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Facial structure and functional findings in patients with progressive muscular dystrophy (Duchenne).

The investigation of 15 patients who have a progressive muscular dystrophy of the Duchenne type (PMD) shows the affection of various groups of muscles of the stomatognathic system, studied by means of electromyography and mechanical force measurements. We observed the following deviations in patients with PMD; transversal overdevelopment and sagittal shortening of the dental arch; reduction of overbite and overjet; sagittal underdevelopment of the cranial, maxillary and mandibular base; retrusion of incisors; concave profile; increase in bizygomatic width; and a difference in time between the attack on the musculi orbicularis oris and that on masticatory muscles. In our study, the activity of the jaw muscles diminished 2 years earlier compared with the perioral muscles. This, and also the enlargement of the hypotonic tongue, causes a transversal expansion of skull and dental arches. This expansion was strongly pronounced in the lower jaw; we invariably observed a posterior crossbite. In contrast to the transversal overdevelopment, we observed a sagittal underdevelopment of skeletal and dental parameters, as well as a retrusion of the incisors. The results of surface electromyography showed the affection of the masseter muscle in patients who had PMD. By means of the Fast-Fourier transformation, we observed a displacement of the median frequency as compared with the lower frequency range.

Adolescent↗

Posttraumatic osteotomies of the jaws.

The increase in road traffic accidents as well as injuries caused by violence has resulted in an increase of fractures of the facial bones. These fractures are not always treated properly. Sometimes they are not treated at all because of their complexity or, more often, because of the associated, mainly neurological, injuries. With the advances in orthognathic surgery a variety of osteotomy techniques have become available. Originally designed to treat congenital deformities, they can also be used to improve posttraumatic malposition of the maxillofacial bones.

Accidents, Traffic↗

[Allergic rhinitis in children].

The diagnosis of allergic rhinitis (AR) in children is mainly based upon history which leads to distinguish between seasonal (periodic or intermittent) pollinic AR and perrenial (permanent or chronic) AR. The consequences of an AR can be severe with major nasal discomfort, repercussion on quality of life and school attendance, and impact on facial bone growth. In addition it can be complicated by asthma. Specific desensitization is the only treatment which may modify the immune response towards allergens. However it must be compared with the other treatments including local or general antihistaminic drugs and intranasal corticosteroids, the choice resting upon the severity of the symptoms, the respiratory repercussion, and the age of the child.

Adrenal Cortex Hormones↗

Development of the parotid gland and its closer neighboring structures in human embryos and fetuses of 19-67 mm CRL.

The fetal development of the human parotid gland was studied by means of serial sections of human embryos and fetuses ranging from 19 mm to 67 mm CRL. Analysis of computer assisted 3-dimensional reconstructions and anatomical drawings leads to the following observations: 1. The parotid gland anlage is found at the most lateral and cranial point of the sulcus buccalis. 2. The location of the orifice of the parotid duct is dependent upon the developmental processes of the fetal skeleton. 3. The anlagen of anatomical structures dominating the parotid bed in the adult are found prior to the enlargement of the parotid gland. 4. The surface of the parotid gland shows impressions of the surrounding structures. 5. There is no evidence that the parotid gland is subdivided into two lobes by the facial nerve.

Adult↗

Airway management of a patient with facial trauma.

Airway management in patients with facial trauma is usually challenging. In this case, we report the airway management of a patient who had multiple midface fractures and a two-inch tree limb implanted into his face.

Adolescent↗

A precision repeat localization head frame for fractionated stereotactic radiotherapy.

An immobilization device was constructed for Fractionated Stereotactic Radiotherapy (FSRT) based on registration of the teeth and facial bones in a single thermoplastic mask system, along with a custom hardened foam pillow for posterior head immobilization relative to the mask. The unit interfaces mechanically with all of our current radiosurgery equipment and can be used with any standard stereotactic planning system. After initial trials to design a reproducible radiographic localization test, we performed a series of daily AP and Lateral port films on 3 patients over five isocenters. Seventy-nine films were reviewed and the maximum deviation in anatomical projection in both sagittal and coronal planes was less than 2 mm, with over 60% of films showing no distinguishable deviations from initial port films. Ninety-three percent of the test films showed a repositioning accuracy of less than 1 mm for all tested structures. We have developed an accurate, non-invasive means of repeat head immobilization that, when properly constructed, can facilitate precise fractionated stereotactic radiation therapy with patient comfort, ease of construction and long term stability.

Humans↗

Craniofacial access to the anterior and middle cranial fossae and skull base.

A profusion of surgical approaches to gain access to the anterior and middle cranial fossa and skull base have been described. An attempt has been made to simplify the position by suggesting a classification of surgical approaches and to describe a standard approach (of at most 4 defined osteotomies, or at least 2) to give a craniofacial approach to the anterior and middle cranial fossae, the infratemporal fossa, the orbit and the superior nasal cavity. The full extent of the osteotomies have been likened to an ancient Corinthian face mask, or to the stylized face-mask worn by the comic book hero, Batman.

Adult↗

Experience with regional flaps in the comprehensive treatment of maxillofacial soft-tissue injuries in war victims.

This article presents our experience with regional flaps in the treatment of facial soft-tissue defects and deformities in 33 patients with various facial injuries from warfare during the period from 1986 to 1999. Thirty-two males and 1 female aged between 8 and 53 years (mean 24.18 years) were treated with facial soft-tissue injuries from high velocity projectiles and varying degrees of associated hard-tissue injuries. Bullets were the most common cause (70%), followed by injuries from shrapnel (21%), land mines (6%), and breech blocks (3%). The perioral region was involved in 15 cases (45%), the midface and cheeks were involved in 13 cases (39%), and the periorbital area was involved in 5 cases (15%). All soft-tissue injuries were treated primarily by debridement and primary closure and by combining, modifying, and tailoring standard regional flap techniques to fit the location of the injury and compensate for the extent of tissue loss. These procedures consisted basically of local-advancement or rotation-advancement flaps, used in conjunction with pedicled fat or subcutaneous supporting flaps, nasolabial, cheek, cervical, Dieffenbach, and Abbe-type flaps. Scar revision, tissue repositioning, and lengthening procedures, such as W, V-Y, Z, or multiple Z-plasty techniques were also used both primarily and secondarily. Revisions and secondary operations were done in 48% of the patients. Initial healing of the flaps was favourable in 76% of the patients. Postoperative discharge from the suture sites was seen in 24% of the patients, but this usually resolved within several weeks using daily irrigation, and these cases underwent scar revision subsequently. None of the soft-tissue flaps sloughed or developed necrosis. Form and function of the soft-tissue reconstructed regions usually recovered within one year postoperatively. The aesthetic results obtained were favourable. None required facial nerve grafting as only the terminal branches were injured in our cases and functional recovery was acceptable. Application of local tissue transfer procedures in our series of facial warfare injuries yielded acceptable tissue form, texture, and colour match, especially when these procedures were used in combination, and tailored to surgically fit the individual case. Moreover, application of these procedures is relatively easy and postoperative morbidity is limited, provided the general condition of the patient is stable, and the surgical techniques used have good indications and flap principles.

Adolescent↗

Anatomic guidelines for the placement of external references for maxillary repositioning.

OBJECTIVE: External reference points, particularly Kirschner pins (K-wire), placed in the region of the nasion have been shown to improve the accuracy of maxillary vertical repositioning. Although no complications associated with this technique have been reported, there is a potential for injury to the anterior cranial fossa or frontal sinus. The purpose of this study was to measure the shortest distance from the nasion to the anterior cranial fossa and from the nasion to the frontal sinus. These measurements were used to establish anatomic guidelines governing safe placement of external reference point pins. STUDY DESIGN: Twenty-seven cadaver heads were sectioned in the midsagittal plane for gross study. Using a Boley gauge, two specific measures were obtained: (1) distance from deepest depression of nasion to the most anterior and inferior projection of the anterior cranial fossa, and (2) distance from nasion to the most inferior aspect of the frontal sinus. All measurements were made in the midsagittal plane. RESULTS: The average distance from nasion to anterior cranial fossa was 16.9 mm (range 13.0 to 20.0 mm) and the smallest distance, 13.0 mm, was seen in two specimens. The average distance from nasion to the frontal sinus was 6.2 mm (range 2.0 to 10.0 mm) and the smallest distance, 2.0 mm, was seen in three specimens. CONCLUSION: Based on our findings, we recommend the following: (1) place pin to a depth of no more than 8 mm into bone, (2) place pin 5 to 10 mm inferior to soft tissue nasion, and (3) place pin in an anterosuperior to posteroinferior direction (i.e., roughly perpendicular to the nasal dorsum). When these anatomic guidelines are followed, one would expect minimal morbidity associated with the placement of ERP pins.

Aged↗

Treacher Collins Syndrome with choanal atresia: a case report and review of disease features.

Treacher Collins Syndrome--or mandibulofacial dysostosis--is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.

Adolescent↗

Parapharyngeal lipoma.

Lipomatous tumours of the parapharyngeal space are rare. A case of lipoma of the parapharyngeal space is reported for its rarity, characteristic radiological findings, and effect on growth of the facial bones.

Child↗

Hypopituitarism following closed head injury.

We describe four young patients (age 19-34 years) with hypopituitarism following closed head injury. The diagnosis was made by demonstration of low basal pituitary hormone levels and dynamic tests showing low pituitary reserve. The time interval between the injury and diagnosis of hypopituitarism was between three weeks and two months demonstrating the difficulty and complexity of making this diagnosis. Three of our patients (all patients suffering from anterior pituitary hormone deficiency) had ACTH deficiency, a condition which may be life threatening if left undiagnosed; these patients also demonstrated central hypothyroidism. Hypogonadotrophic hypogonadism occurred in three of the patients and was treated with hormonal replacement. Diabetes insipidus was the only insult in one of our patients, accompanied other hormonal deficits in two, and did not appear at all in another patient. Information about skull damage was available for three of the patients, and included skull base and facial bone fractures, probably reflecting the extent of injury necessary to cause hypopituitarism. All patients regained normal lives with adequate hormonal replacement therapy.

Adrenocorticotropic Hormone↗

Prevalence and pathogenesis of sleep apnea and lung disease in acromegaly.

Respiratory disorders are common and important complications in acromegaly. Patients suffering from acromegaly display a 1.6-3.3 fold increase in mortality rate, which is due to respiratory disorders in 25% of cases. In these patients, mortality for lung disease is 2-3 fold higher than in the general population. Every portion of the respiratory system may be involved. Deformities of facial bones, edema and hypertrophy of the mucosae and pharyngeal and laryngeal cartilages, enlargement of the tongue and inspiratory collapse of the hypopharinx, all may contribute to respiratory alterations. Nasal polyps, "hormonal rhinitis", changes of the voice and snoring are common occurrences. Though rarely, a laryngocele may ensue. Pneumomegaly is frequently observed and, as suggested by functional studies, might be due to an increased number rather than volume of the alveoli. An obstructive respiratory syndrome caused by mucosal thickening of the upper airways and bronchi is observed in 25% of female and 70% of male patients. The sleep apnea syndrome (SAS) affects 60-70% of acromegalic patients. SAS may be of obstructive, central or mixed type. Obstructive SAS is the prevailing form in acromegaly. It is due to intermittent obstruction of upper airways with preserved activity of the respiratory center, as testified by the remarkable thoracic and abdominal respiratory efforts. The pathogenesis of the central type of SAS is more complex. Narrowing of the upper airways may induce reflex inhibition of the respiratory center. Moreover, increased GH levels and, possibly, defects in the somatostatinergic pathways, may increase the ventilatory response of the respiratory center to carbon dioxide, thereby leading to respiratory arrest. In the mixed type of SAS, the phenomena underlying the other two forms coexist. Oxygen desaturation concomitant with the apneic episodes accounts for the frequent nocturnal wakening and diurnal drowsiness. Among the clinical correlates of SAS, arterial hypertension is of particular interest due to the close correlation existing between the two disorders. Sleep deprivation related to SAS seems per se to favor the appearance of hypertension. Moreover, short lasting hypoxemia may induce prolonged elevations of blood pressure, mediated by decreased endothelial generation of nitric oxide. Thus, since cardiovascular events are the main cause of mortality in patients with acromegaly, it is reasonable to hypothesize that SAS is involved in the reduced life span of these patients.

Acromegaly↗

Current status and future needs in craniofacial imaging.

Imaging is a keystone in understanding and delivery of craniofacial health care. Recent developments in imaging have brought forth many diverse technologies and approaches. This review provides the current status of methods in craniofacial imaging and discusses future needs and directions. New developments in three-dimensional dental and skeletal imaging, as well as three-dimensional facial image acquisition and animation of jaw movement and facial expression are reviewed. Visualization and convergence of the data from these technologies for construction of patient-specific models is described. Insights on how these technologies and approaches could impact research, education as well as the future of craniofacial health care will be discussed.

Computer Simulation↗

The effect of increasing vertical dimension of occlusion on facial aesthetics.

AIM: To investigate the effect of increasing the vertical dimension of occlusion on facial aesthetics. SETTING: General practice. METHOD: Questionnaires were sent to 96 patients who had been treated in the practice during the period of July 1998 to December 2000, resulting in an overall 72% response rate. All these patients had had their occlusal vertical dimension increased. Photographs of patients were taken before, during and after treatment. The questionnaire asked their opinion on the effects of the treatment on their facial features. To obtain an objective view to substantiate the opinions of the patients, a panel of five judges reviewed the before and after photographs and filled in their own questionnaires. RESULTS: Of the patients who responded to the questionnaire, 79.7% said they looked younger after the treatment. The panel thought 81.2% of the patients treated whose photographs they reviewed looked younger. CONCLUSION: Increasing the vertical dimension of occlusion can have far reaching effects on facial aesthetics, not just on the peri-oral areas but on the whole face.

Adult↗