission.

Inadequate tummy time. Inadequate time spent in the prone position is a critical risk factor for plagiocephaly and brachycephaly for reasons outlined previously.

Torticollis. Most children with positional plagiocephaly have torticollis with variable degrees of severity.5,6 Torticollis is the unilateral tightening of the sternocleidomastoid muscle. This forces the head to tilt in the direction of the tightness and turn to the opposite direction (Figure 4). As long as the cranial sutures are open, the head is malleable. The constant pressure on either side causes the occipital and occasionally the parietal bone to become flattened or misshapen. Thus, it is nearly impossible to keep the child's head in the proper position until the torticollis is corrected. Torticollis is a major risk factor for positional plagiocephaly. There are several causes of torticollis such as hypotonia, muscle weakness, and abnormal intrauterine positioning.

Baby with torticollis.

Figure 4.

Baby with torticollis.

Abnormal intrauterine positioning. Abnormal intrauterine fetal positions (eg, breech, transverse, and occiput posterior) can be detected on fetal ultrasound. These types of positions pose a risk for skull deformation as the mother's organs or pelvic bones exert pressure on the baby's head. An ultrasound may not detect intrauterine crowding, but mothers may report that the baby was “stuck.” Large birth weight or multiple fetuses are risk factors for intrauterine crowding. Hypotonia or muscle weakness caused by an underlying neurologic disorder often causes the baby to remain in one position throughout gestation.

Prolonged labor. Prolonged labor is defined as labor that lasts for 20 hours or longer in primiparous women or 14 hours in multiparous women. The same factors that cause abnormal intrauterine positioning, such as a large birth weight, multiple fetuses, and abnormal positioning, may cause prolonged labor. A small pelvis can result in a difficult passage of the fetus through the birth canal, resulting in prolonged labor. Labor may be prolonged by worry, stress, or fear, as well as use of pain medication. Spending hours in the birth canal is enough to misshape a malleable head or lead to torticollis. Suction, forceps, or other mechanical delivery devices used during prolonged labor may exert pressure on the skull, causing deformation.

Premature or post-due date birth. A premature baby may have muscle weakness, developmental delay, and certain medical concerns preventing prone positioning of the infant. Babies born past 40 weeks gestation are at risk for larger birth weight, abnormal intrauterine positioning, intrauterine crowding, and prolonged labor.

Complex medical conditions. Complex medical conditions, such as those requiring open-heart surgery, artificial airways (intubation or tracheostomy), and feeding tubes (nasogastric or gastrostomy), may preclude prone positioning. Conditions like gastrointestinal reflux or developmental dysplasia of the hip do not allow for sufficient tummy time. Even with excellent nursing care, physical therapy, and parental vigilance, these children may still need a cranial molding helmet.

Prolonged hospitalization. Prolonged hospitalization is often required for care of a child with complex medical conditions, and babies born premature might need long hospitalizations simply to grow. A prolonged hospitalization combined with several medical problems increases the risk for plagiocephaly and brachycephaly.

Developmental delay. Neuromuscular diseases, spinal disorders, and genetic syndromes may cause hypotonia or poor muscle development, which leads to motor developmental delays. This affects the child's ability to lie in the prone position. Prolonged hospitalization increases developmental delay, as the child lacks an appropriate environment to meet milestones.4

Supportive and convenience devices. There are many toys and play devices that are intended to “help” a child to walk, stand, jump, or sit up straight. Examples are bouncy seats, car seats, swings, and saucers. This list is not all inclusive. In fact, many of these devices actually hinder children from reaching their milestones because their muscles rely on the device to do the work for them. Sometimes the age of the child is not appropriate for the developmental milestone the equipment proposes to help attain. Children who are placed in these apparati for prolonged periods of time have poor or abnormal muscle development, leading to decreased core strength and inability to roll over to the prone position unassisted. Because these devices limit movement, they add an additional risk of constant pressure to the back and sides of the head. There are instances when this equipment may be appropriate, such as when the parent needs both hands to complete a task or under the strict guidance of a physical therapist. The child should always be monitored while using these devices, and they should not be used longer than 10 to 15 minutes at a time.

Assessment

The primary care provider will perform a basic risk assessment (Table 1) and physical examination. A determination can then be made about when and where to refer. In addition to a risk factor assessment, the provider should inquire about a family history of head shape abnormalities such as large, flat, or scaphocephalic head shape or craniosynostosis.

Basic Risk Assessment for Plagiocephaly and Brachycephaly

Table 1.

Basic Risk Assessment for Plagiocephaly and Brachycephaly

The differential diagnosis of abnormal head shape includes positional plagiocephaly, craniosynostosis, macrocephaly, and hemifacial macrosomia. The most important part of the evaluation is determining whether the abnormal head shape is due to positional plagiocephaly or craniosynostosis (Figure 5). Infants with craniosynostosis should be referred to a craniofacial plastic surgeon or neurosurgeon. Both plagiocephaly and brachycephaly cause characteristic head morphologies. Positional plagiocephaly results in anterior movement of the ear, forehead, and zygoma on the flattened side, giving a characteristic parallelogram shape to the head. Hemifacial microsomia (Figure 6) is a condition in which the lower quadrant of the face is underdeveloped. At first glance, this can mimic the appearance of facial asymmetry seen with severe positional plagiocephaly. True cases of hemifacial microsomia should be referred to a pediatric plastic surgeon or otolaryngologist. In brachycephaly, the child will have bilateral frontal (forehead) bossing and increased biparietal width, which causes the appearance of protruding ears. The head may also appear macrocephalic (Figure 7). Macrocephaly is defined as head circumference that is greater than 2 standard deviations (SD) from age-appropriate normal. These patients should be referred to a neurosurgeon for evaluation of possible underlying brain abnormality.

Types of craniosynostosis. Reprinted from Marshall15 with permission.

Figure 5.

Types of craniosynostosis. Reprinted from Marshall15 with permission.

Hemifacial microsomia. Reprinted from Marshall15 with permission.

Figure 6.

Hemifacial microsomia. Reprinted from Marshall15 with permission.

Macrocephaly.

Figure 7.

Macrocephaly.

A patient with any of these more concerning conditions may be referred to a geneticist as there may be an underlying chromosomal abnormality.

Children with positional plagiocephaly or brachycephaly are best referred to a head shape clinic at the time of diagnosis or when the torticollis is identified. Head shape clinics use a team approach with a nurse practitioner or physician's assistant and/or plastic surgeon, a physical therapist, and an orthotist. Once referred to a head shape clinic, the assessment is made using a white light scanner or calipers to quantify the cranial vault asymmetry (plagiocephaly) or cranial index (brachycephaly). As shown in Figure 8, the indices are age dependent.

Cranial vault asymmetry index (CVAI) and cranial index (CI). A, diagonal A; A/P, anterior/posterior B, diagonal B; BiP, biparietal. Adapted from Wilbrand et al.14

Figure 8.

Cranial vault asymmetry index (CVAI) and cranial index (CI). A, diagonal A; A/P, anterior/posterior B, diagonal B; BiP, biparietal. Adapted from Wilbrand et al.14

Treatment

The provider should understand that the child will not simply “grow out of” plagiocephaly and brachycephaly. Treatment involves physical therapy and may also include cranial molding helmet therapy.5,7–9 As seen in Figure 8, physical therapy alone is most effective for mild and moderate cases. Physical therapy plus a helmet is most effective for moderate to severe cases. The goal is to prevent progression of a mild defect to moderate or severe defect.

Physical therapy or helmet therapy may not completely correct the facial asymmetry seen with plagiocephaly. Facial asymmetry improves in the toddler years, as the midface grows and elongates.

Correcting a head shape abnormality should not be discounted as merely a cosmetic issue. Differences in appearance predispose to poor self-esteem, bullying, withdrawal, and lack of confidence. Safety issues include improper fit of commercially available bicycle or sports helmets. Medical concerns include glasses that do not fit the shape of the head due to facial asymmetry. Data show that auditory processing disorders, mandibular asymmetry (affecting feeding and speech), and visual field effects can be associated with plagiocephaly.10,11

Physical Therapy

Treatment of torticollis alone often corrects or prevents the head deformity. Physical therapists teach the parents tummy time techniques and focus on strengthening core, neck, and back muscles. Strengthening all of the body muscles to develop proper posture and body alignment are treatments for developmental delay and muscle weakness seen with positional plagiocephaly.5,7–9

Tummy time is supervised time the child spends in the prone position. It is used to strengthen the back and neck muscles attached to the base of the skull. As these muscles develop, they pull against the skull bones at the nuchal ridge causing a rounding effect. If the back muscles are underdeveloped the child will resist the prone position. If the child is particularly weak, physical therapy should commence at short durations and frequent intervals. Eventually patients will get stronger, making prone positioning easier, and they will begin to enjoy this time. Parents should keep their child engaged during tummy time to decrease fatigue.

Parents occasionally oppose the concept of tummy time because their child dislikes it or easily tires. Physical therapists teach tummy time “equivalents,” which are exercises limiting the time a child spends on their back. These exercises are integrated into carrying the baby and play time. Tummy time or tummy time equivalents should encompass more than 50% of the daily awake time.2 See Figure 9 for examples of tummy time equivalents.

Examples of tummy time equivalents.. (A) Right tilt with head righting. (B) Prop on hip and lean forward. (C) Left tilt. (D) “Superman” carry. (E) Prone on lap. Reprinted from Marshall15 with permission.

Figure 9.

Examples of tummy time equivalents.. (A) Right tilt with head righting. (B) Prop on hip and lean forward. (C) Left tilt. (D) “Superman” carry. (E) Prone on lap. Reprinted from Marshall15 with permission.

Helmet Therapy

A cranial molding helmet may be beneficial in a number of situations. A complex medical condition preventing prone positioning is an indication for helmet use. In addition, if the defect is already moderate or severe, or the child has failed physical therapy alone, helmet therapy may be beneficial. Helmet therapy is not a replacement for physical therapy but is used in conjunction with physical therapy sessions. Helmets are typically worn for 4 to 6 months. The duration of treatment depends on the severity of the deformation and the age of the child. The older the child when starting helmet therapy, the longer they are likely to wear the helmet. Helmet therapy is ideally initiated at age 4 to 6 months.12,13

Early referral and initiation of physical therapy often proves to be successful. Physical therapy may not eliminate the need for a helmet in all cases but will minimize the use and increase the success of the helmet. For the severe cases, early determination for helmet use provides the best outcomes for the child.

Illustrative Case Outcome

The infant started physical therapy at age 6.25 months. The treatment lasted 2.5 months. The helmet was worn 23 hours per day. The patient's cephalic index changed from 98.5 (+5 SD) to 90.3 (+1.9 SD) and his cranial vault asymmetry index improved from 6.1 to 1.4 mm.

The patient initially presented with increased width-to-length ratio with bilateral parietal occipital flattening, right greater than left (cranial vault asymmetry reduced from 8 mm to 2 mm), left frontal flattening, bulged squamoids, and increased posterior head height.

At the end of treatment, he demonstrated increased occipital length and rounding with resolution to asymmetry. The appearance of posterior head height was decreased and the squamoid bulging blended (Figure 10).

Before and after photos. (A) Top vertex view (before). (B) Top vertex view (after). (C) Right lateral view (before). (D) Right lateral view (after).

Figure 10.

Before and after photos. (A) Top vertex view (before). (B) Top vertex view (after). (C) Right lateral view (before). (D) Right lateral view (after).

Fiscal Considerations

Helmets can be costly and always require a letter of medical necessity. Physical therapy addresses many developmental issues that have lasting effects on the child. Parents should be made aware of the financial responsibilities that come with physical and helmet therapy.

Limited Data

Research, articles, or data relating assessment, treatment plans, and outcomes of treatment of plagiocephaly or brachycephaly are limited. Of interest would be an article on long-term sequelae such as residual occipital flatness or asymmetry, both of the cranial vault as well as the facial structures.

Conclusion

By knowing the causes and treatment of positional plagiocephaly, primary care providers can more effectively manage this common condition. Education for the families should include not only tenets of the Safe to Sleep campaign, but the importance of tummy time and activities that will prevent head shape abnormalities.

References

  1. American Academy of PediatricsAAP Task Force on infant positioning and SIDS. Positioning and SIDS. Pediatrics. 1992;89(6 Pt 1):1120–1126. PMID:1503575
  2. The Canadian Foundation for the Study of Infant Deaths, Canadian Institute of Child Health, Canadian Paediatric Society, Health Canada. Positional plagiocephaly and sleep positioning: an update to the joint statement on sudden infant death syndrome. Paediatr Child Health. 2001;6(10):788–789. doi:10.1093/pch/6.10.788 [CrossRef]
  3. Jin SW, Sim KB, Kim SD. Development and growth of the normal cranial vault : an embryologic Review. J Korean Neurosurg Soc. 2016;59(3):192–196. doi:10.3340/jkns.2016.59.3.192 [CrossRef] PMID:27226848
  4. Majnemer A, Barr RG. Influence of supine sleep positioning on early motor milestone acquisition. Dev Med Child Neurol. 2005;47(6):370–376. doi:10.1017/S0012162205000733 [CrossRef] PMID:15934485
  5. Looman WS, Flannery AB. Evidence-based care of the child with deformational plagiocephaly, part I: assessment and diagnosis. J Pediatr Health Care. 2012;26(4):242–250. doi:10.1016/j.pedhc.2011.10.003 [CrossRef] PMID:22726709
  6. Stellwagen L, Hubbard E, Chambers C, Lyons Jones K. (2013). Torticollis, facial asymmetry and plagiocephaly in normal newborns. Arch Dis Child. 2008;93(10):827–831. doi:10.1136/adc.2007.124123 [CrossRef] PMID:18381343
  7. Flannery AB, Looman WS, Kemper K. Evidence-based care of the child with deformational plagiocephaly, part II: management. J Pediatr Health Care. 2012;26(5):320–331. doi:10.1016/j.pedhc.2011.10.002 [CrossRef] PMID:22920774
  8. Barabé DS. Is congenital muscular torticollis equally resolved in infants who initiate physical therapy intervention after 5 months of age in comparison to infants who initiate therapy before 5 months of age? Capstone Project. University of New Mexico; 2014. Accessed September 28, 2020. https://core.ac.uk/display/78933140
  9. van Vlimmeren LA, van der Graaf Y, Boere-Boonekamp MM, L'Hoir MP, Helders PJ, Engelbert RH. Effect of pediatric physical therapy on deformational plagiocephaly in children with positional preference: a randomized controlled trial. Arch Pediatr Adolesc Med. 2008;162(8):712–718. doi:10.1001/archpedi.162.8.712 [CrossRef] PMID:18678802
  10. Collett BR, Starr JR, Kartin D, et al. Development in toddlers with and without deformational plagiocephaly. Arch Pediatr Adolesc Med. 2011;165(7):653–658. doi:10.1001/archpediatrics.2011.92 [CrossRef] PMID:21727278
  11. Knight SJ, Anderson VA, Meara JG, et al. Early neurodevelopment in infants with deformational plagiocephaly. J Craniofac Surg. 2013;24(4):1225–1228. doi:10.1097/SCS.0b013e318299777e [CrossRef] PMID:23851774
  12. Tamber MS, Nikas D, Beier A, et al. Congress of Neurological Surgeons systematic review and evidence-based guideline on the role of cranial molding orthosis (helmet) therapy for patients with positional plagiocephaly. Neurosurgery. 2016;79(5):E632–E633. doi:10.1227/NEU.0000000000001430 [CrossRef]
  13. Kluba S, Kraut W, Reinert S, Krimmel M. What is the optimal time to start helmet therapy in positional plagiocephaly?Plast Reconstr Surg. 2011;128(2):492–498. doi:10.1097/PRS.0b013e31821b62d6 [CrossRef] PMID:21788840
  14. Wilbrand JF, Schmidtberg K, Bierther U, et al. Clinical classification of infant nonsynostotic cranial deformity. J Pediatr.2012;161(6):1120–1125. doi:10.1016/j.jpeds.2012.05.023 [CrossRef] PMID:22727872
  15. Marshall JM. (2017, October31). Plagiocephaly and brachycephaly: assessment, treatment, and when to refer. Child's Doctor Online CME Rounds: Ann & Robert H. Lurie Children's Hospital of Chicago. Accessed September 28, 2020. http://luriechildrens.peachnewmedia.com/store/seminar/seminar.php?seminar=102383

Basic Risk Assessment for Plagiocephaly and Brachycephaly

Criterion Answer Answer
Torticollis Yes No
Quantify tummy time (<50% of awake time) Yes No
Prematurity Yes No
Post-maturity Yes No
Large birth weight Yes No
Abnormal intrauterine positioning Yes No
Prolonged labor (>20 hours in primiparous and 14 hours in multiparous) Yes No
Delayed motor development Yes No
Hypotonia Yes No
Neuromuscular disease Yes No
Complex medical history Yes No
Cardiac surgery Yes No
Feeding tubes Yes No
Gastroesophageal reflux disease Yes No
Artificial airway Yes No
Breathing problems Yes No
Hip dysplasia Yes No
Use of supportive devices greater than 15 minutes at a time Yes No
Authors

Judith M. Marshall, MS, APRN, is an Advanced Practice Provider, Division of Genetics, Ann & Robert H. Lurie Children's Hospital of Chicago. Farooq Shahzad, MBBS, is a Plastic Surgeon, Division of Plastic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago.

Address correspondence to Judith M. Marshall, MS, APRN, Division of Genetics, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Avenue, Chicago, IL 60611; email: jmmarshall@luriechildrens.org.

Disclosure: The authors have no relevant financial relationships to disclose.

The authors thank Mary Kay McGuire, OTR/L (Cranial Technologies, Inc.), Timothy R. Littlefield, MS (Cranial Technologies, Inc.), Matthew Howard (NorthShore University), and Joseph R. Hageman, MD, (Comer Children's Hospital) for their contributions to this article.

10.3928/19382359-20200922-02

Sign up to receive

Journal E-contents