المؤلفون: Marie-Lise C. van Veelen, Mark J W van der Oest, Irene M.J. Mathijssen, Marjolein H G Dremmen, Bianca K. den Ottelander, Catherine A de Planque

المساهمون: Plastic and Reconstructive Surgery and Hand Surgery, Radiology & Nuclear Medicine, Neurosurgery

المصدر: Journal of Plastic, Reconstructive and Aesthetic Surgery, 75(2), 797-805. Churchill Livingstone

مصطلحات موضوعية: musculoskeletal diseases, congenital, hereditary, and neonatal diseases and abnormalities, Apert syndrome, Craniosynostosis, Craniosynostoses, Clivus, Humans, Medicine, Retrospective Studies, Skull Base, Foramen magnum, business.industry, Craniofacial Dysostosis, Infant, Newborn, Infant, Crouzon syndrome, Syndrome, Anatomy, Acrocephalosyndactylia, Synostosis, medicine.disease, Hydrocephalus, Skull, medicine.anatomical_structure, Surgery, business

الوصف: Purpose: Cerebellar tonsillar herniation (TH) occurs frequently in syndromic craniosynostosis; however, the exact pathogenesis is unknown. This study evaluates the association between skull base deformities and TH in syndromic craniosynostosis. Methods: Retrospective study MRI study comparing syndromic craniosynostosis to controls. Measured parameters included clivus length, skull base angle, Boogard's angle, foramen magnum area, and cerebellar tonsillar position (TP). The association between skull base parameters and TP was evaluated with linear mixed models, correcting for age and risk factors for TH in craniosynostosis (hydrocephalus, intracranial hypertension, craniocerebral disproportion, and lambdoid synostosis). Results: Two hundred and eighty-two scans in 145 patients were included, and 146 scans in 146 controls. The clivus was smaller at birth, and its growth was retarded in all syndromes. The skull base angle was smaller at birth in Apert and Crouzon syndromes, and the evolution through time was normal. Boogard's angle was smaller at birth in Apert syndrome, and its evolution was disturbed in Apert and Saethre-Chotzen syndromes. The foramen magnum was smaller at birth in Crouzon and Saethre-Chotzen syndromes, and its growth was disturbed in Apert, Crouzon, and Saethre-Chotzen syndromes. TP was higher at birth in Apert syndrome, but lowered faster. In Crouzon syndrome, TP was lower at birth and throughout life. A smaller clivus and larger foramen magnum were associated with a lower TP in controls (p

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ed2e6a46c4a1de0b0ebc6a9bd91413d9Test
https://pure.eur.nl/en/publications/d4e2c0e2-b100-4088-b063-db27ed20eaf3Test

المؤلفون: Luca Massimi, Gianpiero Tamburrini, F. Bianchi, Alberto Benato, Paolo Frassanito

المصدر: Child's Nervous System

مصطلحات موضوعية: Posterior cranial fossa, Basilar invagination, Craniosynostoses, Craniosynostosis, Achondroplasia, Medicine, Humans, Foramen magnum, Focus Session, business.industry, Precision medicine, General Medicine, Anatomy, medicine.disease, Magnetic Resonance Imaging, Hypoplasia, Syringomyelia, Chiari I malformation, Arnold-Chiari Malformation, medicine.anatomical_structure, Cranial Fossa, Posterior, Pediatrics, Perinatology and Child Health, Neurology (clinical), business, Hydrocephalus

الوصف: Background The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic. Methods The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia. Results and conclusions In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e120ba293e4806ebd6a022769fdd4729Test
http://europepmc.org/articles/PMC8510968Test

المؤلفون: Alon Kashanian, Moise Danielpour, James A Stadler, Mari L. Groves

المصدر: Neurosurgery clinics of North America. 33(1)

مصطلحات موضوعية: musculoskeletal diseases, Adult, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Axial skeleton, Spinal stenosis, Constriction, Pathologic, Achondroplasia, medicine, Humans, Foramen Magnum, Child, Foramen magnum, business.industry, Infant, General Medicine, Anatomy, medicine.disease, Hydrocephalus, Cervicomedullary compression, medicine.anatomical_structure, Child, Preschool, Surgery, Neurology (clinical), Neurosurgery, business, Ventriculomegaly

الوصف: Much of the current medical discussion for within centers for skeletal dysplasia and specifically patients with achondroplasia focuses on infancy and early childhood. Most neurosurgical concerns arise due to a defect in the endochondral ossification, resulting on early fusion of the synchondrosis. As patients age, the neurosurgical focus shifts from primarily cranial to spinal concerns. Often pediatric neurosurgeons may continue to follow their patients with skeletal dysplasia. However, general adult neurosurgeons and orthopedic surgeons may see these graduated adults in their practice. This article provides a review of the common neurosurgical concerns for patients with achondroplasia.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c24070b8db58feee17460a9714e7a679Test
https://pubmed.ncbi.nlm.nih.gov/34801139Test

المؤلفون: Nathan Albrecht, Anurag Arora, Chetan C. Shah

المصدر: Radiology Case Reports, Vol 16, Iss 2, Pp 262-267 (2021)
Radiology Case Reports

مصطلحات موضوعية: lcsh:Medical physics. Medical radiology. Nuclear medicine, lcsh:R895-920, Clivus hypoplasia, Case Report, CNS, central nervous system, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, 0302 clinical medicine, Clivus, stomatognathic system, medicine, otorhinolaryngologic diseases, Radiology, Nuclear Medicine and imaging, Foramen magnum, US, ultrasonography, business.industry, Anatomy, medicine.disease, Hypoplasia, CT, computed tomography, Hydrocephalus, Vertebra, Shunt (medical), medicine.anatomical_structure, Clival hypoplasia, Chiari 1 Malformation, Craniocervical junction anomalies, Brainstem, business, MRI, magnetic resonance imaging, 030217 neurology & neurosurgery

الوصف: Chiari 1 Malformation in children is defined as 6 mm or more caudal extension of cerebellar tonsils below the foramen magnum. It is one of the deformities in the spectrum of craniovertebral junction anomalies. We report an unusual case of newborn girl with hypoplasia of bassiocciput of the clivus and extension of brainstem into the oropharynx, in addition to extension of cerebellar tonsils below the foramen magnum. Right anterior arch and right posterior arch of C1 vertebra are absent. Dens of C2 vertebra is asymmetric. Laryngoscope showed a membrane covering the brainstem in the oropharynx. Endotracheal and orogastric tubes were placed. A ventriculo-peritoneal shunt was placed for hydrocephalus. The patient was discharged home at 4.5 months of age with home Hospice care. To the best of our knowledge, this has not been described in the literature. The case illustrates a rare defect in the clivus and unusual protrusion of brainstem into oropharynx.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e282555f6e34e55a947e11c24feb97fdTest
http://www.sciencedirect.com/science/article/pii/S1930043320305914Test

المؤلفون: Hirotaka Ito, Shigeki Yamada, Marie Oshima, M. Ishikawa, K. Yamamoto, M. Yamaguchi, Kazuhiko Nozaki

المصدر: AJNR Am J Neuroradiol

مصطلحات موضوعية: Male, 030218 nuclear medicine & medical imaging, Stress (mechanics), Motion, 03 medical and health sciences, 0302 clinical medicine, Cerebrospinal fluid, Alzheimer Disease, medicine, Foramen, Shear stress, Humans, Radiology, Nuclear Medicine and imaging, Aged, Cerebrospinal Fluid, Foramen magnum, business.industry, Adult Brain, Cerebral Aqueduct, Anatomy, Stroke volume, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Hydrocephalus, Normal Pressure, Hydrocephalus, medicine.anatomical_structure, Cerebral aqueduct, Female, Stress, Mechanical, Neurology (clinical), business, 030217 neurology & neurosurgery

الوصف: Background and purpose:Oscillatory shear stress could not be directly measured in consideration of direction, although cerebrospinal fluid has repetitive movements synchronized with heartbeat. Our aim was to evaluate the important of oscillatory shear stress in the cerebral aqueduct and foramen magnum in idiopathic normal pressure hydrocephalus by comparing it with wall shear stress and the oscillatory shear index in patients with idiopathic normal pressure hydrocephalus.
Materials and methods:By means of the 4D flow application, oscillatory shear stress, wall shear stress, and the oscillatory shear index were measured in 41 patients with idiopathic normal pressure hydrocephalus, 23 with co-occurrence of idiopathic normal pressure hydrocephalus and Alzheimer-type dementia, and 9 age-matched controls. These shear stress parameters at the cerebral aqueduct were compared with apertures and stroke volumes at the foramen of Magendie and cerebral aqueduct.
Results:Two wall shear stress magnitude peaks during a heartbeat were changed to periodic oscillation by converting oscillatory shear stress. The mean oscillatory shear stress amplitude and time-averaged wall shear stress values at the dorsal and ventral regions of the cerebral aqueduct in the idiopathic normal pressure hydrocephalus groups were significantly higher than those in controls. Furthermore, those at the ventral region of the cerebral aqueduct in the idiopathic normal pressure hydrocephalus group were also significantly higher than those in the co-occurrence of idiopathic normal pressure hydrocephalus with Alzheimer-type dementia group. The oscillatory shear stress amplitude at the dorsal region of the cerebral aqueduct was significantly associated with foramen of Magendie diameters, whereas it was strongly associated with the stroke volume at the upper end of the cerebral aqueduct rather than that at the foramen of Magendie.
Conclusions:Oscillatory shear stress, which reflects wall shear stress vector changes better than the conventional wall shear stress magnitude and the oscillatory shear index, can be directly measured on 4D flow MR imaging. Oscillatory shear stress at the cerebral aqueduct was considerably higher in patients with idiopathic normal pressure hydrocephalus.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a1a358958f41deb29e1c6f0dc78673a8Test
https://doi.org/10.3174/ajnr.a6941Test

المؤلفون: Laurent Sakka, Guillaume Coll, M. Delion, F. Abed Rabbo, F. Di Rocco, Vincent Jecko

المساهمون: Service de Neurochirurgie [CHU Clermont-Ferrand, CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Institut Pascal (IP), SIGMA Clermont (SIGMA Clermont)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Centre National de la Recherche Scientifique (CNRS), Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), Physiopathologie, diagnostic et traitements des maladies osseuses / Pathophysiology, Diagnosis & Treatments of Bone Diseases (LYOS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurochirurgie [CHU Clermont-Ferrand]

المصدر: Neurochirurgie
Neurochirurgie, Elsevier Masson, 2019, 65, pp.221-227. ⟨10.1016/j.neuchi.2019.09.005⟩
Neurochirurgie, 2019, 65, pp.221-227. ⟨10.1016/j.neuchi.2019.09.005⟩

مصطلحات موضوعية: musculoskeletal diseases, congenital, hereditary, and neonatal diseases and abnormalities, [SDV]Life Sciences [q-bio], Synchondrosis, Complex craniosynostosis, Craniosynostoses, 03 medical and health sciences, 0302 clinical medicine, Cranial vault, medicine, Humans, Receptor, Fibroblast Growth Factor, Type 2, Child, Foramen magnum, business.industry, Skull, Infant, Newborn, Infant, Crouzon syndrome, Syndrome, Anatomy, medicine.disease, Hydrocephalus, medicine.anatomical_structure, Cranial Fossa, Posterior, Posterior cranial fossa, Child, Preschool, 030220 oncology & carcinogenesis, Mutation, Pfeiffer syndrome, Surgery, Neurology (clinical), business, 030217 neurology & neurosurgery

الوصف: Background The growth of the posterior fossa in syndromic craniostenosis was studied in many papers. However, few studies described the pathophysiological growth mechanisms in non-operated infants with fibroblast growth factor receptor (FGFR) type 2 mutation (Crouzon, Apert or Pfeiffer syndrome), although these are essential to understanding cranial vault expansion and hydrocephalus treatment in these syndromes. Objective A review of the medical literature was performed, to understand the physiological and pathological growth mechanisms of the posterior fossa in normal infants and infants with craniostenosis related to FGFR2 mutation. Discussion Of the various techniques for measuring posterior fossa volume, direct slice-by-slice contouring is the most precise and sensitive. Posterior fossa growth follows a bi-phasic pattern due to opening of the petro-occipital, occipitomastoidal and spheno-occipital sutures. Some studies reported smaller posterior fossae in syndromic craniostenosis, whereas direct contouring studies reported no difference between normal and craniostenotic patients. In Crouzon syndrome, synchondrosis fusion occurs earlier than in normal subjects, and follows a precise pattern. This premature fusion in Crouzon syndrome leads to a stenotic foramen magnum and facial retrusion.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::78934adbf63f487265329b14e2057a40Test
https://hal.archives-ouvertes.fr/hal-03488488Test

المؤلفون: Arun Kumar Srivastava, Kumar Ashish, Anant Mehotra, Jayesh Sardhara, Kuntal Kanti Das

المصدر: Indian Journal of Neurosurgery, Vol 03, Iss 02, Pp 106-109 (2014)

مصطلحات موضوعية: 0301 basic medicine, lcsh:Surgery, Kyphosis, 030105 genetics & heredity, Os Odontoideum, Short stature, lcsh:RC346-429, surgery, 03 medical and health sciences, medicine, craniovertebral junction, lcsh:Neurology. Diseases of the nervous system, Foramen magnum, acromesomyelia, business.industry, lcsh:RD1-811, Anatomy, medicine.disease, Osteochondrodysplasia, Hydrocephalus, medicine.anatomical_structure, Acromesomelic dysplasia Maroteaux type, medicine.symptom, business, marotaeux variant, Syringomyelia

الوصف: Acromesomelic dysplasia Maroteaux type (AMDM) is a rare autosomal recessive osteochondrodysplasia. The responsible gene, AMDM gene, in human beings, has been mapped on 9p13-q12 chromosome by homozygous mapping and pathogenic mutation was later identified in natriuretic receptor B (NPR-B) which has been implicated in the regulation of skeletal growth. Till now, around 40 to 50 cases of AMDM have been described in the world literature. Association of the congenital craniovertebral (CV) junction anomaly has not been reported. Here we are presenting a case of AMDM, with CV junction anomaly. A 10-year boy presented with short stature (122 cm) with short distal limbs, symptomatic for thoracic kyphoscoliosis with back pain. On examination there were no neurological deficits. On radiological investigation, he was found to have short and broad phalanges and toes, thoracic kyphoscoliosis, abnormal pelvic ring, mild ventriculomegaly, cervical syringomyelia and tonsillar descent below foramen magnum, hydrocephalus, os odontoideum with Klippel-Feil anomaly. This was diagnosed as AMDM with congenital os odontoideum, Klippel-Feil anomaly with Arnold-Chiari malformation (ACM) type-1. The patient underwent posterior fossa decompression by removal of foramen magnum ring along with C1 arch for ACM type-1. Kyphosis was left for conservative treatment till further observation and required orthopedic correction in his further age. To the best of our knowledge this is a very rare entity of AMDM with congenital CV junction anomaly.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4aa332f9ced12eeed8bccf0b773b6273Test
https://doi.org/10.4103/2277-9167.138919Test

المؤلفون: Thangamadhan Bosemani, Andrea Poretti, Thierry A.G.M. Huisman, Benedikt Hergan, Gunes Orman, Kathryn A. Carson

المصدر: Child's Nervous System. 31:129-133

مصطلحات موضوعية: Male, musculoskeletal diseases, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Constriction, Pathologic, Emissary veins, Achondroplasia, Humans, Medicine, Foramen Magnum, cardiovascular diseases, Achondroplasia in children, Child, Retrospective Studies, Foramen magnum, business.industry, Ventricular dilatation, Infant, Newborn, Infant, General Medicine, Anatomy, musculoskeletal system, medicine.disease, Cerebral Veins, Skull, medicine.anatomical_structure, Dysplasia, Case-Control Studies, Child, Preschool, Pediatrics, Perinatology and Child Health, cardiovascular system, Female, Neurology (clinical), Radiology, business, Hydrocephalus, Ventriculomegaly

الوصف: Achondroplasia is a skeletal dysplasia with diminished growth of the skull base secondary to defective enchondral bone formation. This leads to narrowing of the foramen magnum and jugular foramina, which further leads to ventricular dilatation and prominence of the emissary veins. The primary goal of our study was to determine a correlation between the degree of ventricular dilatation, jugular foramina and foramen magnum narrowing, as well as emissary vein enlargement.Conventional T2-weighted MR images were evaluated for surface area of the foramen magnum and jugular foramina, ventricular dilatation, and emissary veins enlargement in 16 achondroplasia patients and 16 age-matched controls. Ratios were calculated for the individual parameters using median values from age-matched control groups to avoid age as a confounder.Compared to age-matched controls, in children with achondroplasia, the surface area of the foramen magnum (median 0.50 cm(2), range 0.23-1.37 cm(2) vs. 3.14 cm(2), 1.83-6.68 cm(2), p 0.001) and jugular foramina (median 0.02 cm(2), range 0-0.10 cm(2) vs. 0.21 cm(2), 0.03-0.61 cm(2), p 0.001) were smaller, whereas ventricular dilatation (0.28, 0.24-0.4 vs. 0.26, 0.21-0.28, p 0.001) and enlargement of emissary veins (6, 0-11 vs. 0, p 0.001) were higher. Amongst the patients, Spearman correlation and multiple regression analysis did not reveal correlation for severity between the individual parameters.Our study suggests that in children with achondroplasia, (1) the variation in ventricular dilatation may be related to an unquantifiable interdependent relationship of emissary vein enlargement, venous channel narrowing, and foramen magnum compression and (2) stable ventricular size facilitated by interdependent factors likely obviates the need for ventricular shunt placement.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::21971c762b2bbc44172ece8240475208Test
https://doi.org/10.1007/s00381-014-2559-4Test

المؤلفون: Junji Kishimoto, Koji Iihara, Goki Eriguchi, Akio Hiwatashi, Nobuya Murakami, Masayuki Ochiai, Kimiaki Hashiguchi, Osamu Togao, Takato Morioka

المصدر: Child's Nervous System. 32:1069-1078

مصطلحات موضوعية: Male, Meningomyelocele, Neurosurgical Procedures, 030218 nuclear medicine & medical imaging, Cohort Studies, 03 medical and health sciences, Postoperative Complications, 0302 clinical medicine, Cerebrospinal fluid, Cerebellum, medicine, Humans, Retrospective Studies, Surgical repair, Foramen magnum, Models, Statistical, medicine.diagnostic_test, business.industry, Infant, Newborn, Infant, Magnetic resonance imaging, General Medicine, Anatomy, medicine.disease, Magnetic Resonance Imaging, Arnold-Chiari Malformation, Hydrocephalus, Vertebra, Shunting, Spina Bifida Cystica, medicine.anatomical_structure, Cranial Fossa, Posterior, CHIARI MALFORMATION TYPE II, Pediatrics, Perinatology and Child Health, Female, Neurology (clinical), business, 030217 neurology & neurosurgery

الوصف: To document long-term morphological changes of Chiari type II malformation (CM-II) following closure of spina bifida manifesta (SBM). We retrospectively evaluated postnatal magnetic resonance images of the CM-II and posterior fossa (PF) in 28 consecutive cases. We measured changes in vertebral level and length of the cerebellar peg (CP), cerebrospinal fluid (CSF) spaces anterior and posterior to the cerebrospinal junction, PF area, and the anteroposterior diameters of the foramen magnum (FM) and C1 vertebra. We examined the morphological differences between the cases with and without ventriculoperitoneal (VP) shunting and derived predicted means by nonlinear mixed-effect modeling. At birth, there were significant differences in CP length, PF area, and FM and C1 diameters between those who underwent VP shunting and those who did not. In cases with a CP below C1, VP shunting was required in every case but one. In those with visible CSF space at birth, VP shunts were not required. In 17 of 18 cases with a CP below C1, the vertebral level ascended by mean two vertebral levels (range 0–5 levels) within 4–6 months of delivery. In the remaining case, slowly progressive hydrocephalus and delayed CP descent required VP shunting at 8 months. Predicted mean CP length and FM and C1 diameters were greater in those who underwent VP shunting, but there was no difference in predicted mean PF area. The morphology of CM-II and the presence of hydrocephalus influence each other in children who have undergone postnatal SBM repair.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ba6ae1ae92c2ef56db4ab75e9beee324Test
https://doi.org/10.1007/s00381-016-3041-2Test

المؤلفون: Francis Brunelle, Guillaume Coll, Christian Sainte-Rose, Laurent Selek, Eric Arnaud, Federico Di Rocco, Corinne Collet

المصدر: Child's Nervous System. 28:1525-1535

مصطلحات موضوعية: Male, Craniofacial abnormality, Palatine Tonsil, Statistics, Nonparametric, Prolapse, medicine, Humans, Foramen Magnum, Receptor, Fibroblast Growth Factor, Type 2, Lambdoid suture, Chiari malformation, Foramen magnum, business.industry, Craniofacial Dysostosis, Infant, Newborn, Infant, Crouzon syndrome, General Medicine, Anatomy, medicine.disease, Sagittal plane, Hydrocephalus, medicine.anatomical_structure, Biphasic Pattern, Case-Control Studies, Child, Preschool, Mutation, Pediatrics, Perinatology and Child Health, Female, Neurology (clinical), Tomography, X-Ray Computed, business

الوصف: Though the craniovertebral junction is often abnormal in children with Crouzon’s syndrome, no study had measured accurately the size of their foramen magnum (FM). We compared the FM size (area, diameters) on computed tomography examination in 21 children with a genetically confirmed Crouzon’s syndrome prior to any surgery and in 23 control children without craniofacial abnormalities. We extrapolated the growth pattern in both groups. We found a statistically significant smaller FM area (p = 0.0228), FM sagittal diameter (p = 0.0287), and FM sagittal posterior diameter (p = 0.0023) in children with Crouzon’s syndrome. No differences were detected with regard to the transversal diameter. Hydrocephalus in children with Crouzon’s syndrome was associated with a small FM area (p = 0.05), small sagittal diameter (p = 0.023), small sagittal posterior diameter (p = 0.0173), and reduced transversal diameter (p = 0.03985). No association of the aforementioned findings was found with the position of the cerebellar tonsils or the lambdoid suture functional state (open or fused). Comparable results were observed among the two genetic forms (exon 8 or 10 mutations). Concerning the growth pattern, a first phase of rapid increase and a second phase of slow increase could be recognized in all the measurements in both populations, though with some significant differences. The growth of FM follows a biphasic pattern in both Crouzon’s and control children. The sagittal diameter and the global size of the FM are mostly affected in children with Crouzon’s syndrome. The small FM, especially its posterior part, is likely to play a key role in the physiopathology of hydrocephalus.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b99a128ebc7ee7edcb34617cadf54a73Test
https://doi.org/10.1007/s00381-012-1805-xTest