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Background. Craniosynostosis is a group of rare congeni-tal diseases of the skull. They arise from premature ossification of one or more cranial sutures. This represents an obstruction to normal brain growth and leads to specific deformations of the skull, which may in the long term cause intracranial hypertension and cognitive delay. Materials and methods. All children treated for craniosyn-ostosis in the Paediatric Neurosurgery Unit of the University Medical Centre Ljubljana between June 2015 and September 2020 (N = 71; age 12.8 years; follow-up 2.7 years) were analyzed. The following data were recorded: affected suture, syndromic condition, hydrocephalus, Chiari malformation, raised intracranial pressure, age at surgery, surgical technique, need for multiple operations, and surgical complications. Results. Sagittal synostosis were present in 54.9 %, 25.3 % metopic, 14 % unicoronal, 1.4 % bicoronal and 1.4 % unilateral lambdoid. Multiple sutures were affected in 2.8 % of cases. Seven % were syndromic. Overall, 74 surgical procedures were performed: fronto-orbital advancement in 40.5 % of cases; biparietal remodelling in 32.4 %; total cranial vault remodelling in 22.9 %; posterior distraction in 2.7 %; posterior expansion in 1.3 %. Conclusions. The treat-ment of craniosynostosis is exclusively surgical and requires a multidisciplinary, neurosurgical, maxillofacial surgical and plastic and reconstructive surgical approach. Surgical treat-ment is performed not only for aesthetical purposes, but mainly to permit normal development of the brain.

Izhodišče. Kraniosinostoze so redke prirojene bolezni lobanje, ki nastanejo ob prezgodnji zakostenitvi enega ali več lobanjskih šivov. Ker možgani ne morejo več prosto rasti, pride do deformacij lobanje, ki lahko dolgoročno povzročijo znotrajlobanjsko hipertenzijo in kognitivni zaostanek. Preiskovanci in metode. V Enoti za otroško nevrokirurgijo UKC Ljubljana smo pregledali vse otroke, ki so bili od junija 2015 do septembra 2020 zdravljeni zaradi kraniosinostoze (N = 71; starost 12,8 let, obdobje spremljanja 2,7 let). Zbrali smo naslednje podatke: prizadet šiv, sindromska bolezen, hidrocefalus, Chiarijeva malformacija, povišan znotrajlobanjski tlak, starost ob operaciji, kirurška tehnika, potreba po več operacijah in zapleti kirurškega zdravljenja. Rezultati. Sagitalnih je bilo 54,9 %, 25,3 % metopičnih, 14 % koronarnih enostranskih, 1,4 % koronarnih obojestranskih in 1,4 % lambdoidnih enostranskih. V 2,8 % primerov je bilo prizadetih več lobanjskih šivov. Sindromskih primerov je bilo 7 %. Skupaj smo opravili 74 posegov: 40,5 % frontoorbitalno podaljšanje, 32,4 % biparietalno remodeliranje, 22,9 % remodeliranje celotnega svoda, 2,7 % posteriorno distrakcijo in 1,3 % posteriorno ekspanzijo. Zaključki. Zdravljenje kraniosinostoz zahteva multidisciplinarni nevrokirurški, maksilofacialni in oralnokirurški pristop ter posege plastične in rekonstruktivne kirurgije. Zdravljenje kraniosinostoz je izključno kirurško. Poseg ni potreben le v kozmetične namene, temveč predvsem zato, da možganom omogočimo normalno rast.