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[Hemifacial microsomia: cephalometric evaluation].

Among the facial deformities one of the most frequent is the hemifacial microsomia. The Authors after a revision of the literature refer about their study. They utilize lateral, antero-posterior cephalometry and orthopantomogram to exactly identify the deformities. The Authors report the results of the analysis and emphasize that the functional alterations of the stomatologic system are of basical importance in the genesis of the syndrome.

Cephalometry↗

Hemifacial microsomia: assessment of classification systems.

Hemifacial microsomia (HFM) represents a spectrum of congenital malformations involving embryological derivatives of the first and second branchial arches. The multiple anomalies that may exist in HFM present considerable variability in patients with the diagnosis. Numerous classification systems have been devised to facilitate the individualized components of this complex condition. Because multiple HFM classification systems exist, their utility is frequently confusing and selection of a particular system is indeterminate. Several of the better known systems are evaluated using patients with variable expressivity of HFM. This review suggests that there currently exists no ideal classification system for this association of developmental malformations. The systems that were found to be most complete are acknowledged and recommendations or modifications are suggested.

Abnormalities, Multiple↗

[Recessive dystrophic epidermolysis bullosa. Oral manifestations apropos of 3 cases].

Epidermolysis Bullosa (EB) constitutes a group of genetic disorders characterized by bullae and vesicle formation on the skin and mucosa. Three cases of recessive EB that may be clinically classified as recessive dystrophic EB (RDEB) are described. In two cases dental treatment consisted of extractions and restorative therapy under local anesthesia. In all three cases individual home and professional preventive programs were established and no new decays were observed during the next years. Dental management under local anesthesia is emphasized by the authors.

Child↗

Rehabilitation of a patient with limited oral opening following glossectomy.

Rehabilitation of the glossectomy patient is one of the most difficult and challenging problems for the prosthodontist and speech pathologist. The tongue plays an important role in articulation, control of secretions, formation of a bolus, propulsion of the bolus toward the pharynx, clearing the palate, and initiation of the swallow reflex. This paper presents speech and prosthodontic considerations for a patient who underwent a glossectomy and had the additional problem of reduced oral opening.

Aged↗

[Three-dimensional morphological analysis for craniofacial deformity].

Morphological analysis of the craniofacial deformity has been performed by cephalometric radiography, but even with this method, it is difficult to analyze the cases with complicated deformity. The purpose of the present study is to develop the new 3-D CT morphological analyzing system in order to investigate complicated craniofacial deformity. Using the 67 landmarks, we established the 3 D line drawing method. Then the reproducibility of the landmarks were examined and the clinical cases were investigated. The measurement of landmarks were carried out 10 times by the same operator, and standard deviation of 67 landmarks were calculated. Some landmarks which were difficult to identify showed high value of standard deviation. However, it was almost same value between standard deviation of most of the landmarks and the CT system error. Therefore, location of landmarks in the system were found to be reproducible. This new analyzing system was performed in clinical cases with craniofacial deformity. In such cases, cranium deformity and complicated deformity such as twist-form arrangement of cranium, maxilla and mandible were detected. The 3-D CT morphological analysis is effective for analyzing severe craniofacial deformity which was impossible to recognize with conventional cephalometric analysis.

Cephalometry↗

[Hemifacial microsomia: development, diagnosis and surgical correction].

Hemifacial microsomia is the second most common congenital facial anomaly. It occurs as a spontaneous event during embryogenesis and involves structures derived from the first and second branchial arch. In the past treatment of the skeletal defect was delayed until the child ceased growing and end-stage of the deformity was reached. More recent approach to analysis and treatment of this anomaly, emphasizing early treatment, to give the patient the maximum benefit of a combined surgical and orthodontic therapy, is described.

Branchial Region↗

[Orthodontic and maxillofacial confrontation of a hemifacial microsomic mandible, comprising electromyographic check].

A rare case of hemifacial microsomia of the mandible whose etiological entity is delineated from the bad posture of the foetus in the womb during the foetal period, is described. In this case, the combination of orthodontic and maxillofacial treatment, gave satisfactory results as regards the aesthetic problem of the person, the occlusal disturbances and the function, of the masseter and the temporalis-muscles. These functions were checked before, during and after the treatment by means of electromyograms, and a more balanced activity of these muscles could be detected after the combined treatment.

Adult↗

[Combination of plastic and orthognathic procedures, for the surgical correction of the hemifacial microsomia].

This syndrome is made up of a constellation of congenitally malformed facial structures which arises from the first and second branchial arches. A case of hemifacial microsomia, with the physical characteristics of unilateral left underdevelopment of the external and middle ear, mandible and zygoma is presented. The treatment took place in two stages: Firstly the auricle was reconstructed with the method of tissue expander and silicone frame and secondly the mandibular prognathism and laterognathism with sagittal splitting was corrected. We present our case and the results that have been gained by combining the modalities of plastic and orthognathic surgery.

Adult↗

Anthropometry of the face in lateral facial dysplasia: the unilateral form.

The skull, face and ears of 46 patients with the unilateral form of lateral facial dysplasia were examined at the Hospital for Sick Children, Toronto, using anthropometric methods for the purpose of contributing to the morphology of this syndrome. The classical picture of unilateral facial asymmetry with microtia was found in 13 cases (28.3%). The study showed bilaterally defective mandibular arc measurements in 28 per cent of the cases. The facial damage was associated in 15 per cent with ears normal in size but defective in location, level, and shape. The anthropometric examination revealed a great variety of defects on both sides of the face, often barely detectable by routine inspection. It was also found that great deficiencies in the surface measurements were accompanied by great bone deficiencies.

Adolescent↗

A new surgical procedure for correction of lip deformity in cranio-carpo-tarsal dysplasia (whistling face syndrome).

A child with cranio-carpo-tarsal dysplasia or whistling face syndrome is described together with a new surgical procedure for the correction of the mouth deformity. The syndrome is rare; only approximately 40 cases have been described in the literature since 1938. Correction of the facial appearance is of great importance to the affected individuals because of emotional problems they may develop as a reaction of their anomalies. Although the majority of cases have been sporadic, the syndrome can be transmitted as an autosomal dominant trait. Therefore, genetic counseling for affected persons should be offered.

Carpal Bones↗

Craniocarpotarsal dysplasia: the whistling face syndrome.

Currently, 50 cases of craniocarpotarsal dysplasia, or whistling face syndrome (WFS), have been reported, with more than 60 anatomical anomalies involving the head, hands, and feet, in addition to the face. In spite of the fact that there is much information concerning the surgical correction of many of these deformities, such as the surgical program for the hands outlined by Call and Strickland in 1981, there is little information concerning the surgical correction of facial deformities. In view of this, we present here a case report of a patient with WFS and our surgical approach to the facial deformities involved. Since January 24, 1983, when this 7-year-old girl first came into our care, she has successfully undergone a total forehead reshaping, correction of congenital upper eyelid ptosis, and a bilateral commissuroplasty. Follow-up for more than three years has shown stable surgical results.

Abnormalities, Multiple↗