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Dental, orthodontic, and oral/maxillofacial evaluation and treatment in Apert syndrome.

Although the cause of the midfacial anomalies in Apert syndrome is still elusive, a great experience has accrued in the management of these physically and psychologically handicapping deformities. An interdisciplinary approach that includes input from an orthodontist, pediatric dentist, and oral and maxillofacial surgeon should be an integral part of the management of affected patients. This theme of surgical-orthodontic-dental communication and interaction is stressed and illustrated throughout the article.

Acrocephalosyndactylia↗

[Hanhart syndrome (aglossia-adactylia syndrome). Report of 2 cases].

We discuss two cases of Hanhart syndrome, diagnosed at 3 days and 4 months respectively. Minimal diagnostic criteria (micrognathia and peromelia) are reported, and various etiologic hypothesis are discussed. This syndrome includes others as: aglossia-adactylia, hypoglossia-hypodactylia, oro-acral, oro-mandibular-limb-hipogenesis, ankyloglosia superior, glossopalatine ankylosis, peromelia and micrognathia. Emphasize the impossibility of prevention; the patients may die because of food aspiration, both parents an children being subsidiary of psychological support. The possibilities of logopedical treatment and prothesis of peromelic limb must be evaluated.

Abnormalities, Multiple↗

Four-step mandibular lengthening to correct a bird face deformity. A case report.

A technique for extreme lengthening of the mandible is presented. In one operation it combines several procedures already known, modifying some of them. Its advantage is that the movement is subdivided in 4 steps. Thereby the need for grafting is reduced or even absent and the amount of relapse is probably less important than if fewer but bigger movements were applied.

Adolescent↗

[Goldenhar syndrome: apropos of 2 cases].

The arc branch syndrome are a group of diseases whose classification and etiology are unknown yet. We has 2 patients with Goldenhar's syndrome and we have studied the etiopathogenicity and treatment in these diseases.

Adult↗

Craniofacial osseous landmark determination from stereo computer tomography reconstructions.

The accurate and reproducible determination of the three dimensional (3D) co-ordinate positions of anatomical landmarks from computer tomography (CT) images has been limited even though potentially the data have been available for several years. This paper describes a method of acquisition of osseous landmark positions using an off-line computing technique based on multiple stereo images of 3D CT reconstructions. The use of stereo pairs greatly enhances the consistent identification of osseous landmarks. Further, the technique is of particular value where access to the CT scanner is restricted due to heavy clinical demand and separate high quality graphics facilities are unavailable. Osseous landmark position data were determined for dried skulls and patients with craniofacial conditions. Accuracies of the order 1.7mm (median) were obtained. These results encourage the use of the technique for acquisition of landmark positions for the study of the craniofacial complex in three dimensions.

Acrocephalosyndactylia↗

[A successful surgical repair of anomalous right pulmonary venous connection with Goldenhar syndrome].

A ten-year-old girl with Goldenhar syndrome underwent intracardial repair for partial anomalous right pulmonary venous connection into the superior vena cava. In surgical procedure the proximal tract of superior vena cava was reconstructed into double floor, low floor was tract for anomalous pulmonary venous blood drained into left atrium through the atrial septal defect which was made with resection of prinum septum, upper floor was a real tract for superior venous blood. After operation, arrhythmia did not detect. One month after operation, no stenosis along the superior vena cava or right pulmonary vein were evidenced in catheterization. This surgical technique is superior to using baffle for preventing thrombogenesis. The result suggested that this surgical technique is preferable to the reconstruction of baffle in superior vena cava.

Child↗

Tessier clefts revisited with a third dimension.

The classification by Tessier of rare craniofacial clefts brought, for the surgeon, order to a previously confusing array of anatomic and developmental descriptions. An ordered two-dimensional categorization of severe clefting malformations evolved from his clinical, radiologic, and surgical observations. The purpose of this paper is to report a complete series of facial clefts studied with computed tomography (CT) and three-dimensional reconstruction. The CT analysis supports some, but contradicts other, hypotheses and speculations presented by Tessier. The CT data reveal the scale of the reconstructive challenge and allow the assessment of our therapeutic interventions.

Adolescent↗

[Facial malformations and asymmetries due to first and second branchial arch syndromes].

The first and second branchial arch syndrome is a congenital affection, among whom one can range as well hereditary as non-hereditary entities. They always cause a badly mutilating facial asymmetry. In this article, the first and second branchial arch syndrome were divided in hemifacial and bilateral dysostoses. We pointed out the hemifacial microsomia, the most occurring entity in these series. From two case reports, we attempt to give a treatment synopsis of this syndrome, which is always done by a multi-disciplinary team, including dentists, orthodontists, maxillofacial surgeons and plastic surgeons.

Adolescent↗

[Goldenhar syndrome. Report of 5 cases in association with cardiac malformations].

The paper presents five cases of the Syndrome described by Goldenhar, which consists in oculo-auriculo-vertebral alterations. Classic descriptions relate the presence of epibulbar dermoid, pre-auricular appendixes, mandible hypoplasia and vertebral anomalies. Excepting by some vertebral alterations, all the other anomalies were common to the five cases. This syndrome may be accompanied by congenital cardiopathy in a percentage varying from 15 to 50%, depending on the publications. The reason of hospitalization was the real presence of congenital cardiopathy of important clinical repercussion. Three patients had Tetrad of Fallot, one transposition of the great arteries, and the last one total annomalous pulmonary venous connection, which died due to clinical complications. The four patients were submitted to surgical correction of their cardiac defects, being the face defects programmed to the corrected later on.

Child, Preschool↗

[Treacher-Collins syndrome].

Because of two clinical cases, the authors, present a review of the literature. The wide variance in expression that allows for different clinical forms of the condition has led to erroneous interpretations of some cases, which have been described as separate entities. Franceschetti and Klein have emphased this phenotypic variability.

Child↗

Orbital protrusion index in Treacher-Collins syndrome: a tool for determining the degree of soft-tissue damage.

The relationship of measurements of the intercanthal width (en-en), the biocular width (ex-ex) and both eye fissures (ex-en), constituting the orbital protrusion index ex-ex x 100 (en-en) + (en-ex, r) + (en-ex, l) was used to analyze preoperative morphological developments in the orbits of 23 patients with Treacher Collins syndrome. The index permitted distinction between mildly and severely defective orbits, based on the degree of defective measurements and variations in relative sagittal positions. Optimal index values (mean, 94.0) were seen in 11 subjects with a slightly different sagittal level between the inner and outer commissures of the eye fissures, which created slightly protruding but still normal soft-tissue relief. The eye fissures in seven patients were moderately reduced in length. Orbits with higher indices (mean 98.1) (12 subjects) showed less differences in level between the two commissures of the eye fissures, producing a flattening in the surface relief. Eye fissure length was markedly subnormal in all patients.

Adolescent↗