Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “ACROCEPHALOSYNDACTYLIA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

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

At least 793 records · Page 44Linked to original sources
PubMed1988

[Apert's syndrome].

Explore the source record for details and available documents.

Acrocephalosyndactylia↗
PubMed1985

[Human genetic aspects of inborn ear, nose, and throat diseases].

The genetic analysis of malformations enables us to distinguish four groups of different anomalies: 1. Chromosomal disorders (gene and chromosome mutations). 2. Single (monogenic) hereditary anomalies and malformations (gene mutations: point-mutations). 3. Malformations with polygenic multifactorial basis (combination of genetic and exogenous factors). 4. Exogenous non-hereditary malformations. We were able to classify malformations in one of the four groups using the three most important methods of human genetic investigations: (a) chromosomal analysis, (b) family investigations, (c) twin method. These methods can complement each other, but each has its own individual place. This review quotes examples of the four different etiological groups in otology, especially those affecting parts of the ear as well as those affecting whole ear.

Abnormalities, Drug-Induced↗
PubMed1988

Rhinoplastic implications of maxillary osteotomy.

Maxillary osteotomies are done to correct discrepancies in structural facial harmony. The deformities involve retrusion or protrusion, as well as pathological shortening and elongation of the maxilla. Surgical orthopedics of the maxilla move the nose in part, or in whole, in relationship to the rest of the face. Corrective rhinoplasty is sometimes necessary to correct esthetic discrepancies thus produced, as well as other deformities that may be the usual concomitant of the syndrome addressed by the facial surgery. Le Fort I osteotomies produce more marked changes than pyramidal or Le Fort III type osteotomies, specifically as the tip relates to the dorsum. Segmental osteotomies done to correct discrepancies in vertical height of the maxilla produce specific alterations in the tip-lip relationship.

Acrocephalosyndactylia↗
PubMed1988

[Apert syndrome].

Explore the source record for details and available documents.

Acrocephalosyndactylia↗
PubMed1986

Skull morphology after early craniotomy in patients with premature synostosis of the coronal suture.

The degree of normalization of the shape of the skull was studied in 12 craniosynostosis patients who had been treated with different types of craniotomies in infancy. Three diagnoses were represented: isolated bicoronal synostosis; Crouzon's syndrome; and Apert's syndrome; there were four subjects in each group. Selected cranial dimensions were measured on roentgencephalograms taken in infancy and at follow-up examinations at 2 to 3 years of age. The measurements were compared with control values obtained from patients with cleft lip. Cranial dimensions in the synostosis patients seldom corresponded well with the values of the controls, either before or after surgery. Cranial height, in particular, was increased, especially in patients with Crouzon's or Apert's syndrome. This resulted in modulus values above the control mean for 11 of the 12 patients at the follow-up examinations.

Acrocephalosyndactylia↗
PubMed1986

Stability of Le Fort III advancement surgery in children with Crouzon's, Apert's, and Pfeiffer's syndromes.

The records of 19 children who had undergone Le Fort III advancement surgery were used to assess the stability of the midfacial segment following surgery. Cephalometric radiographs of each individual were analyzed longitudinally. The average forward movement of the maxilla at the time of surgery was 12.4 mm. The average amount of relapse for the group was 9.4 percent in the horizontal plane of space and 5.5 percent in the vertical plane of space. The data obtained from this sample indicate that the Le Fort III advancement is a relatively stable procedure. The amount of relapse or stability that occurs following Le Fort III advancement is related to rigid stabilization of the midfacial segment with buttressing bone grafts, transosteotomy wiring, and intermaxillary fixation or bone plating during the postoperative healing phase.

Acrocephalosyndactylia↗
PubMed1986

Midface position after Le Fort III advancement: a long-term follow-up study.

In 1983, we evaluated the stability of midface position after Le Fort III advancement for midface hypoplasia secondary to Crouzon's or Apert's syndromes. The patients were operated upon from 1972 to 1980, and mean follow-up was 5.8 years (Kaban et al, 1984). In this study, we present an additional 3 1/2 year follow-up of these patients to look at what happens to midface position after Le Fort III osteotomy in growing and nongrowing patients.

Acrocephalosyndactylia↗
PubMed1986

Pre- and postsurgical facial growth in patients with Crouzon's and Apert's syndromes.

Our report deals with 8 patients with Crouzon's and Apert's syndromes followed longitudinally with roentgencephalometric examinations during the growth period. The purpose of the study was to analyze: presurgical facial growth, the displacement of the maxillary complex in connection with Le Fort III advancement, the stability of the surgical result, and postsurgical facial growth. Presurgical facial growth was characterized by lack of maxillary sutural growth and abnormal remodeling of the maxilla. The surgical displacement of the maxilla consisted of an average advancement of about 10 mm and a backward rotation. The maxilla remained stable following surgery. However, onlay bone grafts tended to resorb over the long term. Postsurgical facial growth revealed lack of maxillary displacement with development of exophthalmos, relative mandibular prognathism, and malocclusion. However, it was concluded that the positive effects of early craniofacial surgery in patients with severe forms of Crouzon's and Apert's syndromes outweigh these disadvantages.

Acrocephalosyndactylia↗
PubMed1986

Stability of the maxilla after Le Fort III advancement in craniosynostosis syndromes.

The purpose of this study was to assess long-term position of the maxilla after surgical Le Fort III advancement of midfacial structures in patients with craniosynostosis syndromes. Data from preoperative, short and long-term postoperative lateral head films on 21 consecutive patients with Crouzon's syndrome, Pfeiffer's syndrome, or Apert's syndrome were analyzed. A method of evaluating the position of the postoperative maxilla on lateral head films is described. On average, the position of the maxilla did not change significantly in the postoperative periods studied, although some individual variation was demonstrated. Postoperative management, utilizing a registration bite splint after the intermaxillary fixation has been removed, similar to our treatment following bone grafting procedures in patients with hemifacial microsomia, is described.

Acrocephalosyndactylia↗
PubMed1987

Cervical spine anomalies in the craniosynostosis syndromes.

Cervical spine radiographs of 34 patients with a variety of craniosynostosis syndromes were reviewed to determine the incidence and nature of skeletal anomalies and to evaluate the findings in terms of syndromal patterns. Intervertebral fusions occurred in 30 percent of Pfeiffer's, 38 percent of Crouzon's, and 71 percent of Apert's syndrome children. In Crouzon's and Pfeiffer's syndromes, isolated upper cervical fusions were most common; in Apert's syndrome, the fusions were more likely to be complex involving C5-C6. This syndromal variation, however, was not specific enough for diagnostic differentiation.

Abnormalities, Multiple↗