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GeneralIntroduction111theskullisobstructed Thiscanresultinanabnormalskullshapeoracranium withtoolittle volume for theunderlyingbrain to developproperly Thisin turn can causeincreasedintracranialpressureandcanleadtobehaviouralordevelopmentaldisorders4Craniosynostosisoccursinaboutonein2200birthswhichmeansthatthere are about100newbornpatients a yearin theNetherlands56 Craniosynostosiscanbe apart of a syndrome orbepresentin anisolated formDepending on whichsutureisprematurelyfuseddifferenttypesofcraniosynostosiscanbeclassified(Figure1)Scaphocephalyisthemostcommonformofcraniosynostosisoccurringin55to60%ofallthecasesItistheresultofapartialorcompleteprematurefusionofthesagittalsutureAsaresultgrowthperpendiculartothesagittalsutureisinhibitedresultinginareducedlateraldiameteroftheheadAdditionallycompensatorygrowthintheantero-posteriordirectionresultsinanelongatedskullScaphocephalyhasamaletofemaleratioofalmost417–10TrigonocephalyreferstothetypicaltriangularshapeoftheforeheadduetotheprematurefusionofthemetopicsutureinthefrontoftheheadAsaresultasteeply curved frontalheadis formed Trigonocephalyis seenin15% of theisolatedcraniosynostosiscasesandismostcommoninmales7Plagiocephalyiscausedbytheunilateralprematurefusionoftheleftorrightcoronal suture711Unilateral coronal synostosisresultsin an asymmetrichead shapeas well asin facial asymmetryInpronounced casesharlequin eye deformity whichoccursbecausetheorbitontheaffectedsideiselevatedispresentInadditionobliquityofthefacialmidlinemaybeobservedBesides(unilateral)plagiocephalyboththecoronalsuturescouldbefused Thisresultsinbrachycephalyaconditionthatdisplaysnoasymmetryofthefacebutresultsinaskullshapeinwhichtheanteriorpartoftheskullistallandflatandisthereforesometimescalledthe ‘flatheadsyndrome’.7IncasemoresuturesareaffectedthedysmorphologyoftheskullismoresevereOxycephaly(orturricephaly)forexampleistheresultofthesynostosisofbothcoronal andlambdoidal sutures and forces the skull to growin theupward direction