Positional cranial deformation in children: A plea for the efficacy of the cranial helmet in children.

BACKGROUND: Cranial deformations have historically aroused the interest of people worldwide. One of the more debated points about positional plagiocephaly is the choice of the treatment. In this article, the senior author (CM) shares his experience on almost 30 years of use of the helmet molding therapy in children with deformation plagiocephaly. METHODS AND POPULATION: We retrospectively and systematically reviewed the cases of 2188 patients (75% males and 25% females) presenting positional head deformity and treated between 1991 and 2013 with a cranial helmet. To assess the effectiveness of the helmet, we compared the cranial index in bilateral plagiocephaly and the Cranial Diagonals Difference (CDD) in unilateral plagiocephaly at the beginning and at the end of the treatment. RESULTS: The cranial indexes ranged between 94.4% and 124.2% before the treatment and decreased significantly between 86.8% and 121.4% after the treatment (P<0.01). The CDD ranged between 0.3 cm and 4.5 cm with an average of 1.50±0.54 cm before the treatment and decreased significantly between 0.1 cm and 2.5 cm with an average of 0.72±0.37 cm after the treatment (P<0.01). For unilateral plagiocephaly, at the beginning of the treatment, 2.5% children presented a mild plagiocephaly, 19.6% a moderate plagiocephaly and 77.9% a severe plagiocephaly. At the end of the treatment, the deformation was classified as mild in 40.2% children, moderate in 44.3% children and severe in 15.5% children with significantly less children in the most severe subgroups (P<0.01) Facial symmetry pre-existed before the treatment in 13.7% of children. This rate was significantly increased at the end of the treatment to 66.7% (P<0.01). In only 8 cases (0.2%), the helmet therapy did not allow to obtain correct clinical results and a surgical posterior cranial remodeling was performed. CONCLUSIONS: The results observed in this series confirms that cranial helmet is a simple and well tolerated alternative which bring satisfying results. Its success implies a good collaboration with parents and a management both by orthoptist, physiotherapist and doctor. Nevertheless, it remains many controversies in the literature concerning in particular long-term cosmetic and functional outcomes. A long-term multicentric prospective study could enable to remove doubts.

Identification and Management of Cranial Anomalies in Perinatology.

Neonatal skull and head shape anomalies are rare. The most common cranial malformations encountered include craniosynostosis, deformational plagiocephaly, cutis aplasia, and encephalocele. Improved prenatal imaging can diagnose morphologic changes as early as the second trimester. Prenatal identification also provides perinatologists and neonatologists with valuable information that helps to optimize care during and after delivery. Cranial anomalies require a multidisciplinary team approach and occasionally a lifetime of care. Today, care begins with the perinatologist as many cranial anomalies can be identified in utero with recent advances in prenatal testing.

[Synostosic and non-synostosic cranial deformities]. / Deformidades craneales sinostósicas y no sinostósicas.

In the last decades alterations in the skull shape have increased at the expense of plagiocephaly, as consequence of the American Academy of Pediatrics recommendations to sleep the infant in the supine position. The clinician must differentiate between positional plagiocephaly and craniosynostosis, since if a cranial synostosis is proven, the therapeutic behavior will be potentially neurosurgical. Although three-dimensional skull tomography with bone window is the study of greater sensitivity and diagnostic specificity, the majority of cases can be confirmed by the clinic, reserving the radiography or ultrasound with a suture approach for doubtful cases. Craniosynostosis must be early referral to a craniofacial team to define the indication, opportunity and most appropriate surgical technique, in order to avoid future neurocognitive and psychosocial complications. Children with positional plagiocephaly regardless of the treatment of cranial deformity may have a higher risk of motor development delay. For correction, repositioning and physiotherapy are suggested in mild to moderate forms, reserving the use of cranial orthesis in severe forms.

Deformidades craneales sinostósicas y no sinostósicas / Synostosic and non-synostosic cranial deformities

Las alteraciones de la forma del cráneo han aumentado en las últimas décadas a expensas de las plagiocefalias, como consecuencia de las recomendaciones de la Academia Americana de Pediatría de dormir al lactante en decúbito supino. El clínico debe diferenciar entre plagiocefalia posicional y craneosinostosis, ya que la conducta terapéutica será potencialmente neuroquirúrgica de comprobar una sinostosis craneal. Si bien la tomografía de cráneo tridimensional con ventana ósea es el estudio de mayor sensibilidad y especificidad diagnóstica, la mayoría de los casos pueden confirmarse por la clínica, reservando la radiografía simple o de preferencia la ecografía con enfoque de suturas para casos dudosos. Las craneosinostosis deben derivarse tempranamente a un equipo craneofacial para definir la indicación, oportunidad y técnica quirúrgica más adecuada para evitar futuras complicaciones neurocognitivos y psicosociales. Los niños con plagiocefalias posicionales, independientemente del tratamiento de la deformidad craneal, pueden tener más riesgo de retraso del desarrollo motor. Para su corrección se sugiere reposicionamiento y fisioterapia en formas leves a moderadas, reservando el uso de ortésis craneal en formas graves.

In the last decades alterations in the skull shape have increased at the expense of plagiocephaly, as consequence of the American Academy of Pediatrics recommendations to sleep the infant in the supine position. The clinician must differentiate between positional plagiocephaly and craniosynostosis, since if a cranial synostosis is proven, the therapeutic behavior will be potentially neurosurgical. Although three-dimensional skull tomography with bone window is the study of greater sensitivity and diagnostic specificity, the majority of cases can be confirmed by the clinic, reserving the radiography or ultrasound with a suture approach for doubtful cases. Craniosynostosis must be early referral to a craniofacial team to define the indication, opportunity and most appropriate surgical technique, in order to avoid future neurocognitive and psychosocial complications. Children with positional plagiocephaly regardless of the treatment of cranial deformity may have a higher risk of motor development delay. For correction, repositioning and physiotherapy are suggested in mild to moderate forms, reserving the use of cranial orthesis in severe forms.

Relationship between starting age of cranial-remolding-orthosis therapy and effectiveness of treatment in children with deformational plagiocephaly.

PURPOSE: The aim of this study was to investigate the optimal age for starting cranial-remolding-orthosis therapy in children with deformational plagiocephaly. METHODS: Medical records of 310 patients with deformational plagiocephaly were retrospectively reviewed and the initial and final cranial vault asymmetry index (CVAI), age when starting therapy, duration of therapy, mean change of CVAI, improvement rate, and treatment success were analyzed. We compared outcomes according to the groups divided by ages starting therapy. RESULTS: There were no significant differences in improvement rate and duration of cranial-remolding-orthosis therapy among patients starting therapy at the age of 3, 4, and 5 months. However, when starting therapy after the age of 6 months, the rates of CVAI improvement were significantly lower and the duration of therapy was significantly increased. CONCLUSION: Considering the spontaneous resolution effect according to the head growth nature, the age 5 month is the optimal period to start cranial-remolding-orthosis therapy for deformational plagiocephaly.

Plagiocephaly Severity Scale to Aid in Clinical Treatment Recommendations.

Studies have attempted to categorize infant cranial asymmetry in a variety of ways using both observational and quantitative techniques, but none have created a clinical tool that can serve as a treatment guide based on clinical outcomes. In 2006, a research team from Children's Healthcare of Atlanta published the results of a prospective analysis of 224 patients with cranial asymmetries and their treatment outcomes. As a continuation of the previous work, the researchers have identified a plagiocephaly severity scale based on those outcomes to assist medical professionals who treat patients with cranial abnormalities. Our hypothesis is to validate the proposed severity scale that categorizes the clinical presentation and severity of plagiocephaly.Of the 224 patients enrolled, 207 patients were placed in an experimental group and 17 patients who refused treatment were placed in a control group. Digital head shape data were collected. Cross-correlation matrices were computed across variables and regression models resulted in the identification of 5 meaningful variables. A 5-level clinical classification scale was created. Five 1 × 5 analyses of variance were computed to compare each classification level.Four of the 5 analyses of variance identified significant overall effects for classification. A model was developed from the empirical data and the model was tested for accuracy, resulting in 12.1% overall error. The model was validated for both experimental and control groups.The results show that the severity scale is a meaningful outcome-based scale that assists clinicians in developing a treatment plan for treating plagiocephaly. The scale has been validated across a large heterogeneous study sample.

Nonsynostotic Posterior Brachycephaly with Hindbrain Herniation.

BACKGROUND: Positional plagiocephaly is the most common cause of cranial asymmetry. The underlying cause of Chiari-1 malformation has many possible theories, and anecdotally some pediatric neurosurgeons have had experience of severe cases of positional brachycephaly with Chiari-1. However, to date, there have been no published cases linking nonsynostotic plagiocephaly with Chiari-1 malformation. CASE DESCRIPTION: An 18-month-old boy presented with a head injury. On examination he had a Glasgow Coma Score of 15 with no focal neurologic deficits, but he was noted to have marked posterior brachycephaly. A computed tomography scan showed a slim left-sided hemispheric acute subdural hematoma with no mass effect, which was treated conservatively. Of note, all of his cranial vault sutures were open, and a diagnosis of incidental positional plagiocephaly was made. Subsequent magnetic resonance imaging as part of a work-up to exclude nonaccidental injury showed a small posterior fossa with a steep tentorium and herniation of the cerebellar tonsils to the level of the body of the second cervical vertebra. CONCLUSIONS: Chronic hindbrain herniation is well reported in cases of craniosynostosis, but to our knowledge this is the first published case associated with nonsynostotic deformational plagiocephaly. We hypothesize that severe posterior plagiocephaly can cause disproportion of the posterior fossa: hindbrain volume ratio and acquired chronic cerebellar herniation. Nevertheless, positional plagiocephaly and Chiari-1 are common entities, and it is possible that the dual diagnoses were coincidental in this case. This report serves to raise awareness of a putative causal relationship between positional plagiocephaly, reduced posterior fossa volume, and hindbrain herniation.

Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancy-how serious is it?

PURPOSE: We test the hypothesis that ventriculoperitoneal (VP) shunt insertion significantly increases contralateral positional plagiocephaly. METHODS: We reviewed 339 children who had a VP shunt inserted at Birmingham Children's Hospital between 2006 and 2013, noting laterality of shunt insertion and frontal or occipital position. We ascertained the presence of post-operative positional plagiocephaly using the cranial vault asymmetry index. Multinomial logistic regression modelling was used to examine relationships between plagiocephaly, shunt position, gender and age. Adjusted odds and risk ratios for effect of variables on plagiocephaly were calculated. RESULTS: Children with occipital VP shunts are at significant risk of developing contralateral positional plagiocephaly, particularly in the first 12 months of life. CONCLUSIONS: We recommend careful follow-up and advice regarding head positioning following surgery. There should be consideration for active monitoring to avoid plagiocephaly, including physiotherapy and health visitor interventions. Endoscopic third ventriculostomy in selected cases or anterior shunt placement could be considered. A larger national study would be of interest to evaluate the extent of an otherwise correctable problem.

Deformational plagiocephaly and craniosynostosis: trends in diagnosis and treatment after the "back to sleep" campaign.

BACKGROUND: In 1992, the American Academy of Pediatrics discouraged prone sleeping positions because of its association with sudden infant death syndrome. After this was an increased incidence of deformational plagiocephaly (DP). METHODS: A retrospective review was completed for patients with DP and craniosynostosis seen by plastic surgeons at a tertiary medical center during a 19-year period. Two groups of patients were evaluated before (1988-1995) and after (1996-2007) implementation of the "Back to Sleep" campaign. RESULTS: Of the 5169 patients, those with craniosynostosis (n = 279) had a mean age at initial evaluation before and after 1996 of 12.4 versus 5.6 months (P = 0.0008). There was a trend of decreasing age at initial evaluation and first surgery after 1996. For patients with DP (n = 4890), the mean age at initial evaluation before and after 1996 was 11.5 versus 6.0 months (P = 0.10). There was a trend of decreasing age at initial evaluation and DP correction after 1996. The majority of patients had right-sided DP (50.2%), followed by left-sided (24.7%) and bilateral (18.9%). There was no significant difference in DP correction rate (67% versus 87%) or the mean age that DP was corrected (12.8 versus 11.8 mo) before and after 1996. Compared with 1996 to 1999, there was a 214% and 390% increase in DP referrals from 2000 to 2003 and 2004 to 2007. For craniosynostosis, there was a 27% and 129% increase in referrals. CONCLUSIONS: The increasing incidence of DP since the Back to Sleep campaign is concerning, but a positive outcome is that patients are being referred and treated at a younger age.

Surgical correction for unilateral lambdoid synostosis: a systematic review.

BACKGROUND: Unilateral lambdoid synostosis is considered to be the rarest form of craniosynostosis. Since the introduction of the Sudden Infant Death Syndrome "back to sleep" campaign, the incidence of unilateral lambdoid synostosis was reportedly increasing. This was proven to be false and a consequence of non-specific diagnostic criteria in excluding suture fusion from deformational changes. This, in turn, led to ambiguity in the literature in terms of features and surgical correction in the 1980s to 1990s. OBJECTIVE OF REVIEW: We aimed to navigate the literature for true studies of unilateral lambdoid synostosis and examine the results of their surgical corrections. TYPE OF REVIEW: A systematic review with a defined search strategy. SEARCH STRATEGY: A search on MEDLINE and Google Scholar using strategy: (Unilateral AND ((lambdoid* AND Synostosis) OR (lambdoid* AND Craniosynostosis) OR (Posterior AND Plagiocephaly)) AND (Surgery). EVALUATION METHOD: Articles were reviewed, and data were compiled into tables for analysis. RESULTS: Seventeen studies were included in this review. A total of 188 patients with unilateral lambdoid synostosis were identified. No patients had major complications (venous sinus tear, neurologic injury, or cerebrospinal fluid leakage). Facial asymmetry was widely noted, and only limited improvement was achieved postoperatively. Auricular displacement was variable and often persisted despite surgery. Neurodevelopment did not deteriorate postoperatively and even improved in several patients. Endoscopic techniques revealed shorter periods of hospitalization, reduced mean estimated blood loss, and no blood transfusion requirements. The timing of surgery at 6 to 12 months was found to be the optimal balance in preventing deterioration in neurodevelopment and allowing favorable cranial growth and morphology. CONCLUSIONS: The current evidence is difficult to navigate because of deformational plagiocephaly being misdiagnosed as unilateral lambdoid synostosis. Despite this, we present the only systematic review of all truly identifiable cases of unilateral lambdoid synostosis. Long-term quantitative studies are required to assess the benefits of the various surgical procedures.

Posterior remodeling flap for posterior plagiocephaly.

INTRODUCTION: Nonsynostotic posterior plagiocephaly has become the most common skull deformation since pediatricians have suggested the supine position for the newborns to reduce the risk of sudden death. Prevention of such a "positional" deformation or its management once it has occurred is mainly based on physical maneuvers such as physiotherapy and active positional corrective measures. SELECTION CRITERIA: Surgical correction, however, may be suggested in rare cases where deformation of the skull is so severe or the referral of the child is so late that physical corrective treatment cannot be taken into consideration. Surgical management is based on the creation of a posterior bone flap to be repositioned after the opportune contouring and rotation. PURPOSE: The aim of this paper is to describe the surgical technique used for posterior vault remodeling in posterior plagiocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations.

Deformational plagiocephaly and orthotic treatment: indications and limitations.

PURPOSE: As the incidence of deformational plagiocephaly (DP) has risen, so has the demand on clinicians to make appropriate treatment recommendations. While knowledge of risk factors and natural history continue to evolve, there is uncertainty and controversy regarding intervention approaches. The purpose of this report is to review current treatment approaches for DP, in particular the use of orthotic helmets. METHODS: A narrative literature review was conducted to summarize current evidence supporting preventative measures and re-positional and orthotic interventions. RESULTS: When started early at under 2 months of age, positional efforts and 'tummy time' can be effective in preventing and improving DP, but these strategies need to be better promoted to caregivers. The timing, severity and parental concerns as indicators for orthotic treatment are reviewed. Limitations discussed include high cost, lower level of evidence and poor understanding of long-term outcomes and caregiving implications. CONCLUSION: For optimal outcome, current evidence supports use of repositioning in young infants and start of orthotic helmet treatment for infants with significant DP at 4-6 months. Further research is needed to better understand the parameters for use (stratified by age and severity), long-term outcomes and natural history and the impact on caregiving, as well as the cultural significance of head shape.

Avoiding CT scans in children with single-suture craniosynostosis.

INTRODUCTION: During the last decades, computed tomography (CT) has become the predominant imaging technique in the diagnosis of craniosynostosis. In most craniofacial centers, at least one three-dimensional (3D) computed tomographic scan is obtained in every case of suspected craniosynostosis. However, with regard to the risk of radiation exposure particularly in young infants, CT scanning and even plain radiography should be indicated extremely carefully. MATERIAL AND METHODS: Our current diagnostic protocol in the management of single-suture craniosynostosis is mainly based on careful clinical examination with regard to severity and degree of the abnormality and on ophthalmoscopic surveillance. Imaging techniques consist of ultrasound examination in young infants while routine plain radiographs are usually postponed to the date of surgery or the end of the first year. CT and magnetic resonance imaging (MRI) are confined to special diagnostic problems rarely encountered in isolated craniosynostosis. The results of this approach were evaluated retrospectively in 137 infants who were referred to our outpatient clinic for evaluation and/or treatment of suspected single suture craniosynostosis or positional deformity during a 2-year period (2008-2009). RESULTS: In 133 (97.1%) of the 137 infants, the diagnosis of single-suture craniosynostosis (n = 110) or positional plagiocephaly (n = 27) was achieved through clinical analysis only. Two further cases were classified by ultrasound, while the remaining two cases needed additional digital radiographs. In no case was CT scanning retrospectively considered necessary for establishing the diagnosis. Yet in 17.6% of cases, a cranial CT scan had already been performed elsewhere (n = 16) or had been definitely scheduled (n = 8). CONCLUSION: CT scanning is rarely necessary for evaluation of single-suture craniosynostosis. Taking into account that there is a quantifiable risk of developing cancer in further lifetime, every single CT scan should be carefully indicated.

Deficient language acquisition in children with single suture craniosynostosis and deformational posterior plagiocephaly.

PURPOSE: This study examined early language acquisition in children with single suture craniosynostosis (SSC) and in children with deformational posterior plagiocephaly. Our purpose was to determine whether infants with SSC have normal language acquisition at the age of 3 years, and whether infants with deformational posterior plagiocephaly demonstrate parallel development when compared with children with SSC. METHODS: The study population includes 61 infants. Twenty of them had synostosis of the sagittal suture, 12 synostosis of other suture and 29 deformational posterior plagiocephaly. Forty-nine of them were operated on a mean age of 10.6 months, and 12 were non-operated children with deformational posterior plagiocephaly. Language skills of participants were prospectively evaluated at the mean age of 3 years 4 months. RESULTS: About one half of the subjects (49%) had normal linguistic development, 30% had slight developmental problems and 21% had severe disorders in speech-language-related skills. These figures showed the prevalence of severe language disorders to be three times higher in our study population when compared with the general population. Children with sagittal synostosis managed better in all language skills compared with other types of SSC. Defective language development was found in deformational posterior plagiocephaly, both operated and non-operated. CONCLUSIONS: We found a noticeable developmental risk for specific language impairment in children with nonsyndromic SSC, and that the deviant language development is observable already in early infancy. Contrary to previous beliefs, the developmental risk for defective language development in deformational posterior plagiocephaly was found in both operated and non-operated subjects.

Hemangioma causing deformational plagiocephaly.

Hemangioma is the most common tumor of infancy, occurring mostly in the head and neck region. Intervention during the proliferating phase is indicated if the lesion poses a threat to life or function. Posterior head and neck hemangioma causing deformational plagiocephaly is a further indication for intervention.

Divided parietal bone in plagiocephaly.

Supernumerary cranial sutures represent a rare cause of plagiocephaly. In the case of an extra suture dividing the parietal bone, the presenting features are often inconsistent with the typical presentation of deformational or synostotic plagiocephaly. Disagreement exists as to how the presence of this suture affects the shape of the skull. We present a case of a supernumerary suture in the parietal bone leading to plagiocephaly and discuss the role this suture plays in cranial growth.

Severe deformational plagiocephaly: long-term results of surgical treatment.

INTRODUCTION: Deformational plagiocephaly (DP), a benign infantile condition, is often thought to improve spontaneously but can in fact lead to permanent skull asymmetry if left untreated. Deformational plagiocephaly can be completely corrected by nonsurgical treatments if recognized in the first year of life. Nevertheless, efficacy of these treatments drastically diminishes after 9 months as the calvaria thickens. Beyond 15 months, if the asymmetry is severe, surgical remodeling becomes an option. In this study, we analyzed the surgical morbidities and the morphologic outcome of surgical remodeling in DP. METHODS: This study was designed as a retrospective analysis of a craniofacial database combined with an outcome study. Between January 1992 and August 2006, 2363 occipital DP cases were treated by repositioning. Thirty patients were operated on, always after the age of 15 months (1.23%). Mean (SD) age at the time of the first clinic was 19.2 (± 5.8) months (range, 11-34 mo). A switch cranioplasty (A) was first used, progressively replaced since 1995 by a large occipital monobloc graft rotated 180 degrees and fixed with tongue-in-groove pillars (B). Morbidities were extracted from the database. Long-term morphologic outcomes were evaluated during a free clinic visit with a 4-point Likert-type outcome scale. RESULTS: Mean (SD) age at the time of surgery was 20.2 (± 5.6) months. Incidents (dural tears or venous sinus breaches) occurred in 28% (A) and 36% (B) or patients. One complication required repeat operation (extradural hematoma). No cerebrospinal fluid leaks and/or meningitis were recorded. Mean (SD) hospital stay was 5.3 (± 0.95) days. There was no statistical difference in the long-term morphologic outcome between the 2 groups (median of group A being "good but still visible"; median of group B being "excellent, unnoticeable"). CONCLUSIONS: Surgical correction of DP is a potentially life-threatening procedure, which should be indicated only in very severe cases, after exhaustion of all other nonsurgical methods, in a reference craniofacial center combining pediatric neurosurgery and plastic surgery.