Frequency of craniosynostosis in Yorkshire, UK.
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Craniosynostoses↗
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Surgical correction of craniofacial malformations.
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Bone Transplantation↗
Frontonasal suture expansion in the rabbit using titanium screws.
PURPOSE: This study investigated the ability to use titanium screws to provide stable skeletal points in the growing craniofacial region of the rabbit for suture expansion. These screws provided sites for direct application of external forces to alter growth and anatomic form. MATERIAL AND METHODS: Twenty-one rabbits (30 days old) were divided into three groups: control (n = 9), experimental (n = 9, and sham (n = 3). Four four-holed AO/ASIF commercially pure titanium craniofacial plates were contoured into an L-shape with a 90 degree angle at the midpoint. The plates were placed bilaterally in the frontal and nasal bone sites and secured with 2.0-mm diameter, 4-mm long, commercially pure titanium screws in both the sham and experimental groups. After 4 week of healing, a spring mechanism with a distraction force of 55 g was activated between ipsilateral plates and across the frontonasal sutures bilaterally in the experimental group for 5 weeks. No force was applied between the plates in the sham group. A preliminary evaluation of the bone-implant interface and the changes in the suture was done histologically. Morphologic changes were measured using cephalometric radiographs and direct anatomic measurements. RESULTS: The experimental group showed a significant increase in growth across the frontonasal suture in comparison with the sham group (P < .05). In addition, an increase in the length of the nasal and frontal bones in the expanded group was observed in comparison with the control and sham groups (P < .05). Histologically, a mixture of woven and lamellar bone was seen in the suture region and lamellar bone was seen in the screw-bone interface. CONCLUSION: This study indicates that titanium screws in the developing rabbit skull can provide stable sites for the direct application of external forces, producing secondary changes in skeletal morphology. This laboratory models provides a useful system for the further study of growth modification using such external mechanical forces.
Animals↗
Magnetic resonance imaging to visualize the internal anatomy in the Baller-Gerold syndrome.
The Baller-Gerold syndrome is identified by radial aplasia with craniosynostosis. Only 12 cases have been reported previously in the literature; none of the reports describe surgical treatment for the radial aplasia. We report the case of a 10-month-old female infant with this syndrome, including severe bilateral radial clubhands. A radial anlage, which was tethering growth of the ulna, was suspected on physical examination and confirmed with magnetic resonance imaging. Resection of the anlage resulted in dramatic improvement in elbow extension, but additional follow-up will be necessary to determine the complete functional effect of the procedure.
Craniosynostoses↗
Craniocerebral computed tomography in the neonate.
Craniocerebral computed tomography (CT) is an extremely important component of the armanentarium for diagnosis of neurological disorders in the neonate. The indications, techniques, normal anatomy, and pathologic abnormalities of CT scans in newborn infants are presented. Hydrocephalus and intracranial hemorrhage are discussed in depth.
Astrocytoma↗
A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation.
A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported. The patient developed congenital hydrocephalus, upper airway obstruction and breath holding spells. Ventriculo-peritoneal shunt improved the hydrocephalus, but the patient progressively developed apneic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervical vertebrae and craniectomy at the occipital bone were performed to relieve the brain stem compression. Postoperatively, the respiratory symptoms improved slightly, but 8 weeks later the patient suddenly died. Literature survey revealed that this is the second case report of CLS associated with Chiari malformation. It was assumed that the respiratory problems in our patient are ascribable to the Chiari malformation as well as the upper airway obstruction. The patient here reported seems to be a case of Beare-Stevenson cutis gyrata syndrome, in view of an association of multiple characteristic dysmorphic features, in addition to CLS.
Abnormalities, Multiple↗
Value of computed tomography in the evaluation of craniosynostosis.
Forty-seven patients with abnormal cranial shape and the clinical diagnosis of craniosynostosis were assessed by computed tomography (CT). In 13 patients the diagnosis of craniosynostosis was established on the basis of skull radiographs and radionuclide bone scans. Eight out of 13 patients had normal cerebral anatomy demonstrated by CT. In four of the 13 patients CT revealed abnormalities not amenable to surgical correction. The only patient in whom CT scanning influenced therapy presented with elevated intracranial pressure secondary to shunt failure.
Adolescent↗
A population-based study of craniosynostosis.
A population-based study of the incidence of craniosynostosis was conducted among residents of Olmsted County, Minnesota, born between 1 January 1976 and 31 December 1985. This study included only those with primary craniosynostosis who were less than 5 years of age at the time of diagnosis. Cases of craniosynostosis were classified as definite or probable. Definite cases had craniectomies or radiographic evidence confirming craniosynostosis, while patients for whom there was enough concern about craniosynostosis to order a skull radiograph were classified as probable cases. With these definitions, the incidence of craniosynostosis in Olmsted County was 3.1/10,000 births for definite cases (95% CI, 0.4-5.8) and 13.6/10,000 for all cases combined (95% CI, 7.9-19.3). These rates were compared to non-population-based minimal and maximal estimates of craniosynostosis frequency, 4/10,000 births and respectively. The rate observed in this study for definite cases, 3.1/10,000 births, is statistically consistent with the previous low estimate (observed/expected ratio 0.8, 95% CI, 0.2-1.8), and the rate observed for all cases in this study, 13.6, is consistent with the high estimate (observed/expected ratio 1.4, 95% CI, 0.8-2.1). Differences between definite and probable cases in this study are presented, along with comparisons with recent population-based data.
Child, Preschool↗
Finding genes involved in human developmental disorders.
Recent advances in the human genome initiative have accelerated positional cloning efforts toward identification of a number of genes responsible for human developmental anomalies, particularly those involving the skeletal system. Genotype/phenotype comparison and functional analysis of these genes will further elucidate pathways of normal and abnormal human development of the skeletal and other organ systems.
Abnormalities, Multiple↗
Fibroblast growth factors in mammalian development.
Polypeptide growth factors are secreted signalling molecules that function as intercellular communicators. Detailed analyses of the expression and function of members of the fibroblast growth factor (FGF) family and their recepotors have demonstrated that the FGF signalling pathways play essential roles in regulating cellular proliferation, differentiation and tissue patterning during vertebrate embryogenesis. Recent studies on the molecular basis of human dysmorphic syndromes have revealed that aberrant FGF signalling during limb and skeletal development can lead to pathogenesis.
Achondroplasia↗
Impacting infant head shapes.
Infant sleep position impacts the development of head shape. Changes in infant sleep position, specifically the movement toward supine sleep, have led to a redefinition of normal head shape for infants in the United States. Historically, a dolichocephalic (elongated) head shape was the norm. Currently the norm has changed to a more brachycephalic (shorter and broader) shape. Since the American Academy of Pediatrics' Back to Sleep Campaign, the incidence of positional plagiocephaly has increased dramatically with a concurrent rise in the incidence of torticollis. Infants who require newborn intensive care, particularly premature infants, are more prone to positional plagiocephaly and dolichocephaly. Both can be prevented or minimized by proper positioning. The infant with an abnormal head shape requires careful evaluation; treatment varies according to the etiology. Craniosynostosis, a less common but pathological etiology for plagiocephaly, should be considered in the diagnostic process. Successful treatment of positional plagiocephaly and dolichocephaly includes systematic positioning changes to overcome the mechanical forces of repetitive positioning, physical and/or occupational therapy to treat underlying muscle or developmental challenges, and in some cases, molding helmet therapy.
Craniofacial Abnormalities↗
[Anaesthesia for craniosynostosis: increase water and electrolyte therapy].
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Anesthesiology↗
[Study of the morphologic variations of the scaphocephaly. Deduction for their systematisation].
PURPOSE: The scaphocephaly is the most frequent craniosynostosis secondary to a premature synostosis of the sagittal suture. Despite this univocal pathogeny there are a great heterogeneity in the skull deformation and their systematisation stay imprecise. The aim of this study was to analyse the different morphology of the scaphocephaly by 3D CT-scan to clarify the different shapes. MATERIAL AND METHODS: This retrospective study was done from the morphometric analysis of forty-two 3D CT-scan (10 girls and 32 boys). The scaphocephaly was classified in two groups according to the systematisation of Stricker and Montaut: the sphenocephaly (large forehead) and the leptocephaly (narrow forehead). The variation of the calvaria shape was analysed and different index were calculated. RESULTS: There were 30 sphenocephaly and 12 leptocephaly. Excepted for the forehead deformation there was a morphometric difference of the occipital pole that was backward rotated in the leptocephaly. In the two groups there were retro-coronal constriction (40.5%) or pre-lambdoid constriction (9.5%) independently of the sagittal synostosis degree. CONCLUSIONS: The morphometric analysis shows that the deformation of the skull secondary to the premature synostosis of the sagittal suture can be different. The adaptative defect of the metopic suture can be to explain this difference. This deformation can be harmonious, or with a retro-coronal constriction resulting in a bitemporal narrowing, or with a pre-lambdoid constriction resulting in an occipital chignon. These results conduce to qualify the surgical correction to adapt at the different shapes.
Child, Preschool↗
[Role of lipo-filling in the treatment of sequelae in craniosynostosis surgery].
AIM: This study presents an innovative technique of correction of cranioplasty sequelae by lipo-filling in 9 patients treated for cranioplasty in childhood with a long-term follow-up. PATIENTS AND METHODS: A prospective study was conducted from January 2001 to February 2005, including all patients treated with lipo-filling technique in the Plastic Surgery department of Lille. For a period of 4 years and 5 months, 144 patients were operated on with 206 lipo-filling procedures: 30 patients for cosmetic purposes and 114 patients for reconstructive surgery. In the current investigation, we examined the 9 cases of lipo-filling which were performed in complement to craniofacial surgery. All patients presented with a sub-cutaneous fronto-temporal deformation which in 3 of the cases was associated to a frontal medial defect. The assessment of results was performed in the context of post-operative follow-up visits by an observer. RESULTS: Nine patients, with a mean age of 16,7 years and a ratio of 1 female for 2 males underwent a total of 17 lipo-filling procedures. The mean time lapse was 15,06 months. 9 patients judged the result to be good. No complication was observed. CONCLUSION: The S.R. Coleman technique of fat grafting seems to offer several advantages, namely the simplicity of the procedure itself, its apparent innocuity, its reproducibility, as well as patients and surgeons satisfaction. It appears to be an alternative of choice in the corrective surgery of craniosynostosis.
Adipose Tissue↗
Sustained delivery of bioactive cytokine using a dense collagen gel vehicle collagen gel delivery of bioactive cytokine.
OBJECTIVE: The use of cytokines as localized therapeutic agents is limited by the lack of a satisfactory delivery system. The aim of the current investigation was to determine the release kinetics and bioactivity of a simplified cytokine/collagen gel system designed to achieve extended, local delivery of bioactive cytokines at sites of premature cranial suture fusion (craniosynostosis). DESIGN: Cytokine release was determined by ELISA measurements of Tgf-beta3 collected in media. Cytokine bioactivity was determined by measuring the effect of conditioned media, containing released Tgf-beta3, on mink lung epithelial cell proliferation and osteoblast alkaline phosphatase activity. Osteoblast response was evaluated by measuring proliferation of cells cultured on collagen gel containing Tgf-beta3 using an AlamarBlue assay. RESULTS: Gels loaded with 100 and 500 ng of Tgf-beta3 produced a sustained release over 14 days with a pattern of initial large release followed by a gradual reduction in the amount released over the time. The reduced release over time was correlated to the amount initially loaded. Mink lung epithelial cell assay results indicated that Tgf-beta3 released from the collagen gel retained its bioactivity following incorporation into the collagen gel and release into the media. This bioactivity was further illustrated by a decreased alkaline phosphatase activity measured in osteoblasts cultured on the gels loaded with Tgf-beta3. Osteoblast proliferation assays demonstrated that the collagen gel has an inherent inhibitory effect on osteoblast cell number. CONCLUSIONS: This collagen gel/cytokine delivery system can retain and release bioactive cytokine over a prolonged period. These results will allow for better optimization of future in vitro and in vivo studies directed at improving the treatment of craniosynostosis.
Alkaline Phosphatase↗
Cranial reshaping employing distraction and contraction in the treatment of sagittal synostosis.
We treated four patients with scaphocephaly using a combination of distraction and contraction techniques and achieved satisfactory results. Radial osteotomies in the frontal and occipital bones flattened these abnormal bossing bones and accelerated the disappearance of bony bumps created by distraction. This technique facilitates the achievement of the desired shape of the skull through fine adjustments of the distraction and contraction devices.
Craniosynostoses↗
Endoscopic approach to coronal craniosynostosis.
Seventy-two patients who presented with nonsyndromic coronal craniosynostosis were treated using endoscopic-assisted techniques. Release of the stenosed suture was accomplished with the creation of a 5-mm osteotomy extending from the anterior fontanelle to the pterion. All patients tolerated the procedure well with no mortalities, morbidities, or need for blood transfusions. Correction of the vertical dystopia, nasal deviation, and orbital resection was accomplished in most cases. This approach offers a viable alternative for the early treatment of coronal craniosynostosis.
Craniosynostoses↗