CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS

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...

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Main Authors: Peter Spazzapan, Miha Kočar, Andreja Eberlinc, Tomaž Velnar
Format: Article
Language:Slovenian
Published: The Society for Children with Metabolic Disorders 2021-06-01
Series:Slovenska pediatrija
Subjects:
Online Access: http://www.slovenskapediatrija.si/Portals/0/Clanki/2021/Slovpediatr-2021-2-01en.pdf
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author Peter Spazzapan
Miha Kočar
Andreja Eberlinc
Tomaž Velnar
author_facet Peter Spazzapan
Miha Kočar
Andreja Eberlinc
Tomaž Velnar
author_sort Peter Spazzapan
collection DOAJ
description 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.
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spelling doaj.art-bd92e872ecdd49a2921149ca18b54c812022-12-21T19:07:59ZslvThe Society for Children with Metabolic DisordersSlovenska pediatrija1318-44232712-39602021-06-01282677710.38031/slovpediatr-2021-2-01en13184423CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSISPeter Spazzapan0Miha Kočar1Andreja Eberlinc2Tomaž Velnar3 Enota za pediatrično nevrokirurgijo, Klinični oddelek za nevrokirurgijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija Klinični oddelek za maksilofacialno in oralno kirurgijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija Klinični oddelek za maksilofacialno in oralno kirurgijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija Enota za pediatrično nevrokirurgijo Klinični oddelek za nevrokirurgijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija 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. http://www.slovenskapediatrija.si/Portals/0/Clanki/2021/Slovpediatr-2021-2-01en.pdf craniosynostosisintracranial hypertensionapnoeahydrocephaluschiari malformation
spellingShingle Peter Spazzapan
Miha Kočar
Andreja Eberlinc
Tomaž Velnar
CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
Slovenska pediatrija
craniosynostosis
intracranial hypertension
apnoea
hydrocephalus
chiari malformation
title CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
title_full CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
title_fullStr CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
title_full_unstemmed CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
title_short CRANIOFACIAL RECONSTRUCTIONS IN CHILDREN WITH CRANIOSYNOSTOSIS
title_sort craniofacial reconstructions in children with craniosynostosis
topic craniosynostosis
intracranial hypertension
apnoea
hydrocephalus
chiari malformation
url http://www.slovenskapediatrija.si/Portals/0/Clanki/2021/Slovpediatr-2021-2-01en.pdf
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