Mandibular Spatial Reorientation and Morphological Alteration of Crouzon and Apert Syndrome

Background From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. Methods One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analysis. Results In Crouzon syndrome, the angle between the cranial base and gnathion was increased prior to 6 months of age by 10.29 degrees (P
Source: Annals of Plastic Surgery - Category: Cosmetic Surgery Tags: Research Source Type: research

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Conclusions: Occipitofrontal circumference and overall intracranial volume is not always consistent in individual subunits of Apert syndrome. Detailed and segmental anterior, middle, and posterior cranial fossae volumes and morphology should be analyzed to see what impact this may have related to surgical planning.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Original Articles Source Type: research
Background: Apert syndrome is frequently combined with respiratory insufficiency, because of the midfacial deformity which, in turn, is influenced by the malformation of the skull base. Respiratory impairment resulting from Apert syndrome is caused by multilevel limitations in airway space. Therefore, this study evaluated the segmented nasopharyngeal and laryngopharyngeal anatomy to clarify subcranial anatomy in children with Apert syndrome and its relevance to clinical management. Methods: Twenty-seven patients (Apert syndrome, n = 10; control, n = 17) were included. All of the computed tomographic scans were obtaine...
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Ideas and Innovations Source Type: research
Conclusions: Bicoronal synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined pansynostosis patients have flatter cranial base, whereas the combined unilateral coronal synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.
Source: Plastic and Reconstructive Surgery – Global Open - Category: Cosmetic Surgery Tags: Original Article Source Type: research
This study aims to explore the chronology and pathogenesis of the development of craniofacial anatomic relationships and to verify the positional correlates between skull and facial structures in Apert syndrome. Fifty-four computed tomography scans (Apert, nā€Š=ā€Š18; control, nā€Š=ā€Š36) were included and divided into 3 age subgroups. Craniofacial 3-dimensional cephalometries were analyzed by Materialize software. The angle between sella-nasion plane and maxillary plane widens 7.74° (Pā€Š=ā€Š0.003) prior to 6 months of age; thereafter, this widening increases by 10.36° (Pā€Š
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Original Articles Source Type: research
We present the CT scan-derived turricephaly index (TI) as a quotient of the maximal occipito-frontal length of the skull to the distance from the centre of the sella to the highest point on the vertex as a validated tool for assessing turricephaly and evaluating surgical techniques aimed at reducing it.Materials and MethodsMeasurements taken from CTs of non-operated children with Apert syndrome and age-matched controls were analysed using Centricity PACS system (from the lateral scout image) and the thick-sliced Osirix tool. CTs from non-operated children with Apert syndrome were used to investigate the natural history of ...
Source: Journal of Cranio Maxillofacial Surgery - Category: ENT & OMF Source Type: research
In this study, we aimed to explore the evolution of cranial basal dysmorphology and the chronology of these deformities in Apert syndrome, by objectively analyzing three-dimensional measurements. Fifty-four CT scans from unoperated patients (Apert, n = 18; control, n = 36) were included in this study, with age range from 3 days to 24 years. Before 6 months of age, Apert's anterior cranial base was widened 60%. Between 6 months and 2 years of age, the whole cranial base length, anterior cranial base length and posterior cranial base length decreased 8%, 8% and 14%, respectively. The greater sphenoid wing...
Source: Journal of Cranio Maxillofacial Surgery - Category: ENT & OMF Source Type: research
Conclusions: Apert syndrome children have a larger intracranial volume than children with other syndromic craniosynostotic conditions and unaffected children but maintain a similar occipitofrontal circumference. This study demonstrates high correlation between intracranial volume and occipitofrontal circumference with clinical care implications. The authors’ reference growth curves can be used to monitor intracranial volume change over time and correct operative change for underlying growth.
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Original Articles Source Type: research
Publication date: Available online 15 October 2018Source: British Journal of Oral and Maxillofacial SurgeryAuthor(s): X. Lu, A.J. Forte, R. Sawh-Martinez, R. Wu, R. Cabrejo, D.M. Steinbacher, M. Alperovich, N. Alonso, J.A. PersingAbstractBony malformations of the orbit and alterations to the soft tissue in Apert syndrome contribute to ophthalmic dysfunction. Recognised structural malformation of the sphenoid and ethmoid sinuses, together with corresponding deformities in the anterior and middle cranial base, are characteristic. Our aim was to explore the underlying structural components of disfigurement and the consequent ...
Source: British Journal of Oral and Maxillofacial Surgery - Category: ENT & OMF Source Type: research
In this study, we aimed to explore the evolution of cranial basal dysmorphology and the chronology of these deformities in Apert syndrome, by objectively analyzing three-dimensional measurements. Fifty-four CT scans from unoperated patients (Apert, n  = 18; control, n = 36) were included in this study, with age range from 3 days to 24 years.
Source: Journal of Cranio-Maxillofacial Surgery - Category: ENT & OMF Authors: Source Type: research
This study aimed to assess the postoperative ossification of the anterior skull base and pedicled flaps following frontofacial monobloc advancement, and to identify factors influencing this ossification. Methods: Measurements of the skull base only and of the ossified pedicled flaps together with the skull base were performed on computed tomographic scans at the nasofrontal and the nasoethmoid frontal junctions. The total thickness of the skull vault was measured and a qualitative defect score for the anterior skull base was computed. Results: Twenty-two patients who underwent frontofacial monobloc advancement at a m...
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Original Articles Source Type: research
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