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At least 613 records · Page 34Linked to original sources
PubMed1985

Progressive facial asymmetry as a result of early closure of the left coronal suture.

A longitudinal growth analysis of a patient with premature closure of the left coronal suture is presented. The resultant facial asymmetry of patients with unilateral synostosis of the coronal suture is accompanied by diagnostic facial characteristics and radiographic skull distortions. The midfacial structures distort and grow toward the site of synostosis while the lower face grows away from the site of synostosis. Unilateral craniosynostosis of the coronal suture results in distortions and compensations within the cranial vault and the cranial base. Therefore, with growth of the contiguous facial structures, the facial distortions are magnified.

Brain↗
PubMed1988

Orbital expansion in plagiocephaly.

In moderate to severe plagiocephaly, the transverse diameter of the orbit on the affected side is consistently decreased. In these cases, the supraorbital rim has been expanded by the required amount and bone grafted. This makes for greater orbital symmetry and ease in obtaining the correct supraorbital convexity. Ten patients have been handled in this way with satisfactory postoperative results.

Craniosynostoses↗
PubMed1992

Relationship between intracranial pressure and intracranial volume in craniosynostosis.

Premature fusion of cranial sutures in craniosynostosis has been thought to lead to craniostenosis, which in turn may lead to increased intracranial pressures. In 41 consecutive patients with craniosynostosis, intracranial pressure and intracranial volume were measured. Of the 41 patients, 38 (92.6%) had raised intracranial pressure but only 4 (9.7%) had a decreased skull volume. In the present study, there is no correlation between intracranial volume and intracranial pressure. This study confirms that the measurement of intracranial volume, a non invasive procedure, cannot be used to assess intracranial pressure and to avoid an invasive procedure.

Aging↗
PubMed1994

Fronto-orbital re-operation in craniosynostosis.

The need for fronto-orbital re-advancement is reported from all cases of craniosynostosis operated on in the Birmingham and Oxford Craniofacial Units since 1979. Analysis of the 219 cases demonstrates a significantly higher re-operation rate in cases of syndromal craniofacial dysostosis or unilateral coronal synostosis initially operated on below the age of 6 months. Mild bicoronal, sagittal and metopic synostotic cases, however, did uniformly well with the timing of initial surgery having little influence on the re-operation rates.

Age Factors↗
PubMed1988

Frontonasal malformation as a field defect and in syndromic associations.

Several new "syndromes" have been described that have in common facial findings identical or similar to those seen in frontonasal malformation (FNM), previously termed frontonasal dysplasia. Some of those new syndromes are inherited, whereas FNM is an isolated finding. Thus there is a need for differentiation among those conditions. A review of the literature was undertaken to identify and classify the various reports that describe FNM as it occurs alone and in association with a syndrome.

Classification↗
PubMed1988

Craniofrontonasal dysplasia: phenotypic expression in females and males and genetic considerations.

The phenotype and pattern of inheritance of craniofrontonasal dysplasia were analyzed in 66 affected persons from 18 families, including one four-generation kindred personally studied. Females were more severely affected than males. Affected females had hypertelorism, broad nasal root, frontal bossing, craniosynostosis, syndactyly of toes and fingers, and vertical grooving of nails. Males had increased bony interorbital distances and distances between the inner canthi of the eyes, broad nasal root, broad halluces, and vertical grooving of nails but no craniosynostosis. Affected men transmitted the condition to all of their daughters but to no sons, while affected women transmitted the disorder to about half their daughters and half their sons. This pattern is compatible with X-linked dominant inheritance, but the far milder manifestation of the syndrome in males cannot be explained by simple mendelian genetics.

Abnormalities, Multiple↗
PubMedDate not supplied

Congenital craniofacial anomalies of ophthalmic importance.

Congenital craniofacial abnormalities frequently require ophthalmic evaluation and surgical management. Called upon to perform as part of the craniofacial team managing the often severely deformed craniofacial patient, the ophthalmologist must bring a basic knowledge of craniofacial syndromes and developmental anatomy, as well as clinical acumen to help preserve or improve ocular and adnexal function. As an introduction to this area of ophthalmology, the clinical features, classification, appropriate facial embryology, assessment and surgical considerations of the various congenital craniofacial abnormalities are reviewed. The expanding availability of craniofacial surgeons and surgical teams along with improved surgical results will ultimately require an increasing involvement by many more ophthalmologists in the evaluation and management of these congenital abnormalities.

Craniofacial Dysostosis↗
PubMed1984

Intracranial changes with unilateral coronal synostosis.

Intracranial changes in two patients with unilateral coronal synostosis were evaluated by computed tomography (CT). Preoperative CT scans showed local compression of the brain by the flattened frontal bone with definite shift of the midline structures, suggesting that unilateral coronal synostosis may be harmful to the growing brain in infants. Postoperative restoration of the intracranial changes led me to draw the conclusion that unilateral coronal synostosis should be treated as early as possible not only for cosmetic reasons but also for the prevention of parenchymal damage.

Brain↗
PubMed1984

Intracranial pressure changes in craniostenosis.

Intracranial pressure changes occurring in patients with craniostenosis were studied by continuous monitoring of intracranial pressure in 20 children. Although clinical or radiologic evidence of intracranial hypertension was present in only one patient, 13 (65%) had persistent or episodic raised intracranial pressure. Intracranial hypertension occurred with involvement of both single and multiple sutures and in four of six patients with hydrocephalus and craniostenosis. Recordings after surgical treatment in nine patients revealed that several months elapsed before intracranial pressure improved significantly or returned to normal, despite apparently adequate bony decompression. This study confirms that continuous monitoring of intracranial pressure adds significantly to clinical assessment in determining the need for, and the efficacy of, surgical treatment in craniostenosis.

Adolescent↗
PubMed1986

Management of trigonocephaly.

Trigonocephaly, resulting from premature fusion of the metopic suture, is one of the least common forms of craniosynostosis referred to our institution. We describe a method of treating this condition that has been satisfactory in our hands. A bicoronal scalp flap is reflected down to the glabella and supraorbital ridges and a "gull-wing" bone flap is then formed in the frontal region, hinged forward on the metopic ridge. The midline strut in the area of the fused metopic suture is then burred down using a high-speed drill, but is not removed. We feel that this method results in an excellent immediate cosmetic result and is technically easier then the morselization procedures which have previously been described. We have found this to give a satisfactory result in 13 patients who underwent this procedure.

Congenital Abnormalities↗
PubMed1991

Midline craniectomy for sagittal suture synostosis: comparative efficacy of two barriers to calvarial reclosure.

This report quantitatively compares long-term head remolding achieved by two methods for retarding bone reunion after midline craniectomy for sagittal suture synostosis. In one group of six children the adjacent bone edges were overlayed with 1.0-cm-wide U-channel silicone strips, and in the other group, composed of seven patients, a considerably larger extent of the adjacent cranium was covered with silicone sheets. Mean percent change in the cephalic indices of the former group 18 months postoperatively was 9.1% +/- S.D. = 6.7, and of the latter group 23.7% +/- S.D. = 6.0 (p congruent to 0.01). These results indicate that the use of large silicone sheets lead to a significantly greater normalization of cranial proportions.

Craniosynostoses↗
PubMed1991

Hydrocephalus: overdrainage by ventricular shunts. A review and recommendations.

Selected literature review of the clinical course of patients with ventricular shunts for hydrocephalus shows that the effects of cerebrospinal fluid overdrainage are subdural hematoma, craniosynostosis, slit ventricle syndrome, and low intracranial pressure syndrome. These occur sequentially at different age groups, but approximate averages of incidence and time of occurrence after first shunt reveal an overall incidence of 10%-12% for at least one of these appearing at 6.5 years after shunting. The basic etiology, diagnosis, and variety of treatment modalities available are reviewed, including the need for shunt closing intracranial pressure control. Included is a hydrocephalus program designed to minimize the need for long-term extracranial shunts and to maximize therapeutic intracranial procedures for hydrocephalus.

Cerebral Ventricles↗
PubMed1992

A plea for early operation for craniosynostosis.

In times when much is written of complex craniofacial operations for synostosis, it is important to reassess the efficacy of simpler operations on the cranium when carried out in the first few weeks of life. Examples of sagittal, coronal, and lambdoid synostosis are shown, as well as one patient with multiple suture involvement.

Craniosynostoses↗
PubMed1995

Transcranial Doppler analysis of cerebral hemodynamics in primary craniosynostosis: study in progress.

BACKGROUND: Regional elevations in intracranial pressure (ICP) have been monitored adjacent to areas of cranial stenosis by some authors [9,19,20]; however, noninvasive techniques such as transcranial Doppler (TCD) would be preferable if increased ICP could be detected just as reliably. METHODS: TCD examinations of basal cerebral arteries were performed in 31 children with skull deformities. Skull X rays and computed tomography (CT) scans or magnetic resonance imaging (MRI) were analyzed for degree of cranial vault abnormality, as well as for presence or absence of intracranial pathology. Neurodevelopmental examination and clinical signs of increased ICP, if any, were recorded. RESULTS: The overall incidence of increased resistivity indices (RI) was 70%. Patients with asymmetric skull deformities (plagiocephaly) or sagittal synostosis (scaphocephaly) were statistically more prone to exhibit increased RI compared to previously reported prevalences. CONCLUSIONS: Because TCD is a convenient, rapid, accurate, and noninvasive method by which to gauge ICP, it should be considered in the preliminary work-up of a patient with craniosynostosis.

Craniosynostoses↗
PubMed1993

A mutation in the homeodomain of the human MSX2 gene in a family affected with autosomal dominant craniosynostosis.

Craniosynostosis, the premature fusion of calvarial sutures, is a common developmental anomaly that causes abnormal skull shape. The locus for one autosomal dominant form of craniosynostosis has been mapped to chromosome 5qter. The human MSX2 gene localizes to chromosome 5, and a polymorphic marker in the MSX2 intron segregates in a kindred with the disorder with no recombination. Moreover, a histidine substitutes for a highly conserved proline at position 7 of the MSX2 homeodomain exclusively in affected members. In the mouse, transcripts of the Msx2 gene are localized to calvarial sutures. These results provide compelling evidence that the mutation causes this craniosynostosis syndrome.

Acrocephalosyndactylia↗