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At least 307 records · Page 17Linked to original sources
PubMed1996

A comparison of three-dimensional computed tomography scans and stereolithographic models for evaluation of craniofacial anomalies.

PURPOSE: This article describes the use of stereolithographic (SL) models as an adjunct to treatment planning in patients with Apert's syndrome, scaphocephaly, brachycephaly, and turricephaly. PATIENTS AND METHODS: Four syndrome patients had computed tomography (CT) scans done presurgically, and one of these patients was additionally scanned postoperatively. SL models and three-dimensional (3D) CT image reconstructions were produced from the CT data, and linear measurements were compared between the two modalities. RESULTS: All cases showed mean differences between measurements with 3D-CT and SL models of -0.3 to 0.8 mm, except on the CT scans with a gantry tilt (-1.7 mm) and the brachycephalic patient in whom larger deviations were demonstrated (-9.5 mm). CONCLUSION: The results of the study indicate that the accuracy of the SL technique is not always sufficient, necessitating additional validation studies before it can be recommended for routine clinical use.

Acrocephalosyndactylia↗
PubMed1996

Distraction osteogenesis in maxillofacial surgery using internal devices: review of five cases.

PURPOSE: The purpose of this report is to show the feasibility and potential advantages of using internal devices for distraction osteogenesis in the management of maxillofacial skeletal deficiencies. PATIENTS AND METHODS: Distraction osteogenesis was used to correct a variety of maxillofacial skeletal deformities in five patients. One patient underwent bilateral Le Fort III advancement aided by distraction, three patients underwent mandibular ramus lengthening, and one patient underwent segmental alveolar reconstruction by distraction. The devices were activated by either a transcutaneous or transmucosal pin. After achievement of the desired skeletal transport, the activating pins were disengaged and removed from the distraction device. This allowed the distraction device to remain submerged and to stabilize the site of the consolidating bone. RESULTS: All patients achieved lengthening of their jaws. However, premature consolidation was noted in two patients, and one patient had significant relapse. CONCLUSIONS: Development of internal distraction devices is important to address the limitations of currently available biphasic systems. Potential benefits of internal devices include 1) elimination of skin scarring caused by translation of transcutaneous fixation pins, 2) improved patient compliance during the fixation or consolidation phase because there is no external component, and 3) improved stability of the attachment of the device to the bone.

Acrocephalosyndactylia↗
PubMed1980

Temporal double inversion method in reshaping the temporal bulging in a case of Apert's syndrome.

A case is reported of Apert's syndrome with severe bilateral balloonings of the temporal region in addition to other typical craniofacial deformities. He had had three previous strip craniectomies. His mentality and intelligence were normal. In order to improve his deformities, the following steps were carried out: 1. 1.5 cm frontal advancement with reshaping the horizontal arc of the supraorbital ridge by out-fracturing. 2. Bilateral temporal double turnover method with internal decompression of the temporal lobe by resection of the sphenoid wing abnormally roofing the temporal lobe. 3. Reduction of the height of the cranial vault. The result was gratifying.

Acrocephalosyndactylia↗
PubMed1984

Oculo-motor disorders in craniofacial malformations.

Based on 77 observations including telorbitism, craniofacial stenosis (C.F.S.), and plagiocephalies, oculo-motor disorders involving horizontal and vertical imbalance occur very frequently. Abnormalities in the transverse plane (telorbitisms, craniofacial stenoses) give orbital divergence and exotropia. Orbital extorsion with a significant antimongoloid palpebral fissure, may be the cause of rectus muscle extorsion. Abnormalities in the vertical plane (plagiocephalies) induce hypertropia. Abnormalities in the sagittal plane (C.F.S.) lead to exotropia with exorbitism, the V syndrome with double up-shoot. These oculo-motor disorders seem to be the consequence of these orbital structure abnormalities, sometimes combined with sensory factors.

Acrocephalosyndactylia↗
PubMed1987

Congenital embryonal rhabdomyosarcoma of the hand and Apert's syndrome.

A unique case of congenital embryonal rhabdomyosarcoma of the hand in a patient with Apert's syndrome is presented. Immediately after birth a 1 cm soft, bluish mass of the right thenar eminence was clinically diagnosed as a hemangioma. Several months later when the lesion started rapidly enlarging the nature of the tumor was identified, and it was treated with a multidisciplinary approach that included surgical resection, chemotherapy, and radiation.

Acrocephalosyndactylia↗
PubMed1997

Two-stage reconstruction of apert acrosyndactyly.

This report retrospectively reviews presenting radiographs and surgical treatment of 28 hands in 14 children with Apert acrosyndactyly with the purpose of developing a classification system to describe the decision-making process used to determine the type and staging of hand reconstruction. The average patient age at last follow-up evaluation was 7 years (range, 3-17 years). Type I deformities (7 hands) had little or no angular deformity at the metacarpophalangeal (MP) joint; two-stage reconstruction created a four-fingered hand. Type IIA deformities (11 hands) had mild MP joint angular deformity and a more proximal complex syndactyly of the middle three digits; two-stage reconstruction created a three-fingered hand with ray resection of the third digit. Type IIB deformities (7 hands) had pronation of digit 2 superimposed on the thumb and radial angulation at the MP joint of digit 2; two-stage reconstruction created a three-fingered hand with ray resection of the second digit. Type IIC deformities (3 hands) had supination of digit 4 superimposed on digit 5 with ulnar angulation at the MP joint of digits 4 and 5; two-stage reconstruction created a three-fingered hand with ray resection of the fourth digit. This report presents a classification system and four different treatment strategies based on presenting radiographs.

Acrocephalosyndactylia↗
PubMed1983

[Syndactyly: an angiographic study].

Systematic angiographic examination (87 angiographies) done in all the congenital malformations of the hand and in particular in cases of syndactyly in patients 6-11 years of age, has given the authors the opportunity to observe anomalies of the vascular topography of the hand (distal displacement of the bifurcation of the common digital arteries, anomalies of excess or lack of arteries of the palm: common digital arteries, proper digital arteries, etc.) and of performing surgery carefully according to the angiographic appearance of the malformation.

Acrocephalosyndactylia↗
PubMed1984

[Digital videoangiography of the hand].

We have been using digital subtraction angiography for a year. The advantages as compared with customary angiography are pointed out. Some examples are used to illustrate its use.

Acrocephalosyndactylia↗
PubMed1999

Syndactyly in Apert syndrome. Utility of a prognostic classification.

The authors report a series of 36 patients of Apert's Syndrome in whom a genetic analysis was performed. 2 major types of mutation were found (S252W and P253R). The correlation between the type of mutation and certain clinical abnormalities allowed validation of Upton's modification of Blauth's classification and also helped add a prognostic indicator for the intellectual development of the child. Thus, global treatment of the child should take into account not just the clinical abnormalities but also its intellectual prospects.

Acrocephalosyndactylia↗
PubMedDate not supplied

Rapid protyping technology in medicine--basics and applications.

Using medical models built with Rapid Prototyping (RP) technologies represents a new approach for surgical planning and simulation. These techniques allow one to reproduce anatomical objects as 3D physical models, which give the surgeon a realistic impression of complex structures before a surgical intervention. The shift from the visual to the visual-tactile representation of anatomical objects introduces a new kind of interaction called 'touch to comprehend'. As can be seen, from the presented case studies of maxillo-cranio-facial surgery, the RP models are very well suited for use in the diagnosis and the precise preoperative simulation of skeleton modifying interventions.

Acrocephalosyndactylia↗
PubMed1995

Hypertelorism: interorbital growth, measurements, and pathogenetic considerations.

Normal pre- and post-natal changes in the interorbital distance are described. Causes of illusory hypertelorism include flat nasal bridge, epicanthic folds, exotropia, widely-spaced eyebrows, narrow palpebral fissures, and dystopia canthorum. Measurements of hypertelorism may involve soft tissues or bone, and a number of indices have also been proposed. Various types of measurements are evaluated and recommendations suggested. Possible pathogenetic mechanisms for hypertelorism include: early ossification of the lesser wings of the sphenoid; failure in nasal capsule development allowing the primitive brain vesicle to protrude into the space normally occupied by the capsule resulting in morphokinetic arrest in the position of the eyes; and disturbances of the cranial base in Apert syndrome. Associations with increased interorbital distance are also discussed: orofacial clefting, nonprotruding lipomas of the corpus callosum, calcification of the falx cerebri, duplication of the crista galli, wrinkling of the nose, and tissue tags of the nose. Finally, experimental models of hypertelorism in animals are discussed.

Abnormalities, Multiple↗
PubMed1992

New indirect method for estimating the birth prevalence of the Apert syndrome.

An indirect method for estimating the birth prevalence of the Apert syndrome is presented. The fraction of Apert syndrome patients in large clinical surveys of all cases of craniosynostosis is calculated and the fractional component obtained is multiplied by the known birth prevalence of craniosynostosis in general. Apert syndrome makes up approximately 4% of all cases of craniosynostosis. Using a weighted average estimate, birth prevalence was calculated to be 13.7/1,000,000. The results of the indirect method compare favorably with those obtained by the direct method. Nevertheless, because the indirect method is based on a number of assumptions that are easily violated, we cannot recommend its general use except under special conditions.

Acrocephalosyndactylia↗
PubMed1995

Perspectives on craniofacial asymmetry. V. The craniosynostoses.

The craniosynostoses are both etiologically and pathogenetically heterogeneous and many syndromes have been delineated. The present paper highlights well-known craniosynostoses that can present asymmetrically. These include plagiocephaly resulting from premature unilateral synostosis of the coronal or lambdoid sutures. Apert syndrome, and Saethre-Chotzen syndrome.

Acrocephalosyndactylia↗
PubMed1996

A clinical study of the craniofacial features in Apert syndrome.

A clinical study of the craniofacial features in Apert syndrome is based on our experience with 136 cases. Characteristics included hyperacrobrachycephaly, steep wide forehead, flat occiput, common craniofacial asymmetry, ocular hypertelorism and proptosis, downslanting palpebral fissures, divergent upgaze and esotropic downgaze, a tendency towards large ears, and marked depression of the nasal bridge. The nose is short and wide with a bulbous tip, and the anterior facial height is reduced. Common features during infancy included horizontal grooves above the supraorbital ridges that disappear with age, a break in the continuity of the eyebrows, and a trapezoidal-shaped mouth at rest. Radiographic aspects of Apert syndrome were also assessed. Tables are provided which compare the craniofacial features of Apert and Crouzon syndromes.

Acrocephalosyndactylia↗
PubMed1993

Surgical caution with Carpenter's syndrome.

Carpenter's syndrome is a relatively rare craniofacial deformity which will occasionally present to craniofacial surgeons for treatment. Some abnormal anatomical features encountered in two cases which led to unexpected severe intraoperative blood loss are described.

Acrocephalosyndactylia↗