Dental maturation in children with the syndrome of crouzon and apert.

Purpose : Developing teeth are used to assess maturity and estimate age in a number of disciplines. The purpose of this investigation was to study the dental maturation in children with Crouzon or Apert syndrome compared with nonsyndromic controls. Patients and Methods : Records of 40 children with Crouzon syndrome (18 boys and 22 girls, aged 4.0 to 17.9 years) and 28 children with Apert syndrome (10 boys and 18 girls, aged 3.9 to 15.1 years) were referred to the Department of Orthodontics, Cleft Palate Team and Craniofacial Team, Erasmus MC-Sophia. Data from syndromic children were compared with data from 451 nonsyndromic children (225 boys and 226 girls, aged 2.9 to 16.9 years). From panoramic radiographs, dental maturation was determined for patients with Crouzon and Apert syndromes and compared with data collected from control children. Logistic functions were constructed for dental maturation over time for syndromes and gender. Results : Statistically significant gender differences in dental maturation scores were found for girls with Crouzon (P < .05) and Apert syndrome (P < .05). Patients with Apert syndrome demonstrated a significantly delayed dental maturation (P < .05), while patients with Crouzon syndrome showed a nonsignificant delay. Conclusions : Dental maturation in patients with Apert syndrome was more delayed than in patients with Crouzon syndrome. The delay of tooth formation in patients with Crouzon or Apert syndrome suggests a possible common genetic association.

Patterns of tooth agenesis in patients with crouzon or apert syndrome.

PURPOSE: Dental agenesis is the most common anomaly of dental development and can be a component of a congenital syndrome. The purpose of this study was to evaluate the prevalence of agenesis and to describe patterns of tooth agenesis in patients with Crouzon or Apert syndrome compared with nonsyndromic controls. PATIENTS AND METHODS: Longitudinal records of 67 patients with Crouzon syndrome (n = 39) or Apert syndrome (n = 28) from the Erasmus Medical Centre were examined. Syndromic patients were compared with patients in a nonsyndromic control group (n = 284). RESULTS: Prevalence of tooth agenesis in patients with Crouzon syndrome (35.9%) and patients with Apert syndrome (46.4%) was significantly higher than the prevalence in control subjects (27.5%) (P < .005). In all groups third molars were the most likely to be agenetic. Tooth agenesis excluding third molars was significantly higher in syndromic patients than in control subjects (P < .001). Bilateral agenesis of mandibular second premolars occurred significantly more often in patients with Crouzon and Apert syndrome than in control subjects (P < .001). CONCLUSIONS: Tooth agenesis is more prevalent in patients with Crouzon or Apert syndrome than in control subjects. Tooth agenesis and mandibular symmetrical patterns of second premolar agenesis are more prevalent in syndromic patients.

A longitudinal study of dental arch morphology in children with the syndrome of Crouzon or Apert.

The aim of this study was to compare changes in dental arch morphology between patients with Crouzon syndrome or Apert syndrome and controls. Children between 4 and 14 yr of age with Crouzon syndrome (n = 40) or Apert syndrome (n = 28) were compared with non-syndromic controls (n = 457) in terms of arch widths, depths, and length dimensions. Multilevel statistical modeling techniques were used to evaluate changes over time. Dental arch dimensions were found to be smaller in patients with Crouzon syndrome or Apert syndrome compared with control subjects. Maxillary intercanine width for patients with Apert syndrome were increased, whilst other arch width variables showed no change. Patients with Crouzon syndrome showed increases in maxillary intercanine width, whilst intermolar width showed no change over time. Dental arch dimensions in syndromic patients were thus found to be consistently smaller than in control subjects between 4 and 14 yr of age, implying that patients with Crouzon syndrome and Apert syndrome had a diminished growth potential.

Craniofacial Stability in Patients With Crouzon or Apert Syndrome After Le Fort III Distraction Osteogenesis.

Objective : Le Fort III osteotomy with distraction osteogenesis (DO) is used to improve the retruded midface in patients with Crouzon or Apert syndrome. This study aimed to evaluate sagittal and vertical preoperative and postoperative cephalometric changes of DO of the midface in patients with Crouzon or Apert syndrome. Design : Population-based case-control study. Patients and Methods : Records of patients with the syndrome of Crouzon (N = 6) or Apert (N = 7) were compared, before and after Le Fort III DO, with a nonsyndromic untreated control group (N = 486). Main Outcome Measures : Sagittal and vertical cephalometric maxillary landmarks and measurements were used to predict and measure midface advancement and rotation after Le Fort III DO. Cephalograms were taken before surgery (T0), 4 months after surgery at removal of the distraction device (T1), and 1 year after removal of the distraction device (T2). Analysis : Z scores were performed to compare cephalometric measures of syndromic patients with control subjects. Results : Cephalograms of 13 patients with Crouzon syndrome (N = 6) or Apert (N = 7) (age range 8.2 to 19.8 years) were evaluated. Treatment changes (T1-T2) showed statistically significant maxillary advancement, with no significant differences between the patients with the Crouzon or Apert syndrome. Conclusions : DO of the midface in patients with Crouzon or Apert syndrome seems to be stable in the sagittal direction after follow-up. Although Crouzon and Apert differ after DO, anteroposterior craniofacial dimensions were significantly improved and were closer to patterns of normal subjects.

Facial growth in patients with apert and crouzon syndromes compared to normal children.

OBJECTIVE: To evaluate vertical and sagittal facial growth in children with Apert and Crouzon syndromes and compare it to the growth patterns of a nonsyndromic control group. DESIGN: Case-control study. SETTING: Department of Orthodontics, Children's Hospital Erasmus Medical Centre, Sophia, Rotterdam, The Netherlands. PATIENTS, PARTICIPANTS: Sixty-two patients (37 patients with Crouzon syndrome and 25 patients with Apert syndrome) born between 1971 and 2001 (age range 3.9 to 32 years) and 482 nonsyndromic children as a control group. INTERVENTIONS: Lateral cephalograms performed prior to any midfacial surgery of 62 patients and 482 nonsyndromic children were traced and horizontal and vertical measurements were digitized. MAIN OUTCOME MEASURES: Cephalometric measurements of SNA, SNB, ANB, NSMe, and SN/palatal plane angles and lower facial height ratio. RESULTS: Horizontal measurements for the syndromic groups showed no change in SNA angle during growth. SNA angles were lower in patients with Apert syndrome compared to patients with Crouzon syndrome. The syndromic groups showed smaller values for ANB angles compared to the nonsyndromic group. Vertical measurements showed increased lower facial height ratios for the syndromic groups compared to control subjects. There was an increasing counterclockwise rotation of the palatal plane in relation to the anterior cranial base in syndromic patients. NSMe angles among the three groups were not significantly different. CONCLUSIONS: Based on the growth differences identified, the sagittal and vertical jaw relationships differ in patients with Crouzon syndrome, patients with Apert syndrome, and control subjects. Syndromic patients show aggravation of midfacial underdevelopment and anterior rotation of the mandible.

Craniofacial morphology of Dutch patients with bilateral cleft lip and palate and noncleft controls at the age of 15 years.

OBJECTIVE: Comparison of craniofacial morphology in bilateral cleft lip and palate patients to that of a noncleft control group at the age of 15 years. DESIGN: A cross-sectional study of cephalometric data. SUBJECTS AND METHODS: Cephalometric records of 41 consecutive patients (32 boys and 9 girls) with nonsyndromic complete bilateral cleft lip and palate born between 1973 and 1987. The patients were treated by the cleft teams of the Erasmus Medical Centre in Rotterdam and the VU University Amsterdam. The control group of normal Dutch subjects was followed in the Nijmegen Growth Study. From this population, mean cephalometric data were used. Differences in cephalometric measurements and other variables were calculated between the bilateral cleft lip and palate group and the control group. RESULTS: Independent-sample t tests indicated that there was a statistically significant difference between the mean of the cephalometric values of the bilateral cleft lip and palate patients and the control group with respect to all cephalometric variables. Pearson correlation coefficients calculated between angle ANB and the number of operators, number of surgical procedures before 15 years of age, and the year of birth were not significant. CONCLUSIONS: Bilateral cleft lip and palate patients treated in the Amsterdam and Rotterdam cleft centers differed significantly from the control group in all measurements. A Class III development due to a less forward positioned maxilla was observed. The vertical measurements indicated a more divergent growth pattern in bilateral cleft lip and palate patients (Ans-Me/N-Me, GoGn-SN, and SN-FFH).

Presurgical orthopedics and satisfaction in motherhood: a randomized clinical trial (Dutchcleft).

OBJECTIVE: To study the effect of infant orthopedics on satisfaction in motherhood. DESIGN: Prospective two-arm randomized controlled trial in parallel with three participating academic cleft palate centers. Treatment allocation was concealed and was performed by means of a computerized balanced allocation method. SETTING: Cleft palate centers of Amsterdam, Nijmegen, and Rotterdam, the Netherlands. PATIENTS: Two groups of infants with complete unilateral cleft lip and palate and no other malformations. INTERVENTIONS: Group IO+ (n = 27) wore passive maxillary plates during the first year of life, group IO- (n = 27) did not. MAIN OUTCOME MEASURE(S): Mean satisfaction scores were obtained from completed questionnaires at 6, 24, and 58 weeks of age. A 4-point scale was used (1 = very satisfactory to 4 = very unsatisfactory). RESULTS: The range of the mean scores for the individual items on the questionnaires for both groups ranged between 1.1 and 2.4. No differences were found between groups. Mothers appear to be satisfied in motherhood, least satisfied with the available time for themselves, and very satisfied with hugging and walking their babies. No differences were found between groups. CONCLUSIONS: Results from the present study show that infant orthopedics, with a passive plate during the first year of life, in children with a unilateral cleft lip and palate has no influence on the mothers' satisfaction in motherhood.

Effect of long-term repeated deflections on fatigue of preloaded superelastic nickel-titanium archwires.

INTRODUCTION: The aim of this in-vitro study was to investigate the changes in force delivery of superelastic nickel-titanium archwires used in combination with a self-ligating bracket system after dynamic fatigue-loading in a 3-bracket model under controlled temperature. METHODS: Samples of 2 superelastic nickel-titanium (active austenitic) wires, a conventional nickel-titanium wire, and a stainless steel wire, all 0.014-in round, were divided into 2 groups: static deflection and dynamic deflection. The static specimens were under a constant deflection of 3.0 mm. The dynamic specimens had the same constant deflection of 3.0 mm but were subjected to additional repeated deflections of 0.5 mm, applied by a fatigue tester. The test situation simulates a patient's archwire under deflection and subjected to occlusal contact during 1, 100, 10,000, and 100,000 cycles. Fatigue changes were assessed with a 3-point bending test. RESULTS: Type of wire, loading or unloading, and number of cycles as within-subject factors were statistically significantly different. No statistically significant difference between the test condition, static vs dynamic, was found. The repeated deflections of 0.5 mm were not enough to induce an extra effect of fatigue. CONCLUSIONS: Occlusal forces transferred to a considerably deflected archwire, such as in the large malalignments in the early stages of orthodontic treatment, will have no fatigue effect on the unloading force of that archwire.

The overbite and intraoral mandibular distraction osteogenesis.

PURPOSE: The purpose of this study was to quantify the overbite reduction in skeletal Angle Class II malocclusions and discuss the management of the overbite during and after intraoral distraction of the mandible, and during orthodontic treatment with fixed appliances. MATERIAL AND METHODS: Cephalograms of 26 patients with an Angle Class II malocclusion and orthodontic appliances and distraction osteogenesis of the horizontal part of the mandible before (T0) and at least 1 year after treatment (T1) were evaluated. Mean age of the patients at the time of distraction was 14.6 years (range 12.8-15.9 years) and at the final registration 17.3 years (range 14.6-20.4 years). RESULTS: The overbite decreased significantly and the SpP/MP value increased significantly. The increase in the value of the Y-axis and the MP/SN angle before and after treatment was statistically insignificant. CONCLUSION: Opening of the bite during distraction of the mandible can be expected. The use of the 'floating bone' technique did not correct the overbite permanently. The patients in whom an open bite has already been treated are not ideal for mandibular lengthening by means of distraction osteogenesis.

Masticatory muscle right-left differences in controls and hemifacial microsomia patients.

One aim of this study was to assess right-left differences in the volume of masticatory muscles in controls so as to be able to distinguish between normal variations and pathological differences. The other aim was to compare the masticatory muscle volume of the nonaffected side of hemifacial microsomia patients with the smaller side of controls so as to test the compensation hypothesis. The study group consisted of 39 children with hemifacial microsomia and the control group of 52 children. Computed tomography scans were made using a Philips Tomoscan 350 and a Pro Speed S Fast Spiral General Electric scanner. The normal right-left differences in volume of the masticatory muscle of the controls, calculated as a percentage of the total, demonstrated small mean differences of 2.57% to 2.88% (SD:1.98-2.74). For hemifacial microsomia patients, the relative difference was about 10 times the difference for controls. For controls, the averages of the right-left differences (%) were all below the generally accepted SEM of 4%. Testing the compensation hypothesis of all effects, only the age effect was multivariately significant (P < 0.001). No overcompensation of the masticatory muscles of the nonaffected side of hemifacial microsomia patients could be demonstrated.

Perception of symmetry in the face.

One purpose of this study was to assess the perception of (a)symmetry of the face by professionals, lay persons, and sculptors using the ratio estimation scale technique. The other purpose was to compare the perceptions of the faces of patients with those of the controls. Three groups of examiners were involved in this study: lay persons (n = 37), professionals (5 orthodontists and 11 surgeons, n = 16), and sculptors (n = 22). The total number of examiners was 75. All groups agreed on the ordering of the patients according to the severity of the affliction. The stage of asymmetry by group identity interaction effect (lay persons, professionals, and sculptors) was significant (P = 0.004). The between-examiner consensus was high for the original asymmetrical photograph, but the groups diverged further and further over the next steps. The subject identity by stage of asymmetry interaction effect was significant (P = 0.001), whereas the examiner's main effect was not. For all patients, each step resulted in a smaller need for treatment. A need for treatment was not found for any of the control subjects, with 1.5 being the highest need for treatment score for controls. The subject identity effect (patient/control) was significant (P < 0.001). Even at step 5 (the reconstructed symmetrical photograph), all patients were perceived to have an inferior appearance to that of control subjects. It is possible to obtain reliable assessment using the ratio estimation scale technique. Professionals, lay persons, and sculptors have different perceptions of the degree of asymmetry but not of the need for treatment. All patients were scored as asymmetrical compared with controls. Asymmetry of the patients was obvious for all the examiners.

Longitudinal results of mandibular distraction osteogenesis in hemifacial microsomia.

The aim of this study was to evaluate mandibular distraction therapy by three-dimensional (3-D) computed tomography (CT) imaging so as to be able to improve the treatment results. The study group consisted of eight children (3 male and 5 female) with hemifacial microsomia. For each child, CT scans of the head were available (Pro Speed S Fast Spiral scanner; General Electric). Longitudinal measurements of the mandible, bony and soft tissue 3-D reconstructions, and masticatory muscles were demonstrated. Three-dimensional CT scans provide important data concerning the results of mandibular distraction therapy and should be used in treatment evaluation. In some patients and for some muscles only, a small increase in the volume of the affected side of the masticatory muscles in comparison to the normal side was found 3 years after mandibular distraction. In around 50% of the cases, there seems to be a relapse occurring 1 year after distraction osteogenesis, and this relapse has a progressive character when seen 3 years after distraction osteogenesis in comparison to 15 weeks after distraction osteogenesis.