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Clinical and radiographic experience as well as histological findings leave no doubt that the term "condylar hyperplasia" refers only to hyperplasia of the condyle alone and should therefore not be used to mean the two hemimandibular anomalies as is the case in the literature today. There are two basically different malformations of one side of the mandible which we call hemimandibular hyperplasia and hemimandibular elongation respectively. We are convinced that there exist pure and mixed forms of both growth anomalies because we have observed several such clinical cases. The stimulus for the abnormal growth either lies within the fibrocartilaginous layer or is produced by it. Different histological patterns within the condylar growth zone were seen in the two anomalies. The pathophysiological bases of the abnormal growth are discussed. They seem to contribute to the understanding of the normal and abnormal mandibular growth and consequently also of many of the mandibular anomalies. The explanations are demonstrated by the illustrations of some cases.

This study was designed to establish the extent of neuromuscular abnormalities in different types of hemifacial microsomia and to relate these findings to the existing skeletal malformations. Neuromuscular recruitment patterns were established in twenty-three normal subjects. These patterns served as baselines for comparison with recruitment patterns obtained from forty-eight subjects with hemifacial microsomia. The anterior and posterior temporalis, masseter, suprahyoid, buccinator, platysma, mentalis, and upper and lower lips were studied electromyographically. Consistent presence or absence of recruitment for a given task was considered a significant finding with which the hemifacial microsomia data were compared. The subjects with hemifacial microsomia were divided into five groups according to type of mandibular malformation. Abnormal recruitment was found in all recorded muscles except the lips, mentalis, and buccinator muscles. There were variations in recruitment patterns within each group as well as among the groups. A relationship was found between the development of the gonial angle area and the recorded masseter activity. Muscle activity was recorded in the area of the temporal muscle, even in the absence of a coronoid process. It was found that after insertion into a new coronoid process the temporal muscle developed mass and palpable contraction. This study shows that in persons with hemifacial microsomia certain neuromuscular patterns may differ from the norm because of missing or underdeveloped muscles and because of the different relationship between the mandible, its attached muscles, and other structures.

Twenty craniofacial indices composed of 26 surface measurements taken directly from the head and face were determined in 14 Apert's syndrome patients 18 days to 5 years old (younger subgroup) and 14 patients ages 6 years to 15 years old (older subgroup). All of the patients were North American Caucasians who had undergone early suture release but no facial repair. The indices were compared with those in healthy controls of the same age and sex. The wide intercanthal distance in relation to the narrow soft nose was the most frequent (81.5%) and extensive (17.7% above the maximum normal index value) disproportion. Abnormal indices occurred most often with the combination of one abnormal and one normal measurement (61.0%). All seven of the 16 disproportions seen in both age subgroups increased in frequency nonsignificantly with age: the supernormal cephalic, intercanthal, nasal, and vertical mandibulofacial indices and the subnormal nasofacial, upper face, and jaws' arcs indices. Of the six disproportions that decreased in frequency with age, four changed significantly (the supernormal frontoparietal and frontozygomatic indices and the subnormal mandibulofacial and nasozygomatic indices) and two changed nonsignificantly (the supernormal intercanthoalar and subnormal cheilozygomatic indices). With the exception of two nasal proportions, the extent of the disproportionality decreased in all of the indices that increased with age.

Three cases of gross craniofacial polyostotic fibrous dysplasia are presented, together with a brief review of the condition, and pertinent points regarding the cases are discussed. As complete excision of the lesion and immediate reconstruction is rarely feasible or possible, and partial excision may result in accelerated growth of the lesion during the patient's active growth phase, resection only to protect, maintain or restore certain important functions (e.g. vision) during this period are advocated, after which close follow-up is mandatory until the lesion becomes quiescent, when further surgical procedures may be undertaken.

Unilateral electrolytic lesions were produced in the left trigeminal motor nucleus (TMN) of six guinea pigs at 49 days of age. Masticatory mandibular movement and EMG data were collected prior to lesioning and at 4 and 12 days postlesion. After the animals were killed 60 days postlesion, dissection and maceration revealed muscular atrophy and craniofacial asymmetries on the lesion side. Analyses of prelesion and postlesion mandibular movement and EMG data indicated significant (p less than 0.05; p less than 0.01) changes in chew cycle durations, dimensions, and EMG activity patterns. Shifts in EMG durations of working- and balancing-side muscles were strongly related to most of the observed skeletal asymmetries. Data indicated that muscular paresis and altered neuromuscular activity can effect skeletal changes in the mammalian craniofacial complex; total muscular paralysis is not a prerequisite for profound morphologic changes. Altered manifestation of masticatory central pattern generators within the central nervous system may also account for variations in craniofacial form and function.

Patients with Treacher Collins syndrome were studied regarding 10 nasal, 1 orbital, and 4 facial measurements as well as 10 facial proportion indexes. Data from 15 to 24 patients were related to normal values. The most normal feature was found to be the nose, whose basic measurements were optimal. Because harmony between the nose and the face is a basic requirement of aesthetic balance in a healthy face, the nose in these patients is the key for calculating changes in the markedly damaged general framework of the face. Abnormal proportion indexes must be corrected by adjusting the abnormal measurement to its optimal level. Thus, the abnormally short bizygomatic width had to be increased by a mean of 13 to 21 mm. Both the nose and the face were usually normal-long, producing acceptable proportions in these patients. Some size disproportions were found between the root and the soft nose. Their adjustment should be part of the general plan to correct the face of a patient with Treacher Collins syndrome.

A case of true hemifacial hyperplasia is described. This is an unusual condition which produces facial asymmetry by a marked, unilateral, localised overgrowth of all the tissues in the affected area, including, the facial soft tissues, bones and teeth. The patient is an eight-year-old Caucasian girl with congenital hemihyperplasia of the right side of her face.

A particular characteristic of dentomaxillary dysharmony, probably the most frequently encountered anomaly in dentofacial orthopedic practice, is often the result of the presence of physiologic facial asymmetry. Attention is drawn to a syndrome, which could be thought to be paradoxical, and which associates facial asymmetry affecting the left side and dentomaxillary dysharmony that is curiously more marked on this same side.

A boy born at full-term died after 14 days from cardiac failure. At autopsy DiGeorge complex was diagnosed. The father was found to have facial dysmorphia and hypocalcaemia. Investigations revealed no cause other than hypoparathyroidism associated with normal serum 1,25-dihydroxyvitamin D concentrations and normal renal handling of phosphate. Immunological tests, performed on two occasions with an interval of 9 months, revealed a decrease in the number of CD8+ lymphocytes, compatible with a partial thymus deficiency. The combination of facial dysmorphia with dysfunction of the thymus and the parathyroid glands can constitute a partial DiGeorge complex. The findings in this family are compared with reports of four other families with DiGeorge complex in two generations. In genetic counseling DiGeorge complex should be considered a heterogenous disorder. Screening of the parents for somatic stigmata, hypocalcaemia, disturbed cellular immunity, cardiac and chromosomal abnormalities is essential.

A number of cephalometric analyses are presently being used in the assessment of dentofacial deformities. These cephalometrics are mostly based on hard tissue assessment alone, although a few methods using soft tissue only or partially hard and partially soft tissues exist. Most of the analyses use angular and linear measurements, although some are based mainly on measurements of relationships. When the various cephalometric analyses are compared, considerable inconsistency comes to light; so much so, that cephalometrics sometimes cannot be considered as a primary diagnostic tool. A combination of two relationship analyses, one based on soft tissue assessment and one based on hard tissue assessment, incorporating the craniofacial complex, is presented to provide a higher degree of diagnostic accuracy. This combination analysis is based on only a few critical hard tissue landmarks of the cranial base that are used for the total assessment of the facial hard, dental, and soft tissues. This has eliminated inappropriate landmarks and lines that existed in each of the original analyses. The cephalophotometric and architectural-structural craniofacial analyses have been adjusted accordingly and renamed the profilocephalometric analysis.

A case of hemifacial microsomia is reported in association with rare cardiac, lung and laryngeal abnormalities. Our case presents both atrial and ventricular septal defects, hypoplasia of the right lung and of the larynx. This variety of malformations is rarely present in other reported cases. The literature of hemifacial microsomia is reviewed and differential diagnosis is discussed.

A three-dimensional, multiplane cephalometric analysis is presented. This analysis permits visualization of skeletal midlines at selected depths of the craniofacial complex. When the midlines and associated anatomic structures are studied sequentially, the individual midlines may be combined conceptually into a warped midsagittal "plane." This localizes craniofacial asymmetry in the posteroanterior and basilar views. The study of structures in various coronal and transverse planes makes it possible to measure and record the three-dimensional relationships of anatomic structures to one another. A case of hemicraniofacial microsomia in which this analysis was used is presented.

Hemifacial microsomia is variable with respect to the degree of the deformity it presents, thus, treatment varies. Mild degrees of the condition are usually treated adequately by waiting until adolescence, when definitive surgical correction of any skeletal or soft tissue asymmetry can be dealt with. Some mild and moderate cases may benefit from orthodontic functional appliance therapy during growth, but orthodontic opinion is divided on the value of such therapy. Children with severe forms of hemifacial microsomia are improved in appearance during their school years by early surgery performed in the preschool period. A number of surgical approaches have been used at this stage. The technique currently being used by the author involves composite transfer of vascularized soft tissue for contouring, together with a vascularized costochondral strut to lengthen the affected side of the mandible. Early results using this method are satisfactory, but further work is required to assess the advantages of this over other techniques.

This is a study of normal children, evaluating the degree of subtle asymmetry that can be expected in all of us. Asymmetry was found to be very common, but average differences between right and left measurements were mild (3 mm or 3%), with the right side usually the largest. The most common and largest asymmetries were found in the upper third of the face.

Changes have occurred in the original concepts which led to the current use, either totally or partially, of the various cephalometric tests, and the terminology employed varies from one author to another. A comparative study of the principal measurements used was therefore conducted, based on this acquired knowledge. After discussing the relative values of the reference points and constructions, the authors attempt to establish a relationship between the different evaluation methods used for each element, bony, alveolar, and dental, which are necessary for diagnosis, and for which functional coexistence dominates the prognosis for harmony of the buccodental system.

Very little information is available in the literature regarding the morphology and growth of the cranial base, the orbits and the jaws, in patients with premature craniosynostosis. The present report deals with the craniofacial morphology and growth in plagiocephaly and Crouzon syndrome. For each anomaly a dry skull was compared with a clinical case followed longitudinally with roentgencephalometry. Metallic implants in the jaws were employed in one case. Cranial base involvement was demonstrated for both types of premature craniosynostosis. In Crouzon syndrome the cartilagenous cranial base was affected. Marked compensatory and dysplastic growth changes were observed in the facial skeleton in both anomalies. The methodological problems related to roentgencephalometric examination of patients with plagiocephaly are discussed, and a roetgencephalometric method for evaluation of exophthalmos in patients with Crouzon syndrome is presented.

The width and the length of the face in frontal view as important factors in facial harmony are analysed. Two groups of patients with mandibular prognathism were photographed, analysed and evaluated. The lower third of the face generally becomes shorter after a sagittal splitting operation as well as after a sagittal splitting plus additional medial ostectomy of the lower incisor region. In both groups we did not find a significant change in the width of the face. The impression of broadening the face by the sagittal splitting method is therefore subjective. In both groups, the face became more harmonious indimension following the osteotomies.

An anesthesia induction technique not widely utilized is described for a pediatric patient presenting with severe hemifacial hypertrophy, severe mental retardation, as well as a seizure disorder and other congenital anomalies. The anatomically and/or pathologically altered airway as well as other anomalies increase the risks of anesthesia.

True hemihypertrophy involves not only the soft tissues of the body but the hard tissues as well. It may be classified as being total or partial. Total, if an entire side of the body is involved and partial, if only a portion. This report presents 2 cases of true hemifacial hypertrophy in an adult aged 29 years and a child of 12 years. Three-stage reconstruction is described in the older patient.

Various attempts to describe the skeletal characteristics of unilateral craniofacial microsomia have been made with the use of cephalometric and panoramic roentgenograms. Previous studies have been only descriptive in nature. To date, a detailed (quantitative) cephalometric analysis of the mandibular deformity has not been reported. The purpose of this study was to describe the skeletal jaw deformity by means of cephalometric landmarks in the lateral view. The patient population consisted of sixteen boys and eight girls who ranged in age from 6 to 16 years. They were compared to the University of Michigan normal control population for the following measures: gonial angle, mandibular plane angle, overall oblique length of the mandible, ramal height, and body length. The affected side showed a larger gonial angle and mandibular plane angle. The oblique length of the mandible (Cd-Gn) was decreased on both sides, as were ramal height and body length. Paradoxically, body length appeared shorter on the unaffected side than on the affected side. This paradoxical observation could be attributed to a shift of the mandible in relation to the midsagittal plane of the cranial base, the film cassette, and the path of the x-ray beam. Observation of the mandible in the basilar cephalogram explained the geometry of the projection error found in the lateral view. Similar projection errors exist for patients with other types of craniofacial asymmetry. It is suggested that two radiographic views, orthogonal to each other, should be used to define the x, y, and z planes for studies of craniofacial abnormality.

Craniofacial fibrous dysplasia occurring in a mother and two of her three daughters is reported. In all three patients, the condition was characterized by extensive bilateral and bimaxillary involvement. All patients exhibited radiographic evidence of abnormal maxillary and mandibular bone and tooth displacement. Morphologic examination of deciduous teeth from the daughters showed replacement of normal pulp tissue by fibro-osseous lesion. Because of the familial occurrence of the cases, it is proposed that they comprise an example of inherited craniofacial fibrous dysplasia.

Four patients with neurofibromatosis are described: all displayed the feature of an increased depth of the sigmoid notch probably due to an expansive growing neurofibroma of the inferior alveolar nerve. One patient had a local neurofibroma with an invasive growth pattern and only this patient shows hyperplasia of the bone. The suggestion is made, contrary to what is found in the literature, that only the invasive growth type gives rise to hyperplasia and this type is always concomitant with a localized form of neurofibromatosis.

A patient with pulsating exophthalmos is presented. He had facial asymmetry, multiple cutaneous café-au-lait spots and a subcutaneous nodule, biopsy of which showed features consistent with neurofibromatosis. Radiographs of the orbit showed a deficiency of the superolateral orbital wall, a feature previously recognized as part of neurofibromatosis. This feature probably occurs as a result of mesodermal dysplasia. The differential diagnosis and management are discussed.

A review of the anatomical changes in patients with various "first arch" syndromes shows that some anomalies (e.g., micrognathia, ear defects) generally appear together. This study tested the hypothesis that the mandible, zygomatic arch, and middle ear ossicles are a developmental field (i.e., when any of these structures is anomalous, the other two will be also). The hypothesis was tested using data from 25 patients with mandibulofacial dysostosis (MFD) and 40 patients with hemifacial microsomia (HFM). Analysis of the pooled data showed that the hypothesis of character association was generally supported. However, the medians suggested that different factors probably played a role in determining how these three anatomical structures were associated in MFD and HFM. Errors in chondrogenesis may have been primarily responsible for the HFM phenotype. Alterations in Meckel and palatoquadrate cartilages would account for the size and shape changes observed in the ossicles and mandible, while changes in cranial base cartilages may explain the changes noted in the zygomatic arch. Since all three structures were equally affected in MFD, it is problematic to use an interference with chondrogenesis as an explanation for the phenotype. We conclude that although the mandible, zygomatic arch, and middle ear ossicles appear to form a "developmental field," the association between structures varies for HFM and MFD. The relatively lesser involvement of the zygomatic arch in HFM than in MFD suggests different pathogeneses for the two diagnostic groups and maybe a useful criterion for judging animal models of HFM.

The noses of 34 attractive young North American Caucasian women were analyzed quantitatively, based on 19 nasal measurements (6 single and 7 paired linear measurements, 3 angles, and 3 inclinations) and 15 craniofacial measurements (10 linear measurements and 5 inclinations) taken directly from the face of the women. The relationship between the nasal measurements was studied in 16 proportion indices and the relationship between the nasal and the other craniofacial measurements in 13 interareal indices. The findings were also compared with those in 21 women with below-average faces. Two types of facial harmony disruption were identified: disharmony, a normal index with a visually apparent failure of proportionality, and disproportion, an index value outside of the normal range. The percentage of disharmonies and disproportions was significantly higher in the group of 21 women with below-average faces. The study revealed a wide variety of "ideal" noses. Only a small portion of the measurements (12%) and proportion indices (7%) were at the mean value. At least two-thirds of the interareal proportion indices were located in the mean +/- 1 standard deviation portion of the normal range. Only about one-fifth of the interareal indices were disharmonious and 2.8% disproportionate. The disproportions were more areal in the attractive faces and more interareal in the below-average faces. The greatest disproportion in the attractive face was the moderately short columella in relation to the tip protrusion and in the below-average face the long nasal bridge related to the upper-lip height. Disproportions were associated with combinations of normal and abnormal measurements, or with two normal measurements of unequal quality, which resulted in a slightly smaller disfigurement. Analysis of ethnic and racial differences showed the soft nose as the main feature of the most characteristic differences. The study revealed that the key to restoration of facial harmony is the renewal of the uniformity of proportion index qualities by elimination disharmonies and/or disproportionate relationships.

A case of bilateral facial microsomia associated with separations of the posterior portion of the maxilla is presented. The X-ray findings revealed hypoplasia of the condyles, absence of the coronoid processes of the mandible, and displacement of the pterygoid processes. Cephalometric analysis revealed a characteristic feature of the facial skeleton a rotation clockwise to the anterior cranial base.

Computer-assisted medical imaging--transaxial computed tomography (CT) scans and three-dimensional surface reconstructions--was used to study the muscles of mastication and their osseous origins and insertions in 24 patients with untreated unilateral hemifacial microsomia (HFM). The relationship between the volume of a muscle of mastication and the shape and size of its origin and insertion in such patients varies widely. Comparison of mean volumes of specific muscles documents a statistically significant decrease among patients who have moderate to marked mandibular dysmorphology as compared with those with minimally dysmorphic mandibles. This study supports the hypothesis that the shape and size of the mandible are related to the muscles that originate and insert upon it. However, the variation among individual patients means that assumptions regarding muscle mass and, in turn, function cannot be made regarding an individual patient on the basis of osseous dysmorphology that has been demonstrated on skull radiographs alone.

The purpose of this investigation was to determine if any significant differences existed with regard to dental arch and facial asymmetries between persons having malocclusions with well-defined asymmetric characteristics, specifically Angle's Class II subdivision malocclusions, and persons having normal occlusions. Secondarily, it was sought to determine the nature of the differences that contributed to the asymmetric occlusal relationship of buccal segments observed in Class II subdivision malocclusions. The sample consisted of 28 subjects in each of the two groups. All possessed a full complement of permanent teeth including first molars. The average age of subjects in both groups was 17 years. Measurements primarily of a linear nature were obtained from posteroanterior and lateral cephalometric radiographs in addition to dental models. Specific measurements were summed to produce various indices of asymmetry. Variables were analyzed with multivariate discriminant analysis. A significant discrimination between the two groups resulted; variables describing asymmetry of the dentoalveolar region of the mandible appeared to be the primary contributors to the difference obtained. Asymmetry of the maxillary dentoalveolar region appeared to make a secondary contribution.

Condylar hyperplasia poses a problem in planning treatment because it is a self-limiting process for some but not all patients. Continued growth creates a progressive deformity that requires condylectomy, whereas an enlarged condyle can be left in place after hyperplastic growth ceases, even if ramus surgery is needed to correct asymmetry. Bone scan with 99mtechnetium phosphate can be used to assist in making the differential diagnosis. In the two patients reported here, abnormal metabolic activity revealed by bone scans supported clinical and historical evidence that the condylar hyperplasia was active and required surgical correction, including condylectomy. The two cases demonstrate different approaches to postsurgical orthodontic treatment, depending on the amount of normal growth expected.

Hemimaxillofacial dysplasia is a newly recognized disorder consisting of unilateral enlargement of the maxillary alveolar bone and the gingiva associated with hypoplastic teeth, facial asymmetry, and hypertrichosis of the facial skin on the ipsilateral side. The overall pattern of abnormalities in both of the patients presented in this article differs significantly from previously reported types of maxillofacial asymmetry. The occurrence of the same abnormalities in two or more unrelated patients suggests, but does not prove, that pathogenesis in both cases may be the same. The present disorder needs further delineation. At present, neither the cause nor the pathogenesis is understood.

This is a case report of a three month old girl with a unilateral facial dysplasia in whom the rudiments of external auditory organs were displaced towards the zygomatic region. The carotid arteriography demonstrated a persistent stapedial artery and absence of some branches of the external carotid artery (the middle meningeal artery and the superficial temporal artery). The autopsy study, undertaken at six months of age, confirmed the presence of the vascular anomaly and severe defects in the auditory organ. There was nerve tissue in the facial mass. This is the first reported case in which an association of a unilateral first arch anomaly and persistent stapedial artery has been confirmed by arteriography.

As improvements in diagnosis, treatment planning, and surgical techniques have evolved it has become increasingly more common to consider simultaneous mobilization of the maxilla and mandible in order to achieve optimal correction of selected dentofacial deformities. In the experiences of the authors, there are patients with specific deformities of the Class III, Class II, Class I, and asymmetry types who can benefit from simultaneous mobilization of both jaws. It is the purpose of this article, the first in a series of three, to detail those specific conditions which warrant consideration for simultaneous mobilization of the maxilla and mandible. In the second article the details of treatment planning for simultaneous mobilization of the maxilla and the mandible will be presented, and in the third article the results of treatment in 100 consecutive cases will be evaluated.

This article describes the dental skeletal and aesthetic features that characterize Mandibulo-facial dysostosis and Hemifacial microsomia. Each patient has been studied with three different cephalometric analysis in the laterolateral projection. The data are fully reported and discussed. Particular emphasis is put on the differences and correlations between the two syndromes from the point of view both of morphology and physiopathology.