Rev Paul Pediatr. 2016;34(4):495---502

REVISTA PAULISTADE PEDIATRIA

www.rpped.com.br

REVIEW ARTICLE

Diagnosis of infant synostotic and nonsynostotic cranialdeformities: a review for pediatricians

Enrico Ghizonia,∗, Rafael Denadaib, Cesar Augusto Raposo-Amaralb,Andrei Fernandes Joaquima, Helder Tedeschia, Cassio Eduardo Raposo-Amaralb

a Universidade Estadual de Campinas (Unicamp), Campinas, SP, Brazilb Instituto de Cirurgia e Plástica Crânio Facial, Hospital SOBRAPAR, Campinas, SP, Brazil

Received 20 October 2015; accepted 11 January 2016Available online 23 February 2016

KEYWORDSCraniofacialabnormalities;Craniosynostosis;Diagnosis;Pediatricians

AbstractObjective: To review the current comprehensive care for nonsyndromic craniosynostosis andnonsynostotic cranial deformity and to offer an overall view of these craniofacial conditions.Data source: The review was conducted in the PubMed, SciELO, and LILACS databases withouttime or language restrictions. Relevant articles were selected for the review.Data synthesis: We included the anatomy and physiology of normal skull development of chil-dren, discussing nuances related to nomenclature, epidemiology, etiology, and treatment ofthe most common forms of nonsyndromic craniosynostosis. The clinical criteria for the dif-ferential diagnosis between positional deformities and nonsyndromic craniosynostosis werealso discussed, giving to the pediatrician subsidies for a quick and safe clinical diagnosis. Ifpositional deformity is accurately diagnosed, it can be treated successfully with behavior mod-ification. Diagnostic doubts and craniosynostosis patients should be referred straightaway to amultidisciplinary craniofacial center.Conclusions: Pediatricians are in the forefront of the diagnosis of patients with cranial defor-

mities. Thus, it is of paramount importance that they recognize subtle cranial deformities as itmay be related to premature fusion of cranial sutures.© 2016 Sociedade de Pediatria de Sao Paulo. Published by Elsevier Editora Ltda. This is an openaccess article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

∗ Corresponding author.E-mail: ghizonie@gmail.com (E. Ghizoni).

http://dx.doi.org/10.1016/j.rppede.2016.02.0052359-3482/© 2016 Sociedade de Pediatria de Sao Paulo. Published by Elsevier Editora Ltda. This is an open access article under the CC BYlicense (http://creativecommons.org/licenses/by/4.0/).

496 Ghizoni E et al.

PALAVRAS-CHAVEAnormalidadescraniofaciais;Craniossinostose;Diagnóstico;Pediatras

Diagnóstico das deformidades cranianas sinostóticas e não sinostóticas em bebês:uma revisão para pediatras

ResumoObjetivo: Revisar o atendimento integral atual de craniossinostose não sindrômica e deformi-dade craniana não sinostótica e oferecer uma visão global dessas condicões craniofaciais.Fontes de dados: A revisão foi realizada nas bases de dados PubMed, SciELO, LILACS e semrestricões de tempo ou idioma. Artigos relevantes foram selecionados para a revisão.Síntese dos dados: Foram incluídas a anatomia e fisiologia do desenvolvimento normal do crânioem criancas, discutindo nuances relacionadas à nomenclatura, epidemiologia, etiologia e trata-mento das formas mais comuns de craniossinostose não sindrômica. Também foram discutidos oscritérios clínicos para o diagnóstico diferencial entre deformidades posicionais e craniossinos-tose não sindrômica, dando aos pediatras subsídios para um diagnóstico clínico rápido e seguro.Se deformidades posicionais forem diagnosticadas com precisão, elas podem ser tratadas comsucesso através da modificacão do comportamento. Dúvidas de diagnóstico e pacientes porta-dores de craniossinostose devem ser encaminhados imediatamente a um centro multidisciplinarcraniofacial.Conclusões: Os pediatras estão na vanguarda do diagnóstico de pacientes com deformidadescranianas. Assim, é de suma importância que reconhecam deformidades cranianas sutis, poiselas podem estar relacionadas à fusão prematura das suturas cranianas.© 2016 Sociedade de Pediatria de Sao Paulo. Publicado por Elsevier Editora Ltda. Este e umartigo Open Access sob uma licenca CC BY (http://creativecommons.org/licenses/by/4.0/).

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ranial deformities are a common complaint in pediatricnits, since 25% of infants of single pregnancies and 50% ofultiple pregnancies have some degree of skull deformity atirth. In general, parents usually recognize these changes inhe first weeks or months of life.1 However, in some sce-arios the diagnosis may be overlooked by the family thatends to deny the problem. In these cases, pediatriciansust be aware of these issues and counsel the family for

he importance to seek a craniofacial team. In addition, it isf fundamental importance that pediatrician be prepared atrst consultation to make the differential diagnosis between

positional deformity and craniosynostosis, considering thathildren born with a positional deformity does not need toe exposed to the ionizing radiation of a computed tomo-raphy (CT), apart from the costs of the procedure and theedation risks to achieve it.2

In this report, we review the anatomy and physiology oformal skull development of children, discussing nuanceselated to nomenclature, epidemiology, etiology, and treat-ent of the most common forms of craniosynostosis. The

linical criteria for the differential diagnosis between posi-ional deformities and craniosynostosis are also presented,llowing the pediatrician subsidies for a quick and safe clin-cal diagnosis.

ethod

he present study is a literature review, with a descrip-ive approach. We performed a literature review byearching the Medline (PubMed), SciELO, and LILACSatabases without time or language restrictions. The final

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iterature review was performed on July 2015. To iden-ify all relevant articles (review articles, clinical trials,nd cohort studies) about the current comprehensive careor nonsyndromic craniosynostosis and nonsynostotic cra-ial deformity the following search terms were used:‘nonsyndromic craniosynostosis’’, ‘‘nonsynostotic cranialeformity’’, ‘‘positional deformity’’. ‘‘nonsynostotic pos-erior plagiocephaly’’, and ‘‘positional plagiocephaly’’.ach relevant study was individually reviewed to iden-ify information concerning normal skull developmentf children, nomenclature, epidemiology, etiology, diag-osis, and treatment of the most common forms ofonsyndromic craniosynostosis and nonsynostotic cranialeformity.

ranial anatomy

he skull of a newborn is composed of multiple bones andutures that make it malleable and subject to external forceshat deform it, to enable its passage through the birth canalnd to accommodate the encephalon, since the brain volumes quadrupled in the first two years of life.3

The skull is composed of four major sutures (metopic,agittal, coronal, and lambdoid), three secondary suturesfrontonasal, temporal squamosal, and frontosphenoidal),nd four main bones (temporal, frontal, parietal, and occip-tal). The metopic suture separates the frontal bones fromach other, the sagittal suture from the parietal bones; theoronal suture from the parietal and frontal bones, and

he lambdoid suture from the parietal and occipital bones.3

esides the bones and sutures, soft and membranous spaceeparating the skull bones are named fontanelle, being ofreat importance are the anterior or bregmatic (bounded by

Diagnosis of infant synostotic and nonsynostotic cranial deformities 497

Table 1 Major and secondary skull sutures and age at theonset of fusion3

Sutures Beginning of fusion

Metopic 2 monthsSagittal 22 monthsCoronal 24 monthsLambdoid 26 monthsFrontonasal 68 monthsFrontosphenoidal 22 monthsTemporal squamosal 35---39 months

Table 3 Classification of craniosynostoses.

1. PrimarySimple (involving a single suture): Sagittal, Coronal,Metopic, and LambdoidComplex (fusion of two or more sutures)Nonsyndromic: BicoronalSyndromic: Crouzon, Apert, Pfeiffer, and Saethre-Chotzen

2. SecondaryMetabolic disorders: Hyperthyroidism, Inborn Errors ofMetabolismVarious malformations: Microcephaly, EncephaloceleAfter ventricular shunt with excessive drainage of CSF(cerebrospinal fluid)Fetal exposure to certain substances: Valproic acid,Phenytoin

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the frontal and parietal bones) and the posterior or lambdoid(bounded by the occipital bone and parietal bones).

Under physiological conditions, the cranial suturesprogress into fusion with different initial periods of fusionaccording to each major suture (Table 1). The same occurswith the fontanelles, which usually close themselves by thesecond year (Table 2).

An important detail is that the skull bones are membra-nous (without a prior cartilaginous phase), which results ingrowth through the bone deposit in the region of the sutures;this growth occurs perpendicular to the suture. For exam-ple, coronal sutures allow the anterior-posterior growth ofthe skull, while the sagittal suture allows growth of thebiparietal skull.3

Craniosynostosis

The definition of craniosynostosis is the premature fusionof one or more cranial sutures. The occurrence is approx-imately one for 2000 to 2500 live births.4 The prematurefusion of sutures prevents perpendicular growth of the skull,and an increase in brain volume leads to a compensatorygrowth of the skull parallel to this.

Craniosynostosis are classified as primary or secondary.Primary craniosynostosis results from genetic and environ-mental influences, being classified as simple and complex.Complex craniosynostosis are divided even further intononsyndromic and syndromic (Table 3).5---8 Due to thegreater prevalence, we will discuss the diagnosis of sim-ple primary craniosynostosis, namely sagittal, coronal,metopic, and lambdoid synostoses (Fig. 1). Then the nonsyn-ostotic posterior plagiocephaly (positional plagiocephaly)will be presented, emphasizing important features fordifferential diagnosis with lambdoid synostosis (posterior

plagiocephaly).

Table 2 Age of closure of cranial fontanelles3

Fontanelles Age of closure

Anterior or bregmatic 24 monthsPosterior or lambdoid 3 monthsAnterolateral (Sphenoid) 6---24 monthsPosterolateral (Mastoid) 6---24 months

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enetic aspects

ultiple hypotheses have been proposed to explain theathogenesis of abnormal suture fusion. Both environ-ental (especially intrauterine fetal head constraint)

nd genetics factors (single gene mutations, chromosomebnormalities and polygenic background) predispose toraniosynostosis.9---11 Mutations in 7 genes (namely, FGFR1,GFR2, FGFR3, TWIST1, EFNB1, MSX2 and RAB23) arenequivocally associated with Mendelian forms of syndromicraniosynostosis.9---11 In contrast, the genetic etiology ofonsyndromic craniosynostosis remained poorly understoodntil very recently.9---11 In the last years, epidemiologicnd phenotypic studies clearly demonstrate that nonsyn-romic craniosynostosis is a complex and heterogeneousondition supported by a strong genetic component accom-anied by environmental factors that contribute to theathogenesis network of this birth defect.9,10 In fact, rareutations in FGFRs, TWIST1, LRIT3, ALX4, IGFR1, EFNA4,UNX2, and FREM1 have been reported in a minor fraction ofatients with nonsyndromic craniosynostosis.9,10 The mini-um molecular genetic tests recommended for each clinicalresentation (syndromic and nonsyndromic craniosynosto-is) have been previously published review10 and are out ofhe scope of this report. Further research of a large popu-ation with phenotypically homogeneous subsets of patientss required to understand the complex genetic, maternal,nvironmental, and stochastic factors contributing to non-yndromic craniosynostosis.9---11

eneral diagnostic approach

ediatricians are expected to be able to recognize skulleformities and to diagnose them as either craniosynostosisr a positional skull deformity. If a skull deformity is present,he physical examination and clinical history (key fea-ures described in the next subheads) are the most helpful

nd revealing pieces of information in the child’s evalua-ion. A previous published anamnestic flowchart12 serves as

guideline to distinguish craniosynostosis from positionalkull deformities. The key questions to differentiate the

498 Ghizoni E et al.

Figure 1 (A, Left) Frontal photograph of patient with premature fusion of sagittal suture showing the characteristic temporalpinching. (Right) Lateral photograph reveling increase in the anterior-posterior diameter of the skull (long narrow skull), the frontalbossing and occipital bulging (occipital bullet), which are the main clinical characteristics of sagittal craniosynostosis. (B, Left)Frontal photograph of patient with premature fusion of the right coronal suture showing the retrusion of the ipsilateral frontal bonefusion and compensatory contralateral bulging, asymmetry of the eyebrows, orbits, ears, nose, jaw, as well as convergent strabismusof the left eye. (Right) 3D CT reconstruction showing the premature fusion of the right coronal suture and the elevation of theipsilateral sphenoid wing leading to an elongate orbit, recognized as the ‘‘harlequin orbit’’. (C, Left) Frontal photograph of patientwith premature fusion of metopic suture showing the triangular aspect of the forehead with retruded crests of the orbits bilaterallyand hypoteleorbitism (approximation of orbits). (Right) Basal view revealing the triangular appearance of the skull. (D) Lateralphotograph of patient with premature fusion of lambdoid suture showing the turricephalic aspect of the skull. Two-dimensionalp AR Hp

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hotographs and radiological documentations belong to SOBRAPatient’s parents.

raniosynostoses from the nonsynostotic deformities are: (1)‘Is deformity present at birth?’’ Craniosynostosis is presentt birth, whereas nonsynostotic deformities develop in theeonatal period; (2) Is there a preferred sleep position?; (3)‘Is there improvement of the deformity?’’ Craniosynostosisets worse with time, whereas the nonsynostotic deformi-ies improve as the child develops head control and the skullo longer has localized pressure for long periods’’.12

In rare and difficult cases, when the examination andistory are not diagnostic, a good-quality four-view radio-raphic series (anteroposterior, Towne and two lateralrojections) might be sufficient to exclude craniosynosto-is and avoid further radiation exposure.13 If it is unclear,ecause of the very young age of the patient, it is rec-

mmended to repeat X-skull after 1 or 2 months.14 CT isot the recommended modality for screening because ofhe associated radiation exposure and high imaging costs

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ospital’s archives. Informed consent forms were signed by the

nd diagnostics by pediatricians with CT is associated withurther delay in referral.13,14

After the clinical suspicion (or confirmation) of craniosyn-stosis, the children should be referred to a multidisciplinaryeam specialized in craniofacial anomalies (anesthesiol-gist, plastic surgeon, speech therapist, neurosurgeon,rthodontist, otorhinolaryngologist, and psychologist).15 Inhese centers, the radiological exam of choice is the three-imensional CT scan that contributes to elucidation of thextension of suture fusion and the consequent craniofacialeformity and subsequent surgical planning. It is notewor-hy that the cephalic perimeter generally does not changeue to compensatory growth of other bones in the majorityf cases with simple craniosynostosis.

In this context, patients with craniosynostosis noturgically-treated can develop several complications suchs16---18: Intracranial hypertension (ICH) occurs in up to 60%

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Diagnosis of infant synostotic and nonsynostotic cranial defo

of children with complex craniosynostosis and 20% of carri-ers of simple craniosynostosis; cognitive and developmentaldisorders, poor weight gain, visual, hearing, and languagedisorders; and psychological problems such as low self-esteem and social isolation. Therefore, the objective ofsurgical treatment is to prevent ICH and to correct cranio-facial abnormalities. Overall, the optimal timing of surgicalcorrection in most cases is between 6 and 9 months of age.The motivations for performing the surgery before 1 yearof age include the ability of the child younger than 1 yearto completely reossify, the malleable character of the cal-varia during this age, and the tremendous brain growth thatoccurs during the first year, which allows good remodeling ofthe skull.19 Satisfactory craniofacial form and esthetic pleas-ing outcomes have also been associated with craniofacialsurgical interventions performed before 1 year of age.18,19

It is noteworthy that the presence of ICH signs (irritability,swelling of the papilla, bulging fontanelle, and imaging find-ings) may result in the need for earlier surgical intervention,to perform decompression procedures or ventricular shuntsurgery if associated with hydrocephalus.

Sagittal synostosis

It is the most common form of simple craniosynostosisand accounts for 40---60% of cases, being more prevalentamong males (75---85%). The skull has an elongated andnarrow shape, similar to a boat, hence being called scapho-cephaly (Fig. 1A).20 Upon physical examination, a ridge canbe palpated on the sagittal suture. It should be noted thatthe anterior fontanelle may not be closed. Compensatoryfrontal bossing and occipital protrusion may occur in varyingdegrees.

Surgical treatment is indicated between 3 and 12 monthsof age, and procedures may vary from a simple endoscopicresection of the sagittal suture to total reconstruction ofthe skull, depending on the severity of the clinical pre-sentation. In our service, we recommend surgery between6 and 9 months of age and use the craniectomy in a‘‘�’’ fashion (named Hung Spun procedure),21 associatedwith several osteotomies (bone cuts) parallel rectangles ofapproximately two centimeters long in the parietal bone,between the coronal and lambdoid sutures, which permitgreater lateral space for further accommodation of thebrain. Barrel stave osteotomies (lateral bone cuts) still allowfor reduction of the anterior posterior diameter and betterremodeling of the skull, with excellent esthetic results.22

Coronal synostosis

It is the second most common form, accounting for upto 25% of craniosynostosis cases. The closure of a coronalsuture is called anterior plagiocephaly,23,24 while the closureof two sutures is termed brachycephaly (commonly foundin syndromic craniosynostosis).6---8 It predominantly affectsfemales (60%), with similar incidence on both sides.

Early fusion of the coronal suture leads to a flatten-

ing of the frontal bone and the ipsilateral orbital rimto the fusion, with a compensatory contralateral frontalbossing.23,24 Strabismus is a common finding (50---60% ofcases) and is the result of morphological changes in the

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rbital roof and trochlea, altering the function of the supe-ior oblique muscle.25 Elevation of the ipsilateral sphenoiding can be seen in simple skull radiography and is recog-ized as the ‘‘harlequin orbit’’ (Fig. 1B).26 Premature fusionf the coronal suture also causes a deviation of the skullase, changing the position of the orbits, asymmetry ofhe eyebrows, asymmetry of the ear position, deviation ofhe mandible, and change of occlusion, with an importantsthetic effect.23,24

Therefore, surgical treatment is indicated for correctionf the morphological skull deformity and its repercussionsn the face, but also because of strabismus and risks ofeveloping ICH. We recommend the procedure between 6nd 9 months of age, when there is already sufficient boneaturity for remodeling. Basically, a front-orbital advance-ent associated with frontal remodeling is performed and

eleasing both coronal sutures.24 Further procedures mighte necessary as fat injection in the craniofacial skeleton toecrease facial asymmetries and correction of eyelid pto-is. At the end of facial growth orthognatic surgery andhinoplasty with septoplasty may be performed by a plasticurgeon with a craniofacial training.

etopic synostosis

orresponds to 10% of all craniosynostosis and predominatesn males (75---85% of cases). Early fusion of the metopicuture restricts the transversal growth of frontal bones,nd in more severe cases can restrict the expansion ofhe anterior fossa, which leads to hypoteleorbitism, andonsequently to trigonocephaly (Fig. 1C). Metopic cranio-ynostosis is the single suture synostosis most frequentlyssociated with more cognitive disorders, primarily due tohe growth restriction of the frontal lobes.27

The increase in the anterior fossa volume is the mainbjective in the treatment of patients with trigonocephaly,s well as frontal remodeling and fronto-orbital advance-ent. The best time for treatment is between 6 and 9onths of age.

amboid synostosis

his is the rarest form of simple craniosynostosis, with anncidence of about 0.3 per 10,000 live births, correspond-ng to approximately 1.0---5.5% of all craniosynostosis. Whenvaluated in a population of children with occipital flatten-ng (also called posterior plagiocephaly), it is responsible fornly 0.9---4.0% of the cases.28

Fusion of a lambdoid suture leads to an occipital defor-ity (posterior plagiocephaly) with a trapezoidal shape,hile the fusion of both lambdoid sutures leads to brachy-ephaly (Fig. 1D). In addition to posterior deformityflattening, poor positioning of ears, parietal compensatoryossing) caused by premature fusion of the lambdoid suture,ignificant morphological changes may occur concomitantlyn the posterior fossa. This craniosynostosis is associatedith herniated cerebellar tonsils (also known as Chiari

alformation type I) and fusion of the jugular foramen,

esulting in a high risk of venous hypertension. Thus, thiss the form of simple craniosynostosis with the greatest riskf ICH.29

500 Ghizoni E et al.

Table 4 Important characteristics to subsidize the differential diagnosis of positional plagiocephaly versus lamboidsynostosis21,24,25

Characteristics Positional plagiocephaly Lambdoid craniosynostosis

Age at onset Several weeks postnatally BirthPreferred position Common RareTorticollis Present Absent/PresentBony ridge along the lambdoid suture Absent PresentBulging mastoid Absent PresentFrontal bossing Ipsilateral ContralateralDisplacement of the ipsilateral ear Anterior PosteriorSkull shape Parallelogram Trapezoid

Diagnosis Clinical, through medical history andphysical examination

Three-dimensional computedtomography

Treatment Clinical Surgical

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Surgical treatment is based on volume expansion of theosterior portion of the skull (parietal and occipital region)nd releasing the lambdoid sutures. However, this region hasarge venous drainage, with innumerable scalp veins thatross the bone toward the dural sinuses, greatly increasinghe surgical risk of a craniotomy and bone remodeling. Inur service, we choose a posterior distraction technique,n which the cranial volume is gradually increased, signif-cantly reducing the risk of bleeding and need for bloodransfusions.30

ositional plagiocephaly

he term plagiocephaly means oblique skull and correspondso a unilateral or bilateral occipital flattening, which mayrise due to the continual influence of external forces on themmature skull (nonsynostotic posterior plagiocephaly) orecause of premature fusion of one or both lambdoid suturessynostotic posterior plagiocephaly). Anterior plagiocephalyan be used to define the cranial deformation characterizedy premature unilateral fusion of the coronal suture.

Positional or deformational plagiocephaly is the mostommon cause of plagiocephaly (prevalence of 5---48% inealthy newborn infants)31 versus an incidence of 0.003%f synostotic plagiocephaly (lamboid synostosis).28

Based on the introduction of the campaign to preventudden Infant Death Syndrome (‘‘Back to Sleep’’), in theeginning of the 1990s --- which recommended that babiesemain in the supine position --- a significant increase inhe incidence of children with positional plagiocephaly wasoticed (5---48%).31 This deformity results from an ongoingction of gravitational forces on the occipital region, caus-ng a flattened region of the posterior craniofacial skeleton.

f no intervention is performed, the deformity can continuend, in severe cases, evolve with facial deformities. Posi-ional plagiocephaly occurs more often on the right side70% of cases) and affects more males. The major risk

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actors include: torticollis, prematurity, multiparity, and axed sleeping position.

Diagnosis is eminently clinical, and the differentiationith synostotic plagiocephaly (unilateral fusion of the lamb-oid or coronal sutures) is essential.28 Anamnesis andhysical examination are sufficient to establish the dif-erential diagnosis between a positional deformity andraniosynostosis in the vast majority of cases (Table 4).lassically, patients with premature fusion of the lambdoiduture already have the deformity at birth, while thoseith a positional deformity have a normal skull at birth andevelop the deformity in the subsequent weeks or months.hen asked, parents may mention that there is a pre-

erred position of the baby’s positioning in patients withositional plagiocephaly, while in patients with synostoticlagiocephaly, there is no preferred position.

Pediatricians should perform a physical examination withhe aid of the parents; initially, the patient remains on theother/father’s lap facing the pediatrician and after facing

he parents; finally, the examiner should observe the childrom a superior view. During the physical examination, sym-etries between the skull, forehead, and ears should be

arefully analyzed.Positional plagiocephaly presents a format of a parallelo-

ram skull, while synostotic posterior plagiocephaly has thehape of a trapezoid.32 Still, an ipsilateral bulging in theastoid region and a ridge on the fused lambdoid suture

an be seen and palpated. In moderate to severe casesn both deformities, compensatory frontal bossing can bebserved, ipsilateral in positional plagiocephaly and con-ralateral in synostotic plagiocephaly. This involvement ofhe forehead may progress, leading to a facial scoliosis inoth pathologies, changing the facial symmetry. In patientsith lambdoid suture craniosynostosis, the ipsilateral ear

tenosis tends to be in a posterior position and downwards,

s if the suture pulled it. While the positional plagiocephalyends to be in an anterior position, as if it had been pushedFig. 2). The physical examination should also includevaluation of the cervical region and look for evidence of

Diagnosis of infant synostotic and nonsynostotic cranial deformities 501

Figure 2 Representation of positional plagiocephaly and true (synostotic) posterior plagiocephaly. (A) Positional plagiocephalyshowing: absence of lambdoid suture stenosis, format of a parallelogram skull, ipsilateral compensatory frontal bossing, ipsilateralear in an anterior position, as if it had been pushed. (B) True posterior plagiocephaly showing: presence of lambdoid suture stenosis,shape of a trapezoid, ipsilateral bulging in the mastoid region, contralateral compensatory frontal bossing, ipsilateral ear stenosis

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congenital torticollis and/or thickening of the sternoclei-domastoid muscle, which are directly related to positionalplagiocephaly.

The diagnosis and treatment of positional plagiocephalyare clinical.32 Some guidance should be given to parents atthe first childcare consultation, how to avoid bad posturepositions for sleeping or on the changing table, to note thepresence of torticollis, to spend as little time as possible inthe ‘‘baby comfort’’, and to encourage that the baby stayfor a longer time in the ventral decubitus position, whileunder supervision. It is important to diagnose any cervicalrestriction (e.g., congenital torticollis or thickening of thesternocleidomastoid muscle) and guide parents about theneed of early physiotherapy treatment.

In addition to the guidelines, therapeutic measures canbe employed, such as how to force the baby to sleep on thecontralateral side of the deformity, to encourage movingthe location of the crib and changing table, in order to forcethe baby to turn his head to the side he is on, besides stim-ulating the baby to sit. Such measures are effective until4---6 months as treatment of positional plagiocephaly.32,33

The prevalence of positional deformity tends to decreasewith age and may be as low as 3.3% at 2 years of age, whichhighlights the natural ability of skull remodeling.32 However,

after 6 months of age, the use of a specific helmet maybe indicated in patients with severe deformities to aid inremodeling the skull. It should be emphasized that the hel-met requires at least 23h of use per day to be effective,

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hich may result in pressure sores and local abrasions,esides the high cost of the appliance and bothersome tohe child.34

onclusions

ranial deformities are common complaints and highlyrevalent in the routine of pediatricians. Although the vastajority of children present positional deformities, earlyiagnosis of craniosynostosis and referring them to special-zed treatment in a timely manner is critical to optimizeurgical outcomes. The diagnosis of positional deformities issually clinical, and treatment consists of simple guidelinesnd measures to prevent worsening the condition. Whenhere is a diagnosis of craniosynostosis, a multidisciplinarypproach of the child with craniosynostosis is crucial for areater surgical success rate and to minimize complications.

unding

his study did not receive funding.

onflicts of interest

he authors declare no conflicts of interest.

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