Eziopatogenesi e manifestazioni cliniche della sindrome di Crouzon: revisione della letteratura

Translated title of the contribution: Crouzon syndrome: Etiopathogenesis and clinical manifestations

C. Maspero, L. Giannini, F. Iazzetti, S. Tronca, G. Farronato

Research output: Contribution to journalArticle

Abstract

Objectives: The term Craniofacial dysostosis (CFD) is used to describe familiar forms of synostosis involving different sutures of the cranial base and midface. The aim of this work consists in a systematic review of literature on aetiology, etiopathogenesis, epidemiology, diagnosis, clinical, systemic, and oral manifestations and therapeutic options of Crouzon syndrome. Materials and methods: A systematic review of literature through Medline data bank [www.ncbi.nim.nih.gov/pubmed] was done using "Craniofacial Dysostosis", "Craniosynostosis", and "Crouzon Syndrome" as keywords. Results: Crouzon syndrome is the most common craniosynostosis. It develops after an early fusion of superior and posterior sutures of the maxilla with orbital ones, with consequent underdevelopment of the midface and ocular proptosis. Oral manifestations are maxillary hypoplasia, maxillary dental crowding, ogival palatus, and relative mandibular prognathism. Diagnosis can be done either through the evaluation of clinical phenotypes or DNA analysis with polymerase chain reaction (PCR). Treatment is mainly surgical. Orthodontic therapy is performed during the early skeletal maturation. Conclusions: Thanks to the important innovations in surgical techniques and to a muldisciplinary approach, patients affected by this syndrome may aspire to normal cerebral development, physical status, and social relationships.

Original languageItalian
Pages (from-to)249-260
Number of pages12
JournalMondo Ortodontico
Volume35
Issue number5
DOIs
Publication statusPublished - Nov 2010

Fingerprint

Craniofacial Dysostosis
Oral Manifestations
Craniosynostoses
Sutures
Angle Class III Malocclusion
Synostosis
Exophthalmos
Malocclusion
Skull Base
Maxilla
Orthodontics
Epidemiology
Therapeutics
Databases
Phenotype
Polymerase Chain Reaction
DNA

ASJC Scopus subject areas

  • Orthodontics

Cite this

Eziopatogenesi e manifestazioni cliniche della sindrome di Crouzon : revisione della letteratura. / Maspero, C.; Giannini, L.; Iazzetti, F.; Tronca, S.; Farronato, G.

In: Mondo Ortodontico, Vol. 35, No. 5, 11.2010, p. 249-260.

Research output: Contribution to journalArticle

@article{ed49a17b8aad474c8327cadd0075c12c,
title = "Eziopatogenesi e manifestazioni cliniche della sindrome di Crouzon: revisione della letteratura",
abstract = "Objectives: The term Craniofacial dysostosis (CFD) is used to describe familiar forms of synostosis involving different sutures of the cranial base and midface. The aim of this work consists in a systematic review of literature on aetiology, etiopathogenesis, epidemiology, diagnosis, clinical, systemic, and oral manifestations and therapeutic options of Crouzon syndrome. Materials and methods: A systematic review of literature through Medline data bank [www.ncbi.nim.nih.gov/pubmed] was done using {"}Craniofacial Dysostosis{"}, {"}Craniosynostosis{"}, and {"}Crouzon Syndrome{"} as keywords. Results: Crouzon syndrome is the most common craniosynostosis. It develops after an early fusion of superior and posterior sutures of the maxilla with orbital ones, with consequent underdevelopment of the midface and ocular proptosis. Oral manifestations are maxillary hypoplasia, maxillary dental crowding, ogival palatus, and relative mandibular prognathism. Diagnosis can be done either through the evaluation of clinical phenotypes or DNA analysis with polymerase chain reaction (PCR). Treatment is mainly surgical. Orthodontic therapy is performed during the early skeletal maturation. Conclusions: Thanks to the important innovations in surgical techniques and to a muldisciplinary approach, patients affected by this syndrome may aspire to normal cerebral development, physical status, and social relationships.",
keywords = "Craniofacial dysostosis, Craniosynostosis, Crouzon syndrome, Maxillo-facial surgery, Orthodontics",
author = "C. Maspero and L. Giannini and F. Iazzetti and S. Tronca and G. Farronato",
year = "2010",
month = "11",
doi = "10.1016/j.mor.2010.01.002",
language = "Italian",
volume = "35",
pages = "249--260",
journal = "Mondo Ortodontico",
issn = "0391-2000",
publisher = "Elsevier",
number = "5",

}

TY - JOUR

T1 - Eziopatogenesi e manifestazioni cliniche della sindrome di Crouzon

T2 - revisione della letteratura

AU - Maspero, C.

AU - Giannini, L.

AU - Iazzetti, F.

AU - Tronca, S.

AU - Farronato, G.

PY - 2010/11

Y1 - 2010/11

N2 - Objectives: The term Craniofacial dysostosis (CFD) is used to describe familiar forms of synostosis involving different sutures of the cranial base and midface. The aim of this work consists in a systematic review of literature on aetiology, etiopathogenesis, epidemiology, diagnosis, clinical, systemic, and oral manifestations and therapeutic options of Crouzon syndrome. Materials and methods: A systematic review of literature through Medline data bank [www.ncbi.nim.nih.gov/pubmed] was done using "Craniofacial Dysostosis", "Craniosynostosis", and "Crouzon Syndrome" as keywords. Results: Crouzon syndrome is the most common craniosynostosis. It develops after an early fusion of superior and posterior sutures of the maxilla with orbital ones, with consequent underdevelopment of the midface and ocular proptosis. Oral manifestations are maxillary hypoplasia, maxillary dental crowding, ogival palatus, and relative mandibular prognathism. Diagnosis can be done either through the evaluation of clinical phenotypes or DNA analysis with polymerase chain reaction (PCR). Treatment is mainly surgical. Orthodontic therapy is performed during the early skeletal maturation. Conclusions: Thanks to the important innovations in surgical techniques and to a muldisciplinary approach, patients affected by this syndrome may aspire to normal cerebral development, physical status, and social relationships.

AB - Objectives: The term Craniofacial dysostosis (CFD) is used to describe familiar forms of synostosis involving different sutures of the cranial base and midface. The aim of this work consists in a systematic review of literature on aetiology, etiopathogenesis, epidemiology, diagnosis, clinical, systemic, and oral manifestations and therapeutic options of Crouzon syndrome. Materials and methods: A systematic review of literature through Medline data bank [www.ncbi.nim.nih.gov/pubmed] was done using "Craniofacial Dysostosis", "Craniosynostosis", and "Crouzon Syndrome" as keywords. Results: Crouzon syndrome is the most common craniosynostosis. It develops after an early fusion of superior and posterior sutures of the maxilla with orbital ones, with consequent underdevelopment of the midface and ocular proptosis. Oral manifestations are maxillary hypoplasia, maxillary dental crowding, ogival palatus, and relative mandibular prognathism. Diagnosis can be done either through the evaluation of clinical phenotypes or DNA analysis with polymerase chain reaction (PCR). Treatment is mainly surgical. Orthodontic therapy is performed during the early skeletal maturation. Conclusions: Thanks to the important innovations in surgical techniques and to a muldisciplinary approach, patients affected by this syndrome may aspire to normal cerebral development, physical status, and social relationships.

KW - Craniofacial dysostosis

KW - Craniosynostosis

KW - Crouzon syndrome

KW - Maxillo-facial surgery

KW - Orthodontics

UR - http://www.scopus.com/inward/record.url?scp=78049530815&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78049530815&partnerID=8YFLogxK

U2 - 10.1016/j.mor.2010.01.002

DO - 10.1016/j.mor.2010.01.002

M3 - Articolo

AN - SCOPUS:78049530815

VL - 35

SP - 249

EP - 260

JO - Mondo Ortodontico

JF - Mondo Ortodontico

SN - 0391-2000

IS - 5

ER -