Cranial Deformation and Craniosynostosis


Concern about abnormal head shape in infants is common. Infants born prematurely are at higher risk for cranial deformation (often dubbed “positional plagiocephaly”) than those born at term, though it occurs in about 20% of the latter and has become more common since the American Academy of Pediatrics instituted the “Back to Sleep” campaign in 1994. [Rogers: 2011] [Rogers: 2011] [Task: 2000]
Cranial deformation occurs in response to external forces either in utero or after birth, and sutures close normally. Craniosynostosis, involving premature closure of 1 or more of the cranial sutures, is far less common for both preterm and term infants but can look similar to some deformational abnormalities. Craniosynostosis is estimated to occur in 1:2500 infants. It is thought to be caused by combinations of genetics, bone, and epigenetic factors, with a small subset linked to a genetic syndrome. Maternal thyroid disease during pregnancy or prenatal use of clomiphene citrate, a fertility medication, is associated with increased risk of craniosynostosis.

ICD-10 Coding

Q 67.3, Plagiocephaly (can result from either craniosynostosis or external forces [nonsynostotic plagiocephaly])
  • Congenital positional plagiocephaly
  • Positional plagiocephaly
  • Positional plagiocephaly, congenital
Q 75.0, Craniosynostosis (includes scaphocephaly, trigonocephaly, brachycephaly, turribrachycephaly, oxycephaly, imperfect skull fusion)
  • Complex craniosynostosis
  • Craniosynostosis syndrome
  • Simple craniosynostosis

Pearls and Alerts

Early referral of craniosynostosis is critical
Most of these are evident at birth or within the first 2-3 weeks of life after swelling from birth subsides. Positional deformities usually become increasingly evident in the weeks and months after birth and may have a normal appearance at birth.
Timing of fontanelle closure
Be aware that 10% close by 3 months, 90% by 18 months. “If the shape is normal, ignore the fontanelle.” [Kestle: 2020] This means you do not need to refer for evaluation of early fontanelle closure unless head shape is abnormal.
Ridged sutures
If the overall shape is normal but there are ridges on the sutures, these do not need referrals.
Radiographs are not advised. Consultation with pediatric plastic surgery and/or pediatric neurosurgery prior to ordering CT imaging is prudent.
Prevention of deformation
Provide anticipatory guidance to parents of newborns about how to prevent deformation. In addition to routine safe sleep recommendations, encourage positioning the infant’s head to alternating sides during sleep. Stimulate the baby from all directions by arranging toys around the infant and approaching the infant from different sides. Encourage “tummy time” when awake and avoid extended time in bouncers and car seats.

Differentiating Deformation from Synostosis

Differentiating craniosynostosis from deformational abnormalities requires a thorough history and physical of the infant. It also may require imaging. Since many infants will have deformational cranial abnormalities, the decision to image the infant using head computerized tomography (CT) may be best left to the specialist, perhaps reducing needless radiation exposure. [Siddiqi: 2020]

Normal Head Shape with Sutures

Cranial Sutures

Differentiating Craniosynostosis from Deformational Abnormalities

This table provides some basic characteristics that differentiate these 2 cranial abnormalities. [Looman: 2012] [Ursitti: 2011] [Nield: 2007])
Cranial Deformation vs. Craniosynostosis

Evaluation of Cranial Deformation

Flattening occurs when cranial expansion and growth are consistently resisted in one area of the skull by an external force. [Rogers: 2011] [Rogers: 2011] Limited independent head mobility in very young infants or those with congenital muscular torticollis contribute to the flattening. Growth of the cranium is most rapid in the first few months of life, and treatment is most effective in that time frame. Premature infants have more limited head mobility for a longer period of time than full-term infants, which may explain their higher frequency of deformational cranial abnormalities. This brief video reviews how to evaluate for torticollis: Torticollis Quick Screening Guide by Lisa Hwang, DPT, Dsc Candidate. Also see, Premature Infant Follow-Up
Cranial deformation takes several forms:
A drawing showing deformational plagiocephaly, which is the flattening of one side of the posterior skull, creating an oblique or slanted head when viewed from the top
Deformational Plagiocephaly
Deformational plagiocephaly, or positional plagiocephaly, is the flattening of one side of the posterior skull, creating an oblique or slanted head when viewed from the top. Ipsilateral (along the same side) frontal bossing and asymmetric facial features result; however, the degree of flattening of the occipital skull is more pronounced and results in a parallelogram shape when viewed from above the infant’s head. The ear may be positioned more anteriorly on the side of the flattening.
Deformational Brachycephaly
Deformational brachycephaly is the symmetric flattening of the occipital skull with compensatory bi-parietal widening, giving the appearance of a large head when viewed from the front. Some degree of asymmetry is commonly seen. These infants may also have a posterior protrusion at the top of the head. When looking at the infant skull from the side, the skull appears to slope downward towards the anterior portion of the head. This is called “turricephaly” or a “tall head.” There can be a combination of deformational brachycephaly and plagiocephaly, resulting in some asymmetry.
Deformational Scaphocephaly
Deformational scaphocephaly (also known as dolichocephaly) is an uncommon variant seen in preterm infants who have been positioned on their sides, resulting in flattening of the sides of the head and compensatory increase in the anterior posterior dimension of the cranium. Preventive measures instituted in NICUs have decreased the incidence of this.
Anthropomorphic measurements can be obtained, and there are age-matched normative values for the cephalic index (CI), which is the maximum width of the head divided by the anteroposterior length and the plagiocephaly index which compares the differences between the two oblique diameters. However, the interpretation of normal is culturally based and may not affect the decision to treat. The author, Rogers, suggests the strongest indication to treat is the parent’s opinion of deformity. [Rogers: 2011] [Rogers: 2011]
Common Head Shape Measurements
Cephalic Index = BPD/OFD x 100
  • Normocephalic or plagiocephaly: 76-90%
  • Brachycephaly: >90%
  • Dolichocephaly: <76%
Plagiocephaly Index (aka Oblique Diameter Difference) = [X – Y] (in mm)
  • Normal: 0-4 mm
  • Mild: 5-9 mm
  • Moderate: 10-15 mm
  • Severe: >15 mm

Management of Cranial Deformation

Treatment for positional deformation is repositioning and helmets. Evidence does not support the use of helmets in moderate-severely affected infants ages 5-6 months. A 2016 randomized controlled trial observed equal improvements in head shape in follow-up of infants who were helmeted vs. natural course; however, the study excluded premature infants <35 weeks and those with torticollis or other deformities. [van: 2014] Helmets may not be covered by insurance; coverage improves if there is also torticollis. Based on the timing of fontanelle closures, helmeting is most likely to be effective if initiated by 4 months of age, but it may not improve outcomes.
Initial treatment considerations include: [Siddiqi: 2020]
  • Mechanical adjustments and exercises: These can be done at home. They include positioning the infant’s rounded side of the head against the mattress and changing the crib’s position so the infant would need to look away from the flattened side to see people enter the room. [Laughlin: 2011] [Morris: 2016] Some specialists advise placing a wedge or rolled up blanket under the torso on 1 side to appropriately position the child. Repositioning only works prior to the infant acquiring independent head control, which typically occurs by 4 months of age, corrected. Within 2-3 months, most infants will show improvement. [Task: 2000] This brief video demonstrates holding positions for parents to help stretch the baby’s neck muscles: Torticollis Holding Positions -COA PTOT.
  • Physical therapy addresses congenital muscular torticollis (CMT) and positional preferences. A physical therapist can teach parents to address congenital muscular torticollis (CMT) and positional preferences with 3 repetitions of stretches performed several times daily, such as at each diaper change. [Laughlin: 2011] The stretches usually involve placing one hand on the child’s upper chest while the other gently moves the chin until it touches the shoulder. This is held for 10 seconds and then repeated for the other side. [Laughlin: 2011] This short video demonstrates exercises that parents can do at home to augment physical therapy for torticollis: How to Treat Torticollis - DadLabs Video.
Consider referral to a pediatric neurosurgeon or plastic surgeon or to a craniofacial specialist if improvement does not improve by 4 months of age. Treatments that might then be considered are: [Laughlin: 2011]
  • Sleep orthotic or cradle devices position the infant’s occiput on a concave rather than a flat surface. This redistributes the surface pressure on the occiput but maintains the infant in the supine position. This approach only works prior to 3-4 months of age corrected, when infants develop sufficient head control to defeat its purpose.
  • Infant in car seat with a helmet on to help manage head shape
    Helmet therapy
    Helmet therapy is an orthotic that is typically used for moderate to severe deformational flattening. [Rogers: 2011] [Rogers: 2011] Several principles are important:
    • Helmets would only have the potential to be effective when there is remaining skull growth.
    • Younger children correct quicker than older children, consistent with the rate of growth of the cranium.
    • Helmets do not apply pressure to the cranium but rather have foam selectively cut away from the area in which growth is desired. They do not mold or squeeze the cranium.
    • A skilled orthotist is needed to monitor the growth of the infant’s head and the fit of the helmet.
Prevention includes:
  • Preventive counseling by 4 weeks of age: Provide information about tummy time while the infant is awake and observed (at least 30-60 minutes) and alternating head position at night during sleep. Encourage full, symmetric, unrestricted movements while awake. [Morris: 2016] Too much time in safety seats or swings should be discouraged. [Rogers: 2011]

Evaluation of Craniosynostosis

Head shape is dependent on which sutures are fused.
Sagittal synostosis is the most common type of craniosynostosis, early closure of the suture running along the top of the head, results in an elongated head shape (scaphocephaly), which can appear bullet-shaped posteriorly and downward sloping.
Sagittal craniosyntosis
Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities
Coronal synostosis is when one of the coronal sutures running from the ear to the sagittal bone closes early, and anterior plagiocephaly develops on the side of the early closure. When coronal synostosis occurs, the contralateral frontal bone protrudes, orbits appear asymmetric, and the eye on the affected side appears more open (“Harlequin eye”). The bony part of the nose may be pulled toward the fused suture, and the nasal tip may deviate away from it.
Coronal craniosyntosis
Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities
Bicoronal synostosis results in turribrachycephaly with a broad, tall appearance to the head. Bicoronal synostosis is more common in syndromic conditions that present with other atypical physical findings likely to be present, e.g., Crouzon, Apert, and Pfeiffer syndromes.
Metopic synostosis with trigonocephaly is rare with early fusion of the metopic suture, which runs from the top of the nose mid-forehead to the sagittal suture, leading to a trigonocephalic or triangular shape with a mid-forehead prominence. Hypotelorism may be present. Less severe “mild trigonocephaly” with mildly wider back compared to front of the head may not require surgical intervention. A ridge over the metopic suture without trigonocephaly does not require imaging or surgical intervention.
Lambdoid synostosis, the rarest type of synostosis, occurs in the lambdoid sutures at the back of the head. Early closure of one of these results in flattening on the back on the ipsilateral side, and the ear on that side may be pulled back or down. A mastoid bony bulge may be present on the affected side. The forehead will likely appear normal. It may appear trapezoidal when viewed from the back.
Complex synostosis involves multiple sutures and is quite rare and more likely associated with a syndrome.
While many cases are obvious at birth or within the first few weeks of life, more subtle cases may be difficult to identify. Declining occipital-frontal circumference (OFC) percentiles over time may provide a clue, as well as sleep apnea, hydrocephalus, speech or developmental delays, headaches, vomiting, and papilledema may be present if there is increased intracranial pressure (ICP). Use OFC charts (Head Circumference-for-Age Percentiles: Boys, Birth to 36 Months (CDC) (PDF Document 48 KB), Head Circumference-for-Age Percentiles: Girls, Birth to 36 Months (CDC) (PDF Document 45 KB)) or online OFC calculator (Infant Growth Chart Calculator: CDC 0-3 Years-Head Circumference For Age Percentile) for ages 0-3 to evaluate the growth of the infant’s head diameter over time. See Resources, below, for international OFC charts ages 0-5.


Radiographs are not advised. A CT may be performed to best evaluate suture abnormalities. Low-radiation CT imaging protocols are gaining traction to greatly reduce the risk of developing head cancers later in life. Consultation with pediatric plastic surgery and/or pediatric neurosurgery prior to ordering imaging is prudent. [Siddiqi: 2020]

Management of Craniosynostosis

Once swelling from birth has subsided in the first week or 2 of life, most cases of synostosis are readily identifiable. Early referral to pediatric plastic surgery or pediatric neurosurgery within the first month of life is advised. In some centers, a synostosis clinic may be available. Pediatric neurosurgery and pediatric plastic surgery often collaborate on care for these infants. Contacting the specialists with photos or via video may be useful as well. [Siddiqi: 2020]
When surgery is indicated, operating as early as 2-3 months of age (endoscopic-assisted strip craniectomy) may be recommended; however, the actual timing of surgery will vary depending on which suture is affected, the age at presentation, and the recommended treatment plan. [Naran: 2017] Open cranial vault surgery by 9-12 months is optimal due to the relative plasticity of the cranial vault that hardens significantly by 15 months of age. The expert recommendation for early surgical intervention is based on limited evidence; later surgical intervention has not been shown to improve outcomes, and complications are lowest in the earlier surgery groups. Earlier surgical interventions are associated with better neurodevelopmental outcomes, shorter operative times, shorter hospitalizations, reduced blood loss, and reduced swelling. [Mandela: 2019] [Bellew: 2019]
Treatment promotes a more typical head shape to protect the development of the orbits, provide adequate space for brain growth, and manage intracranial pressure. [Morris: 2016] Multiple studies have investigated the impact of abnormal head shape on children’s emotional well-being; however, there is a lack of high-quality evidence regarding this outcome. [Chummun: 2016] Elevated ICP is present in about 14-24% of children with isolated synostosis and can correlate with lower developmental outcomes; if multiple sutures are present, the risk increases. [Naran: 2017]
With the endoscopic-assisted strip craniectomy approach, serial helmeting is performed post-operatively for 6-12 months. Continue to monitor children post-operatively for symptoms and signs of increased ICP, developmental or behavioral concerns. Provide routine monitoring for problems with dentition, hearing, or vision and refer as indicated. Follow up within a multidisciplinary craniosynostosis program is advised, when available. [Siddiqi: 2020]


These specialists often work together to manage head shape problems in babies.
Pediatric Plastic Surgery: Provides expertise in evaluation of head shape deformations and management of injuries sustained during the NICU hospitalization. Pediatric Plastic Surgery (see MT providers [1]).
Orthotist: A person skilled in providing supportive equipment for a desired functional outcome. In the case of cranial deformation abnormalities, this individual makes the helmet and closely monitors its function throughout its use on the infant’s head to ensure the best growth and prevent skin sores and discomfort for the infant.
Pediatric Neurosurgery: Provide evaluations, recommendations, and management for nervous system problems and severe or persistent cranial deformations. Pediatric Neurosurgery (see MT providers [2]).
Craniosynostosis Program: Multidisciplinary evaluation, treatment, and follow-up program.
Physical Therapy: Refer to help treat torticollis associated with plagiocephaly. Physical Therapy (see MT providers [44]).
Early Intervention: Referral to early intervention can be helpful as up to half of infants with craniosynostosis will have developmental and/or speech delays. Risk factors include prematurity and low birth weight. Early Intervention for Children with Disabilities/Delays (see MT providers [30]).
Pediatric ENT: Refer if a submucous cleft is suspected; this is a fairly common comorbidity in non-syndromic synostosis. Pediatric Otolaryngology (see MT providers [5]).
Pediatric Dentistry: Refer to manage specialized dental issues. Pediatric Dentistry (see MT providers [52]).


Information & Support

For Parents and Patients

Craniosynostosis and Positional Plagiocephaly Support (CAPPS)
CAPPS — which stands for Craniosynostosis and Positional Plagiocephaly Support — is the pioneer and leader in supporting and educating families diagnosed with Craniosynostosis and/or Positional Plagiocephaly. The site includes a provider directory.

Practice Guidelines

Dias MS, Samson T, Rizk EB, Governale LS, Richtsmeier JT.
Identifying the Misshapen Head: Craniosynostosis and Related Disorders.
Pediatrics. 2020;146(3). PubMed abstract
The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. The intent is to improve pediatric care providers’ recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes; American Academy of Pediatrics.


Head Circumference-for-Age Percentiles: Boys, Birth to 36 Months (CDC) (PDF Document 48 KB)
This printable reference page provides an extended growth chart for head circumference for boys; Centers for Disease Control.

Head Circumference-for-Age Percentiles: Girls, Birth to 36 Months (CDC) (PDF Document 45 KB)
This printable reference page provides an extended growth chart for head circumference for girls; Centers for Disease Control.

Head circumference-for-age BOYS Birth to 5 years (percentiles) (WHO) (PDF Document 47 KB)
Chart to graph age of child vs occipital frontal circumference (OFC) of the child's head, internationally standardized; World Health Organization.

Head circumference-for-age BOYS Birth to 5 years (percentiles) (WHO) (PDF Document 47 KB)
Chart to graph age of child vs occipital frontal circumference (OFC) of the child's head, internationally standardized; World Health Organization.

Infant Growth Chart Calculator: CDC 0-3 Years-Head Circumference For Age Percentile
A calculator for OFC for boys and girls that shows the head circumference percentile based on age when compared to other infants. Requires the baby's date of birth, date of examination, gender, and OFC in either cm or in.

Services for Patients & Families in Montana (MT)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: May 2020; last update/revision: August 2020
Current Authors and Reviewers:
Author: Jennifer Goldman-Luthy, MD, MRP, FAAP
Reviewer: Faizi A. Siddiqi, MD FACS
Authoring history
2014: update: Sarah Winter, MDA
2014: first version: Jennifer Goldman-Luthy, MD, MRP, FAAPA
AAuthor; CAContributing Author; SASenior Author; RReviewer

Page Bibliography

Bellew M, Mandela RJ, Chumas PD.
Impact of age at surgery on neurodevelopmental outcomes in sagittal synostosis.
J Neurosurg Pediatr. 2019:1-8. PubMed abstract
A study of whether age at surgery has an impact on later neurodevelopmental outcomes for children with sagittal synostosis

Chummun S, McLean NR, Flapper WJ, David DJ.
The Management of Nonsyndromic, Isolated Sagittal Synostosis.
J Craniofac Surg. 2016;27(2):299-304. PubMed abstract
This systematic review assessed cranial index and neuropsychological outcome following surgery for isolated, nonsyndromic sagittal synostosis.

Kestle J.
ECHO presentation.
Dr. John Kestle, ECHO presentation 4/8/2020.

Laughlin J, Luerssen TG, Dias MS.
Prevention and management of positional skull deformities in infants.
Pediatrics. 2011;128(6):1236-41. PubMed abstract / Full Text
This AAP practice guideline was retired in 2018.

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):242-50; quiz 251-3. PubMed abstract / Full Text

Mandela R, Bellew M, Chumas P, Nash H.
Impact of surgery timing for craniosynostosis on neurodevelopmental outcomes: a systematic review.
J Neurosurg Pediatr. 2019;23(4):442-454. PubMed abstract
This systematic review summarizes and assesses evidence on whether there is an optimal age for surgery in terms of neurodevelopmental outcomes.

Morris LM.
Nonsyndromic Craniosynostosis and Deformational Head Shape Disorders.
Facial Plast Surg Clin North Am. 2016;24(4):517-530. PubMed abstract
This article provides an overview of etiology, epidemiology, pathology, diagnosis, and treatment of nonsyndromic craniosynostosis, indications for surgical intervention and management options. Deformational plagiocephaly is also presented with treatment options including repositioning, physical therapy, and helmet therapy.

Naran S, Miller M, Shakir S, Ware B, Camison L, Ford M, Goldstein J, Losee JE.
Nonsyndromic Craniosynostosis and Associated Abnormal Speech and Language Development.
Plast Reconstr Surg. 2017;140(1):62e-69e. PubMed abstract
The article details language acquisition and speech development in children with nonsyndromic craniosynostosis.

Nield LS, Brunner MD, Kamat D.
The infant with a misshapen head.
Clin Pediatr (Phila). 2007;46(4):292-8. PubMed abstract

Rogers GF.
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part I: terminology, diagnosis, and etiopathogenesis.
J Craniofac Surg. 2011;22(1):9-16. PubMed abstract

Rogers GF.
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part II: prevention and treatment.
J Craniofac Surg. 2011;22(1):17-23. PubMed abstract

Siddiqi F, Kestle J.
Specialty Recommendations in Primary Care.
Pediatrics TeleECHO Spring 2020 Module. 2020; April 8, 2020. Salt Lake City: University of Utah;

Task Force on Infant Sleep Position and Sudden Infant Death Syndrome.
Changing concepts of sudden infant death syndrome: implications for infant sleeping environment and sleep position. American Academy of Pediatrics. Task Force on Infant Sleep Position and Sudden Infant Death Syndrome.
Pediatrics. 2000;105(3 Pt 1):650-6. PubMed abstract / Full Text

Ursitti F, Fadda T, Papetti L, Pagnoni M, Nicita F, Iannetti G, Spalice A.
Evaluation and management of nonsyndromic craniosynostosis.
Acta Paediatr. 2011;100(9):1185-94. PubMed abstract / Full Text

van Wijk RM, van Vlimmeren LA, Groothuis-Oudshoorn CG, Van der Ploeg CP, Ijzerman MJ, Boere-Boonekamp MM.
Helmet therapy in infants with positional skull deformation: randomised controlled trial.
BMJ. 2014;348:g2741. PubMed abstract / Full Text