Congress of Neurological Surgeons (CNS) and the AANS/CNS Joint Section on Pediatric Neurosurgery
Joint Guidelines Committee of the American Association of Neurological Surgeons (AANS) and the Congress of Neurological Surgeons (CNS) and American Academy of Pediatrics (AAP)
Catherine Mazzola, MD1, Lissa C. Baird, MD2, David F. Bauer, MD3, Alexandra Beier, DO4, Susan Durham, MD5, Paul Klimo, Jr., MD6, Alexander Y. Lin, MD7, Catherine McClung-Smith, MD8, Laura Mitchell, MA9, Dimitrios Nikas, MD10, Mandeep S. Tamber, MD, PhD11, Rachana Tyagi, MD12, Ann Marie Flannery, MD13
- Goryeb Children's Hospital of Atlantic Health Systems, Morristown, New Jersey, USA
- Department of Neurological Surgery, Oregon Health and Science University, Portland, Oregon, USA
- Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA
- Division of Pediatric Neurosurgery, University of Florida Health Jacksonville, Jacksonville, Florida, USA
- Division of Neurosurgery, University of Vermont Medical Center, Burlington, Vermont, USA
- Semmes-Murphey Neurologic & Spine Institute; Department of Neurosurgery, University of Tennessee Health Science Center; Le Bonheur Children’s Hospital, Memphis, Tennessee, USA
- St. Louis Cleft-Craniofacial Center, SSM Health Cardinal Glennon Children's Hospital at Saint Louis University, Division of Plastic Surgery, Saint Louis University School of Medicine, St. Louis, Missouri, USA
- Department of Neurological Surgery, Palmetto Health University of South Carolina Medical Group, Columbia, South Carolina, USA
- Guidelines Department, Congress of Neurological Surgeons, Schaumburg, Illinois, USA
- Department of Neurosurgery, University of Illinois at Chicago, Chicago, Illinois, Advocate Children's Hospital, Oak Lawn, Illinois, USA
- Department of Pediatric Neurological Surgery, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA
- Department of Surgery, Division of Neurosurgery, Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA
- Kids Specialty Center, Women’s & Children’s Hospital, Lafayette, Louisiana, USA
Catherine Mazzola, MD
New Jersey Pediatric Neurosurgical Associates
Morristown, NJ, USA
No part of this article has been published or submitted for publication elsewhere.
Disclaimer of Liability
This clinical systematic review and evidence-based guideline was developed by a physician volunteer task force as an educational tool that reflects the current state of knowledge at the time of completion. The presentations are designed to provide an accurate review of the subject matter covered. This guideline is disseminated with the understanding that the recommendations by the authors and consultants who have collaborated in its development are not meant to replace the individualized care and treatment advice from a patient's physician(s). If medical advice or assistance is required, the services of a physician should be sought. The recommendations contained in this guideline may not be suitable for use in all circumstances. The choice to implement any particular recommendation contained in this guideline must be made by a managing physician in light of the situation in each particular patient and on the basis of existing resources.
Background: No evidence-based guidelines exist for imaging of patients with positional plagiocephaly.
Objective: To answer the question: Is imaging necessary for infants with positional plagiocephaly in order to make a diagnosis?
Methods: The National Library of Medicine Medline database and the Cochrane Library were queried using MeSH headings and keywords relevant to imaging as a means to diagnose plagiocephaly. Abstracts were reviewed and an evidentiary table was assembled summarizing the studies and the quality of evidence (Classes I-III). Based on the quality of the literature, a recommendation was rendered (Level I, II, or III).
Results: A total of 42 full-text articles selected for review. Of these, 10 were eliminated. There were 32 full-text manuscripts selected. There was no Class I evidence, but 2 Class II and 30 Class III studies were included. Three-dimensional cranial topographical imaging, ultrasound, skull x-rays, computed tomography, and magnetic resonance imaging were investigated.
Conclusion: Clinical examination is most often sufficient to diagnose plagiocephaly (quality, Class III; strength, Level III). Within limits of this systematic review, the evidence suggests that imaging is rarely necessary and should be reserved for cases where the clinical examination is equivocal. Many of the imaging studies were not designed to address the diagnostic utility of the imaging modality, and authors were actually assessing the utility of the imaging in longitudinal follow-up, not initial diagnosis. For this reason, some of the studies reviewed were downgraded in Level of Evidence. When needed, 3-dimensional cranial topographical photo, skull x-rays, or ultrasound imaging is almost always sufficient for definitive diagnosis. Computed tomography scanning should not be used to diagnose plagiocephaly, but it may be necessary to rule out craniosynostosis.
Short Title: Guideline for the Diagnosis of Patients with Positional Plagiocephaly: The Role of Imaging
Key Words: imaging; infants; diagnosis; plagiocephaly, non-synostotic; positional plagiocephaly
- Clinical examination is recommended for the diagnosis of plagiocephaly and imaging is rarely necessary, except in cases in which clinical diagnosis is equivocal.
Strength of recommendation: Level III—low clinical certainty
- In cases in which the clinical examination is equivocal, skull x-rays or ultrasound imaging of the suspect suture is recommended.
Strength of recommendation: Level II—moderate clinical certainty
- In cases in which the clinical examination is equivocal, surface imaging (computer-based topographical scans) or stereophotogrammetry is recommended for the assessment of infants with plagiocephaly without synostosis.
Strength of recommendation: Level III—low clinical certainty
- Only for infants in whom x-rays or ultrasound are non-diagnostic, a CT scan is recommended for definitive diagnosis.
Strength of recommendation: Level III—low clinical certainty
Infants may present with abnormal head shape any time after birth. The reason for referral is commonly “to rule out craniosynostosis.” The diagnosis of true craniosynostosis is important because this condition is amenable to surgical correction, whereas positional, posterior plagiocephaly without synostosis (PWS) is adequately treated with repositioning, physical therapy, or, in moderate to severe cases, a cranial molding helmet.1,2
It has been the experience of many craniofacial specialists, including those on the Plagiocephaly Task Force (hereinafter referred to as the “task force”), that most infants with plagiocephaly can be adequately diagnosed through a detailed clinical examination. Three dimensional (3-D) topographical scanning may be useful for diagnosis and baseline assessment of severity. In those rare cases in which the clinical examination was equivocal, skull x-rays or an ultrasound of the suture in question could be used to rule out craniosynostosis. Only if those radiological studies are equivocal, should a computed tomography (CT) scan of the head be performed.
The Congress of Neurological Surgeons (CNS) and the Section on Pediatric Neurosurgery initiated a systematic review of the literature and evidence-based guideline relevant to the management of positional plagiocephaly. Additional information about the methods utilized in this systematic review is provided below and within the introduction and methodology chapter of the guideline.
Potential Conflicts of Interest
All guideline task force members were required to disclose all potential conflicts of interest (COIs) prior to beginning work on the guideline, using the COI disclosure form of the Joint Guidelines Committee of the American Association of Neurological Surgeons (AANS) and the CNS. The CNS Guidelines Committee and the task force chair reviewed any disclosures and either approved or disapproved the nomination and participation on the task force. The CNS Guidelines Committee and guideline task force chair may approve nominations of task force members with possible conflicts and restrict the writing, reviewing, and/or voting privileges of that person to topics that are unrelated to the possible COIs.
The task force members collaborated with medical librarians to search the National Library of Medicine/PubMed database and the Cochrane Library for the period from 1966 to October 2014 using the MeSH subject headings and PubMed search strategies provided in Appendix A. Manual searches of bibliographies were also conducted.
Our searches resulted in 204 abstracts. The task force selected 42 full-text articles for review. Of these, 10 were rejected for not meeting inclusion criteria or for being off-topic (Figure 1). For example, some studies were not performed on human subjects, not limited to infants, or were mainly reporting study results of infants with craniosynostosis. Thirty-two articles were selected for systematic review (Table 2).
Rating Quality of Diagnostic Evidence
For diagnostic-type papers, evidence classification had definitions targeted toward diagnosis. The issues addressed by papers on diagnosis are related to the ability of the diagnostic test to successfully distinguish between patients who have and do not have a disease or pertinent finding. This speaks to the validity of the test and is illustrated in Table 1. Additional information regarding the hierarchy classification of evidence can be located here: https://www.cns.org/guidelines/guideline-procedures-policies/guideline-development-methodology.
Many of the imaging studies were not designed to address the diagnostic utility of the imaging modality, and authors were actually assessing the utility of the imaging in longitudinal follow-up, not initial diagnosis. For this reason, some of the studies reviewed were downgraded in Level of Evidence.
Clinical examination is recommended for the diagnosis of plagiocephaly and imaging is rarely necessary, except in cases in which clinical diagnosis is equivocal. Within the limits of this systematic review, we found that imaging is rarely necessary and should be reserved for when the clinical examination is equivocal. In a Class III, 2013 study done by Kuang and Bergquist, the effectiveness and safety of pediatric nurse practitioners for the diagnosis of infants with plagiocephaly was evaluated.3 They completed a retrospective review of the electronic medical records of 1228 infants seen in a craniofacial clinic from 2005 to 2011. The authors concluded that a nurse practitioner was able to effectively and safely diagnose and treat plagiocephaly without the need of imaging.3 However, in their study, it was the surgical team who ruled out craniosynostosis by clinical examination alone in 325 of the 590 infants that the nurse practitioners believed to have craniosynostosis.3 It was the craniofacial surgeons who made the correct diagnosis of plagiocephaly in most of the infants. The nurse practitioners in the Kuang study each had 12-20 years of prior experience working with the pediatric neurosurgeon on the craniofacial team.
In another report by Linz, 269 infants with abnormal head shape were evaluated clinically, resulting in 258 diagnosed with deformational plagiocephaly, and 8 were “definitely” diagnosed with craniosynostosis; in 3 infants, the diagnosis of plagiocephaly was suspected but could not be made definitively.4 All 269 had cranial ultrasounds and 8 had x-rays of the skull performed. Of the 261 infants with plagiocephaly, in 258 the correct diagnosis was made by clinical examination alone.4 Of the 3 infants whose diagnosis was uncertain and clinical examination could not definitely rule out craniosynostosis, ultrasounds did confirm open sutures. Skull x-rays were ordered for 8 infants suspected of having craniosynostosis, and in those 8 infants, craniosynostosis was confirmed on x-ray.4 This protocol, whereby skull x-rays were ordered only in equivocal cases, was followed by many authors.4-6
There are well-described cranial shape differences between infants with PWS and craniosynostosis of the lambdoid suture.3,4,7 From the posterior coronal view, infants with PWS had ipsilateral occipital-parietal flattening and contralateral occipital prominence but an essentially “normal” head shape from behind. However, in infants with true unilateral lambdoid synostosis, skull growth persists from either end of the fused suture, leading to ipsilateral occipital-mastoid bossing and contralateral parietal bossing, forming a diamond shape from behind that is wider posteriorly than anteriorly, with skull base tilt.4,7 From the aerial vertex view, in PWS there is posterior flattening with ipsilateral frontal prominence leading to an aerial parallelogram-shaped head. In patients with true unilateral lambdoid synostosis, frontal bossing is contralateral instead, though sometimes minimally prominent, which, when combined with occipital flattening on the synostotic side, forms an aerial-view trapezoid with ipsilateral ear pulled posteriorly.3,4,7
In a Class III, retrospective analysis of 287 infants seen at their craniofacial clinic, Hutchison et al reported that only 2% (n = 7) were referred for CT scanning and that clinical examination and head shape measurements were usually sufficient for diagnosis.8 Of those 7 infants in whom the diagnosis could not be made on clinical examination alone, 4 had confirmed craniosynostosis, 1 had an abnormal "extra" suture, and 2 had severe positional plagiocephaly without true craniosynostosis. In the Mulliken et al study, only 11/115 infants required CT scans.5 In most studies, CT scanning is not indicated for all infants with plagiocephaly, only those in whom the clinical diagnosis is not apparent and skull x-rays or other imaging is equivocal or non-diagnostic.5,6,8
Anthropometric measurements have been used as part of the clinical examination in several studies.6,9-11 In 1 Class III study, the repeatability and reliability of intra-rater measurements were found to be +/- 1 mm.9 A total of 14 measurements were recorded using spreading and sliding calipers and a linen measuring tape, at the initial assessment and at various intervals thereafter. Intra-rater and inter-rater caliper-based anthropometric measurement reliability was compared to measurements made from stereophotographs and were found to be accurate. There was low variability in the variance component analysis in the study done by Shaaf.10
3D Surface Imaging and/or Stereophotogrammetry
In cases in which the clinical examination is equivocal, surface imaging (computer based tomography scans) or stereophotogrammetry is recommended for the assessment of infants with plagiocephaly without synostosis.
In 2012, there was a prospective, non-randomized study done by Collett et al that utilized clinical assessment and cranial topographical scanning to follow infants with and without PWS.12 Even though the study was a prospective design, the lack of blinded or randomized comparison between the utility of topographical scanning versus independent clinical examination made this Class III data. Three-dimensional (3D) topographical scanning is not performed in the radiology department but is often done in the clinic or practice setting and does not expose the infant to radiation. Topographical scanning, as mentioned in several studies, usually involves a light source and recorder that utilizes data obtained from the reflection of light at various angles from the infant’s head to construct a computer-generated 3D image.12-18 There is no exposure to ionizing radiation. Although the Collett and Heike study demonstrated improvement in symmetries, there was no blinded or randomized comparison between the utility of topographical scanning versus independent clinical examination, and for this reason we downgraded this to Class III evidence.12 This was true in the Katzel, Thompson, and Moghaddam studies as well, making the overall recommendation Level III.13,17,18 Most authors found that 3D topogrammetry provided better objectivity, but often the significance, sensitivity, and specificities for diagnosis were not reported or compared to independent clinical observation for various reasons.
In a Class III study by Kluba et al, clinical examination and 3D stereophotogrammetry demonstrated equipoise in assessment of head shape asymmetry, but 3D topographical scanning was superior in the assessment of “ear shift” seen in positional plagiocephaly.14 Stereophotogrammetry involves multiple camera systems that provide 3D imagery.14,16,19 Three-dimensional cranial topography of infants with severe positional plagiocephaly was performed before and after cranial molding therapy.10,14,16,19 The cranial vault asymmetry index (CVAI) and ear shift were measured and statistically compared.14 Three craniofacial surgeons also evaluated the 3D stereophotogrammetry results independently and reported their assessment with a standard questionnaire. The results were compared with the three-dimensional, anthropometric measurements, and there existed a good correlation.10,14 Other authors have utilized 3D topographical scanning and stereophotogrammetry for the analysis of initial head shape and the clinical follow-up of infants with plagiocephaly, as well.15,16,19 Other groups have used digital photography to record, evaluate, and follow infants with plagiocephaly.11
Skull X-ray or Ultrasound
In cases in which the clinical examination is equivocal, a skull x-ray or ultrasound imaging of the suspect suture is recommended.
When the clinical examination is equivocal, skull x-rays or ultrasound imaging is recommended for definitive diagnosis.20-23 In well-done Class II studies with prospective clinical comparisons, Krimmel et al and Sze et al evaluated the value of high-resolution ultrasound (US) in the differential diagnosis of scaphocephaly and occipital plagiocephaly.20,23 In Krimmel’s study, radiological data from 54 infants under 12 months of age with plagiocephaly were presented. The 2 comparison groups were 47 infants with simple positional plagiocephaly and 7 infants with true craniosynostosis. Under the premise that the inconclusive US findings are regarded as false-positive and false-negative results, the US method had at least a sensitivity of 71.4% (95% confidence interval: 35.5%, 100%), a specificity of 95.7%, a positive predictive value of 71.4%, and a negative predictive value of 95.7%.20 In 96% (45/47) of infants with positional plagiocephaly, the ultrasound images did confirm patent sutures. In 4% (2/47) of the infants studied, the ultrasound was inconclusive. In 5 out of 7 infants with craniosynostosis, the ultrasound did show closed cranial sutures. In only 2 of the 7 infants with craniosynostosis, the ultrasound findings were inconclusive and were followed by a CT in 1 patient and a skull x-ray in the other. In conclusion, the authors felt that ultrasound effectively distinguished between open and closed sutures.20 Sze et al reported in 2003 that ultrasonography of the lambdoid sutures shows “excellent promise as a screening test of lambdoid sutural patency.”23 Their group prospectively evaluated ultrasound as a screening test of suture patency using CT as the gold standard. The reported mean sensitivity and specificity of cranial ultrasound in distinguishing a patent from fused suture by 3 blinded pediatric radiologists was 100% and 89%, respectively. This was a Class II study used to support ultrasound as a Level II recommendation as a study that can be used for infants in whom clinical diagnosis is uncertain. In a prospective Class III study done by Linz et al, 269 infants with plagiocephaly without synostosis (PWS, n = 261) and 8 infants with lambdoid synostosis were clinically examined to outline the specific clinical features of true positional deformity vs craniosynostosis.4 After clinical examination, cranial ultrasound of the sutures was performed. Ultrasound revealed either a lambdoid synostosis or a patent lambdoid suture in cases of PWS. In 96% (258/269) of PWS infants, clinical examination was able to confirm the diagnosis. Of note, in 3 infants who were initially diagnosed with PWS, a lambdoid synostosis was found on ultrasound. In all true lambdoid synostosis cases, ultrasound did support the clinical diagnosis. Their conclusion was that CT scan was not necessary in the diagnosis of true lambdoid synostosis and that there are quite distinctive clinical features apparent in infants with lambdoid synostosis when compared to infants with PWS. Additionally, the group concluded that ultrasonography done in infants ≤12 months can be used to confirm the diagnosis of synostosis.4 These findings have been supported by additional studies.22
Many authors followed protocols whereby infants with plagiocephaly were evaluated with skull x-rays, and only if the skull x-rays were non-diagnostic would CT scans be performed.6,21,24 In the study by David et al, skull x-rays done for 204 infants with posterior plagiocephaly showed 202 infants with patent sutures. Less than 1% (2/204) of the infants were found to have true synostosis. The authors concluded that clinical examination and skull x-rays were sufficient for diagnosis, and CT imaging should be reserved for only those patients in whom both the clinical exam and radiological skull x-ray results are equivocal.24
Only for infants in whom x-rays or ultrasound is non-diagnostic, a computed tomography scan is recommended for definitive diagnosis.
It is our opinion that rarely is CT ever needed for the diagnosis of plagiocephaly or to rule out craniosynostosis. Furthermore, children, particularly infants, should be spared unnecessary radiation. In many clinical studies, the utility of CT has been investigated in relationship to the differential diagnosis of PWS versus craniosynostosis.5,8,21,22,25-27 In a Class III study conducted by Fisher et al, the authors pointed out that only in equivocal or severe cases of skull deformity could confusion with true synostosis arise, and the diagnosis must be confirmed to correctly identify infants with true craniosynostosis.25 The diagnosis can be determined by clinical examination of the head shape and, only if absolutely necessary, established with a CT scan.25 However, for purposes of our study, the study by Fisher et al was Class III study supporting a Level III recommendation because, as the authors pointed out, the purpose of their study was 2-fold: (1) to evaluate the incidence of true metopic synostosis in infants with plagiocephaly and (2) to examine the morphology of the metopic suture, forehead, and interorbital distance among the positional plagiocephaly group and to compare it with the morphologies of the suture and forehead and interorbital distances of infants with classic metopic synostosis and trigonocephaly. Additionally, there was no comparison of CT findings to clinical observations or physical examination, or even skull x-rays as diagnostic modalities. Losee et al published a Class III study evaluating CT scanning as a modality to diagnose lambdoid synostosis.27 Thirty-three infants with posterior plagiocephaly were imaged; 26 did not have craniosynostosis, and 7 infants did have lambdoid synostosis diagnosed after 10 suture characteristics were evaluated.27 Although this was a well-done study, it was a Class III study because there was no comparison of imaging modalities or comparison to clinical examination alone.
In another Class III retrospective review of 204 patients with occipital plagiocephaly, all of whom had clinical examination and skull x-rays, 2 CT scans were done in cases in which the diagnosis was in question.24 In a Class III retrospective analysis of 287 infants seen at their craniofacial clinic, Hutchison et al reported that only 2% (n = 7) were referred for CT scanning and that clinical examination and head shape measurements were usually sufficient for diagnosis.8 In a similar Class III retrospective analysis of 287 infants seen at their craniofacial clinic, Hutchison et al reported that only 2% of their patients (n = 7) were referred for CT scanning and that clinical examination and head shape measurements were usually sufficient for diagnosis.8 Of those 7 infants in whom the diagnosis could not be made on clinical examination alone, 4 had confirmed craniosynostosis, 1 had an abnormal “extra” suture, and 2 had severe positional plagiocephaly without true craniosynostosis. In the authors’ opinion, CT scanning is not indicated for all infants with plagiocephaly, only those in whom the skull deformation is severe and the suture is “ridged.”8
While CT scans are not necessary in most infants with plagiocephaly, CT scanning may be useful for differential diagnosis in difficult or equivocal cases.5,21,22,26,28,29 In a Class III retrospective review of 102 CT scans performed on infants with skull asymmetry, of whom 82 had deformational plagiocephaly and 20 had craniosynostosis, skull base asymmetries were evaluated and compared.29 Although there were obvious and different asymmetries recorded, the value of these measurements over a simple clinical examination was not established, and there was no consideration of radiation exposure to these children.29 Similar findings were published by Lo et al and Netherway et al26, 30 CT scans of infants with plagiocephaly and craniosynostosis did show differences in external perimeter analysis, cranial fossae symmetries, and cranial midline angulation; however, in most of these studies, the absolute needs for CT imaging for diagnosis and comparison to expert clinical examination were not done.26,30 Differences in 3D anatomy that are made obvious on imaging studies have allowed clinicians to follow improvement in cranial symmetry over time. Although some studies have documented better symmetry in head shape in infants with PWS treated with cranial orthotics, the absolute need for 3D CT imaging for “follow-up” was never demonstrated.2,30 Mulliken et al reported that only 11/115 infants required CT scans.5 In most studies, CT scanning is not indicated for all infants with plagiocephaly, only those in whom the clinical diagnosis is not apparent, and skull x-rays or other imaging is equivocal or non-diagnostic.
In a Class III, retrospective comparative study of 202 infants, 66 with PWS and CT data, Abbott et al demonstrated that intracranial volumes are similar to the intracranial volumes of “normal” infants.1 Intracranial volume calculated for the 66 infants with deformational plagiocephaly was not statistically different compared to “normal,” age-matched infants.1 This information was validated in another study by Bruner et al, who prospectively studied 3D CT data and calculated intracranial volume in infants with PWS treated with cranial molding therapy.2 In several studies, 3D scans were found “useful” for evaluating the results of helmet therapy for infants with deformational plagiocephaly.2,30 However, the purpose of the Bruner study was to use 3D CT to assess the efficacy of helmet therapy. Their analysis was not undertaken in order to compare clinical examination for diagnosis to CT imaging, and so, for our purposes, we did downgrade the Bruner study to Class III evidence. Criticisms of the study include that (1) no mention was made of radiation dose exposure to infants, (2) only 34/69 infants completed the study, and (3) no comparison was made to initial or clinical assessment of the severity of deformity/plagiocephaly. Although other studies have also demonstrated that 3D CT scanning does show differences in craniofacial skeletal development in infants with plagiocephaly or unilateral coronal synostosis as compared to normal infants, this does not prove the need for CT scanning.28,30,31 In fact, in most of these studies, there was no consideration or discussion of radiation dose or exposure, and there was no evidence to suggest that CT scans were necessary for diagnosis. Risk of radiation exposure in infancy is of obvious concern, and unnecessary CT scans should be avoided.
Magnetic Resonance Imaging
The utility of magnetic resonance imaging (MRI) for plagiocephaly has also been investigated.32 No recommendation can be made for MRI, however, because there was only a single study, and the study did not assess the true need for MRI in the diagnosis of craniosynostosis.
In a Class III, prospective, non-randomized study, Collett and Aylward assessed brain volume and shape in infants with deformational plagiocephaly.32 The authors compared 2 cohorts of infants: 20 infants with deformational plagiocephaly and 21 “normal” infants. This was a magnetic resonance imaging (MRI) study of various intracranial measurements and volumes in infants with or without deformational plagiocephaly. The authors concluded that: (1) the shape of the brain is affected or controlled by the skull shape and (2) degree of asymmetry is associated with neurodevelopmental outcomes. Criticisms include that the authors were unable to establish whether plagiocephaly develops because of developmental delays or vice versa. Additionally, although there were 78 infants with plagiocephaly identified, only 50 consented to the study, and only 30 MRI studies were attempted, and of those, only 20 completed the MRI study successfully. The cost and time involved in obtaining the MRI studies were not calculated, and there was no comparison between clinical assessment of head asymmetry and findings on MRI.32
Within the limits of this systematic review, we found that imaging is unnecessary as a first step in infants with plagiocephaly and should be reserved for when the clinical examination is equivocal. In these situations, skull x-rays or ultrasound are almost always sufficient for definitive diagnosis. CT is the gold-standard but should be used sparingly, always making sure the benefit of making a diagnosis is worth the radiation exposure. MRI plays no role.
- Clinical examination is recommended for the diagnosis of plagiocephaly and imaging is rarely necessary, except in cases in which clinical diagnosis is equivocal. Strength of recommendation: Level III—low clinical certainty
- In cases in which the clinical examination is equivocal, skull x-rays or ultrasound imaging of the suspect suture is recommended. Strength of recommendation: Level II—moderate clinical certainty
- In cases in which the clinical examination is equivocal, surface imaging (computer-based topographical scans) or stereophotogrammetry is recommended for the assessment of infants with plagiocephaly without synostosis. Strength of recommendation: Level III—low clinical certainty
- Only for infants in whom x-rays or ultrasound are non-diagnostic, a CT scan is recommended for definitive diagnosis. Strength of recommendation: Level III—low clinical certainty
The guidelines task force would like to acknowledge the Congress of Neurological Surgeons Guidelines Committee for their contributions throughout the development of this guideline, the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Guidelines Committee for their review, comments, and suggestions, as well as Pamela Shaw, MSLIS, MS, and Mary Bodach, MLIS, for their assistance with the literature searches. Throughout the review processs, the reviewers and authors were blinded from one another. At this time, the guidelines task force would like to acknowledge the following individual peer reviewers for their contributions: Sepideh Amin-Hanjani, MD; Maya Babu, MD; Kimon Bekelis, MD; Faiz Ahmad, MD; Daniel Resnick, MD; Patricia Raksin, MD; Jeffrey Olson, MD; and Krystal Tomei, MD.
These evidence-based clinical practice guidelines were funded exclusively by the Congress of Neurological Surgeons and the Section on Pediatric Neurosurgery of the Congress of Neurological Surgeons and the American Association of Neurological Surgeons, which received no funding from outside commercial sources to support the development of this document. The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.
- Abbott AH, Netherway DJ, Moore MH, et al. Computer tomography determined intracranial volume of infants with deformational plagiocephaly: a useful "normal"? The Journal of craniofacial surgery. 1998;9(6):493-503.
- Bruner TW, David LR, Gage HD, Argenta LC. Objective outcome analysis of soft shell helmet therapy in the treatment of deformational plagiocephaly. The Journal of craniofacial surgery. 2004;15(4):643-650.
- Kuang AA, Bergquist C, Crupi L, Oliverio M, Selden NR. Effectiveness and safety of independent pediatric nurse practitioners in evaluating plagiocephaly. Plastic and reconstructive surgery. 2013;132(2):414-418.
- Linz C, Collmann H, Meyer-Marcotty P, et al. Occipital plagiocephaly: unilateral lambdoid synostosis versus positional plagiocephaly. Arch Dis Child. 2015;100(2):152-157.
- Mulliken JB, Vander Woude DL, Hansen M, LaBrie RA, Scott RM. Analysis of posterior plagiocephaly: deformational versus synostotic. Plastic and reconstructive surgery. 1999;103(2):371-380.
- O'Broin ES, Allcutt D, Earley MJ. Posterior plagiocephaly: proactive conservative management. Br. J. Plast. Surg. 1999;52(1):18-23.
- Huang MH, Gruss JS, Clarren SK, et al. The differential diagnosis of posterior plagiocephaly: true lambdoid synostosis versus positional molding. Plastic and reconstructive surgery. 1996;98(5):765-774; discussion 775-766.
- Hutchinson DL, Stewart AW, Mitchell EA. Characteristics, head shape measurements and developmental delay in 287 consecutive infants attending a plagiocephaly clinic. Acta Paediatrica. 2009;98:1494-1499.
- Littlefield TR, Beals SP, Manwaring KH, et al. Treatment of craniofacial asymmetry with dynamic orthotic cranioplasty. The Journal of craniofacial surgery. 1998;9(1):11-17; discussion 18-19.
- Schaaf H, Pons-Kuehnemann J, Malik CY, et al. Accuracy of three-dimensional photogrammetric images in non-synostotic cranial deformities. Neuropediatrics. 2010;41(1):24-29.
- Zonenshayn M, Kronberg E, Souweidane MM. Cranial index of symmetry: an objective semiautomated measure of plagiocephaly. Technical note. Journal of neurosurgery. 2004;100(5 Suppl Pediatrics):537-540.
- Collett BR, Heike CL, Atmosukarto I, Starr JR, Cunningham ML, Speltz ML. Longitudinal, three-dimensional analysis of head shape in children with and without deformational plagiocephaly or brachycephaly. The Journal of pediatrics. 2012;160(4):673-678 e671.
- Katzel EB, Koltz PF, Sbitany H, Girotto JA. Treatment of plagiocephaly with helmet molding therapy: do actual results mimic perception? The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. 2011;48(2):205-209.
- Kluba S, Schreiber R, Kraut W, Meisner C, Reinert S, Krimmel M. Does helmet therapy influence the ear shift in positional plagiocephaly? The Journal of craniofacial surgery. 2012;23(5):1301-1305.
- Lipira AB, Gordon S, Darvann TA, et al. Helmet versus active repositioning for plagiocephaly: a three-dimensional analysis. Pediatrics. 2010;126(4):e936-945.
- Meyer-Marcotty P, Bohm H, Linz C, et al. Head orthesis therapy in infants with unilateral positional plagiocephaly: an interdisciplinary approach to broadening the range of orthodontic treatment. Journal of orofacial orthopedics = Fortschritte der Kieferorthopadie : Organ/official journal Deutsche Gesellschaft fur Kieferorthopadie. 2012;73(2):151-165.
- Moghaddam MB, Brown TM, Clausen A, DaSilva T, Ho E, Forrest CR. Outcome analysis after helmet therapy using 3D photogrammetry in patients with deformational plagiocephaly: the role of root mean square. Journal of plastic, reconstructive & aesthetic surgery : JPRAS. 2014;67(2):159-165.
- Thompson JT, David LR, Wood B, Argenta A, Simpson J, Argenta LC. Outcome analysis of helmet therapy for positional plagiocephaly using a three-dimensional surface scanning laser. The Journal of craniofacial surgery. 2009;20(2):362-365.
- Schaaf H, Malik CY, Streckbein P, Pons-Kuehnemann J, Howaldt HP, Wilbrand JF. Three-dimensional photographic analysis of outcome after helmet treatment of a nonsynostotic cranial deformity. The Journal of craniofacial surgery. 2010;21(6):1677-1682.
- Krimmel M, Will B, Wolff M, et al. Value of high-resolution ultrasound in the differential diagnosis of scaphocephaly and occipital plagiocephaly. International journal of oral and maxillofacial surgery. 2012;41(7):797-800.
- Pollack IF, Losken HW, Fasick P. Diagnosis and management of posterior plagiocephaly. Pediatrics. 1997;99(2):180-185.
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