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Craniofacial features in osteogenesis imperfecta: A cephalometric study

Identifieur interne : 007540 ( Main/Exploration ); précédent : 007539; suivant : 007541

Craniofacial features in osteogenesis imperfecta: A cephalometric study

Auteurs : Janna Waltimo-Sirén [Finlande] ; Marina Kolkka [Finlande] ; Seppo Pynnönen [Finlande] ; Kaija Kuurila [Finlande] ; Ilkka Kaitila [Finlande] ; Outi Kovero [Finlande]

Source :

RBID : ISTEX:03479D4261085202B24E5F088C1F016088D77DBA

Descripteurs français

English descriptors

Abstract

Osteogenesis imperfecta (OI) is a heterogeneous group of connective tissue diseases that mainly manifest as bone fragility and skeletal deformity. In most families it segregates as a dominant trait and results from mutations in type I collagen genes. In this study we analyzed the size and form of the bony structures in heads of 59 consecutive patients with OI types I, III, or IV (Sillence classification), using lateral radiographs. Paired controls were matched for gender and age. The purpose was to obtain baseline information of craniofacial development in OI patients that have not received bisphosphonate treatment. In OI type I we found smaller than normal linear measurements, indicating a general growth deficiency, but no remarkable craniofacial deformity. In OI types III and IV, the growth impairment was pronounced, and the craniofacial form was altered as a result of differential growth deficiency and bending of the skeletal head structures. We found strong support both for an abnormally ventral position of the sella region due to bending of the cranial base, and for a closing mandibular growth rotation. Vertical underdevelopment of the dentoalveolar structures and the condylar process were identified as the main reasons for the relative mandibular prognathism in OI. Despite of the widespread intervention with bisphosphonates, the facial growth impairment will probably remain characteristic for many OI patients, and their orthodontic treatment should be further developed. © 2005 Wiley‐Liss, Inc.

Url:
DOI: 10.1002/ajmg.a.30523


Affiliations:


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Le document en format XML

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<term>Abnormal dentin</term>
<term>Alveolar height</term>
<term>Angular measurements</term>
<term>Anterior</term>
<term>Anterior calvaria</term>
<term>Anterior cranial base</term>
<term>Anterior position</term>
<term>Anterior skull base</term>
<term>Baseline information</term>
<term>Bisphosphonate treatment</term>
<term>Blue sclerae</term>
<term>Bone fragility</term>
<term>Brittle bone disease</term>
<term>Calvaria</term>
<term>Case report</term>
<term>Cephalograms</term>
<term>Cephalometric</term>
<term>Cephalometric analysis</term>
<term>Cephalometric values</term>
<term>Chain association</term>
<term>Condylar process</term>
<term>Contemporary orthodontics</term>
<term>Control groups</term>
<term>Cranial</term>
<term>Cranial base</term>
<term>Cranial base angle</term>
<term>Craniofac genet</term>
<term>Craniofacial</term>
<term>Craniofacial bones</term>
<term>Craniofacial development</term>
<term>Craniofacial features</term>
<term>Craniofacial form</term>
<term>Craniofacial growth</term>
<term>Craniofacial phenotype</term>
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<term>Dentin</term>
<term>Dentoalveolar</term>
<term>Dentoalveolar structures</term>
<term>Dermal bones</term>
<term>Different types</term>
<term>Esthetic problems</term>
<term>External pressure</term>
<term>Facial</term>
<term>Facial angle</term>
<term>Facial growth</term>
<term>Facial growth rotation</term>
<term>Facial height</term>
<term>Finland</term>
<term>Finnish women dentists</term>
<term>Genetic heterogeneity</term>
<term>Glorieux</term>
<term>Gonial angle</term>
<term>Growth impairment</term>
<term>Growth reduction</term>
<term>Growth rotation</term>
<term>Harvold</term>
<term>Harvold mandibular measurement</term>
<term>Harvold maxillary measurement</term>
<term>Healthy controls</term>
<term>Helsinki</term>
<term>Horizontal base line</term>
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<term>Incisor</term>
<term>Labially proclined</term>
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<term>Lateral cephalograms</term>
<term>Lateral radiographs</term>
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<term>Lower incisors</term>
<term>Lower molar cusp</term>
<term>Mandible</term>
<term>Mandibular</term>
<term>Mandibular base</term>
<term>Mandibular bone</term>
<term>Mandibular condyle</term>
<term>Mandibular growth rotation</term>
<term>Mandibular lines</term>
<term>Mandibular ramus</term>
<term>Maxilla</term>
<term>Maxillary</term>
<term>Maxillary bone</term>
<term>Maximum distance</term>
<term>Mosby year book</term>
<term>Multiple fractures</term>
<term>Mutation</term>
<term>Nasion point</term>
<term>Natural size</term>
<term>Neural tissue</term>
<term>Neurocranium</term>
<term>Normal population</term>
<term>Occlusal</term>
<term>Occlusal relationship</term>
<term>Oral cavity</term>
<term>Oral surg</term>
<term>Orthodontic consultation</term>
<term>Orthodontic treatment</term>
<term>Orthognathic surgery</term>
<term>Osteoclastic activity</term>
<term>Osteogenesis</term>
<term>Osteogenesis imperfecta</term>
<term>Osteogenesis imperfecta patients</term>
<term>Palatal</term>
<term>Palatal plane</term>
<term>Palatal planes</term>
<term>Partial gene deletions</term>
<term>Patient group</term>
<term>Posterior calvaria</term>
<term>Posterior neurocranium</term>
<term>Posterior parts</term>
<term>Prognathism</term>
<term>Ramus</term>
<term>Reference points</term>
<term>Relative height</term>
<term>Relative mandibular prognathism</term>
<term>Sagittal</term>
<term>Sagittal growth</term>
<term>Sagittal position</term>
<term>Sagittal relationship</term>
<term>Second edition</term>
<term>Sella</term>
<term>Sella region</term>
<term>Severe bone fragility</term>
<term>Short stature</term>
<term>Sillence</term>
<term>Skeletal class</term>
<term>Skeletal deformities</term>
<term>Skeletal head structures</term>
<term>Skull</term>
<term>Skull base</term>
<term>Statistical analyses</term>
<term>Surgical management</term>
<term>Treatment possibilities</term>
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<term>Upper incisor edge</term>
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<term>Anterior cranial base</term>
<term>Anterior position</term>
<term>Anterior skull base</term>
<term>Baseline information</term>
<term>Bisphosphonate treatment</term>
<term>Blue sclerae</term>
<term>Bone fragility</term>
<term>Brittle bone disease</term>
<term>Calvaria</term>
<term>Case report</term>
<term>Cephalograms</term>
<term>Cephalometric</term>
<term>Cephalometric analysis</term>
<term>Cephalometric values</term>
<term>Chain association</term>
<term>Condylar process</term>
<term>Contemporary orthodontics</term>
<term>Control groups</term>
<term>Cranial</term>
<term>Cranial base</term>
<term>Cranial base angle</term>
<term>Craniofac genet</term>
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<term>Craniofacial bones</term>
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<term>Craniofacial phenotype</term>
<term>Craniofacial structures</term>
<term>Defect</term>
<term>Dentin</term>
<term>Dentoalveolar</term>
<term>Dentoalveolar structures</term>
<term>Dermal bones</term>
<term>Different types</term>
<term>Esthetic problems</term>
<term>External pressure</term>
<term>Facial</term>
<term>Facial angle</term>
<term>Facial growth</term>
<term>Facial growth rotation</term>
<term>Facial height</term>
<term>Finland</term>
<term>Finnish women dentists</term>
<term>Genetic heterogeneity</term>
<term>Glorieux</term>
<term>Gonial angle</term>
<term>Growth impairment</term>
<term>Growth reduction</term>
<term>Growth rotation</term>
<term>Harvold</term>
<term>Harvold mandibular measurement</term>
<term>Harvold maxillary measurement</term>
<term>Healthy controls</term>
<term>Helsinki</term>
<term>Horizontal base line</term>
<term>Imperfecta</term>
<term>Incisor</term>
<term>Labially proclined</term>
<term>Lateral</term>
<term>Lateral cephalograms</term>
<term>Lateral radiographs</term>
<term>Long bones</term>
<term>Lower incisor apex</term>
<term>Lower incisor edge</term>
<term>Lower incisors</term>
<term>Lower molar cusp</term>
<term>Mandible</term>
<term>Mandibular</term>
<term>Mandibular base</term>
<term>Mandibular bone</term>
<term>Mandibular condyle</term>
<term>Mandibular growth rotation</term>
<term>Mandibular lines</term>
<term>Mandibular ramus</term>
<term>Maxilla</term>
<term>Maxillary</term>
<term>Maxillary bone</term>
<term>Maximum distance</term>
<term>Mosby year book</term>
<term>Multiple fractures</term>
<term>Mutation</term>
<term>Nasion point</term>
<term>Natural size</term>
<term>Neural tissue</term>
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<term>Normal population</term>
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<term>Occlusal relationship</term>
<term>Oral cavity</term>
<term>Oral surg</term>
<term>Orthodontic consultation</term>
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<term>Orthognathic surgery</term>
<term>Osteoclastic activity</term>
<term>Osteogenesis</term>
<term>Osteogenesis imperfecta</term>
<term>Osteogenesis imperfecta patients</term>
<term>Palatal</term>
<term>Palatal plane</term>
<term>Palatal planes</term>
<term>Partial gene deletions</term>
<term>Patient group</term>
<term>Posterior calvaria</term>
<term>Posterior neurocranium</term>
<term>Posterior parts</term>
<term>Prognathism</term>
<term>Ramus</term>
<term>Reference points</term>
<term>Relative height</term>
<term>Relative mandibular prognathism</term>
<term>Sagittal</term>
<term>Sagittal growth</term>
<term>Sagittal position</term>
<term>Sagittal relationship</term>
<term>Second edition</term>
<term>Sella</term>
<term>Sella region</term>
<term>Severe bone fragility</term>
<term>Short stature</term>
<term>Sillence</term>
<term>Skeletal class</term>
<term>Skeletal deformities</term>
<term>Skeletal head structures</term>
<term>Skull</term>
<term>Skull base</term>
<term>Statistical analyses</term>
<term>Surgical management</term>
<term>Treatment possibilities</term>
<term>Triple helix</term>
<term>Upper incisor apex</term>
<term>Upper incisor edge</term>
<term>Upper incisors</term>
<term>Upper molar cusp</term>
<term>Vertical growth</term>
<term>Viola players</term>
<term>Wormian bones</term>
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<front>
<div type="abstract" xml:lang="en">Osteogenesis imperfecta (OI) is a heterogeneous group of connective tissue diseases that mainly manifest as bone fragility and skeletal deformity. In most families it segregates as a dominant trait and results from mutations in type I collagen genes. In this study we analyzed the size and form of the bony structures in heads of 59 consecutive patients with OI types I, III, or IV (Sillence classification), using lateral radiographs. Paired controls were matched for gender and age. The purpose was to obtain baseline information of craniofacial development in OI patients that have not received bisphosphonate treatment. In OI type I we found smaller than normal linear measurements, indicating a general growth deficiency, but no remarkable craniofacial deformity. In OI types III and IV, the growth impairment was pronounced, and the craniofacial form was altered as a result of differential growth deficiency and bending of the skeletal head structures. We found strong support both for an abnormally ventral position of the sella region due to bending of the cranial base, and for a closing mandibular growth rotation. Vertical underdevelopment of the dentoalveolar structures and the condylar process were identified as the main reasons for the relative mandibular prognathism in OI. Despite of the widespread intervention with bisphosphonates, the facial growth impairment will probably remain characteristic for many OI patients, and their orthodontic treatment should be further developed. © 2005 Wiley‐Liss, Inc.</div>
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