Cephalometric study of the Apert syndrome in adolescence and adulthood.

This paper reports a cephalometric analysis of the craniofacial morphology in adolescents and adults with Apert syndrome. The sample comprised 26 patients with Apert syndrome (15 males and 11 females). The control group consisted of 153 adults (102 males and 51 females). Both lateral and frontal cephalograms were studied. The data were presented as mean plots of the craniofacial region together with data on some of the most significant findings. Marked differences were found in nearly all craniofacial regions except the mandible. The calvaria was increased in height and width but length was decreased. The cranial base showed marked protrusion of the greater wing of the sphenoid, which contributed to severe reduction of orbital volume and protrusion of the eyeglobe. Orbital volume was further reduced by maxillary hypoplasia in all three planes of space together with retrognathia. Maxillary height was extremely short and so was the nose. The width of the nasal cavity, height and depth of the bony nasopharynx, and the nasopharyngeal airway were all markedly reduced in size. The mandible was of fairly normal size and shape but was posteriorly inclined. Head posture was extended in relation to the cervical column. Total facial height was increased, whereas upper facial height was markedly reduced. Incisor occlusion showed mandibular overjet and open bite. Apert syndrome patients were then compared to a group of Crouzon syndrome patients. Marked and significant differences were found between the two syndromes in nearly all craniofacial regions, and craniofacial dysmorphology was generally more severe in Apert syndrome patients.

Iliac versus cranial bone for secondary grafting of residual alveolar clefts.

Secondary bone grafting of the maxilla in the mixed transitional dentition stage has become a well-accepted procedure in the surgical protocol for rehabilitation of patients with residual alveolar clefts. This retrospective study was undertaken to evaluate and compare the long-term results obtained with iliac or cranial cancellous bone graft material in the area of alveolar clefts and was based on the independent experience of two plastic surgeons from the same center using exclusively cranial or iliac cancellous bone, respectively. The criteria for surgery were similar. The surgical technique, with the exception of the bone-grafting material, also was similar, and all patients were treated by the same group of orthodontists. Fifteen patients from each group, from a total of over 100 patients, were randomly selected and included in the study. All patients were followed up from 18 to 60 months. Operative and perioperative parameters, donor-site morbidity, and long-term results were evaluated, compared, and analyzed. There were no significant differences between the two groups, and equally good results in terms of bone incorporation, tooth eruption, and appearance were obtained with both iliac and cranial bone grafts. We conclude from our study that successful bone grafting is primarily achieved by adherence to meticulous surgical technique, simultaneous closure of coexisting oronasal or palatal fistulae, use of cancellous bone particles only, and coverage of the grafts with well-vascularized flaps. The source of bone graft does not seem to primarily influence the success of the outcome.

The role of gingival mucoperiosteal flaps in the repair of alveolar clefts.

Secondary bone grafting of alveolar clefts has become a well-established procedure. However, little attention has been given to the soft-tissue coverage of these grafts. We present our experience with 32 patients in which gingival mucoperiosteal flaps were used exclusively in conjunction with bone grafting for patients with residual alveolar clefts. The indications and timing of the procedure as well as the steps of the technique are presented. The advantages of bone grafting at the stage of mixed dentition and the superiority of gingival mucoperiosteal flaps, including teeth eruption through the graft, additional teeth support, dental hygiene and aesthetic appearance of the alveolar ridge, are discussed. Our results are compared with those obtained with similar or other techniques.

Tooth maturation in cleft lip, cleft palate, or both.

The relation between chronologic age and the development of permanent teeth in patients with cleft lip, cleft palate, or both was investigated according to the method described by Demirjian et al (1973). The patients were divided into three groups: (1) those with bilateral cleft lip and palate (BCLP), (2) those with unilateral cleft lip and palate (UCLP), and (3) those with cleft palate only (CP). Panoramic radiographs of 107 Caucasian children (66 boys and 41 girls) aged 4 to 12 years were evaluated. The degree of maturation of each of the permanent teeth on the left side of the mandible was determined, and a dental maturation score was computed for each child. The scores were compared with those obtained in a previous study of dental maturation in Caucasian children without cleft from the Chicago area (Loevy, 1983). Evaluation of the data demonstrated that there was no significant difference in dental maturity between girls with cleft lip, cleft palate, or both and with the sample of those free from cleft. There was a significant difference in dental maturity in boys when all cleft groups were evaluated together. There was no significant difference from the normal sample in boys with bilateral cleft lip and cleft palate or with cleft palate alone, but there was a highly significant difference from the normal sample in the group of boys with unilateral cleft lip and palate.

Tooth eruption and craniofacial development in congenital hypothyroidism: report of case.

The long-term effects of severe hypothyroidism on craniofacial growth and dental development are illustrated in this case. It is apparent that given a favorable diet, the primary dentition can persist for a long period (early childhood to at least the age of 19) without the development of dental caries. It is also clear that the dental structures can still respond to the effects of L-thyroxine at a relatively late age, with the exfoliation of primary dentition and eruption of the secondary dentition. Impacted mandibular second molars appear to be rare. The lack of proper growth of the mandible and failure of normal resorption of the internal aspect of the ramus associated with deposition of bone on the external aspect with the development of normal-size teeth, resulted in a lack of space for the eruption of mandibular second molars. The impaction of the mandibular second molars in this patient seems to be caused by a dissociation of ramus growth and dental development, resulting in insufficient space for proper eruption of these teeth.

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.

Craniofacial growth data for cleft lip patients infancy to 6 years of age: potential applications.

The present work was undertaken to provide craniofacial roentgenocephalometric growth data on a group of patients with cleft lip ranging in age from infancy to 6 years. Included are variables related to the neurocranium and orbit. Mensurational data were derived from cephalograms of 72 patients (44 male and 28 female subjects) with cleft lip only (n = 38) or cleft lip with varying degrees of alveolar cleft (n = 34). The data in this mixed longitudinal sample are reported for ten age groups. On the basis of comparison with reported noncleft roentgenocephalometric values, it is suggested that the data may serve as a reference for early craniofacial growth. Measurements from four persons with different types of craniofacial anomalies are also presented to illustrate the application of the reported data in the assessment of craniofacial growth.

Form, function, growth, and craniofacial surgery.

The introduction of craniofacial surgery in the early 1970's provided new treatment opportunities for patients previously ineligible for care and increased the number of patients presenting to multidisciplinary teams and private practitioners. Otolaryngologists together with other medical and paramedical professionals began to see patients whose overall phenotype was relatively unfamiliar. Complex craniofacial malformations raised questions concerned with the severity of the malformation process, the effect of growth, and the effect of surgery. For example, how does the anomalous craniofacial skeleton grow? Does the deformity become worse with time? Does it get better? Or does it stay the same? Additionally, how does surgery that is designed to restore form and function affect growth of the craniofacial complex? To answer these questions, this article examines the form, function, and pattern of craniofacial growth in four categories of malformations: cleft lip and palate, hemifacial microsomia, mandibulofacial dysostosis, and two of the craniofacial synostoses, the Apert and Crouzon syndromes. Each of these malformations is amenable to surgery. The timing and effect of surgery on growth are critical to successful habilitation and are discussed as part of the natural history of those anomalies.