- Email: [email protected]
Clinical Classification of Infant Nonsynostotic Cranial Deformity
Clinical Classification of Infant Nonsynostotic Cranial Deformity Jan-Falco Wilbrand, MD, DMD1, Kerstin Schmidtberg, DMD1, Uta Bierther, MD, DMD1, Philipp Streckbein, MD, DMD1, Joern Pons-Kuehnemann, PhD2, Petros Christophis, MD, PHD3, Andreas Hahn, MD, PHD4, Heidrun Schaaf, MD, DMD, PHD1, and Hans-Peter Howaldt, MD, DMD, PHD1 Objective Based on a pilot study including >400 children and a comprehensive database analysis of >2500 children, we sought to define the craniofacial norm and to objectify the categorization of positional head deformity.
Study design A database was created containing clinical information on children assessed for nonsynostotic cranial deformity. The findings of standardized anthropometric measurements were compared with data from a group of 401 healthy children with a normal head shape collected in terms of a prospective pilot study. Using a statistical analysis of all anthropometric craniofacial measurements, cut-off percentiles for discriminating different groups of deformation and severity classes were generated. Results Normative percentiles for all dimensions in cranial vault anthropometric measurements during the first year of life were calculated. Children with definite nonsynostotic head deformity could be clearly allocated into 3 different groups: positional plagiocephaly (abnormal Cranial Vault Asymmetry Index), positional brachycephaly (abnormal Cranial Index), and combined positional plagiocephaly and brachycephaly (abnormal Cranial Vault Asymmetry Index and Cranial Index). Additionally, a reliable 3-level severity categorization (mild, moderate, and severe) for each group of cranial deformation could be obtained according to age and sex. Conclusions Our results allow a meaningful and reliable classification of nonsynostotic early childhood cranial deformity. (J Pediatr 2012;161:1120-5).
T
he incidence of sudden infant death syndrome declined significantly after the American Academy of Pediatrics initiated the Back to Sleep campaign in 1992.1,2 However, increasing numbers of nonsynostotic head deformities were subsequently reported.3,4 Positional infant plagiocephaly is characterized by asymmetric occipital flattening with a flat area on the contralateral forehead and scoliosis of the face.5,6 As described by Argenta et al, central deformational plagiocephaly or brachycephaly shows distinct occipital flattening with increased head width or height.6 Due to increasing experience with these patients, positional head deformities can usually be identified visually, without using radiographic methods.7,8 Indication for helmet therapy usually is decided after the initial clinical assessment, but the process is imprecise. Nevertheless, there is a definite need for an objective classification of the type and severity of cranial deformity to improve the reliability of clinical diagnosis and treatment.9-12 This study systematizes nonsynostotic craniofacial deformities, in a manner similar to that used in the classification of premature craniosynostoses, by creating a comprehensive database. A comparative analysis with normal anthropometric measurements was performed to improve diagnostic accuracy and obtain reliable increments for positional head deformity. Using a statistical cluster analysis of the patients, a 3-level grouping method of nonsynostotic deformities and age- and sexdependent percentiles for severity classifications are proposed. The resulting classification is routinely used in our clinic for decision making and during therapy of craniofacial deformity.
Methods Preliminary to our study, approval of the local ethic board was given. Parental informed consent was assured before intervention. A prospective cross-sectional cohort pilot-study of 401 children was performed to obtain normative values of cranial vault growth in the first year of life. Children were grouped into 4 age groups (group 1: 0-3 months, group 2: 4-6 months, group 3: 7-9 months, group 4: 10 months and older). Seven respective anthropometric measurements (cranial circumference, length, breadth, transversal and sagittal hemicircumference, and transcranial diagonals A and B) were collected by a trained examiner (U.B.) following a standardized protocol defined previously.13 Normative values for the derived indices (Cranial Index and Cranial Vault Asymmetry Index [CVAI]) were calculated and respective percentiles (3rd, 10th, 25th, 50th, 75th, 90th, and 97th) were generated (Table; available at www.jpeds.com). Furthermore, a Windows Access (Microsoft Corporation, Redmond, Washington) database for the systematization of nonsynostotic cranial deforFrom the Departments for Cranio-Maxillofacial Surgery, Plastic Surgery, Medical Statistics, Neurosurgery, and mities was generated. This database includes the results of standardized initial Neuropediatrics, University Hospital Giessen, Giessen, 1
2
3
4
Germany The authors declare no conflicts of interest.
CVAI
Cranial Vault Asymmetry Index
0022-3476/$ - see front matter. Copyright ª 2012 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2012.05.023
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Vol. 161, No. 6 December 2012 and follow-up anthropometric craniofacial measurements, the patient history, and other details for each patient, such as age and sex, preterm birth, risk factors, and therapeutic approaches used to treat the craniofacial deformity in advance. All patients seen in the Cranio-Maxillofacial Surgery Department of the University Hospital, Giessen, Germany, and diagnosed with a nonsynostotic cranial deformity in the 6-year period between May 2005 and August 2011 were included in the database. Of the 2571 patients in the database, 1512 patients showed a head deformity requiring orthotic treatment clinically and met the inclusion criteria for the classification study. Children with a diagnosis of premature suture craniosynostosis or without a head deformity requiring orthotic treatment clinically were excluded from the study. The anthropometric measurements included in the database were obtained using a standardized protocol,13 which consisted of cranial circumference, length, width, and diagonals A and B. The Cranial Index (cranial width/length 100) and (CVAI) ([(diagonal A diagonal B)/diagonal A] 100, where diagonal A < diagonal B) were calculated for each patient (Figure 1). Data from the initial craniofacial assessment were analyzed statistically and compared with the anthropometric data collected from the 401 healthy white children in the pilot study on normal values. The CVAI was compared in plagiocephalic children and children with combined plagiocephaly and brachycephaly. The Cranial Index was compared in children with brachycephaly and combined plagiocephaly and brachycephaly. In a first statistical evaluation, the arrangement of the data was described. Then, a cluster analysis was used to find a meaningful grouping method.
Results From the entire database of 2571 children, 1512 (58.8%) children (1095 males [72.4%] and 417 females [27.6%]) met the clinical criteria for therapy with an orthotic helmet. Physiotherapy treatments had been performed in 1167 (77.2%) of the patients. A significant improvement in the cranial shape due to these methods subjectively was observed by the parents in our clinic in only 63 (5.3%) cases. Of the children with a clinically significant head deformity, 24.3% were born before the 37th week of pregnancy and therefore were preterm. The mean age at the first appointment in our clinic was 7.5 months. The median duration of a helmet therapy was 5.7 months. From all children in our database, we could clearly allocate 2506 (97.5%) into 1 of 3 groups: 1535 children (61.3%; 498 females and 1037 males) had an abnormal CVAI only; 229 children (9.1%; 72 females and 157 males) had an abnormal Cranial Index only; and 742 children (29.6%; 200 females and 542 males) had an abnormal CVAI and Cranial Index. The 3 groups were called the plagiocephaly, brachycephaly, and combined plagiocephaly and brachycephaly groups, respectively. Comparing the anthropometric data of the orthotically treated children (n = 1512) with those of the control group (n = 401) showed that in the treated group with plagiocephaly alone (n = 878) or in combination (n = 527), 83.3% were above the normative 97th percentile curve for CVAI. Those were defined as severely deformed. Additionally, 95.8% of the children in this group were above the 90th percentile. We defined a CVAI between the 90th and 97th percentiles as moderate deformation and a CVAI between the 75th and 90th percentiles as mild deformation. Of the treated children with plagiocephaly, 99.1% were above the 75th percentile (Figure 2).
Figure 1. Anthropometric landmarks for measurement of cranial length (glabella point [g]- opisthocranion point [op]), cranial width (eurion point [eu]-eu), and respective transcranial diagonals A and B (frontotemporale point [ft]-lambdoid point [ld]). 1121
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Figure 2. Pretreatment CVAI values of A, male children (n = 1018) and B, female children (n = 382), with plagiocephaly, or combination of plagiocephaly and brachycephaly, treated with an individual molding helmet plotted on normative percentile curves for CVAI in male and female children in the first year of life.
Revisiting the data for the Cranial Index, we found that 93.5% of the treated children with brachycephaly alone (n = 107) or combined with plagiocephaly (n = 527) were above the 97th percentile curve. Hence, we defined this as severe brachycephalic head deformity. As 99.2% of these children were above the 90th percentile, this was defined as the cut-off for moderate deformation; 99.9% were above the 1122
75th percentile, which we set as the cut-off for mild deformation (Figure 3). Orthotic therapy was administered to 57.2% of the plagiocephalic children, 46.7% of the brachycephalic children, and 71.0% of children with a combined deformity, whereas 1059 patients (41.2% of all patients) were not given helmet therapy. Wilbrand et al
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Figure 3. Pretreatment Cranial Index values of A, male children (n = 473) and B, female children (n = 158), with brachycephaly, or combination of plagiocephaly and brachycephaly, treated with an individual molding helmet plotted on normative percentile curves for Cranial Index in male and female children in the first year of life.
Discussion A reliable instrument is needed to classify nonsynostotic plagiocephaly and brachycephaly in the diagnosis and therapy of pediatric craniofacial deformity.9,11 This should Clinical Classification of Infant Nonsynostotic Cranial Deformity
be based on clear cut-offs and repeatable anthropometric measurements to ensure objectivity and high clinical validity.13-15 Furthermore, cranial shape should be judged in respect of age and sex, to obtain comparability to work from other authors.14,15 Several classification methods have been proposed.6,9 Hutchison et al proposed that a Cranial Index 1123
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$93% was the cut-off for brachycephaly, and an oblique cranial length ratio of 106% was used as the cut-off for plagiocephalic head deformities.16 No sex differences were proposed in that study. However, the classification was based on a small number of children, a different measurement method, and different anthropometric parameters than those used in our study.16 Mortenson and Steinbok evaluated the clinical applicability of anthropometric caliper measurements and the clinical appearance of their patients.9 Their results were unsatisfactory, and they spoke of the need for a reliable instrument for measuring and classifying infant cranial deformities. In 2004, Argenta et al proposed what has become a widely used system that classifies nonsynostotic cranial deformation exclusively by the clinical appearance of the patient.6 However, studies of the reliability of this method have shown that it lacks clinical value.11 Loveday and de Chalain described a model that defined significant plagiocephaly as a CVAI >3.5% and a normal Cranial Index as between 75% and 85%.17 However, they did not examine the effects of sex or age, and their patient series was small. All these proposals for the classification of infant head deformity, however, were not based on a comprehensive statistical analysis taking the “norm” into account. Based on almost 2 decades of evaluating nonsynostotic cranial deformities in Europe,18 our classification based on percentile curves of the normal cranial vault growth during the first year of life appears to provide a secure diagnostic and therapeutic instrument for pediatric craniofacial practitioners. Our craniofacial measurements are based on reliable anthropometric examinations with high clinical validity.13 We propose ageand sex-specific cut-offs for the definitions of severe, moderate, and mild head deformities, as well as an objective classification of positional head deformities into 3 individual groups. Any therapeutic recommendations for nonsynostotic head deformities should include physiotherapy approaches and bedding modifications.19-21 We make such recommendations when children are #4 months old and when the craniofacial deformity is classified as “mild” to “moderate.” Nevertheless, based on subjective assessments by the parents in our clinic, a significant improvement in cranial shape is rarely achieved. The low level of improvement in our group might be due to a preselection of patients. Physiotherapy methods are the current American Academy of Pediatrics recommendations for an initial treatment of head deformities. Cervical mobility is improved by these advances and therefore they should be implemented early. Presentation of severe head deformity should lead to an orthotic therapy, if parental request is given, at a minimum age of 4 months.22 Furthermore, eventual improvement of cranial shape after physical therapy, osteopathic medicine, or bedding modifications should be evaluated and should influence the decision about the therapeutic pathway. Careful follow-up is needed and measurements should be taken to determine whether physical therapy can be sufficient or whether orthotic therapy is necessary. The percentile curves generated in this study allow exact tracing of the developmental course of cranial deformity in all dimensions. 1124
Vol. 161, No. 6 A good way to acquire exact assessment of cranial deformity is ensured by 3-dimensional photography.23 It is a fast, examiner-independent, and noninvasive method to objectify cranial shape.24 The anthropometric measurements with spreading calipers used in our study, however, have the advantage of general availability, simplicity, and cost-effectiveness. In our experience, a combined head deformity is the most stigmatizing cranial deformity and usually requires treatment (71.1%). By contrast, only 46.8% of all brachycephalic deformities were treated. Recommendations for orthotic helmet therapy should be established for the nonsynostotic cranial deformities (ie, plagiocephaly and brachycephaly alone or in combination). n The authors would like to thank Mr Herbert Kattenborn (University Hospital Giessen, Germany) for programming the database and helping to summarize the collected data. Submitted for publication Feb 14, 2012; last revision received Apr 16, 2012; accepted May 10, 2012. Reprint requests: Jan-Falco Wilbrand, MD, DMD, Department for CranioMaxillofacial Surgery, Plastic Surgery, Klinikstraße 33, 35385 Giessen, Germany. E-mail: [email protected]
References 1. Moon RY, Horne RS, Hauck FR. Sudden infant death syndrome. Lancet 2007;370:1578-87. 2. American Academy of Pediatrics Task Force on Infant Positioning and SIDS. Positioning and SIDS. Pediatrics 1992;89:1120-6. 3. Kane AA, Mitchell LE, Craven KP, Marsh JL. Observations on a recent increase in plagiocephaly without synostosis. Pediatrics 1996;97: 877-85. 4. Turk AE, McCarthy JG, Thorne CH, Wisoff JH. The “back to sleep campaign” and deformational plagiocephaly: is there cause for concern? J Craniofac Surg 1996;7:12-8. 5. Mulliken JB, Vander Woude DL, Hansen M, LaBrie RA, Scott RM. Analysis of posterior plagiocephaly: deformational versus synostotic. Plast Reconstr Surg 1999;103:371-80. 6. Argenta L, David L, Thompson J. Clinical classification of positional plagiocephaly. J Craniofac Surg 2004;15:368-72. 7. Cunningham ML, Heike CL. Evaluation of the infant with an abnormal skull shape. Curr Opin Pediatr 2007;19:645-51. 8. Ruiz-Correa S, Sze RW, Starr JR, Lin HT, Speltz ML, Cunningham ML, et al. New scaphocephaly severity indices of sagittal craniosynostosis: a comparative study with cranial index quantifications. Cleft Palate Craniofac J 2006;43:211-21. 9. Mortenson PA, Steinbok P. Quantifying positional plagiocephaly: reliability and validity of anthropometric measurements. J Craniofac Surg 2006;17:413-9. 10. van Vlimmeren LA, Takken T, van Adrichem LN, van der Graaf Y, Helders PJ, Engelbert RH. Plagiocephalometry: a non-invasive method to quantify asymmetry of the skull; a reliability study. Eur J Pediatr 2006;165:149-57. 11. Spermon J, Spermon-Marijnen R, Scholten-Peeters W. Clinical classification of deformational plagiocephaly according to Argenta: a reliability study. J Craniofac Surg 2008;19:664-8. 12. McGarry A, Dixon MT, Greig RJ, Hamilton DR, Sexton S, Smart H. Head shape measurement standards and cranial orthoses in the treatment of infants with deformational plagiocephaly: a systematic review. Dev Med Child Neurol 2008;50:568-76. 13. Wilbrand JF, Wilbrand M, Pons-Kuehnemann J, Blecher JC, Christophis P, Howaldt HP, et al. Value and reliability of anthropometric measurements of cranial deformity in early childhood. J Craniomaxillofac Surg 2011;39:24-9.
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December 2012 14. Kolar JC, Salter EM. Craniofacial anthropometry. Springfield (IL): Charles C Thomas; 1997. 15. Farkas LG. Anthropometry of the head and face. 2nd ed. Toronto (Canada): Raven Press; 1994. 16. Hutchison BL, Hutchison LA, Thompson JM, Mitchell EA. Quantification of plagiocephaly and brachycephaly in infants using a digital photographic technique. Cleft Palate Craniofac J 2005;42:539-47. 17. Loveday BP, de Chalain TB. Active counterpositioning or orthotic device to treat positional plagiocephaly? J Craniofac Surg 2001;12: 308-13. 18. Blecher JC, Howaldt HP. Treatment of non-synostotic, pediatric skull deformities with dynamic head orthosis. Mund Kiefer Gesichtschir 1998;2(Suppl 1):S81-5. 19. Pollack IF, Losken HW, Fasick P. Diagnosis and management of posterior plagiocephaly. Pediatrics 1997;99:180-5.
Clinical Classification of Infant Nonsynostotic Cranial Deformity
ORIGINAL ARTICLES 20. Neufeld S, Birkett S. What to do about flat heads: preventing and treating positional occipital flattening. Axone 2000;22:29-31. 21. Moss SD. Nonsurgical, nonorthotic treatment of occipital plagiocephaly: what is the natural history of the misshapen neonatal head? J Neurosurg 1997;87:667-70. 22. Kluba S, Kraut W, Reinert S, Krimmel M. What is the optimal time to start helmet therapy? Plast Reconstr Surg 2011;128:492-8. 23. 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. J Craniofac Surg 2010;21:1677-82. 24. Wilbrand JF, Szczukowski A, Blecher JC, Pons-Kuehnemann J, Christophis P, Howaldt HP, et al. Objectification of cranial vault correction for craniosynostosis by three-dimensional photography. J Craniomaxillofac Surg 2012; doi: 10.1016/J.jcms.2012.01.007.
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Circumference, cm Group 0-3 mo (M = 69, F = 56)
4-6 mo (M = 56, F = 47)
CVA, cm
Obs-Obs, cm
Nas-Ini, cm
Cranial Index, %
CVAI, %
Percentiles
M
F
M
F
M
F
M
F
M
F
M
F
M
F
M
F
3 10 25 50 75 90 97 3 10 25 50 75 90 97 3 10 25 50 75 90 97 3 10 25 50 75 90 97
33.6 35.5 36.5 37.7 39 40.5 42.5 38.2 40 41 42.6 43.3 44 45.1 40.7 42.3 43.6 44.8 45.5 46.4 46.9 42.3 44.3 45.4 46.3 47.5 48.6 49.7
31.6 34.8 35.9 37.4 38.7 39.6 40.1 37.3 38.3 39.4 41 41.8 43.2 43.8 40.4 41.7 42.2 43.5 44 45.2 46 42.7 43.3 44.3 45 46 46.9 47.8
8.2 8.9 9.6 10 10.6 11.4 12.2 10.1 10.8 11.2 11.5 12 12.5 12.7 10.3 11.2 11.8 12 12.6 13.1 13.4 10.9 12 12.2 12.6 13 13.9 14.6
8.5 9.2 9.5 10.2 10.4 10.9 11.4 9.8 10.4 10.6 11.2 11.6 12.1 12.9 10.6 11 11.2 11.8 12 12.1 12.4 11.2 11.4 11.8 12.2 12.6 12.9 13.5
11.2 11.4 12.2 12.4 13 13.4 14 12.1 13 13.4 13.8 14.4 14.6 14.8 13.3 13.7 14.2 14.8 15.2 15.6 16.1 13.3 13.9 14.8 15.4 16 16.4 17.2
10.8 11.3 11.8 12.4 12.8 13.4 13.7 11.2 12.4 12.8 13.4 13.8 14.2 14.7 13.2 13.2 13.9 14.4 15.1 15.4 15.6 13.5 14.1 14.6 15.2 15.5 16.1 16.3
0 0 0.2 0.2 0.4 0.6 0.8 0 0 0.2 0.4 0.6 0.8 0.9 0 0 0.2 0.2 0.4 0.7 0.8 0 0 0.2 0.2 0.4 0.6 0.7
0 0 0.2 0.2 0.4 0.6 0.7 0 0 0.2 0.2 0.4 0.6 0.9 0 0.1 0.2 0.4 0.4 0.7 0.8 0 0.1 0.2 0.2 0.4 0.6 0.8
18.4 19.2 20.2 21.2 22 23.2 24.2 20.8 22.4 23.2 24.2 25 25.9 27.6 22.6 24 24.4 25.7 26.4 27.4 28 22.6 25.2 26 26.8 27.5 28.6 30.3
17.5 18.5 19.6 21 22 22.9 24.3 17 21.8 22.4 23.5 24.4 25.2 28.5 22 23.8 24.2 24.7 26 26.7 27.2 23 24.5 25 25.8 26.8 28.2 33.1
18.4 20.3 21.2 22.2 22.9 24.4 27.2 20.8 22.7 24.3 25.9 26.8 27.4 27.8 23.1 24.3 26.2 27.5 29 30.2 31.4 21.7 26.1 27.5 29.4 30.6 31.5 32.9
17 18.5 20 22.1 23.3 24.5 25.9 22.5 23.3 24.4 25.4 27 28 29.4 21.2 24.5 25.2 26.6 28 29.2 29.3 24.8 26 27.6 28.3 30 30.6 35.6
67.9 72.3 77 79 85.7 91.8 98.4 74.9 78.5 79.5 83.5 87 93 95.9 72.4 74.7 77.6 82.2 87 91.4 96.6 73.5 75.5 78.6 82.1 86.5 92.1 97
72.7 75.7 78 79.7 85.4 87.2 90.3 70.3 76.8 79.4 83.6 86.6 92.9 99.8 72.3 74.8 77.2 80.2 82.9 89 90.9 72.5 74 76.5 80.5 85.9 89.5 94.8
0 0 1.6 1.9 3.7 5.2 7 0 0 1.6 3.1 4.7 6.6 7.3 0 0 1.4 1.6 3.2 5.9 11.1 0 0 1.4 1.5 2.9 4.4 5.3
0 0 1.7 1.9 3.6 5.4 6.9 0 0 1.5 1.7 3.2 5.6 9.4 0 0.4 1.5 2.9 3.2 5.9 10 0 1.4 1.5 1.6 3.2 4.7 6
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10-12 mo (M = 55, F = 43)
Length, cm
7-9 mo (M = 43, F = 32)
Width, cm
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Table. Anthropometric measurements and derived indices during the first year of life in 401 healthy white children
CVA, cranial vault asymmetry; Obs, otobasion; Nas, nasion; Ini, inion. Circumference indicates head circumference. The median values are given in bold.
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Study design A database was created containing clinical information on children assessed for nonsynostotic cranial deformity. The findings of standardized anthropometric measurements were compared with data from a group of 401 healthy children with a normal head shape collected in terms of a prospective pilot study. Using a statistical analysis of all anthropometric craniofacial measurements, cut-off percentiles for discriminating different groups of deformation and severity classes were generated. Results Normative percentiles for all dimensions in cranial vault anthropometric measurements during the first year of life were calculated. Children with definite nonsynostotic head deformity could be clearly allocated into 3 different groups: positional plagiocephaly (abnormal Cranial Vault Asymmetry Index), positional brachycephaly (abnormal Cranial Index), and combined positional plagiocephaly and brachycephaly (abnormal Cranial Vault Asymmetry Index and Cranial Index). Additionally, a reliable 3-level severity categorization (mild, moderate, and severe) for each group of cranial deformation could be obtained according to age and sex. Conclusions Our results allow a meaningful and reliable classification of nonsynostotic early childhood cranial deformity. (J Pediatr 2012;161:1120-5).
T
he incidence of sudden infant death syndrome declined significantly after the American Academy of Pediatrics initiated the Back to Sleep campaign in 1992.1,2 However, increasing numbers of nonsynostotic head deformities were subsequently reported.3,4 Positional infant plagiocephaly is characterized by asymmetric occipital flattening with a flat area on the contralateral forehead and scoliosis of the face.5,6 As described by Argenta et al, central deformational plagiocephaly or brachycephaly shows distinct occipital flattening with increased head width or height.6 Due to increasing experience with these patients, positional head deformities can usually be identified visually, without using radiographic methods.7,8 Indication for helmet therapy usually is decided after the initial clinical assessment, but the process is imprecise. Nevertheless, there is a definite need for an objective classification of the type and severity of cranial deformity to improve the reliability of clinical diagnosis and treatment.9-12 This study systematizes nonsynostotic craniofacial deformities, in a manner similar to that used in the classification of premature craniosynostoses, by creating a comprehensive database. A comparative analysis with normal anthropometric measurements was performed to improve diagnostic accuracy and obtain reliable increments for positional head deformity. Using a statistical cluster analysis of the patients, a 3-level grouping method of nonsynostotic deformities and age- and sexdependent percentiles for severity classifications are proposed. The resulting classification is routinely used in our clinic for decision making and during therapy of craniofacial deformity.
Methods Preliminary to our study, approval of the local ethic board was given. Parental informed consent was assured before intervention. A prospective cross-sectional cohort pilot-study of 401 children was performed to obtain normative values of cranial vault growth in the first year of life. Children were grouped into 4 age groups (group 1: 0-3 months, group 2: 4-6 months, group 3: 7-9 months, group 4: 10 months and older). Seven respective anthropometric measurements (cranial circumference, length, breadth, transversal and sagittal hemicircumference, and transcranial diagonals A and B) were collected by a trained examiner (U.B.) following a standardized protocol defined previously.13 Normative values for the derived indices (Cranial Index and Cranial Vault Asymmetry Index [CVAI]) were calculated and respective percentiles (3rd, 10th, 25th, 50th, 75th, 90th, and 97th) were generated (Table; available at www.jpeds.com). Furthermore, a Windows Access (Microsoft Corporation, Redmond, Washington) database for the systematization of nonsynostotic cranial deforFrom the Departments for Cranio-Maxillofacial Surgery, Plastic Surgery, Medical Statistics, Neurosurgery, and mities was generated. This database includes the results of standardized initial Neuropediatrics, University Hospital Giessen, Giessen, 1
2
3
4
Germany The authors declare no conflicts of interest.
CVAI
Cranial Vault Asymmetry Index
0022-3476/$ - see front matter. Copyright ª 2012 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2012.05.023
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Vol. 161, No. 6 December 2012 and follow-up anthropometric craniofacial measurements, the patient history, and other details for each patient, such as age and sex, preterm birth, risk factors, and therapeutic approaches used to treat the craniofacial deformity in advance. All patients seen in the Cranio-Maxillofacial Surgery Department of the University Hospital, Giessen, Germany, and diagnosed with a nonsynostotic cranial deformity in the 6-year period between May 2005 and August 2011 were included in the database. Of the 2571 patients in the database, 1512 patients showed a head deformity requiring orthotic treatment clinically and met the inclusion criteria for the classification study. Children with a diagnosis of premature suture craniosynostosis or without a head deformity requiring orthotic treatment clinically were excluded from the study. The anthropometric measurements included in the database were obtained using a standardized protocol,13 which consisted of cranial circumference, length, width, and diagonals A and B. The Cranial Index (cranial width/length 100) and (CVAI) ([(diagonal A diagonal B)/diagonal A] 100, where diagonal A < diagonal B) were calculated for each patient (Figure 1). Data from the initial craniofacial assessment were analyzed statistically and compared with the anthropometric data collected from the 401 healthy white children in the pilot study on normal values. The CVAI was compared in plagiocephalic children and children with combined plagiocephaly and brachycephaly. The Cranial Index was compared in children with brachycephaly and combined plagiocephaly and brachycephaly. In a first statistical evaluation, the arrangement of the data was described. Then, a cluster analysis was used to find a meaningful grouping method.
Results From the entire database of 2571 children, 1512 (58.8%) children (1095 males [72.4%] and 417 females [27.6%]) met the clinical criteria for therapy with an orthotic helmet. Physiotherapy treatments had been performed in 1167 (77.2%) of the patients. A significant improvement in the cranial shape due to these methods subjectively was observed by the parents in our clinic in only 63 (5.3%) cases. Of the children with a clinically significant head deformity, 24.3% were born before the 37th week of pregnancy and therefore were preterm. The mean age at the first appointment in our clinic was 7.5 months. The median duration of a helmet therapy was 5.7 months. From all children in our database, we could clearly allocate 2506 (97.5%) into 1 of 3 groups: 1535 children (61.3%; 498 females and 1037 males) had an abnormal CVAI only; 229 children (9.1%; 72 females and 157 males) had an abnormal Cranial Index only; and 742 children (29.6%; 200 females and 542 males) had an abnormal CVAI and Cranial Index. The 3 groups were called the plagiocephaly, brachycephaly, and combined plagiocephaly and brachycephaly groups, respectively. Comparing the anthropometric data of the orthotically treated children (n = 1512) with those of the control group (n = 401) showed that in the treated group with plagiocephaly alone (n = 878) or in combination (n = 527), 83.3% were above the normative 97th percentile curve for CVAI. Those were defined as severely deformed. Additionally, 95.8% of the children in this group were above the 90th percentile. We defined a CVAI between the 90th and 97th percentiles as moderate deformation and a CVAI between the 75th and 90th percentiles as mild deformation. Of the treated children with plagiocephaly, 99.1% were above the 75th percentile (Figure 2).
Figure 1. Anthropometric landmarks for measurement of cranial length (glabella point [g]- opisthocranion point [op]), cranial width (eurion point [eu]-eu), and respective transcranial diagonals A and B (frontotemporale point [ft]-lambdoid point [ld]). 1121
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Figure 2. Pretreatment CVAI values of A, male children (n = 1018) and B, female children (n = 382), with plagiocephaly, or combination of plagiocephaly and brachycephaly, treated with an individual molding helmet plotted on normative percentile curves for CVAI in male and female children in the first year of life.
Revisiting the data for the Cranial Index, we found that 93.5% of the treated children with brachycephaly alone (n = 107) or combined with plagiocephaly (n = 527) were above the 97th percentile curve. Hence, we defined this as severe brachycephalic head deformity. As 99.2% of these children were above the 90th percentile, this was defined as the cut-off for moderate deformation; 99.9% were above the 1122
75th percentile, which we set as the cut-off for mild deformation (Figure 3). Orthotic therapy was administered to 57.2% of the plagiocephalic children, 46.7% of the brachycephalic children, and 71.0% of children with a combined deformity, whereas 1059 patients (41.2% of all patients) were not given helmet therapy. Wilbrand et al
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Figure 3. Pretreatment Cranial Index values of A, male children (n = 473) and B, female children (n = 158), with brachycephaly, or combination of plagiocephaly and brachycephaly, treated with an individual molding helmet plotted on normative percentile curves for Cranial Index in male and female children in the first year of life.
Discussion A reliable instrument is needed to classify nonsynostotic plagiocephaly and brachycephaly in the diagnosis and therapy of pediatric craniofacial deformity.9,11 This should Clinical Classification of Infant Nonsynostotic Cranial Deformity
be based on clear cut-offs and repeatable anthropometric measurements to ensure objectivity and high clinical validity.13-15 Furthermore, cranial shape should be judged in respect of age and sex, to obtain comparability to work from other authors.14,15 Several classification methods have been proposed.6,9 Hutchison et al proposed that a Cranial Index 1123
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$93% was the cut-off for brachycephaly, and an oblique cranial length ratio of 106% was used as the cut-off for plagiocephalic head deformities.16 No sex differences were proposed in that study. However, the classification was based on a small number of children, a different measurement method, and different anthropometric parameters than those used in our study.16 Mortenson and Steinbok evaluated the clinical applicability of anthropometric caliper measurements and the clinical appearance of their patients.9 Their results were unsatisfactory, and they spoke of the need for a reliable instrument for measuring and classifying infant cranial deformities. In 2004, Argenta et al proposed what has become a widely used system that classifies nonsynostotic cranial deformation exclusively by the clinical appearance of the patient.6 However, studies of the reliability of this method have shown that it lacks clinical value.11 Loveday and de Chalain described a model that defined significant plagiocephaly as a CVAI >3.5% and a normal Cranial Index as between 75% and 85%.17 However, they did not examine the effects of sex or age, and their patient series was small. All these proposals for the classification of infant head deformity, however, were not based on a comprehensive statistical analysis taking the “norm” into account. Based on almost 2 decades of evaluating nonsynostotic cranial deformities in Europe,18 our classification based on percentile curves of the normal cranial vault growth during the first year of life appears to provide a secure diagnostic and therapeutic instrument for pediatric craniofacial practitioners. Our craniofacial measurements are based on reliable anthropometric examinations with high clinical validity.13 We propose ageand sex-specific cut-offs for the definitions of severe, moderate, and mild head deformities, as well as an objective classification of positional head deformities into 3 individual groups. Any therapeutic recommendations for nonsynostotic head deformities should include physiotherapy approaches and bedding modifications.19-21 We make such recommendations when children are #4 months old and when the craniofacial deformity is classified as “mild” to “moderate.” Nevertheless, based on subjective assessments by the parents in our clinic, a significant improvement in cranial shape is rarely achieved. The low level of improvement in our group might be due to a preselection of patients. Physiotherapy methods are the current American Academy of Pediatrics recommendations for an initial treatment of head deformities. Cervical mobility is improved by these advances and therefore they should be implemented early. Presentation of severe head deformity should lead to an orthotic therapy, if parental request is given, at a minimum age of 4 months.22 Furthermore, eventual improvement of cranial shape after physical therapy, osteopathic medicine, or bedding modifications should be evaluated and should influence the decision about the therapeutic pathway. Careful follow-up is needed and measurements should be taken to determine whether physical therapy can be sufficient or whether orthotic therapy is necessary. The percentile curves generated in this study allow exact tracing of the developmental course of cranial deformity in all dimensions. 1124
Vol. 161, No. 6 A good way to acquire exact assessment of cranial deformity is ensured by 3-dimensional photography.23 It is a fast, examiner-independent, and noninvasive method to objectify cranial shape.24 The anthropometric measurements with spreading calipers used in our study, however, have the advantage of general availability, simplicity, and cost-effectiveness. In our experience, a combined head deformity is the most stigmatizing cranial deformity and usually requires treatment (71.1%). By contrast, only 46.8% of all brachycephalic deformities were treated. Recommendations for orthotic helmet therapy should be established for the nonsynostotic cranial deformities (ie, plagiocephaly and brachycephaly alone or in combination). n The authors would like to thank Mr Herbert Kattenborn (University Hospital Giessen, Germany) for programming the database and helping to summarize the collected data. Submitted for publication Feb 14, 2012; last revision received Apr 16, 2012; accepted May 10, 2012. Reprint requests: Jan-Falco Wilbrand, MD, DMD, Department for CranioMaxillofacial Surgery, Plastic Surgery, Klinikstraße 33, 35385 Giessen, Germany. E-mail: [email protected]
References 1. Moon RY, Horne RS, Hauck FR. Sudden infant death syndrome. Lancet 2007;370:1578-87. 2. American Academy of Pediatrics Task Force on Infant Positioning and SIDS. Positioning and SIDS. Pediatrics 1992;89:1120-6. 3. Kane AA, Mitchell LE, Craven KP, Marsh JL. Observations on a recent increase in plagiocephaly without synostosis. Pediatrics 1996;97: 877-85. 4. Turk AE, McCarthy JG, Thorne CH, Wisoff JH. The “back to sleep campaign” and deformational plagiocephaly: is there cause for concern? J Craniofac Surg 1996;7:12-8. 5. Mulliken JB, Vander Woude DL, Hansen M, LaBrie RA, Scott RM. Analysis of posterior plagiocephaly: deformational versus synostotic. Plast Reconstr Surg 1999;103:371-80. 6. Argenta L, David L, Thompson J. Clinical classification of positional plagiocephaly. J Craniofac Surg 2004;15:368-72. 7. Cunningham ML, Heike CL. Evaluation of the infant with an abnormal skull shape. Curr Opin Pediatr 2007;19:645-51. 8. Ruiz-Correa S, Sze RW, Starr JR, Lin HT, Speltz ML, Cunningham ML, et al. New scaphocephaly severity indices of sagittal craniosynostosis: a comparative study with cranial index quantifications. Cleft Palate Craniofac J 2006;43:211-21. 9. Mortenson PA, Steinbok P. Quantifying positional plagiocephaly: reliability and validity of anthropometric measurements. J Craniofac Surg 2006;17:413-9. 10. van Vlimmeren LA, Takken T, van Adrichem LN, van der Graaf Y, Helders PJ, Engelbert RH. Plagiocephalometry: a non-invasive method to quantify asymmetry of the skull; a reliability study. Eur J Pediatr 2006;165:149-57. 11. Spermon J, Spermon-Marijnen R, Scholten-Peeters W. Clinical classification of deformational plagiocephaly according to Argenta: a reliability study. J Craniofac Surg 2008;19:664-8. 12. McGarry A, Dixon MT, Greig RJ, Hamilton DR, Sexton S, Smart H. Head shape measurement standards and cranial orthoses in the treatment of infants with deformational plagiocephaly: a systematic review. Dev Med Child Neurol 2008;50:568-76. 13. Wilbrand JF, Wilbrand M, Pons-Kuehnemann J, Blecher JC, Christophis P, Howaldt HP, et al. Value and reliability of anthropometric measurements of cranial deformity in early childhood. J Craniomaxillofac Surg 2011;39:24-9.
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December 2012 14. Kolar JC, Salter EM. Craniofacial anthropometry. Springfield (IL): Charles C Thomas; 1997. 15. Farkas LG. Anthropometry of the head and face. 2nd ed. Toronto (Canada): Raven Press; 1994. 16. Hutchison BL, Hutchison LA, Thompson JM, Mitchell EA. Quantification of plagiocephaly and brachycephaly in infants using a digital photographic technique. Cleft Palate Craniofac J 2005;42:539-47. 17. Loveday BP, de Chalain TB. Active counterpositioning or orthotic device to treat positional plagiocephaly? J Craniofac Surg 2001;12: 308-13. 18. Blecher JC, Howaldt HP. Treatment of non-synostotic, pediatric skull deformities with dynamic head orthosis. Mund Kiefer Gesichtschir 1998;2(Suppl 1):S81-5. 19. Pollack IF, Losken HW, Fasick P. Diagnosis and management of posterior plagiocephaly. Pediatrics 1997;99:180-5.
Clinical Classification of Infant Nonsynostotic Cranial Deformity
ORIGINAL ARTICLES 20. Neufeld S, Birkett S. What to do about flat heads: preventing and treating positional occipital flattening. Axone 2000;22:29-31. 21. Moss SD. Nonsurgical, nonorthotic treatment of occipital plagiocephaly: what is the natural history of the misshapen neonatal head? J Neurosurg 1997;87:667-70. 22. Kluba S, Kraut W, Reinert S, Krimmel M. What is the optimal time to start helmet therapy? Plast Reconstr Surg 2011;128:492-8. 23. 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. J Craniofac Surg 2010;21:1677-82. 24. Wilbrand JF, Szczukowski A, Blecher JC, Pons-Kuehnemann J, Christophis P, Howaldt HP, et al. Objectification of cranial vault correction for craniosynostosis by three-dimensional photography. J Craniomaxillofac Surg 2012; doi: 10.1016/J.jcms.2012.01.007.
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Circumference, cm Group 0-3 mo (M = 69, F = 56)
4-6 mo (M = 56, F = 47)
CVA, cm
Obs-Obs, cm
Nas-Ini, cm
Cranial Index, %
CVAI, %
Percentiles
M
F
M
F
M
F
M
F
M
F
M
F
M
F
M
F
3 10 25 50 75 90 97 3 10 25 50 75 90 97 3 10 25 50 75 90 97 3 10 25 50 75 90 97
33.6 35.5 36.5 37.7 39 40.5 42.5 38.2 40 41 42.6 43.3 44 45.1 40.7 42.3 43.6 44.8 45.5 46.4 46.9 42.3 44.3 45.4 46.3 47.5 48.6 49.7
31.6 34.8 35.9 37.4 38.7 39.6 40.1 37.3 38.3 39.4 41 41.8 43.2 43.8 40.4 41.7 42.2 43.5 44 45.2 46 42.7 43.3 44.3 45 46 46.9 47.8
8.2 8.9 9.6 10 10.6 11.4 12.2 10.1 10.8 11.2 11.5 12 12.5 12.7 10.3 11.2 11.8 12 12.6 13.1 13.4 10.9 12 12.2 12.6 13 13.9 14.6
8.5 9.2 9.5 10.2 10.4 10.9 11.4 9.8 10.4 10.6 11.2 11.6 12.1 12.9 10.6 11 11.2 11.8 12 12.1 12.4 11.2 11.4 11.8 12.2 12.6 12.9 13.5
11.2 11.4 12.2 12.4 13 13.4 14 12.1 13 13.4 13.8 14.4 14.6 14.8 13.3 13.7 14.2 14.8 15.2 15.6 16.1 13.3 13.9 14.8 15.4 16 16.4 17.2
10.8 11.3 11.8 12.4 12.8 13.4 13.7 11.2 12.4 12.8 13.4 13.8 14.2 14.7 13.2 13.2 13.9 14.4 15.1 15.4 15.6 13.5 14.1 14.6 15.2 15.5 16.1 16.3
0 0 0.2 0.2 0.4 0.6 0.8 0 0 0.2 0.4 0.6 0.8 0.9 0 0 0.2 0.2 0.4 0.7 0.8 0 0 0.2 0.2 0.4 0.6 0.7
0 0 0.2 0.2 0.4 0.6 0.7 0 0 0.2 0.2 0.4 0.6 0.9 0 0.1 0.2 0.4 0.4 0.7 0.8 0 0.1 0.2 0.2 0.4 0.6 0.8
18.4 19.2 20.2 21.2 22 23.2 24.2 20.8 22.4 23.2 24.2 25 25.9 27.6 22.6 24 24.4 25.7 26.4 27.4 28 22.6 25.2 26 26.8 27.5 28.6 30.3
17.5 18.5 19.6 21 22 22.9 24.3 17 21.8 22.4 23.5 24.4 25.2 28.5 22 23.8 24.2 24.7 26 26.7 27.2 23 24.5 25 25.8 26.8 28.2 33.1
18.4 20.3 21.2 22.2 22.9 24.4 27.2 20.8 22.7 24.3 25.9 26.8 27.4 27.8 23.1 24.3 26.2 27.5 29 30.2 31.4 21.7 26.1 27.5 29.4 30.6 31.5 32.9
17 18.5 20 22.1 23.3 24.5 25.9 22.5 23.3 24.4 25.4 27 28 29.4 21.2 24.5 25.2 26.6 28 29.2 29.3 24.8 26 27.6 28.3 30 30.6 35.6
67.9 72.3 77 79 85.7 91.8 98.4 74.9 78.5 79.5 83.5 87 93 95.9 72.4 74.7 77.6 82.2 87 91.4 96.6 73.5 75.5 78.6 82.1 86.5 92.1 97
72.7 75.7 78 79.7 85.4 87.2 90.3 70.3 76.8 79.4 83.6 86.6 92.9 99.8 72.3 74.8 77.2 80.2 82.9 89 90.9 72.5 74 76.5 80.5 85.9 89.5 94.8
0 0 1.6 1.9 3.7 5.2 7 0 0 1.6 3.1 4.7 6.6 7.3 0 0 1.4 1.6 3.2 5.9 11.1 0 0 1.4 1.5 2.9 4.4 5.3
0 0 1.7 1.9 3.6 5.4 6.9 0 0 1.5 1.7 3.2 5.6 9.4 0 0.4 1.5 2.9 3.2 5.9 10 0 1.4 1.5 1.6 3.2 4.7 6
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10-12 mo (M = 55, F = 43)
Length, cm
7-9 mo (M = 43, F = 32)
Width, cm
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Table. Anthropometric measurements and derived indices during the first year of life in 401 healthy white children
CVA, cranial vault asymmetry; Obs, otobasion; Nas, nasion; Ini, inion. Circumference indicates head circumference. The median values are given in bold.
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