SEARCH · PubMed Health

Results for “CRANIOSYNOSTOSES”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 127 records · Page 7Linked to original sources

PubMed1997

Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis.

OBJECTIVE: The current study compared the mental and psychomotor development of infants with nonsyndromic sagittal synostosis (SS) with a demographically matched comparison group without congenital defects. Within the SS group, we tested the hypothesis that age of cranial release would be inversely correlated with mental development. DESIGN: The design was prospective and longitudinal. Participants were assessed at 4, 12, and 24 months of age. SETTING: The study was conducted in a craniofacial clinic at an urban children's hospital. PARTICIPANTS: Participants were 19 infants with SS (consecutive craniofacial program referrals) and 19 demographically matched comparison infants recruited from the community. One infant with SS did not attend the 24-month assessment. MAIN OUTCOME MEASURES: Mental and Psychomotor Indices from the Bayley Scales of Infant Development were the primary outcome measures. Subdomains of development were created using Kohen-Raz scoring procedures. All measures were determined a priori. RESULTS: Repeated-measures MANOVAs revealed no statistically significant differences in the developmental trajectories of the two groups. None of the SS group infants received Mental Development Index (MDI) scores in the mentally retarded or borderline range of intellectual functioning (i.e., below 78). An inverse correlation (r = -.30) was found between the age at surgery and Bayley growth curve coefficients; however, this association was not statistically significant (p = .10, one-tailed). CONCLUSIONS: Results are consistent with previous studies of the mental and psychomotor development of infants with nonsyndromic craniosynostoses in relation to normative test data. The relation between surgery age and developmental outcome merits further study in a larger sample with a greater range of surgery ages.

Age Factors↗

PubMed1999

Three-dimensional analysis of craniofacial form in a familial rabbit model of nonsyndromic coronal suture synostosis using Euclidean distance matrix analysis.

OBJECTIVE: Simple craniosynostoses produce predictable morphologies of the cranial vault, with growth deficits in a direction parallel to the synostosed suture and compensatory growth at sutures that are perpendicular to and attached to the synostosed one. In coronal suture synostosis, anteroposterior growth is inhibited, with compensatory growth in a transverse direction. Information on growth patterns and influence on other craniofacial regions are not as clear. This study tested the hypotheses that (1), both juvenile and adult rabbits with familial, nonsyndromic coronal suture synostosis exhibit significant size and shape differences of the entire craniofacial region relative to normal rabbits as a result of altered growth patterns and that (2), shape differences of the calvaria will precede those of the basicranium. DESIGN: Fifty anatomic landmarks were located on 94 New Zealand white rabbit crania. The crania were divided into a juvenile, six-week-old age category (n = 53) and an adult, 18-week-old category (n = 41) in order to assess shape differences at different ages. Each age category was sorted into three groups based on growth at the coronal suture: normal sutural growth, delayed onset synostosis, and complete synostosis. Landmarks were digitized in three-dimensions, and statistical analyses on shape differences were carried out using Euclidean distance matrix analysis (EDMA). RESULTS AND CONCLUSIONS: Results showed that delayed onset synostosis did not produce craniofacial morphology that was different from normal at any age. However, complete synostosis yielded predictable and global craniofacial shape differences at both ages relative to normal skulls, producing an overall shorter, wider cranium with the most markedly compensating regions in a posterosuperior position of the skull. In addition, delayed onset synostosed crania showed no shape differences in the basicranium, relative to normal crania, suggesting primacy of the calvaria in this model of coronal synostosis. However, further investigations are necessary to verify primacy of the calvaria in this model.

Age Factors↗

PubMed2004

Craniofacial reconstruction with a fast resorbing polymer: a 6- to 12-month clinical follow-up review.

OBJECT: Resorbable polymer implants have become a compelling option in the treatment of acquired and congenital craniofacial deformities. In particular, the resorbable polylactide and polyglycolide polymers have demonstrated excellent safety profiles in multiple in vitro, animal, and clinical studies and are currently being used in a wide variety of craniofacial applications. In pediatric craniofacial reconstruction a desirable attribute of fixation is early resorption, which may limit the duration of any effect on cranial growth. In this paper the authors discuss the biomaterial properties of a fast resorbing polymer (FRP) and the clinical results in a series of patients who participated in a 6- to 12-month study. METHODS: The authors performed craniofacial reconstruction by using FRP implants in 29 patients beginning in August 2002. All patients experienced maintenance of stable bone fixation followed by bone healing. Cosmetic results were rated satisfactory or excellent, except for one unsatisfactory cosmetic result caused by disease progression. CONCLUSIONS: Results of this study support the effectiveness of an FRP implant in a variety of craniofacial surgical procedures including craniosynostoses, fibrous dysplasia, cranial defects, and encephaloceles.

Absorbable Implants↗

PubMed1984

Computerized tomography of cranial sutures. Part 2: Abnormalities of sutures and skull deformity in craniosynostosis.

Preoperative computerized tomographic (CT) scans of 24 children who had surgery for either single or multiple craniosynostoses were compared with skull radiographs and operative and pathological findings. In addition to providing accurate imaging of calvarial and skull base deformities secondary to premature suture closure, high-resolution CT with bone definition algorithms supplied valuable detail of anatomical changes at the abnormally developed suture. The CT findings varied with the location of the suture. Thickened bony ridges predominated at the sagittal suture, focal bone thickening and erosions were more likely to be found at the metopic suture, and parasutural sclerosis was the prevalent finding on one side of the lambdoid suture. No evidence of the suture could be detected in the majority of patients with complete coronal craniosynostosis. Radiographs of the skull were shown to be a relatively insensitive means of imaging the zone of limited fusion, especially the lambdoid suture. An excellent correlation was found between the CT scan and the operative and pathological findings. There was histological evidence of progressive suture fusion in virtually all patients. An asymmetrically narrowed lucent zone with parasutural sclerosis or bony ridges seen on CT scans correlated well with fibrous union of the suture found on histological examination. The authors conclude that high-definition CT used in conjunction with bone windows and thin and coronal slices for the evaluation of sagittal sutures is a useful imaging method for the evaluation of craniosynostosis.

Cranial Sutures↗

PubMed2006

Simulation of surgery for craniosynostosis: a training model in a fresh cadaveric sheep cranium. Technical note.

The authors present a training model in sheep crania that allows residents in neurosurgery and plastic surgery to practice the frontoorbital remodeling procedure used in the surgical correction of simple craniosynostoses such as plagiocephaly, trigonocephaly, and brachiocephaly. The model comprises a three-step approach: subperiosteal and subperiorbital dissection; elevation of the bifrontal bone flap and the supraorbital bar; and finally, frontoorbital remodeling. The authors conclude that this training model, based on the use of cadaveric sheep crania, represents a fairly useful method to accustom trainees to the required surgical techniques and simulates well the steps of standard pediatric and adult craniofacial surgery for simple craniosynostosis.

Animals↗

PubMed2001

New Zealand Maori family with the pro250arg fibroblast growth factor receptor 3 mutation associated with craniosynostosis.

BACKGROUND: A large New Zealand Maori family has non-syndromic coronal craniosynostosis, which is inherited as an autosomal dominant mutation with variable expression. The aim of the study is to determine whether the family has the pro250arg mutation in the gene for fibroblast growth factor receptor 3 (FGFR3), a mutation found in patients with various types of craniosynostosis. PATIENTS: Fourteen members of a New Zealand Maori family were evaluated, of whom five have coronal synostosis. A family pedigree tracing six generations was recorded. METHODS: Blood samples were drawn for genomic DNA analysis from 14 family members. Polymerase chain reaction, restriction-enzyme digestion and DNA sequencing was performed to identify the pro250arg mutation in FGFR3. RESULTS: Seven family members were heterozygous for the pro250arg mutation in FGFR3. The mutation showed autosomal dominance with reduced penetrance and variable expressivity. CONCLUSION: Our data and those of other investigators suggest that we should begin integrating molecular diagnosis with phenotypic diagnosis of craniosynostoses.

Arginine↗

PubMedDate not supplied

[Surgery of multi-sutured craniosynostosis in childhood].

According to own critical experience with the treatment of craniosynostoses (1954-81), using various methods from partial morcellement to total ablation of the cranial vault, the author applied (1982-2000) compound surgical decompression in 40 cases of turricephalic craniostenosis due to multisutural craniosynostosis. The surgical approach to the problem was supported by pre- and postoperative observations of computed three-dimensional bone reconstruction, furthermore by investigation of alterations of SPECT detecting the critical areas of most pronounced intracranial tightness of the skull leading to localized and general cerebral blood flow impairment of the developing brain. The application of newer diagnostic methods contributed to deeper understanding of mechanisms concerning neurodevelopmental retardation and behaviour or defect, disturbances as epilepsy, cranial nerve palsy, spastic paresis, hypothalamic-pituitary insufficiency or poor intelectual performance. This made the possible treatment of preoperative clinical symptoms more efficient. By an early operation we solve the disproportion between the too small skull volume limited by premature ossification of sutures which resulted previously in compression of the growing in size, of the maturing brain. In cases of more complex pathology orbitotomies, with a reconstructive advancement of the orbital rim and maxillas should be performed with multidisciplinary cooperation.

Adolescent↗

PubMed2003

Advances in distraction techniques for craniofacial surgery.

Distraction osteogenesis has been applied to the craniofacial skeleton as well as the long bones of the extremities. This technique does not require bone grafting and allows correction of craniofacial deformities with less invasion. Moreover, the distraction procedures can expand the overlying soft tissues simultaneously. We determined the indications of distraction osteogenesis, analyzed the types of devices available, and examined patients treated with distraction for the mandible, midface, and cranium. In all three sites, the devices tended to be the buried type and made of absorbable materials. Administration of some cytokines for shortening the consolidation period may be considered. Among disorders indicated for distraction osteogenesis, there are several syndromic craniosynostoses, which involve mutations in the fibroblast growth factor receptor (FGFR) 2 gene. The FGFR 2 mutation was suggested to clinically accelerate osteogenesis at the distraction site. The usefulness and appropriateness of the distraction protocol must be assessed for each individual disorder. Although distraction osteogenesis in the craniofacial skeleton has advanced technologically, all possible risks must be discussed with the patient and family members when obtaining preoperative informed consent, especially until establishment of fully safe distraction procedures.

Adult↗

PubMed2004

[The correction of fronto-orbital deformity in infant craniosynostosis--a one year experience].

OBJECTIVE: To evaluate the correction of fronto-orbital deformation in infant craniosynostosis and to discuss the timing of treatment and surgical technique. METHODS: Eleven consecutive patients with craniosynostoses underwent bilateral fronto-orbital osteotomies and advancement via coronal approaches. There were two females and 9 males with an age range from 6 to 9 months. Among the patients, six had trigonocephaly secondary to metopic synostosis, two had non-syndromic plagiocephaly secondary to unilateral coronal synostosis, one had turricephaly secondary to multi-sutural synostosis and two patients had brachycephaly due to syndromic synostosis (Apert syndrome and Saethre-Chotzen syndrome). RESULTS: The shape of forehead, bilateral orbit and bilateral temples in all patients markedly improved with 2-11 months follow-up. There were not obvious complications except that the unilateral parietal bossing happened to the child with turricephaly postoperatively. CONCLUSIONS: Satisfactory results show that fronto-orbital advancement is safe and effective way to correct frontal and orbital retrusion secondary to craniosynostosis.

Craniosynostoses↗

PubMed1991

The cranial base in normal and abnormal skull growth.

In the normal growth of the cranial base, an outline of prenatal development stressed the common vertebrate plan, the patterning of mesenchymal tissues formed from and influenced by migrating neural crest cells. Details are given of the sequences of chondrification and ossification. Postnatal growth is approached in two ways--sagittal growth and transverse growth--involving detailed consideration of sutural growth and of resorptive expansion and remodeling in the whole base. New conclusions have been reached about the growth of the ethmoid, which continues for a longer time than was generally thought, and of its relation to orbital growth. The dependence of fossa growth upon the early, almost explosive growth of the brain is shown in the sequences of basicranial growth, with the maturation of the fossae in a rostrocaudal direction. Of all the suture systems affecting the base, the coronal ring is most important (with the spheno-occipital synchondrosis next in importance). An off-shoot of the coronal ring is noted, the pterygoid buttress, and its importance stressed in relation to maxillary and midface growth. These considerations of normal morphology are widened to explore the dysmorphologies of the nonsyndromic and syndromic craniosynostoses. The abnormal patterning of these synostoses is shown to lie partly in the common pathways of expressivity of the calvarial and basicranial suture systems, again particularly the coronal ring, and partly in the development and responsiveness of the bony units themselves, whether normal or hypoplastic, and of their soft-tissue matrices.

Craniosynostoses↗

PubMedDate not supplied

Surgical treatment of craniosynostosis.

The authors report their experience with the cranial recontouring techniques in the treatment of craniosynostoses. The results obtained treating sixteen children suggest that these techniques can be tolerated well by patients, with very limited postoperative complications. It is underlined that the precocity of the surgical treatment is an indispensable prerequisite in order to obtain satisfactory clinical and cosmetic results.

Craniosynostoses↗

PubMed1986

Cranial base changes following surgical treatment of craniosynostosis.

Three-dimensional osseous surface images from CT scans have been used to study the endo- and exocranial bases of 87 patients with a variety of craniosynostoses. Patients were studied prior to cranial surgery in infancy, perioperatively, and 1 year postoperatively. The dysmorphology of the endocranial base is diagnostically specific for synostosis of the metopic, sagittal, unicoronal, and bicoronal sutures. Cranio-orbital surgery in infancy for nonsyndromal solitary and bicoronal synostosis seems to induce normalization of endocranial symmetry in the first postoperative year. This normalization occurs to a lesser degree in patients with multiple synostoses. These findings suggest that the cranial base dysmorphology of craniosynostosis is a secondary manifestation of an undefined primary disorder. Furthermore, persistent postoperative dysmorphology may reflect abnormal neural rather than osseous growth.

Child↗

PubMed1987

Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients.

Treatment options for the craniosynostoses vary from conservative observation until completion of growth to radical remodeling in infancy. To further define the timing and type of treatment necessary in these complex disorders, we have retrospectively analyzed all patients operated on for this deformity during the past 12 years. One-hundred and sixty-four patients with craniosynostosis were analyzed and subgrouped into asymmetrical (predominantly unilateral) and symmetrical (bilateral) deformities, in addition to segregation by age and type of procedure performed. This was done recognizing that no deformity, like no normal human face, is truly symmetrical. Results of treatment were categorized on the basis of the need for additional surgery and varied from no refinements necessary (category I) to major reduplication of the initial procedure (category IV). Analysis of the data led us to conclude that excellent results can be expected in the asymmetrical deformities group treated in infancy by a unilateral approach. Similarly, for the mild symmetrical deformities, treatment at this time by bilateral orbital advancement gives satisfactory results in the majority of patients. By contrast, the more severe symmetrical groups treated in childhood have a high incidence of requiring secondary major reconstructions, and consideration should be given to delaying craniofacial surgery until age 7 or older, although earlier cranial surgery may be advisable.

Age Factors↗

PubMed1987

Treatment of craniosynostosis in infancy.

Craniosynotosis affects approximately one infant out of 1000. The increase of intracranial pressure and the risks of functional problems are more frequent than usually estimated, especially in monosutural synostosis. Frontocranial remodeling will correct both functional and aesthetic consequences of craniosynostosis. The best operative period is the first year of life, 2 to 3 months of age for the brachycephalies, and 6 to 9 months of age for the other craniosynostoses. Not only does growth not adversely affect the results of the forehead remodeling, but the adjacent orbitonasal areas improve with time. In Crouzon's disease and Apert's syndrome, early frontal advancement does not prevent the midface retrusion, and a radical frontofacial advancement may be indicated in very severe cases.

Child↗

PubMed1977

[Carpenter's syndrome].

A newborn boy presented with an acrocephaly characterized by a coronal craniosynostoses, open sagittal sutures and abnormally high and straight forehead. He was the only child of young, unrelated, healthy parents; there was no familial history of dysmorphy. Facial asymmetry was important and associated with posterior cleft palate, syndactylia of the tips and polydactylia of feet, due to a splitting of the first metatarsus. The child also had a congenital heart disease, like in half of the 15 published cases. In older children, mental retardation is usually observed, often associated with obesity and hypogonadism. Polydactylia permitted to exclude Apert's acrocephalosyndactylia in which there is a normal number of finger arms and which seems to be a dominant mutation, while the transmission of Carpenter's syndrome appears autosomal recessive, thus requiring restrictive genetic counselling.

Abnormalities, Multiple↗

PubMed1995

Evaluation of the effect of early mobilization of the supraorbital bar on the frontal sinus and frontal growth.

Consequences of early frontocranial remodeling are controversial. It has been said that secondary operations are more difficult and that the frontal sinus does not develop well, with an adverse effect on forehead aesthetics. Some illustrative cases are presented, among 820 operated craniosynostoses, to demonstrate that (1) an early, well-performed frontal advancement and/or remodeling is followed by satisfactory reossification and permits easy secondary surgery, (2) frontal sinus development, which is always impaired in anterior craniosynostosis, is only moderately diminished by early supraorbital bar remodeling (when the frontal bar is repositioned after remodeling, as with trigonocephaly, the frontal sinus develops in 83 percent of cases, whereas when the advancement is significant, as with brachycephaly, the development of the frontal sinus is observed in only 50 percent of the cases), and (3) forehead aesthetics are not linked closely to the development of the frontal sinus. After a significant advancement, even if the sinus does not develop, the frontal bar projection can remain satisfactory.

Child↗

PubMed1994

Surgical correction of metopic suture synostosis.

Premature fusion of the metopic suture is an uncommon form of craniosynostosis, historically reported with an incidence of less than 10% among the various forms of craniosynostoses. Despite its infrequency, it is the most obvious deformity associated with premature fusion of a single suture with its prominent frontal keel, narrow forehead, and close-set eyes. This article discusses the timing, long-term results, and recent advances of surgical techniques.

Child, Preschool↗

PubMed1994

[Glabellar morphology after frontocranial remodeling for craniosynostosis in infancy].

This retrospective clinical and radiographic study concerns 75 cases of craniosynostoses among 850 cases operated in the Craniofacial Unit of Necker's Hospital for Sick Children (1976-1988, Paris, France). The correlation between frontal sinus development and glabellar morphology was analyzed. When there was a significant advancement of the supraorbital ridge the projection of the glabella was satisfactory without any frontal sinus. When no significant advancement was performed, the pneumatization of the frontal bone was as frequent as in the general population and was dependent upon the underlying frontal sinus.

Adolescent↗