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At least 37 records · Page 2Linked to original sources
PubMed1996

Birmingham Craniofacial Score: a scoring system for craniofacial deformities.

A system was devised to score facial and head appearance in craniofacial patients in an attempt to quantify the severity of craniofacial deformities and measure the effect of corrective operations. It was deliberately kept simple to be "user friendly," and it must be regarded as incomplete. It has all the limitations of other scoring systems, but nevertheless it consistently reflects the results of operations, allowing quantitative comparisons and statistical analysis. It should be emphasized that deformity, not aesthetics, is measured. Fifteen features are scored. For every normal feature 1 point is awarded; for every abnormal feature, no point is scored. The range, therefore, extends from 0 to 15. In practice, there seems to be good correlation between the total score and the degree of deformity. A score of 13 or greater indicates that the appearance is cosmetically very good and the patient could be regarded as normal; a score ranging from 9 to 12 indicates a definite deformity; and a score of 8 or lower indicates the deformity is severe.

Cephalometry↗
PubMed1996

Craniofacial dysostosis: airway obstruction and craniofacial surgery.

Craniofacial surgery in craniofacial dysostosis on airway obstruction was studied retrospectively in a consecutive series of patients. The records of 76 patients were reviewed, 27 with Apert syndrome, 47 with Crouzon's syndrome, and two with Pfieffer's syndrome. Of 172 operations, 148 were done for cosmetic reasons, hydrocephalus, or papillary oedema, and 24 were done for airway distress. Forty patients (23%) were children less than 13 years of age, and 22 underwent midface advancement procedures. Only 13 of these operations had been done for airway distress. Two were cured by operation and seven improved. After operation had failed to relieve the airway obstruction, a nasal continuous positive airway pressure device (nCPAP) was fitted to seven patients. The nCPAP relieved or improved airway obstruction recorded by a sleep study. As midface advancement in childhood rarely results in lasting improvement in breathing or aesthetics, it may well be advisable to postpone operation until the early teens. We conclude that with nCPAP operation can in most cases be deferred until a time when a permanent result can be achieved.

Airway Obstruction↗
PubMed1996

Neural crest apoptosis and the establishment of craniofacial pattern: an honorable death.

During development of the vertebrate head neural crest cells emigrate from the hindbrain and populate the branchial arches, giving rise to distinct skeletal elements and muscle connective tissues in each arch. The production of neural crest from the hindbrain is discontinuous and crest cells destined for different arches, carrying different positional cues, are separated by regions of apoptosis centered on rhombomeres (r) 3 and r5. This cell death program is under the interactive control of the neighboring hindbrain segments. Both r3 and r5 produce large numbers of crest cells when freed from their flanking rhombomere, but when conjoined with their neighbor the cell death program is restored. Two key components of this program are Bmp 4 and msx-2, both of which are expressed in the apoptotic foci of r3 and r5 and which are also regulated by neighbor interactions. Importantly, the addition of recombinant Bmp 4 to isolated cultures of r3 and r5 induces the expression of Bmp 4 and msx-2 and restores the cell death program. This early neural crest segregation is maintained during development and it has profound effects upon the final craniofacial pattern. Even though crest cells from different axial origins will contribute to compound skeletal elements, these distinct populations do not intermingle. Furthermore head muscle connective tissues are exclusively anchored to skeletal domains arising from neural crest from the same axial level. Thus the discontinuous production of neural crest sculpts the crest into nonmixing streams and consequently ensures the fidelity of patterning.

Animals↗
PubMed1977

Applications of reconstructive craniofacial techniques to acute craniofacial trauma.

Increasing familiarity with elective reconstructive approaches to craniofacial deformity has led to the application of a similar multispecialty approach to repair of selected cases of massive frontal and upper facial trauma. The fundamental steps in craniofacial reconstruction are outlined. The process of patient selection is emphasized. Illustrative case histories review the management of this type of injury. Contributions from anesthesiology, neurosurgery, ophthalmology, and plastic surgery are required. Specialized facilities for postoperative care are mandatory.

Adult↗
PubMed1983

Cerebro-craniofacial and craniofacial malformations: an embryological analysis.

A macro- and/or microscopical study on the normal and abnormal development of the forebrain with the eyes, nose, and cranium, was performed in 139 mouse embryos, 120 normal and 19 abnormal human embryos and fetuses, and in about 2,300 human skulls. The results suggest that from the embryological point of view, a distinction should be made between facial defects involving the brain and/or the neural elements of the eyes, i.e., the cerebro-craniofacial dysplasias, and malformations of the face and cranium only, called the craniofacial dysplasias. Both groups can be subdivided into early or primary defects (in embryos less than or equal to 17 mm C-RL) and late or secondary defects (in embryos greater than or equal to 17 mm C-RL). Almost all of the primary defects can be considered to originate from disorders occurring during the transformation of the brain and face. The secondary defects concern defective differentiation of neurectoderm and of the mesenchyme into bone centers, cartilage, and muscles. All of the defects in question can be explained by insufficient cell proliferation, degeneration, and/or differentiation. New terminology is proposed.

Animals↗
PubMed1995

Craniofacial osteotomies and rigid fixation in the correction of post-traumatic craniofacial deformities.

Post-traumatic craniofacial deformities may result from failure to diagnose craniomaxillofacial injuries or from less than adequate repair. The skeletal deformities may be corrected by procedures employing extended craniofacial exposure, segmental osteotomies and bony repositioning. A graduated approach to the correction of regional bony post-traumatic deformities has been formulated. Bony depressions are corrected by onlay grafts, rigidly fixed with lag screws. Malposition of anatomically normal bone is corrected by osteotomy and repositioning. Anatomically abnormal bone is replaced with bone grafts. The use of rigid fixation techniques prevents the late skeletal relapse seen with previous techniques. In the established post-traumatic deformity, soft tissue distortion from contracted underlying scar tissue and adherence to bony depressions and defects is the limiting factor in restoring the pre-injury appearance.

Adolescent↗
PubMed2004

Global strategies to reduce the health care burden of craniofacial anomalies: report of WHO meetings on international collaborative research on craniofacial anomalies.

Although several significant research projects have arisen from international cooperation, especially in the field of genetics, these have been the exception rather than the rule. However, those of us who had the privilege and delight of participating in the World Health Organization meetings were struck by a common realization of the vast potential of systematic international cooperation. It is clear that the global model will be the most effective approach for tackling the big questions in craniofacial anomalies, be they concerned with cause, treatment, or prevention. This report will serve as a road map for making collaboration the rule and not the exception and hopefully be a stimulus for the creation of partnerships between international research teams and funding agencies.

Craniofacial Abnormalities↗
PubMed1987

Radical treatment of craniofacial anomalies in infancy and the use of miniplates in craniofacial surgery.

The radical treatment of craniofacial anomalies in infancy is indicated for medical, technical, and psychosocial reasons. The major problems around the cranium, the orbits, and the face may be safely corrected by osteotomy, suture resection and disjunction, mobilization, repositioning, and reshaping of the various skeletal segments. This radical approach has been possible through the introduction of miniplate fixation. The plates and screws are removed after 3 to 6 months to take optimal advantage of the great formative power of the growing brain, which expands rapidly within the first 2 years of life. In infants, disjunction is more important than advancement, making this approach a dynamic one in contrast with the static procedures for the adolescents and adults. The complete, one-stage procedures are superior to a delayed or two-stage procedure. Experienced administration of pediatric anaesthesia and intensive care are mandatory.

Acrocephalosyndactylia↗
PubMed2001

Skeletal analysis of craniofacial deformities in brachycephaly: comparison with craniofacial deformities in plagiocephaly.

The deformities of craniofacial bones in brachycephaly (n = 2) and plagiocephaly (n = 2) were compared using three-dimensional skull replicas. In brachycephaly the bilateral deformities of the cranial base were similar to the unilateral deformities on the affected side in plagiocephaly. Shortening of the anterior-middle cranial fossas and expansion of the middle cranial fossas suggest downward and anterior displacement of both temporomandibular (TM) joints resulting in the underdevelopment of the middle-inferior facial bones, though this is less conspicuous in brachycephaly for three reasons. The most important one is that the patients have an imposing facial morphology characterised by high, protruding facial bones, as found on the affected side in plagiocephaly. Secondly, the frontal bones are flattened and positioned posteriorly in the anteroposterior direction, which also helps to mask the underdevelopment of the middle-inferior facial bones. Finally, as bony deformities are symmetrical in brachycephaly, they are not as obvious as they would be if they were unilateral.

Child↗
PubMed1994

Vitamin K--its essential role in craniofacial development. A review of the literature regarding vitamin K and craniofacial development.

The normal vitamin K status of the human embryo appears to be close to deficiency. Maternal dietary deficiency or use of a number of therapeutic drugs during pregnancy, may result in frank vitamin K deficiency in the embryo. First trimester deficiency results in maxillonasal hypoplasia in the neonate with subsequent facial and orthodontic implications. A rat model of the vitamin K deficiency embryopathy shows that the facial dysmorphology is preceded by uncontrolled calcification in the normally uncalcified nasal septal cartilage, and decreased longitudinal growth of the cartilage, resulting in maxillonasal hypoplasia. The developing septal cartilage is normally rich in the vitamin K-dependent protein matrix gla protein (MGP). It is proposed that functional MGP is necessary to maintain growing cartilage in a non-calcified state. Developing teeth contain both MGP and a second vitamin K-dependent protein, bone gla protein (BGP). It has been postulated that these proteins have a functional role in tooth mineralization. As yet this function has not been established and abnormalities in tooth formation have not been observed under conditions where BGP and MGP should be formed in a non-functional form.

1-Carboxyglutamic Acid↗
PubMed2000

[A Study On Dental and Craniofacial Structure of Normal Shanghai Adults Occlusion-III The Analysis of Dental Capacity and Craniofacial Structure]

OBJECTIVE:To examine the relationships between dental capacity and cranioficial structures.METHODS:Models, posterioanterior and lateral cephalometric films of 90 adults with normal occlusion in Shanghai area were measured and analyzed.RESULTS:The canine width of lower arch extended an important influence on the upper and lower arch. The dental capacity was highly correlated not only with the width and depth of cranioficial structure but with the height as well. The database of Coben analysis for normal Shanghai adults occlusion was established.CONCLUSION:The growth between dental and cranioficial structure was harmonized in three dimensions.

Journal Article↗