Clinical and radiological assessment of scoliosis in Koolen-de Vries syndrome.

The Koolen-de Vries syndrome (KdVS) is a multisystem disorder characterized by developmental delay, intellectual disability, characteristic facial features, epilepsy, cardiovascular and urogenital malformations, and various musculoskeletal disorders. Scoliosis is a common feature. The aim of this study is to fill the gap in the current knowledge about scoliosis in individuals with KdVS and to provide recommendations for management and follow-up. In total, 54 individuals with KdVS were included in the study, with a mean age of 13.6 years (range 1.9-38.8 years). Spine radiographs, MR scans, and corresponding radiology reports were analyzed retrospectively for scoliosis and additional anomalies. The presence of scoliosis-related clinical conditions was assessed in participants' medical records and by use of a parent survey. Scoliosis was present in 56% of the participants (30/54) with a mean age of onset of 10.6 years and curve progression during the growth spurt. Prevalence at age 6, 10, and 18 years was, respectively, 9%, 41%, and 65%. Most participants were diagnosed with a single curve (13/24, 54%), of which five participants had a long C-curve type scoliosis. No significant risk factors for development of scoliosis could be identified. Severity was mostly classified as mild, although 29% (7/24) of the curves were larger than 30° at last follow-up. Bracing therapy was received in 13% (7/54), and surgical spinal fusion was warranted in 6% (3/54). Remarkably, participants with scoliosis received less often physical therapy compared to participants without scoliosis (P = 0.002). Scoliosis in individuals with KdVS should be closely monitored and radiologic screening for scoliosis and vertebrae abnormalities is recommended at diagnosis of KdVS, and the age of 10 and 18 years.

Bone Health in Adults With Prader-Willi Syndrome: Clinical Recommendations Based on a Multicenter Cohort Study.

CONTEXT: Prader-Willi syndrome (PWS) is a rare complex genetic syndrome, characterized by delayed psychomotor development, hypotonia, and hyperphagia. Hormone deficiencies such as hypogonadism, hypothyroidism, and growth hormone deficiency are common. The combination of hypotonia, low physical activity, and hypogonadism might lead to a decrease in bone mass and increase in fracture risk. Moreover, one would expect an increased risk of scoliosis due to hypotonia and low physical activity. OBJECTIVE: To study the prevalence and risk factors for skeletal problems (reduced bone mineral density, fractures, and scoliosis) in adults with PWS. METHODS: We retrospectively collected patient characteristics, medical history, medication, biochemical measurements, dual-energy X-ray absorptiometry scans, and spinal X-rays and reviewed the current literature. RESULTS: We included 354 adults with PWS (median age 31 years; 43% males), of whom 51 (14%) had osteoporosis (T-score below -2.5) and 143 (54%) had osteopenia (T-score -1 to -2.5). The most prevalent modifiable risk factors for osteoporosis were hypogonadism, insufficient dairy intake, sedentary lifestyle, and corticosteroid use. Male sex was associated with osteoporosis (P = .005). Growth hormone treatment was not associated with osteoporosis. A history of vertebral fractures was present in 10 (3%) and nonvertebral fractures in 59 (17%). Scoliosis was present in 263 (80%), but no modifiable risk factors were identified. CONCLUSION: Besides scoliosis, osteoporosis is common in adults with PWS. Based on the literature and the risk factors for osteoporosis found in our cohort, we provide practical clinical recommendations to avoid skeletal complications in these vulnerable patients.

The course of skull deformation from birth to 5 years of age: a prospective cohort study.

In a continuation of a prospective longitudinal cohort study in a healthy population on the course of skull shape from birth to 24 months, at 5 years of age, 248 children participated in a follow-up assessment using plagiocephalometry (ODDI-oblique diameter difference index, CPI-cranio proportional index). Data from the original study sampled at birth, 7 weeks, 6, 12, and 24 months were used in two linear mixed models. MAIN FINDINGS: (1) if deformational plagiocephaly (ODDI <104%) and/or positional preference at 7 weeks of age are absent, normal skull shape can be predicted at 5 years of age; (2) if positional preference occurs, ODDI is the highest at 7 weeks and decreases to a stable lowest value at 2 and 5 years of age; and (3) regarding brachycephaly, all children showed the highest CPI at 6 months of age with a gradual decrease over time. CONCLUSION: The course of skull deformation is favourable in most of the children in The Netherlands; at 5 years of age, brachycephaly is within the normal range for all children, whereas the severity of plagiocephaly is within the normal range in 80%, within the mild range in 19%, and within the moderate/severe range in 1%. Medical consumption may be reduced by providing early tailored counselling. What is Known: • Skull deformation prevalence increased after recommendations against Sudden Infant Death Syndrome, little is known about the longitudinal course. • Paediatric physical therapy intervention between 2 and 6 months of age reduces deformational plagiocephaly at 6 and 12 months of age. What is New: • The course of skull deformation is favourable in most of the children in The Netherlands; at 5 years of age, deformational brachycephaly is within the normal range for all children, whereas the severity of deformational plagiocephaly is within the normal range in 80%, within the mild range in 19%, and within the moderate to severe range in only 1%. • Paediatric physical therapy intervention does not influence the long-term outcome; it only influences the earlier decrease of the severity of deformational plagiocephaly.

Insufficient vitamin D supplement use during pregnancy and early childhood: a risk factor for positional skull deformation.

Vitamin D insufficiency during pregnancy is associated with disturbed skeletal homeostasis during infancy. Our aim was to investigate the influence of adherence to recommendations for vitamin D supplement intake of 10 µg per day (400 IU) during pregnancy (mother) and in the first months of life (child) on the occurrence of positional skull deformation of the child at the age of 2 to 4 months. In an observational case-control study, two hundred seventy-five 2- to 4-month-old cases with positional skull deformation were compared with 548 matched controls. A questionnaire was used to gather information on background characteristics and vitamin D intake (food, time spent outdoors and supplements). In a multiple variable logistic regression analysis, insufficient vitamin D supplement intake of women during the last trimester of pregnancy [adjusted odds ratio (aOR) 1.86, 95% (CI) 1.27-2.70] and of children during early infancy (aOR 7.15, 95% CI 3.77-13.54) were independently associated with an increased risk of skull deformation during infancy. These associations were evident after adjustment for the associations with skull deformation that were present with younger maternal age and lower maternal education, shorter pregnancy duration, assisted vaginal delivery, male gender and milk formula consumption after birth. Our findings suggest that non-adherence to recommendations for vitamin D supplement use by pregnant women and infants are associated with a higher risk of positional skull deformation in infants at 2 to 4 months of age. Our study provides an early infant life example of the importance of adequate vitamin D intake during pregnancy and infancy.

Is Motor Performance in 5.5-Year-Old Children Associated with the Presence of Generalized Joint Hypermobility?

OBJECTIVE: To determine the prevalence of generalized joint hypermobility (GJH) in Dutch children aged 5.5 years, and to examine the association between GJH and motor performance and development over time. STUDY DESIGN: A prospective cohort of 249 children was recruited. GJH was assessed with the Beighton test at age 5.5 years. Motor performance was evaluated at age 2.0 years using the Bayley Scales of Infant Development, Second Edition and at age 5.5 years using the Movement Assessment Battery for Children-Second Edition (subscore categories: manual dexterity, aiming and catching, and static and dynamic balance). RESULTS: In 249 children, the prevalence of GJH, defined by the Beighton test score, was 34.1% for a score ≥ 4, 22.5% for a score ≥ 5, and 16.5% for a score ≥ 6. No significant association was found between GJH and total motor performance. Manual dexterity in girls (Beighton score ≥ 4) was positively associated with higher level of motor performance (ß [SE] = 0.38 [0.17]; P = .028), ranging from +0.04 SD to +0.72 SD, even after correction for covariates. A significant interaction between GJH and body mass index (BMI) growth was found, indicating that the effect of GJH on the rate of development of motor performance declines with increasing BMI growth (ß = 0.05 [0.02]; P = .031). CONCLUSION: In this healthy pediatric cohort, GJH was present in one-third of the sample, and no significant association was found between GJH and total motor performance. The effect of GJH on the rate of development of motor performance appears to decline with increasing BMI growth. Longitudinal prospective studies are recommended to detect influences of GJH on motor performance over time, as well as the influence of body composition and Beighton cutoff points.

Helmet therapy in infants with positional skull deformation: randomised controlled trial.

OBJECTIVE: To determine the effectiveness of helmet therapy for positional skull deformation compared with the natural course of the condition in infants aged 5-6 months. DESIGN: Pragmatic, single blinded, randomised controlled trial (HEADS, HElmet therapy Assessment in Deformed Skulls) nested in a prospective cohort study. SETTING: 29 paediatric physiotherapy practices; helmet therapy was administered at four specialised centres. PARTICIPANTS: 84 infants aged 5 to 6 months with moderate to severe skull deformation, who were born after 36 weeks of gestation and had no muscular torticollis, craniosynostosis, or dysmorphic features. Participants were randomly assigned to helmet therapy (n=42) or to natural course of the condition (n=42) according to a randomisation plan with blocks of eight. INTERVENTIONS: Six months of helmet therapy compared with the natural course of skull deformation. In both trial arms parents were asked to avoid any (additional) treatment for the skull deformation. MAIN OUTCOME MEASURES: The primary outcome was change in skull shape from baseline to 24 months of age assessed using plagiocephalometry (anthropometric measurement instrument). Change scores for plagiocephaly (oblique diameter difference index) and brachycephaly (cranioproportional index) were each included in an analysis of covariance, using baseline values as the covariate. Secondary outcomes were ear deviation, facial asymmetry, occipital lift, and motor development in the infant, quality of life (infant and parent measures), and parental satisfaction and anxiety. Baseline measurements were performed in infants aged between 5 and 6 months, with follow-up measurements at 8, 12, and 24 months. Primary outcome assessment at 24 months was blinded. RESULTS: The change score for both plagiocephaly and brachycephaly was equal between the helmet therapy and natural course groups, with a mean difference of -0.2 (95% confidence interval -1.6 to 1.2, P=0.80) and 0.2 (-1.7 to 2.2, P=0.81), respectively. Full recovery was achieved in 10 of 39 (26%) participants in the helmet therapy group and 9 of 40 (23%) participants in the natural course group (odds ratio 1.2, 95% confidence interval 0.4 to 3.3, P=0.74). All parents reported one or more side effects. CONCLUSIONS: Based on the equal effectiveness of helmet therapy and skull deformation following its natural course, high prevalence of side effects, and high costs associated with helmet therapy, we discourage the use of a helmet as a standard treatment for healthy infants with moderate to severe skull deformation. TRIAL REGISTRATION: Current Controlled Trials ISRCTN18473161.

Response to pediatric physical therapy in infants with positional preference and skull deformation.

BACKGROUND: Pediatric physical therapy seems to reduce skull deformation in infants with positional preference. However, not all infants show improvement. OBJECTIVE: The study objective was to determine which infant and parent characteristics were related to responses to pediatric physical therapy in infants who were 2 to 4 months old and had positional preference, skull deformation, or both. DESIGN: This was a prospective cohort study. METHODS: Infants who were 2 to 4 months old and had positional preference, skull deformation, or both were recruited by pediatric physical therapists at the start of pediatric physical therapy. The primary outcome was a good response or a poor response (moderate or severe skull deformation) at 4.5 to 6.5 months of age. Potential predictors for responses to pediatric physical therapy were assessed at baseline with questionnaires, plagiocephalometry, and the Alberta Infant Motor Scale. Univariate and multiple logistic regression analyses with a stepwise backward elimination method were performed. RESULTS: A total of 657 infants participated in the study. At follow-up, 364 infants (55.4%) showed a good response to therapy, and 293 infants (44.6%) showed a poor response. Multiple logistic regression analysis resulted in the identification of several significant predictors for a poor response to pediatric physical therapy at baseline: starting therapy after 3 months of age (adjusted odds ratio [aOR]=1.50, 95% confidence interval [95% CI]=1.04-2.17), skull deformation (plagiocephaly [aOR=2.64, 95% CI=1.67-4.17] or brachycephaly [aOR=3.07, 95% CI=2.09-4.52]), and a low parental satisfaction score (aOR=2.64, 95% CI=1.67-4.17). A low parental satisfaction score indicates low parental satisfaction with the infant's head shape. LIMITATIONS: Information about pediatric physical therapy was collected retrospectively and included general therapy characteristics. Because data were collected retrospectively, no adjustment in therapy for individual participants could be made. CONCLUSIONS: Several predictors for responses to pediatric physical therapy in infants who were 2 to 4 months old and had positional preference, skull deformation, or both were identified. Health care professionals can use these predictors in daily practice to provide infants with more individualized therapy, resulting in a better chance for a good outcome.

Functional, motor developmental, and long-term outcome after the component separation technique in children with giant omphalocele: a case control study.

BACKGROUND/PURPOSE: The objective of this study was to evaluate the long-term functional and motor development and abdominal muscle quantity in children operated on for giant omphalocele (GOC) with the Component Separation Technique (CST). METHODS: Between 2004 and 2007, CST was applied in eleven consecutive infants with GOC. Eight underwent ultrasound of the abdominal wall and muscles, assessment of functional and motor development using the Movement Assessment Battery for Children, 2nd Edition (M-ABC-2), and an observational physical examination focused on possible abnormalities in stature and movements related to GOC. Findings were compared with those in age-matched controls. The parents filled in a questionnaire on the children's functioning in daily life. RESULTS: The mean age at evaluation was 71 months (range, 42-141 months) with a median time of follow-up of 54 months (range, 38-84 months). Ultrasound of the abdominal wall muscles showed normal muscle thickness. In seven of the eight children, a rectus diastasis was seen without any protrusion. The MABC-2 was within the normal range, and stature and motor coordination did not differ from those in controls. CONCLUSIONS: After 4.5 years, these children show normal thickness of all abdominal wall muscles and motor function within the normal range, despite a rectus diastasis. The CST seems to be a promising closure technique for GOC.

Objective evaluation of muscle strength in infants with hypotonia and muscle weakness.

The clinical evaluation of an infant with motor delay, muscle weakness, and/or hypotonia would improve considerably if muscle strength could be measured objectively and normal reference values were available. The authors developed a method to measure muscle strength in infants and tested 81 typically developing infants, 6-36 months of age, and 17 infants with Prader-Willi Syndrome (PWS) aged 24 months. The inter-rater reliability of the measurement method was good (ICC=.84) and the convergent validity was confirmed by high Pearson's correlations between muscle strength, age, height, and weight (r=.79-.85). A multiple linear regression model was developed to predict muscle strength based on age, height, and weight, explaining 73% of the variance in muscle strength. In infants with PWS, muscle strength was significantly decreased. Pearson's correlations showed that infants with PWS in which muscle strength was more severely affected also had a larger motor developmental delay (r=.75).

HElmet therapy Assessment in infants with Deformed Skulls (HEADS): protocol for a randomised controlled trial.

BACKGROUND: In The Netherlands, helmet therapy is a commonly used treatment in infants with skull deformation (deformational plagiocephaly or deformational brachycephaly). However, evidence of the effectiveness of this treatment remains lacking. The HEADS study (HElmet therapy Assessment in Deformed Skulls) aims to determine the effects and costs of helmet therapy compared to no helmet therapy in infants with moderate to severe skull deformation. METHODS/DESIGN: Pragmatic randomised controlled trial (RCT) nested in a cohort study. The cohort study included infants with a positional preference and/or skull deformation at two to four months (first assessment). At 5 months of age, all children were assessed again and infants meeting the criteria for helmet therapy were asked to participate in the RCT. Participants were randomly allocated to either helmet therapy or no helmet therapy. Parents of eligible infants that do not agree with enrolment in the RCT were invited to stay enrolled for follow up in a non-randomisedrandomised controlled trial (nRCT); they were then free to make the decision to start helmet therapy or not. Follow-up assessments took place at 8, 12 and 24 months of age. The main outcome will be head shape at 24 months that is measured using plagiocephalometry. Secondary outcomes will be satisfaction of parents and professionals with the appearance of the child, parental concerns about the future, anxiety level and satisfaction with the treatment, motor development and quality of life of the infant. Finally, compliance and costs will also be determined. DISCUSSION: HEADS will be the first study presenting data from an RCT on the effectiveness of helmet therapy. Outcomes will be important for affected children and their parents, health care professionals and future treatment policies. Our findings are likely to influence the reimbursement policies of health insurance companies.Besides these health outcomes, we will be able to address several methodological questions, e.g. do participants in an RCT represent the eligible target population and do outcomes of the RCT differ from outcomes found in the nRCT? TRIAL REGISTRATION: ISRCTN18473161.

The effect of growth hormone treatment or physical training on motor performance in Prader-Willi syndrome: a systematic review.

Although motor problems in Prader-Willi syndrome (PWS) are prominent in infants, and continue into childhood and adulthood, there is little insight into the factors important for clinical management. The literature was reviewed to: (1) provide an overview of the characteristics and prevalence of motor problems and (2) evaluate the effects of growth hormone (GH) treatment and physical training on motor performance. A systematic search revealed 34 papers: 13 on motor performance; 12 on GH treatment; and nine on physical training. In infants, motor development is 30-57% of the normal reference values, and children and adults also have significant problems in skill acquisition, muscle force, cardiovascular fitness, and activity level. GH treatment positively influenced motor performance in infants, children, and adults, although not all studies demonstrated an effect. All studies on physical training demonstrated beneficial effects in PWS patients. We suggest a combination of GH treatment and physical training to be started as soon as possible, especially in infants, to improve motor development as this will positively influence general development.

Motor problems in Prader-Willi syndrome: a systematic review on body composition and neuromuscular functioning.

Motor problems in Prader-Willi syndrome (PWS) are presumably related to abnormal body composition and certain neuromuscular abnormalities. The authors reviewed the literature to evaluate the extent to which body composition is affected and gathered all findings on neuromuscular functioning in PWS. A systematic review was conducted in four databases (1956-2010). The methodological quality of each included article was evaluated. Thirty-eight papers were included: body composition (9 studies), neuromuscular functioning (7) and growth hormone (GH) effect studies (23). Increased fat mass and decreased lean body mass are characteristics of PWS. As a result, muscle mass is decreased by 25-37%, which might explain partly the weakness and hypotonia. However, there are also structural and functional muscle abnormalities, and cortical motor areas are hypo-excitable in PWS patients. Moreover, disuse as result of decreased activity in PWS could also contribute. GH treatment positively influences body composition, but does not normalize it. Training could prevent disuse and improves body composition. Therefore GH treatment and training will probably enhance one another.

Effect of pediatric physical therapy on deformational plagiocephaly in children with positional preference: a randomized controlled trial.

OBJECTIVE: To study the effect of pediatric physical therapy on positional preference and deformational plagiocephaly. DESIGN: Randomized controlled trial. SETTING: Bernhoven Hospital, Veghel, the Netherlands. PARTICIPANTS: Of 380 infants referred to the examiners at age 7 weeks, 68 (17.9%) met criteria for positional preference, and 65 (17.1%) were enrolled and followed up at ages 6 and 12 months. INTERVENTION: Infants with positional preference were randomly assigned to receive either physical therapy (n = 33) or usual care (n = 32). MAIN OUTCOME MEASURES: The primary outcome was severe deformational plagiocephaly assessed by plagiocephalometry. The secondary outcomes were positional preference, motor development, and cervical passive range of motion. RESULTS: Both groups were comparable at baseline. In the intervention group, the risk for severe deformational plagiocephaly was reduced by 46% at age 6 months (relative risk, 0.54; 95% confidence interval, 0.30-0.98) and 57% at age 12 months (0.43; 0.22-0.85). The numbers of infants with positional preference needed to treat were 3.85 and 3.13 at ages 6 and 12 months, respectively. No infant demonstrated positional preference at follow-up. Motor development was not significantly different between the intervention and usual care groups. Cervical passive range of motion was within the normal range at baseline and at follow-up. When infants were aged 6 months, parents in the intervention group demonstrated significantly more symmetry and less left orientation in nursing, positioning, and handling. CONCLUSION: A 4-month standardized pediatric physical therapy program to treat positional preference significantly reduced the prevalence of severe deformational plagiocephaly compared with usual care. CLINICAL TRIAL REGISTRATION: isrctn.org Identifier: ISRCTN84132771.

Validation of a simple method for measuring cranial deformities (plagiocephalometry).

Craniofacial measuring is essential for diagnosis or evaluation of growth and therapies. Skull deformities in children are mainly caused by craniosynostosis or by external pressure in positional skull deformations. Traditional anthropometry does not sufficiently analyze craniofacial shape. In computed tomography (CT) scanning, radiation loads are considerable. Both CT and magnetic resonance imaging (MRI) scanning require anaesthesia in children for accurate imaging, due to their long acquisition time. This makes CT and MRI unsuitable for long term follow-up of pediatric patients unless there is a compelling reason. Other noninvasive three-dimensional (3D) surface scanners still have limited practical use. van Vlimmeren et al presented plagiocephalometry (PCM) as a simple and versatile instrument to quantify skull deformities with high intrarater and interrater reliability, but no comparison was made with the actual skull shape. At the Erasmus University Medical Center Rotterdam, Sophia Children's Hospital PCM was compared to 3D-CT scanning in 21 children with craniosynostosis early in life. The PCM ring proved to fit closely to the skin with mean differences less than 1 mm (P < 0.05). The shape of the PCM ring was not significantly changed when taken off the head (P > 0.05). Finally, no significant differences are shown between measurements on the skull (CT-scan) and PCM ring off the head (P > 0.05). This study proves that PCM is a reliable method for analysis of skull deformities. The measurements are in agreement with 3D-CT scanning as golden standard. Although only 2-dimensional measurements are performed by PCM, the combination of simplicity, reliability, and validity make it a promising tool for daily practice.

Risk factors for deformational plagiocephaly at birth and at 7 weeks of age: a prospective cohort study.

OBJECTIVE: The purpose of this work was to identify risk factors for deformational plagiocephaly within 48 hours of birth and at 7 weeks of age. PATIENTS AND METHODS: This was a prospective cohort study in which 380 healthy neonates born at term in Bernhoven Hospital in Veghel were followed at birth and at 7 weeks of age. Data regarding obstetrics, sociodemographics, asymmetry of the skull, anthropometrics, motor development, positioning, and care factors related to potentially provoking deformational plagiocephaly were gathered, with special interest for putative risk factors. The main outcome measure at birth and at 7 weeks of age was deformational plagiocephaly, assessed using the plagiocephalometry parameter oblique diameter difference index, a ratio variable, calculated as the longest divided by the shortest oblique diameter of the skull x 100%. A cutoff point of > or = 104% was used to indicate severe deformational plagiocephaly. RESULTS: Only in 9 of 23 children who presented deformational plagiocephaly at birth was deformational plagiocephaly present at follow-up, whereas in 75 other children, deformational plagiocephaly developed between birth and follow-up. At birth, 3 of 14 putative risk factors were associated with severe flattening of the skull: gender, birth rank, and brachycephaly. At 7 weeks of age, 8 of 28 putative risk factors were associated with severe flattening: gender, birth rank, head position when sleeping, position on chest of drawers, method of feeding, positioning during bottle-feeding, and tummy time when awake. Early achievement of motor milestones was a protective factor for developing deformational plagiocephaly. Deformational plagiocephaly at birth was not a predictor for deformational plagiocephaly at 7 weeks of age. There was no significant relation between supine sleeping and deformational plagiocephaly. CONCLUSIONS: Three determinants were associated with an increased risk of deformational plagiocephaly at birth: male gender, first-born birth rank, and brachycephaly. Eight factors were associated with an increased risk of deformational plagiocephaly at 7 weeks of age: male gender, first-born birth rank, positional preference when sleeping, head to the same side on chest of drawers, only bottle feeding, positioning to the same side during bottle feeding, tummy time when awake < 3 times per day, and slow achievement of motor milestones. This study supports the hypothesis that specific nursing habits, as well as motor development and positional preference, are primarily associated with the development of deformational plagiocephaly. Earlier achievement of motor milestones probably protects the child from developing deformational plagiocephaly. Implementation of practices based on this new evidence of preventing and diminishing deformational plagiocephaly in child health care centers is very important.

Plagiocephalometry: a non-invasive method to quantify asymmetry of the skull; a reliability study.

UNLABELLED: Deformational plagiocephaly (DP) in newborns and very young children is a common problem in daily practice. The intrarater and interrater reliability of plagiocephalometry (PCM), a new, non-invasive, inexpensive instrument to assess and quantify the asymmetry of the skull, is evaluated at the outpatient Department of Physical Therapy of the Bernhoven Hospital at Veghel, The Netherlands. Using a thermoplastic material to mould the outline of the infant's skull, a reproduction of the skull shape is performed on paper, allowing for accurate cephalometric measurements. Fifty children (aged 0-24 months), with or without positional preference of the head, and with or without DP, were measured three times by two separate, experienced pediatric physical therapists. Intraclass correlation coefficients (ICC) regarding the measurements of the drawn lines were all above 0.92 (intrarater reliability) and 0.90 (interrater reliability). The ICCs of the plagiocephaly indicators ear deviation (ED), antero-sinistra-antero-dextra (ASAD), postero-dextra-postero-sinistra (PDPS) and oblique diameter difference (ODD) were 0.88, 0.57, 0.92 and 0.96, respectively, for the intrarater reliability and 0.90, 0.65, 0.94 and 0.96, respectively, for the interrater reliability. The ICCs of the two indices oblique diameter difference index (ODDI) and cranial proportional index (CPI) were 0.97 and 0.96, respectively, for the intrarater reliability and 0.95 and 0.92, respectively, for the interrater reliability. The limits of agreement according to Bland Altman, comprising 95% of the differences between two measurements (2 sd), were 4.3 mm (ED), 5.9 mm (ASAD), 3.0 mm (PDPS), 3.4 mm (ODD), 2.7% (ODDI) and 4.5% (CPI) for the intrarater reliability, and 3.7 mm (ED), 5.2 mm (ASAD), 2.4 mm (PDPS), 3.3 mm (ODD), 2.9% (ODDI) and 5.8% (CPI) for the interrater reliability. CONCLUSION: We conclude that PCM is an easy-to-apply, non-invasive and reliable measurement instrument to assess skull asymmetry with good clinical accuracy and low application costs. PCM might serve as an instrument to be used in all levels of care for children with DP, and might provide information concerning the natural course of DP, as well as the assessment of the effects of conservative treatment strategies on DP.

Diagnostic strategies for the evaluation of asymmetry in infancy-a review.

UNLABELLED: Asymmetry in infancy is a diagnosis with a large spectrum of features, expressing an abnormal shape of parts of the body or unequal postures and movements. Symptoms may be structural and/or functional, with localised or generalised expression. Within the last decade, many professionals have focused on the adverse consequences on motor performance of infants, associated with the recommendations on the prevention of sudden infant death. The purpose of the present study was to highlight different aspects of asymmetry in infancy based on best available evidence in the current literature and to present a flow chart illustrating different diagnostic pathways of asymmetry in infancy. CONCLUSION: We conclude that asymmetry in infancy is a diagnosis with a large spectrum of features and a multifactorial aetiology without consensus in definition, nomenclature and classification. Systematic diagnostic management of asymmetry in infancy is indicated. The presented diagnostic flow chart might serve as a basis.