Cranial Fossa Volume and Morphology Development in Apert Syndrome

Conclusions: Malformation of the middle cranial fossa is an early, perhaps the initial, pivotal cranial morphologic change in Apert syndrome. Increased cranial fossae depth is an inherent characteristic of the maldevelopment. Normalization of cranial volume and circumference overall may not achieve a normal skull structure, as it does not correct regional craniocerebral disproportion.
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Original Articles 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
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
Background: Orbital dysmorphology and midface retrusion are the hallmarks of Crouzon and Apert syndromes. The precise nature of this deficiency is not known. Methods: Untreated Crouzon and Apert syndrome patients and age- and sex-matched controls were included. Computed tomographic scans were digitized and reconstructed. Craniometric and volumetric data relating to the orbit were collected. Results: Thirty-one scans were included (control, n = 12; Crouzon; n = 9; Apert, n = 10). The mean age of the Apert group was 5.31 ± 5 years; Crouzon, 5.77 ± 2.7 years; and control, 6.4 ± 3.6 years. The bony orbit l...
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Original Articles Source Type: research
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