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Apert syndrome: Cranial procedures and brain malformations in a series of patients
BACKGROUND: Apert syndrome is one of the most severe craniofacial disorders. This study aims to describe the craniofacial surgeries and central nervous system malformations of a cohort of children with Apert syndrome treated in the past 20 years and to compare these data with previously published da...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Scientific Scholar
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7655990/ https://www.ncbi.nlm.nih.gov/pubmed/33194294 http://dx.doi.org/10.25259/SNI_413_2020 |
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author | Munarriz, Pablo M. Pascual, Beatriz Castaño-Leon, Ana M. García-Recuero, Ignacio Redondo, Marta de Aragón, Ana Martínez Romance, Ana |
author_facet | Munarriz, Pablo M. Pascual, Beatriz Castaño-Leon, Ana M. García-Recuero, Ignacio Redondo, Marta de Aragón, Ana Martínez Romance, Ana |
author_sort | Munarriz, Pablo M. |
collection | PubMed |
description | BACKGROUND: Apert syndrome is one of the most severe craniofacial disorders. This study aims to describe the craniofacial surgeries and central nervous system malformations of a cohort of children with Apert syndrome treated in the past 20 years and to compare these data with previously published data. METHODS: Retrospective analysis of a series of patients with Apert syndrome treated between 1999 and 2019 in our hospital. Information was analyzed regarding craniofacial procedures, hydrocephalus and presence of shunts, Chiari malformation Type 1, and other brain malformations such as corpus callosum and septum pellucidum anomalies. RESULTS: Thirty-seven patients were studied. Ventriculoperitoneal shunt prevalence was 24.3%, and 8.1% of patients required decompressive surgery for Chiari malformation. All of them needed at least one cranial vault remodeling procedure. The median age for this procedure was 8 months. In 69.7% of patients, the first cranial vault intervention was performed in the fronto-orbital region. In 36.4% of patients, a midface advancement had been performed at the time of this review, although this proportion was very dependent on the follow-up period and the age of the patients. The median age for the midface advancement procedure was 5.25 years. Anomalies of the corpus callosum and the septum pellucidum were reported in 43.2% and 59.5% of patients, respectively. CONCLUSION: Apert syndrome is a type of syndromic craniosynostosis, and patients usually require one or more cranial and facial surgeries. In comparison with other syndromic craniosynostosis types, Apert syndrome less frequently requires a VP shunt or treatment for a Chiari malformation. |
format | Online Article Text |
id | pubmed-7655990 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Scientific Scholar |
record_format | MEDLINE/PubMed |
spelling | pubmed-76559902020-11-13 Apert syndrome: Cranial procedures and brain malformations in a series of patients Munarriz, Pablo M. Pascual, Beatriz Castaño-Leon, Ana M. García-Recuero, Ignacio Redondo, Marta de Aragón, Ana Martínez Romance, Ana Surg Neurol Int Original Article BACKGROUND: Apert syndrome is one of the most severe craniofacial disorders. This study aims to describe the craniofacial surgeries and central nervous system malformations of a cohort of children with Apert syndrome treated in the past 20 years and to compare these data with previously published data. METHODS: Retrospective analysis of a series of patients with Apert syndrome treated between 1999 and 2019 in our hospital. Information was analyzed regarding craniofacial procedures, hydrocephalus and presence of shunts, Chiari malformation Type 1, and other brain malformations such as corpus callosum and septum pellucidum anomalies. RESULTS: Thirty-seven patients were studied. Ventriculoperitoneal shunt prevalence was 24.3%, and 8.1% of patients required decompressive surgery for Chiari malformation. All of them needed at least one cranial vault remodeling procedure. The median age for this procedure was 8 months. In 69.7% of patients, the first cranial vault intervention was performed in the fronto-orbital region. In 36.4% of patients, a midface advancement had been performed at the time of this review, although this proportion was very dependent on the follow-up period and the age of the patients. The median age for the midface advancement procedure was 5.25 years. Anomalies of the corpus callosum and the septum pellucidum were reported in 43.2% and 59.5% of patients, respectively. CONCLUSION: Apert syndrome is a type of syndromic craniosynostosis, and patients usually require one or more cranial and facial surgeries. In comparison with other syndromic craniosynostosis types, Apert syndrome less frequently requires a VP shunt or treatment for a Chiari malformation. Scientific Scholar 2020-10-29 /pmc/articles/PMC7655990/ /pubmed/33194294 http://dx.doi.org/10.25259/SNI_413_2020 Text en Copyright: © 2020 Surgical Neurology International http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Original Article Munarriz, Pablo M. Pascual, Beatriz Castaño-Leon, Ana M. García-Recuero, Ignacio Redondo, Marta de Aragón, Ana Martínez Romance, Ana Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title | Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title_full | Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title_fullStr | Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title_full_unstemmed | Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title_short | Apert syndrome: Cranial procedures and brain malformations in a series of patients |
title_sort | apert syndrome: cranial procedures and brain malformations in a series of patients |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7655990/ https://www.ncbi.nlm.nih.gov/pubmed/33194294 http://dx.doi.org/10.25259/SNI_413_2020 |
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